2019 Medicaid Preapproval Criteria

Home , Alglucosidase alfa, Idursulfase, Lusutrombopag, Pegvaliase, Xanthoma

ABILIFY MAINTENA ...... 13 ACTHAR HP ...... 14 ACTIMMUNE ...... 16 ADAGEN & REVCOVI ...... 17 ADCIRCA ...... 18 ADEMPAS ...... 19 AFINITOR ...... 21 AFINITOR DISPERZ ...... 22 ALDURAZYME ...... 23 ALGLUCOSIDASE ALFA ...... 24 ALPHA-1 PROTEINASE INHIBITORS ...... 25 AMPYRA ...... 26 ANTIEMETICS ...... 27 ANTIHEMOPHILIC FACTORS ...... 29 ARCALYST ...... 31 ARISTADA ...... 32 ARIKAYCE ...... 33 ATGAM ...... 34 ATRYN ...... 35 AUBAGIO ...... 36 AUSTEDO ...... 37 AVASTIN ...... 38 AVONEX ...... 39 AZEDRA ...... 40 BANZEL ...... 41 BELEODAQ ...... 42 BENLYSTA ...... 43

BESPONSA ...... 44 BETASERON ...... 45 BLINCYTO ...... 46 BONJESTA & DICLEGIS ...... 47 BOSULIF ...... 48 BOTOX ...... 49 BRINEURA ...... 51 CABLIVI ...... 52 CALQUENCE ...... 53 CAPRELSA ...... 54 C1 ESTERASE INHIBITOR ...... 55 CARBAGLU ...... 56 CAYSTON ...... 57 CERDELGA ...... 58 CEREZYME ...... 59 CGRP Inhibitors ...... 60 CHOLBAM ...... 62 CIMZIA ...... 63 CINQAIR IV ...... 66 COAGADEX ...... 67 COMPOUNDED MEDICATIONS ...... 68 CONTINUOUS GLUCOSE MONITORS ...... 69 CORLANOR ...... 70 COTELLIC ...... 72 CRESEMBA ...... 73 CRYSVITA ...... 74 CYSTADANE ...... 75 CYSTAGON ...... 76 DALIRESP ...... 77

DEFITELIO ...... 78 DESMOPRESSIN ...... 80 DIABETIC TEST STRIPS ...... 81 DIACOMIT ...... 82 DOPTELET ...... 83 DUPIXENT ...... 85 ELAPRASE ...... 87 ELELYSO ...... 88 ELIQUIS ...... 89 ELZONRIS ...... 90 ENBREL ...... 91 ENTERAL NUTRITION/ORAL NUTRITION SUPPLEMENTS ...... 94 ENTYVIO ...... 96 EPIDIOLEX ...... 98 EPOPROSTENOL ...... 99 ERGOT ALKALOIDS ...... 100 ERIVEDGE ...... 101 ERYTHROPOIESIS STIMULATING AGENTS (ESAs) ...... 102 EXJADE, JADENU ...... 104 EXONDYS 51 ...... 105 EXTAVIA ...... 106 FABRAZYME...... 107 FARYDAK ...... 108 FASENRA SUBCUTANEOUS ...... 109 FDA APPROVED DRUG – Below the Medicaid Line of Coverage ...... 110 FDA APPROVED DRUG – Not Yet Reviewed By Plan for Formulary Placement ...... 111 FERRIPROX ...... 112 FIRDAPSE ...... 113 FLUCYTOSINE ...... 114

GALAFOLD ...... 115 GILENYA...... 116 GILOTRIF ...... 117 GLATIRAMER ...... 118 GLUCAGON-LIKE PEPTIDE-1 AGONISTS ...... 119 GROWTH HORMONES (somatropin) Injectables ...... 120 ESBRIET ...... 122 EVENITY...... 123 FLUOCINOLONE OCULAR IMPLANT ...... 124 GAMIFANT ...... 126 HEMLIBRA ...... 128 HEPATITIS C DIRECT-ACTING ANTIVIRALS ...... 129 Table 1: Recommended Treatment Regimens for Chronic Hepatitis C...... 132 HERCEPTIN ...... 135 HEREDITARY TYROSINEMIA (HT-1) ...... 136 HETLIOZ ...... 137 HUMIRA ...... 138 HYALURONIC ACID DERIVATIVES ...... 142 HYCAMTIN ...... 143 IBRANCE ...... 144 ICLUSIG ...... 145 IDHIFA ...... 146 ILARIS ...... 147 ILUMYA ...... 149 IMATINIB ...... 150 IMBRUVICA ...... 151 IMPAVIDO ...... 152 INCRELEX ...... 153 INFLIXIMAB ...... 154

INLYTA ...... 158 INTRAVITREAL ANTI-VEGF THERAPY ...... 159 INTRON-A ...... 160 INVEGA SUSTENNA ...... 161 INVEGA TRINZA ...... 162 IMMUNE GLOBULIN ...... 163 ISOTRETINOIN ORAL...... 166 ITRACONAZOLE ...... 167 JAKAFI ...... 169 JETREA ...... 171 JYNARQUE ...... 172 KALBITOR...... 173 KALYDECO ...... 174 KANUMA ...... 175 KINERET ...... 176 KRYSTEXXA ...... 177 KUVAN ...... 178 KYMRIAH ...... 179 KYNAMRO ...... 181 LEMTRADA ...... 183 LETAIRIS ...... 184 LEUKINE ...... 185 LEUPROLIDE ...... 187 LIDOCAINE PATCH ...... 189 LUXTURNA ...... 190 LYRICA ...... 191 MAKENA ...... 192 MAVENCLAD ...... 193 MAYZENT ...... 195

MEPSEVII ...... 197 MIACALCIN ...... 198 MITOXANTRONE ...... 200 MULPLETA ...... 202 MYALEPT ...... 203 MYELOID GROWTH FACTORS ...... 205 NAGLAZYME ...... 208 NATPARA ...... 209 NILANDRON...... 210 NINLARO...... 211 NPLATE ...... 212 NUCALA ...... 213 NUEDEXTA ...... 215 OCALIVA ...... 216 OCREVUS ...... 217 ODOMZO ...... 218 OFEV ...... 219 OLUMIANT ...... 220 ONCOLOGY AGENTS ...... 222 ONFI ...... 223 ONPATTRO ...... 224 OPIOID QUANTITY ABOVE 90 MORPHINE MILLIGRAM EQUIVALENTS (MME) ...... 225 ORAL-INTRANASAL FENTANYL ...... 226 ORENCIA ...... 227 ORENITRAM ...... 230 ORILISSA ...... 231 ORKAMBI ...... 232 OTEZLA ...... 233 OXERVATE ...... 235

PALYNZIQ ...... 236 PARATHYROID HORMONE ANALOGS...... 238 PARSABIV ...... 239 PEGASYS ...... 240 PEGINTRON ...... 242 PERJETA ...... 243 PERSERIS...... 244 PHENOXYBENZAMINE ...... 245 PLEGRIDY ...... 246 POMALYST ...... 247 PROBUPHINE ...... 248 PROLIA ...... 249 PROMACTA...... 251 PROVENGE ...... 253 PULMOZYME ...... 254 RADICAVA ...... 255 RAYALDEE ...... 256 REBIF ...... 257 REMODULIN ...... 258 REPATHA ...... 259 REVLIMID ...... 262 RIBAVIRIN ...... 264 RIBAVIRIN INHALATION ...... 265 RITUXAN ...... 266 RYDAPT ...... 269 SABRIL ...... 270 SELF-ADMINISTERED DRUGS (SAD) ...... 271 SAMSCA ...... 272 SEROSTIM ...... 273

SIGNIFOR ...... 274 SIGNIFOR LAR ...... 275 SILDENAFIL ...... 277 SIMPONI ...... 278 SIRTURO ...... 281 SODIUM PHENYLBUTYRATE ...... 282 SOLIRIS ...... 283 SOMATOSTATIN ANALOGS ...... 285 SORIATANE ...... 287 SPINRAZA ...... 288 SPRAVATO ...... 290 SPRYCEL ...... 292 STELARA ...... 294 STRENSIQ...... 296 SUBCUTANEOUS IMMUNE GLOBULIN ...... 297 SUBLOCADE ...... 298 SUCRAID ...... 300 SUPPRELIN ...... 301 SUTENT ...... 302 SYLVANT ...... 303 SYMDEKO ...... 304 SYMLIN ...... 305 SYNAGIS ...... 306 TACROLIMUS OINTMENT ...... 308 TAFAMIDIS ...... 309 TAKHZYRO ...... 310 TARCEVA ...... 312 TARGRETIN ...... 313 TASIGNA ...... 314

TAVALISSE ...... 315 TECENTRIQ ...... 316 TECFIDERA ...... 317 THALOMID ...... 318 THYMOGLOBULIN ...... 320 TEGSEDI ...... 321 TOBRAMYCIN INHALATION ...... 322 TESTOPEL AND TRANSDERMAL TESTOSTERONE ...... 323 TRACLEER ...... 324 TRIPTROELIN ...... 325 TROGARZO ...... 326 TYKERB ...... 327 TYSABRI ...... 328 TYVASO ...... 330 ULTOMIRIS ...... 331 UNITUXIN ...... 332 VAGINAL PROGESTERONE ...... 333 VALCHLOR ...... 334 VARIZIG ...... 335 VECTIBIX ...... 336 VENTAVIS ...... 337 VERZENIO ...... 338 VFEND ...... 339 VIMIZIM...... 340 VISTOGARD ...... 341 VIVITROL...... 342 VOTRIENT ...... 343 VPRIV ...... 344 XALKORI ...... 345

XELJANZ ...... 346 XENAZINE ...... 348 XEOMIN, DYSPORT and MYOBLOC ...... 349 XGEVA ...... 351 XIAFLEX ...... 352 XIFAXAN ...... 353 XOFIGO ...... 354 XOLAIR ...... 355 XTANDI ...... 357 XURIDEN ...... 358 XYREM ...... 359 YESCARTA ...... 360 ZAFIRLUKAST ...... 361 ZAVESCA ...... 362 ZEJULA ...... 363 ZELBORAF ...... 364 ZINPLAVA ...... 365 ZOLINZA ...... 366 ZONTIVITY ...... 367 ZORBTIVE ...... 368 ZYDELIG ...... 369 ZYKADIA...... 370 ZYTIGA ...... 371

POLICY NAME: ACTEMRA Affected Medications: ACTEMRA (tocilizumab)

Covered  All FDA-approved indications not otherwise excluded by plan design. Uses: Required  Documentation of moderate to severe disease despite current treatment (indication must be Medical documented in chart notes within the last 6 months) Information:  Documentation of complete and current treatment course  Documented current level of disease activity/disease control  Patient weight Rheumatoid Arthritis  Laboratory test confirming diagnosis of rheumatoid arthritis (anti-CCP, RF)  Documented current level of disease activity with one of the following:  The Disease Activity Score derivative for 28 joints (DAS-28) greater than 3.2  The Simplified Disease Activity Index (SDAI) greater than 11  The Clinical Disease Activity Index (CDAI) greater than 10  Weighted RAPID3 of at least 2.3 Giant Cell Arteritis (GCA)  GCA is only approved for subcutaneous (SC) formulation  Diagnosis based on temporal artery biopsy or color doppler ultrasound OR  Large vessel giant cell arteritis (GCA) diagnosis by advanced imaging of the vascular tree with computed tomography (CT), magnetic resonance imaging(MRI), magnetic resonance angiography (MRA), positron emission tomography (PET) or PET with CT

Appropriate Rheumatoid arthritis Treatment  Has failed minimum 12 weeks trial of combination disease-modifying antirheumatic drugs Regimen & therapy: methotrexate 25 mg (IM or SQ) With hydroxychloroquine and/or sulfasalazine, or Other leflunomide Criteria:  Has failed minimum 12 weeks of Humira AND minimum 12 weeks of Enbrel individually  For intravenous request: Treatment failure with subcutaneous route  Dosing: 4 mg/kg once every 4 weeks; may be increased to 8 mg/kg once every 4 weeks based on clinical response (maximum dose: 800 mg)  Polyarticular juvenile idiopathic arthritis  Failure with a minimum 12 weeks of Humira AND minimum 12 weeks of Enbrel individually  IV Dosing – o less than 30 kg: 10 mg/kg once every 4 weeks o 30 kg or greater: 8 mg/kg once every 4 weeks  SC Dosing – o less than 30 kg: 162 mg once every 3 weeks o 30 kg or greater: 162 mg once every 2 weeks

Systemic juvenile idiopathic arthritis  Has failed minimum 12 weeks of Humira AND minimum 12 weeks of Enbrel individually  IV Dosing: o Less than 30 kg: 12 mg/kg once every 2 weeks o 30 kg or greater: 8 mg/kg once every 2 weeks  SC Dosing: o Less than 30 kg: 162 mg once every 2 weeks o 30 kg or greater: 162 mg once weekly

Giant cell arteritis  Documented resistant disease with treatment failure to glucocorticoids  QL- 0.9ml (126 mg) given once weekly. 3 month supply  Reauthorization: Achieving remission or improvement defined as absence or a decrease of GCA signs and symptoms, normalization of erythrocyte sedimentation rate (ESR) (to less than 30 mm/hr without an elevation at least or greater than 30 mm/hr attributable to GCA) normalization of C-reactive protein to less than 1mg/dL and successful adherence to prednisone taper

Cytokine Release Syndrome (CRS)- IV formulation only  For patients less than 30kg, recommended dose is 12mg/kg; patients 30kg or greater recommended dose is 8mg/kg up to maximum of 800mg  Maximum of 4 doses total may be administered

Reauthorization: documentation of treatment success

Exclusion  ANC less than 2,000/mm3, platelets less than 100,000/mm3, or ALT or AST 1.5 times above the Criteria: upper limit of normal  History of GI perforation or diverticulitis  Prior intolerance or allergic reaction to Actemra  Concurrent use of biologic DMARDs such as Orencia (abatacept), Rituxan (rituximab), Humira (adalimumab), Remicade (infliximab), Cimzia (certolizumab), Simponi (golimumab), Kineret (anakinra), Xeljanz (tofacitinib).  Hepatitis B, positive tuberculosis or herpes zoster  Severely uncontrolled hyperlipidemia Age  Rheumatoid arthritis: 18 years or older Restriction:  Polyarticular JIA or Systemic JIA: Ages 2 to 16 Prescriber  Prescribed by or in consultation with a rheumatologist Restrictions: Coverage  Initial approval: 4 months initiation, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: ABILIFY MAINTENA Affected Medications: ABILIFY MAINTENA (aripiprazole suspension, reconstituted) (**Medical benefit only)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Diagnosis of schizophrenia and on maintenance treatment Information:  The patient has received at least ONE of the following: oral aripiprazole (Abilify), Abilify Maintena or Abilify solution

Appropriate Treatment  Documented failure or contraindication to Risperdal Consta Regimen & Other Criteria: Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria: Age Restriction:

Prescriber Restrictions:  Psychiatrist or receiving input from a psychiatry practice

Coverage Duration:  Approval: 12 months, unless otherwise specified.

POLICY NAME: ACTHAR HP Affected Medications: ACTHAR HP (repository corticotripin injection)

Covered Uses:  All FDA-approved indications  Diagnostic adrenocortical function Required  Diagnosis of infantile spasms and the patient is currently receiving treatment with HP Medical Acthar gel and has shown substantial clinical benefit from therapy, OR the patient has not Information: received previous treatment with HP Acthar gel and the patient is less than 2 years of age (If yes, skip directly to exclusion criteria), OR  HP Acthar gel is being requested for diagnostic testing of adrenocortical function and the patient cannot be tested with Cosyntropin, OR  Acthar Gel is requested for serum sickness and the patient had an inadequate response to parenteral corticosteroids, OR  Acthar Gel is requested for rheumatic diseases, used as adjunctive treatment, and the patient had an inadequate response to parenteral corticosteroids, OR  The patient has a diagnosis of nephrotic syndrome, the therapy is being requested for induction of diuresis or for remission proteinuria, and the patient had an inadequate response to parenteral corticosteroids, OR  The therapy is requested for multiple sclerosis (MS) exacerbation and the patient had an inadequate response to parenteral corticosteroids, OR  The patient has Collagen diseases (eg, systemic lupus erythematosus (SLE), dermatomyositis, or polymyositis), Dermatologic disorders (eg, severe erythema multiforme, Stevens-Johnson syndrome), Ophthalmic disorders, acute or chronic (eg, iritis, keratitis, optic neuritis), or Symptomatic sarcoidosis AND the patient had an inadequate response to parenteral corticosteroids. Appropriate  MS exacerbation: Failure to generic oral AND intravenous glucocorticoids Treatment  SLE: Failure to hydroxychloroquine or chloroquine AND generic glucocorticoids Regimen & Other Criteria: Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion  Receipt of live or live attenuated vaccines within 6 weeks of H.P. Acthar Gel Criteria:  Suspected congenital infection (infants)  Scleroderma  Osteoporosis  Systemic fungal infections  Peptic ulcer disease  Ocular herpes simplex  Congestive heart failure  Recent surgery  Uncontrolled hypertension  Known hypersensitivity to porcine proteins 14

 Primary adrenocortical insufficiency or hyperfunction Age Restriction: Prescriber Restrictions: Coverage  Approvals: Duration: Infantile Spasms, Rheumatic Diseases, Nephrotic Syndrome, Collagen Diseases, Dermatologic Diseases, Ophthalmic Disorders, or Symptomatic Sarcoidosis = 6 months, unless otherwise specified Diagnostic Use = 1 dose, (30 days), unless otherwise specified Serum Sickness = 1 month, unless otherwise specified MS Exacerbation = 3 weeks, unless otherwise specified

POLICY NAME: ACTIMMUNE Affected Medications: ACTIMMUNE (Interferon Gamma 1 b)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  FDA approved indication must be documented in the member's chart notes within Information: the most recent 12 months  Patient's body surface area (BSA) must be documented along with the prescribed dose.  Pediatrics with BSA less than 0.5 m2: weight must be documented along with prescribed dose.  Recent CBC with differential and platelet counts, liver function test Chronic granulomatous disease  Patient is on prophylaxis regimen: antibacterial and antifungal Appropriate Reauthorization will require documentation of treatment success and a clinically Treatment significant response to therapy Regimen & Other Criteria: Exclusion Criteria:  Labs outside of normal limits must have documentation of benefit of thearpy outweighing risk (bone marrow toxicity and hepatotoxicity)

 Doses above 50 mcg/m2

Age Restriction: Prescriber  Chronic granulomatous disease: prescribed by or in consultation with a Restrictions: rheumatologist or an infectious disease specialist  Severe, malignant osteoporosis: prescribed by or in consultation with an oncologist Coverage Duration:  Approval = 12 months, unless otherwise specified

POLICY NAME: ADAGEN & REVCOVI Affected Medications: ADAGEN (pegademase bovine)

Covered Uses:  All FDA approved indications not otherwise excluded by plan design.  Treatment of adenosine deaminase severe combined immune deficiency (ADA-SCID) in pediatric and adult patients

Required Medical  A confirmed diagnosis of adenosine deaminase severe combined immune Information: deficiency (ADA-SCID) o Absent ADA levels in lysed erythrocytes o A marked increase in deoxyadenosine triphosphate (dATP) levels in erythrocyte lysates o A significant decrease in ATP concentration in red blood cells o Absent or extremely low levels of N adenosylhomocysteine hydrolase in red blood cells o Increase in 2'-deoxyadenosine in urine and plasma

Appropriate  Documentation showing that neither gene therapy nor a matched sibling or Treatment family donor for HCT (hematopoietic cell transplantation) is available, or that Regimen & Other gene therapy or HCT was unsuccessful AND Criteria:  For Revcovi requests- documentation that treatment with Adagen was unsuccessful

Exclusion  Other forms of autosomal recessive SCIDs Criteria:  All uses not listed under covered uses are considered experimental

Age Restriction:

Prescriber  Immunologist or prescriber experienced in SCID Restrictions: Coverage  Initial Authorization: 4 months, unless otherwise specified Duration:  Reauthorization: 6 months, unless otherwise specified

POLICY NAME: ADCIRCA Affected Medications: ADCIRCA (tadalafil)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.

Required Medical  Pulmonary arterial hypertension (PAH) (WHO Group 1) confirmed by right heart Information: catheterization  Etiology of PAH (idiopathic, heritable, or associated with connective tissue disease)  NYHA/WHO Functional Class II or III symptoms  Documentation of Acute Vasoreactivity Testing (positive result requires trial/failure to calcium channel blocker)

Appropriate  Inadequate response or intolerance to sildenafil citrate tablets (Revatio) Treatment  Subsequent approvals require documentation of treatment success such as Regimen & Other improved walking distance or improvements in functional class Criteria: Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Concomitant nitrate therapy on a regular or intermittent basis  Concomitant use of Guanylate Cyclase Stimulators (eg. riociguat) Age Restriction: Prescriber  Cardiologist or Pulmonologist Restrictions:

Coverage Duration:  12 months, unless otherwise specified

POLICY NAME: ADEMPAS Affected Medications: ADEMPAS (riociguat)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.

Required Medical Chronic thromboembolic pulmonary hypertension (CTEPH) Information:  WHO Group 4 with documented thromboembolic occlusion of proximal or distal pulmonary vasculature and mean pulmonary arterial pressure of at least 25 mmHg at rest in the absence of elevated pulmonary capillary wedge pressure (i.e. PCWP not more than 15 mmHg)

Pulmonary arterial hypertension (PAH)  WHO Group 1 confirmed by right heart catheterization  Etiology of PAH (idiopathic, heritable, or associated with connective tissue disease)  NYHA/WHO Functional Class II to III symptoms  Documentation of Acute Vasoreactivity Testing (positive result requires trial/failure to calcium channel blocker)

 Liver Function Test and creatinine clearance, baseline exercise testing (6MWD)

Appropriate CTEPH Treatment  Documentation of failure of or inability to receive pulmonary endarterectomy surgery Regimen & Other  Current therapy with anticoagulants Criteria: PAH  The following supportive care should be considered: anticoagulants, diuretics, oxygen, digoxin  Failure/Contraindication to the following therapy classes: PDE5 inhibitors AND endothelin receptor antagonists  Efficacy was shown in patients on Adempas monotherapy or in combination with endothelin receptor antagonists or prostanoids

 Safety and efficacy have not been demonstrated in patients with creatinine clearance less than or equal to 15 ml/min or on dialysis  Safety and efficacy have not been demonstrated in patients with severe (Child-Pugh class C) hepatic impairment

 Reauthorization: Subsequent approvals require documentation of treatment success such as improved walking distance or improvements in functional class Exclusion  Pregnancy Criteria:  Concomitant use with nitrates or nitric oxide donors (such as amyl nitrite)

 Concomitant use with specific PDE-5 inhibitors (such as sidenafil, tadalafil, or vardenafil) or non-specific PDE inhibitors (such as dipyridamole or theophylline)  Use in patients with symptomatic pulmonary hypertension associated with in idiopathic interstitial pneumonias (PH-IIP) Age Restriction:

Prescriber  Cardiologist or Pulmonologist Restrictions:

Coverage  12 months, unless otherwise specified Duration:

POLICY NAME: AFINITOR Affected Medications: AFINITOR

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, all prior therapies used, and prescribed Information: treatment regimen Documentation of use with NCCN 2A or higher level of evidence regimen

Appropriate  Documentation of medication review and / or avoidance with strong CYP3A4 Treatment inhibitors, CYP3A4 inducers, PgP inhibitors Regimen & Other  Reauthorization requires documentation of disease responsiveness to therapy Criteria:

Exclusion Criteria:  Hypersensitivity to rapamycin derivatives  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction:

Prescriber  Oncologist Restrictions:

Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: AFINITOR DISPERZ Affected Medications: AFINITOR DISPERZ

Covered Uses:  NCCN indications with evidence level of 2A or higher  FDA-approved indications not otherwise excluded by plan design Required Medical SUBEPENDYMAL GIANT CELL ASTROCYTOMA (SEGA) INDICATION: Information:  Diagnosis of SEGA TUBEROUS SCLEROSIS COMPLEX (TSC)-ASSOICATED PARTIAL-ONSET SEIZURES  Documentation of monotherapy failure for seizure control with 2 different Anti- Epleptic regimens AND  Documentation of failure with at least 1 alternative adjunct therapy for seizure control  Documentation that therapy is being used as adjunct therapy for seizures

Appropriate SEGA INDICATION: Treatment  Patient has SEGA associated with a tuberous sclerosis complex (TSC) that requires Regimen & Other therapeutic intervention but is not a candidate for curative surgical resection. Criteria:  Documentation of medication review and / or avoidance with strong CYP3A4 inhibitors, CYP3A4 inducers, PgP inhibitors

 Reauthorization requires documentation of disease responsiveness to therapy Exclusion Criteria: Age Restriction:  Greater than or equal to 1 year Prescriber SEGA INDICATION: Restrictions:  Must be prescribed by or in consultation with an oncologist TSC-ASSOICATED PARTIAL-ONSET SEIZURES:  Neurologist or specialist in the treatment of TSC Coverage Duration:  Initial Authorization: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: ALDURAZYME Affected Medications: ALDURAZYME (laronidase)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Diagnosis of one the following type I mucopolysaccharidosis: Information: . Hurler Mucopolysacchardiosis I (MPS I H) . Herler-Scheie Mucopolysaccharidosis I (MPS I H/S) . Scheie form of Mucopolysacchardiosis (MPS I S) with moderate to severe symptoms  Diagnosis confirmed by an essay assay showing deficiency of alpha-L-iduronidase enzyme activity or by DNA testing  Patient weight

Appropriate  Appropriate medical support readily available when Aldurazyme is administered in Treatment case of anaphylaxis or severe allergic reaction Regimen & Other  Pretreatment with antipyretics and/or antihistamines prior to infusion Criteria:  QL: 0.58 mg/kg intravenous once weekly  Reauthorization: documentation of treatment success define as improvement in percent predicted forced vital capacity (FVC), six-minute walk test, sleep apnea, shoulder flexion, and activities of daily living Exclusion Criteria:  Treatment of central nervous system manifestation of the disorder Age Restriction:

Prescriber Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified

POLICY NAME: ALGLUCOSIDASE ALFA Affected Medications: LUMIZYME (alglucosidase alfa)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Diagnosis of Pompe disease confirmed by an enzyme assay demonstrating a deficiency Information: of acid α-glucosidase (GAA) enzyme activity or by DNA testing that identifies mutations in the GAA gene.  Patient weight and planned treatment regimen. Appropriate  Appropriate medical support is readily available when medication is administered in the Treatment event of anaphylaxis, severe allergic reaction, or acute cardiorespiratory failure. Regimen & Other Criteria: Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria: Age Restriction: Prescriber Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified.

POLICY NAME: ALPHA-1 PROTEINASE INHIBITORS Affected Medications: ARALAST NP, GLASSIA, PROLASTIN-C, ZEMAIRA

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Documentation of severe alpha-1-antitrypsin (AAT) deficiency with emphysema (or Information: Chronic Obstructive Pulmonary Disease)  Baseline (pretreatment) alpha 1 antitrypsin serum concentration less than or equal to 11 micronM (11 micromol/L or 57 mg/dL by nephelometry  Forced Expiratory Volume (FEV1) 30-65% predicted OR Forced Expiratory Volume (FEV1) reduction equal to or greater than 120mL per year Appropriate  Documentation of non-smoker or has quit smoking for at least the prior 6 months Treatment  Patient has not received a liver or lung transplantation Regimen & Other  Dosing: 60 mg/kg IV once weekly Criteria: Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Use in the management of:  Cystic fibrosis  COPD without alpha1-antitryspin deficiency  Alpha1-antitrypsin deficiency without lung disease (even if deficiency-induced hepatic disease is present  Bronchiectasis (without alpha1-antitrypsin deficiency)  Patients with IgA deficiency (less than or equal to 15 mg/dL) or IgA antibody deficiency Age Restriction: Prescriber Restrictions: Coverage Duration:  Approval: 12 months, unless otherwise specified

POLICY NAME: AMPYRA Affected Medications: AMPYRA (dalfampridine)

Covered Uses:  All FDA approved indications not otherwise excluded from plan design.

Required Medical  Documentation of dosing and patient renal function (height / weight and serum Information: creatinine OR eGFR OR CrCl).  If dosage > 20mg per day, then documentation supporting using greater than maximum recommended FDA dose.  Documentation of baseline walking ability Appropriate For initial approval for MS: Treatment  Authorize for 90 days; Regimen & Other  After up to 90 days of dalfampridine extended release therapy, if MS patient has had a Criteria: response to therapy as determined by prescribing physician (eg, increased walking distance, improved leg/limb strength, improvement in activities of daily living), then an additional authorization is allowed.

Exclusion  History of seizures Criteria:  Dose > 10 mg twice daily OR  Creatinine clearance ≤ 50 mL/min Age Restriction: Prescriber  Prescribed by or after consultation with a neurologist or an MS specialist. Restrictions:

Coverage  Initial approval: 3 months, unless otherwise specified Duration:  Reauthorization: 12 months, based on treatment response unless otherwise specified

POLICY NAME: ANTIEMETICS Affected Medications: Aloxi (palonosetron HCl 25 mcg), Akynzeo (fosnetupitant 235mcg and palonosetron 0.25mg), Emend IV (fosprepitant 1 mg), Cinvanti (aprepitant 1 mg), Varubi (rolapitant 0.5mg)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design  Aloxi (palonosetron) o Prevention of chemotherapy-induced nausea and vomiting (CINV) in adult and pediatric patients o Prevention of post-operative nausea and vomiting (PONV) in adults  Emend (fosaprepitant) o Prevention of acute and delayed nausea and vomiting associated with initial and repeat courses of highly emetogenic cancer chemotherapy (HEC) including high-dose cisplatin o Prevention of delayed nausea and vomiting associated with initial and repeat courses of moderately emetogenic cancer chemotherapy (MEC)  Cinvanti (aprepitant) o Prevention of acute and delayed nausea and vomiting associated with initial and repeat courses of highly emetogenic cancer chemotherapy (HEC) including high-dose cisplatin. o Prevention of nausea and vomiting associated with initial and repeat courses of moderately emetogenic cancer chemotherapy (MEC).  Varubi (rolapitant) o Prevention of delayed nausea and vomiting associated with initial and repeat courses of emetogenic cancer chemotherapy, including, but not limited to, highly emetogenic chemotherapy  Akynzeo (fosnetupitant and palonosetron) o Prevention of acute and delayed nausea and vomiting associated with initial and repeat courses of highly emetogenic cancer chemotherapy. o Akynzeo injection is not approved for use in anthracycline or cyclophosphamide- based chemotherapy or chemotherapy not considered highly emetogenic Required  For CINV- documentation of planned chemotherapy regimen Medical  For PONV- planned date of procedure Information:  Highly emetogenic chemotherapy (HEC): Carboplatin, carmustine, cisplatin, cyclophosphamide, dacarbazine, doxorubicin, epirubicin, ifosfamide, mechlorethamine, streptozocin, FOLFOX regimen  The following can be considered HEC in certain patients: Dactinomycin, daunorubicin, irinotecan, methotrexate (250 mg/m2 or greater), oxaliplatin, trabectedin

Appropriate Prevention of Chemotherapy induced Nausea and vomiting (CINV) in Adults Treatment  Aloxi: o Documentation of highly emetogenic chemotherapy (HEC); OR

Regimen & o Failure with another 5HT3-antagonist (i.e. ondansetron or granisetron) while Other Criteria: receiving the current chemotherapy regimen defined as two or more documented episodes of vomiting attributed to the current chemotherapy  Emend/Cinvanti/Varubi: o Documentation of highly emetogenic chemotherapy (HEC); OR o Moderately emetogenic chemotherapy and failure with a 5HT3-antagonist (i.e. ondansetron or granisetron) in combination with dexamethasone while receiving the current chemotherapy regimen  Akynzeo requires a highly emetogenic chemotherapy (HEC) regimen AND failure with another generically available 5-HT3 receptor antagonist (e.g. ondansetron, granisetron or palonosetron) and NK1 receptor antagonist (e.g. aprepitant, fosaprepitant or rolapitant) while receiving the current chemotherapy regimen  Aloxi and Akynzeo are NOT covered for: Breakthrough emesis or repeat dosing in multi-day emetogenic chemotherapy regimens

Prevention of Chemotherapy induced Nausea and vomiting (CINV) in Pediatric Patients (1 month to less than 17 years old)  Documentation of emetogenic chemotherapy  Aloxi and Varubi - Not being used for acute nausea and vomiting

Prevention of post-operative nausea and vomiting (PONV) in Adults [Aloxi only]  Dosing: 0.075 mg given immediately before anesthesia

Maximum 1 vial per 7 days for Aloxi, Emend, Akynzeo and Cinvanti; 1 vial per 14 days for Varubi

Reauthorization requires documentation of treatment success and initial criteria to be met. Exclusion Criteria: Age Restriction: Prescriber  Prescribed by or in consultation with an oncologist (For CINV) Restrictions:

Coverage  Initial Authorization: 6 months, unless otherwise specified Duration:  Reauthorization (no renewal for PONV): 6 months, unless otherwise specified

POLICY NAME: ANTIHEMOPHILIC FACTORS Affected Medications: Advate, Adynovate, Afstyla, Alphanate, Alphanine SD, Alprolix, Benefix, Corifact, Elocate, Feiba NF, Helixate FS, Hemofil M, Humate-P, Idelvion, Ixinity, Jivi, Koate DVI, Kogenate FS, Kovaltry, Monoclate-P, Mononine, NovoEight, Novoseven RT, Nuwiq, Obizur, Rebinyn, Recombinate, Riastap, Rixubis, Tretten, Vonvendi, Wilate, Xyntha

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Documentation of dose based on reasonable projections and current dose utilization Information: and product labeling, diagnosis, baseline factor level, circulating factor activity (% of normal or units/dL) and rationale for use  Patient weight  Documentation with one of the following diagnostic categories:  Treatment of acute moderate to severe bleed in patients with severe hemophilia A, severe hemophilia B, or severe von Willebrand Disease  Treatment of bleeding prevention in surgical or invasive procedure in patients with hemophilia A, hemophilia B, or von Willebrand Disease  Use as primary prophylactic therapy in patient with severe hemophilia A, severe hemophilia B, or severe von Willebrand’s disease (less than 1% of normal factor)  Documentation of treatment of acute bleeding in patients with severe hemophilia, OR primary prophylactic therapy to maintain factor levels greater than 1% of normal OR  Documentation of treatment and management of acute bleeding episodes in patients with mild hemophilia (factor levels greater than 5 and less than 30%) OR actual levels for mild hemophilia is 5-49%  Moderate hemophilia (factor levels 1% to 5%) OR  Documentation of the management of acute bleeding in clinical situations in patients with von Willebrand disease that are at an increased risk of bleeding  Reauthorization: requires documentation of planned treatment dose, number of acute bleeds since last approval with severity and cause of bleed, past treatment history and titer inhibitor level to factor VIII, and IX as appropriate Appropriate  Approval based on necessity and laboratory titer levels Treatment  Documentation of Bethesda Titer level, number of bleeds in past 3 months with Regimen & Other severity and cause of bleed Criteria:  Confirmed diagnosis of von Willebrand disease with plasma von Willebrand factor (VWF) antigen, plasma VWF activity, and factor VIII activity

Hemophilia A (factor VIII deficiency)  Documented treatment failure or contraindication to Stimate (demopressin) in mild (greater than 5%) hemophilia  For Benefix, Idelvion and Rebinyn: documentation of failure or contraindication to Rixubis  For NovoEight, Afstyla, and Nuwiq: Must have documentation of failure or contraindication to Advate or Hemofil M. 29

 For Eloctate: documentation of failure or contraindication to Advate, Helixate FS, Kogenate FS, Xyntha, or NovoEight  For Alprolix: documentation of contraindication to Rixibus in perioperative management  For Vonvendi: documentation of failure or contraindication to Humate P AND Alphanate  Documentation indicates requested medication is to achieve or maintain but not to exceed maximum functional capacity in performing daily activities

Exclusion Criteria:  History of anaphylaxis or severe hypersensitivity to any component of the chosen agent  Acute thrombosis, embolism or symptoms of disseminated intravascular coagulation (DIC)  Obizur will not be approved for the treatment of congenital hemophilia A or von Willebrands disease  Tretten will not be approved for the diagnosis of congenital factor XIII B-subunit deficiency  Jivi and Adynovate will not be approved for the treatment of von Willebrand disease, and is not for patients less than 12 years old  Idelvion will not be approved for immune tolerance induction in patients with Hemophilia B  Vonvendi will not be approved for treatment of congenital hemophilia A or hemophilia B, and is not for patient less than 18 years old  Afstyla and Nuwiq are not indicated for the treatment of von Willebrand disease  Rebinyn will not be approved for routine prophylaxis Age Restriction:  Subject to review of FDA label for each product Prescriber  Hematologist Restrictions:

Coverage  Initial approval: 3 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified  Perioperative management: 1 month, unless otherwise specified

POLICY NAME: ARCALYST Affected Medications: ARCALYST (Rilonacept)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.

Required Medical  Patient has a diagnosis of cryopyrin-associated periodic syndromes (CAPS), including Information: familial cold auto-inflammatory syndrome (FCAS) and Muckle-Wells syndrome (MWS)  Patient has failed or has contraindications to Anakinra  Negative TB test prior to initiation

Appropriate  Arcalyst is only approved for monotherapy biologic use Treatment  QL – loading dose 320 mg subcutaneous followed by 160 mg once weekly Regimen & Other Criteria: Reauthorization: documentation of treatment success

Exclusion Criteria:  Active or chronic infection, concurrent therapy with other biologics  Tuberculosis latent or active Age Restriction:  12 years of age and older Prescriber  Prescribed by or in consultation with a rheumatologist Restrictions:

Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: ARISTADA Affected Medications: ARISTADA (aripiprazole lauroxil), ARISTADA INITIO

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  Diagnosis of schizophrenia Information:  Documentation of established tolerability with oral aripiprazole for a minimum of 14 days prior to initiating treatment with Aristada.  For initial authorization only: documented plan for ensuring oral adherence during first 21 days of initial Aristada is required  Documentation of anticipated dosing based on oral aripiprazole maintenance dose.  Documentation of comprehensive antipsychotic treatment regimen (including dosing and frequency of all formulations)  Documentation of Food and Drug Administration (FDA) approved dose and frequency for the requested formulation  For Aristada Initio: Documentation of clinical rationale to avoid 21 day oral aripiprazole loading dose due to history of patient non-compliance or risk for hospitalization

Appropriate Treatment  Reauthorization: Documentation of clinically significant response to therapy. Regimen & Other Criteria: Exclusion Criteria:  Repeated dosing (greater than 1 dose) of Aristada Initio  Women who are pregnant, lactating, or breastfeeding.  Patients with dementia-related psychosis  Prior inadequate response to oral aripiprazole (unless poor adherence was a contributing factor)  No current, or within the last 2 years, diagnosis of : . Major Depressive Disorder . Comorbid schizoaffective disorder . Amnestic or other cognitive disorder . Bipolar disorder . Dementia . Delirium Age Restriction:  18 years of age or older Prescriber  Prescribed by or in consultation with a psychiatrist or behavioral health specialist Restrictions:

Coverage Duration: Aristada lauroxil  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified Aristada Initio  Approval: 1 month, unless otherwise specified 32

POLICY NAME: ARIKAYCE Affected Medications: ARIKAYCE (Amikacin inhalation suspension)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required  Diagnosis of Mycobacterium avium complex (MAC) lung disease confirmed by a MAC- Medical positive sputum culture Information:  Documentation of failure to obtain a negative sputum cultures after a minimum of 6 consecutive months of a multidrug background regimen therapy for MAC lung disease such as clarithromycin (or azithromycin), rifampin and ethambutol

Appropriate  Arikayce must be used as part of a multi-drug regimen and will not be approved for use as Treatment a single agent treatment Regimen & Other  To be used with Lamira Nebulizer system only Criteria: Reauthorization requires documentation of negative sputum culture obtained within the last 30 days.

 The ATS/IDSA guidelines state that patients should continue to be treated until they have negative cultures for 1 year. Treatment beyond the first recertification approval (after 18 months) will require documentation of a positive sputum culture to demonstrate the need for continued treatment. Patients that have had negative cultures for 1 year will not be approved for continued treatment. Exclusion  Diagnosis of non-refractory MAC lung disease Criteria:

Age Restriction:

Prescriber  Prescribed by or in consultation with infectious disease specialist Restrictions:

Coverage  Initial Approval: 6 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: ATGAM Affected Medications: ATGAM (Antithymocyte globulin)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design  Management of allograft rejection in renal transplant patients  Treatment of moderate to severe aplastic anemia in patients unsuitable for bone marrow transplantation  NCCN (National Comprehensive Cancer Network) indications with evidence level of 2A or better  Myelodysplastic Syndromes Required Medical  For myelodysplastic syndromes: Documentation of performance status, disease Information: staging, all prior therapies used, and anticipated treatment course

Appropriate  Dosing Treatment o Aplastic anemia: 10 to 20 mg/kg once daily for 8 to 14 days, then if needed, Regimen & Other may administer every other day for 7 more doses for a total of 21 doses in 28 Criteria: days OR 40 mg/kg daily for 4 days o MDS: 40 mg/kg once daily for 4 days Exclusion Criteria:  All uses not listed in covered uses are considered experimental and are excluded from coverage  Oncology: Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater  Use in patients with aplastic anemia who are suitable candidates for bone marrow transplantation or in patients with aplastic anemia secondary to neoplastic disease, storage disease, myelofibrosis, Fanconi’s syndrome, or in patients known to have been exposed to myelotoxic agents or radiation Age Restriction: Prescriber  Specialist in oncology, hematology or transplant medicine Restrictions:

Coverage Duration:  Approval: Maximum 4 weeks per dosing above

POLICY NAME: ATRYN Affected Medications: ATRYN (antithrombin alfa)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  Diagnosed hereditary antithrombin deficiency via reduced plasma antithrombin Information: level (not in midst of acute illness or surgery that could give falsely low antithrombin levels)  Can be given for prophylaxis if negative personal/family history of thromboembolic events in high risk-settings as in surgery and pregnancy.  Patient weight  Documentation of intended dose based on reasonable projections and current dose utilization and product labeling. Appropriate  Confirmed diagnosis of Hereditary Antithrombin deficiency Treatment Regimen & Other Peri-partum thromboembolic prophylaxis Criteria:  If positive personal/family history of VTE, ATryn recommended prior to and at the time of delivery when anticoagulation cannot be administered, and used until anticoagulation can be resumed  If negative personal history of VTE, patient may need single dose of ATryn  ATryn use is limited to third trimester  If positive personal/family history of VTE, ATryn recommended  Can be concomitantly given with LMWH or heparin

Peri-operative thromboembolic event prophylaxis  Used during warfarin interruption leading up to surgical procedure (with or without heparin)  Utilized until patient can resume warfarin therapy Exclusion Criteria:  Hypersensitivity to goats and goat milk protein  Administration within first two trimesters of pregnancy  Active thromboembolic event Age Restriction:  18 – 65 years of age Prescriber  OB-GYN, MD Restrictions: Coverage Duration:  Approval: 1 month, unless otherwise specified

POLICY NAME: AUBAGIO Affected Medications: AUBAGIO (Teriflunomide)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  Documentation of diagnosis of relapsing forms of Multiple Sclerosis confirmed with Information: magnetic resonance imaging (MRI)  Transaminase, bilirubin, and complete blood count (CBC) within 6 months before initiation of Aubagio.  Transaminase levels at least monthly for 6 months thereafter. Appropriate  For use in MS, patient has a relapsing form of MS and patient has failed at least one Treatment preferred product (Avonex, glatiramer 20mg, glatiramer 40mg, glatopa 20mg, Regimen & Other Extavia, Gilenya, Tecfidera) Criteria:  Reauthorization: documentation of treatment success

Exclusion Criteria:  Patients with known liver disease should not begin treatment with teriflunomide  Not used in pregnancy or plan on having children (both genders)  No concurrent use of medications indicated for treatment of relapsing-remitting multiple sclerosis.  Not approved for primary and secondary progressive multiple sclerosis. Age Restriction: Prescriber  Prescribed by or after consultation with a neurologist or an MS specialist. Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified.

POLICY NAME: AUSTEDO Affected Medications: AUSTEDO (deutetrabenazine)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical Chorea related to Huntington’s Disease: Information:  Diagnosis of Huntington’s Disease with Chorea requiring treatment  Total functional capacity score of 5 or higher on a scale of 13 (A score <5 indicates moderate to severe impairment of function, requiring a full-time caregiver- was excluded from clinical trials)

Tardive Dyskinesia:  Diagnosis of tardive dyskinesia requiring treatment defined as 10 or greater on AIMS.  History of dopamine receptor antagonist (Antipsychotic, metoclopramide) use for 3 months if < 60 years old.  History of dopamine receptor antagonist (Antipsychotic, metoclopramide) use for 1 month if 60 years old and older. Appropriate Chorea related to Huntington’s Disease: Treatment  Maximum labeled dose: 48 mg/day (Dose is typically started at 6 mg/day and titrated Regimen & Other upward to effect or tolerability) Criteria:  Reauthorization requires documentation of treatment success defined as a clinically significant improvement in function or decrease in Chorea o If disease has progressed to the point of inability to walk/need for a full-time caregiver reauthorization is not appropriate Tardive Dyskinesia:  Documented inability to discontinue offending agent or persistent dyskinesia in spite of cessation  Maximum labeled dose: 48 mg/day (Dose is typically started at 6 mg/day and titrated upward to effect or tolerability)  Reauthorization requires documentation of treatment success defined as a clinically significant improvement with a decrease in AIMS score from baseline. Exclusion Criteria:  Untreated or inadequately treated depression or suicidal ideation  Concomitant use of an MAOI (monoamine oxidase inhibitor) (must be >14 days post discontinuing therapy)  Concomitant use of tetrabenazine (Xenazine)  Severe hepatic impairment Age Restriction:  Safety and effectiveness in pediatric patients have not been established. Prescriber  Prescribed by or in consultation with a neurologist Restrictions:

Coverage Duration:  Initial approval: 4 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: AVASTIN Affected Medications: AVASTIN (bevacizumab)

Covered Uses:  NCCN indications with evidence level of 2 or higher

Required Medical  Documentation of disease staging, all prior therapies used, and anticipated treatment Information: course AND  As indicated per NCCN, documentation of performance status 0-1 AND  If patient is at risk of thrombocytopenia: Documentation that risks (DVT, intra- abdominal thrombosis, gastrointestinal perforations, hemorrhage) have been reviewed and that benefit of therapy outweighs risks Appropriate Non-Small Cell Lung Cancer (NSCLC) : Treatment  Approval will be limited to NCCN category 1 recommended therapies for first line Regimen & Other treatment of advanced NSCL cancer Criteria:  Reauthorization: documentation of disease responsiveness to therapy

Epithelial Ovarian, Fallopian Tube, or Primary Peritoneal Cancer  Approval will be limited for up to 22 cycles of therapy

Exclusion Criteria: Age Restriction: Prescriber  Oncologist Restrictions: Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: AVONEX Affected Medications: AVONEX (Interferon beta-1a)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Documentation of anticipated dosing per FDA label Information:  Documentation of diagnosis of relapsing forms of multiple sclerosis confirmed with Magnetic Resonance Imaging (MRI) in accordance with the Revised McDonald diagnostic criteria for multiple sclerosis  Documentation of recent liver function tests, CBC, and platelet counts. Appropriate  Not approved for primary and secondary progressive multiple sclerosis Treatment  Titrate weekly to recommended dose of 30 mcg once weekly Regimen & Other  Reauthorization: Documentation of treatment success Criteria: Exclusion Criteria: Age Restriction: Prescriber  Neurologist Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified.

POLICY NAME: AZEDRA Affected Medications: AZEDRA (IOBENGUANE I-131)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.  NCCN (National Comprehensive Cancer Network) indications with evidence level of 2A or higher Required Medical  Official diagnosis of pheochromocytoma or paraganglioma documented in Information: member’s chart AND  Laboratory confirmed diagnosis - Two-fold elevation above upper limit of normal in urine catecholamines or - Elevated urine metanephrines o Nmet greater than 900 mcg per 24 hours or o Met greater than 400 mcg per 24 hours or - “Significant increase” in fractionated plasma metanephrines AND  Positive adrenal/abdominal MRI or CT scan AND  Prior positive MIBG scan with dosimetry

 Reauthorization: Reauthorization will require documentation of disease responsiveness to therapy Appropriate Treatment Dosimetric Dose Regimen & Other Criteria:  Patients weighing greater than 50 kg: 185 to 222 MBq (5 or 6 mCi)  Patients weighing 50 kg or less: 3.7 MBq/kg (0.1 mCi/kg)

Therapeutic Dosage: administer 2 therapeutic doses intravenously a minimum of 90 days apart  Patients weighing greater than 62.5 kg: 18,500 MBq (500 mCi)  Patients weighing 62.5 kg or less: 296 MBq/kg (8 mCi/kg) Exclusion Criteria:

Age Restriction:  Must be at least 12 years old

Prescriber Restrictions:  Oncologist

Coverage Duration:  Initial approval: 4 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: BANZEL Affected Medications: BANZEL (rufinamide)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical Information:  Diagnosis of Lennox-Gastaut Syndrome

Appropriate Treatment  QL: 3200 mg daily Regimen & Other Criteria:  Reauthorization: documentation of treatment success

Exclusion Criteria:  Familial Short QT syndrome

Age Restriction:  1 year of age and older

Prescriber Restrictions:  Neurologist

Coverage Duration:  Approval: 12 months, unless otherwise specified

POLICY NAME: BELEODAQ Affected Medications: BELEODAQ (belinostat)

Covered Uses:  NCCN indications with evidence level 2A or higher. Required Medical  Documentation of staging, all prior therapies used, performance status and Information: anticipated treatment course  CBC with differential, creatinine clearance (CrCl), liver function tests  Documentation of UGT1A1*28 allele status Appropriate Treatment  Appropriate dose reduction based on absolute neutrophil count (ANC) OR Regimen & Other homozygous UGT1a1*28 allele Criteria:  Reauthorization: documentation of disease responsiveness to therapy

Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction:

Prescriber Restrictions:  Oncologist Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: BENLYSTA Affected Medications: Benlysta (Belimumab)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Documentation of systemic lupus erythematosus with disease staging AND Information:  Documentation of patient’s current weight AND  Failure with standard therapy (clinical failure with at least 2 or more of the following in combination: hydroxychloroquine, chloroquine, azathioprine, methotrexate, mycophenolate, cyclophosphamide)

Appropriate  For adult patients (18 years of age and and older): Intravenous (IV) formulation Treatment requires documented inability to use subcutaneous formulation. Regimen & Other  Dosing: Criteria: Patients 5 years of age and older: o Benlysta Intravenous (IV) Solution: IV Initial- 10 mg/kg every 2 weeks for 3 doses; Maintenance: 10 mg/kg every 4 weeks. Patients 18 years of age and older: o Benlysta Subcutaneous: 200 mg once weekly.

 Reauthorization: Documentation of treatment success defined as a clinically significant improvement in SLE Responder Index-4 (SRI-4) or decrease in flares/corticosteroid use.

Exclusion Criteria:  Benlysta is not approved to be used in combination with other biologic therapies or intravenous cyclophosphamide  Benlysta is not approved to be used in severe active lupus nephritis or severe active central nervous system lupus Age Restriction:

Prescriber  By or in consultation with a specialist in immunology or rheumatology Restrictions: Coverage  Authorization: 12 months, unless otherwise specified Duration:

POLICY NAME: BESPONSA Affected Medications: BESPONSA (Inotuzumab Ozogamicin)

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Peripheral blast count of less than or equal to 10,000 / mm3 Information:  Documentation of disease staging, all prior therapies used, anticipated treatment course, and performance status AND  Documentation of relapsed or refractory B-cell precursor acute lymphoblastic leukemia AND  Philadelphia chromosome status AND  Documentation that Black Box Warnings (hepatotoxicity including veno-occlusive disease, increased risk of non-relapse mortality post-HSCT) have been fully reviewed and patient understands and accepts risks. Appropriate  For patients proceeding to HSCT, recommended treatment duration is 2 cycles. May Treatment utilize 3 cycles maximum. Regimen & Other  For patients not proceeding to HSCT, recommended treatment duration is up to 6 cycles Criteria: maximum.  Reauthorization requires documentation of clinically significant improvement and member not achieving complete remission or complete remission with incomplete hematologic recovery and have minimal residual disease negativity after completing 2 cycles. Exclusion  Severe hepatic impairment Criteria:  Less 5% bone marrow blasts Age Restriction:  Safety and effectiveness in pediatric patients have not been established. Prescriber  Prescribed by an oncologist Restrictions: Coverage Approval for transplant candidate: 3 months, unless otherwise specified Duration: Approval for non-transplant candidate:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 3 months, unless otherwise specified

POLICY NAME: BETASERON Affected Medications: BETASERON (Interferon beta-1b)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Documentation of diagnosis of relapsing form of multiple sclerosis confirmed with Information: magnetic resonance imaging (MRI)  Complete blood count, basic metabolic panel one, three, and six months following introduction of Betaseron therapy and then periodically thereafter

Appropriate  Must fail at least one preferred product (Avonex, glatiramer 20mg, glatiramer 40mg, Treatment glatopa 20mg, Extavia, Gilenya, Tecfidera) Regimen & Other  Reauthorization: documentation of treatment success Criteria:

Exclusion Criteria:  Concurrent use of medications indicated for the treatment of relapsing form of multiple sclerosis  For treatment of primary and secondary progressive multiple sclerosis Age Restriction:

Prescriber  Neurologist Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified

POLICY NAME: BLINCYTO Affected Medications: BLINCYTO (blinatumomab)

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of disease staging, all prior therapies used, performance status and Information: anticipated treatment course AND  Philadelphia chromosome status AND  Documentation of ECOG performance status of 1 or 2 OR Karnofsky performance score greater than 50% Appropriate  Blincyto should permanently be discontinued for the following adverse reactions: Treatment grade 4 cytokine release syndrome, grade 4 neurological toxicity, or two Blincyto Regimen & Other induced seizures Criteria:  Maximum approval: 5 cycles for Relapsed or Refractory ALL, 4 cycles for ALL in 1st or 2nd remission with MRD Exclusion Criteria: Age Restriction: Prescriber  Oncologist Restrictions: Coverage Duration:  Initial approval: 30 weeks for Relapsed or Refractory ALL; 24 weeks for ALL in 1st or 2nd remission with MRD  Reauthorization: None (maximum of 5 cycles approved), unless otherwise specified

POLICY NAME: BONJESTA & DICLEGIS Affected Medications: BLINCYTO (doxylamine succinnate and pyridoxine hydrochloride extended-release oral tablets), DICLEGIS (Doxylamine-Pyridoxine Tab Delayed Release 10-10 mg)

Covered Uses: ● All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Estimated Delivery Date Information:  Documentation of all therapies tried/failed Appropriate ● Documentation of trial and education on non-pharmacologic methods of Treatment controlling nausea and vomiting related to pregnancy (avoidance of triggers, Regimen & Other proper rest, etc.) Criteria: Documented failure, intolerance or clinical rationale for avoidance to ALL of the following:

● OTC pyridoxine with OTC doxylamine AND ● Dopamine antagonist (prochlorperazine, metoclopramide, etc.) OR ● H1 antagonist (promethazine, meclizine, dimenhydrinate, diphenhydramine, etc.) OR ● Ondansetron Exclusion Criteria: Age Restriction: Prescriber Restrictions: Coverage Duration: ● Approval: 12 weeks, unless otherwise specified

POLICY NAME: BOSULIF Affected Medications: BOSULIF (bosutinib)

Covered Uses:  NCCN indications with evidence level of 2A or higher Required Medical  Documentation of performance status, all prior therapies used, and prescribed Information: treatment regimen  Documentation of use with NCCN 2A or higher level of evidence regimen  If applicable, test results demonstrating Philadelphia chromosome-positive status  If applicable, BCR-ABL1 transcript levels

Appropriate  Should not be used in combination with strong or moderate CYP3A4 inducers or Treatment inhibitors Regimen & Other  Proton pump inhibitors may decrease bosutinib drug levels. Consider short-acting Criteria: antacids in place of proton pump inhibitors  Reauthorization requires documentation of disease responsiveness to therapy (based on applicable markers examples BCR-ABL1 transcript levels, cytogenetic response, molecular relapse)

Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber  Oncologist Restrictions: Coverage Duration:  Initial approval: 3 months  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: BOTOX Affected Medications: BOTOX (onabotulinumtoxinA)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design

Required  Pertinent medical records and diagnostic testing Medical  Complete description of the site(s) of injection Information:  Strength and dosage of botulinum toxin used Appropriate  Approved first-line for: focal dystonia, drug-induced orofacial dyskinesia, blepharospasm, Treatment severe writer's cramp, laryngeal spasm or dysphonia, upper and lower limb spasticity or Regimen & other conditions of central focal spasticity botulinum toxin is the preferred mode of Other Criteria: therapy.  For use in all other FDA-approved indications not otherwise excluded by benefit design, failure of first-line recommended and conventional therapies is required  Idiopathic or neurogenic detrusor over-activity (Overactive Bladder (OAB)): Inadequate response to, or intolerance to, ≥2 incontinence antimuscarinic drugs  Urinary incontinence associated with neurologic condition: Documentation of neurologic condition (e.g. spinal cord injury, multiple sclerosis) AND Inadequate response or intolerance to greater than 2 anticholinergic medications.  Chronic migraine: Documentation of chronic migraine defined as headaches on at least 15 days per month of which at least 8 days are with migraine AND documented failure with an adequate trial (at least 8 weeks) of an oral migraine preventive therapy (beta-blocker, calcium channel blocker, anticonvulsant or tricyclic antidepressant) as follows: o Propranolol 40 mg daily, Metoprolol 100 mg daily o Amitriptyline 25 mg daily, o Topiramate 50 mg daily, Valproic acid, Divalproex sodium  Achalasia and cardiospasm (must meet 1 of the following): o Failed conventional therapy, myotomy, or dilatation o high risk of complications from pneumatic dilation or surgical myotomy o Epiphrenic diverticulum or hiatal hernia Number of treatments must not exceed the following: o OAB: 2 treatments/12 months o Urinary incontinence associated with neurologic condition: 1 treatment/12 months o Chronic migraine: initial treatment limited to two injections given 3 months apart, subsequent treatment approvals limited to 4 treatments per 12 months o All other indications maximum of 4 treatments/12 months unless otherwise specified Reauthorization: Documentation of improvement and clinically significant response to therapy;  Chronic migraine continuation of treatment: Additional treatment requires that the member has achieved or maintained a 50% reduction in monthly headache frequency since starting therapy with Botox.

 Idiopathic or neurogenic detrusor over-activity (Overactive Bladder (OAB)): Additional treatment requires documented positive response to therapy. Positive response to therapy is defined as a reduction of urinary frequency of greater or equal to 8 episodes per day or urinary incontinence of greater or equal to 2 episodes per day compared to baseline frequency.

Exclusion  Cosmetic procedures Criteria:  For intradetrusor injections: documented current/recent urinary tract infection or urinary retention  Current aminoglycoside use (or current use of other agents interfering with neuromuscular transmission)  Hemifacial spasm: no longer above the line on the prioritized list  Possible medication overuse headache: headaches occurring 15 or more days each month in a patient with pre-existing headache-causing condition possibly due to o Use of ergotamines, triptans, opioids, or combination analgesics greater than or equal to 10 days per month for greater than or equal to three months o Use of simple analgesics (acetaminophen, aspirin, or an NSAID) greater than or equal to 15 days per month for greater than or equal to 3 months o Use of combination of any previously mentioned products without overuse of any one agent if no causative pattern can be established  Combined use with Calcitonin Gene-Related Peptide (CGRP) Receptor Antagonists (Ajovy, Aimovig or Emgality) for the treatment of migraine Age Restriction:

Prescriber  Blepharospasm, strabismus: ophthalmologist or optometrist Restrictions:  Chronic migraine: treatment is administered in consultation with a neurologist or headache specialist.  OAB or urinary incontinence due to neurologic condition: urologist or neurologist  Documentation of consultation with any of the above specialists mentioned Coverage Chronic migraine: Duration:  Initial approval: 6 months  Reauthorization: 12 months, unless otherwise specified Overactive Bladder:  Initial approval: 3 months  Reauthorization: 12 months, unless otherwise specified All other indications:  Approval 12 months, unless otherwise specified

POLICY NAME: BRINEURA Affected Medications: BRINEURA (carliponase alfa)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required  Confirmed diagnosis of infantile neuronal ceroid lipofuscinosis type 2 (CLN2) with one of Medical the following: Information: - Documented deficient tripeptidyl peptidase-1 (TPP1) activity in leukocytes - Pathogenic variants/mutations in each allele of TPP1/CLN2 gene AND baseline motor, speech and vision function documented by the physician  Documentation of baseline performance of mobility confirming functional impairment (use The Motor domain of a CLN2 Clinical Rating Scale confirmed disease progression) with mild to moderate disease and a two-domain score of 3 to 6 on motor and language domains of the Hamburg Scale with a score of at least 1 in each of these two domains  Planned Treatment Regimen including doses, frequency  Planned monitoring parameters for infections and side effects Appropriate  Dosing: 300 mg administered once every other week by intraventricular infusion Treatment Regimen & Reauthorization: Other Criteria:  Documentation of continuing meeting initial review criteria AND  Documentation of clinical responsiveness to therapy with disease stability/improvement defined as a score of 1 or higher in the motor domain of the Clinical Scoring System for LINCL.

Exclusion  Patients with acute intraventricular access device-related complications (e.g., leakage, Criteria: device failure or signs of device-related infection such as swelling, erythema of the scalp, extravasation of fluid, or bulging of the scalp around or above the intraventricular access device)  Other form of neuronal ceroid lipofuscinosis  Patients with ventriculoperitoneal shunts Age Restriction:  Below 3 years or greater than 16 years of age Prescriber  Must be prescribed by a neurologist or in consultation with a neurologist with expertise in Restrictions: the diagnosis of CLN2  Must be administered by, or under the direction of a physician knowledgeable in intraventricular administration Coverage  Initial approval: 3 months, unless otherwise specified Duration:  Reauthorization: 6 months, unless otherwise specified

POLICY NAME: CABLIVI Affected Medications: CABLIVI (caplacizumab)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design  Treatment of adult patients with acquired thrombotic thrombocytopenic purpura (aTTP), in combination with plasma exchange and immunosuppressive therapy  Refractory or relapsed aTTP despite plasma exchange and immunosuppressive therapy  Initial therapy for patients with documented severe features of aTTP Required Medical  Documentation of severe features of aTTP: Information: o Neurologic findings such as seizures, focal weakness, aphasia, dysarthria, confusion, coma o Symptoms suggesting encephalopathy o High serum troponin levels  Trial or unresponsive to treatment with daily plasma exchange alongside immunosuppressive agents (glucocorticoids and rituximab)  ADAMTS13 activity  Platelet count Appropriate Dosing: Treatment  First day of treatment: IV followed by SubQ: 11 mg IV at least 15 minutes prior to Regimen & Other plasma exchange, followed by 11 mg SubQ after completion of plasma exchange on Criteria: day 1.  Subsequent treatment days (during daily plasma exchange): SubQ: 11 mg once daily following plasma exchange.  Treatment after plasma exchange period: SubQ: 11 mg once daily, continuing for 30 days following the last daily plasma exchange; if sign(s) of persistent underlying disease remain present (eg, suppressed ADAMTS13 activity levels) after initial treatment course, treatment may be extended up to a maximum of 28 days.  Discontinuation: Discontinue caplacizumab if >2 recurrences of acquired thrombotic thrombocytopenic purpura (aTTP) occur during treatment.

 Reauthorization requires documented signs of ongoing disease (eg, suppressed ADAMTS13 activity levels) Exclusion Criteria: Age Restriction:  18 years and older Prescriber  Treatment by or in consultation with a hematology specialist Restrictions: Coverage Duration:  Initial Authorization: 1 month, unless otherwise specified  Reauthorization: 1 month, unless otherwise specified

POLICY NAME: CALQUENCE Affected Medications: CALQUENCE (acalabrutinib)

Covered Uses:  NCCN (National Comprehensive Cancer Network) indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, disease staging, all prior therapies used, and Information: anticipated treatment course

Appropriate  Documentation of least one prior treatment for Mantle Cell Lymphoma Treatment Regimen & Other Reauthorization: documentation of disease responsiveness to therapy Criteria: Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater  Prior use of Bruton Tyrosine Kinase (BTK) inhibitors. Age Restriction: Prescriber  Oncologist Restrictions: Coverage Duration:  Initial approval: 4 months  Reauthorization: 12 months

POLICY NAME: CAPRELSA Affected Medications: CAPRELSA (vandetanib)

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, disease staging, all prior therapies used, and Information: prescribed treatment regimen  Documentation of use with NCCN 2A or higher level of evidence regimen Appropriate  Avoid administration with agents that prolong the QT interval and strong CYP3A4 Treatment inducers Regimen & Other  Reauthorization requires documentation of disease responsiveness to therapy Criteria: Exclusion Criteria:  Congenital long QT syndrome  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction:

Prescriber  Oncologist Restrictions:

Coverage Duration:  Initial approval: 3 months  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: C1 ESTERASE INHIBITOR Affected Medications: BERINERT, CINRYZE, FIRAZYR*, HAEGARDA, RUCONEST* (*COVERED UNDER MEDICAL BENEFITS ONLY)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Hereditary angiogenesis (HAE) official diagnosis documented in member’s chart AND Information:  Number of hereditary angiogenesis attacks per month must be ≥2. AND  Laboratory confirmed diagnosis: -C4 antigenic level -C1-inhibitor antigenic level -C1-inhibitor functional level

Appropriate  Berinert: Prophylaxis: 20 units/kg IV twice a week; avoid doses less than 20 units/kg. Treatment Treatment of acute attacksL 20 units/kg IV Regimen & Other  Cinryze: Prophylaxis: 1000 units IV twice a week. Doses up to 2,500 units (not Criteria: exceeding 100 units/kg) may be appropriate if inadequate response with 1000 units.  Firazyr: Treatment of acute attacks: 30mg SC. Additional doses may be administered at 6 hour intervals if response is inadequate or symptoms recur. Maximum 3 doses in 24 hours.  Haegarda: Prophylaxis: 60 units/kg SC twice a week  Ruconest: 50 units/kg IV, not to exceed 4200 units per dose. If attack symptoms persist, a second dose may be administered. Not to exceed 2 doses in 24 hours. (Effectiveness not demonstrated in patients with laryngeal attacks)

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria: N/A Age Restriction:  Berinert: Indicated for treatment and prophylaxis of HAE in adult and pediatric patients  Cinryze: Safety and effectiveness has not been established in neonates, infants or children.  Firazyr: Approved for treatment of acute HAE attacks in patients 18 and older  Haegarda: Approved for patients 12 and older  Ruconest: Indicated for treatment and prophylaxis of HAE in adult and pediatric patients Prescriber  Must be prescribed by, or in consultation with, an allergist/immunologist or physician Restrictions: that specializes in HAE or related disorders. Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: CARBAGLU Affected Medications: CARBAGLU (carblumic acid)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Diagnosis of N-acetyl glutamate synthase (NAGS) deficiency with hyperammonemia Information: (plasma ammonia levels > 70 mcg/dL) Appropriate  For patients with acute hyperammonemia, Carbaglu should always be used in Treatment combination with other methods to lowering plasma ammonia levels such as Regimen & Other hemodialysis, other pharmacologic therapy (Sodium phenylacetate and sodium Criteria: benzoate), and dietary protein restriction.

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Carbaglu should not be used to treat hyperammonemia due to disorders other than urea cycle disorder (UCD) specifically caused by NAGS deficiency  Carbaglu should not be used in patients with UCD caused by other enzyme deficiencies that lead to hyperammonemia. This includes: o Carbamyl phosphate synthetase I (CPSI) deficiency o Ornithine transcarbamylase (OTC) deficiency o Argininosuccinate synthetase (ASS) deficiency o Argininosuccinate lyase (ASL) deficiency o Arginase deficiency Age Restriction:

Prescriber  Carbaglu treatment should be initiated by a physician experienced in the treatment of Restrictions: metabolic disorders. Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: CAYSTON Affected Medications: CAYSTON (aztreonam inhalation)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Diagnosis of cystic fibrosis Information:  Culture and sensitivity report confirming presence of Pseudomonas aeruginosa in the lungs o To reduce the development of drug-resistant bacteria and maintain the effectiveness of aztreonam and other antibacterial drugs, only use aztreonam to treat patients with cystic fibrosis known to have P. aeruginosa in the lungs.  Baseline FEV1 greater than 25% but less than 75% predicted  Documented failure, contraindication, or resistance to inhaled tobramycin  Anticipated treatment duration Appropriate  Dosing 28 days on and 28 days off Treatment Reauthorization: documentation of improved respiratory symptoms including improved Regimen & Other FEV1, reduced bacterial density in sputum, and need for long-term use such as history of Criteria: frequent exacerbations resulting in hospitalizations due to pseudomonas aeruginosa infection Exclusion Criteria:  Baseline FEV1 less than 25% or greater than 75% predicted Age Restriction:  Age 7 years or older Prescriber Restrictions:

Coverage Duration:  Initial approval: 1 month, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: CERDELGA Affected Medications: CERDELGA (eliglustat)

Covered Uses:  All FDA approved indications not otherwise excluded by plan design

Required Medical  FDA approved diagnosis must be documented in the members chart notes within the Information: past 6 months  Diagnosis of Gaucher disease with enzyme assay documenting type I disease (GD1)  Documentation of CY2D6 Genotype by a FDA approved test indicating 2D6 extensive metabolizers, intermediated metabolizers, or poor metabolizers  Documentation of complete and current treatment course  Documentation of baseline tests such as hemoglobin level, platelet count, liver function tests, renal function tests. Appropriate  Documentation of failure, intolerance, or clinical rationale for the avoidance of Treatment combination therapy with Cerezyme, and failure with Cerezyme monotherapy Regimen & Other Criteria: Extensive or Immediate Metabolizers of CYP2D6  QL- 84 mg capsules #60 per 30 days Poor Metabolizers of CYP2D6  QL- 84 mg capsules #30 per 30 days  Reauthorization: documentation of treatment success: Labs have not significantly deteriorated compared to baseline. Exclusion Criteria:  Patient must not be a CYP2D6 ultra-rapid metabolizer, OR an indeterminate metabolizer Patients on concomitant medication that inhibit CYP2D6 and CYP3A4 isoenzymes  Pre-existing Cardiac disease (CHF, MI, Bradycardia, heart block, arrhythmias, and long QT syndrome)  Treatment with Class 1A (quinidine, procainaminde) and Class III (amiodarone, sotalol) antiarrhythmic medications  Presence of moderate to severe renal impairment or end stage renal disease Age Restriction:  18 years of age or older Prescriber  Metabolic disease specialist Restrictions:

Coverage Duration:  Approval: 3 months, unless otherwise specified  Reapproval: 12 months, unless otherwise specified

POLICY NAME: CEREZYME Affected Medications: CEREZYME (imiglucerase)

Covered Uses:  All FDA approved indications not otherwise excluded by plan design Required Medical  Diagnosis of Type 1 (nonneuronopathic) Gaucher disease that has resulted in 1 or more Information: of the following conditions: anemia, bone disease, hepatomegaly or splenomegaly, and thrombocytopenia, OR  Diagnosis of Type 3 (chronic neuronopathic) Gaucher disease, OR  Patients with following high-risk genotypes:  L444P/L444P (c.14486>C homozygote)  D409H/D409H (c.1342G> C homozygote)  L444P/D409H (c.1448T>C/c.1342G>C heteroygote), AND  Documented patient weight, dose and frequency

Appropriate  Not approved for Type 2 (acute neuronopathic disease) Gaucher Disease Treatment  Documented adult patients with symptomatic disease: platelet count less than Regimen & Other 60,000/microL, liver greater than 2.5 times normal size, spleen greater 15 times normal Criteria: size, radiologic evidence of skeletal disease, etc.  Documented symptomatic children: includes those with malnutrition, growth retardation, impaired psychomotor development, and/or fatigue (early presentation is associated with more severe disease)

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:

Age Restriction:

Prescriber Restrictions: Coverage  Initial approval: 3 months Duration:  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: CGRP Inhibitors Affected Medications: Aimovig, Ajovy, Emgality

Covered Uses:  All FDA approved indications not otherwise excluded by plan design o Preventive treatment of migraine in adults Required Medical  Diagnosis of chronic migraine defined as headaches on at least 15 days per month of Information: which at least 8 days are with migraine at baseline  Diagnosis of episodic migraine with at least 8 migraines per month at baseline Appropriate  Chronic or episodic migraine: documented treatment failure with an adequate trial Treatment (at least 8 weeks) of an oral migraine preventive therapy as follows: Regimen & Other o Propranolol 40 mg daily, Metoprolol 100 mg daily Criteria: o Amitriptyline 25 mg daily o Topiramate 50 mg daily, Valproic acid, Divalproex sodium AND  For treatment of chronic migraine: treatment failure after at least 6 months (2 injection series) of Botox

 Dosing: o Aimovig: 70 mg (1x 70 mg pen) or 140 mg (2x 70 mg pen pack) once monthly [70 mg/mL pen & 140 dose 2x 70 mg/mL pen] o Ajovy: 225 mg every 30 days or 675 mg (3x 225 mg pen) every 90 days [225 mg/1.5 mL pen] o Emgality: 240 mg single loading dose then 120 mg every 30 days [120 mg/mL pen or syringe]

Reauthorization requires that the member has achieved or maintained a 50% reduction in monthly headache frequency since starting therapy Exclusion Criteria:  Combined use with Botox for the treatment of migraine  Possible medication overuse headache: headaches occurring 15 or more days each month in a patient with pre-existing headache-causing condition possibly due to o Use of ergotamines, triptans, opioids, or combination analgesics at least 10 days per month for at least three months o Use of simple analgesics (acetaminophen, aspirin, or an NSAID) at least 15 days per month for at least 3 months o Use of combination of any previously mentioned products without overuse of any one agent if no causative pattern can be established Age Restriction:

Prescriber  Neurologist or headache specialist Restrictions: Coverage Duration:  Initial: 6 months , unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: CHOLBAM Affected Medications: CHOLBAM (cholic acid) 50 & 250mg capsules

Covered Uses:  All FDA approved indications not otherwise excluded by plan design Required  Patient weight, dose and frequency Medical  Baseline liver function tests (AST, ALT, GGT, ALP, total bilirubin, INR) Information:  Diagnosis confirmed by assessment of serum or urinary bile acid levels using mass spectrometry (Fast Atom Bombardment ionization - Mass Spectrometry (FAB-MS) analysis) Appropriate  Dose: 10 to 15 mg/kg orally once daily, or in two divided doses Treatment  Dose if concomitant familial hypertriglyceridemia: 11 to 17 mg/kg orally once daily, or in Regimen & two divided doses Other Criteria:  Reauthorization requires documentation of clinically significant improvement in liver function as determined by meeting TWO of the following criteria: ALT or AST values reduced to less than 50 U/L, or baseline levels reduced by 80%; total bilirubin values reduced to less than or equal to 1 mg/dL; no evidence of cholestasis on liver biopsy; body weight increased by 10% or stable at greater than the 50th percentile  Treatment should be discontinued if liver function does not improve after 3 months of start of treatment Exclusion  Treatment of extrahepatic manifestations (such as neurologic symptoms) of bile acid Criteria: synthesis disorders due to single enzyme defects or peroxisomal disorders including Zellweger spectrum disorders Age Restriction:  3 weeks and older Prescriber  Prescribed by or in consultation with hepatologist or gastroenterologist Restrictions: Coverage  Initial: 3 months Duration:  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: CIMZIA Affected Medications: CIMZIA (certolizumab)

Covered Uses: o All FDA-approved indications not otherwise excluded by plan design. Required Medical o Documentation of moderate to severe disease despite current treatment (Indication Information: must be documented in chart notes within the last 6 months) o Documentation of complete and current treatment course o Documented current level of disease activity/disease control Rheumatoid arthritis o Laboratory test confirming diagnosis of rheumatoid arthritis (anti-CCP, RF) o Documented current level of disease activity with one of the following: o The Disease Activity Score derivative for 28 joints (DAS-28) greater than 3.2 o The Simplified Disease Activity Index (SDAI) greater than 11 o The Clinical Disease Activity Index (CDAI) greater than 10 Crohn’s disease o Documentation of Crohn’s Disease Activity Index (CDAI) greater than 220

Plaque psoriasis (PP) o Documentation of disease that is severe in nature, which has resulted in functional impairment (e.g., inability to use hands or feet for activities of daily living, or significant facial involvement preventing normal social interaction) AND one or more of the following: o At least 10% body surface area involvement; or o Hand, foot or mucous membrane involvement

Psoriatic Arthritis (PsA) with Axial Involvement  Documentation of active disease defined by Bath ankylosing spondylitis disease activity index (BASDAI) at least 4 AND  Scaroiliitis on imaging AND at least 1 Spondyloarthritis (SpA) feature: o Inflammatory back pain (4 of 5 features met): . Onset of back discomfort before the age of 40 years . Insidious onset . Improvement with exercise . No improvement with rest . Pain at night (with improvement upon arising) o Arthritis o Enthesitis o Uveitis o Dactylitis (inflammation of entire digit) o Psoriasis o Crohn’s disease/ulcerative colitis o Good response to NSAIDs o Family history of SpA

o Elevated CRP OR  HLA-B27 genetic test positive AND at least 2 SpA features

Appropriate Rheumatoid Arthritis (RA) Treatment  Has failed minimum 12 week trial of combination disease-modifying anti-rheumatic drug Regimen & Other therapy: methotrexate 25 mg (IM or SQ) With hydroxychloroquine and/or sulfasalazine, Criteria: or leflunomide AND  Has failed minimum 12 weeks on Humira AND minimum 12 weeks on Enbrel  QL – Initial (one time only) – 6 ml per 28 day supply  QL – Continuation – 2 ml per 28 day supply

Psoriatic Arthritis (PsA)  Failure of at least 12 weeks with methotrexate, or if unable to tolerate or contraindicated to methotrexate, another disease modifying antirheumatic drug (sulfasalazine, cyclosporine, leflunomide) AND  Has failed minimum 12 weeks on Humira AND minimum 12 weeks on Enbrel  QL – Initial (one time only) – 6 ml per 28 day supply  QL – Continuation – 2 ml per 28 day supply

Crohn’s Disease  Has failed at least two oral treatments for minimum of 12 week trial: corticosteroids, azathioprine, cyclosporine, 6-mercaptopurine, methotrexate, sulfasalazine, balsalazide, tacrolimus, mesalamine AND  Has failed minimum 12 weeks on Humira  QL – Initial (one time only) – 6 ml per 28 day  QL – Continuation – 2 ml per 28 day supply

Plaque psoriasis  Has failed at least two systemic therapies with minimum of 12 week trial: methotrexate, cyclosporine, isotretinoin or phototherapy (UVB, PUVA) AND  Has failed minimum 12 weeks on Humira AND failed minimum 12 weeks on Enbrel

Reauthorization: documentation of treatment success

Ankylosing Spondylitis (AS) and Psoriatic Arthritis (PsA) with Axial Involvement  Has failed two daily prescription strength nonsteroidal anti-inflammatory drugs (ibuprofen, naproxen, diclofenac, meloxicam, etc.) with minimum 1 month trial each  For isolated sacroiliitis, enthesitis, peripheral arthritis: documented treatment failure with locally administered parenteral glucocorticoid  Has failed minimum 12 weeks on Humira AND minimum 12 weeks on Enbrel  QL – Initial (one time only) – 6 ml per 28 day supply  QL – Continuation – 2 ml per 28 day supply

 Reauthorization: Improvement of at least 50% in the BASDAI score or an absolute change of 2 units. Exclusion  Concurrent us of biologic DMARDs: Orencia (abatacept), Rituxan (rituximab), Actemra Criteria: (tocilizumab), Enbrel (etanercept), Remicade (infliximab), Simponi (golimumab), Humira (adalimumab), Kineret (anakinra), Xeljanz (tofacitinib)  Concurrent use of Otezla (Apremilast)  Positive tuberculosis; active hepatitis B, hepatitis C or herpes zoster infection  Use for the management of plaque psoriasis Age Restriction:  18 years of age or older Prescriber  CD/UC: prescribed by or in consultation with a GI specialist Restrictions:  RA/PsA/AS: prescribed by or in consultation with a rheumatologist Coverage  Initial approval: 4 months unless otherwise specified Duration:  Reauthorization: 12 months unless otherwise specified

POLICY NAME: CINQAIR IV Affected Medications: CINQUAIR IV (reslizumab-interleukin-5 antagonist monoclonal antiboty (IgG4 kappa))

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  Diagnosis of severe asthma with an eosinophilic phenotype defined as: Information:  eosinophilic count greater than 400 cells/ul within 1 year  Baseline FEV1  Documentation of all current asthma medications and all prior treatments tried and failed  medium-to-high doses of inhaled corticosteroid based with or without another controller drug (including oral corticosteroids)  Documentation of multiple asthma-related exacerbations within the past year Appropriate  Documentation of managing medication adherence, inhaler technique, environmental Treatment control, and comorbidities prior to stepping up therapy per the National Institute of Regimen & Other Health (NIH guidelines) Criteria:  Asthma symptoms not controlled for minimum of 2 months by concomitant use of high dose inhaled corticosteroid AND long-acting beta agonist (LABA)  If LABA contraindicated or not tolerated, sustained-release theophylline OR a leukotriene modifier must be tried AND  Documented inadequate asthma control demonstrated by hospitalization OR increasing need for short-acting beta agonist (SABA) as demonstrated by using rescue inhaler for greater than 4 times a day  Documentation of optimal management of other asthma triggers  Subsequent approvals: documentation of treatment success defined as reduction in daily recuse inhalers use, prednisone use, or improved FEV1 from baseline Exclusion Criteria:  Not indicated for treatment of other eosinophilic conditions  Not indicated for acute bronchospasm or status asthmaticus  Eosinophilic less than 150 cells/ul Age Restriction:  Patients 18 years old and older Prescriber  Prescribed by or in consultation with an allergist, immunologist, or pulmonologist Restrictions:

Coverage  Approval: 12 months, unless otherwise specified Duration:

POLICY NAME: COAGADEX Affected Medications: COAGADEX (Factor X)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  Documentation of dose based on reasonable projections and current dose utilization Information: and product labeling, diagnosis, baseline factor level, circulating factor activity (% of normal or units/dL) and rationale for use  Patient weight  Documentation with one of the following diagnostic categories: o On-demand treatment and control of bleeding episodes o Perioperative management of bleeding in patients with mild and moderate hereditary Factor X deficiency o Routine prophylaxis to reduce the frequency of bleeding episodes

Reauthorization (Routine Prophylaxis only): requires documentation of planned treatment dose, number of acute bleeds since last approval with severity and cause of bleed

Appropriate  Food and Drug Administration (FDA)-approved dosing Treatment Regimen & Other Criteria:

Exclusion Criteria:

Age Restriction:  12 years and older Prescriber  Hematologist Restrictions:

POLICY NAME: COMPOUNDED MEDICATIONS Affected Medications: ALL COMPOUNDED MEDICATIONS

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  All compounded ingredients must be submitted on the pharmacy claim Information:

Appropriate  Compounded medications will only be payable after ALL commercially available or Treatment formulary products have been exhausted. Regimen & Other  In the case of a payable claim, only compound ingredients that are covered on the Criteria: applicable formulary will be reimbursed under this policy.  Compounds above a certain dollar threshold will be stopped by the claim adjudication system. Exclusion Criteria:  Compounds for experimental or investigational uses will not be covered.  Compounds containing non-FDA approved ingredients will not be covered  Non-FDA approved compounded medications will not be covered when an FDA approved, commercially available medication is on the market for treatment of requested condition Age Restriction: Prescriber Restrictions:

Coverage Duration:  3 months unless otherwise specified

POLICY NAME: CONTINUOUS GLUCOSE MONITORS Affected Medications: FREESTYLE LIBRE and DEXCOM G6

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical 1. Diagnosis of Type 1 diabetes currently on an insulin pump Information: 2. Diagnosis of Type 1 diabetes not currently using an insulin pump with one of the following:  Baseline HbA1c Level 8.0% or higher  Frequent or severe hypoglycemia  Impaired awareness of hypoglycemia

3. Pregnant women or actively attempting to conceive and have a diagnosis of Type 1 diabetes

4. Children and adolescents under 21 with a diagnosis of Type 1 diabetes

Appropriate Treatment Regimen & Other Criteria: Exclusion Criteria:  Type 2 diabetes Age Restriction: Prescriber Restrictions:

Coverage Duration:  Authorization 12 months, unless otherwise specified

POLICY NAME: CORLANOR Affected Medications: CORLANOR (ivabradine) 5 mg and 7.5 mg tablets

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.  Inappropriate sinus tachycardia Required Medical Chronic heart failure Information:  Documentation of chronic heart failure with left ventricular ejection fraction (LVEF) 35% or less AND  Resting heart rate of at least 70 beats per minute (bpm)  Documentation of tried or currently receiving one beta blocker (metoprolol succinate extended release, carvedilol, or carvedilol extended release) at the maximally tolerated dose for heart failure treatment OR  Documentation of medical reason for avoidance of beta-blockers

Inappropriate sinus tachycardia  Heart rate of at least 100 beats per minute, with average mean heart rate of at least 90 beats per minute over 24 hours not due to appropriate physiologic response or primary abnormality (hyperthyroidism or anemia)  Symptomatic (palpitations, shortness of breath, dizziness, and/or decreased exercise capacity)  Documentation for absence of identifiable causes of sinus tachycardia and exclusion of atrial tachycardia Appropriate  Effective contraception is recommended in women of child-bearing age Treatment Regimen & Other  Reauthorization will require documentation of treatment success and a clinically Criteria: significant response to therapy; development of atrial fibrillation while on therapy will exclude patient from reauthorization Exclusion Criteria:  Acute, decompensated heart failure  Blood pressure less than 90/50 mm Hg  Resting heart rate of less than 60 bpm prior to treatment  Sick sinus syndrome, sinoatrial block, third-degree atrioventricular block (unless stable with functioning demand pacemaker)  Severe hepatic impairment (Child-Paugh class C)  Heart rate maintained exclusively by pacemaker  Concomitant use with strong CYP3A4 inhibitors/inducers Age Restriction:  18 years or older Prescriber  Prescribed by or in consultation with a cardiologist Restrictions:

Coverage  Initial Authorization: 12 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: COTELLIC Affected Medications: COTELLIC (cobimetinib fumarate)

Covered Uses:  NCCN indications with evidence level 2A or higher.

Required Medical  Documentation of performance status, disease staging, all prior therapies used, Information: and prescribed treatment regimen

Unresectable or metastatic melanoma:  Documentation of BRAF V600E or V600K mutation  Documentation of use in combination with venurafenib Appropriate Treatment  Reauthorization requires documentation of disease responsiveness to therapy Regimen & Other Criteria: Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber Restrictions:  Oncologist Coverage Duration:  Initial approval: 3 months  Reauthorization: 12 months

POLICY NAME: CRESEMBA Affected Medications: CRESEMBA (isavuconazonium sulfate)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  Diagnosis of Invasive Aspergillosis Information:  Diagnosis of Invasive Mucormycosis Aspergillosis:  Documented treatment failure or contraindication to voriconazole Mucormycosis:  Documented treatment failure or contraindication to amphotericin B

Appropriate All Indications: Treatment  Susceptibility cultures matching isavuconazonium activity Regimen & Other  Exceptions made for empiric therapy as long as treatment is adjusted when Criteria: susceptibility cultures are available

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Concurrent use of strong CYP3A4 inhibitors (ketoconazole, high-dose ritonavir [400 mg every 12 hours]) and strong CYP3A4 inducers (rifampin, carbamazepine, St. John’s Wort, long-acting barbiturates) (Hypericum perforatum)  Familial short QT syndrome Age Restriction: Prescriber Restrictions:

Coverage  Initial approval: 3 months Duration:  Reauthorization: 3 months

POLICY NAME: CRYSVITA Affected Medications: CRYSVITA (burosumab-twza)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.

Required Medical X-Linked Hypophosphatemia Information:  Documentation of diagnosis by:  A blood test demonstrating: . Decreased phosphate AND . Increased FGF-23 AND . Decreased 1,25-(OH)2D AND . Normal parathyroid hormone (PTH) AND  A urine test demonstrating:  Decreased tubular reabsorption of phosphate corrected for glomerular filtration rate (TmP/GFR)

Appropriate  Documentation of trial/failure with oral phosphate and calcitriol supplementation. Treatment  Dosing Regimen & Other o Adults, Criteria: . Initial: 1 mg/kg, rounded to nearest 10 mg, subQ every 4 weeks; up to maximum of 90 mg every 4 weeks. o Pediatrics, . Initial: 0.8 mg/kg rounded to nearest 10 mg, subQ every 2 weeks; up to a maximum of 90 mg, or 2 mg/kg every 4 weeks.  Reauthorization: requires documentation of normalization of serum phosphate levels.

Exclusion Criteria:  Oral phosphate and active vitamin D analogs within the last week  Renal impairment and/or end stage renal disease

Age Restriction:  Patients is at least 1 year of age Prescriber  Must be administered by a healthcare provider. Restrictions:  Prescribed by or in consultation with a Nephrologist or Endocrinologist Coverage Duration:  Initial approval: 6 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: CYSTADANE Affected Medications: CYSTADANE (betaine)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Documentation of one of the following: Information:  Cystathionine beta-synthase (CBS) deficiency  5,10-methylenetetrahydrofolate reductase (MTHFR) deficiency  Cobalamin cofactor metabolism (cbl) defect  Vitamin B12 and folic acid serum levels Appropriate Treatment  Vitamin B6, B12, and folate supplementation Regimen & Other Criteria: Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Uncorrected vitamin B12 or folic acid levels Age Restriction: Prescriber Restrictions: Coverage Duration:  Approval: 12 months, unless otherwise specified.

POLICY NAME: CYSTAGON Affected Medications: CYSTAGON (cysteamine bitartrate)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.

Required Medical  Diagnosis of nephropathic cystinosis Information:  The diagnosis was confirmed by the presence of increased cysteine concentration in leukocytes (generally 3-23 nmol half-cystine/mg protein) or by DNA testing (mutations in the CTNS gene) or by demonstration of cysteine corneal crystals by the slit lamp examination Appropriate Treatment Regimen & Other Criteria: Exclusion  Documented history of hypersensitivity to cysteamine or penicillamine Criteria:

Age Restriction:

Prescriber Restrictions:

Coverage  Approval: 12 months unless otherwise specified Duration:

POLICY NAME: DALIRESP Affected Medications: DALIRESP (roflumilast)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical Information:  Documentation of Stage III, or Stage IV COPD  Documentation of recent FEV1, and FVC.  Documentation of current treatment regimen.

Appropriate Treatment  Documentation that this product is being used in combination with a Regimen & Other Criteria: long acting anti-muscarinic agent or a long acting bronchodilator that is approved for the treatment of COPD

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Moderate or severe hepatic impairment (Child-Pugh class B or C).

Age Restriction:

Prescriber Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified

POLICY NAME: DEFITELIO Affected Medications: DEFITELIO (defibrotide sodium)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical  Diagnosis of hepatic veno-occlusive disease (VOD), also known as sinusoidal obstruction Information: syndrome (SOS), AND  Renal and/or pulmonary dysfunction following hematopoietic stem cell transplantation (HSCT) AND  Weight prior to HSCT, dose and frequency AND  Renal function data o Serum creatinine (SCr) prior to admission for HSCT conditioning, during conditioning before HSCT, or Creatinine clearance (CrCl) or glomerular filtration rate (GFR) prior to admission o Current SCr, CrCl, or GFR  Pulmonary function data o Oxygen saturation on room air or requirement for oxygen supplementation/ventilator dependence

Reauthorization Criteria  21 days of therapy have been completed AND  Total bilirubin level is still above normal (normal varies by lab, ~0.1-1.2 mg/dL or 1.71- 20.5 microM/L) Appropriate Treatment Regimen & Other Criteria: Exclusion Criteria:  Renal dysfunction secondary to an alternate etiology  Insufficiently severe renal dysfunction defined as: o SCr less than 3x the value at admission for HSCT conditioning OR o SCr less than 3x the lowest value during conditioning before HSCT OR o CrCl or GFR greater than 40% of admission value OR o Not dialysis dependent after HSCT  Pulmonary dysfunction secondary to an alternate etiology  Insufficiently severe pulmonary dysfunction o Oxygen saturation greater than 90% on room air OR o No documented requirement for oxygen supplementation/ventilator dependence  Preexisting liver cirrhosis

 Any of the following without diagnosis of VOD or SOS with renal or pulmonary dysfunction following HSCT: hyperbilirubinemia, ascites, weight gain, and/or hepatomegaly  Prior solid organ transplant  Dialysis dependence at the time of HSCT  Oxygen dependence during conditioning  Hemodynamic instability (requirement for multiple pressors or inability to maintain mean arterial pressure with single-pressor support).  Concomitant use of medications increasing hemorrhagic risk (e.g. anticoagulants and/or fibrinolytics)  Presence of active bleeding Age Restriction:  Prescriber  Restrictions: Coverage  Authorization: 1 month Duration:  Reauthorization: 2 weeks, may only reauthorize total of two times

POLICY NAME: DESMOPRESSIN Affected Medications: DDAVP, STIMATE (desmopressin)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  Diagnosis of central Diabetes insipidus OR Information:  Diagnosis of Hemophilia A OR  Von Willebrand Disease AND  Documentation of complete and current treatment course Appropriate  Desmopressin is ineffective for treatment of nephrogenic diabetes insipidus Treatment  Desmopressin is not indicated for the treatment of hemophilia A with factor VIII Regimen & Other coagulant activity levels less than or equal to 5%, for the treatment of hemophilia B, or Criteria: in patients with factor VIII antibodies  Documentation of appropriate use

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Tablet, Injection: hyponatremia or history of hyponatremia; moderate-to-severe renal impairment (CrCl less than 50 ml/minute)  Prior intolerance or allergic reaction to requested medication  Below the line (BTL) indications: o primary nocturnal enuresis o polyuria o polydipsia

Age Restriction: Prescriber Restrictions: Coverage Duration:  Approval: 12 months, unless otherwise specified.

POLICY NAME: DIABETIC TEST STRIPS Affected Medications: DIABETIC TEST STRIPS (FREESTYLE, FREESTYLE LITE, FREESTYLE INSULINX, FREESTYLE PRECISION NEO)

Covered Uses:  All FDA approved indications not otherwise excluded by plan design  Diabetes Mellitus (DM) Required Medical  Documentation of complete & current treatment course Information: Appropriate Treatment Preferred products must be prescribed (If a patient requires a new meter, please Regimen & Other Criteria: call 541-330-4999):  Freestyle  Freestyle Lite  Freestyle InsuLinx  Freestyle Precision Neo

Standard Quantity Limits:  Insulin dependent DM: #100 test strips per 25 days (4x/day)  Non-insulin dependent DM: #100 test strips per 25 days (4x/day)

Quantity Limit exceptions:  Uncontrolled (HbA1c >10), new onset, or gestational: #150 test strips per 25 days (6x/day)  New onset Pediatric DM or Insulin Pump Start: #250 test strips per 25 days (10x/day)

Exclusion Criteria:

Age Restriction:

Prescriber Restrictions: Coverage Duration:  Approval: 12 months

POLICY NAME: DIACOMIT Affected Medications: Diacomit capsules, Diacomit powder for suspension

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. o Seizures associated with Dravet syndrome (DS) in patients 2 years of age and older taking clobazam Required Medical  Patient Weight Information:  Documentation that therapy is being used as adjunct to clobazam for seizures  Documentation of at least 4 generalized clonic or tonic-clonic seizures in the last month while on stable antiepileptic drug therapy  Documented treatment and inadequate control of seizures with at least four guideline directed therapies including: o Valproate and o Onfi and o Topiramate and o Clonazepam, levetiracetam, or zonisamide Appropriate Treatment  Dosing: 50 mg/kg/day, in 2 or 3 divided doses, not to exceed 3,000mg/day Regimen & Other Criteria:  Reauthorization will require documentation of at least 50% reduction in generalized clonic or tonic-clonic seizure frequency

Exclusion Criteria:

Age Restriction:  2 years of age or older

Prescriber  Prescribed by or in consultation with a neurologist Restrictions: Coverage Duration:  Initial Authorization: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: DOPTELET Affected Medications: DOPTELET (avatrombopag maleate)

Covered Uses:  All FDA approved indications not otherwise excluded by plan design. Required Medical  Complete blood count with differential and platelet count Information:  Liver function tests  Documentation of planned procedure including date  Documentation of current platelet count is required for prescribing

Appropriate Thrombocytopenia in patients with Chronic Liver Disease undergoing medical or dental Treatment procedures Regimen & Other  Dosage as either: Criteria:  Platelet count less than 40 x 109/L: 60 mg orally once daily with food for 5 consecutive days beginning 10 to 13 days prior to scheduled procedure OR  Platelet count 40 to less than 50 x 109/L: 40 mg orally once daily with food for 5 consecutive days beginning 10 to 13 days prior to scheduled procedure

 Documented inability to respond adequately to Promacta  Consideration for reapproval of therapy requires response to treatment with platelet count of at least 50,000/mcL or above without significant liver function abnormalities during procedure

Exclusion Criteria:  Platelet count above 50,000/mcL at baseline  History of thrombosis  Platelet transfusion or receipt of blood containing platelets within 7 days of screening for procedure  Use of heparin, warfarin, NSAIDs, ASA, verapamil, or antiplatelet therapy with ticlopidine or glycoprotein IIb/IIIa antagonists, or erythropoietin stimulating agents within 7 days of screening for procedure Age Restriction: • 18 years of age or older Prescriber  Prescribed by or in consultation with hematologist or gastroenterologist Restrictions:

Coverage Duration:  Approval: 1 month (5 days of treatment maximum), unless otherwise specified

POLICY NAME: DUOPA Affected Medications: DUOPA (carbidopa/levodopa enteral suspension)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  Diagnosis of idiopathic Parkinson’s Disease (PD) based on presence of bradykinesia and Information: at least one other cardinal PD feature (tremor, rigidity, postural instability) AND  Levodopa responsive with clearly defined “On” periods AND  Persistent motor complications with disabling “Off” periods for a minimum of 3 hours/day, despite optimal medical therapy with oral levodopa-carbidopa, and at least two other classes of anti-PD therapy (i.e. COMT, MAO-B inhibitor, or dopamine agonist) Appropriate  Duopa is delivered as a 16-hour infusion through either a naso-jejunal tube for SHORT- Treatment term administration or through a PEG-J for LONG-term administration Regimen & Other Criteria: Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion  Atypical Parkinson’s syndrome (“Parkinson’s Plus” syndrome) or secondary Parkinson’s Criteria:  Non-levodopa responsive PD  Contraindication to percutaneous endoscopic gastro-jejunal (PEG-J) tube placement or long-term use of a PEG-J  Concomitant use with nonselective MAO inhibitors or have recently (within 2 weeks) taken a nonselective MAO inhibitor Age Restriction:

Prescriber  Prescribed by a neurologist Restrictions: Coverage  12 months, unless otherwise specified Duration:

POLICY NAME: DUPIXENT Affected Medications: DUPIXENT (dupilumab)

Covered Uses:  All FDA approved indications not otherwise excluded by plan design. o Treatment of patients greater than or equal to 12 years of age with moderate-to- severe atopic dermatitis whose disease is not adequately controlled with topical prescription therapies or when those therapies are not advisable o Add-on maintenance treatment in patients with moderate-to-severe asthma aged 12 years and older with an eosinophilic phenotype or with oral corticosteroid dependent asthma. Required Medical Atopic Dermatitis (AD) Information: Meet ALL of the following:  Clinical documentation of severe inflammatory skin disease defined as having functional impairment (e.g. inability to use hands or feet for activities of daily living, or significant facial involvement preventing normal social interaction) despite current treatment AND  Body Surface Area (BSA) of at least 10% OR hand, foot or mucous membrane involvement

Asthma  A history of 2 or more asthma exacerbations requiring oral or systemic corticosteroid treatment in the past 12 months OR  Requiring chronic daily oral corticosteroid use  Reduced lung function at baseline [pre-bronchodilator FEV1 below 80% in adults, and below 90% in adolescents] despite regular treatment with high dose inhaled corticosteroid (ICS) plus a long-acting beta agonist (LABA)  A baseline blood eosinophil count of at least 300 cells/μL Appropriate Atopic Dermatitis (AD) Treatment  Failed therapy with tacrolimus 0.1% ointment for 6 weeks AND Regimen & Other  Documented failure with two of the following alternatives: Phototherapy, Cyclosporine, Criteria: Azathioprine, Methotrexate for a minimum of 12 weeks.

 Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Dosing  AD: Initial dose of 600 mg (two 300 mg injections), followed by 300 mg given every other week  Asthma: Initial dose of 400 mg (two 200 mg injections) followed by 200 mg given every other week or initial dose of 600 mg (two 300 mg injections) followed by 300 mg given every other week Exclusion  Atopic dermatitis not meeting the above criteria is considered a below the line diagnosis Criteria: per Oregon Health Authority (OHA). Please refer to OHA GUIDELINE NOTE 21, SEVERE INFLAMMATORY SKIN DISEASE Line 430. 85

Age Restriction:  AD: 12 years of age or older  Asthma: 12 years of age or older Prescriber  Prescribed by, or in consultation with, a specialist, (e.g., dermatologist, allergist or Restrictions: immunologist)

Coverage  Initial approval: 4 months, unless otherwise specified; Initial PA: Please approve 6 mLs Duration: (three 300 mg/2mL syringes) for the first 30 days then maintenance dose of 4 mL/28 days  Reauthorization: 6 months, unless otherwise specified

POLICY NAME: ELAPRASE Affected Medications: ELAPRASE (idursulfase)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design Required  Diagnosis of Hunter syndrome or (Mucopolysaccharidosis type II, (MPS II)) Medical  Diagnosis confirmed by enzyme assay demonstrating a deficiency of iduronate 2-sulfatase Information: enzyme activity or by DNA testing that shows pathologic iduronate 2-sulfatase gene mutation  Documentation of baseline values for 6-minute walk test (6-MWT) and/or percent predicted forced vital capacity (FVC)  Must have symptoms attributable to MPS II such as: developmental delay, cognitive impairment, frequent infections, hearing loss, hepatosplenomegaly, hernias, impaired respiratory function, joint pain, skeletal deformities, sleep apnea or valvular heart disease Appropriate  In case of anaphylaxis or severe allergic reaction, there will be appropriate medical Treatment support readily available when Elaprase is administered Regimen &  QL- 0.5 mg/kg infusion once weekly Other Criteria:

Reauthorization: Documentation of clinical response and toleration of agent -Clinical Response: Demonstrated a response to therapy compared to pretreatment baseline: stabilization or improvement in 6-MWT and/or FVC AND -Toleration of agent: absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include the following: severe hypersensitivity including anaphylactic reactions, antibody development and serious adverse reactions, acute respiratory complications, acute cardiorespiratory failure, etc Exclusion Criteria:

Age Restriction:  5 years of age and older Prescriber  Prescribed by or in consultation with a physician who specializes in the treatment of Restrictions: inherited metabolic disorders

Coverage  Initial approval 3 months, unless otherwise specified Duration:  Subsequent approval 12, months unless otherwise specified

POLICY NAME: ELELYSO Affected Medications: ELELYSO (taliglucerase alfa)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  Diagnosis of Type 1 Gaucher Disease Information:  Diagnosis confirmed by enzyme assay demonstrating a deficiency of beta- glucocerebrosidase enzyme activity  At least one of the following disease complications: anemia, thrombocytopenia, bone disease, hepatomegaly, or splenomegaly Appropriate Treatment Reauthorization will require documentation of treatment success and a clinically Regimen & Other significant response to therapy Criteria: Exclusion Criteria:  Patients currently taking miglustat (Zavesca) Age Restriction:  4 years of age or older Prescriber Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified

POLICY NAME: ELIQUIS Affected Medications: Eliquis (Apixaban)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Age, body weight, and a recent serum creatinine. Information: o Dose should be reduced to 2.5 mg twice daily, if the patient has two or more of the following: o Age > 80 years, o body weight < 60 kg, or o SCr > 1.5 mg/dL

Appropriate  Authorize use of Eliquis for patients with: non-valvular atrial fibrillation or flutter or Treatment prophylaxis of deep vein thrombosis/pulmonary embolism after hip or knee Regimen & Other replacement surgery. Criteria:  Additional indications evaluated by a pharmacist and/or a physician on a case-by-case basis.

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  CrCl < 15 mL/minute.

Use in the management of acute coronary syndromes,  primary and secondary prevention of chronic coronary arter disease (eg, unstable angina, non-ST segment elevation myocardial infarction [MI], ST-elevation MI, post percutaneous coronary intervention or intracoronary stent placement),  prevention of thromboembolic events related to hereditary factor deficiencies or clotting disorders (eg, protein S deficiency, hereditary antithrombin deficiency), or  Management of mechanical heart valves or indwelling cardiac assistive devices. Age Restriction:  Age ≥ 18 Prescriber Restrictions: Coverage  Approval: 12 months, unless otherwise specified. Duration:

POLICY NAME: ELZONRIS Affected Medications: ELZONRIS (tagraxofusp-erzs)

Covered Uses:  Food and Drug Administration (FDA)-approved indications not otherwise excluded by plan design o Treatment of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in adults and in pediatric patients at least 2 years of age Required Medical  Diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN) made by a board Information: certified Hematopathologist or Dermatopathologist.  If diagnosis of BPDCN is based on skin biopsy, clear plasmacytoid dendritic blast cells are present by morphology and confirmed by IHC and flow cytometry. Features excluding AML and Leukemia cutis must be present.  If BPDCN presents as the leukemic form or it there is bone marrow involvement, AML, T-cell lymphoblastic leukemia, and NK-cell Leukemia must be excluded.  Documentation of performance status, disease staging, all prior therapies used, and anticipated treatment course.

Appropriate  The recommended dose and schedule is 12 mcg/kg administered intravenously over 15 Treatment minutes once daily on day 1 to 5 of a 21-day cycle. Regimen & Other Criteria: Reauthorization: documentation of disease responsiveness to therapy

Exclusion Criteria:  Renal toxicity: Withhold tagraxofusp until serum creatinine is ≤1.8 mg/dL or CrCl is ≥60 mL/minute.  Hepatotoxicity: Withhold tagraxofusp until AST and/or ALT are ≤2.5 times ULN  Persistent clinically significant toxicities from prior chemotherapy  Receiving immunosuppressive therapy  Pregnancy Age Restriction:  For adults and pediatric patients 2 years and older only Prescriber  Must be prescribed by or in consultation with a prescriber experienced in the treatment Restrictions: of BPDCN

Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: ENBREL Affected Medications: ENBREL (etanercept); ENBREL MINI

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required  Documentation of moderate to severe disease while on treatment (Indication must be Medical documented in chart notes within the last 6 months) Information:  Documentation of complete and current treatment course  Documented current level of disease activity/disease control

Rheumatoid arthritis (RA)  Documented current level of disease activity with one of the following (or equivalent objective scale): o The Disease Activity Score derivative for 28 joints (DAS-28) greater than 3.2 o The Simplified Disease Activity Index (SDAI) greater than 11 o The Clinical Disease Activity Index (CDAI) greater than 10 o Weighted RAPID3 of at least 2.3

Juvenile idiopathic arthritis (JIA)  Documented current level of disease activity with physician global assessment (MD global score) or active joint count

Plaque psoriasis (PP)  Documentation of disease that is severe in nature, which has resulted in functional impairment (e.g., inability to use hands or feet for activities of daily living, or significant facial involvement preventing normal social interaction) AND one or more of the following: o At least 10% body surface area involvement; or o Hand, foot or mucous membrane involvement

Psoriatic Arthritis (PsA)  Documentation of CASPAR criteria score greater than 3 based on chart notes: o Skin psoriasis: present – two points, OR previously present by history – one point, OR a family history of psoriasis, if the patient is not affected – one point o Nail lesions (onycholysis, pitting): one point o Dactylitis (present or past, documented by a rheumatologist): one point o Negative rheumatoid factor (RF): one point o Juxtaarticular bone formation on radiographs (distinct from osteophytes): one point

o Inflammatory back pain (4 of 5 features met): . Onset of back discomfort before the age of 40 years . Insidious onset . Improvement with exercise . No improvement with rest . Pain at night (with improvement upon arising) o Arthritis o Enthesitis o Uveitis o Dactylitis (inflammation of entire digit) o Psoriasis o Crohn’s disease/ulcerative colitis o Good response to NSAIDs o Family history of SpA o Elevated CRP OR o HLA-B27 genetic test positive AND at least 2 SpA features

Rheumatoid arthritis (RA) Appropriate o Documented clinical failure with at least 12 weeks of combination disease-modifying Treatment antirheumatic drug (DMARD) therapy [Methotrexate plus sulfasalazine, methotrexate Regimen & plus hydroxychloroquine, sulfasalazine plus hydroxychloroquine, leflunomide plus Other Criteria: sulfasalazine, or leflunomide plus hydroxychloroquine] o QL – 4 ml (4 pens) per 28 day supply

Juvenile idiopathic arthritis (JIA) o Documented failure with glucocorticoid joint injections or oral corticosteroids AND o At least one of the following agents for a minimum of 12 weeks: methotrexate, leflunomide o QL – 4 ml (4 pens) per 28 day supply

Psoriatic arthritis (PsA) o Failure of at least 12 weeks with methotrexate, or if unable to tolerate or contraindicated to methotrexate, another disease modifying antirheumatic drug (sulfasalazine, cyclosporine, leflunomide) o QL – 4 ml (4 pens) per 28 day supply

Plaque psoriasis (PP) o Documented failure with 12 weeks of at least two systemic therapies (methotrexate, cyclosporine, acitretin, phototherapy [UVB, PUVA]) o QL – Initial – 8 ml (8 pens) per 28 days for 3 month supply o QL – Continuation – 4 ml (4 pens) per 28 day supply

Graft Versus Host Disease (GVHD)

o Managed by transplant center AND has tried or currently receiving conventional treatment with high dose SC, CSA, tacrolimus, etc.

Reauthorization: Documentation of treatment success and a clinically significant response to therapy

Ankylosing spondylitis and Psoriatic Arthritis (PsA) with Axial Involvement o Documented failure with two daily prescription strength nonsteroidal anti-inflammatory drugs (ibuprofen, naproxen, diclofenac, meloxicam, etc.) with minimum 1 month trial each o For isolated sacroiliitis, enthesitis, peripheral arthritis: documented treatment failure with locally administered parenteral glucocorticoid o QL – 4mL (4 pens) per 28 day supply Reauthorization: Improvement of at least 50% in the BASDAI score or an absolute change of 2 units. Exclusion  Concurrent us of biologic DMARDs: Orencia (abatacept), Rituxan (rituximab), Actemra Criteria: (tocilizumab), Humira (adalimumab), Remicade (infliximab), Simponi (golimumab), Cimzia (certolizumab), Kineret (anakinra), Xeljanz (tofacitinib)  Active infection including tuberculosis  Intra-articular injection of Enbrel  Positive tuberculosis, hepatitis B, hepatitis C or herpes zoster infection  Use in the management of alopecia areata, alopecia totalis, alopecia universalis, asthma, Crohn's disease, dermatomyositits/polymyositis, inclusion body myositis, Graves ophthalmopathy, hepatitis C, alcoholic hepatitis, idiopathic pulmonary fibrosis, immune- mediated cochleovestibular disorders, immune thrombocytopenic purpura, myelodysplastic syndrome, prevention of peri-prosthetic osteolysis, primary sclerosing cholangitis, recurrent spontaneous pregnancy loss, ocular sarcoidosis, pulmonary sarcoidosis, sciatica, Sjogren's syndrome, Takayasu's arteritis, Wegener's granulomatosis, cancer anorexia/weight loss syndrome, new-onset diabetes mellitus type 1, keloids, and Alzheimer's disease, myositis, nephrotic syndrome, septic shock/sepsis, uveitis Age  PA, PsA, PP, AS: 18 years of age or older Restriction:

Prescriber  PP: prescribed by or in consultation with a dermatologist Restrictions:  RA/PsA/AS: prescribed by or in consultation with a rheumatologist

Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: ENTERAL NUTRITION/ORAL NUTRITION SUPPLEMENTS Affected Medications: ENTERAL NUTRITION

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Nutritional Deficiency identified by one of the following: Medical  Documentation of chronic and permanent illness/trauma resulting in inability to be Information: maintained through oral feeding and must rely on enteral/parenteral nutrition therapy. (i.e. permanent enteral/parenteral prosthetic device is required) OR  Documentation of functioning GI tract who, due to pathology to, or non-function of, the structures that normally permit food to reach the digestive tract (oral feeding), cannot maintain weight and strength commensurate with his/her general condition. (ex. head/neck cancer with reconstructive surgery and CNS disease leading to interference with the neuromuscular mechanism) OR  Documentation of use for training in the ketogenic diet for children with epilepsy in cases where the child has failed or not tolerated conventional therapy

Oral nutritional supplements may be approved when the following criteria has been met:

Clients age 6 and above:  Must have a nutritional deficiency identified by one of the following: o Recent low serum protein levels OR o Recent registered dietician assessment shows sufficient caloric/protein intake is not obtainable through regular, liquefied or pureed foods OR o Must meet all of the following: . Prolonged history (i.e. years) of malnutrition, and diagnosis or symptoms of cachexia . Client residence in home, nursing facility, or chronic home care facility . Where the above conditions be futile and invasive AND  Must have a recent unplanned weight loss of at least 10%, PLUS one of the following: o Increased metabolic need resulting from severe trauma OR o Malabsorption difficulties (e.g., short-gut syndrome, fistula, cystic fibrosis, renal dialysis) OR o Ongoing cancer treatment, advanced Acquired Immune Deficiency Syndrome (AIDS) or pulmonary insufficiency. Note: Weight loss criteria may be waived if body weight is being maintained by supplements due to patient’s medical condition (e.g., renal failure, AIDS)

Clients under age 6:  Diagnosis of failure to thrive AND  Must meet same criteria as above, with the exception of % of weight loss.

Appropriate Treatment Regimen & Other Criteria: Exclusion Criteria:

Age Restriction: Prescriber  Practitioner licensed to prescribe medications Restrictions: Coverage  Initial approval: 6 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: ENTYVIO Affected Medications: Entyvio (vedolizumab)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design

Required Medical  Documentation of moderate to severe disease despite current treatment (Indication Information: must be documented in chart notes within the last 6 months )  Documentation of complete and current treatment course  Documented current level of disease activity/disease control

Crohn’s disease  Documentation of Crohn’s Disease Activity Index (CDAI) greater than 220 Ulcerative Colitis  Diagnosis supported by endoscopy/colonoscopy/sigmoidoscopy or biopsy and a Mayo Clinic Score for Assessment of Ulcerative Colitis Activity Appropriate Crohn’s disease Treatment  Has failed at least two oral treatment for minimum of 12 weeks trial: corticosteroids, Regimen & Other azathioprine, cyclosporine, 6-mercaptopurine, methotrexate, sulfasalazine, balsalazide, Criteria: tacrolimus, mesalamine AND  Has failed minimum 12 weeks on Humira  Dosing of 300mg every 8 weeks

Ulcerative Colitis  Has failed at least two oral treatment for minimum of 12 weeks trial: corticosteroids, sulfasalazine, azathioprine, mesalamine, balsalazide, cyclosporine, 6-mercaptopurine AND  Has failed minimum 12 weeks on Humira  Dosing of 300mg every 8 weeks

For consideration of every 4 week dosing, must meet all of the following:  Documented clinical failure to Entyvio at standard dosing for at least 6 months o Clinical failure defined as failure to achieve a clinical response (greater than or equal to 70 point improvement in CDAI score for Crohn’s)  Documented failure to minimum of 12 weeks on two alternative Tumor necrosis factor- alpha (TNF) inhibitors

Reauthorization: documentation of treatment success Exclusion  Concurrent use with or tumor necrosis factor (TNF) alfa inhibitors (eg, infliximab, Criteria: adalimumab, certolizumab pegol) Age Restriction:  18 years of age or older Prescriber  Prescribed by or in consultation with a gastroenterologist specialist Restrictions:

Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: EPIDIOLEX Affected Medications: Epidiolex (cannabidiol)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design

Required Medical  Documented diagnosis of Lennox-Gastaut syndrome (LGS) or Dravet syndrome (DS) Information:  Patient Weight  Documentation that therapy is being used as adjunct therapy for seizures

 Lennox-Gastaut syndrome (LGS) . Documentation of at least 8 drop seizures per month . Documented treatment and inadequate control of seizures with at least three guideline directed therapies including  Valproate and  Lamotrigine and  Rufinamide, topiramate, flebamate, or Onfi  Dravet Syndrome (DS) . Documentation of at least 4 convulsive seizures in the last month while on stable antiepileptic drug therapy . Documented treatment and inadequate control of seizures with at least four guideline directed therapies including  Valproate and  Onfi and  Topiramate and  Clonazepam, levetiracetam, or zonisamide Appropriate Dosing: Not to exceed 20mg/kg daily Treatment Regimen & Other Reauthorization will require documentation of treatment success and a clinically Criteria: significant response to therapy

Exclusion Criteria: Age Restriction:  2 years of age or older Prescriber  Prescribed by or in consultation with a neurologist Restrictions: Coverage Duration:  Initial approval: 6 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

POLICY NAME: EPOPROSTENOL Affected Medications: Epoprostenol, Veletri, Flolan

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical Pulmonary arterial hypertension (PAH) WHO Group 1 Information:  Documentation of PAH confirmed by right-heart catheterization  Documentation of acute vasoreactivity testing  Patient weight, planned dose and frequency Appropriate  PAH: for initiation of therapy patient must have mean pulmonary artery pressure at Treatment least 25mm Hg at rest or at least 30 mm Hg with exertion Regimen & Other AND Criteria:  Failure of the following therapy classes: PDE5 inhibitors AND  Endothelin receptor antagonists (exception for severe disease, WHO class IV)  Subsequent approval requires documentation of treatment success: exercise endurance, echocardiographic testing, hemodynamic testing, BNP, functional class

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Folan: Heart failure caused by reduced left ventricular ejection fraction  Veletri: Long-term use in patients with heart failure due to severe left ventricular systolic dysfunction; long-term use patients who develop pulmonary edema during dose initiation

Age Restriction:  18 years of age and older

Prescriber  Prescribed by or in consultation with cardiologist or pulmonologist Restrictions:

Coverage Duration:  Initial coverage, 3 months, unless otherwise specified  Subsequent coverage, 12 months unless otherwise specified

POLICY NAME: ERGOT ALKALOIDS Affected Medications: Dihydroergotamine Mesylate Injection, Dihydroergotamine Mesylate Nasal Solution

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical  Request for injection: documentation of status migrainosus Information:  Request for nasal solution: documentation of migraines described as being moderate-severe AND  Documentation of inadequate response or contraindication to all of the following: - Minimum of two prescription strength NSAIDs or combination analgesics (e.g. ibuprofen, naproxen, or acetaminophen plus aspirin plus caffeine) - Minimum of 1 oral 5-hydroxytryptamine-1 (5HT1) receptor agonists (e.g. sumatriptan, naratriptan, rizatriptan, or zolmitriptan) - Minimum of 1 NON-oral 5HT1 agonist (e.g. sumatriptan, zolmitriptan) Appropriate  Injection doses should not exceed 3 mg in a 24 hour period, and 6 mg in one week Treatment  QL 12mL/30 days Regimen & Other  Nasal solutions should not exceed 2 mg per day, no additional benefit shown Criteria:  QL 2mL/30 days (or 8mg/30 days)

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Hemiplegic or basilar migraine  Uncontrolled hypertension  Ischemic heart disease (e.g. angina pectoris, history of myocardial infarction, history of silent ischemia)  Peripheral artery disease  Pregnancy or breastfeeding  Documented severe chronic liver disease  Severe renal impairment  Use in combination with 5HT1 receptor agonist such as sumatriptan Age Restriction:  Patients 18 years and older Prescriber Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified

100

POLICY NAME: ERIVEDGE Affected Medications: ERIVEDGE (vismodegib)

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, all prior therapies used, and prescribed Information: treatment regimen.  Documentation that Erivedge is being used as a NCCN 2A level of evidence regimen Appropriate Treatment  Documentation of surgery performed or reason why the patient is not a Regimen & Other Criteria: candidate for surgery  Documentation of why the patient is not a candidate for radiation  Reauthorization requires documentation of treatment success

Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber Restrictions:  Oncologist

Coverage Duration:  Initial approval: 3 months (2 week initial partial fill), unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

101

POLICY NAME: ERYTHROPOIESIS STIMULATING AGENTS (ESAs) Affected Medications: Aranesp (darbepoetin alfa), Epogen (epoetin alfa), Mircera (methoxy polyethylene glycol-epoetin beta), Procrit (epoetin alfa)

Covered Uses:  All FDA (Food and Drug Administration)-approved indications not otherwise excluded by plan design Epogen & Procrit & Aranesp & Mircera  Treatment of anemia due to chronic kidney disease (CKD), including patients on dialysis and not on dialysis to decrease the need for red blood cell (RBC) transfusion Epogen & Procrit & Aranesp  Treatment of anemia in patients with non-myeloid malignancies where anemia is due to the effect of concomitant myelosuppressive chemotherapy, and upon initiation, there is a minimum of two additional months of planned chemotherapy Epogen & Procrit only  To reduce the need for allogeneic RBC transfusions among patients with perioperative hemoglobin greater than 10 to 13 or less g/dL who are at high risk for perioperative blood loss from elective, noncardiac, nonvascular surgery  Treatment of anemia due to zidovudine administered at ≤ 4200 mg/week in patients with HIV-infection with endogenous serum erythropoietin levels of ≤ 500 mUnits/mL Compendia-supported uses  Symptomatic anemia in Myelodysplastic syndrome  Allogenic bone marrow transplantation  Anemia associated with Hepatitis C (HCV) treatment  Anemia associated with rheumatoid arthritis (RA)/ rheumatic disease

Required Medical  One of the following in accordance with FDA (Food and Drug Administration)-approved Information: label or compendia support: o Anemia associated with chronic renal failure o Anemia secondary to chemotherapy with a minimum of two additional months of planned chemotherapy o Anemia secondary to zidovudine-treated Human Immunodeficiency Virus (HIV) patients o Anemia in patients scheduled to undergo elective, non-cardiac, nonvascular surgery o Symptomatic anemia in Myelodysplastic syndrome o Allogenic bone marrow transplantation o Anemia associated with Hepatitis C (HCV) treatment o Anemia associated with rheumatoid arthritis (RA)/ rheumatic disease Appropriate  Coverage for the non-preferred drugs (Epogen, Procrit, Aranesp, Mircera) is provided Treatment when any of the following criteria is met: Regimen & Other  For Epogen or Procrit, a documented intolerable adverse event to the preferred product Criteria: Retacrit, and the adverse event was not an expected adverse event attributed to the active ingredient 102

 For Aranesp or Mircera, a documented inadequate response or intolerable adverse event to the preferred product, Retacrit  Currently receiving treatment with either Aranesp or Mircera, excluding via samples or manufacturer’s patient assistance programs

Exclusion  Use in combination with another erythropoiesis stimulating agent (ESA) Criteria:

Age Restriction: Prescriber  Must be prescribed by, or in consultation with, a specialist (hematologist, oncologist, Restrictions: nephrologist)

Coverage  Approval: 6 months, unless otherwise specified Duration:

103

POLICY NAME: EXJADE, JADENU Affected Medications: EXJADE (deferasirox), JADENU (deferasirox)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required  Diagnosis of transfusion-dependent anemia with chronic iron overload due to blood Medical transfusions (transfusional hemosiderosis). Information:  Pretreatment serum ferritin level within the last 60 days of at least 1000 mcg/L Baseline and then monthly, or more frequently as indicated, monitoring of serum ferritin, serum creatinine/creatinine clearance, serum transaminases, bilirubin, and urinalysis (urine protein).  On renewal, for patients with serum ferritin below 500 mcg/L, temporary interruption of Exjade/Jadenu therapy should be considered.  For patients with persistent or severe increases in creatinine or liver function tests, the prescriber will consider dose modification or interruption of treatment.  Diagnosis of chronic iron overload in patients 10 years and older with nontransfusion- dependent thalassemia syndromes and with a liver iron concentration (LIC) of at least 5 mg of iron per gram of liver dry weight (mg Fe/g dw) and a serum ferritin greater than 300 mcg/L on at least 2 measurements 1 month apart Appropriate  Exjade/Jadenu should not be used with Desferal (deferoxamine) Treatment Regimen & Reauthorization will require documentation of treatment success and a clinically significant Other Criteria: response to therapy

Exclusion  CrCl < 40 mL/min. Criteria:  Severe hepatic impairment.  Platelet count <50,000/mcL  Patient with poor performance status and high-risk myelodysplastic syndrome (MDS) or advanced malignancies Age Restriction:  2 years of age and older for chronic iron overload due to blood transfusions  10 years of age and older for non-transfusion-depending thalassemia Prescriber  Hematologist Restrictions:

Coverage  12 months, unless otherwise specified Duration:

104

POLICY NAME: EXONDYS 51 Affected Medications: Exondys 51 (Eteplirsen)

Covered Uses:  Eteplirsen is not considered medically necessary due to insufficient evidence of therapeutic value Required Medical Information:

Appropriate Treatment Regimen & Other Criteria: Exclusion Criteria: Age Restriction: Prescriber Restrictions: Coverage Duration:

105

POLICY NAME: EXTAVIA Affected Medications: Extavia (interferon beta-1b)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Documentation of diagnosis of relapsing forms of multiple sclerosis confirmed Information: with MRI

Appropriate Treatment  No concurrent use of medications indicated for the treatment of relapsing- Regimen & Other remitting multiple sclerosis Criteria:  Not approved for primary and secondary progressive multiple sclerosis

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria: Age Restriction:

Prescriber Restrictions:  Prescribed by or after consultation with a Neurologist or an MS specialist. Coverage Duration:  Authorization will be for 12 months, unless otherwise specified.

106

POLICY NAME: FABRAZYME Affected Medications: FABRAZYME (agalsidase beta)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design Required  Diagnosis of Fabry disease Medical  Diagnosis confirmed by enzyme assay demonstrating a deficiency of alpha- Information: galactosidase enzyme activity or by DNA testing  The patient has clinical signs and symptoms of Fabry disease.  The patient is male OR  The patient is female and the patient has documented substantial disease manifestations (Renal dysfunction, Cardiovascular dysfunction, Cerebrovascular complications, Pulmonary complications, Neurologic/neuropathic dysfunction (pain) and diagnosis has been confirmed with genetic testing  Patient weight

Appropriate Reauthorization will require documentation of treatment success and a clinically Treatment significant response to therapy Regimen & Other Criteria: Exclusion Criteria: Age Restriction: Prescriber  Prescribed by or in consultation with a prescriber experienced in the treatment Restrictions: of Fabry disease

Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Subsequent approval: 12 months, unless otherwise specified

107

POLICY NAME: FARYDAK Affected Medications: Farydak (panobinostat)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design  NCCN indications with evidence level of 2A or higher Required Medical  Documentation of performance status, all prior therapies used, and prescribed Information: treatment regimen

Multiple Myeloma:  Documentation of progression on 2 prior regimens, including bortezomib and an immunomodulatory agent  Use in combination with dexamethasone and bortezomib Appropriate Treatment  Max duration 16 cycles Regimen & Other  Note Substrate for CYP3A4. Consider dose reduction with CYP3A4 inhibitors Criteria:  Note prolongation of QT interval  Reauthorization: documentation of disease responsiveness to therapy Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber Restrictions:  Oncologist Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 9 months , unless otherwise specified

108

POLICY NAME: FASENRA SUBCUTANEOUS Affected Medications: Fasenra (benralizumab)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical  A history of 3 or more asthma exacerbations requiring oral or systemic corticosteroid Information: treatment in the past 12 months OR  Requiring chronic daily oral corticosteroid use  Reduced lung function at baseline [pre-bronchodilator FEV1 below 80% in adults, and below 90% in adolescents] despite regular treatment with high dose inhaled corticosteroid (ICS) plus a long-acting beta agonist (LABA)  A baseline blood eosinophil count of at least 300 cells/μL Appropriate  Dosing: Administered once every 4 weeks for the first 3 doses, and then every 8 weeks Treatment thereafter. Maintenance dosing at every 4 weeks was studied but was not found to be Regimen & Other more effective than every 8 weeks. Criteria:  Reauthorization requires documented treatment success defined as a reduction in the frequency of exacerbations from baseline Exclusion Criteria:

Age Restriction:  Age 12 years and older Prescriber  Prescribed by or in consultation with an Allergist or Immunologist Restrictions:

Coverage  Initial approval: 6 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

109

POLICY NAME: FDA APPROVED DRUG – Below the Medicaid Line of Coverage Affected Medications: ALTRENO LOTION (tretinoin lotion), MINOLIRA (minocycline), MOTEGRITY (prucalopide), CEQUA (cyclosporine ophthalmic solution), ESKATA (Hydrogen Peroxide topical), MACRILEN (Macimorelin Acetate for oral solution 60mg), MOTEGRITY (prucalopride), NOCDURNA (desmopressin), OTIPRIO (ciprofloxacin suspension for otic administration), QBREXZA (glycopyrronium tosylate topical), SEYSARA (sarecycline), SINUVA (mometasone sinus implant)

Covered Uses:  FDA-approved indications not otherwise excluded by benefit design Required Medical  This medication is currently not covered by PacificSource Community Solutions as it is Information: below the OHA-funded line of the Prioritized List of Health Services.  To review the line as well as examine guidelines to see if patient meets certain criteria for approval, please refer to the following website https://intouch.pacificsource.com/LineFinder/.

Appropriate  Drug must be dosed according to package insert requirements Treatment Regimen & Other Criteria: Exclusion  Exclusion based on package insert requirements Criteria:

Age Restriction:  Age based on package insert requirements Prescriber  Prescriber restrictions based on package insert requirements Restrictions:

Coverage  Case by case Duration:

110

POLICY NAME: FDA APPROVED DRUG – Not Yet Reviewed By Plan for Formulary Placement Affected Medications: Not Yet Reviewed By Plan for Formulary Placement

Covered Uses:  FDA-approved indications not otherwise excluded by benefit design Required Medical  Documentation of disease state, level of control, and therapies failed Information:  Documentation of failure with all available formulary products for treatment of disease state  Documentation that delay in treatment will cause loss of life, limb, function or other extreme pain

Appropriate Treatment  Drug must be dosed according to package insert requirements Regimen & Other Criteria: Exclusion Criteria:  Exclusion based on package insert requirements Age Restriction:  Age based on package insert requirements Prescriber Restrictions:  Prescriber restrictions based on package insert requirements Coverage Duration:  Case by case based on member need

111

POLICY NAME: FERRIPROX Affected Medications: Ferriprox (Deferiprone)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Patient complete blood count including absolute neutrophil count (ANC) level Information:

Appropriate Treatment  Do not start treatment if patient is exhibiting signs of infection Regimen & Other  Upon renewal: If ANC 500/mm3-1500/mm3, treatment should be not be Criteria: restarted and the patient should be monitored

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria: Age Restriction: Prescriber Restrictions: Coverage Duration:  Approval: 12 months, unless otherwise specified

112

POLICY NAME: FIRDAPSE Affected Medications: FIRDAPSE (amifampridine phosphate)

Covered Uses:  All food and Drug Administration (FDA) approved indications not otherwise excluded by plan design. o Lambert-Eaton myasthenic syndrome

Required Medical Information: Lambert-Eaton myasthenic syndrome to reduce symptoms  Documentation of diagnosis of Lambert-Eaton myasthenic syndrome (LEMS) confirmed by all of the following: o Electrodiagnostic studies, including repetitive nerve stimulation (RNS) o Anti-P/Q-type voltage-gated calcium channel (VGCC) antibody testing o Repetitive nerve stimulation (RNS) records o Reproducible post-exercise increase in compound muscle action potential (CMAP) amplitude of at least 60 percent compared with pre-exercise baseline value or a similar increment on high-frequency repetitive nerve stimulation without exercise.  Documented clinical failure to at least 12 weeks of each of the following: o Guanidine or pyridostigmine o Immunosuppressive agents such as Corticosteroids (dosed at 1mg/kg/day), Azathioprine and Mycophenolate o Intravenous Immune Globulin (IVIG) Appropriate Treatment Lambert-Eaton myasthenic syndrome to reduce symptoms Regimen & Other Criteria: 15 to 30 mg/day in 3 to 4 divided doses; May increase based on response and tolerability in 5 mg increments every 3 to 4 days. Maximum 80 mg/day.

Reauthorization requires documentation of treatment success  Electromyography records Exclusion Criteria:  Seizure disorder  Active brain metastases  Clinically significant long QTc interval on ECG in previous year OR history of additional risk factors for torsade de pointes Age Restriction:  18 years of age or older

Prescriber Restrictions:  Prescribed by or in consultation with Neurologist

Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

113

POLICY NAME: FLUCYTOSINE Affected Medications: FLUCYTOSINE

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical Information:  Susceptibility cultures matching flucytosine activity

Appropriate Treatment  Dosing: maximum 150 mg/kg/day Regimen & Other Criteria: Exclusion Criteria:

Age Restriction:

Prescriber Restrictions: Coverage Duration:  Approval: 6 weeks, or lesser requested duration

114

POLICY NAME: GALAFOLD Affected Medications: GALAFOLD (migalastat)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required  Diagnosis of Fabry disease Medical  Diagnosis confirmed by enzyme assay demonstrating a deficiency of alpha- Information: galactosidase enzyme activity or by DNA testing  Presence of at least one amenable (responsive) GLA variant (mutation)

 The patient has clinical signs and symptoms of Fabry disease.  The patient is male OR The patient is female and the patient has documented substantial disease manifestations (Renal dysfunction, Cardiovascular dysfunction, Cerebrovascular complications, Pulmonary complications, Neurologic/neuropathic dysfunction (pain) and diagnosis has been confirmed with genetic testing

Appropriate  Reauthorization will require documentation of treatment success and a clinically Treatment significant response to therapy Regimen & Other Criteria: Exclusion  The safety and efficacy of Galafold used concurrently with Fabrazyme has not Criteria: been established.

Age Restriction:

Prescriber  Prescribed by or in consultation with a prescriber experienced in the treatment of Restrictions: Fabry disease

Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Subsequent approval: 12 months, unless otherwise specified

115

POLICY NAME: GILENYA Affected Medications: GILENYA (fingolimod)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Diagnosis of relapsing forms of multiple sclerosis with MRI Information:  Recent documentations of CBC, LFT and ECG Appropriate Treatment  No concurrent use of any medications indicated for the treatment of relapsing- Regimen & Other remitting multiple sclerosis Criteria:  Not approved for diagnosis of primary progressive or secondary progressive multiple sclerosis  Maximum dose: 0.5 mg once daily  Documentation of varicella serology and varicella zoster virus vaccination if antibody negative for those without a history of chicken pox or prior vaccination

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Varicella or Zostervax vaccination within the last month  MI, unstable angina, stroke, TIA, decompensated heart failure requiring hospitalization or Class III/IV heart failure in the last 6 months  History or presence of Mobitz Type II second-degree or third-degree AV block or sick sinus syndrome, unless patient has a functioning pacemaker  Baseline QTc interval ≥ 500 msec  Current use of Class Ia or Class III anti-arrhythmic drugs Age Restriction:  Greater than or equal to 10 years old (per FDA Labeling)

Prescriber Restrictions:  Prescribed by a Neurologist or an MS specialist Coverage Duration:  Authorization will be for 12 months, unless otherwise specified

116

POLICY NAME: GILOTRIF Affected Medications: GILOTRIF (afatanib)

Covered Uses:  NCCN indications with evidence level of 2A or higher Required Medical  Documentation of performance status, disease staging, all prior therapies used, and Information: prescribed treatment regimen  Documentation of use with NCCN 2A or higher level of evidence regimen  Documentation of known sensitizing EGFR mutation for metastatic non-squamous non- small cell lung cancer (NSCLC): exon 19 deletions or exon 21 (L858R) substitution mutations

Appropriate  Co-administration of P-gp inhibitors can increase afatinib exposure. Reduce dose by 10 mg Treatment per day if not tolerated. Co-administration of chronic Pgp inducers orally can decrease Regimen & Other afatinib exposure. Increase dose by 10 mg per day as tolerated Criteria:  Reauthorization requires documentation of disease responsiveness to therapy

Exclusion Criteria:  Comorbid diagnosis of interstitial lung disease  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber  Oncologist Restrictions: Coverage  Initial approval: 3 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

117

POLICY NAME: GLATIRAMER Affected Medications: GLATIRAMER 20MG, GLATIRAMER 40MG, GLATOPA 20MG

Covered Uses:  All FDA approved indications

Required Medical  Documentation of diagnosis of relapsing forms of multiple sclerosis confirmed Information: with MRI

Appropriate Treatment  Documentation of dose and frequency as the 20 mg/mL and 40 mg/mL Regimen & Other formulations are not interchangeable Criteria:  No concurrent use of medications indicated for the treatment of relapsing- remitting multiple sclerosis  Not approved for primary and secondary progressive multiple sclerosis

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria: Age Restriction: Prescriber Restrictions:  Prescribed by or after consultation with a neurologist or an MS specialist. Coverage Duration:  Approval = 12 months, unless otherwise specified.

118

POLICY NAME: GLUCAGON-LIKE PEPTIDE-1 AGONISTS Affected Medications: BYETTA Subcutaneous (Exenatide), BYDUREON Subcutaneous (Exenatide), BYDUREON BCise Subcutaneous (Exenatide), VICTOZA Subcutaneous (Liraglutide), TRULICITY Subcutaneous (dulaglutide)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required  The patient is diagnosed as having type-2 diabetes with an HbA1C level greater than 7. Medical  The patient demonstrated an inadequate treatment response, intolerance or Information: contraindication to an adequate trial of: metformin AND an additional oral antidiabetic agent (e.g. sulfonylurea (e.g. glyburide, glimepiride, glipizide), pioglitazone, Dipeptidyl peptidase-4 (DPP4) inhibitor (e.g. Januvia, Tradjenta), or Sodium-glucose co-transporter 2 (SGLT2) inhibitor (Invokana, Jardiance).  For reauthorization, patient demonstrated an expected reduction in hemoglobin A1c (HbA1C) since starting therapy of at least 0.5%

Appropriate Treatment Regimen & Other Criteria: Exclusion  Weight Loss Criteria:

Age Restriction:  Greater than or equal to 18 years. Prescriber Restrictions: Coverage  Approval: 12 months, unless otherwise specified Duration:

119

POLICY NAME: GROWTH HORMONES (somatropin) Injectables Affected Medications: GENOTROPIN®, HUMATROPE®, NORDITROPIN FLEXPRO®, NORDITROPIN NORDIFLEX®, NUTROPIN AQ, NUSPIN®, NUTROPIN AQ®, OMNITROPE®, SAIZEN®, TEV-TROPIN®, ZOMACTON

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design  The following disorders are funded pediatric FDA indications: o Pituitary dwarfism (short stature disorder due to growth hormone deficiency) . Growth hormone deficiency without short stature NOT a funded indication o Turner’s syndrome o Prader-Willi syndrome o Noonan’s syndrome o Short stature homeobox-containing gene (SHOX) deficiency o Chronic kidney disease (stages 3, 4, 5 or ESRD) o Renal transplant  Pediatric FDA indications not listed above are NOT funded indications  FDA indications for adults AND pediatric indications after adult height reached as determined by bone age are NOT funded indications Required Medical All indications: Information:  Documentation of baseline height, height velocity, and bone age

Growth hormone deficiency or Pituitary dwarfism  For initial approval, documentation of the following is required: o Diagnosis of growth hormone deficiency or pituitary dwarfism AND o Low serum values for GH stimulation test, IGF-I, and IGFBP-3 AND . Height standard deviation score (SDS) of -2.5 (0.6th percentile) OR . Height velocity impaired AND . Height SDS of -2 (2.3rd percentile) for bone age Turner’s syndrome  For initial approval, documentation of the following is required: o Diagnosis of Turner Syndrome done through genetic testing AND o Age greater than or equal to 2 years of age AND o Height below the 5th percentile for bone age Noonan’s syndrome  For initial approval, documentation of the following is required: o Diagnosis of Noonan’s syndrome done through genetic testing AND . Height standard deviation score (SDS) of -2.5 (0.6th percentile) OR . Height velocity impaired AND . Height SDS of -2 (2.3rd percentile) for bone age Short stature homeobox-containing gene (SHOX) deficiency  For initial approval, documentation of the following is required: o Diagnosis of SHOX deficiency done through genetic testing 120

. Height standard deviation score (SDS) of -2.5 (0.6th percentile) OR . Height velocity impaired AND . Height SDS of -2 (2.3rd percentile) for bone age Chronic kidney disease stage 3 and greater OR kidney transplant  For initial approval, documentation of the following is required: o Diagnosis of chronic kidney disease stage 3 or higher (CrCl less than 60mL/min) o Height velocity (SDS) less than -1.88 for bone age. Prader-Willi syndrome  For initial approval, documentation of the following is required: o Diagnosis of Prader-Willi syndrome through genetic testing AND o Height velocity impaired

 Reauthorization: requires a documented growth rate increase of at least 2.5 cm over baseline per year AND evaluation of epiphyses (growth plates) documenting they remain open. Appropriate  Patient must try Norditropin prior to use of any other growth hormone agent. Treatment  Height velocity impairment Regimen & Other Criteria:

Exclusion Criteria:  Adult growth hormone deficiency Age Restriction: Prescriber  Pediatric endocrinologist Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified

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POLICY NAME: ESBRIET Affected Medications: ESBRIET CAPSULE 267 MG ORAL, ESBRIET TABLET 267 MG ORAL, ESBRIET TABLET 801 MG ORAL

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design

Required Medical  Documentation of diagnosis of idiopathic pulmonary fibrosis Information:  Presence of usual interstitial pneumonia (UIP) or high resolution computed tomography (HRCT), and/or surgical lung biopsy AND  Documentation of baseline forced vital capacity (FVC) greater than or equal to 50 percent of the predicted value AND  Documentation of Predicted diffuse capacity for carbon monoxide (DLCO) greater than or equal to 30 percent.  Documentation of baseline liver function tests, monthly for first 6 months, then every 3 months thereafter

Appropriate  Esbriet is not approved for use in combination with Ofev. Treatment  ESBRIET is not recommended for use in patients with severe (Child Pugh Class C) Regimen & Other hepatic impairment. Criteria:  Reauthorization requires documentation of treatment success. Exclusion Criteria:  Concomitant administration of moderate or strong CYP1A2 inhibitors / inducers should be avoided while taking Esbriet.  Transaminases more than 5 times the upper limit of normal or elevated transaminases accompanied by symptoms (jaundice, hyperbilirubinemia). Age Restriction:  18 years of age or older Prescriber  Must be prescribed by or in consultation with a pulmonologist Restrictions: Coverage Duration:  Initial approval: 6 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: EVENITY Affected Medications: EVENITY (romosozumab-aqqg)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design o Treatment of osteoporosis in postmenopausal women at high risk for fracture, defined as a history of osteoporotic fracture, or multiple risk factors for fracture; or patients who have failed or are intolerant to other available osteoporosis therapy Required Medical  For Treatment of Osteoporosis: Documentation of T Score equal to or less than -2.5 or Information: FRAX Score indicating Major fracture risk greater than 20% or HIP Fracture greater than 3%, or non-traumatic fracture. Appropriate  Documentation of clinical failure or intolerance with intravenous bisphosphonate (e.g., Treatment zoledronic acid [Reclast] or ibandronate [Boniva]) OR Regimen & Other  If the patient has severe renal impairment (e.g., creatinine clearance less than 35 mL/min) Criteria: AND  Documentation of clinically significant worsening osteoporosis on Prolia  If the patient has multiple osteoporotic fractures in the setting of T-scores less than -3.5 no treatment failure to Prolia required  Dosage is 210 mg once monthly  Documentation of taking a minimum 500mg calcium and 600 IU vitamin D daily or contraindication Exclusion  Heart attack or stroke event within 1 year of starting this medication Criteria:  Concurrent use of bisphosphonates (e.g. alendronate, risendronate), parathyroid hormone analogs (e.g. Forteo, Tymlos), or RANK ligand inhibitors (e.g. Prolia, Xgeva)  Preexisting hypocalcemia  Use beyond 12 months of therapy Age Restriction:  18 years of age or older Prescriber Restrictions: Coverage  Approval: 12 months lifetime maximum Duration:

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POLICY NAME: FLUOCINOLONE OCULAR IMPLANT Affected Medications: ILUVIEN, RETISERT, YUTIQ (fluocinolone acetonide intravitreal implant)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Iluvien Medical  Diagnosis of clinically significant diabetic macular edema AND Information:  Documentation of past treatment with corticosteroids without a clinically significant rise in intraocular pressure AND  Documentation of insufficient response to initial therapy with intravitreal bevacizumab (or another anti-VEGF therapy) AND  Documentation of insufficient response to laser photocoagulation

Retisert and Yutiq  Diagnosis of recurrent non-infectious uveitis with documentation of slit-lamp examination and dilated fundus examination  Authorization for Retisert requires documented clinical failure with Yutiq Appropriate Iluvien Treatment  One intravitreal implant per 36 months as monotherapy Regimen &  If the physician determines that adjunctive therapy with anti-VEGF is necessary (e.g. Other Criteria: worsening visual acuity, retinal volume, or fluorescein leakage with Iluvien monotherapy), the request will be reviewed and determination will be made based on medical necessity. Adjunctive therapy with Avastin (bevacizumab) will be the preferred option.

Retisert and Yutiq  One intravitreal implant per 30 months (Retisert) or 36 months (Yutiq)  Documented failure with  A 12-week trial with a systemic corticosteroid (such as prednisone) AND  At least one immunosuppressive agent: methotrexate, azathioprine, mycophenolate AND  At least one calcineurin inhibitor (cyclosporine, tacrolimus) AND  At least two of the following ocular steroids: Ozurdex, Triesence, Trivaris AND  Authorization for Retisert requires documented clinical failure with Yutiq Exclusion  Active or suspected ocular or periocular infections Criteria:  Glaucoma or documentation of past treatment with corticosteroids with a clinically significant rise in intraocular pressure  Concurrent use of intravitreal implants and injections: Ozurdex (dexamethasone), Triesence (triamcinolone), Trivaris (triamcinolone) Age Restriction: Prescriber  Ophthalmologist Restrictions:

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Coverage Iluvien: 36 months, unless otherwise specified Duration: Retisert: 30 months, unless otherwise specified Yutiq: 36 months, unless otherwise specified

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POLICY NAME: GAMIFANT Affected Medications: GAMIFANT (emapalumab-lzsg)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design o Treatment of adult and pediatric (newborn and older) patients with primary hemophagocytic lymphohistiocytosis (HLH) with refractory, recurrent or progressive disease or intolerance with conventional HLH therapy. Required Diagnosis of primary hemophagocytic lymphohistiocytosis (HLH) Medical  Medical records (e.g., chart notes, laboratory values) confirming the following: Information: o Confirmation of a gene mutation known to cause primary HLH (e.g., PRF1, UNC13D); AND o Confirmation that 5 of the following clinical characteristics are present:  Fever 101.3°F or higher  Splenomegaly  Two of the following cytopenias in the peripheral blood:  Hemoglobin less than 9 g/dL; or  Platelet count less than 100 x 109/L; or  Neutrophils less than 1 x 109/L  One of the following:  Hypertriglyceridemia defined as fasting triglycerides 3 mmol/L or higher or 265 mg/dL or higher; or  Hypofibrinogenemia defined as fibrinogen 1.5 g/L or lower  Hemophagocytosis in bone marrow or spleen or lymph nodes with no evidence of malignancy  Low or absent natural killer cell activity (according to local laboratory reference)  Ferritin 500 mg/L or higher  Soluble CD25 (i.e., soluble IL-2 receptor) 2,400 U/ml or higher AND

 Patient has refractory, recurrent or progressive disease or intolerance with conventional HLH therapy (i.e., etoposide + dexamethasone); and  Emapalumab will be administered with dexamethasone; and  Patient is a candidate for stem cell transplant; and  Emapalumab is being used as part of the induction or maintenance phase of stem cell transplant, which is to be discontinued at the initiation of conditioning for stem cell transplant; and  Dosing is in accordance with the United States Food and Drug Administration approved labeling; and  Approval is for no more than 6 months

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Appropriate Treatment Regimen & Other Criteria: Exclusion  Emapalumab for the treatment of secondary HLH Criteria:

Age Restriction: Prescriber  Must be prescribed by or in consultation with a prescriber experienced in the Restrictions: treatment of HLH

Coverage  Initial Authorization: 2 months, unless otherwise specified Duration:  Reauthorization: 4 months, unless otherwise specified (not to exceed 6 months total of treatment)

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POLICY NAME: HEMLIBRA Affected Medications: HEMLIBRA (Emicizumab-kxwh)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Documented diagnosis of severe hemophilia A (baseline factor level less than Information: 1%) with or without inhibitors  Prescribed for routine prophylaxis to prevent or reduce the frequency of bleeding episodes Appropriate  Documented inadequate response to current therapy with a bypassing agent or Treatment inhibitors to Factor VIII OR Regimen & Other  A documented history of at least two episodes of spontaneous bleeding into Criteria: joints  Prophylactic agents must be discontinued o Factor VIII Inhibitors: after the first week of HEMBLIRA o Bypassing Agents: one day before starting HEMBLIRA

Loading Dose:  3 mg/kg once every week for 4 weeks o Maximum 1,380 mg per 28 day supply

Maintenance dose:  1.5 mg/kg once every week or  3 mg/kg once every 2 weeks or  6 mg/kg once every 4 weeks  Any increases in dose must be supported by an acceptable clinical rationale (i.e. weight gain, increase in breakthrough bleeding when patient is fully adherent to therapy, etc.)

Product Availability:  Single-dose vials for injection: 30 mg/mL, 60 mg/0.4 mL, 105 mg/0.7 mL, 150 mg/mL  Dose-rounding to the nearest vial size within 10% of the prescribed dose will be enforced

Reauthorization requires documentation of treatment success defined as a reduction in spontaneous bleeds requiring treatment, as well as documentation of bleed history since last approval Exclusion Criteria: Age Restriction: Prescriber  Hematologist Restrictions:

Coverage Duration:  Approval duration: 6 months, unless otherwise specified

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POLICY NAME: HEPATITIS C DIRECT-ACTING ANTIVIRALS

Affected Medications: ZEPATIER (Elbasvir/Grazoprevir), EPCLUSA (Sofosbuvir/Velptasvir), VOSEVI (Sofosbuvir/Velpatasvir/Voxilaprevir), MAVYRET (Glecaprevir/Pibrentasvir)

Approval Criteria

1. What diagnosis is being treated? Record ICD10 code.

2. Is the request for treatment of chronic Hepatitis Yes: Go to #3 No: Pass to RPh. C infection (B18.2)? Deny; medical appropriateness.

3. Is expected survival from non-HCV- Yes: Go to #4 No: Pass to RPh. associated morbidities more than 1 year? Deny; medical appropriateness.

4. Has all of the following pre-treatment Yes: Record results of No: Pass to RPh. testing been documented: each test and go to #5 Request updated a. Genotype testing in past 3 years is testing. required if the patient has cirrhosis, any Note: If the patient has prior treatment experience, and if HIV or HBV co-infection, it prescribed a regimen which is not pan- is highly recommended genotypic; that a specialist be b. Baseline HCV RNA level in past 6 consulted prior to months; treatment. c. Current HBV status of patient d. Pregnancy test in past 30 days for a Currently treatment is not woman of child-bearing age; and recommended during e. History of previous HCV treatment and pregnancy due to lack of outcome safety and efficacy data f. Presence or absence of cirrhosis as clinically determined (e.g., clinical, laboratory, radiologic evidence, etc)?

Note: Direct-acting antiviral agents can re- activate hepatitis B in some patients. Patients with history of HBV should be monitored carefully during and after treatment for flare- up of hepatitis. Prior to treatment with a DAA, all patients should be tested for HBsAG, HBsAb, and HBcAB status. HIV testing is also recommended.

5. Which regimen is requested? Document and go to #6

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Approval Criteria

6. Does the patient have clinical, Yes: Go to #7 No: Go to #8 radiologic or laboratory evidence of complications of cirrhosis (ascites, portal hypertension, hepatic encephalopathy, hepatocellular carcinoma, esophageal varices)? Yes: Go to #8 7. Is the regimen prescribed by, OR is the patient No: Pass to RPh. in the process of establishing care with or in Deny; medical consultation with a hepatologist, appropriateness. gastroenterologist, or infectious disease specialist? Recommend prescriber document referral to a specialist prior to initiating treatment.

8. Is there attestation that the patient and Yes: Go to #9 No: Pass to RPh. provider will comply with all case Deny; medical management interventions to promote the appropriateness. best possible outcome for the patient and adhere to monitoring requirements required by the Oregon Health Authority, including measuring and reporting of a post- treatment viral load?

Case management includes assessment of treatment barriers and offer of patient support to mitigate potential barriers to regimen adherence as well as facilitation of SVR12 evaluation to assess treatment success.

9. Is the prescribed drug: Yes: Go to #10 No: Go to #11 a) Elbasvir/grazoprevir for GT 1a infection; or b) Daclatasvir + sofosbuvir for GT 3 infection?

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Approval Criteria

10. Has the patient had a baseline NS5a Yes: Pass to RPh; deny No: Go to #11 resistance test that documents a resistant for appropriateness variant to one of the agents in #16? Document test and result. Note: Baseline NS5A resistance testing is required.

11. Is the prescribed regimen include a Yes: Go to #12 No: Go to #13 NS3/4a protease inhibitor (elbasvir, glecaprevir, simeprevir, paritaprevir, voxilaprevir)?

12. Does the patient have moderate-severe Yes: Pass to RPh; deny No: Go to #13 hepatic impairment (Child-Pugh B or Child- for appropriateness Pugh C)?

13. Is the prescribed regimen for the Yes: Pass to RPh; Deny No: Go to #14 retreatment after failure of a DAA due to and refer to medical noncompliance or lost to follow-up? director for review

14. Is the prescribed drug regimen a Yes: Approve for 8-16 No: Pass to RPh. recommended regimen based on the weeks based on duration Deny; medical patient’s genotype, treatment status of treatment indicated appropriateness. (retreatment or treatment naïve) and for approved regimen cirrhosis status (see Table 1)?

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Table 1: Recommended Treatment Regimens for Chronic Hepatitis C.

Treatment History Cirrhosis Status Recommended Regimen Genotype 1 DAA-Treatment naive Non-cirrhotic EBV/GZR x 12 weeks** SOF/VEL x 12 weeks G/P x 8 weeks

Compensated Cirrhosis EBV/GZR x 12 weeks** SOF/VEL x 12 weeks G/P x 12 weeks

Decompensated Cirrhosis SOF/VEL + RBV x 12 week Treatment experienced (Prior Non-cirrhotic EBV/GZR x 12 weeks** PEG/RBV) SOF/VEL x 12 weeks G/P x 8 weeks Compensated cirrhosis EBV/GRZ 12weeks** SOF/VEL x 12 weeks G/P x 12 weeks

Treatment Experienced (Prior Non-cirrhotic or SOF/VEL x 12 weeks sofosbuvir) compensated cirrhosis G/P x 12 weeks

Treatment Experienced (Prior Non-cirrhotic or SOF/VEL x 12 weeks NS3A/4A inhibitor) compensated cirrhosis EBV/GZR + RBV x 12 weeks** G/P x 12 weeks

Treatment Experienced (prior Non-cirrhotic or G/P x 16 weeks NS5A-containing regimen) compensated cirrhosis Genotype 2 Naïve Non-cirrhotic SOF/VEL x 12 weeks G/P x 8 weeks Compensated cirrhosis SOF/VEL x 12 weeks G/P x 12 weeks Decompensated SOF/VEL + RBV x 12 weeks Treatment Experienced (prior Non-cirrhotic SOF/VEL x 12 weeks PEG/RBV) G/P x 8 weeks Compensated cirrhosis SOF/VEL x 12 weeks G/P x 12 weeks

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Treatment Experienced (SOF + Non-cirrhotic or SOF/VEL x 12 weeks RBV) compensated cirrhosis G/P x 12 weeks Treatment Experienced (prior Non-cirrhotic or SOF/VEL/VOX x 12 weeks NS5A-containing regimen) compensated cirrhosis Genotype 3 Naïve Non-cirrhotic SOF/VEL X 12 weeks G/P x 8 weeks Compensated cirrhosis SOF/VEL + RBV x 12 weeks G/P x 12 weeks Decompensated Cirrhosis SOF/VEL + RBV x 12 weeks Treatment Experienced (prior Non-cirrhotic or SOF/VEL x 12 weeks PEG/RBV only) compensated cirrhosis G/P x 16 weeks

Treatment Experienced (SOF + Non-cirrhotic or G/P x 16 weeks RBV) compensated cirrhosis Experienced (prior NS5A- Non-cirrhotic or SOF/VEL/VOX x 12 weeks containing regimen) compensated cirrhosis Genotype 4 Treatment Naïve Non-cirrhotic SOF/VEL x 12 weeks EBV/GZR x 12 weeks G/P x 8 weeks Compensated cirrhosis SOF/VEL x 12 weeks EBV/GZR x 12 weeks G/P x 12 weeks

Decompensated Cirrhosis SOF/VEL + RBV x 12 week Treatment Experienced (prior Non-cirrhotic SOF/VEL x 12 weeks PEG/RBV only) EBV/GZR x 12 weeks G/P x 8 weeks

Compensated cirrhosis SOF/VEL x 12 weeks EBV/GZR x 12 weeks G/P x 12 weeks

Treatment Experienced (prior Non-cirrhotic or SOF/VEL/VOX x 12 weeks NS5A-containing regimen OR compensated cirrhosis sofosbuvir)

Genotype 5/6 Treatment Naïve or Experienced Non-cirrhotic SOF/VEL x 12 weeks (prior PEG-IFN/RBV only) G/P x 8 weeks

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Compensated cirrhosis SOF/VEL x 12 weeks G/P x 12 weeks Decompensated cirrhosis SOF/VEL + RBV x 12 weeks Experienced (prior NS5A- containing Non-cirrhotic or SOF/VEL/VOX x 12 weeks regimen OR sofosbuvir) compensated cirrhosis

Abbreviations: CTP = Child-Turcotte-Pugh; DAA = direct acting antiviral; EBV/GZR = elbasvir/grazoprevir; G/P = glecaprevir and pibrentasvir; PEG = pegylated interferon; RAV = resistance- associated variant; RBV = ribavirin; SOF = sofosbuvir; SOF/VEL = sofosbuvir/velpatasvir; SOF/VEL/VOX = sofosbuvir/velpatasvir/voxilaprevir

**No baseline NS5A RAVs. For genotype 1a patients with baseline NAS5A RAVs, extend duration to 16 weeks. ±^ EvidenceRarely, genotyping is insufficient assays if the may addition indicate of RBVthe presencemay benefit of asubjects mixed infection with GT3 (e.g., and cirrhosis.genotypes If 1aRBV and is 2).not usedTreatment with regimen, data for mixedthen baseline genotypes RAV with testing direct should-acting be antivirals done prior are to limited. treatment However, to rule inout these the Y93cases, a polymorphism.pangenotypic regimen is appropriate. Ribavirin-containing regimens are absolutely contraindicated in pregnant women and in the male partners of women who are pregnant. Documented use of two forms of birth control in patients and sex partners for whom a ribavirin containing regimen is chosen is required.

Regimens other than glecaprevir/pibrentasvir (G/P;) and elbasvir/grazoprevir (EBV/GZR) should not be used in patients with severe renal impairment (GRF < 30 mL/min) or end stage renal disease requiring dialysis.

All regimens containing a protease inhibitor (elbasvir, glecaprevir, simeprevir, paritaprevir, voxilaprevir) should not be used in patients with moderate to severe hepatic impairment (CTP B and C). There is limited data supporting DAA regimens in treatment- experienced patients with decompensated cirrhosis. These patients should be handled on a case by case basis with the patient, prescriber, and CCO or FFS medical director.

*Criteria adopted from Oregon Health Authority

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POLICY NAME: HERCEPTIN Affected Medications: HERCEPTIN IV (trastuzumab) & HERCEPTIN HYLECTA

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, disease staging, all prior therapies used, Information: and prescribed dosing regimen  Baseline evaluation of left ventricular function  Documentation of Her 2 positivity based on 3+ IHC testing or positive fish test Appropriate Treatment  Max duration for adjuvant breast cancer therapy is 12 months Regimen & Other  Reauthorization requires documentation of disease responsiveness to therapy Criteria:

Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction:

Prescriber Restrictions: Coverage Duration:  Initial approval: 4 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: HEREDITARY TYROSINEMIA (HT-1) Affected Medications: NITYR, ORFADIN

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical  Diagnosis of hereditary tyrosinemia type 1 confirmed by biochemical testing (e.g. Information: detection of succinylacetone in urine) and appropriate clinical picture of the patient or by DNA testing  Current patient weight Appropriate  Use as an adjunct to dietary restriction of tyrosine and phenylalanine Treatment  Dosing: Initial- 0.5 mg/kg twice daily Regimen & Other o Maximum: 2 mg/kg/day Criteria:  Orfadin requires documented failure with or contraindication to Nityr  Reauthorization: documentation of treatment success confirmed by urine or plasma succinylacetone reduction since starting therapy and documented adherence to medical/nutritional therapy Exclusion Criteria: Age Restriction:

Prescriber  Prescribed by, or in consultation with physicians that specializes in the treatment of Restrictions: hereditary tyrosinemia or related disorders

Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: HETLIOZ Affected Medications: HETLIOZ (tasimelteon)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Documentation of being legally blind Information:  Documentation of 30 day sleep log or actigraphy  Circadian biochemical analysis (collected over several weeks)

Appropriate  If applicable: Beta-blocker must be discontinued unless clinically inappropriate, then Treatment documentation as to clinical need of continuation of Beta-blocker and acknowledgment Regimen & Other of likely diminished efficacy. Criteria:  Documentation of 30 day attempt of other treatments options attempted: melatonin and chronotherapy.  Polysomnogram with documentation of treatment or having ruled out other sleep disorders.

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria: Age Restriction: Prescriber  Neurologist, Internist board certified in Sleep Medicine, Sleep Specialist Restrictions: Coverage Duration:  Approval: 6 months, unless otherwise specified.

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POLICY NAME: HUMIRA Affected Medications: HUMIRA (adalimumab)

Juvenile idiopathic arthritis (JIA)  Documented current level of disease activity with physician global assessment (MD global score) or active joint count

Crohn’s disease  Documentation of Crohn’s Disease Activity Index (CDAI) greater than 220

Ulcerative Colitis (UC)  Diagnosis supported by endoscopy/colonoscopy/sigmoidoscopy or biopsy and a Mayo Clinic Score for Assessment of Ulcerative Colitis Activity 138

Ankylosing Spondylitis (AS) and Psoriatic Arthritis (PsA) with Axial involvement  Documentation of active disease defined by Bath ankylosing spondylitis disease activity index (BASDAI) at least 4 AND o Scaroiliitis on imaging AND at least 1 Spondyloarthritis (SpA) feature: o Inflammatory back pain (4 of 5 features met): . Onset of back discomfort before the age of 40 years . Insidious onset . Improvement with exercise . No improvement with rest . Pain at night (with improvement upon arising) o Arthritis o Enthesitis o Uveitis o Dactylitis (inflammation of entire digit) o Psoriasis o Crohn’s disease/ulcerative colitis o Good response to NSAIDs o Family history of SpA o Elevated CRP OR o HLA-B27 genetic test positive AND at least 2 SpA features

Intermediate, Posterior, and Panuveitis  Baseline brain MRI result in patient with intermediate uveitis/pars planitis Appropriate Rheumatoid arthritis (RA) Treatment  Documented clinical failure with at least 12 weeks of combination disease-modifying Regimen & antirheumatic drug (DMARD) therapy [Methotrexate plus sulfasalazine, methotrexate plus Other Criteria: hydroxychloroquine, sulfasalazine plus hydroxychloroquine, leflunomide plus sulfasalazine, or leflunomide plus hydroxychloroquine]  QL – 1.6 ml (2 pens) per 28 day supply

Juvenile idiopathic arthritis (JIA)  Documented failure with glucocorticoid joint injections or oral corticosteroids AND  At least one of the following agents for a minimum 12 weeks: methotrexate, leflunomide  QL – 1.6 ml (2 pens) per 28 day supply

Plaque psoriasis (PP) 139

 Documented failure with 12 weeks of at least two systemic therapies (methotrexate, cyclosporine, acitretin, phototherapy [UVB, PUVA])  QL – Initial (one time only)– 3.2 ml (4 pens) per 28 day supply  QL – Continuation – 1.6 ml (2 pens) per 28 day supply

Crohn’s disease  Documented failure with at least two oral treatments for a minimum of 12 weeks: corticosteroids, azathioprine, cyclosporine, 6-mercaptopurine, methotrexate, sulfasalazine, balsalazide, tacrolimus, mesalamine  QL – Initial (one time only) – 4.8 ml (6 pens) per 28 day supply  QL – Continuation – 1.6 ml (2 pens) per 28 day supply

Ulcerative Colitis (UC)  Documented failure with at least two oral treatments for a minimum of 12 weeks: corticosteroids, sulfasalazine, azathioprine, mesalamine, balsalazide, cyclosporine, azathioprine, 6-mercaptopurine  QL – Initial (one time only)– 4.8 ml (6 pens) per 28 day supply  QL – Continuation – 1.6 ml (2 pens) per 28 day supply

Intermediate and Panuveitis  Documented failure with at least one immunosuppressive agent: methotrexate, azathioprine, mycophenolate AND  At least one calcineurin inhibitor (cyclosporine, tacrolimus)  QL – initial (one-time only)- 3.2 ml (4 pens) per 28 day supply  QL – Continuation- 1.6 ml (2 pens) per 28 day supply

Posterior Uveitis  Documented trial and clinical failure of Yutiq AND Retisert  QL – initial (one-time only)- 4 pens per 28 day supply  QL – Continuation- 2 pens per 28 day supply

Reauthorization: documentation of treatment success

Ankylosing spondylitis (AS) and Psoriatic arthritis (PsA) with Axial Involvement  Documented failure with two daily prescription strength nonsteroidal anti-inflammatory drugs (ibuprofen, naproxen, diclofenac, meloxicam, etc.) with minimum 1 month trial each  For isolated sacroiliitis, enthesitis, peripheral arthritis: documented treatment failure with locally administered parenteral glucocorticoid  QL – 1.6 ml (2 pens) per 28 day supply Reauthorization: Improvement of at least 50% in the BASDAI score or an absolute change of 2 units.

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Exclusion  Concurrent us of biologic DMARDs: Orencia (abatacept), Rituxan (rituximab), Actemra Criteria: (tocilizumab), Enbrel (etanercept), Remicade (infliximab), Simponi (golimumab), Cimzia (certolizumab), Kineret (anakinra), Xeljanz (tofacitinib)  Concurrent use of Otezla (Apremilast)  Positive tuberculosis; active hepatitis B, hepatitis C or herpes zoster infection  Use in the management of osteoarthritis, recurrent spontaneous pregnancy loss, in-vitro fertilization  Intra-articular injection of Humira  Hidradenitis Suppurativa (HS) – not covered by plan  Use in management of anterior uveitis Age Restriction:  RA, PsA, PP, AS, CD : 18 years of age or older  JIA 2 years of age or older  Pediatric CD: 6 years of age or older Prescriber  CD/UC: prescribed by or in consultation with a gastroenterologist specialist Restrictions:  PP: prescribed by or in consultation with a dermatologist  Other indications: prescribed by or in consultation with a rheumatologist Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: HYALURONIC ACID DERIVATIVES Affected Medications: EUFLEXXA, GENVISC, GEL-ONE, GEL-SYN, HYALGAN, HYMOVIS, MONOVISC, ORTHOVISC, SUPARTZ, SYNVISC, SYNVISC-ONE, TRI-VISC

Covered Uses:  Coverage of Hyaluronic Acids is excluded based Oregon Health Authority: Viscosupplementation of the knee (CPT 20610) is not covered for treatment of osteoarthritis of the knee.

Required Medical Information: Appropriate Treatment Regimen & Other Criteria: Exclusion Criteria: Age Restriction: Prescriber Restrictions: Coverage Duration:

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POLICY NAME: HYCAMTIN Affected Medications: HYCAMTIN (topotecan)

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, all prior therapies used, and prescribed Information: treatment regimen.  Documented monitoring of bood cell counts, renal function tests, and bilirubin.  Performance status 0-2 Appropriate  Avoid use with CYP 450 inhibitors such as ritonavir, cyclosporine, saquinavir, Treatment ketoconazole, as these drugs increase concentration of hycamtin Regimen & Other  Patients of child-bearing potential are instructed on the importance and proper Criteria: utilization of appropriate contraceptive methods for Hycamtin use.

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater

Age Restriction:

Prescriber  Prescribed by or in consultation with an oncologist Restrictions:

Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Subsequent approval: 12 months, unless otherwise specified

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POLICY NAME: IBRANCE Affected Medications: IBRANCE (palbociclib)

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of disease staging and all prior therapies used Information:  Documentation of ECOG performance status of 1 or 2 OR Karnofsky performance score greater than 50%

Appropriate  Reauthorization: documentation of disease responsiveness to therapy Treatment Regimen & Other Criteria:

Exclusion Criteria:  Previous progression on any agents within the class (Kisqali, Verzenio) Age Restriction:

Prescriber  Oncologist Restrictions:

Coverage Duration:  Initial approval: 4 months, unless otherwise specified  Subsequent approval: 12 months, unless otherwise specified

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POLICY NAME: ICLUSIG Affected Medications: ICLUSIG (ponatinib)

Covered Uses:  NCCN indications with evidence level of 2A or higher. Required Medical  Documentation of performance status, disease staging, all prior therapies used, and Information: prescribed treatment regimen  Documentation of use with NCCN 2A or higher level of evidence regimen  If applicable, documentation of Philadelphia chromosome-positive  If applicable, documentation of T315I mutation-positive

Appropriate  Avoid concurrent use with strong CYP3A inhibitors, or reduce Iclusig dose if co- Treatment administration cannot be avoided. Avoid concurrent use with strong CYP3A inducers. Regimen & Other  Reauthorization requires documentation of disease responsiveness to therapy Criteria: Exclusion Criteria:  Congenital long QT syndrome  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber  Oncologist Restrictions: Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: IDHIFA Affected Medications: Idhifa™ (enasidenib mesylate tablet –Celgene)

Covered Uses:  NCCN (National Comprehensive Cancer Network) indications with evidence level of 2a or higher

Required Medical  Diagnosis of Acute Myeloid Leukemia with an isocitrate dehydrogenase-2 (IDH2) Information: mutation as detected by a Food and Drug Administration (FDA)-approved test. Appropriate  Reauthorization: documentation of disease responsiveness to therapy Treatment Regimen & Other Criteria:

Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber  Oncologist Restrictions:

Coverage Duration:  Initial approval: 4 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: ILARIS Affected Medications: ILARIS (canakinumab)

Covered Uses:  All FDA approved indications not otherwise excluded by plan design. Required  Patient weight Medical Information: Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS)  Confirmed diagnosis of TRAPS with frequent and/or severe recurrent disease AND documented genetic defect of TNFRSF1A gene  Documented clinical failure to Nonsteroidal anti-inflammatory drugs (NSAIDs), glucocorticoids (prednisone or prednisolone), Enbrel

Hyperimmunoglobulin D syndrome (HIDS)  Confirmed diagnosis including presence of heterozygous or homozygous mutation in the mevalonate kinase (MVK) gene  Documented treatment failure with nonsteroidal anti-inflammatory drugs (NSAIDs), glucocorticoids, and episodic anakinra  Documented frequent and severe attacks with substantive quality-of-life detriment

Familial Mediterranean Fever (FMF).  Documented Treatment failure with maximal tolerable dose of colchicine (3 mg daily in adults and 2 mg daily in children) AND  Documentation of frequent and/or severe recurrence disease despite adequate treatment with Anakinra

Systemic Juvenile Idiopathic Arthritis (SJIA)  Documentation of frequent and/or severe recurrence disease despite adequate treatment with each of with minimum of 12 weeks trial each: 1. NSAIDS or Glucocorticoids AND 2. Anakinra AND 3. Actemra

Appropriate  Initial approval for MWS or FCAS, authorize one dose Treatment  After up to 8 weeks of therapy if the patient has had a response to therapy as determined by Regimen & prescribing physician an additional 6 months authorization is allowed Other Criteria:  Reauthorization: documentation of treatment success Exclusion  Treatment of neonatal onset multisystem inflammatory disorder (NOMID) or chronic Criteria: infantile neurological cutaneous and articular syndrome (CINCA), juvenile idiopathic arthritis (JIA), gout, rheumatoid arthritis, chronic obstructive pulmonary disease (COPD), type 2 diabetes mellitus

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 When used in combination with tumor necrosis factor (TNF) blocking agents (e.g. Enbrel, Humira, Cimzia, Remicade, Simponi), Kineret, Arcalyst  Coverage is not recommend for circumstances not listed under covered uses Age Restriction: Prescriber  Prescribed by or in consultation with allergist/Immunologist/Rheumatologist Restrictions:

Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 6 months, unless otherwise specified

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POLICY NAME: ILUMYA Affected Medications: ILUMYA (tildrakizumab-asmn)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design

Required Medical  Documentation of moderate to severe disease despite current treatment (Indication must Information: be documented in chart notes within the last 6 months)  Documented current level of disease activity/disease control  Documentation of disease that is severe in nature, which has resulted in functional impairment (e.g., inability to use hands or feet for activities of daily living, or significant facial involvement preventing normal social interaction) AND one or more of the following: . At least 10% body surface area involvement; or . Hand, foot or mucous membrane involvement

Appropriate Plaque psoriasis Treatment  Documented failure with at least two systemic therapies for a minimum of 12 weeks Regimen & Other (methotrexate cyclosporine, acitretin, phototherapy [UVB, PUVA]) AND Criteria:  Documented failure with Humira AND Enbrel  Dose: 100 mg at Weeks 0, 4, and every twelve weeks thereafter

Reauthorization: documentation of treatment success Exclusion  Positive TB test or treatment of latent infection should be started prior to therapy Criteria:  Avoid use of live vaccines in patients treated with Ilumya  Dosing beyond the FDA-approved amount Age Restriction:  Adults age 18 and older Prescriber  Prescribed by or in consultation with a Dermatologist Restrictions: Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: IMATINIB Affected Medications: Imatinib

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, all prior therapies used, and prescribed treatment Information: regimen  Documentation of Philadelphia chromosome-positive mutation status  Documentation of PDGFR (platelet-derived growth factor receptor) gene re-arrangements, Kit (CD117) positive mutation status, or FIP1L1-PDGFR-alpha fusion kinase status (mutational analysis or FISH demonstration of CHIC2 allele deletion) if applicable  Documentation of use with NCCN 2A or higher level of evidence regimen Appropriate  Reauthorization requires documentation of disease responsiveness to therapy (including Treatment applicable markers, examples: BCR-ABL1 transcript levels, cytogenetic response, molecular Regimen & Other relapse) Criteria:

Exclusion Criteria:  Use with warfarin  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber  Oncologist Restrictions: Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: IMBRUVICA Affected Medications: IMBRUVICA (ibrutinib)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design (Non-Oncology use only)  NCCN indications with evidence level of 2A or higher Required Medical Oncology indication Information:  Documentation of performance status, disease staging, all prior therapies used, and anticipated treatment course

Chronic Graft Versus Host Disease (cGVHD)  Managed by transplant center  Documentation confirming tried or currently receiving conventional treatment with high dose corticosteroids, calcineurin inhibitor (eg. cyclosporine, tacrolimus), etc.

Appropriate  Mantle cell lymphoma:560 mg taken orally once daily Treatment  Chronic Lymphocytic Leukemia, Waldenstrom’s Macroglobinemia, and GVHD: 420 mg Regimen & Other taken orally once daily Criteria: Reauthorization: documentation of disease responsiveness to therapy

Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction:  18 years of age or greater Prescriber  Oncology: Oncologist Restrictions:

Coverage Duration:  Initial approval: 4 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: IMPAVIDO Affected Medications: IMPAVIDO (miltefosine)

Covered Uses:  All FDA approved indications not otherwise excluded by plan design. Required Medical  Current weight Information:  Documentation of Visceral leishmaniasis OR Cutaneous leishmaniasis OR Mucosal leishmaniasis

Appropriate  FDA-approved dosing of 30 to 44 kg: one 50 mg capsule twice daily for 28 consecutive Treatment days OR 45 kg or greater: one 50 mg capsule three times daily for 28 consecutive days Regimen & Other  Documentation of plan to monitor LFTs and Platelets during therapy Criteria:  Age 12 years or older  Weight equal to or greater than 30kg (66lbs)

Exclusion Criteria:  Pregnancy (category D)  Sjögren-Larsson-Syndrome

Age Restriction:  Age less than 12 years of age  Weight less than 30 kg (66 lbs) Prescriber  Infectious Disease Specialist Restrictions: Coverage Duration:  Initial coverage: 1 month unless otherwise specified  Subsequent coverage: 1 month unless otherwise specified

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POLICY NAME: INCRELEX Affected Medications: INCRELEX (mecasermin)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  Diagnosis of severe primary insulin-like growth factor-1 (IGF-1) deficiency (Primary IGFD) Information: or with growth hormone (GH) gene deletion with neutralizing antibodies to GH.  Prior to starting therapy, a height at least 3 standard deviations below the mean for chronological age and sex, and an IGF-1 level at least 3 standard deviations below the mean for chronological age and sex.  One stimulation test showing patient has a normal or elevated GH level.  For continuation of therapy, patient grew more than 2 cm/year over baseline. Appropriate  Initial: 40-80 mcg/kg SQ twice daily. Treatment  Maintenance: Up to 0.12 mg/kg SQ twice daily. Regimen & Other Criteria: Exclusion Criteria:  Epiphyseal closure, active or suspected neoplasia malignancy, or concurrent use with GH therapy.  Patient has secondary causes of IGF1 deficiency (e.g., hypothyroidism, malignancy, chronic systemic disease, skeletal disorders, malnutrition, celiac disease). Age Restriction:  For patients 2 to 18 years of age. Prescriber  Endocrinologist Restrictions: Coverage Duration:  Approval: 12 months , unless otherwise specified

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POLICY NAME: INFLIXIMAB Affected Medications: REMICADE (infliximab), INFLECTRA (infliximab-dyyb), RENFLEXIS (infliximab-abda) Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.  Juvenile idiopathic arthritis (JIA) Required  Documentation of moderate to severe disease despite current treatment (Indication must be Medical documented in chart notes within the last 6 months ) Information:  Documentation of complete and current treatment course  Documented current level of disease activity/disease control  Patient’s current weight  Coverage for the non-preferred drug (Remicade) is provided when any of the following criteria is met: o Use in Pediatric ulcerative colitis for patients age 6 years and under. o Member has tried and experienced a documented intolerable adverse event to the preferred products (Renflexis and Inflectra), and the adverse event was not an expected adverse event attributed to the active ingredient o Documented failure with a minimum 12 weeks on Renflexis AND minimum 12 weeks on Inflectra Rheumatoid arthritis  Documented current level of disease activity with one of the following (or equivalent objective scale): . The Disease Activity Score derivative for 28 joints (DAS-28) greater than 3.2 . The Simplified Disease Activity Index (SDAI) greater than 11 . The Clinical Disease Activity Index (CDAI) greater than 10 . Weighted RAPID3 of at least 2.3 Juvenile idiopathic arthritis (JIA)  Documented current level of disease activity with physician global assessment (MD global score) or active joint count Plaque psoriasis  Minimum body surface area (BSA) involvement greater than 10% Crohn’s disease  Documentation of Crohn’s Disease Activity Index (CDAI) greater than 220 Ulcerative Colitis  Diagnosis supported by endoscopy/colonoscopy/sigmoidoscopy or biopsy and a Mayo Clinic Score for Assessment of Ulcerative Colitis Activity Psoriatic Arthritis (PsA) with Axial Involvement  Documentation of active disease defined by Bath ankylosing spondylitis disease activity index (BASDAI) at least 4 AND  Scaroiliitis on imaging AND at least 1 Spondyloarthritis (SpA) feature: o Inflammatory back pain (4 of 5 features met): . Onset of back discomfort before the age of 40 years . Insidious onset . Improvement with exercise . No improvement with rest 154

. Pain at night (with improvement upon arising) o Arthritis o Enthesitis o Uveitis o Dactylitis (inflammation of entire digit) o Psoriasis o Crohn’s disease/ulcerative colitis o Good response to NSAIDs o Family history of SpA o Elevated CRP OR  HLA-B27 genetic test positive AND at least 2 SpA features

Psoriatic Arthritis (PsA)  Documentation of CASPAR criteria score greater than 3 based on chart notes o Skin psoriasis: present – two points, OR previously present by history – one point, OR a family history of psoriasis, if the patient is not affected – one point o Nail lesions (onycholysis, pitting): one point o Dactylitis (present or past, documented by a rheumatologist): one point o Negative rheumatoid factor (RF): one point o Juxtaarticular bone formation on radiographs (distinct from osteophytes): one point Appropriate Rheumatoid arthritis Treatment  Documented clinical failure with at least 12 weeks of combination disease-modifying Regimen & antirheumatic drug (DMARD) therapy [Methotrexate plus sulfasalazine, methotrexate plus Other Criteria: Hydroxychloroquine, sulfasalazine plus hydroxychloroquine, leflunomide plus sulfasalazine, or leflunomide plus hydroxychloroquine] AND  Documented clinical failure of a minimum 12 weeks on Humira AND failure of a minimum 12 weeks on Enbrel  QL – Initial (one time only) – 3mg/kg at 0, 2, and 6 weeks  QL – Continuation – 3mg/kg per 8 weeks

Psoriatic Arthritis (PsA)  Failure of at least 12 weeks with methotrexate, or if unable to tolerate or contraindicated to methotrexate, another disease modifying antirheumatic drug (sulfasalazine, cyclosporine, leflunomide) AND  Documented clinical failure of a minimum 12 weeks on Humira AND failure of a minimum 12 weeks on Enbrel  QL – Initial (one time only) – 5mg/kg at 0, 2, and 6 weeks  QL – Continuation – 5mg/kg per 8 weeks

Plaque psoriasis  Documented clinical failure of at least two systemic therapies with minimum of 12 week trial: methotrexate, cyclosporine, isotretinoin or phototherapy (UVB, PUVA) AND

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 Documented clinical failure of a minimum 12 weeks on Humira AND failure of a minimum 12 weeks on Enbrel  QL – Initial (one time only) – 5mg/kg at 0,2, and 6 weeks  QL – Continuation – 5mg/kg per 8 weeks

Crohn’s disease  Documented clinical failure with at least two oral treatments for minimum of 12 weeks trial: corticosteroids, azathioprine, cyclosporine, 6-mercaptopurine, methotrexate, sulfasalazine, balsalazide, tacrolimus, mesalamine AND  Documented clinical failure of a minimum 12 weeks on Humira  QL – Initial (one time only) – 5mg/kg at 0,2, and 6 weeks  QL – Continuation – 5mg/kg per 8 weeks

Ulcerative Colitis  Documented clinical failure with at least two oral treatments for minimum of 12 week trial: corticosteroids, sulfasalazine, azathioprine, mesalamine, balsalazide, cyclosporine, azathioprine, 6-mercaptopurine AND  Documented clinical failure of a minimum 12 weeks on Humira  QL – Initial (one time only) – 5mg/kg at 0,2, and 6 weeks  QL – Continuation – 5mg/kg per 8 weeks

Reauthorization: documentation of treatment success

Ankylosing spondylitis (AS) and Psoriatic Arthritis (PsA) with Axial Involvement  Documented clinical failure with two daily prescription strength nonsteroidal anti- inflammatory drugs (ibuprofen, naproxen, diclofenac, meloxicam, etc.) with minimum 1 month trial each  For isolated sacroiliitis, enthesitis, peripheral arthritis: documented treatment failure with locally administered parenteral glucocorticoid  Documented clinical failure with minimum 12 weeks on Humira AND Enbrel  QL – Initial (one time only) – 5mg/kg at 0,2, and 6 weeks  QL – Continuation – 5mg/kg per 6 weeks Reauthorization: Improvement of at least 50% in the BASDAI score or an absolute change of 2 units. Exclusion  Concurrent use of biologic DMARDs: Orencia (abatacept), Rituxan (rituximab), Actemra Criteria: (tocilizumab), Enbrel (etanercept), Humira (adalimumab), Simponi (golimumab), Cimzia (certolizumab), Kineret (anakinra), Xeljanz (tofacitinib)  Concurrent use of Otezla (Apremilast)  Positive tuberculosis; active hepatitis B, hepatitis C or herpes zoster infection  Dose greater than 5mg/kg in patients with moderate-severe heart failure (NYHA Class III/IV) Age Restriction: Prescriber  CD/UC: prescribed by or in consultation with a gastroenterologist specialist Restrictions:  PP: prescribed by or in consultation with a dermatologist 156

 RA/JIA/PsA/AS: prescribed by or in consultation with a rheumatologist Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: INLYTA Affected Medications: INLYTA (axitinib)

Covered Uses:  NCCN indications with evidence level of 2A or higher

Appropriate Treatment  Inlyta should be discontinued with severe hypertension that cannot be controlled Regimen & Other with antihypertensive therapy Criteria:  Avoid administration with strong CYP3A4/5 inhibitors, if unable to avoid combined use, Inlyta dosage should be reduced by half  Avoid administration with strong CYP3A4/5 inducers  For patients with moderate hepatic impairment, decrease the starting dose by approximately half  Reauthorization requires documentation of disease responsiveness to therapy

Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber Restrictions:  Oncologist Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: INTRAVITREAL ANTI-VEGF THERAPY Affected Medications: Lucentis (ranibizumab), Eylea (aflibercept)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  Initial approval requires documented failure to intravitreal Avastin (bevacizumab) after Information: a minimum 3-month trial, defined as worsening vision, such as losing greater than 15 letters of visual acuity  Anticipated treatment course with dose and frequency clearly stated in chart notes. Appropriate Eylea Dosing Treatment  Neovascular (Wet) Age-Related Macular Degeneration (AMD) - 2mg (0.05 ml) every 4 Regimen & Other weeks for the first 5 injections followed by 2 mg (0.05ml) every 8 weeks Criteria:  Continued every 4 week dosing requires documented clinical failure to minimum 3 months of every 8 week maintenance dosing  Macular Edema Following Retinal Vein Occlusion - 2 mg (0.05 mL) every 4 weeks  Diabetic Macular Edema and Diabetic Retinopathy (DR) inpatients with Diabetic Macular Edema - 2mg (0.05 ml) every 4 weeks for the first 5 injections followed by 2 mg (0.05ml) every 8 weeks

Lucentis Dosing  Neovascular (Wet) Age-Related Macular Degeneration (AMD) and Macular Edema Following Retinal Vein Occlusion – maximum 0.5mg every 4 weeks  Diabetic Macular Edema and Diabetic Retinopathy (DR) inpatients with Diabetic Macular Edema – 0.3 mg every 28 days

Reauthorization requires documentation of vision stability defined as losing fewer than 15 letters of visual acuity and/or improvements in visual acuity with evidence of decreased leakage and/or fibrosis (central retinal thickness)

Exclusion Criteria:  Evidence of a current ocular or periocular infections  Active intraocular inflammation (aflibercept) Age Restriction:

Prescriber  Ophthalmologist Restrictions:

Coverage  Initial approval: 6 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: INTRON-A Affected Medications: INTRON A (Interferon Alfa-2B)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  NCCN indications with evidence level of 2A or higher

Required Medical  For Hepatitis B and C: Documentation of intolerance to or clinical rationale for Information: avoidance of PEGylated interferon.  Recent liver function tests, comprehensive metabolic panel, complete blood count with differential, TSH (within past 3 months)  Documentation of performance status, disease staging, all prior therapies used, and anticipated treatment course  Reauthorization: documentation of disease responsiveness to therapy Appropriate Treatment  Patients with preexisting cardiac abnormalities and/or advanced cancer: recent Regimen & Other electrocardiogram Criteria:  Chest X ray for patients with pulmonary disorders  Recent ophthalmologic exam at baseline for all patients  Uncontrolled severe mental health illness should be addressed before use and monitored during treatment Exclusion Criteria:  Autoimmune hepatitis  Decompensated liver disease  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction:  Hepatitis B greater than or equal to 1 year of age  Hepatitis C greater than or equal to 3 years of age  All other indications greater than or equal to 18 years of age Prescriber Restrictions: Coverage Duration:  Initial approval: 4 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: INVEGA SUSTENNA Affected Medications: INVEGA SUSTENNA (Paliperidone Palmitate Extended-Release Injectable Suspension)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  Diagnosis of acute and maintenance treatment of schizophrenia AND Information:  The patient has a history of non-compliance and/or refuses to utilize oral medication AND  The patient has received at least ONE of the following: o three test doses of oral Risperdal (risperidone) o three test doses of oral Invega o previous use of Invega Sustenna.  If the patient is increasing the dose of Invega Sustenna, the patient must have a history of two prior injections of Invega Sustenna.

Appropriate Treatment Reauthorization will require documentation of treatment success and a clinically Regimen & Other significant response to therapy Criteria:

Exclusion Criteria:  Diagnosis of dementia-related psychosis.  Prior use of risperidone demonstrated a hypersensitivity reaction. Age Restriction: Prescriber  Psychiatrist or receiving input from a psychiatry practice Restrictions: Coverage Duration:  Approval: 12 months, unless otherwise specified.

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POLICY NAME: INVEGA TRINZA Affected Medications: INVEGA TRINZA (Paliperidone Palmitate Extended-Release Injectable Suspension)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  Diagnosis of acute and maintenance treatment of schizophrenia. AND Information:  The patient has a history of non-compliance and/or refuses to utilize oral medication, or cannot be stabilized on oral medications AND  Patient has been stable on Invega Sustenna for at least 4 months  Documented anticipated dose and dosing schedule based on maintenance Invega Sustenna maintenance dose.  Documented recent renal function with CrCl greater than 50mL/min

Appropriate  If concomitant use with QT prolonging drugs, obtain QT interval prior to initiating Treatment therapy Regimen & Other  If >500msec, documented evaluation of risk for TdP Criteria:  Dosed every 3 months

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Diagnosis of dementia-related psychosis.  Prior hypersensitivity reaction to risperidone

Age Restriction:

Prescriber  Psychiatrist or in consultation with a psychiatrist/psychiatric practice Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified.

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POLICY NAME: IMMUNE GLOBULIN Affected Medications: BIVIGAM, CARIMUNE-NF, FLEBOGAMMA, GAMMAGARD LIQUID/S-D, GAMMAPLEX, GAMUNEX-C, GAMASTAN, OCTAGAM, PRIVIGEN, PANZYGA, VENOGLOBULIN, VIVAGLOBIN

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design  Pemphigus Vulgaris, Pemphigus Foliaceus, Bullous Pemphigoid, Mucous, Membrane Pemphigoid (a.k.a., Cicatricial Pemphigoid), Epidermolysis Bullosa Acquisita  Idiopathic Thrombocytopenic Purpura (ITP) in Pregnancy  Neurological disorders: Myasthenia Gravis, Guillain-Barre Syndrome and Chronic Inflammatory Demyelinating Neuropathy (CIDP), OR select neurological conditions, such as Polymyositis, Multiple Myeloma, Multifocal Motor Neuropathy (MMN)  Chronic Lymphocytic Leukemia with associated hypogammaglobulinemia  Bone Marrow/Stem Cell Transplantation  Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)  Transplantation rejection, kidney or stem cell, antibody-mediated  Stiff-man syndrome  Desensitization for a pre-kidney transplantation Required  Covered indications without specifics require documented failure of or contraindications to Medical conventional therapies Information:  Serum immunoglobulin concentrations and patient weight  Used first line for the following conditions: Immunodeficiency Syndrome, Primary thrombocytopenia, Post-transfusion purpura, Guillian-Barré syndrome, Eaton-Lambert Syndrome, Polyneuropathy, chronic inflammatory demyelinating, Kawasaki disease, Wiskott-Aldrich Syndrome, Anemia due to pure red cell aplasia

Desensitization for a pre-kidney transplantation  panel reactive antibody (PRA) of 80% or below

Neurological Conditions  Failure of or contraindications to conventional therapies (absolute requirement for Dermatomyositis and MS); OR rapidly progressive form of the disease  Documentation of diagnosis based on non-subjective standard of care (ex. electromyography (EMG), spinal fluid tests, serum tests and biopsy finding)

Pemphigus Vulgaris, Pemphigus Foliaceus, Bullous Pemphigoid, Mucous Membrane Pemphigoid (a.k.a., Cicatricial Pemphigoid), Epidermolysis Bullosa Acquisita, Pemphigoid gestationis, Pyoderma gangrenosum  Failure of or contraindications to conventional therapies  Rapidly progressive disease requiring IVIG therapy concomitantly with conventional therapy until conventional therapy reaches efficacy (short-term)

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Idiopathic Thrombocytopenic Purpura (ITP) in Pregnancy  Failure of or contraindications to conventional OR rapidly progressive form of the disease and one of the following  Previously delivered infants with autoimmune thrombocytopenia OR  Platelet counts less than 75,000/mm3 during the current pregnancy OR  Splenectomy

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), CIDP variants, and Multifocal motor neuropathy(MMN)  Diagnosis must be made by Neurologist or Rheumatologist with CIPD expertise  Uncertain diagnosis should be confirmed by prednisone trial (if appropriate), IVIG trial (MMN only), and documented exclusions of alterative diagnosis  When used for pain management: documentation of clinically significant pain improvement with prednisone trial

Common Variable Immunodeficiency (CVID)  Documentation of all of the following must be met: o Serum immunoglobulin G (IgG) below 200mg/dL o Low immunoglobulin A (IgA) and/or immunoglobulin M (IgM) o Poor or absent response to immunizations (both protein- and polysaccharide-based vaccines) o An absence of any other defined immunodeficiency state (examples include secondary hypogammaglobulinemia, specific antibody deficiency, Hyperimmunoglobulin M syndromes, IgG subclass deficiencies)

Acute ITP  Approved for patients with one of the following: Management of acute bleeding due to severe thrombocytopenia (platelet count less than or equal to30,000 mm3) OR To increase platelet counts prior to invasive major surgical procedures (splenectomy) OR In patients with severe thrombocytopenia (platelet counts less than or equal to 20,000 mm3) considered to be at risk for intracerebral hemorrhage.

Chronic Refractory ITP  For first line use in pediatric ITP OR in combination with steroids if rapid platelet response is justified or to avoid splenectomy OR when steroids are contraindicated, otherwise approve for second line treatment following treatment with corticosteroids for splenectomy or when platelet counts are persistently less than or equal to 20,000 mm3

Symptomatic Human Immunodeficiency Virus (HIV)  Entry CD4+ lymphocyte counts greater than or equal to 200/mm3  Clinically symptomatic or asymptomatic, but immunologically abnormal  Current bacterial infections, despite appropriate antimicrobial prophylaxis  400 mg/kg every 28 days

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Chronic Lymphocytic Leukemia with associated hypogammaglobulinemia  Baseline IgG level less than 400 mg/dL AND  Evidence of specific antibody deficiency and the presence of repeated bacterial infections

Bone Marrow/Stem Cell Transplantation  Covered indication for transplant  Cytomegalovirus (CMV) positive OR  CMV negative with CMV positive donor

Reauthorization requires documentation of initial clinical improvement

Appropriate Neurological Conditions and Chronic Inflammatory Demyelinating Polyneuropathy (CIPD) Treatment  Long term use requires regular quantitative monitoring with treatment success (ex. MRC Regimen & scale and activities of daily living (ADL) measurements) Other Criteria:  Long-term treatment success requires documentation of regular dose reduction / discontinuation attempts  Treatment failure requires discontinuation Exclusion  IgA deficiency with antibodies to IgA Criteria:

Age Restriction:  HIV: 13 years and younger  Bone marrow/stem cell transplant: 20 years and older Prescriber  Must be prescribed by or in consultation with a specialist for the treating condition (i.e. Restrictions: neurologist, oncologist/hematologist, immunologist)

Coverage  Initial approval: 3 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

165

POLICY NAME: ISOTRETINOIN ORAL Affected Medications: AMNESTEEM ORAL, MYORISAN ORAL, ZENATANE ORAL

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical  Must have documentation of diagnosis covered by Oregon Health Authority (such Information: as acne conglobata (severe cystic acne))  Prescriber is enrolled in iPLEDGE risk evaluation and mitigation strategy (REMS) management program

Appropriate Treatment Reauthorization will require documentation of treatment success and a clinically Regimen & Other significant response to therapy Criteria: Exclusion Criteria:

Age Restriction:  12 years of age and older Prescriber Restrictions:  Prescribed by or in consultation with a Dermatologist Coverage Duration:  Initial approval: 5 months, unless otherwise specified

166

POLICY NAME: ITRACONAZOLE Affected Medications: ITRACONAZOLE

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical  Diagnosis of onychomycosis or any other susceptible unresolved fungal infection AND Information:  The member has a secondary risk factor that is considered a covered condition per Oregon Health Authority (e.g. diabetes mellitus, peripheral vascular disease, immunocompromised) AND  If the indication is onychomycosis, the diagnosis must be confirmed with a fungal diagnostic test (KOH preparation, fungal culture, or nail biopsy) Appropriate  For tinea pedis, tinea corporis, tinea cruris, and tinea capitis, the member has had an Treatment adequate trial on a topical antifungal agent and either oral griseofulvin or ketoconazole Regimen & Other Criteria:

Exclusion Criteria:  Concomitant use of CYP3A4 substrates, ergot alkaloids, felodipine, irinotecan, lovastatin, lurasidone, methadone, oral midazolam, nisoldipine, pimozide, quinidine, ranolazine, simvastatin, ticagrelor, and triazolam Age Restriction: Prescriber Restrictions:

Coverage  Approval: All but onychomycosis and other tinea infections – 6 months, unless Duration: otherwise specified  Approval: Onychomycosis – fingernails 6 weeks (allows two fills), toenails 10 weeks (allows three fills) , unless otherwise specified  Approval: Other tinea infections – 1 month, unless otherwise specified

167

168

POLICY NAME: JAKAFI Affected Medications: JAKAFI (Ruxolitinib)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Documentation of prior therapies used Information:  Recent liver function, renal function, complete blood count, and platelet count Appropriate  Myelofibrosis: Treatment Platelet count greater than 200 X 10^9/ L: Regimen & Other o Initial dose 20 mg twice daily Criteria:

Platelet count 100 X 109 L to 200 X 10^9/ L: Initial dose 15 mg twice daily

Platelet count greater than 50 X 10^9/ L to less than 100 X 10^9 L: Initial dose 5 mg twice daily

 Polycythemia Vera: Initial dose 10 mg twice daily

 Reduce, interrupt, or discontinue Jakafi doses with Strong CYP3A4 inhibitors or fluconazole. Avoid use of Jakafi with fluconazole doses greater than 200 mg.  Renal Impairment: Reduce Jakafi starting dose or avoid treatment  Hepatic Impairment: Reduce Jakafi starting dose or avoid treatment

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria: Myelofibrosis:  Hepatic impairment with platelet counts less than 50 X 10^9/L  Moderate to severe renal disease with platelet counts less than 50 X 10^9/L  Myelofibrosis and Polycythemia Vera:  End stage renal disease (creatinine clearance less than 15 mL/min) not requiring dialysis Age Restriction:  18 years of age or older Prescriber  Oncologist or in consultation with an oncologist or hematologist Restrictions: Coverage Duration:  12 months, unless otherwise specified

169

170

POLICY NAME: JETREA Affected Medications: JETREA (ocriplasmin)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required  A diagnosis of symptomatic vitreomacular adhesion (VMA) based on Optical coherence Medical tomography (OCT) Information:  Best-corrected visual acuity of 20/25 or less in the eye to be treated

Appropriate  Treatment for bilateral VMA must be done at least 7 days apart Treatment  Dosing: 0.125 mg (0.1 mL of solution) as a single use Regimen &  Repeated administration is not recommended Other Criteria:

Exclusion  Any of the following: Proliferative diabetic retinopathy, Neovascular age-related macular Criteria: degeneration, Retinal vascular occlusion, Aphakia, History of retinal detachment in either eye, Prior vitrectomy in the affected eye, Prior laser photocoagulation of the macula in the affected eye, Prior treatment with ocular surgery, intravitreal injection or retinal laser photocoagulation in the previous 3 months, Uncontrolled glaucoma Age Restriction:  18 years of age and older Prescriber  Ophthalmologist Restrictions:

Coverage  Approval: 1 month (One-time only per affected eye), unless otherwise specified Duration:

171

POLICY NAME: JYNARQUE Affected Medications: JYNARQUE (tolvaptan tablets)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Documentation of baseline serum creatinine. Information:  Documentation of baseline total kidney volume (TKV) at least 750 mL  Documentation of baseline ALT, AST, and bilirubin prior to initiation.

Appropriate  Dosing Treatment o Initial: 45 mg in the morning and 15 mg 8 hours later Regimen & Other o May titrate weekly as tolerated to max of 90 mg and 30 mg 8 hours later Criteria:  Monitoring of liver: Documentation of ALT, AST, and bilirubin at 2 weeks and 4 weeks after initiation, then monthly for the first 18 months and every 3 months thereafter.  Documented risk of rapidly progressing (total kidney volume [TKV] at least 750 mL and age less than 51 years) ADPKD  Documented progression while on maximum ACE inhibitor or ARB therapy to lower blood pressure (target less than 110/75 mmHg)

 Reauthorization: documentation of disease responsiveness to therapy defined as a reduction in the rate of decline in kidney function.

Exclusion Criteria:  A history, signs or symptoms of significant liver impairment or injury. This contraindication does not apply to uncomplicated polycystic liver disease.  Concomitant strong CYP 3A inhibitors.  Uncorrected abnormal blood sodium concentrations.  Uncorrected urinary outflow obstruction or anuria Age Restriction:  Patients < 18 years of age

Prescriber  Nephrologist Restrictions: Coverage Duration:  Initial approval: 6 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

172

POLICY NAME: KALBITOR Affected Medications Kalbitor (ecallantide)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  FDA approved indication must be documented in the member’s chart notes within the Information: most recent 6 months.  Documentation of complete and current treatment course  Number of Hereditary angioedema (HAE) attacks per month must be greater than or equal to 2 (HAE attacks include laryngeal, gastrointestinal, or cutaneous)  Laboratory confirmation of diagnosis (the following levels must be documented):  C4 antigenic level:______ C1-inhibitor antigenic level: ______ C1-inhibitior functional level:______

Appropriate  Documentation of failure, intolerance, or clinical rationale for avoidance to the Treatment following: Androgens: danazol, stanozolol, oxandrolone, oxymetholone, tibolone, or Regimen & Other methyltestosterone. Criteria:  Documentation of maximum of 2 doses on hand at any point

 Reauthorization: Documentation of at least a 50% decrease in the frequency of HAE attacks, significant improvement in severity and duration of attacks, and clinical documentation of functional improvement. Exclusion Criteria:

Age Restriction:  12 years and older

Prescriber  Prescribed by, or in consultation with, an allergist/immunologist or a physician that Restrictions: specializes in the treatment of HAE or related disorders

Coverage  Initial coverage, 3 months unless otherwise specified Duration:  Subsequent coverage, 12 months unless otherwise specified

173

POLICY NAME: KALYDECO Affected Medications Kalydeco (ivacaftor)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  Documentation of cystic fibrosis (CF) diagnosis. Information:  Documentation confirming FDA approved mutation by appropriate genetic or diagnostic testing (FDA approved CF mutation test).  Please provide the diagnostic testing report and/or Cystic Fibrosis Foundation Patient Registry Report  ALT and AST prior to Kalydeco initiation, every 3 mo during first year of treatment, and annually thereafter.  Baseline and routine eye examinations in pediatrics. Appropriate Dosing: Treatment  6 years or older: 150 mg twice daily Regimen & Other  6 months to less than 6 years AND 5 kg to less than 7 kg: 25 mg twice daily Criteria:  6 months to less than 6 years AND 7 kg to less than 14 kg: 50 mg twice daily  6 months to less than 6 years AND greater than 14 kg: 75 mg twice daily

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Homozygous F508del mutation.  Concurrent use of strong CYP3A inducers: rifampin, rifabutin, phenobarbital, carbamazepine, phenytoin, and St. John's wort  Combined use with Calcitonin Gene-Related Peptide (CGRP) Receptor Antagonists (Ajovy, Aimovig or Emgality) for the treatment of migraine Age Restriction:  Ivacaftor oral granules are approved in patients 6 months of age and older.  Ivacaftor oral tablets are approved in patients 6 years of age and older. Prescriber  Prescribed by or in consultation with a pulmonologist or provider who specializes in Restrictions: CF

Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

174

POLICY NAME: KANUMA Affected Medications KANUMA (sebelipase alfa)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design

Required Medical  Diagnosis of lysosomal acid lipase (LAL) deficiency or Rapidly Progressive LAL deficiency Information: within the first 6 months of life via enzyme assay that measures the level and activity of LAL or a genetic sequencing analysis test  Documentation of patient weight  Documentation of prescribed treatment regimen (dose and frequency)  Baseline fasting lipid panel prior to initiating therapy (not required for Rapidly Progressive LAL deficiency) Appropriate  Reauthorization for Rapidly Progressive LAL deficiency requires documentation of Treatment improvement in weight for-age Z-score Regimen & Other  Reauthorization for lysosomal acid lipase (LAL) deficiency requires documentation of Criteria: improvement in fasting lipid panel  If documentation of anti-drug antibodies to Kanuma, documentation of continued response to therapy required Exclusion Criteria:

Age Restriction:  1 month or older Prescriber Restrictions:

Coverage  Initial Approval: 3 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

175

POLICY NAME: KINERET Affected Medications: KINERET (anakinra)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.  Juvenile idiopathic arthritis (JIA), Juvenile rheumatoid arthritis (JRA), polyarticular course (regardless of type of onset)  Systemic onset JIA  Still’s disease (SD)  Neonatal-onset multisystem inflammatory disease (NOMID)  Chronic infantile neurological cutaneous and articular (CINCA) syndrome Required Medical  Indication must be documented in chart notes within the last 6 months Information:  Documentation of complete and current treatment course  Documented latent TB screening with either a TB skin test or an interferon gamma release assay (e.g, QFT-GIT, T-SPOT.TB) with a negative result. Must be receiving or have completed treatment for latent TB prior to initiation.  Recent CrCl or SCr, height, and weight. Dose every other day with CrCl < 30mL/min.  Rheumatoid Arthritis: laboratory test confirming diagnosis of RA (anti-CCP, RF) Appropriate Rheumatoid Arthritis Treatment  Has failed ≥ 12 weeks on Humira AND ≥ 12 weeks Enbrel Regimen & Other  QL – 18.76 ml per 28 day supply Criteria: JIA/JRA (regardless of onset)  Has failed ≥ 12 weeks on Humira AND ≥ 12 weeks Enbrel  QL – 18.76 ml per 28 day supply Systemic onset JIA  Has failed ≥ 12 weeks on Humira AND ≥ 12 weeks Enbrel  QL – 18.76 ml per 28 day supply

Subsequent approval: documentation of treatment success Exclusion Criteria:  Concurrent use with biologic DMARDs: Enbrel, Humira, Remicade, Cimzia, Simponi, Rituxan, Orencia, Rituxan, Actemra, Xeljanz  Sepsis syndrome or graft versus host disease  Use in the management of symptomatic osteoarthritis, lupus arthritis, or type 2 diabetes mellitus. Age Restriction:  Rheumatoid arthritis: ≥18 years of age  Polyarticular JIA or systemic JIA: ≤ 18 years of age Prescriber  Prescribed by or in consultation with a rheumatologist Restrictions:

Coverage  Initial approval: 4 months initiation, unless otherwise specified. Duration:  Reauthorization: 12 months, unless otherwise specified.

176

POLICY NAME: KRYSTEXXA Affected Medications: KRYSTEXXA (pegloticase)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Baseline serum uric acid (SUA) of greater than 8 mg/dl AND Information:  Three (3) or more flares in the past 18 months OR  One (1) or more tophi  Medical contraindication to xanthine oxidase inhibitor(s) or medical history of failure to normalize uric acid (to less than 6 mg/dL) with at least 3 months of treatment at the maximum medically appropriate dose: o Allopurinol AND o Uloric (febuxostat)  Reauthorization requires documentation of uric acid level normalization to less than 6 mg/dl after starting Krystexxa therapy.

Appropriate  Use of probenecid monotherapy should be considered in patients who have failed Treatment xanthine oxidase inhibitors Regimen & Other Criteria:  Referral to a rheumatologist should be considered when o There is an unclear etiology of hyperuricemia o Refractory signs/symptoms of gout o Target serum urate level difficult to reach, especially in renal impairment o Urate lowering therapy causing multiple/serious adverse events

Exclusion Criteria:  Glucose-6-phosphate dehydrogenase (G6PD) Deficiency Age Restriction:

Prescriber  Rheumatologist or in consultation with rheumatologist Restrictions: Coverage  Initial approval: 6 months , unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

177

POLICY NAME: KUVAN Affected Medications: KUVAN (sapropterin)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Documentation of anticipated treatment course, including target phenylalanine Information: (Phe) level set by specialist  Documentation of failure to Phe restricted diet as monotherapy  Current patient weight  Baseline (pre-treatment) blood Phe levels

Baseline Phe concentration must be consistent with the following:  Age less than or equal to 12 years: Phe level must be greater than 6mg/dL (360 microM)  Age greater than or equal to 12 years: Phe level must be greater than 10mg/dL (600 microM)  During pregnancy: Phe level must be greater than 6mg/dL (360 microM) Reauthorization after initial approval requires documentation of updated Phe labs decreased by 30% or greater from baseline  Treatment with Kuvan should be discontinued in patients whose blood Phe has not decreased by at least 30 percent from baseline Reauthorization for continued long-term approval (12 months) requires updated Phe labs meeting one of the following criteria:  Phe level less than 30 percent of baseline OR  Phe level lower than baseline and meets specialist’s target level Appropriate If patient has failed monotherapy with Phe restricted diet and treatment with Kuvan is Treatment warranted, treatment must be consistent with the following: Regimen & Other o Phe restricted diet must be maintained during Kuvan treatment AND Criteria: o Initial dose must be 10mg/kg/day x 1 month . If blood Phe does not decrease from baseline after 1 month, dose can be increased to 20mg/kg/day x 1 month Exclusion Criteria:  Prior intolerance or allergic reaction to requested medication  Doses greater than 20mg/kg/day Age Restriction: Prescriber  Specialist in metabolic disorders or endocrinologist Restrictions:

Coverage Duration:  Initial approval: 2 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

178

POLICY NAME: KYMRIAH Affected Medications: KYMRIAH (Tisagenlecleucel)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of patient’s body weight. Information:  Documentation of patient’s CAR-positive viable T-cells.  Documentation that Black Box Warnings (Cytokine release syndrome, neurological toxicities) have been fully reviewed and patient understands and accepts risks.  Documentation of disease staging, all prior therapies used, and anticipated treatment course

Pediatric and Young Adult Relapsed or Refractory (r/r) B-cell Acute Lymphoblastic Leukemia (ALL)  Documentation of patient being less than 25 years old.  Documentation of Hepatitis B vaccination or protected titer status.  Documentation of relapsed (second or later relapse) or refractory B-cell precursor acute lymphoblastic leukemia AND  Philadelphia chromosome status

Adult Relapsed or Refractory (r/r) Diffuse Large B-Cell Lymphoma (DLBCL)  Documentation of patient being at least 18 years old.  Documentation that treatment is not for primary central nervous system lymphoma.  Documentation of treatment failure with at least two lines of systemic therapy  Documentation of relapsed (second or later relapse) or refractory diffuse large B-cell lymphoma not otherwise specified OR  High grade B-cell lymphoma OR  DLBCL arising from follicular lymphoma Appropriate Pediatric and Young Adult Relapsed or Refractory (r/r) B-cell Acute Lymphoblastic Treatment Leukemia (ALL) Regimen & Other  Completion of lymphodepleting therapy before initiation of Kymriah. Fludarabine (30 Criteria: mg/m2 intravenous daily for 4 days) and cyclophosphamide (500 mg/m2 intravenous daily for 2 days starting with the first dose of fludarabine).  Infuse Kyrmiah 2 to 14 days after completion of lymphodepleting chemotherapy.  Dosing for patients 50 kg or less: administer 0.2 to 5.0 x 106 CAR positive viable T cells per KG of body weight.  Dosing for patients above 50 kg: administer 0.1 to 2.5 x 108 CAR positive viable T cells  Chimeric antigen receptor (CAR)-positive viable T cells based on the patient weight reported at the time of leukapheresis.

179

Reauthorization not supported by compendia.

Adult Relapsed or Refractory (r/r) Diffuse Large B-Cell Lymphoma (DLBCL)  Completion of lymphodepleting therapy 2 to 11 days before initiation of Kymriah. Fludarabine (25 mg/m2 IV daily for 3 days) and Cyclophosphamide (250 mg/m2 IV daily for 3 days starting with the first dose of fludarabine)  Alternative pre-treatment: Bendamustine 90 mg/m2 IV daily for 2 days  Pretreatment with lymphodepleting chemotherapy may be omitted if patient's WBC is 1 x 109/L or less within 1 week prior to tisagenlecleucel  Premedication, give acetaminophen and diphenhydrAMINE or other H1-antihistamine 30 to 60 minutes before infusion. Avoid use of prophylactic systemic corticosteroids, as it may interfere with the activity of tisagenlecleucel  A single dose of KYMRIAH contains 0.6 to 6.0 x 108 CAR-positive viable T cells suspended in one or more patient-specific infusion bag(s) for i.v. infusion.

Reauthorization not supported by compendia. Exclusion Criteria:  Concomitant use of granulocyte colony-stimulating factors.  Unresolved serious adverse reactions from chemotherapy, active uncontrolled infection, active GVHD, or increasing leukemia burden following lymphodepleting chemotherapy. Age Restriction: ALL  Safety and effectiveness in patients 25 years and older have not been established. DLBCL  Safety and effectiveness in patients under 18 years have not been established. Prescriber  Prescribed by an oncologist Restrictions: Coverage  Initial approval: 2 months, unless otherwise specified Duration:  Reauthorization: None

180

POLICY NAME: KYNAMRO Affected Medications: KYNAMRO (Mipomersen Sodium)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required  Diagnosis of homozygous familial hypercholesterolemia confirmed by one of the following: Medical (1) history of genetic testing confirming 2 mutated alleles at the LDLr gene locus, or (2) Information: documented history of untreated LDL-C greater than 500 mg/dL and at least one of the criteria (a) tendinous and/or cutaneous xanthoma prior to age 10 years or (b) documentation of elevated LDL-C greater than 190 mg/dL prior to lipid-lowering therapy consistent with heterozygous familial hypocholesteremia in both parents AND  Recent Lipid Panel (within prior 3 months): For initial approval, LDL-C must be greater than 300 mg/dl on optimal lipid lowering therapy AND  Documentation of baseline LDL-C (untreated) AND  Documentation of failure to daily combination use of the following maximally tolerated doses of lipid lowering medications: statins, ezetimibe, nicotinic acid, bile acid sequestrants, fibrates for minimum 6 months - may review pharmacy profile if available AND  Documentation of failure to a PCSK9 inhibitor for minimum 3 months - may review pharmacy profile if available AND  Documentation of failure or justification for avoidance of LDL apheresis AND  Recent Liver Function Tests (LFTs) AND Child-Pugh Classification OR bilirubin, albumin, INR, ascites status, and encephalopathy status to calculate Child-Pugh score within 3 months AND  Documentation of planned use as adjunct with maximally tolerated oral lipid lower therapy.  Reauthorization requires updated lipid panel and LFTs with documentation that the benefit of therapy is greater than the risk of hepatotoxicity if rise in LFTs

Appropriate  Documented plan to monitor liver function tests every 3 months Treatment Regimen & Other Criteria: Exclusion  Moderate or severe hepatic impairment (Child-Pugh class B or C) Criteria:  Active liver disease or unexplained persistent elevations of serum transaminases  Severe renal impairment  Use of Kynamro as adjunct to LDL apheresis or PCSK9 inhibitor Age Restriction:  18 years of age and older

181

Prescriber  Endocrinologist, cardiologist, or lipid specialist Restrictions:

Coverage  Initial approval: 6 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

182

POLICY NAME: LEMTRADA Affected Medications: LEMTRADA (alemtuzumab)

Covered Uses:  All FDA approved indications.

Required Medical  Diagnosis of relapsing form of multiple sclerosis confirmed with MRI (Revised McDonald Information: diagnostic criteria for multiple sclerosis) AND  Inadequate response to 2 or more medications indicated for MS (per FDA-approved indication) such as interferon beta-1a, pegylated interferon beta-1a, glatiramer 20mg, glatiramer 40mg, glatopa 20mg, natalizumab, dimethyl fumarate, or teriflunomide AND  Patient has completed any necessary immunizations (at least 6 weeks prior to treatment) AND  Corticosteroid prophylaxis will be provided immediately prior to infusions AND  Herpes prophylaxis will be provided starting on the first day of each treatment course and continue for at least two months or until CD4+ lymphocyte count is 200 cells per microliter or greater (whichever occurs later)

Appropriate  Initial dosage of 12mg IV daily on 5 consecutive days. Treatment  For final dosage a year later, 12mg IV daily on 3 consecutive days. Regimen & Other Criteria: Exclusion Criteria:  Patients infected with Human Immunodeficiency Virus (HIV) Age Restriction:  Greater than or equal to 17 years of age Prescriber  Multiple sclerosis specialists and health care facilities enrolled and certified with the Restrictions: Lemtrada REMS program Coverage  Initial: 5 doses for 5 days, unless otherwise specified Duration:  For final dosage a year later (3 doses for 3 days), provide documentation of success

183

POLICY NAME: LETAIRIS Affected Medications: LETAIRIS (ambrisentan)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design

Required Medical Pulmonary arterial hypertension (PAH) WHO Group 1 Information:  Documentation of PAH confirmed by right-heart catheterization  Etiology of PAH (idiopathic, heritable, or associated with connective tissue disease)  NYHA/WHO Functional Class II, III, or IV symptoms  Liver Function Tests within normal limits prior to initiation  Documentation of Acute Vasoreactivity Testing (positive result requires trial/failure to calcium channel blocker) Appropriate  Supportive care should be considered as first-line (anticoagulants, diuretics, oxygen, Treatment digoxin). Regimen & Other  Documentation of trial with at least 1 PDE5 inhibitor (unless contraindicated) OR Criteria: patient at high risk necessitating endothelin receptor antagonist.  Consider Letairis as second-line therapy in patients with WHO-FC IV symptoms

 Not recommended for patients with PAH secondary to heart failure with severe systolic dysfunction  Not recommended for patients with moderate to severe liver impairment

 Subsequent approvals require documentation of treatment success such as improved walking distance or improvements in functional class

Exclusion Criteria:  Pregnancy  Idiopathic Pulmonary Fibrosis (IPF), including IPF patients with PAH (WHO Group 3)

Age Restriction: Prescriber  Cardiologist or Pulmonologist Restrictions: Coverage Duration:  12 months, unless otherwise specified

184

POLICY NAME: LEUKINE Affected Medications: LEUKINE (sargramostim)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical  Complete blood counts with differential and platelet counts will be monitored at baseline Information: and regularly throughout therapy  Documentation of therapy intention (curative, palliative)  Documentation of risk factors both medication therapy regimen and patient specific  Documentation of planned treatment course

Hematopoietic Syndrome of Acute Radiation Syndrome [H-ARS]:  Documentation of acute exposure to myelosuppressive doses of radiation and comprehensive review by medical team  Documentation of planned treatment course and patient weight

Appropriate  For prophylaxis of febrile neutropenia (FN) or other dose-limiting neutropenic events for Treatment patients receiving myelosuppressive anti-cancer drugs: Regimen &  Meets one of the following: Other Criteria: . a) Curative Therapy: High risk (greater than 20% risk) based on chemotherapy regimen OR intermediate risk (10-20% risk) based on chemotherapy regimen with documentation of significant risk factors for development of febrile neutropenia OR has experienced a dose-limiting neutropenic event on a previous cycle of current chemotherapy to be continued and dose-reduction of current chemotherapy to be continued is not an option due to impact on patient survival . b) Palliative Therapy: This will not be approved upfront for prophylaxis of febrile neutropenia. Chemotherapy regimens with a 20% or greater risk of neutropenic events should not be used. If however a dose limiting neutropenic event occurs on a previous cycle of chemotherapy, and the effectiveness of chemotherapy will be reduced with dose reduction, will be approved for secondary prophylaxis on a case by case basis. . Significant risk factors for development of febrile neutropenia include: prior chemotherapy or radiation therapy, persistent neutropenia, bone marrow involvement by tumor, recent surgery and/or open wounds, liver dysfunction (bilirubin greater than 2), renal dysfunction (creatinine clearance less than 50), age greater than 65 years while receiving full chemotherapy dose intensity Exclusion  Leukemic myeloid blasts in peripheral blood of 10% or greater Criteria:

Age Restriction:

185

Prescriber  Prescribed by an oncologist, hematologist, or bone marrow transplant specialist Restrictions:

Coverage  3 months, unless otherwise specified Duration:  H-ARS: 1 month, unless otherwise specified

186

POLICY NAME: LEUPROLIDE Affected Medications: Lupron Depot 3.75 and 11.25mg AND Lupron Depot-Ped 11.25mg; Lupron Depot 7.5, 22.5, 30, and 45mg AND Lupron Depot-Ped 15mg AND Eligard; Leuprolide Acetate or injection solution

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design  NCCN indications level 2A or higher  Gender dysphoria Required Medical Endometriosis Information:  Documentation of treatment failure or contraindication to continuous oral contraceptive regimen (no placebo pills) Preoperative uterine leiomyomata  Documentation of leiomyoma-related surgery in 6 or less months  Documentation of planned use in combination with iron supplements Gender dysphoria  Documentation of baseline estradiol and testosterone levels to confirm onset of puberty  Documentation of current Tanner stage 2 or greater  Documentation from a licensed mental health professional (LMHP) confirming diagnosis and addressing the patient’s general identifying characteristics; I. The initial and evolving gender and any associated mental health concerns, and other psychiatric diagnoses; II. The duration of the referring licensed mental health professional’s relationship with the client, including the type of evaluation and psychotherapy to date; III. The clinical rationale for supporting the client’s request for hormone therapy and statement that the client meets eligibility criteria; and IV. Permission to contact the licensed mental health professional for coordination of care Appropriate  Women of childbirth age should have pregnancy ruled out and a plan to use a non- Treatment hormonal based contraceptive during therapy Regimen & Other Endometriosis Criteria:  Lupron Depot 3.75 and 11.25mg Preoperative uterine leiomyomata  Lupron Depot 3.75 and 11.25mg  Experience has been limited to women 18 years and older and treated for 6 months or less  Must be given in conjunction with iron supplementation Central precocious puberty  Leuprolide Acetate, Lupron Depot-Ped 11.25 and 15mg Gender dysphoria  Comprehensive mental health evaluation should be provided in accordance with most current version of the World Professional Association for Transgender Health (WPATH) Standards of Care  Therapy should be started at onset of puberty, but no earlier than Tanner stages 2-3 187

 Fulfill eligibility and readiness criteria from Journal of Clinical Endocrinology and Metabolism (JCEM) guidelines

Exclusion Criteria:  Undiagnosed and/or abnormal vaginal bleeding  Management of uterine leiomyomata without intention of undergoing surgery.  Pregnancy or breastfeeding  Gender dysphoria patients 18 years or older Age Restriction:  Gender dysphoria: less than 18 years old Prescriber  Prescribed by or in consultation with oncologist, endocrinologist, or gynecologist for Restrictions: endometriosis  Gender Dysphoria: Diagnosis made and prescribed by a mental health specialist with experience in gender dysphoria Coverage Duration:  Uterine leiomyomata: maximum of 6 months, unless otherwise specified, deny reauthorization requests  Endometriosis: 6 months, unless otherwise specified  All other diagnoses: 12 months, unless otherwise specified

188

POLICY NAME: LIDOCAINE PATCH Affected Medications: Lidocaine Patch

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  Diabetic neuropathic pain Required  Diagnosis of post-herpetic neuralgia: requires trial of gabapentin OR Medical  Diagnosis of diabetes (for diabetic neuropathy) Information:  All medications tried/failed for indicated diagnosis

Appropriate Diabetic Neuropathic Pain: Treatment  Documented inadequate treatment response or intolerance to a minimum of 3 other Regimen & pharmacologic therapies commonly used to treat neuropathic pain such as Other Criteria: gabapentin, serotonin norepinephrine reuptake inhibitors (SNRIs): duloxetine, venlafaxine, desvenlafaxine, and tricyclic antidepressants (TCAs)

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:

Age Restriction:

Prescriber Restrictions:

Coverage  Approval: 12 months, unless otherwise specified Duration:

189

POLICY NAME: LUXTURNA Affected Medications: LUXTURNA (voretigene neparvovec-rzyl intraocular suspension for subretinal injection)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.  Inherited Retinal Dystrophies (IRD) caused by mutations in the retinal pigment epithelium-specific protein 65kDa (RPE65) gene Required Medical  Diagnosis of a confirmed biallelic RPE65 mutation-associated retinal dystrophy (e.g. Information: Leber’s congenital amaurosis [LCA], Retinitis pigmentosa [RP] Early Onset Severe Retinal Dystrophy [EOSRD], etc.); AND  Genetic testing documenting biallelic mutations of the RPE65 gene; AND  Visual acuity of at least 20/800 OR have remaining light perception in the eye(s) receiving treatment AND  Visual acuity of less than 20/60 OR a visual field of less than 20 degrees AND  Presence of neural retina and a retinal thickness greater than 100 microns within the posterior pole as assessed by optical coherence tomography with AND have sufficient viable retinal cells as assessed by the treating physician Appropriate Treatment Regimen & Other Criteria:

Exclusion Criteria:  Patient has been previously enrolled in clinical trials of gene therapy for retinal dystrophy RPE65 mutations or been previously been treated with gene therapy for retinal dystrophy in the eye(s) receiving treatment  Patient has other pre-existing eye conditions or complicating systemic diseases that would eventually lead to irreversible vision loss and prevent the patient from receiving full benefit from treatment (eg. severe diabetic retinopathy) Age Restriction:  12 months of age and older Prescriber  Ophthalmologist or retinal surgeon with experience providing sub-retinal injections Restrictions:

Coverage  Approval: 1 month - 1 injection per eye, per lifetime Duration:

190

POLICY NAME: LYRICA Affected Medications: LYRICA (pregabalin)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Initial approval requires documented failure, intolerance, or clinical rationale Information: for avoidance to gabapentin Appropriate Treatment  Subsequent approval requires documentation of treatment success. Regimen & Other Criteria: Exclusion Criteria:

Age Restriction:

Prescriber Restrictions: Coverage Duration:  Initial approval: 12 months, unless otherwise specified

191

POLICY NAME: MAKENA Affected Medications: MAKENA (Hydroxyprogesterone Caproate) and Hydroxyprogesterone Caproate

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  NCCN indications with evidence level of 2A or higher Required Medical  Oncology Indications Information: o Documentation of performance status, all prior therapies used, and prescribed treatment regimen. Consider holding therapy if Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater. o Documentation of trial and failure prescription progesterone products (medroxyprogesterone, progestin-based therapies)  Preterm Labor Prevention o Singleton pregnant patient o History of singleton spontaneous preterm birth (less than 37 weeks) o Expected date of delivery Appropriate Preterm Labor Prevention Treatment  Initial approval requires: Regimen & Other  History of prior singleton preterm birth (less than 37 weeks) OR Criteria:  Documented failure, intolerance, or clinical rationale for avoidance of vaginal progesterone Exclusion Criteria:  Current or history of any of the following: o multiple gestations or other risk factors for preterm birth o Thrombosis or thromboembolic disorders o Known or suspected breast cancer or other hormone-sensitive cancer, or history of these conditions o Undiagnosed abnormal vaginal bleeding unrelated to pregnancy o Cholestatic jaundice of pregnancy o Liver tumors, benign or malignant, or active liver disease o Uncontrolled hypertension.

Age Restriction:  16 years of age or older

Prescriber  Oncology use: Oncologist Restrictions: Coverage  Oncology Duration: o Initial Approval- 4 Months, unless otherwise specified o Reauthorization- 12 months, unless otherwise specified  Preterm Labor Prevention o Approval- 20 weeks, unless otherwise specified

192

POLICY NAME: MAVENCLAD Affected Medications: MAVENCLAD (cladribine)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design o Treatment of relapsing forms of multiple sclerosis (MS), to include relapsing- remitting disease (RRMS) and active secondary progressive (SPMS) disease, in adults. Required Medical  Diagnosis of relapsing or active secondary progressive forms of Multiple Sclerosis Information: (MS) confirmed with MRI (Revised McDonald diagnostic criteria for multiple sclerosis) o Clinical evidence alone will suffice; additional evidence desirable but must be consistent with MS  Documentation of previous therapies tried/failed with duration of trial.  Complete blood count (CBC) with differential including lymphocyte count at baseline.  Transaminase within 6 months before initiation of treatment Appropriate  Documented failure with at least two other disease-modifying therapies (DMTs) for Treatment multiple sclerosis (MS) for at least 3 months Regimen & Other Criteria: Reauthorization (1 time only):  Documentation of clinical treatment success  Administer second course starting at least 43 weeks after the last dose of the first course  Dosing according to the approved label: Dose of MAVENCLAD per Cycle by Patient Weight in Each Treatment Course Weight Range Dose in mg (number of 10 mg tablets) per cycle Kg First Cycle Second Cycle 40* to less than 50 40 mg (4 tablets) 40 mg (4 tablets) 50 to less than 60 50 mg (5 tablets) 50 mg (5 tablets) 60 to less than 70 60 mg (6 tablets) 60 mg (6 tablets) 70 to less than 80 70 mg (7 tablets) 70 mg (7 tablets) 80 to less than 90 80 mg (8 tablets) 70 mg (7 tablets) 90 to less than 100 90 mg (9 tablets) 80 mg (8 tablets) 100 to less than 110 100 mg (10 tablets) 90 mg (9 tablets) 110 and above 100 mg (10 tablets) 100 mg (10 tablets) *The use of MAVENCLAD in patients weighing less than 40 kg has not been investigated Exclusion Criteria:  Patients with current malignancy  Pregnant women or women and men of reproductive potential who do not plan to use effective contraception because of the risk of fetal harm.  Treatment naïve  Treatment beyond 2 years

193

Age Restriction:  Use in patients younger than 18 years of age has not been established.

Prescriber  Prescribed by or in consultation with a neurologist or an MS Specialist Restrictions: Coverage Duration:  Initial Authorization: 2 months, unless otherwise specified  Reauthorization: 2 months – at least 43 weeks after last dose, unless otherwise specified

194

POLICY NAME: MAYZENT Affected Medications: MAYZENT (Siponimod)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design • Treatment of relapsing forms of multiple sclerosis (MS), to include clinically isolated syndrome, relapsing-remitting disease, and active secondary progressive disease, in adults Required Medical  Documentation of diagnosis of relapsing forms of Multiple Sclerosis (MS) confirmed Information: with MRI (Revised McDonald diagnostic criteria for MS)  Documentation of ECG, CBC, liver function tests, ophthalmic evaluation, and CYP2C9 genetic testing  Documentation of antibodies to varicella zoster virus (VZV) or vaccination of antibody- negative patients prior to treatment initiation

Secondary-Progressive MS (SPMS)  Documentation of prior history of relapsing-remitting MS (RRMS) with progressive increase in disability over at least 6 months, independent of, or in the absence of, relapses  Documentation of active disease classified as the presence of clinical relapse or inflammatory activity (i.e. new or enlarging T2 lesions or gadolinium enhancing lesions on MRI) in the last 2 years  Documentation of Expanded Disability Status Scale (EDSS) score of 3.0 to 6.5 Appropriate  After treatment titration, the recommended maintenance dosage of Mayzent is 2 mg Treatment taken orally once daily starting on Day 6. Dosage adjustment is required in patients with Regimen & Other a CYP2C9*1/*3 or *2/*3 genotype Criteria: o If one titration dose is missed for more than 24 hours, treatment needs to be reinitiated with Day 1 of the titration regimen o In patients with a CYP2C9*1/*3 or *2/*3 genotype, after treatment titration, the recommended maintenance dosage of Mayzent is 1 mg taken orally once daily starting on Day 5  Discontinuation of treatment if liver enzymes exceed three times the upper limit of normal (ULN) with signs of liver dysfunction (unexplained nausea, vomiting, abdominal pain, fatigue, anorexia, rash with eosinophilia, or jaundice and/or dark urine)  Reauthorization will require documentation of clinical treatment success as determined by treating provider Exclusion  CYP2C9*3/*3 genotype Criteria:  Recent (in the past 6 months) MI, unstable angina, stroke, TIA, decompensated HF requiring hospitalization or class III or IV HF  Mobitz type II second- or third-degree AV block or sick sinus syndrome (unless patient has functioning pacemaker) Age Restriction:

195

Prescriber  Prescribed by or in consultation with a neurologist or multiple sclerosis specialist Restrictions: Coverage  Initial approval: 12 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

196

POLICY NAME: MEPSEVII Affected Medications: MEPSEVII (vestronidase alfa-vjbk)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design

Required Medical  Definitive diagnosis of Mucopolysaccharidosis VII (MPS VII; Sly syndrome) Information: syndrome confirmed by BOTH of the following:  Beta-glucuronidase enzyme deficiency in peripheral blood leukocytes AND  Detection of pathogenic mutations in the GUSB gene by molecular genetic testing  Baseline value for one or more of the following:  Bruininks-Oseretsky Test of Motor Proficiency  6 minute walk test  Pulmonary function tests Appropriate Treatment  4 mg/kg infusion (maximum 290mg) every 2 weeks Regimen & Other  Reauthorization will require: Criteria:  Documentation of absence of unacceptable toxicity (ex. anaphylaxis or severe allergic reactions) AND  Patient has responded to therapy compared to pretreatment baseline in one or more of the following: o Improvement in Bruininks-Oseretsky Test of Motor Proficiency o Improvement in 6 minute walk test o Reduction in liver and/or spleen volume o Stability or improvement in pulmonary function tests Exclusion Criteria: Age Restriction:  Age 8 - 25 years Prescriber  Prescriber with experience in treating MPS Restrictions: Coverage Duration:  Initial approval: 2 months, unless otherwise specified  Reauthorization: 6 months, unless otherwise specified

197

POLICY NAME: MIACALCIN Affected Medications: MIACALCIN Injection (calcitonin-salmon)

Covered Uses:  Paget’s disease of bone, hypercalcemia.

Required Medical  Hypercalcemia Information: o Documented calcium level greater than or equal to 14 mg/dL (3.5 mmol/L)  Paget’s disease of bone o Documented baseline radiograph findings o Abnormal liver function test (LFT), including alkaline phosphatase o Documented lack of malignancy within the past 3 months

Appropriate  Hypercalcemia Treatment o Documentation that additional methods for lowering calcium (such as Regimen & Other intravenous fluids) did not result in adequate efficacy OR Criteria: o Clinical judgement necessitated immediate administration without waiting for other methods to show efficacy  Paget’s disease of bone o Trial and failure of zoledronic acid AND oral bisphosphonates OR o Documentation indicating zoledronic acid or oral bisphosphonates are not be suitable for the member o Documentation of normal vitamin D level and/or supplementation o Documentation of normal calcium level and/or supplementation o Documentation of symptoms experienced by member necessitating treatment with medication (i.e. pain, bone deformity)

Re-Authorization criteria – Paget’s disease of bone:  Documentation of treatment efficacy (i.e. stable or lowered alkaline phosphatase level, resolution of symptoms)

Exclusion Criteria:  Related to Paget’s disease of bone o History of a skeletal malignancy or bone metastases o Concurrent use of zoledronic acid or oral bisphosphonates o Asymptomatic Paget’s Disease of the bone  Indication of osteoporosis, prevention of osteoporosis

Age Restriction:  18 years or older - for Paget’s disease of bone only Prescriber Restrictions:

Coverage Duration:  Approval = 12 months, unless otherwise specified

198

199

POLICY NAME: MITOXANTRONE Affected Medications: MITOXANTRONE (mitoxantrone)

Covered Uses:  NCCN indications with evidence level of 2A or higher  Breast cancer, recurrent or metastatic  Hodgkin’s lymphoma  Liver carcinoma  Non-Hodgkin lymphoma with following subtypes: Adult T-cell leukemia/lymphoma, AIDS-related B-cell lymphoma, Diffuse large B-cell lymphoma, Follicular lymphoma, Gastric and nongastric MALT lymphoma, Lymphoblastic lymphoma, Mantle cell lymphoma, Mycosis Fungoides/Sézary syndrome, Peripheral T-cell lymphoma, Primary cutaneous B-cell lymphoma, Splenic marginal zone lymphoma, T-cell prolymphocytic leukemia  Ovarian cancer  Multiple sclerosis, Secondary progressive, progressive relapsing, or worsening relapsing-remitting; to reduce neurologic disability and/or frequency of clinical relapses

Required  Referral for mitoxantrone Medical  Assessed for cardiac signs and symptoms by history, physical exam, and ECG prior Information: to starting mitoxantrone  Baseline evaluation of left ventricular ejection fraction  Diagnosis of Non- Hodgkin Lymphoma with one of the subtypes listed in the above section (If yes, skip directly to coverage duration), OR  Diagnosis of any other cancers listed in the above section (If yes, skip directly to coverage duration), OR  Assessed for cardiac signs and symptoms by history, exam, and ECG prior to each dose  Patient will undergo quantitative reevaluation of LVEF prior to each dose using the same methodology that was used to assess baseline LVEF. Additional doses of Mitoxantrone should not be administered to MS patients who have experienced either a drop in LVEF to below the lower limit of normal or a clinically significant reduction in LVEF during Mitoxantrone therapy  Should undergo yearly quantitative LVEF evaluation after stopping Mitoxantrone to monitor for late occurring cardiotoxicity

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Appropriate Dosing for MS Patients: Treatment  12mg/m2 IV every 3 months

200

Regimen & Other Criteria: Exclusion For MS Patients: Criteria:  Baseline LVEF below the lower limit of normal  Receive a cumulative Mitoxantrone dose greater than 140 mg/m2 Age Restriction: Prescriber Restrictions: Coverage  Approval (Non-Hodgkin Lymphoma): 6 months, unless otherwise specified Duration:  Approval (All other covered cancer diagnoses): 6 months, unless otherwise specified  Approval (MS): 12 months, unless otherwise specified

201

POLICY NAME: MULPLETA Affected Medications: MULPLETA (lusutrombopag)

Covered Uses: ● All FDA-approved indications not otherwise excluded by plan design

Required Medical  Complete blood count with differential and platelet count Information:  Liver function tests Appropriate  Documentation of procedure and baseline platelet count is required for Treatment prescribing Regimen & Other  Dosing: Criteria:  Begin Mulpleta dosing 8-14 days prior to a scheduled procedure.  Patients should undergo their procedure 2-8 days after the last dose.  Recommended Dosage: 3 mg orally once daily with or without food for 7 days.  Documented inability to respond adequately to Promacta  Consideration for reapproval of therapy requires response to treatment with platelet count of at least 50,000/mcL or above without significant liver function abnormalities during procedure Exclusion Criteria:  Platelet count above 50,000/mcL at baseline  A history of splenectomy, partial splenic embolization, or thrombosis and those with Child-Pugh class C liver disease, absence of hepatopetal blood flow, or a prothrombotic condition other than chronic liver disease Age Restriction:

Prescriber  Prescribed by or in consultation with hematologist or gastroenterology/liver Restrictions: specialist

Coverage  Approval: 1 month (7 days of treatment), based on planned procedure date Duration:

202

POLICY NAME: MYALEPT Affected Medications: MYALEPT (metreleptin)

Covered Uses:  All Food and Drug Administration (FDA)-approved indications not otherwise excluded by plan design.  Congenital or acquired generalized lipodystrophy.

Required Medical  Weight Information:  Baseline serum leptin levels, HbA1c, fasting glucose, fasting triglycerides, fasting serum insulin  Prior Myalept use will require test of anti-metrepeptin antibodies Appropriate  Serum leptin < 6.0 ng/mL females and < 3.0 ng/mL males, obtained on at least 2 Treatment occasions Regimen & Other  If treating acquired generalized lipodystrophy with concurrent Criteria: hypertriglyceridemia defined as triglycerides ≥ 500 mg/dL despite optimizing with statin and/or fibrate  If treating acquired generalized lipodystrophy with concurrent diabetes, baseline HbA1c ≥ 7% despite optimal treatment with metformin, TZD, sulfonylurea, GLP-1 agonist or DPP-4 inhibitor, SGLT-2, and insulin  Treatment success defined by improvement in HbA1c, fasting glucose, and fasting triglycerides  Worsening metabolic control and/or severe infection = indicators of possible anti-metreleptin antibodies  Maximum daily dose for individuals <40kg = 0.13mg/kg  Maximum daily dose for individuals >40kg = 10mg/day

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Partial lipodystrophy  General obesity not associated with leptin deficiency  HIV-related lipodystrophy  Metabolic disease, including diabetes mellitus and hypertriglyceridemia, without concurrent evidence of generalized lipodystrophy  Age > 65 years Age Restriction:  Age ≥ 1 year Prescriber  Prescribed by, or in consultation with, an Endocrinologist Restrictions:  Myalept is available only through the MYALEPT REMS Program

203

Coverage  Initial: 4 months, unless otherwise specified Duration:  Subsequent: 12 months, unless otherwise specified

204

POLICY NAME: MYELOID GROWTH FACTORS Affected Medications: FULPHILA (pegfilgrastim-jmdb), NEULASTA (pegfilgrastim), UDENYCA (pegfilgrastim-cbqv), NEUPOGEN (filgrastim), NIVESTYM (filgrastim-aafi), GRANIX (tbo-filgrastim), ZARXIO (filgrastim-sndz)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.  Food and Drug Administration (FDA)-approved indications:

Neupogen, Nivestym & Zarxio

Patients with Cancer Receiving Myelosuppressive Chemotherapy  Indicated to decrease the incidence of infection‚ as manifested by febrile neutropenia‚ in patients with non-myeloid malignancies receiving myelosuppressive anti-cancer drugs associated with a significant incidence of severe neutropenia with fever.

Patients With Acute Myeloid Leukemia Receiving Induction or Consolidation Chemotherapy  Indicated for reducing the time to neutrophil recovery and the duration of fever, following induction or consolidation chemotherapy treatment of adults with acute myeloid leukemia.

Patients with Cancer Receiving Bone Marrow Transplant  Indicated to reduce the duration of neutropenia and neutropenia-related clinical sequelae‚ (e.g., febrile neutropenia) in patients with non-myeloid malignancies undergoing myeloablative chemotherapy followed by marrow transplantation.

Patients Undergoing Autologous Peripheral Blood Progenitor Cell Collection and Therapy  Indicated for the mobilization of autologous hematopoietic progenitor cells into the peripheral blood for collection by leukapheresis.

Patients With Severe Chronic Neutropenia  Indicated for chronic administration to reduce the incidence and duration of sequelae of neutropenia (e.g.‚ fever‚ infections‚ oropharyngeal ulcers) in symptomatic patients with congenital neutropenia‚ cyclic neutropenia‚ or idiopathic neutropenia.

Fulphila & Udenyca

205

Granix  Granix is indicated to reduce the duration of severe neutropenia in patients with non-myeloid malignancies receiving myelosuppressive anti-cancer drugs associated with a clinically significant incidence of febrile neutropenia.

Neulasta Patients with Cancer Receiving Myelosuppressive Chemotherapy Patients with Hematopoietic Subsyndrome of Acute Radiation Syndrome  Neulasta is indicated to increase survival in patients acutely exposed to myelosuppressive doses of radiation  Neulasta is not indicated for the mobilization of peripheral blood progenitor cells for hematopoietic stem cell transplantation

Compendia supported uses (Neupogen/Granix/Zarxio/Nivestym):  Treatment of chemotherapy-induced febrile neutropenia in patients with non- myeloid malignancies  Treatment of anemia in patients with myelodysplastic syndromes (MDS)  Treatment of neutropenia in patients with MDS  Following chemotherapy for acute lymphocytic leukemia (ALL)  Stem cell transplantation-related indications  Agranulocytosis  Aplastic anemia  Neutropenia related to HIV/AIDS  Neutropenia related to renal transplantation

Required  Complete blood counts with differential and platelet counts will be monitored at Medical baseline and regularly throughout therapy Information:  Documentation of therapy intention (curative, palliative) for prophylaxis of febrile neutropenia  Documentation of risk factors both medication therapy regimen and patient specific  Documentation of planned treatment course  Documentation of current patient weight Appropriate  Coverage for the non-preferred products, Neupogen or Granix, is provided when Treatment the member meets one of the following criteria: Regimen &  Documented treatment failure or intolerable adverse event to Nivestym AND Other Criteria: Zarxio  Documented latex allergy and the prescriber states that the member must use latex-free vials  Neupogen or Granix are requested for doses less than 180 mcg  Neulasta requests require rationale for use over biosimilar products, Fulphila and Udenyca

 For prophylaxis of febrile neutropenia (FN) or other dose-limiting neutropenic events for patients receiving myelosuppressive anti-cancer drugs: . Meets one of the following: a) Curative Therapy: High risk (greater than 20% risk) OR intermediate risk (10-20% risk) for febrile neutropenia based on chemotherapy regimen with 206

documentation of significant risk factors for serious medical consequences OR has experienced a dose-limiting neutropenic event on a previous cycle of current chemotherapy to be continued b) Palliative Therapy: Neulasta will not be approved upfront for prophylaxis of febrile neutropenia in the palliative setting. Per the NCCN, chemotherapy regimens with a 20% or greater risk of neutropenic events should not be used. If however a dose limiting neutropenic event occurs on a previous cycle of chemotherapy, and the effectiveness of chemotherapy will be reduced with dose reduction, growth factor will be approved for secondary prophylaxis on a case by case basis.

 Neupogen, Nivestym and Zarxio in the use of Severe Chronic Neutropenia, Must meet ALL of the following: . Congenital neutropenia, cyclic neutropenia, OR idiopathic neutropenia . Current documentation of ANC less than 500 cells/microL . Neutropenia symptoms (fever, infections, orophanryngeal ulcers) . CBC with differential and platelet counts, bone marrow morphology, and karyotype Exclusion Criteria: Age Restriction: Prescriber  Prescribed by oncologist, hematologist Restrictions: Coverage  6 months, unless otherwise specified Duration:

207

POLICY NAME: NAGLAZYME Affected Medications: NAGLAZYME (galsulfase)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  The patient has a diagnosis of mucopolysaccharidosis VI (MPS VI or Maroteaux-Lamy Information: syndrome)  The diagnosis was confirmed by an enzyme assay demonstrating a deficiency in N- acetylgalactosamine 4-sulfatase (arylsulfatase B) enzyme activity or by DNA testing  Documented current weight of patient for dosing

Appropriate Dosing: Treatment  The recommended dosage regimen of NAGLAZYME is 1 mg/kg of body weight Regimen & Other administered once weekly as an intravenous infusion. Criteria: Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:

Age Restriction: Prescriber Restrictions:

Coverage Duration:  Approval 3 months, unless otherwise specified  Subsequent approval 12 months, unless otherwise specified

208

POLICY NAME: NATPARA Affected Medications: NATPARA (parathyroid hormone)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  Diagnosis of hypoparathyroidism AND Information:  Hypocalcemia uncontrolled on calcium and active forms of vitamin D alone  25-hydroxyvitamin D levels are sufficient (approximately 30-74 ng/mL). If insufficient, replace to sufficient levels per standard of care  Total serum calcium (albumin-corrected) greater than 7.5 mg/dL

Appropriate  Natpara to be must be used in conjunction with calcium and vitamin D, documentation Treatment of taking at least 2,000g/day of calcium and vitamin d regularly for over a 2 month time Regimen & Other is required for coverage. Criteria: Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:

Age Restriction:

Prescriber  Endocrinologist Restrictions: Coverage Duration:  Initial approval: 6 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

209

POLICY NAME: NILANDRON Affected Medications: NILUTAMIDE

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, disease staging, all prior therapies used, and Information: prescribed treatment regimen Prostate Cancer  Documentation of metastatic prostate cancer  Documentation of no prior treatment with Xtandi (enzalutamide) and Zytiga (abiraterone)  Documented treatment failure with LHRH agonist combined with biclutamide  Documented treatment failure or contraindication to flutamide Appropriate Treatment  Reauthorization: documentation of disease responsiveness to therapy Regimen & Other Criteria: Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater

Age Restriction: Prescriber Restrictions:  Oncologist

Coverage Duration:  Initial approval: 3 month, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

210

POLICY NAME: NINLARO Affected Medications: NINLARO (ixazomib)

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, disease staging, all prior therapies used, Information: and prescribed planned treatment course

Multiple Myeloma  Documentation of progression with at least 1 line of standard therapy  Should be used in combination with lenalidomide and dexamethasone

Appropriate Treatment  Documentation of no disease refractory to lenalidomide or proteasome inhibitors Regimen & Other (Velcade, Kyprolis) Criteria:  Subsequent approval: documentation of disease responsiveness to therapy

Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction:  18 years or older Prescriber Restrictions:  Oncologist Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

211

POLICY NAME: NPLATE Affected Medications: NPLATE (romiplostim)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan o Adult patients with chronic immune thrombocytopenia (ITP) who have had an insufficient response to corticosteroids, immunoglobulins, or splenectomy o Pediatric patients 1 year of age and older with ITP for at least 6 months who have had an insufficient response to corticosteroids, immunoglobulins, or splenectomy Required Medical  Complete blood count with differential and platelet count Information:  Patient Weight

Thrombocytopenia in patients with immune thrombocytopenia pupura (ITP)  All therapies tried/failed  Documentation of splenectomy status Appropriate Thrombocytopenia in patients with ITP Treatment  Documentation of platelet count less than 20 x 109/L AND Regimen & Other  Documentation of clinically significant bleeding AND Criteria:  Must fail at least 2 therapies for ITP, including corticosteroids or immunoglobulin (defined as platelets did not increase to at least 50 x 109/L) OR  Documentation of splenectomy

Reauthorization  Response to treatment with platelet count of at least 50 x 109/L (not to exceed 400 x 109/L) OR  The platelet counts have not increased to a platelet count of at least 50 x 109/L and the patient has NOT been on the maximum dose for at least 4 weeks

Exclusion Criteria:  Treatment of thrombocytopenia due to myelodysplastic syndrome (MDS)  When attempting to normalize platelet count  Using in combination with thrombopoietin receptor agonist (Promacta) or similar treatments. Age Restriction: Prescriber  Prescribed by or in consultation with a hematologist Restrictions:

Coverage Duration:  Initial Approval = 3 months, unless otherwise specified  Renewal with sufficient platelet increase = 12 months, unless otherwise specified

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POLICY NAME: NUCALA Affected Medications: NUCALA (mepolizumab)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical Asthma Information:  Diagnosis of severe asthma with an eosinophilic genotype defined as:  eosinophilic count greater than 300 cells/ul within 1 year  eosinophilic count greater than 150 cells/ul within 6 weeks prior to planned therapy start  Baseline FEV1  Documentation of all current asthma medications and all prior treatments tried and failed

EGPA  Confirmed diagnosis of relapsing or refractory Eosinophilic granulomatosis with polyangiitis (EGPA) with the following: o Chronic rhinosinusitis o Asthma o Blood eosinophilia (at least 1,500 cells/microL and/or 10% eosinophils on differential) at baseline o Diagnosis must be confirmed by a second clinical opinion Appropriate Severe Asthma Treatment  Asthma symptoms not controlled after at least 6 months of adherent use (80% of daily Regimen & Other doses) of high-dose inhaled corticosteroid plus long-acting beta agonist (LABA) Criteria:  If LABA contraindicated or not tolerated, sustained-release theophylline OR a leukotriene modifier must be tried AND  Uncontrolled asthma defined by at least two of the following in the past year: Two or more exacerbations requiring oral corticosteroids, at least one serious exacerbation requiring hospitalization, FEV1 less than 80% of predicted, Poor symptom control (ACT less than 20)  Documentation of optimal management of other asthma triggers Subsequent approvals: documentation of treatment success defined as reduction in prednisone use, or improved FEV1 from baseline

EGPA  Documented relapsing disease while on highest tolerated oral corticosteroid dose AND  Disease refractory to at least two oral immunosuppressive drugs for at least 12 weeks each (Azathioprine, Methotrexate, Leflunomide) Reauthorization: requires documented treatment success such as reduction in relapses from baseline or ability to significantly taper oral corticosteroid dose

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Exclusion  Not indicated for treatment of other eosinophilic conditions Criteria:  Not indicated for acute bronchospasm or status asthmaticus  Eosinophilic less than 150 cells/ul  Administration of Nucala through IV route Age Restriction:  12 years of age or older Prescriber ● Prescribed by or in consultation with an allergist, immunologist, or pulmonologist Restrictions: Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: NUEDEXTA Affected Medications: NUEDEXTA (dextromethorphan and quinidine)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required  Diagnosis of Pseudobulbar affect (PBA) in setting of comorbid diagnosis of one or more of Medical the following neurologic conditions: amyotrophic lateral sclerosis (ALS), extrapyramidal Information: and cerebellar disorders (Parkinson’s disease, multiple system atrophy, progressive supranuclear palsy), multiple sclerosis (MS), traumatic brain injury, Alzheimer’s disease and other dementias, or stroke.  Diagnosis of PBA using the Center for Neurologic Study-Lability Scale (CNS-LS) and a score greater than or equal to 13  Current complete medication list  QT interval at baseline in patients at risk for QTc prolongation  Baseline labs: potassium, magnesium, complete blood count, liver and renal function tests  Documentation of a 30 day trial of a SSRI and TCA  Documentation of failure of similar products (OTC dextromethorphan and compounded quinidine)

Appropriate  Other disease states have been ruled out (Depression, bipolarism, etc.) Treatment  Reauthorization requires documentation of treatment success with need for continuation Regimen & (spontaneous improvement of PBA occurs in some patients) Other Criteria:

Exclusion  Concomitantly taking other drugs containing quinidine, quinine, mefloquine, monoamine Criteria: oxidase inhibitors (MAOIs), or drugs that both prolong QT interval and are metabolized by CYP2D6.  Patient has a prolonged QT interval, congenital long QT syndrome or a history suggestive of torsade de pointes, or heart failure  Patient has complete atrioventricular (AV) block without implanted pacemaker or is at high risk of complete AV block.

Age Restriction:

Prescriber  Prescribed by or in consultation with a neurologist Restrictions:

Coverage  Approval: 12 months, unless otherwise specified Duration:

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POLICY NAME: OCALIVA Affected Medications: OCALIVA (obeticholic acid)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Liver function tests (including alkaline phosphatase and bilirubin) Information:  Child-Pugh score  Lipid profile Appropriate  The patient has an alkaline phosphatase level (ALP) at least 1.67 times the upper limit of Treatment normal and/or bilirubin above the upper limit of while on ursodiol (with demonstrated Regimen & Other adherence) after at least 12 months of therapy or has demonstrated a clinical inability to Criteria: tolerate ursodiol o ULN ALP (118 U/L for females or 124 U/L for males) o ULN of total bilirubin defined as 1.1 mg/dL for females or 1.5 mg/dL for males

 Reauthorization will require documentation of treatment success defined as a significant reduction in Alkaline phosphatase (ALP) and/or bilirubin levels Exclusion  Complete biliary obstruction Criteria: Age Restriction:  18 years and older Prescriber  Prescribed by hepatologist Restrictions: Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: OCREVUS Affected Medications: OCREVUS (ocrelizumab)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  Diagnosis of relapsing or primary progressive forms of Multiple Sclerosis (MS) confirmed Information: with MRI (Revised McDonald diagnostic criteria for multiple sclerosis)  Clinical evidence alone will suffice; additional evidence desirable but must be consistent with MS Appropriate  RRMS: Documented failure with or rationale for avoidance of a trial of Rituximab Treatment  PPMS: Documentation of at least one year of disease progression and Baseline Expanded Regimen & Other Disability Status Scale (EDSS) of 3-6.5 Criteria:  Initial dose: 300 mg infusion followed two weeks later by a second 300 mg infusion  Subsequent doses: Single 600 mg infusion every 6 months

 Reauthorization: o RRMS- Documentation of treatment success o PPMS- Documentation of treatment success as determined by treating provider (based on clinical and MRI findings) o Lack of disability progression (progression defined as when the EDSS score increased by 1 point or more from the baseline EDSS if the baseline EDSS was 5.5 points or less, or by 0.5 points or more if the baseline EDSS was more than 5.5 points) Exclusion  Use outside of the Food and Drug Administration (FDA)-approved indications of relapsing Criteria: or primary progressive forms of Multiple Sclerosis (MS)  Active HBV infection  Use with any other disease-modifying therapy for Multiple Sclerosis  Secondary progressive multiple sclerosis Age Restriction:  Safety and effectiveness of Ocrevus in pediatric patients have not been established  Clinical studies of Ocrevus did not include sufficient numbers of subjects aged 65 and over to determine whether they respond differently from younger subjects Prescriber  Prescribed by or after consultation with a neurologist or an MS specialist Restrictions:

Coverage  Initial approval: 6 months (2 initial infusions as noted above), unless otherwise specified Duration:  Reauthorization: 12 months (2 infusions as noted above), unless otherwise specified

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POLICY NAME: ODOMZO Affected Medications: ODOMZO (sonidegib)

Covered Uses:  NCCN indications with evidence level of 2A or higher. Required Medical  Documentation of performance status, all prior therapies used, and prescribed Information: treatment regimen.  Documentation that Erivedge is being used as a NCCN 2A level of evidence regimen. Appropriate Treatment  Documentation of surgery performed or reason why the patient is not a Regimen & Other Criteria: candidate for surgery.  Documentation of why the patient is not a candidate for radiation.  Reauthorization requires documentation of treatment success.

Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction:

Prescriber Restrictions:  Oncologist Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: OFEV Affected Medications: OFEV CAPSULE 100 MG ORAL, OFEV CAPSULE 150 MG ORAL

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design Required Medical  Documentation of nicotine use. Information:  If active nicotine user, documentation risks have been reviewed including decreased efficacy of therapy AND  Documentation of diagnosis of idiopathic pulmonary fibrosis  Presence of usual interstitial pneumonia (UIP) or high resolution computed tomography (HRCT), and/or surgical lung biopsy AND  Documentation of baseline forced vital capacity (FVC) greater than or equal to 50% of the predicted value AND  Documentation of Predicted diffuse capacity for carbon monoxide (DLCO) greater than or equal to 30%  Documentation of baseline liver function tests, at regular intervals during the first three months, then periodically thereafter or as clinically indicated Appropriate  Ofev is not approved for use in combination with Esbriet Treatment  Pregnancy should be avoided while on Ofev and for at least 3 months after the last Regimen & Other dose. Criteria:  Treatment of patients with moderate (Child Pugh B) and severe (Child Pugh C) hepatic impairment with OFEV is not recommended.  The safety, efficacy, and pharmacokinetics of nintedanib have not been studied in patients with severe renal impairment (less than 30 mL/min CrCl) and end-stage renal disease.  Reauthorization requires documentation of treatment success

Exclusion Criteria:  Concomitant administration of moderate or strong CYP3A4 and P-gp inhibitors / inducers should be avoided while taking Ofev  Transaminases more than 5 times the upper limit of normal or elevated transaminases accompanied by symptoms (jaundice, hyperbilirubinemia). Age Restriction:  18 years of age or older Prescriber  Must be prescribed by or in consultation with a pulmonologist Restrictions:

Coverage Duration:  Initial approval: 6 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME:

OLUMIANT Affected Medications: OLUMIANT (Baricitinib)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.

Required Medical  Documentation of moderate to severe disease despite current treatment (Indication Information: must be documented in chart notes within the last 6 months)  Documentation of complete and current treatment course  Documented current level of disease activity/disease control

Appropriate Rheumatoid arthritis (RA) Treatment  Has failed minimum 12 weeks of Humira AND minimum12 weeks of Enbrel individually Regimen & Other  Quantity Limit Criteria: o QL #1 tablet per day

Subsequent approval: documentation of treatment success determined by Disease Activity Score derivative for 28 joints (DAS-28), Simplified Disease Activity Index (SDAI), or Clinical Disease Activity Index (CDAI)

Exclusion Criteria:  Concurrent use with potent immunosuppressants (azathioprine, cyclosporine), or other biologic DMARDs.  Hepatitis B or Hepatitis C infection  ANC less than 1000 cells/mm3, lymphocytes less than 500 cells/mm3, Hgb less than 9 g/dL, CrCl less than 40 mL/min  History of diverticulitis or GI perforation, unresolved cytopenias, severe hepatic impairment, or severely uncontrolled hyperlipidemia  Evidence of active, latent, or inadequately treated tuberculosis or herpes zoster  Psoriatic arthritis with axial (spinal) involvement

Age Restriction:  Less than or equal to 18 years of age Prescriber  Prescribed by or in consultation with a rheumatologist. Restrictions: Coverage  Initial approval: 4 months, unless otherwise specified. Duration:  Reauthorization: 12 months, unless otherwise specified. 220

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POLICY NAME: ONCOLOGY AGENTS Affected Medications: ABRAXANE (paclitaxel), ADCETRIS, ALKERAN, ALIMTA (pemetrexed disodium), ALIQOPA (copanlisib), ALUNBRIG (brigatinib), ARZERRA (ofatumumab), BAVENCIO (avelumab), BALVERSA (erdafitinib), BENDEKA (bendamustine), BRAFTOVI (encorafenib), CABOMETYX (cabozantinib), COMETRIQ (cabozantinib), COPIKTRA (duvelisib), CYRAMZA (ramucirumab), DACOGEN (decitabine), DAURISMO (glasdegib), EMPLICITI, ERBITUX, ERLEADA (apalutamide), ERWINAZE, EVOMELA, GAZYVA, IMBRUVICA (ibrutinib), IMFINZI (durvalumab), IMLYGIC (talimogene laherparepvec), IRESSA (gefitinib), ISTODAX (romidepsin), IXEMPRA (ixabepilone), JEVTANA (cabazitaxel), Kadcyla (Ado-trastuzumab), KEYTRUDA (pembrolizumab), KISQALI (ribociclib), KISQALI & FEMARA CO-PACK, KYPROLIS (carfilzomib), LARTRUVO, LENVIMA (lenvatinib mesylate), LIBTAYO (cemiplimab-rwlc), LONSURF, LORBRENA, LUMOXITI, LUTATHERA, LYNPARZA, MEKINIST (trametinib), MEKTOVI (binmetinib), MYLOTARG, NEXAVAR (sorafenib tosylate), ONCASPAR, ONIVYDE (irinotecan), OPDIVO (nivolumab), PHOTOFRIN (porfimer), PORTRAZZA (necitumumab), POTELIGEO, RUBRACA, STIVARGA (regorafenib), SYNRIBO (omacetaxine), TAFINLAR (dabrafenib), TAGRISSO, TALZENNA (talazopairb), TEMOZOLOMIDE, TEPADINA (thiotepa), TIBSOVO, TORISEL (temsirolimus), TREANDA (bendamustine), VELCADE (bortezomib), VENCLEXTA (venetoclax), VITRAKVI (larotrectinib), VIDAZA (Azacitidine), VIZIMPRO (dacotiminib), VYXEOS (Daunorubicin and Cytarabine (Liposomal)), XOSPATA (gilteritinib), YERVOY (ipilimumab), YONDELIS (trabectedin), YONSA (abiraterone acetate), ZALTRAP (ziv- aflibercept)

Covered Uses:  NCCN indications with evidence level of 2A or higher. Required Medical  Documentation of performance status, all prior therapies used, disease staging, Information: and anticipated treatment course  Documentation of use with National Comprehensive Cancer Network (NCCN) 2A or higher level of evidence regimen  Patient weight

Appropriate Treatment Reauthorization: documentation of disease responsiveness to therapy Regimen & Other Criteria: Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater  Neutrophil counts less than 1,500 cells/mm3 Age Restriction: Prescriber Restrictions:  Oncologist Coverage Duration:  Initial approval: 4 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: ONFI Affected Medications: ONFI (clobazam)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design  Adjuctive therapy for seizure management

Required Medical a. Documentation of monotherapy failure for seizure control Information: AND b. Documentation of failure with at least 2 alternative adjunct therapy for seizure management (Carbamazepine, lamotrigine, levetiracetam, oxcarbazepine, topiramate, lamotrigine, divlproex, vimpat, zonisamide, phenytoin) i. Established members on Onfi as adjuvant therapy will not be required to fail alternative Appropriate  Documentation that therapy is being used as adjunct therapy for seizures Treatment Regimen & Other Reauthorization will require documentation of treatment success and a clinically significant Criteria: response to therapy

Exclusion Criteria: Age Restriction: Prescriber  Neurologist or affiliated with a neurology practice Restrictions: Coverage  Approval: 12 months, unless otherwise specified. Duration:

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POLICY NAME: ONPATTRO Affected Medications: ONPATTRO (patisiran)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design Required Medical  Documented pathogenic mutation in transthyretin (TTR; e.g. V30M mutation) Information:  Diagnosis of hereditary transthyretin (hATTR) amyloidosis with polyneuropathy  Documentation of baseline polyneuropathy disability (PND) score less than or equal to IIIb or baseline FAP stage I or II  Presence of clinical signs and symptoms of disease (e.g. peripheral/autonomic neuropathy, motor disability, cardiovascular dysfunction, renal dysfunction)  Documented failure with diflunisal

Reauthorization:  Documentation of either continued PND score less than or equal to IIIb OR patient continues to have FAP stage I or II AND Documentation of the patient experiencing positive clinical response to patisiran (e.g. improved neurologic impairment, motor function, cardiac function, quality of life assessment, serum TTR levels, etc)

Appropriate Hereditary transthyretin-mediated (hATTR) amyloidosis Treatment Dosing: Regimen & Other  For patients weighing less than 100 kg, the recommended dosage is 0.3 mg/kg once Criteria: every 3 weeks. For patients weighing 100 kg or more, the recommended dosage is 30 mg once every 3 weeks. Exclusion Criteria:  Previous liver transplantation  NYHA class III or IV Concomitant oligonucleotide (e.g. inotersen) or tafamidis meglumine Age Restriction:  Adults age 18 to 85 years old Prescriber  Physicians experienced in the management of amyloidosis Restrictions: Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: OPIOID QUANTITY ABOVE 90 MORPHINE MILLIGRAM EQUIVALENTS (MME) Affected Medications: OPIOIDS

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design  As of June 17, 2019 chronic use of opioids with a Morphine Milligram Equivalents (MME) per day greater than 90 MME is not funded by PacificSource

Required Medical  Exceptions require that opioid use greater than 90 MME is not chronic and is being Information: used for short term exceptional circumstances

Appropriate Treatment Regimen & Other Criteria:

Exclusion Criteria:  Pain related to current active cancer  Chronic pain related to sickle cell disease  Pain related to hospice care  Surgery or documented acute injury – 1 month approval

Age Restriction:

Prescriber Restrictions:

Coverage Duration:  Based on exceptional circumstance, not to exceed 3 months

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POLICY NAME: ORAL-INTRANASAL FENTANYL Affected Medications: FENTANYL CITRATE LOLLIPOP

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.

Required  Long-Acting opioid is being prescribed for around-the clock treatment of the cancer pain. Medical  The patient is opioid tolerant (Patients are considered opioid tolerant if they have been Information: taking at least 60 mg of oral morphine per day, 25 mcg of transdermal fentanyl/hr, 30 mg of oral oxycodone daily, 8 mg of oral hydromorphone daily, 25 mg oral oxymorphone daily or an equianalgesic dose of another opioid for a week or longer).

Appropriate Documentation for breakthrough pain in patients with cancer: Treatment  patient is unable to swallow, has dysphagia, esophagitis, mucositis, or uncontrollable Regimen & nausea/vomiting OR Other Criteria:  patient is unable to take 2 other short-acting narcotics (eg, oxycodone, morphine sulfate, hydromorphone, etc) secondary to allergy or severe adverse events AND  patient is on or will be on a long-acting narcotic (eg, Duragesic), or the patient is on intravenous, subcutaneous, or spinal (intrathecal, epidural) narcotics (eg, morphine sulfate, hydromorphone, fentanyl citrate).

Exclusion  Patients taking strong or moderate cytochrome P450 3A4 inhibitor(s), who will not be Criteria: carefully monitored and will not have dosing adjustments made if necessary.  Use in the management of acute and/or postoperative pain including surgery/post- surgery, trauma/post-trauma, acute medical illness (acute abdominal pain, pelvic pain, muscle spasm).  Use as pre-anesthesia (preoperative anxiolysis and sedation and/or supplement to anesthesia). Age Restriction:  18 years of age or older Prescriber Restrictions: Coverage  Approval: 12 months, unless otherwise specified. Duration:

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POLICY NAME: ORENCIA Affected Medications: ORENCIA (abatacept)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. o Adult Rheumatoid Arthritis (RA) o Juvenile Idiopathic Arthritis (JIA) o Adult Psoriatic Arthritis (PsA) Required  Documentation of moderate to severe disease despite current treatment (Indication must Medical be documented in chart notes within the last 6 months ) Information:  Documentation of complete and current treatment course  Documented current level of disease activity/disease control  Patient weight

Rheumatoid arthritis (RA)  Laboratory test confirming diagnosis of rheumatoid arthritis (anti-CCP, RF)  Documented current level of disease activity with one of the following: o The Disease Activity Score derivative for 28 joints (DAS-28) greater than 3.2 o The Simplified Disease Activity Index (SDAI) greater than 11 o The Clinical Disease Activity Index (CDAI) greater than 10 o Weighted RAPID3 of at least 2.3

o Dactylitis (inflammation of entire digit) o Psoriasis o Crohn’s disease/ulcerative colitis o Good response to NSAIDs o Family history of SpA o Elevated CRP OR  HLA-B27 genetic test positive AND at least 2 SpA features

Polyarticular Juvenile idiopathic arthritis  Documented current level of disease activity with physician global assessment (MD global score) or active joint count

Appropriate Rheumatoid arthritis (RA) Treatment  Documented clinical failure with at least 12 weeks of combination disease-modifying Regimen & antirheumatic drug (DMARD) therapy [Methotrexate plus sulfasalazine, methotrexate plus Other Criteria: hydroxychloroquine, sulfasalazine plus hydroxychloroquine, leflunomide plus sulfasalazine, or leflunomide plus hydroxychloroquine] AND  Has failed minimum 12 weeks on Humira AND minimum 12 weeks on Enbrel  For intravenous request: Treatment failure with subcutaneous route  QL- SQ – 4 ml per 28 day supply  QL - IV – Initial (one time only ) -3,000 mg per 28 day supply  QL - IV- Continuation – 1,000 mg per 28 day supply

Psoriatic arthritis (PsA)  Failure of at least 12 weeks with methotrexate, or if unable to tolerate or contraindicated to methotrexate, another disease modifying antirheumatic drug (sulfasalazine, cyclosporine, leflunomide) AND  Has failed minimum 12 weeks on Humira AND Enbrel  For intravenous request: Treatment failure with subcutaneous route  QL- SQ – 4 ml per 28 day supply  QL - IV – Initial (one time only ) -3,000 mg per 28 day supply  QL - IV- Continuation – 1,000 mg per 28 day supply

Juvenile idiopathic arthritis (JIA)  Has failed glucocorticoid joint injection or oral corticosteroids AND  Has failed at least one of the following agents with minimum 12 weeks trial: methotrexate, leflunomide AND  Has failed minimum 12 weeks on Humira AND minimum 12 weeks on Enbrel  For intravenous request: treatment failure with subcutaneous route  QL –SQ- 4 ml per 28 day supply  QL - IV – Initial (one time only ) -3,000 mg per 28 day supply  QL - IV- Continuation – 1,000 mg per 28 day supply

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Reauthorization: documentation of treatment success and a clinically significant response to therapy

Psoriatic Arthritis (PsA) with Axial Involvement  Documented failure with a minimum 12 weeks on Humira AND minimum 12 weeks on Enbrel  For intravenous request: Treatment failure with subcutaneous route  QL- SQ – 4 ml per 28 day supply  QL - IV – Initial (one time only ) -3,000 mg per 28 day supply  QL - IV- Continuation – 1,000 mg per 28f day supply Reauthorization: requires improvement of at least 50% in the BASDAI score or an absolute change of 2 units Exclusion  Concurrent use of biologic DMARDs: Rituxan (rituximab), Actemra (tocilizumab), Enbrel Criteria: (etanercept), Remicade (infliximab), Humira (adalimumab), Cimzia (certolizumab), Kineret (anakinra), Xeljanz (tofacitinib), and etc.  Management of plaque psoriasis without psoriatic arthritis  Positive Tuberculosis, Hepatitis B Age Restriction:  Rheumatoid Arthritis: 18 years of age or older Prescriber  Prescribed by or in consultation with a rheumatologist Restrictions:

Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: ORENITRAM Affected Medications: ORENITRAM (treprostinil)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Pulmonary arterial hypertension (PAH) WHO Group 1 Medical  Documentation of PAH confirmed by right-heart catheterization Information:  Etiology of PAH: (idiopathic, heritable, or associated with connective tissue disease)  NYHA/WHO Functional Class II to III symptoms  Documentation of acute vasoreactivity testing (positive result requires trial/failure to calcium channel blocker)

Appropriate  Documentation of failure with Remodulin and Tyvaso Treatment  For initiation of therapy patient must have mean pulmonary artery pressure at least 25 Regimen & mmHg at rest or at least 30 mmHg with exertion Other Criteria: AND  The pulmonary hypertension has progressed despite maximal medical and/or surgical treatment of the identified condition  Treatment with oral calcium channel blocking agents has been tried and failed, or has been considered and ruled out  Documentation that treprostinil is used as a single route of administration (Remodulin, Tyvaso, Orenatriam should not be used in combination)  Not recommended for PAH secondary to pulmonary venous hypertension (e.g., left sided atrial or ventricular disease, left sided valvular heart disease, etc) or disorders of the respiratory system (e.g., chronic obstructive pulmonary disease, interstitial lung disease, obstructive sleep apnea or other sleep disordered breathing, alveolar hypoventilation disorders, etc.)  Subsequent approvals require documentation of treatment success such as improved walking distance or improvements in functional class

Exclusion  Severe hepatic impariment (Child Pugh Class C) Criteria:

Age Restriction:

Prescriber  Cardiologist or Pulmonologist Restrictions:

Coverage  12 months, unless otherwise specified Duration:

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POLICY NAME: ORILISSA Affected Medications: ORILISSA (Elagolix oral tablets)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design Required Medical  Documentation of premenopausal status Information:  Surgical diagnosis of endometriosis in the previous 5 years  Moderate or severe endometriosis-associated pain  Pregnancy has been excluded

Appropriate ● Documented trial and inadequate relief after at least three months of first-line Treatment therapy with nonsteroidal anti-inflammatory drugs (NSAIDs) and hormonal Regimen & Other contraceptives Criteria: ● Dosing: ○ 150 mg once daily- maximum treatment duration of 24 months ○ 200 mg twice daily- maximum treatment duration of 6 months ○ Moderate hepatic impairment (Child-Pugh Class B)- 150 mg once daily for up to 6 months Reauthorization requires documentation of clinical treatment success defined as a significant reduction in pain (150 mg dose only)

Exclusion Criteria:  Chronic pain unrelated to endometriosis  Other clinically significant gynecologic conditions  Z score of less than -1.5 for bone mineral density at the lumbar spine, femoral neck, or total hip at screening  Severe hepatic impairment

Age Restriction:  18 years of age and older Prescriber ● Prescribed by or in consultation with a specialist in obstetrics/gynecology or Restrictions: reproductive endocrinology

Coverage Duration:  Initial Authorization: 6 months, unless otherwise specified  Reauthorization (for 150 mg dose only): 18 months, unless otherwise specified

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POLICY NAME: ORKAMBI Affected Medications: ORKAMBI (lamacaftor/ivacaftor)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design Required Medical  Documentation of cystic fibrosis (CF) diagnosis. Information:  Documentation of Homozygous for the F508 del mutation by FDA-cleared CF mutation test on both alleles of the CFTR gene  Baseline forced expiratory volume in 1 second (FEV1)  Documentation of baseline liver function tests; eye exam (for pediatric patients) Appropriate  2 through 5 years and weighing less than 14 kg: Take one lumacaftor 100 mg/ivacaftor Treatment 125 mg packet of granules every 12 hours Regimen & Other  2 through 5 years and weighing 14 kg or greater: Take one lumacaftor 150 Criteria: mg/ivacaftor 188 mg packet of granules every 12 hours  6 through 11 years Take two lumacaftor 100 mg/ivacaftor 125 mg tablets every 12 hours  12 years and older Take two lumacaftor 200 mg/ivacaftor 125 mg tablets every 12 hours

Reauthorization: Documentation of improvement in FEV1 from baseline, documentation of follow up liver function tests; blood pressure monitoring AND follow up, eye exam for pediatric patients. Exclusion Criteria:  Concurrent use of strong CYP3A inducers: rifampin, rifabutin, phenobarbital, carbamazepine, phenytoin, and St. John’s wort Age Restriction:  2 years and older Prescriber  Prescribed by or in consultation with a pulmonologist or provider who specializes in CF Restrictions:

Coverage Duration:  Initial approval: 6 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: OTEZLA Affected Medications: OTEZLA (apremilast)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design Required Medical  Documentation of moderate to severe disease despite current treatment (Indication Information: must be documented in chart notes within the last 6 months )  Documentation of complete and current treatment course  Documented current level of disease activity/disease control

Plaque Psoriasis  Documentation of disease that is severe in nature, which has resulted in functional impairment (e.g., inability to use hands or feet for activities of daily living, or significant facial involvement preventing normal social interaction) AND one or more of the following: o At least 10% body surface area involvement; or o Hand, foot or mucous membrane involvement

Appropriate Psoriatic Arthritis (PsA) Treatment  Failure of at least 12 weeks with methotrexate, or if unable to tolerate or contraindicated Regimen & Other to methotrexate, another disease modifying antirheumatic drug sulfasalazine, Criteria: cyclosporine, leflunomide) AND  Documented clinical failure with minimum 12 weeks on Humira AND minimum 12 weeks on Enbrel

Plaque Psoriasis  Has failed at least two systemic therapy with minimum of 12 weeks trial: methotrexate, cyclosporine, acitretin, phototherapy (UVB, PUVA) AND  Has failed minimum 12 weeks on Humira AND minimum 12 weeks on Enbrel

Reauthorization: documentation of treatment success and a clinically significant response to therapy

233

Use of Otezla in combination with other biologics for the treatment of Psoriasis is considered experimental and is not covered Exclusion  Uncontrolled depression Criteria:  Use of Otezla in combination with other biologics for the treatment of Psoriasis is considered experimental and is not covered Age Restriction:  Greater than or equal to 18 years Prescriber  Prescribed by or in consultation with a rheumatologist or dermatologist Restrictions:

Coverage  Initial approval: 4 months initiation, unless otherwise specified. Duration:  Reauthorization: 12 months, unless otherwise specified.

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POLICY NAME: OXERVATE Affected Medications: OXERVATE (cenergermin-bkbj)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design Required  Documentation of decreased corneal sensitivity (≤ 4 cm using the Cochet-Bonnet Medical aesthesiometer) within the area of the persistent epithelial defect or corneal ulcer and Information: outside of the area of the defect in at least one corneal quadrant  Documentation of stage 2 or stage 3 neurotrophic keratitis . Stage 2 neurotrophic keratitis o Persistent corneal epithelial defect OR o Descemet’s membrane folds and stromal swelling OR o Anterior chamber inflammatory reaction . Stage 3 neurotrophic keratitis o Corneal ulcer OR o Corneal perforation OR o Corneal stromal melting Appropriate  Documentation of progression in severity with treatment of preservative-free artificial Treatment tears, gel, or ointments AND therapeutic corneal or scleral contact lenses AND amniotic Regimen & membrane transplantation and conjunctival flap surgery OR tarsorrhaphy OR Other Criteria: cyanoacrylate glue OR soft-bandage contact lens  Dose may not exceed more than 1 vial per eye per day  Dosing does not exceed 8 weeks for first treatment  Reauthorization will require documentation of improvement in corneal sensitivity and grade of severity determined by corneal fluorescein staining using the modified Oxford scale Exclusion  Active or suspected ocular or periocular infections Criteria:

Age Restriction:

Prescriber  Ophthalmologist Restrictions:

Coverage  Authorization: 8 weeks Duration:  Reauthorization: 8 weeks, maximum approval (total of 16 weeks)

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POLICY NAME: PALYNZIQ Affected Medications: PALYNZIQ (pegvaliase-PQPZ)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan.

Required Medical  Documentation of anticipated treatment course, including target phenylalanine Information: (Phe) level set by specialist  Documentation of failure to Phe restricted diet as monotherapy  Documentation of failure to Kuvan AND phenylalanine-restricted diet as dual- therapy  Current patient weight  Baseline (pre-treatment) blood Phe levels

Baseline Phe concentration must be consistent with the following:  Phe level must be greater than 10mg/dL (600 microM) .

Reauthorization after initial approval requires documentation of updated Phe labs decreased by 20% or greater from baseline  Treatment with Palynziq should be discontinued in patients whose blood Phe has not decreased by at least 20% from baseline or a blood phenylalanine concentration less than or equal to 600 microM/L after 16 weeks with max dose of 40 mg/day

Reauthorization for continued long-term approval (12 months) requires updated Phe labs meeting one of the following criteria:  Phe level less than 20 percent of baseline OR  Phe level lower than baseline and meets specialist’s target level

Appropriate If patient has failed dual-therapy with Kuvan and Phe restricted diet, and Palynziq is Treatment warranted, treatment must be consistent with the following: Regimen & Other  Initial dose must be 2.5mg once weekly x 4 weeks Criteria:  Titration (after 4-week induction): 2.5 mg twice weekly for 1 week, then 10 mg once weekly for 1 week, then 10 mg twice weekly for 1 week, then 10 mg 4 times/week for 1 week, then 10 mg once daily for 2 week.  Maintenance (after induction and titration): 20 mg once daily for at least 24 weeks. May increase to 40 mg once daily if a response (20% reduction from baseline in blood phenylalanine or blood phenylalanine concentration 600 micromol/L or less) has not been achieved after administering 20 mg once daily for 24 weeks.

Exclusion Criteria:  Prior intolerance or allergic reaction to requested medication  Doses greater than 40mg/day

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Age Restriction:  Adults 18 years and older

Prescriber  Specialist in metabolic disorders or endocrinologist Restrictions:  All approvals are subject to utilization of the most cost effect site of care

Coverage Duration:  Initial approval: 2 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: PARATHYROID HORMONE ANALOGS Affected Medications: FORTEO (teriparatide), TYMLOS (abaloparatide)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical  Documentation of DEXA score within 2 years showing a T-Score less than or equal to - Information: 2.5, OR FRAX score indicating major fracture risk 20% or greater (or hip fracture 3% or greater), OR non-traumatic hip or vertebral fracture.  Liver function test (LFT), including alkaline phosphatase (ALP). Appropriate  Documentation of adequate calcium intake and vitamin D level and/or treatment Treatment  Therapy will be discontinued after a lifetime total of 24 months of treatment Regimen & Other  Documentation of clinically significant worsening osteoporosis or five years of Criteria: continuous treatment on therapeutic doses of bisphosphonates (eg, alendronate, risedronate, ibandronate, zoledronic acid) or contraindication to intravenous bisphosphonate therapy AND  Documentation of clinically significant worsening osteoporosis on Prolia  For Forteo request: documented treatment failure with Tymlos

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Paget’s Disease  Unexplained elevations of alkaline phosphatase  Open epiphyses (ie, pediatric or young adult patient)  Bone metastases or skeletal malignancies  Hereditary disorders predisposing to osteosarcoma  Prior external beam or implant radiation therapy involving the skeleton  Concurrent therapy with bisphosphonates, Prolia, Xgeva, or Forteo Age Restriction:  Pediatric patients or young adults with open epiphyses Prescriber Restrictions: Coverage  Approval= 12 months, unless otherwise specified Duration:

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POLICY NAME: PARSABIV Affected Medications: PARSABIV (etelcalcetide)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design

Required Medical  Diagnosis of secondary hyperparathyroidism on hemodialysis Information:  Documentation of baseline laboratory values: Calcium (corrected or free), Phosphate, Vitamin D  Parathyroid hormone (PTH) levels persistently greater than 2-9 times the Upper Limit of Normal (ULN) for the assay used  Documentation of failure or rationale for avoidance for all standard treatments for hyperparathyroidism: Calcitriol oral (capsule or solution) and injection, Paricalcitol oral and injection, Doxercalciferol oral and injection, Cincalcet

Appropriate  Patient does not have any FDA labeled contraindications to therapy Treatment  Reauthorization will require documentation of reduction of PTH to 2 times the ULN Regimen & Other Criteria:

Exclusion Criteria:  Known hypersensitivity to etelcalcetide or any of its excipients  Diagnosis of parathyroid carcinoma, primary hyperparathyroidism or with chronic kidney disease who are not on hemodialysis Age Restriction: Prescriber Restrictions: Coverage  12 months, unless otherwise specified Duration:

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POLICY NAME: PEGASYS Affected Medications: PEGASYS® (peginterferon alfa-2a)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical Chronic Hepatitis C (CHC): Information:  Documentation chronic hepatitis C virus (HCV) genotype by liver biopsy or by FDA- approved serum test  Baseline HCV RNA level  Documentation of anti-hepatitis C virus regimen to be used with AND anticipated dose and duration of therapy

Chronic Hepatitis B (CHB):  Documentation of HBeAg-positive or HBeAg-negative chronic hepatitis B virus (HBV) infection  Baseline HBV DNA level  Documentation of anti-hepatitis B virus regimen to be used with AND anticipated dose and duration of therapy

Chronic Hepatitis C and B:  Baseline HIV-1 RNA level  Current documentation of hepatic impairment severity with Child-Pugh Classification OR bilirubin, albumin, INR, ascites status, and encephalopathy status to calculate Child-Pugh score within 12 weeks prior to anticipated start of therapy  Current estimated creatinine clearance OR serum creatinine, height, and weight to calculate by Cockcroft-Gault within 12 weeks prior to anticipated start of therapy  Current complete blood count AND liver function tests within 12 weeks prior to anticipated start of therapy  Documentation if HIV/HCV/HBV coinfection  Documentation of abstinence from alcohol and any illegal drug use for at least 6 months Appropriate Chronic Hepatitis C: Treatment  Approve if used in combination with FDA- and/or AASLD/IDSA- recommended regimen Regimen & Other and if not otherwise excluded from PacificSource policies of other medications in the Criteria: regimen  Preferred regimen should include concomitant ribavirin

Chronic Hepatitis B (one of the following 4 scenarios must be met):  HBeAg-positive AND baseline serum HBV DNA greater than 20,000 copies/mL AND baseline serum aminotransferase (ALT) two times greater than the upper limit of normal range  HBeAg-positive AND baseline serum HBV DNA greater than 20,000 copies/mL AND baseline serum aminotransferase (ALT) one to two times greater than the upper limit of normal range AND moderate-severe inflammation/fibrosis

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 HBeAg-negative AND baseline serum HBV DNA greater than 2,000 copies/mL AND baseline serum aminotransferase (ALT) two times greater than the upper limit of normal range  HBeAg-negative AND baseline serum HBV DNA greater than 2,000 copies/mL AND baseline serum aminotransferase (ALT) one to two times greater than the upper limit of normal range AND moderate-severe inflammation/fibrosis

Chronic Hepatitis C and B:  Creatinine clearance less than 50 ml/min, adjust dose: 135 mcg subcutaneously once weekly  Baseline platelet count greater than or equal to 90,000 cells/mm3  Baseline absolute neutrophil count 1,500 cells/mm3 or more

Exclusion Criteria:  Treatment of patients with CHC who have had solid organ transplantation  Autoimmune hepatitis  Hepatic decompensation (Child-Pugh score greater than 6) Age Restriction:  CHC: 5 years of age or older  CHB: 18 years of age or older Prescriber  Prescribed by or in consultation with a gastroenterologist, hepatologist, or infectious Restrictions: disease specialist

Coverage  CHC: 12 weeks, unless otherwise specified (depends on regimen and diagnosis ) Duration:  CHB: 12 months, unless otherwise specified

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POLICY NAME: PEGINTRON Affected Medications: PEGINTRON REDIPEN®(peginterferon alfa-2b), PEGINTRON® (peginterferon alfa-2b)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  Documentation chronic hepatitis C virus (HCV) genotype by liver biopsy or by FDA- Information: approved serum test  Baseline HCV RNA level  Documentation of anti-hepatitis C virus regimen to be used with AND anticipated dose and duration of therapy  Patient weight  Current documentation of hepatic impairment severity with Child-Pugh Classification OR bilirubin, albumin, INR, ascites status, and encephalopathy status to calculate Child-Pugh score within 12 weeks prior to anticipated start of therapy  Current estimated creatinine clearance OR serum creatinine, height, and weight to calculate by Cockcroft-Gault within 12 weeks prior to anticipated start of therapy  Current complete blood count AND liver function tests within 12 weeks prior to anticipated start of therapy  Documentation if HIV/HCV/HBV coinfection  Documentation of abstinence from alcohol and any illegal drug use for at least 6 months Appropriate  Approve if used in combination with FDA- and/or AASLD/IDSA- recommended regimen Treatment and if not otherwise excluded from PacificSource policies of other medications in the Regimen & Other regimen Criteria:  Preferred regimen should include concomitant ribavirin  In patients with moderate renal dysfunction (creatinine clearance 30-50 mL/min), the PegIntron dose should be reduced by 25%  Patients with severe renal dysfunction (creatinine clearance 10-29 mL/min), including those on hemodialysis, should have the PegIntron dose reduced by 50% Exclusion Criteria:  Autoimmune hepatitis  Hepatic decompensation (Child-Pugh score greater than 6) Age Restriction:  3 years of age or older Prescriber  Prescribed by or in consultation with a gastroenterologist, hepatologist, or infectious Restrictions: disease specialist

Coverage  12 weeks, unless otherwise specified (depends on regimen and diagnosis) Duration:

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POLICY NAME: PERJETA Affected Medications: PERJETA (pertuzumab)

Covered  NCCN indications with evidence level of 2A or higher Uses: Required  Documentation of performance status, disease staging, all prior therapies used, and Medical prescribed dosing regimen Information:  Documentation of HER2 status  Baseline evaluation of left ventricular function Appropriate  Neoadjuvant Treatment of Breast Cancer - minimum T2 or N1 Treatment  Use with Herceptin and chemotherapy Regimen &  Adjuvant Treatment of Breast Cancer – minimum N1 Other  Use with Herceptin, max duration of treatment is 12 months Criteria:  Recurrent Breast Cancer  First line or rarely second line  Reauthorization: documentation of disease responsiveness to therapy

 Regular assessment of LVEF for all indications

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion  Stage IV Breast Cancer: Previous failure/progression while on Perjeta (pertuzumab) Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater  Consider withholding therapy for at least 3 weeks for either a drop in LVEF to less than 40% OR LVEF 40-45% with a 10% reduction in LVEF from pre-treatment values Age Restriction: Prescriber Restrictions: Coverage  Initial: 4 months unless otherwise specified Duration:  Reauthorization: 12 months unless otherwise specified

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POLICY NAME: PERSERIS Affected Medications: PERSERIS (risperidone)

Covered  All FDA-approved indications not otherwise excluded by plan design. Uses: Required  Diagnosis of acute and maintenance treatment of schizophrenia AND Medical  The patient has a history of non-compliance and/or refuses to utilize oral medication AND Information:  The patient has received at least two test doses of oral risperidone  Documentation of failure or clinical rationale for avoidance of Risperdal Consta Appropriate  Reauthorization will require documentation of treatment success and a clinically significant Treatment response to therapy Regimen & Other Criteria:

Exclusion  Diagnosis of dementia-related psychosis. Criteria:  Prior use of risperidone demonstrated a hypersensitivity reaction. Age Restriction: Prescriber  Psychiatrist or receiving input from a psychiatry practice Restrictions: Coverage  Approval: 12 months, unless otherwise specified Duration:

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POLICY NAME: PHENOXYBENZAMINE Affected Medications: PHENOXYBENZAMINE

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required  Documentation of use as preoperative medical therapy for diagnosis of Medical pheochromocytoma and anticipated duration of need Information:  If use is projected to be greater than 14 days, documentation of contraindication to selective alpha-1-adrenergic blocking agents (examples: prazosin, terazosin, or doxazosin) is needed as well as documentation of recent myocardial infarction, catecholamine cardiomyopathy, refractory hypertension, and catecholamine-induced vasculitis  For diagnosis of metastatic pheochromocytoma where long-term pharmacologic treatment is indicated, documentation of contraindication or failure to the following selective alpha-1-adrenergic blocking agents: prazosin, terazosin, or doxazosin

Appropriate  An alpha-adrenergic blocker is given 10 to 14 days preoperatively to normalize blood Treatment pressure and expand the contracted blood volume. A longer duration of preoperative Regimen & alpha-adrenergic blockade is indicated in patients with recent myocardial infarction, Other Criteria: catecholamine cardiomyopathy, refractory hypertension, and catecholamine-induced vasculitis  Initial: 10 mg twice daily, increase by 10 mg every other day until optimal blood pressure response is achieved; usual range: 20-40 mg 2-3 times/day. Doses up to 240 mg/day have been reported Exclusion Criteria:

Age Restriction:  18 years of age or older Prescriber Restrictions: Coverage  1 month , unless otherwise specified Duration:

245

POLICY NAME: PLEGRIDY Affected Medications: PLEGRIDY (peglyated interferon beta-1a)

Appropriate Treatment  Must fail at least one preferred product (Avonex, glatiramer 20mg, glatiramer Regimen & Other 40mg, glatopa 20mg, Extavia, Gilenya, Tecfidera) Criteria:  No concurrent use of medications indicated for the treatment of relapsing- remitting multiple sclerosis  Not approved for primary and secondary progressive multiple sclerosis  multiple sclerosis

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria: Age Restriction: Prescriber  Prescribed by or after a consultation with a Neurologist or a MS specialist. Restrictions: Coverage Duration:  Approval: 12 months, unless otherwise specified

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POLICY NAME: POMALYST Affected Medications: Pomalyst (Pomalidomide)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.  NCCN indications with evidence level 2A or higher. Required Medical For Multiple Myeloma Information:  The Patient has received at least TWO prior therapies for multiple myeloma including lenalidomide (Revlimid) and bortezomib (Velcade) AND  Patient has demonstrated disease progression on or within 60 days of completion of last therapy for multiple myeloma. Appropriate For Multiple Myeloma Treatment  Concurrent therapy with dexamethasone unless patient is steroid intolerant Regimen & Other For Systemic Light Chain Amyloidosis Criteria:  Pomalyst should not be used without dexamethasone for this indication For All Indications  Monitor for signs and symptoms of thromboembolism  Female patients of child-bearing potential and male partners are instructed on the importance of proper utilization of appropriate contraceptive methods.  Documented consideration of risk/benefit ratio for anticoagulation therapy  Baseline CBC with documentation of planned weekly CBC monitoring for the first 8 weeks of treatment and monthly thereafter

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Pregnancy  CBC outside of normal limits without documentation of benefit outweighing risk Age Restriction:  18 years of age or older Prescriber  Prescribed by or in consultation with an oncologist. Restrictions:  Prescriber must be certified with the Pomalyst REMS program Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: PROBUPHINE Affected Medications: PROBUPHINE (buprenorphine implant)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.

Required Medical  Documentation of no illicit drug use or desire, no crisis interventions in the past 180 days, Information: and consistent monthly clinic visit  Documentation of buprenorphine dose equal or less than 8 mg per day Appropriate Opioid Dependence: Treatment  Patient must be enrolled in a program of supportive care Regimen & Other  No dependence on alcohol or benzodiazepines Criteria:  Free from significant untreated psychiatric comorbidities  QL: 4 implants/ 6 months

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Daily buprenorphine dose greater than 8mg per day Age Restriction:  Age 16 years or older Prescriber  Certified prescriber per DATA 2000 requirements Restrictions:

Coverage  Initial approval: 6 months, unless otherwise specified (4 implants) Duration:  Reauthorization: 12 months, unless otherwise specified (8 implants)

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POLICY NAME: PROLIA Affected Medications: PROLIA (denosumab)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  For Treatment of Osteoporosis: Documentation of T Score equal to or less than - 2.5 or Information: FRAX Score indicating Major fracture risk greater than 20% or HIP Fracture greater than 3%, or non-traumatic fracture.  For Treatment of glucocorticoid-induced osteoporosis in men and women:  50 years old or greater: Documentation of baseline BMD T-score of less than or equal to -2.0 at the lumbar spine, total hip, or femoral neck; or a BMD T-score ≤ -1.0 at the lumbar spine, total hip, or femoral neck and a history of osteoporotic fracture.  Less than 50 years old: Documentation of history of osteoporotic fracture.  For Treatment to Increase Bone Mass in Women at High Risk for Fracture Receiving Adjuvant Aromatase Inhibitor Therapy for Breast Cancer: Evidence of low bone mass (T- score of -1.0 to -2.5).

Appropriate  Prolia may be approved for treatment of osteoporosis: if the patient has failed an Treatment intravenous bisphosphonate (eg, zoledronic acid [Reclast] or ibandronate[Boniva]) OR Regimen & Other  If the patient has severe renal impairment (eg, creatinine clearance less than 35 mL/min) Criteria: OR  If the patient has multiple osteoporotic fractures in the setting of T-scores less than -3.5.  For Treatment to Increase Bone Mass in Men at High Risk for Fracture Receiving Androgen Deprivation Therapy Prolia may be approved for males: If younger than 70 years: T-score less than -1.0 at any location, or a history of osteoporotic fracture.  For Treatment of glucocorticoid-induced osteoporosis in men and women at high risk for fracture, Prolia may be approved if initiating or continuing systemic glucocorticoids equivalent to 7.5 mg or greater of prednisone and expected to remain on glucocorticoids for at least 6 months  Dosage is 60 mg once every 6 months  Documentation of taking a minimum 1000mg calcium and 400 IU vitamin D daily or contraindication For Reauthorization: documented clinical response to treatment

Exclusion Criteria:  Concurrent use of bisphosphonate therapy or antineoplastic therapy apart from aromatase inhibitors or androgen deprivation therapy.  Preexisting hypocalcemia pregnancy  Pregnancy

Age Restriction:  For Treatment to Increase Bone Mass in Men at High Risk for Fracture Receiving Androgen Deprivation Therapy: Age greater than 70 years if normal bone mineral density or no history of fracture.  Greater than 18 years for all other indications.

249

Prescriber Restrictions:

Coverage  Approval: 24 months, unless otherwise specified Duration:  Reapproval: 24 months, unless otherwise specified

250

POLICY NAME: PROMACTA Affected Medications: PROMACTA (eltrombopag)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical All indications Information:  Complete blood count with differential and platelet count  Liver function test Thrombocytopenia in patients with ITP  All therapies tired/failed  Documentation of splenectomy status Thrombocytopenia in patients with chronic hepatitis C  Documentation of plan to initiate interferon-based therapy  Child-Pugh score Severe aplastic anemia  All immunosuppressive therapies tried/failed  Documentation of planned treatment regimen  Baseline hemoglobin and absolute neutrophil count (ANC) Appropriate Thrombocytopenia in patient with ITP Treatment  Documentation of platelet count less than 20 x 109/L AND Regimen & Other  Documentation of clinically significant bleeding AND Criteria:  Must fail at least 2 therapies for ITP, including corticosteroids or immunoglobulin (defined as platelets did not increase to at least 50 x 109/L) OR  Documentation of splenectomy

Thrombocytopenia in patients with chronic hepatitis C  Documentation of platelet count less than 75,000/mcl AND  Documentation of compensated liver disease  Continuation of therapy requires response to treatment with platelet count of at least  90,000/mcl but less than 400,000/mcl and no significant liver function abnormalities

Severe aplastic anemia  Documentation of platelet count ≤ 30,000/mcl AND  Documentation of insufficient response to at least 1 prior immunosuppressive therapy  Continuation of therapy after initial approval requires hematologic response to treatment defined as meeting 1 or more of the following criteria:

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1) Platelet count increases to 20,000/mcl above baseline, or stable platelet counts with transfusion independence for a minimum of 8 weeks; 2) Hemoglobin increase by greater than 1.5 g/dL, or a reduction in greater than or equal to 4 units RBC transfusions for 8 consecutive weeks; 3) ANC increase of 100% or an ANC increase greater than 0.5 x 109/L  Discontinue therapy if hematologic response not achieved after 16 weeks of treatment, if platelet count greater than 400,000/mcl, or significant liver function abnormalities  Oral suspension formulation requires documented medical inability to use Promacta tablets Reauthorization for the indication of thrombocytopenia in patients with chronic hepatitis C or severe aplastic anemia will require documentation of treatment success and a clinically significant response to therapy Exclusion Criteria: All indications  History of hematological malignancy or myelodysplastic syndrome Thrombocytopenia in patients with chronic hepatitis C  Hepatitis C treatment with direct-acting antiviral agents used without interferon  Child-Pugh score greater than 6  History of ascites or hepatic encephalopathy Age Restriction: Thrombocytopenia in patients with ITP  1 year and older Thrombocytopenia in patients with chronic hepatitis C and patients with severe aplastic anemia  18 years and older Prescriber Thrombocytopenia in patients with ITP and patients with severe aplastic anemia Restrictions:  Prescribed by or consultation with hematologist Thrombocytopenia in patients with chronic hepatitis C  Prescribed by or consultation with hematologist, hepatologist, gastroenterologist, or ID specialist Severe Aplastic Anemia  2 years and older Coverage Thrombocytopenia in patients with ITP Duration:  Initial approval: 3 months, unless otherwise specified  Renewal with sufficient platelet increase = 12 months, unless otherwise specified  Renewal with insufficient platelet increase= 3 months, unless otherwise specified Thrombocytopenia in patients with chronic hepatitis C  Initial: 2 months, unless otherwise specified  Subsequent: 12 months, unless otherwise specified Severe aplastic anemia  Initial: 4 months, unless otherwise specified  Subsequent: 12 months, unless otherwise specified Severe aplastic anemia in combination with cyclosporine and Atgam  Approval: 6 months only POLICY NAME:

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PROVENGE Affected Medication: PROVENGE (sipuleucel-T)

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required  Documentation of performance status 0 or 1, disease staging, all prior therapies documentation: used, and prescribed treatment regimen  Documentation of castrate recurrent recurrent (rising PSA on two separate tests) metastatic (M1) prostate cancer with NO liver metastases  Documented asymptomatic or minimally symptomatic disease with life expectancy greater than 6 months  Testosterone levels  Less than 50 ug  Below lowest level of normal Appropriate  Maximum 3 infusions Treatment Regimen: Exclusion Criteria:  Prior intolerance or allergic reaction to requested medication  Concomitant use of chemotherapy or immunosuppressive therapy  Karnofsky Performance Status less than or equal to 50% or ECOG performance score greater than or equal to 2 Age Restriction: Provider Restriction:  Oncologist or Urologist

Approval Duration:  Approval: 3 infusions or 2 months, unless otherwise specified

253

POLICY NAME: PULMOZYME Affected Medications: PULMOZYME (dornase alfa)

Covered Uses:  All FDA approved indications not otherwise excluded by plan design.

Required Medical  The diagnosis of Cystic Fibrosis (CF) has been confirmed by appropriate diagnostic Information: or genetic testing o Additional testing should include evaluation of overall clinical lung status and respiratory function (eg pulmonary function tests, lung imaging, etc.)

Appropriate Treatment  Pulmozyme will be used in conjunction with standard therapies for cystic fibrosis Regimen & Other Criteria: Reauthorization will require documentation of a clinically significant response to therapy

Exclusion Criteria:  Known hypersensitivity to dornase alfa, Chinese Hamster Ovary cell products, or any component of the product. Age Restriction:  1 month or older Prescriber Restrictions: Coverage Duration:  Approval: 24 months, unless otherwise specified.

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POLICY NAME: RADICAVA Affected Medication: RADICAVA (edaravone)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical  Definite or probable Amyotrophic lateral sclerosis (ALS) based on El Escorial revised Information: criteria  Disease duration of 2 years or less  Normal respiratory function (defined as %FVC ≥ 80%)  Patient currently retains most activities of daily living defined as at least 2 points on all 12 items of the ALS functional rating scale-revised (ALSFRS-R)

Appropriate Treatment  Initial treatment cycle: 60mg intravenous infusion daily for 14 days followed by a Regimen & Other 14 day drug free period Criteria:  Maintenance: 60 mg intravenous infusion daily for 10 days followed by 18 day drug free-period.  Reauthorization: Treatment success as determined by prescriber including retaining most activities of daily living

Exclusion Criteria:

Age Restriction:  Age 20 years and older

Prescriber  By or in consultation with a neurologist or provider with experience in treating ALS Restrictions:

Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: RAYALDEE Affected Medications: RAYALDEE (calcifediol)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Documentation of Secondary Hyperparathyroidism with chronic kidney disease Information:  Documentation of total 25-hydroxyvitamin D levels less than 30 ng/mL  Documentation of failure or clinically significant adverse effects to ONE of the alternatives: . calcitriol . ergocalciferol  Documentation of baseline serum calcium, serum phosphorus, intact PTH levels  Documentation of stage 3 or 4 chronic kidney disease not on dialysis

Appropriate Treatment  Dosing: Adult Secondary hyperparathyroidism: Regimen & Other  Initial: 30 mcg once daily at bedtime; Ensure corrected serum total calcium is Criteria: below 9.8 mg/dL prior to initiating therapy.  May adjust dose to 60 mcg once daily at bedtime after 3 months if intact PTH remains above desired therapeutic range.  Maintenance dose should target total 25-hydroxyvitamin D levels between 30 and 100 ng/mL, intact PTH levels within desired therapeutic range, serum calcium <9.8 mg/dL, and serum phosphorus ≤5.5 mg/dL

 Monitor Serum calcium, serum phosphorus, serum total 25-hydroxyvitamin D and intact PTH levels within 3 months after initiation of therapy or dose adjustment, and subsequently at least every 6 to 12 months; signs and symptoms of hypercalcemia.

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Not indicated for the treatment of secondary hyperparathyroidism in patients with stage 5 chronic kidney disease or in patients with end-stage renal disease (ESRD) on dialysis. Age Restriction: Prescriber  Prescribed by or after consultation with a Nephrologist or Kidney Specialist. Restrictions: Coverage Duration:  Approval: 12 months, unless otherwise specified

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POLICY NAME: REBIF Affected Medications: REBIF (interferon beta-1a)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Diagnosis of relapsing forms of multiple sclerosis confirmed with MRI Information: Appropriate Treatment  Must fail at least one preferred product (Avonex, glatiramer 20mg, glatiramer Regimen & Other 40mg, glatopa 20mg, Extavia, Gilenya, Tecfidera) Criteria:  No concurrent use of medications indicated for the treatment of relapsing- remitting multiple sclerosis  Not approved for primary and secondary progressive multiple sclerosis

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria: Age Restriction:

Prescriber  Prescribed by or after consultation with a neurologist or an MS specialist. Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified.

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POLICY NAME: REMODULIN Affected Medications: REMODULIN (treprostinil)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Pulmonary arterial hypertension (PAH) WHO Group 1 Medical  Documentation of PAH confirmed by right-heart catheterization Information:  Etiology of PAH: approve if PAH secondary to one of the following conditions: connective tissue disease, thromboembolic disease of the pulmonary arteries, human immunodeficiency virus (HIV) infection, cirrhosis, anorexigens or congenital left to right shunts  NYHA/WHO Functional Class II to IV symptoms  Documentation of acute vasoreactivity testing (positive result requires trial/failure to calcium channel blocker)

Appropriate  For initiation of therapy patient must have mean pulmonary artery pressure at least 25 Treatment mmHg at rest or at least 30 mmHg with exertion Regimen & AND Other Criteria:  The pulmonary hypertension has progressed despite maximal medical and/or surgical treatment of the identified condition  Treatment with oral calcium channel blocking agents has been tried and failed, or has been considered and ruled out  Documentation that treprostinil is used as a single route of administration (Remodulin, Tyvaso, Orenatriam should not be used in combination)  Not recommended for PAH secondary to pulmonary venous hypertension (e.g., left sided atrial or ventricular disease, left sided valvular heart disease, etc) or disorders of the respiratory system (e.g., chronic obstructive pulmonary disease, interstitial lung disease, obstructive sleep apnea or other sleep disordered breathing, alveolar hypoventilation disorders, etc.)  Subsequent approvals require documentation of treatment success such as improved walking distance or improvements in functional class

Exclusion Criteria: Age Restriction: Prescriber  Cardiologist or Pulmonologist Restrictions: Coverage  12 months, unless otherwise specified Duration:

258

POLICY NAME: REPATHA Affected Medications: REPATHA (evolocumab)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  Adjunct to diet and maximally tolerated statin therapy to reduce the risk of MI, stroke, and coronary revascularization in adults with established CVD  Adjunct to diet, alone or in combination with other lipid-lowering therapies (e.g. statins, ezetimibe), for the treatment of adults with primary hyperlipidemia, including HeFH, to reduce LDL-C  Adjunct to diet and other LDL-lowering therapies (e.g., statins, ezetimibe, LDL apheresis) for the treatment of patients with homozygous familial hypercholesterolemia (HoFH) who require additional lowering of LDL-C Required All indications: Medical  Documentation of current complete lipid panel within last 3 months Information:  Documentation of baseline LDL-C (untreated)  Documentation of dietary measures being undertaken to lower cholesterol.  Reauthorization will require updated lipid panel (once since starting therapy and then yearly thereafter) showing reduction in LDL AND documentation of adherence to therapy.

HeFH/HoFH:  Diagnosis confirmed based on WHO criteria/Dutch Lipid Clinical Network criteria with score of greater than 8 points OR Simon Broome register diagnostic criteria with a criterion for definite FH OR genotype test confirming mutation of LDL receptor gene or familial defective apo B-100  Documentation of current rosuvastatin 20-40 mg or atorvastatin 40-80 mg and/or ezetimibe or fenofibrate use

Clinical ASCVD:  Documentation of daily high intensity statin (rosuvastatin 20-40 mg or atorvastatin 40-80 mg) and ezetimibe 10 mg use for at least 6 months - may review pharmacy profile if available  Documentation of tobacco status and cessation if applicable  Documentation of clinical ASCVD in medical history: Myocardial infarction, Unstable angina, Coronary revascularization procedure (PCI or CABG), Diagnosis of clinically significant coronary heart disease by coronary angiography, stress test using treadmill, stress echocardiography or nuclear imaging OR a coronary heart disease (CHD) risk- equivalent, defined as documented history of 1 or more of the following: Peripheral arterial disease, Ischemic stroke of atherothrombotic origin, Chronic kidney disease (CrCl 30-60 mL/min, Diabetes mellitus PLUS at least 2 additional risk factors: Hypertension; ankle-brachial index ≤0.90; micro- or macro-albuminuria; retinopathy; or family history of early coronary heart disease

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Hyperlipidemia:  Documentation of failure to 6 months of combination therapy with maximal tolerated statin plus ezetimibe therapy to adequately reduce LDL to target level.  If member was unable to tolerate statin therapy, documentation that combination ezetimibe plus either niacin or bile acid sequestrants for 6 months failed to adequately lower LDL to target levels  If request is due to intolerance to statin therapy, documentation of failure to 8 week trials with each: pravastatin, rosuvastatin, fluvastatin, separated by an adequate ‘holiday’ of 2 weeks and employing strategies such as every other day dosing to minimize muscular effects  If request is due to intolerance of statin therapy, documentation of evaluation of other causes of muscle symptoms (hypothyroidism, vitamin D deficiency, rheumatologic or musculoskeletal disease, exercise, steroid myopathy, antipsychotics, immunosuppressants, bisphosphonates, alcohol or drug abuse, drug or food interactions)

Request for PCSK9 due to rhabdomyolysis:  Documentation of creatinine kinase (CK) levels greater than 10-times upper limit of normal OR  Chart documentation of diagnosis

Request for PCSK9 due to myalgia or myopathy:  Documentation of persistent myalgia or myopathy on 3 separate trials (each trial minimum 8 weeks duration) with pravastatin, rosuvastatin, fluvastatin separated by an adequate ‘holiday’ of 2 weeks  Evaluation of other causes of muscle symptoms (hypothyroidism, vitamin D deficiency, rheumatologic or musculoskeletal disease, exercise, steroid myopathy, antipsychotics, immunosuppressants, bisphosphonates, alcohol or drug abuse, drug or food interactions) Appropriate  Repatha for clinical ASCVD and primary hyperlipidemia : 140 mg every 2 weeks or 420 mg Treatment once monthly; for HoFH: 420 mg once monthly Regimen & Other Criteria: HeFH/HoFH:  Must take along with maximally tolerated doses of statin and ezetimibe if no contraindication

Clinical ASCVD:  Approve only if documentation of cardiovascular event (myocardial infarction, ischemic stroke, coronary revascularization procedure, etc.) while on optimized doses of and optimized adherence (80% or greater) to statin and/or ezetimibe  Must take along with maximally tolerated doses of statin and/or ezetimibe if no contraindication

260

Exclusion  Fasting serum triglycerides greater than 400 mg/dL Criteria:  History of documented ASCVD and LDL-C less than 70 mg/dL  No history of documented ASCVD and LDL-C less than 100 mg/dL Age Restriction: Prescriber  Prescribed by or in consultation with Cardiologist, Endocrinologist, or Lipid Specialist Restrictions:

Coverage  12 months, unless otherwise specified Duration:

261

POLICY NAME: REVLIMID Affected Medications: REVLIMID (lenalidomide)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.  NCCN indications with evidence level of 2A or higher

Required Medical  Reauthorization: documentation of disease responsiveness to therapy Information: Appropriate  Documentation of dose adjustment based on appropriate lab values Treatment Regimen & Other Revlimid is warranted in any of the following settings: Criteria: Active/symptomatic/smoldering myeloma or progressive solitary plasmacytoma  Revlimid is used as a single agent for steroid-intolerance, or in combination with dexamethasone with or without bortezomib or carflizomib or cyclophosphamide or bendamustine  Revlimid is used as maintenance monotherapy for patients responding to primary induction therapy or for patients with stable or responsive disease following stem cell transplant,  Appropriate thromboprophylaxis for Revlimid used in combination with other agents

Low or intermediate-1 risk MDS  In those with a 5q deletion, patients have transfusion-dependent anemia (i.e., greater than 2 units of red blood cells in the previous 8 weeks) or symptomatic anemia  In those without a 5q deletion and symptomatic anemia when patients are ineligible for or have failed a trial of erythropoietin.

NHL  Revlimid is used in relapsed or refractory disease in patients with CLL/SLL  Revlimid is used as monotherapy or in combination with rituximab for relapsed, refractory, or progressive disease in almost all subtypes of NHL (excluding cutaneous T- cell Lymphoma)

Systemic light chain amyloidosis  Revlimid is used in combination with dexamethasone as primary therapy.

Classical Hodgkin Lymphoma  Revlimid is used in relapsed or refractory disease  Documentation of dose adjustment based on appropriate lab values

Exclusion Criteria:  Consider holding therapy if Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater 262

Age Restriction:

Prescriber Restrictions: Coverage  Initial approval: 6 months unless otherwise specified Duration:  Reauthorization: 12 months unless otherwise specified

263

POLICY NAME: RIBAVIRIN Affected Medications: Ribasphere, Ribatab, Ribapak, Rebetol

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  Baseline hemoglobin level greater than 10 g/dL Information:  Baseline creatinine clearance (serum creatinine, height, weight to calculate)  Baseline weight  Documentation chronic hepatitis C virus genotype by liver biopsy or by FDA-approved serum test  Documentation of anti-hepatitis C virus regimen to be used with and anticipated duration of therapy Appropriate  Approve if used in combination with FDA- and/or AASLD/IDSA- recommended Treatment regimen and if not otherwise excluded from PacificSource policies of other Regimen & Other medications in the regimen Criteria:

Exclusion Criteria:  Women who are pregnant  Men whose female partners are pregnant  Patients with autoimmune hepatitis  Patients with hemoglobinopathies (e.g., thalassemia major, sickle-cell anemia)  Patients with creatinine clearance less than 50 mL/min  Coadministration with didanosine  Hemoglobin level less than 8.5 g/dL Age Restriction: Prescriber  Prescribed by or in consultation with gastroenterologist or hepatologist Restrictions:

Coverage Duration:  12 weeks, unless otherwise specified (depends on regimen)

264

POLICY NAME: RIBAVIRIN INHALATION Affected Medications: VIRAZOLE *Medical benefit only

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  Treatment of hospitalized infants and young children with respiratory syncytial virus Information: (RSV) infections documentation of: prematurity, cardiopulmonary disease, or immunosuppression.  Treatment for RSV in adult patients, documentation of : hematopoietic stem cell or heart/lung transplant recipients Appropriate Treatment Regimen & Other Criteria:

Exclusion Criteria: Age Restriction: Prescriber Restrictions:

Coverage Duration:  Approval, 1 month, unless otherwise specified

265

POLICY NAME: RITUXAN Affected Medications: RITUXAN (rituximab), RITUXAN HYCLEA (Rituximab & hyaluronidase subcutaneous– Genentech)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  NCCN indications with evidence level of 2 or higher  Relapsing Remitting Multiple Sclerosis

Required  Indication must be documented in the member’s chart notes within the most recent 6 Medical months Information:  Documentation of disease staging, all prior therapies used, and anticipated treatment course Rheumatoid Arthritis:  Documentation of complete and current treatment course  Laboratory test confirming diagnosis of RA rheumatoid arthritis (anti-CCP, RF)  Documentation of moderate to severe disease despite current treatment  Documented current level of disease activity with one of the following (or equivalent objective scale): . The Disease Activity Score derivative for 28 joints (DAS-28) greater than 3.2 . The Simplified Disease Activity Index (SDAI) greater than 11 . The Clinical Disease Activity Index (CDAI) greater than 10 . Weighted RAPID3 of at least 2.3 Non-Hodgkin’s Lymphoma (NHL):  Documentation of CD20-positve B-Cell NHL Chronic Lymphocytic Leukemia (CLL):  Documentation of advanced or active CLL . Binet Stage A or B with active disease . Binet Stage C . Modified Rai Stage 0, I, or II with symptoms . Modified Rai Stage III or IV Microscopic Polyangiitis (MPA) or Granulomatosis with Polyangiitis (GPA):  Documentation of active GPA or MPA Relapsing Remitting Multiple Sclerosis  Diagnosis of relapsing form of Multiple Sclerosis (MS) confirmed with MRI (Revised McDonald diagnostic criteria for multiple sclerosis) . Clinical evidence alone will suffice; additional evidence desirable but must be consistent with MS Moderate to severe Pemphigus Vulgaris (PV)  Confirmed diagnosis of pemphigus vulgaris: . Multiple nonhealing oral ulcers persisting for at least 1 month, multiple flaccid blisters on normal skin, and positive Nikolsky sign. . Direct immunoflourescence (DIF) showing intercellular localization of immunoglobulin on perilesional skin or mucosal biopsy 266

 Patient has failed a minimum of 12 weeks of therapy with corticosteroids AND  Patient has failed a minimum of 12 weeks of therapy with immunosuppressants (eg, azathioprine, mycophenolate, methotrexate, etc.)

Appropriate Oncology Uses: Treatment  Documentation of ECOG performance status of 1 or 2 OR Karnofsky performance score Regimen & greater than 50% Other Criteria:  Reauthorization: documentation of disease responsiveness to therapy

Rheumatoid Arthritis:  Initial Course: Has failed minimum 12 weeks on Humira AND minimum 12 weeks Enbrel  Repeat Course: Approve if 16 weeks or more after the first dose of the previous rituximab regimen and the patient has responded (eg, less joint pain, morning stiffness, or fatigue, or improved mobility, or decreased soft tissue swelling in joints or tendon sheaths) as determined by the prescribing physician.

Microscopic Polyangiitis and Granulomatosis with Polyangiitis  For initial immunosuppression: in combination with a glucocorticoid in accordance with FDA approval

Relapsing Forms of Multiple Sclerosis • Studied treatment regimens vary slightly • Dose is approved for up to two doses of 1,000 mg annually . Higher doses (e.g., 1,000 mg x 2 every 6 months) will require documentation to support

Reauthorization: documentation of disease responsiveness to therapy

Exclusion  Concurrent use of: abatacept (Orencia), tocilizumab (Actemra), adalimumab (Humira), Criteria: entanercept (Enbrel), infliximab (Remicade), certolizumab (Cimzia), golimumab (Simponi)  Positive hepatitis B test/history of hepatitis B or positive tuberculosis test

Age Restriction:  18 years or older

Prescriber  For RA, GPA, MPA – Prescribed by a rheumatologist or in consultation with a Restrictions: rheumatologist  For CLL, NHL– Prescribed by an oncologist  For MS- Prescribed by or in consultation with a neurologist  For PV- Prescribed by or in consultation with a dermatologist

Coverage  For RA – Approval: 2 doses, 16 weeks or more after, approve 2 more doses if response per Duration: doctor, unless otherwise specified  For Oncology – Initial Approval: 4 months, unless otherwise specified Continuation Approval = 12 months, unless otherwise specified 267

 For MPA/GPA – Approval: 4 weeks, unless otherwise specified  For MS- Initial approval= 6 months (up to two doses of 1,000 mg), Subsequent approvals= 12 months, unless otherwise specified  For PV – Approval = 1 Month, unless otherwise specified  Subsequent approvals= 12 months, unless otherwise specified

268

POLICY NAME: RYDAPT Affected Medications: RYDAPT (midostaurin)

Covered Uses:  NCCN (National Comprehensive Cancer Network) indications with evidence level of 2A or higher Required Medical  Documentation of performance status, disease staging, all prior therapies used, Information: and anticipated treatment course  Diagnosis of acute myeloid leukemia (AML); FLT3 mutation-positive as detected by a FDA approved test Appropriate Treatment  For induction therapy: Rydapt must be used in combination with standard Regimen & Other cytarabine and daunorubicin induction Criteria:  For consolidation therapy: must be used in combination with cytarabine

 Reauthorization: documentation of disease responsiveness to therapy Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater  Use of Rydapt as a single agent Age Restriction: Prescriber Restrictions:  Oncologist Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

269

POLICY NAME: SABRIL Affected Medications: SABRIL (vigabatrin)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  Documentation of baseline vision assessment (no later than 4 weeks after starting Information: Sabril) by an ophthalmologist  Documentation that the potential benefits outweigh the risk of vision loss  Proof that the patient is blind or formally exempt from vision assessments in the Support, Help, And Resources for Epilepsy (SHARE) program

Refractory complex partial seizures  Documentation the patient has tried at least 2 alternative therapies: carbamazepine, phenytoin, levetiracetam, topiramate, oxcarbazepine, or lamotrigine Appropriate Infantile Spasm Treatment  Use as monotherapy for pediatric patients (1 month to 2 years of age) Regimen & Other Criteria: Refractory Complex Partial Seizures  As adjunctive therapy for adult patients who have inadequately responded to several alternative treatments

Reauthorization:  Vision assessment by an ophthalmologist with no documented vision loss from baseline  Documented planned reassessments every 3 months during therapy  Documentation of substantial clinical benefit (within 3 months of initiation; within 2-4 weeks of initiation for patients with infantile spasms or sooner if treatment failure becomes obvious)

Exclusion Criteria:  Use as a first line agent for Complex Partial Seizures Age Restriction: Infantile Spasms: 1 month to 2 years of age Refractory Complex Partial Seizures: greater than 10 years of age Prescriber  Prescriber certified with the SHARE program Restrictions:

Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

270

POLICY NAME: SELF-ADMINISTERED DRUGS (SAD) Affected Medications: atorvastatin, fluticasone, latanoprost, triamcinolone cream, Trulicity, Novolog, Fiasp, Tresiba, Levemir, Lantus, Toujeo, Apidra, Humalog, Humulin/Novolin, Victoza, Symlin, Repatha, leuprolide, methotrexate, Benlysta, Cosentyx, Saizen, Humatrope, cyanocobalamin, Aranesp, Zarxio, sumatriptan, Interferon Beta-1A, Peginterferon Beta-1A, Byetta, Strensiq, Zinbryta, Taltz, Dupixent, Siliq, Alprostadil, Apokyn, Miacalcin, Fortical, Fuzeon, Hizentra, Copaxone, Glatiramer, Glatopa, Firazyr, Betaseron, Increlex, Relistor, Octreotide, Forteo, Egrifta, Kineret, Simponi, Orencia, Zinbryta, Avonex, Aimovig, Humira, Enbrel, Peg-Intron, Kynamro, Signifor, Natpara, Plegridy, Actimmune, Cuvitru, Emgality, Ajovy

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical Information: Appropriate  In the hospital outpatient setting, the pharmacy benefit will cover pharmaceutical Treatment agents that the member could reasonable take or use on their own while the Regimen & Other medical benefit will cover any agents given IV or other forms that the member Criteria: could not have given to themselves.  This is not an all-encompassing list and is subject to reviewer’s discretion.

Exclusion Criteria:

Age Restriction:

Prescriber Restrictions: Coverage Duration:

271

POLICY NAME: SAMSCA Affected Medications: SAMSCA (Tolvaptan Tablets)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  Patients already started on tolvaptan for the treatment of hyponatremia.

Required  Serum sodium at baseline Medical Information: Appropriate  For the treatment of clinically significant hypervolemic and euvolemic hyponatremia with Treatment serum sodium less than 125 mEq/L at baseline OR less marked hyponatremia, defined as Regimen & less than 135 mEq/L at baseline, that is symptomatic (eg, nausea, vomiting, headache, Other Criteria: lethargy, confusion) and has resisted correction with fluid restriction  QL- 60 mg per day Exclusion  Patients requiring intervention to raise serum sodium urgently to prevent or to treatment Criteria: serious neurological symptoms  Concomitant use with strong CYP3A inhibitor  Hypovolemic hyponatremia  Anuric patients Age Restriction: Prescriber Restrictions: Coverage  Approval: 1 month, unless otherwise specified Duration:

272

POLICY NAME: SEROSTIM Affected Medications: SEROSTIM (somatropin)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Patient has a diagnosis of cachexia or wasting syndrome associated with HIV Information: infection.  Serostim is used in combination with antiretroviral therapy.  Alternative causes of wasting (eg, inadequate nutrition intake, malabsorption, opportunistic infections, hypogonadism) have been ruled out or treated appropriately.  Prior to somatropin, patient had a suboptimal response to at least 1 other therapy for wasting or cachexia (eg, megestrol or dronabinol) unless contraindicated or not tolerated.  For continuation of therapy: Patients treated with Serostim for 12 or more weeks have demonstrated a response to therapy (ie, body mass index has improved or stabilized). Appropriate  Weight based dosing (0.1mg/kg) up to max 6mg/day Treatment Regimen & Other Criteria: Exclusion Criteria:  Acute critical illness  Active malignancy  Diabetic retinopathy  Hypersensitivity to Serostim  Preexisting papilledema Age Restriction: Prescriber Restrictions: Coverage Duration:  Approval: 12 weeks, unless otherwise specified

273

POLICY NAME: SIGNIFOR Affected Medications: SIGNIFOR (pasireotide diaspartate)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Diagnosis of Cushing’s Disease Information:  The patient had surgery that was not curative or is not a candidate for surgery Appropriate If the patient is currently receiving Signifor therapy, Treatment  The patient has shown a clinically meaningful reduction in 24-hour urinary free Regimen & Other cortisol levels and/or improvement in signs or symptoms of the disease. Criteria:  ECG obtained prior to dose adjustment If the patient is not currently receiving Signifor,  baseline fasting plasma glucose and/or HbA1c levels were obtained  The patient has controlled blood glucose levels OR the patient is receiving optimized antidiabetic therapy  ECG obtained  LFTs evaluated prior to initiation

Exclusion Criteria:  Poorly controlled diabetes mellitus (HbA1c >8%)  Severe hepatic impairment (Child Pugh C) Age Restriction:

Prescriber  Prescribed by or in consultation with an endocrinologist Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified

274

POLICY NAME: SIGNIFOR LAR Affected Medications: SIGNIFOR LAR (pasireotide diaspartate)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required For Acromegaly: Medical Patient meets the following criteria for initiation of therapy: Information: 1) Clinical evidence of acromegaly, 2) Pre-treatment high IGF-1 level for age/gender, 3) Patient has had an inadequate or partial response to octreotide or lanreotide OR patient is intolerant to or has a contraindication to octreotide or lanreotide AND 4) Patient has had an inadequate or partial response to surgery and/or radiotherapy OR there is a clinical reason for why the patient has not had surgery or radiotherapy (e.g., medically unstable conditions, patient is at high risk for complications of anesthesia because of airway difficulties, lack of an available skilled surgeon, patient refuses surgery or prefers the medical option over surgery, major systemic manifestations of acromegaly including cardiomyopathy, severe hypertension and uncontrolled diabetes). Reauthorization: the IGF-1 level decreased or normalized

For Cushing’s Disease: Patient meets the following criteria for initiation of therapy: 1) Clinical evidence of Cushing’s disease in whom pituitary surgery is not an option or has not been curative 2) Mean urinary free cortisol level (mUFC) between 1.5 and 5 times the upper limit of normal 3) Documented inadequate response, intolerable adverse event, or contraindication to ALL of the following: ketoconazole, cabergoline, mifepristone 4) Initial starting dose is 10mg every 4 weeks, may be increased after 4 months to a maximum of 40mg every 4 weeks if 24-hour Urinary Free Cortisol has not normalized Reauthorization: mUFC equal to or less than the upper limit of normal

Appropriate  Prior to initiation of therapy hypokalemia or hypomagnesemia must be corrected. Treatment  Prior to initiation of therapy baseline HbA1c, Liver function tests, and EKG should be Regimen & obtained Other Criteria:  Blood glucose monitoring should be done weekly for the first 3 months after initiation and the first 4 to 6 weeks after dose increases  New assessment of liver function should be obtained 3 weeks after initiation and then monthly for 3 months  Quantity limit 1 injection (maximum 60 mg) every 28 days Exclusion  Poorly controlled diabetes mellitus (HbA1c greater than 8%) Criteria:  Severe hepatic impairment (Child Pugh C)

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Age Restriction:  Must be 18 years of age or older Prescriber Restrictions:

Coverage  Initial: 6 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

276

POLICY NAME: SILDENAFIL Affected Medications: Sildenafil Citrate TABLET 20 MG

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  Pulmonary Arterial Hypertension (PAH) (WHO Group 1) confirmed by right heart Information: catheterization  Etiology of PAH (idiopathic or associated with connective tissue disease)  NYHA/WHO Functional Class II or III symptoms  Documentation of Acute Vasoreactivity Testing (positive result requires trial/failure to calcium channel blocker)

Appropriate  Subsequent approvals require documentation of treatment success such as Treatment improved walking distance or improvements in functional class Regimen & Other Criteria:

Exclusion Criteria:  Concomitant nitrate therapy on a regular or intermittent basis  Concomitant use of riociguat, a guanylate cyclase stimulator Age Restriction:

Prescriber  Cardiologist or Pulmonologist Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified

277

POLICY NAME: SIMPONI Affected Medications: SIMPONI (golimumab)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  Documentation of moderate to severe disease despite current treatment (Indication Information: must be documented in chart notes within the last 6 months)  Documentation of complete and current treatment course  Documented current level of disease activity/disease control  Patient weight

Rheumatoid arthritis  Laboratory test confirming diagnosis of rheumatoid arthritis (anti-CCP, RF)  Documented current level of disease activity with one of the following: o The Disease Activity Score derivative for 28 joints (DAS-28) greater than 3.2 o The Simplified Disease Activity Index (SDAI) greater than 11 o The Clinical Disease Activity Index (CDAI) greater than 10

o Good response to NSAIDs o Family history of SpA o Elevated CRP OR  HLA-B27 genetic test positive AND at least 2 SpA features

Appropriate Rheumatoid arthritis (RA) Treatment  Has failed minimum 12 week trial of combination disease-modifying anti-rheumatic drug Regimen & Other therapy: methotrexate 25 mg (IM or SQ) With hydroxychloroquine and/or sulfasalazine, Criteria: or leflunomide AND  Has failed minimum 12 weeks on Humira AND minimum 12 weeks on Enbrel  For intravenous request: Treatment failure with subcutaneous route  QL – 0.5 ml per 28 day supply

Ulcerative Colitis (UC)  Has failed at least two oral treatments for minimum of 12 week trial: corticosteroids, sulfasalazine, azathioprine, mesalamine, balsalazide, cyclosporine, azathioprine, 6- mercaptopurine AND  Has failed minimum 12 weeks on Humira  QL – Initial (one time only)– 3 ml per 28 day supply  QL – Continuation – 1 ml per 28 day supply

Reauthorization: documentation of treatment success and a clinically significant response to therapy

Ankylosing spondylitis (AS) and Psoriatic Arthritis (PsA) with Axial involvement  Has failed two daily prescription strength nonsteroidal anti-inflammatory drugs (ibuprofen, naproxen, diclofenac, meloxicam, etc.) with minimum 1 month trial each  For isolated sacroiliitis, enthesitis, peripheral arthritis: documented treatment failure with locally administered parenteral glucocorticoid  Has failed minimum 12 weeks on Humira AND failed minimum 12 weeks on Enbrel  QL – 1 ml per 28 day supply Reauthorization: Improvement of at least 50% in the BASDAI score or an absolute change of 2 units.

279

Exclusion  [Concurrent us of biologic DMARDs: Orencia (abatacept), Rituxan (rituximab), Actemra Criteria: (tocilizumab), Enbrel (etanercept), Remicade (infliximab), Humira (adalimumab), Cimzia (certolizumab), Kineret (anakinra), Xeljanz (tofacitinib)  Concurrent use of Otezla (Apremilast)  Management of plaque psoriasis without psoriatic arthritis  Positive tuberculosis; active hepatitis B Age Restriction:  RA: 18 years of age or older

Prescriber  RA/PsA/AS: prescribed by or in consultation with a rheumatologist Restrictions:  UC: prescribed by or in consultation with a GI specialist Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

280

POLICY NAME: SIRTURO Affected Medications: SIRTURO (bedaquiline fumarate)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  Pulmonary multi-drug resistant tuberculosis (MDR-TB).

Required Medical Patient has failed, is resistant, or is allergic to quad therapy of any combination of Information: the following:  Isoniazid  Rifampin  Ethambutol  Pyrazinamide  Fluoroquinolones  Capreomycin (Kanamycin, Amikacin, Streptomycin)  Ethionamide/Prothinamide  Cycloserine/Terizidone  Aminosalicylic acid (acidic salt) Appropriate Treatment  Documentation of being administered by directly observed therapy (DOT) Regimen & Other Criteria:  Baseline ECG  BMP (including K, Ca, Mg documentation of correction if needed)  LFTs Exclusion Criteria:  Drug-sensitive TB (DS-TB)  Latent infection due to mycobacterium TB  Extrapulmonary TB (e.g. central nervous system)  QTc greater than 500 milliseconds Age Restriction:  18 years of age or older Prescriber Restrictions:

Coverage Duration:  24 weeks, unless otherwise specified

281

POLICY NAME: SODIUM PHENYLBUTYRATE Affected Medications: sodium phenylbutyrate

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design Required Medical  Diagnosis of Urea Cycle Disorder (UCD) Information:  Diagnosis confirmed by blood, enzymatic, biochemical, or genetic testing  The prescribed medication will be used for chronic management of UCD  The patient has UCD that cannot be managed by dietary protein restriction and/or amino acid supplementation alone Appropriate Treatment  The prescribed medication will be used in combination with dietary protein Regimen & Other restriction Criteria: Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Should not be used in the treatment of acute hyperammonemia Age Restriction: Prescriber Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified

282

POLICY NAME: SOLIRIS Affected Medications: SOLIRIS (eculizumab)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical  Diagnosis of paroxysmal nocturnal hemoglobinuria Information:  Diagnosis of atypical hemolytic uremic syndrome  Complete & current treatment course required  4 or more blood transfusions required in the previous 12 months • Complete blood count (CBC), reticulocyte count, lactate dehydrogenase (LDH), packed RBC transfusion requirement • Patients must be administered a meningococcal vaccine at least two weeks prior to initiation of Soliris therapy and revaccinated according to current ACIP guidelines

Paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis:  Platelet count ≥30,000/mcl  LDH levels ≥1.5 times the upper limit of normal range.  Flow cytometry shows GPI deficient red blood cell clone (type III cells) ≥10%

Atypical hemolytic uremic syndrome (aHUS) to inhibit complement-medicated thrombotic microangiopathy:  Clinical presentation of: microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury  LDH levels ≥1.5 times the upper limit of normal range.  ADAMTS13 activity level >10%  Patient has failed to respond to five days of plasma therapy

Generalized Myasthenia Gravis (gMG)  Myasthenia Gravis Foundation of America (MGFA) Clinical Classification Class II to IV  Positive serologic test for anti-acetylcholine receptor (AchR) antibodies  MG-Activities of Daily Living (MG-ADL) total score of ≥6  Documentation of baseline Quantitative Myasthenia Gravis (QMG) score  Patient has failed treatment over at least 1 year with at least 2 immunosuppressive therapies (e.g. azathioprine, cyclosporine, mycophenolate, etc), or has failed at least 1 immunosuppressive therapy and required chronic plasmapheresis, plasma exchange (PE), or intravenous immunoglobulin (IVIG)

Appropriate Paroxysmal nocturnal hemoglobinuria to reduce hemolysis: Treatment o 600 mg weekly for the first 4 weeks, followed by Regimen & Other o 900 mg for the fifth dose 1 week later, then Criteria: o 900 mg every 2 weeks thereafter

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Atypical hemolytic uremic syndrome to inhibit complement-mediated thrombotic microangiopathy:  Appropriate weight based adjustment if younger than 18 years old or less than 40kg; otherwise: o 900 mg weekly for the first 4 weeks, followed by o 1200 mg for the fifth dose 1 week later, then o 1200 mg every 2 weeks thereafter

Generalized Myasthenia Gravis  900 mg weekly for the first 4 weeks, followed by  1200 mg for the fifth dose 1 week later, then  1200 mg every 2 weeks thereafter

Supplemental dosing of is required in the setting of concomitant support with:  Plasmapheresis or plasma exchange; or fresh frozen plasma infusion

Reauthorization requires documentation of treatment success  Serum LDH, Hb, blood transfusion hx, infusion records

Exclusion  Current meningitis infection Criteria:  Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS). Age Restriction:  PNH and gMG: 18 years of age or older  aHUS: 2 months of age or older

Prescriber  PNH: hematologist Restrictions:  aHUS: hematologist or nephrologist  gMG: neurologist

Coverage  Initial approval: 3 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

284

POLICY NAME: SOMATOSTATIN ANALOGS Affected Medications: OCTREOTIDE, SANDOSTATIN LAR, LANREOTIDE (somatuline depot)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design  NCCN indications with evidence level of 2A or higher Required Medical All indications: Information:  For Sandostatin LAR (J2353), patient has received at least 2 weeks of initial treatment with any of the non-LAR formulations and treatment was effective and tolerable. Acromegaly  Initiation of therapy, patient meets the following: o Clinical evidence of acromegaly o Pre-treatment high IGF-1 level for age/gender o Patient has had an inadequate or partial response to surgery and/or radiotherapy OR there is a clinical reason for why patient has not had surgery or radiotherapy  Reauthorization: requires that the IGF-1 level is decreased or normalized Carcinoid syndrome  Documentation of the following: o A positive 5-hydroxyindoleacetic acid (5-HIAA) test OR o Clinical interpretation of imaging consistent with that of a carcinoid tumor  Reauthorization: requires documentation of improvements in flushing and/or diarrhea Vasoactive intestinal peptide-secreting tumor, associated diarrhea (VIPoma-associated diarrhea)  Documentation of two serum vasoactive intestinal polypeptide (VIP) concentrations greater than 75pg/mL  Reauthorization: requires documentation of disease responsiveness to therapy

Gastroenteropancreatic neuroendocrine tumors (Lanreotide)  Documentation of performance status, disease staging, all prior therapies used, and prescribed treatment regimen Appropriate Acromegaly Treatment  Clinical reasons for why patient has not had surgery or radiotherapy could include: Regimen & Other o Medically unstable conditions Criteria: o Patient is at high risk for complications of anesthesia because of airway difficulties o Lack of an available skilled surgeon o Patient refuses surgery or prefers the medical option over surgery o Major systemic manifestations of acromegaly including cardiomyopathy o Severe hypertension o Uncontrolled diabetes Exclusion Criteria: Age Restriction:  Oncologist or Endocrinologist

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Prescriber Restrictions: Coverage Duration:  Initial Approval = 6 months, unless otherwise specified  Reauthorization = 12 months, unless otherwise specified

286

POLICY NAME: SORIATANE Affected Medications: Acitretin

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  Prevention of non-melanoma skin cancers in high risk individuals. Required  If the patient is female and able to bear children (e.g., no hysterectomy, not reached Medical menopause, has achieved menses). AND Information:  The patient is unresponsive to other therapies for the covered diagnoses OR the other therapies for the treatment of the covered diagnoses are contraindicated due to the clinical condition of the patient. AND C  Pregnancy has been excluded as confirmed by 2 negative urine or serum pregnancy tests with a sensitivity of at least 25 mIU/mL. AND  The patient has chosen to use any of the following methods of contraception: one primary form (e.g., tubal ligation, partner's vasectomy, intrauterine devices, birth control pills, injectable/implantable/insertable/topical hormonal birth control products) plus one secondary form (e.g., diaphragms, latex condoms, cervical caps) used in combination with a spermicide OR absolute abstinence. AND  The patient has agreed to use her chosen form of contraception for at least 1 month before initiation of Soriatane therapy, during Soriatane therapy, and for at least 3 years after discontinuation of therapy. AND  The patient has been advised that ethanol must not be ingested by female patients during Soriatane treatment and for 2 months following therapy. AND  The patient will have a negative pregnancy test on a monthly basis.

Appropriate  Female patient or guardian signed a Patient Agreement/Informed Consent. Treatment Regimen & Reauthorization will require documentation of treatment success and a clinically significant Other Criteria: response to therapy

Exclusion  Severely impaired liver function. Criteria:  Severely impaired kidney function.  Chronic abnormally elevated blood lipid values.  Currently taking methotrexate or tetracycline. Age Restriction:

Prescriber Restrictions:

Coverage  Authorization will be for 12 months, unless otherwise noted. Duration:

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POLICY NAME: SPINRAZA Affected Medications: SPINRAZA (nusinersen)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  Patient must have documentation of a confirmed diagnosis of spinal muscular atrophy Information: (SMA) type 1, 2, or 3 defined as ONE of the following genetic tests of 5q13 demonstrating: a. Homozygous SMN1 gene deletion OR b. Homozygous SMN1 gene mutation OR c. Compound heterozygous SMN1 gene mutation  Patient has at least 2 or more copies of the SMN2 gene  Documentation of one of the following baseline motor assessments appropriate for patient age and motor function: • Hammersmith Infant Neurological Examination (HINE-2) • Hammersmith Functional Motor Scale (HFSME) • Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) • Upper Limb Module (ULM) test • 6-Minute Walk Test (6MWT)  Documentation of ventilator use status • Is the patient ventilator dependent (using it at least 16 hours per day on at least 21 of the last 30 days)? • This does not apply to patients who require non-invasive ventilator assistance

Appropriate  Loading dose: 12 mg once every 14 days for 3 doses; then 12 mg once 30 days after the Treatment third dose Regimen & Other  Maintenance dose: 12 mg once every 4 months Criteria:  Reauthorization: documentation of clinically significant improvement from baseline motor function demonstrated by: o Improvement from baseline motor function score documented within one month of renewal request AND o More areas of motor function improved than worsened o HINE-2:  at least a 2-point increases in ability to kick OR  at least a 1-point increase in the motor milestones of head control, rolling, sitting, crawling, standing or walking using Section 2 of the Hammersmith Infant Neurologic Exam (HINE) AND  More areas of motor function improved than worsened o Hammersmith Functional Motor Scale (HFSME)

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 At least 3 points increase in score from pretreatment baseline AND  More areas of motor function improved than worsened o Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND)  At least a 4 point increase in score from the pretreatment baseline AND  More areas of motor function improved than worsened o Upper Limb Module (ULM)  At least a 30 meter increase from pretreatment baseline o 6-Minute Walk Test (6MWT)  At least a 30 meter increase from pretreatment baseline Exclusion Criteria:  SMA type 4  Ventilator dependent (using at least 16 hours per day on at least 21 of the last 30 days) o Does not apply to patients who require non-invasive ventilator assistance

Age Restriction: Prescriber  Prescribed by or in consultation with a pediatric neurologist or provider who is Restrictions: experienced in treatment of spinal muscular atrophy

Coverage  Initial approval: 5 does to be administered in a 6 month period Duration:  Reauthorization: 12 months, unless otherwise specified

289

POLICY NAME: SPRAVATO Affected Medications: SPRAVATO (esketamine nasal spray)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design  Indicated, in conjunction with an oral antidepressant, for the treatment of treatment- resistant depression (TRD) in adults Required  Diagnosis of treatment-resistant depression Medical  Assessment of patient’s risk for abuse or misuse Information:  PHQ-9 Score at baseline (or other standard rating scale)

Appropriate  Failure to clinically respond to four trials of antidepressant drugs at highest tolerated doses Treatment for at least 6 weeks from two or more different classes during the current depressive Regimen & episode as defined by less than 50% reduction in symptom severity using a standard rating Other Criteria: scale that reliably measures depressive symptoms (such as PHQ-9) and at least one trial must have used an augmentation strategy (aripiprazole, lithium, olanzapine, quetiapine, risperidone, thyroid hormone); OR  Demonstrated intolerance to four antidepressant drugs with distinct side effects; AND  Failure to respond to evidence based psychotherapy such as Cognitive Behavioral Therapy (CBT) and/or Interpersonal Therapy as documented by an objective scale such as a PHQ-9 or similar rating scale for depressive symptoms  Will use Spravato in addition to new oral antidepressant therapy  Reauthorization requires documentation of treatment success defined as at least a 50% reduction in symptoms of depression compared to baseline using a standard rating scale that reliably measures depressive symptoms and that Spravato continues to be used in addition to antidepressant therapy

 Dose: Approve #8 dose packs in first 28 days, then limit of #4 per 28 days (maximum). Per table below Recommended Dosage for SPRAVATO

Adults

Induction Phase Weeks 1 to 4: Day 1 starting dose: 56 mg

Administer twice per Subsequent doses: 56 week mg or 84 mg

Maintenance Phase Weeks 5 to 8:

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Administer once weekly 56 mg or 84 mg

Week 9 and after:

Administer every 2 56 mg or 84 mg weeks or once weekly*

*Dosing frequency should be individualized to the least frequent dosing to maintain remission/response

Exclusion  Use as an anesthetic agent Criteria:  Pregnancy  Aneurysmal vascular disease (including thoracic and abdominal aorta, intracranial, and peripheral arterial vessels) or arteriovenous malformation  History of intracerebral hemorrhage  Hypersensitivity to esketamine, ketamine, or any of the excipients Age  18 years and older Restriction:

Prescriber  REMS Program certified (others will be unable to order drug) Restrictions:  Behavioral health specialist Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization:12 months, unless otherwise specified

291

POLICY NAME: SPRYCEL Affected Medications: SPRYCEL (dasatinib)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  NCCN indications with evidence level of 2A or higher Required Medical  Documentation of performance status, all prior therapies used, and prescribed Information: treatment regimen  Documentation of Philadelphia chromosome-positive mutation status  For patients with Chronic myeloid leukemia (CML) and low risk score, documented clinical failure with Imatinib  For patients with acute lymphoblastic leukemia (ALL), documented clinical failure with imatinib.

Appropriate Treatment Reauthorization requires documentation of disease responsiveness to therapy (as Regimen & Other applicable, BCR-ABL1 transcript levels, cytogenetic response) Criteria: Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber  Oncologist Restrictions:

Coverage Duration:  Initial approval: 4 months (2 week initial partial fill), unless otherwise specified  Reauthorization:12 months, unless otherwise specified

292

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POLICY NAME: STELARA Affected Medications: STELARA (ustekinumab)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  Documentation of moderate to severe disease despite current treatment (Indication Information: must be documented in chart notes within the last 6 months )  Documentation of complete and current treatment course  Documented current level of disease activity/disease control Plaque psoriasis  Documentation of disease that is severe in nature, which has resulted in functional impairment (e.g., inability to use hands or feet for activities of daily living, or significant facial involvement preventing normal social interaction) AND one or more of the following: o At least 10% body surface area involvement; or o Hand, foot or mucous membrane involvement Crohn’s Disease  Documentation of Crohn’s Disease Activity Index (CDAI) greater than 220

Psoriatic Arthritis (PsA)  Documentation of CASPAR criteria score greater than 3 based on chart notes . Skin psoriasis: present – two points, OR previously present by history – one point, OR a family history of psoriasis, if the patient is not affected – one point . Nail lesions (onycholysis, pitting): one point . Dactylitis (present or past, documented by a rheumatologist): one point . Negative rheumatoid factor (RF): one point . Juxtaarticular bone formation on radiographs (distinct from osteophytes): one point Appropriate Plaque psoriasis Treatment  Has failed at least two systemic therapy with minimum of 12 weeks trial: methotrexate, Regimen & Other cyclosporine, isotretinoin OR phototherapy (UVB, PUVA) AND Criteria:  Has failed minimum 12 weeks on Humira AND minimum 12 weeks on Enbrel  QL – Initial (one time only)– 0.5 to 1 ml per 28 day supply (based on patient weight)  QL – Continuation – 1 ml per 84 day supply (based on patient weight) Psoriatic Arthritis (PsA)  Failure of at least 12 weeks with methotrexate, or if unable to tolerate or contraindicated to methotrexate, another disease modifying antirheumatic drug (sulfasalazine, cyclosporine, leflunomide) AND  Has failed minimum 12 weeks on Humira AND minimum 12 weeks on Enbrel  QL – Initial (one time only)– 0.5 to 1 ml per 28 day supply  QL – Continuation – 0.5 to 1 ml per 84 day supply Crohn’s Disease

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 Has failed at least two oral treatment for minimum of 12 weeks trial 12 weeks: corticosteroids, azathioprine, cyclosporine, 6-mercaptopurine, methotrexate, sulfasalazine, balsalazide, tacrolimus, mesalamine AND  Has failed minimum 12 weeks on Humira AND Cimzia  QL – 1 ml per 56 day supply

Reauthorization: documentation of treatment success and a clinically significant response to therapy Exclusion  Ustekinumab should not be given in combination with a tumor necrosis factor (TNF) Criteria: antagonist (eg, adalimumab, certolizumab pegol, etanercept, golimumab, infliximab), anakinra, etc  Use in the management of psoriatic arthritis without plaque psoriasis  Use in the management of multiple sclerosis  Positive tuberculosis; active hepatitis B, hepatitis C or herpes zoster infection Age Restriction:  18 years of age or older

Prescriber  Plaque psoriasis: prescribed by or in consultation with a dermatologist Restrictions:  Crohn’s Disease: prescribed by or in consultation with a GI specialist Coverage  Initial approval: 4 months initiation, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

295

POLICY NAME: STRENSIQ Affected Medications: STRENSIQ (asfotase alfa)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Diagnosis of perinatal/infantile hypophosphatasia OR Information:  Diagnosis of juvenile-onset hypophosphatasia  Age of diagnosis Appropriate Treatment Perinatal/Infantile-Onset HPP Regimen & Other Criteria:  QL – 9 mg/ kg per week Juvenile-Onset HPP  QL – 6 mg/ kg per week Subsequent approval: documentation of treatment responsiveness to therapy. Exclusion Criteria:  Adult-onset hypophosphatasia Age Restriction: Prescriber Restrictions:

Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

296

POLICY NAME: SUBCUTANEOUS IMMUNE GLOBULIN Affected Medications: Cuvitru, Gamunex-C, Hizentra, Hyqvia

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  Decent serum immunoglobulin G (IgG) trough concentration AND Information:  Monthly IVIG dose AND  Patient weight

Primary Immunodeficiency (PID)  Type of immunodeficiency AND  Documentation of at least 3 months of IVIG therapy

Appropriate  Meets all criteria for IVIG approval Treatment  Exceptions may be given for patients without prior IV or SC immune Regimen & Other globulin use

Criteria: Primary Immunodeficiency (PID)  Approve for patients if they have previously received immune globulin given intravenously (IV) (eg, Carimune, Privigen, etc.) or immune globulin given subcutaneously (SC)  Approve for patients who are continuing subcutaneous immune globulin therapy (SCIG)

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (Hizentra only)  Used as initial maintenance therapy for prevention of disease relapses after treatment and stabilization with intravenous immunoglobulin (IVIG) OR  Used for re-initiation of maintenance therapy after experiencing a relapse and requiring re-induction therapy with IVIG

Renewal Criteria  Documentation of treatment success AND  Need for continued therapy

Exclusion Criteria:  IgA deficiency with antibodies to IgA  History of hypersensitivity to immune globulin or product components  Hyperprolinemia type I or II Age Restriction:  PID: 2 years of age and older  CIDP (Hizentra only): 18 years of age and older

Prescriber/Site of  PID: prescribed by or in consultation with an immunologist Care Restrictions:  CIDP: prescribed by a neurologist or rheumatologist with CIPD expertise

Coverage Duration:  Approval = 12 months, unless otherwise specified.

297

POLICY NAME: SUBLOCADE Affected Medications: SUBLOCADE (Buprenorphine extended release injection)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical  Documentation that member is part of a comprehensive management program that Information: includes psychosocial support AND  Must have initiated treatment with a transmucosal buprenorphine-containing product, followed by dose adjustment for a minimum of 7 days. Appropriate  Initial approval requires documented failure with a minimum 1-month trial with each Treatment generic oral buprenorphine product (buprenorphine sublingual tablets, Regimen & Other buprenorphine/naloxone sublingual tablets, buprenorphine/naloxone sublingual film) Criteria: AND  Diagnosed moderate to severe Opioid Use Disorder (OUD) as described by the following DSM-5 Criteria for OUD o Problematic pattern of opioid use leading to clinically significant impairment or distress is manifested by four or more of the following within a 12 month period:  Opioids are often taken in large amounts or over a longer period of time than was intended  Persistent desire or unsuccessful efforts to cut down or control opioid use  Great deal of time is spent in activities necessary to obtain, use, or recover from its effects  Craving or strong desire/urge to use opioids  Recurrent opioid use resulting in a failure to fulfill major role obligations at work, school, or home  Continued opioid use despite having persistent or recurrent social or interpersonal problems caused by or exacerbated by the effects of opioids  Important social, occupational, or recreational activities are given up or reduced because of opioid use  Recurrent opioid use in situations in which it is physically hazardous  Continued opioid use despite knowledge of having a persistent or recurrent physical or psychological problem that is likely to have been caused or exacerbated by the substance  Tolerance or withdrawal o Experiencing these symptoms while taking opioids solely under appropriate medical supervision does not meet these criteria for OUD

 Reauthorization: Subsequent approvals require documentation of treatment success

298

Exclusion  Moderate to Severe Hepatic Impairment (Child-Pugh class B or C) Criteria:

Age Restriction:  Age greater than or equal to 18 years Prescriber  SUBLOCADE is available only through a restricted program called the SUBLOCADE REMS Restrictions: Program  Physician (must meet DATA 2000 requirements and has been assigned a unique identification number specific to the prescription of medication assisted therapy (DEA-X)

Coverage  Approval Duration: 12 months Duration:

299

POLICY NAME: SUCRAID Affected Medications: SUCRAID (Sacrosidase)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical  Documentation of confirmed congenital sucrose-isomaltase deficiency, diagnosed by Information: duodenal or jejunal mucosal biopsy assayed for lactase, sucrose, isomaltase, and maltase.  Reauthorization: requires documentation of treatment success (fewer stools, lower number of symptoms) Appropriate Symptoms of congenital sucrose-isomaltase deficiency include: Treatment Regimen & Other  Diarrhea Criteria:  Abdominal pain or cramping  Bloating  Gas  Loose Stools  Abdominal pain or cramping  Bloating  Nausea  Vomiting Exclusion Criteria:  Known hypersensitivity to years, yeast products, glycerin (glycerol), or papain Age Restriction:  5 months or older Prescriber Restrictions: Coverage Duration:  Initial approval: 1 month, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

300

POLICY NAME: SUPPRELIN Affected Medications: SUPPRELIN LA (histrelin acetate)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  Gender Dysphoria

Required Medical Central Precocious puberty Information:  Documentation of central precocious puberty (CPP) confirmed by basal luteinizing hormone (LH), follicle-stimulating hormone (FSH), and either estradiol or testosterone concentrations Gender Dysphoria  Documentation of baseline estradiol and testosterone levels to confirm onset of puberty  Documentation of current Tanner stage 2 or greater  Documentation of planned psychological care/visits during puberty suppression therapy Appropriate QL: 50 mg implant every 12 months Treatment Regimen & Other Gender dysphoria Criteria:  Comprehensive mental health evaluation should be provided in accordance with most current version of the World Professional Association for Transgender Health (WPATH) Standards of Care  Therapy should be started at onset of puberty, but no earlier than Tanner stages 2-3  Fulfill eligibility and readiness criteria from Journal of Clinical Endocrinology and Metabolism (JCEM) guidelines

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria: Age Restriction:  equal or greater than 2 years old and less than 18 years old Prescriber  Prescribed by or in consultation with endocrinologist Restrictions:  Gender Dysphoria: Diagnosis made and prescribed by a mental health specialist with experience in gender dysphoria Coverage Duration:  Approval: 12 months, unless otherwise specified

301

POLICY NAME: SUTENT Affected Medications: SUTENT (sunitinib malate)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, disease staging, all prior therapies used, Information: and prescribed treatment regimen  Documentation that Sutent is being used as a NCCN 2A level of evidence regimen

Appropriate Treatment  Appropriate dose adjustments with strong CYP3A4 inducers or inhibitors Regimen & Other  Reauthorization requires documentation of treatment success Criteria: Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber Restrictions:  Oncologist Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

302

POLICY NAME: SYLVANT Affected Medications: SYLVANT (siltuximab)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  The diagnosis was confirmed by biopsy of lymph gland Information:  HIV and human herpes virus-I (HHV-8) negative  Hematology laboratory tests prior to each dose for the first 12 months and every 3 dosing cycles thereafter

Appropriate  Before first treatment: ANC greater than or equal to 1.0 x109 /L, Platelet count Treatment greater than or equal to 75 x109 /L, Hemoglobin less than 17 g/dL Regimen & Other  Retreatment: ANC greater than or equal to 1.0 x109 /L, Platelet count greater than or Criteria: equal to 50 x109 /L, Hemoglobin less than 17 g/dL

Exclusion Criteria: Age Restriction:  Age >18 years Prescriber  Prescribed by an Oncologist Restrictions: Coverage Duration:  Initial Approval: 3 weeks , unless otherwise specified  Subsequent Approval: 3 months until treatment failure (disease progression, increase in symptoms, decrease in performance status, radiologic progression) or toxicity

303

POLICY NAME: SYMDEKO Affected Medications: SYMDEKO (tezacaftor/ivacaftor tablets)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required  Documentation of cystic fibrosis (CF) diagnosis. Medical  Documentation of Homozygous for the F508 del mutation by FDA-cleared CF mutation Information: test on both alleles of the CFTR gene or who have at least one mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that is responsive to tezacaftor/ivacaftor based on in vitro data and/or clinical evidence.  Baseline forced expiratory volume in 1 second (FEV1)  Documentation of baseline and follow-up liver function tests  Documentation of baseline and follow-up eye exam (for pediatric patients) Appropriate  Reauthorization requires documentation of improvement in FEV1 from baseline, Treatment documentation of follow-up liver function tests AND follow-up eye exam (for pediatric Regimen & patients) Other Criteria:

Exclusion  Concurrent use of strong CYP3A inducers: rifampin, rifabutin, phenobarbital, Criteria: carbamazepine, phenytoin, and St. John’s wort

Age Restriction:  12 years of age and older Prescriber  Prescribed by or in consultation with a pulmonologist or provider who specializes in CF Restrictions:

Coverage  Initial approval: 6 months, unless otherwise specified Duration:  Reauthorization: 12 months unless otherwise specified

304

POLICY NAME: SYMLIN Affected Medications: SYMLINPEN (pramlintide acetate)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.  patient has type 1 or 2 diabetes mellitus. Required Medical Information: Appropriate  If patient received Symlin in previous 3 months, patient demonstrated an expected Treatment reduction in HbA1c since starting Symlin therapy. OR Regimen & Other  The patient has inadequate glycemic control (HbA1c > 7%). AND Criteria:  Patient is currently receiving optimal mealtime insulin therapy.

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Severe hypoglycemia that required assistance during the past 6 months.  Gastroparesis.  Patient requires drug therapy to stimulate gastrointestinal motility.  Hypoglycemia unawareness (i.e., inability to detect and act upon the signs or symptoms of hypoglycemia).  HbA1c level greater than 9 percent.  Weight loss treatment. Age Restriction: Prescriber Restrictions: Coverage Duration:  Approval: 12 months, unless otherwise noted.

305

POLICY NAME: SYNAGIS Affected Medications: SYNAGIS (palivizumab)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical Documentation of one of the following conditions: Information:  1. Congenital heart disease (CHD):  With cardiac transplantation, cardiac bypass, or extra-corporeal membrane oxygenation  That is hemodynamically significant (eg acyanotic heart disease, congestive heart failure, or moderate to severe pulmonary hypertension)

 2. Chronic lung disease (CLD) of prematurity:  In the first year of life, born less than 32 weeks gestation and requiring greater that 21% oxygen for at least the first 28 days of life  In the second year of life necessitating continued medical support within the 6 month period prior to RSV season (eg corticosteroids, diuretics, supplemental oxygen)

 3. Cystic Fibrosis and:  Clinical evidence of CLD and/or nutritional compromise  Severe lung disease (eg previous hospitalization for pulmonary exacerbation in the first year of life or abnormalities on chest radiography or computed tomography that persist when stable)  A weight for length less than the 10th percentile

 4. Congenital airway abnormality or neuromuscular condition (not cystic fibrosis) that impairs the ability to clear airway secretions

 5. Premature infants without above conditions

Appropriate Prevention of serious lower respiratory tract disease caused by respiratory syncytial virus Treatment (RSV) Regimen & Other  The first dose of Synagis should be administered prior to commencement of the RSV Criteria: season  Remaining doses should be administered monthly throughout the RSV season (Exception: dose administration should occur immediately post cardiopulmonary bypass surgery, even if dose is administered earlier than a month from previous dose, then dosing schedule should resume monthly)  No more than 5 monthly doses During the RSV season, November 1 through March 31  Discontinue prophylaxis therapy if hospitalized for RSV

306

Exclusion Criteria:  For use in the treatment of RSV disease

Age Restriction: Refer to numbered conditions above in “Required Medical Information”:  1a. Less than 2 years of age  1b. Less than 1 year of age  2a. Less than 1 year of age; Gestational Age less than 32 weeks  2b. Less than 2 years of age; Gestational Age less than 32 weeks  3a. Less than 1 year of age  3c. Less than 2 years of age  3d. Less than 2 years of age  4. Less than 1 year of age  5. Less than 1 year of age; Gestational Age less than 29 weeks Prescriber Restrictions:

Coverage Approval: Duration:  5 months (November 1 through March 31) 5 monthly doses , unless otherwise specified  1 month for off-season when RSV activity greater than or equal to 10% for the region according to the CDC 1 monthly dose , unless otherwise specified

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POLICY NAME: TACROLIMUS OINTMENT Affected Medications: TACROLIMUS OINTMENT (0.1%, 0.3%)

Covered Uses:  All FDA approved indications not otherwise excluded by plan design. Required Medical  Atopic dermatitis not meeting the following criteria is considered a below the line (non- Information: funded) diagnosis per Oregon Health Authority (OHA):  Meet ALL of the following: . Clinical documentation of SEVERE inflammatory skin disease defined as having functional impairment (e.g. inability to use hands or feet for activities of daily living, or significant facial involvement preventing normal social interaction) despite current treatment AND . Body Surface Area (BSA) of at least 10% OR hand, foot or mucous membrane involvement Appropriate  Documented failure with other topical prescription treatment for atopic dermatitis Treatment (prescription strength topical corticosteroids and emollients) Regimen & Other Criteria:  Reauthorization will require documentation of disease responsiveness to therapy: Body Surface Area (BSA) reduction from baseline Exclusion  Atopic dermatitis not meeting the above criteria is considered a below the line (non- Criteria: funded) diagnosis per Oregon Health Authority (OHA). Please refer to OHA GUIDELINE NOTE 21, SEVERE INFLAMMATORY SKIN DISEASE Line 430. Age Restriction:  Tacrolimus ointment 0.03% for children 2-15 years old  Tacrolimus ointment 0.03% and 0.1% for adults

Prescriber  Prescribed by, or in consultation with, a specialist, (e.g., dermatologist, allergist or Restrictions: immunologist)

Coverage  Initial approval: 3 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: TAFAMIDIS Affected Medications: VYNDAQEL, VYNDAMAX

Covered Uses:  All FDA approved indications not otherwise excluded by plan design. Required Medical  Diagnosis of Heart Failure with NYHA Class I to II symptoms AND Information:  Documentation of treatment failure with diuretics AND  Documentation of amyloid deposits from biopsy tissue that is composed of wild-type or mutant transthyretin (confirmed by immunohistochemistry, scintigraphy, or mass spectrometry) Appropriate  Maximum dosing Treatment o Vyndaqel 80 mg (four 20 mg capsules) Regimen & Other o Vyndamax 61 mg (one 61 mg capsule) Criteria:  Reauthorization: Documentation of treatment success Exclusion Criteria:  Heart Failure NYHA Class III/IV  Presence of primary (light chain) amyloidosis  Prior liver or heart transplant  Implanted cardiac mechanical assist device (i.e. pacemaker) Age Restriction:  18 years and older

Prescriber  Physicians with experience in treating amyloidosis Restrictions:

Coverage Duration:  Initial approval: 6 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: TAKHZYRO Affected Medications: TAKHZYRO (lanadelumab)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Hereditary angiogenesis (HAE) official diagnosis documented in member’s chart AND Information:  Treatment with acute therapy (i.e. Kalbitor, Firazyr, Berinert or Ruconest) did not result in meaningful outcomes such as decreased severity of attacks, avoidance of hospitalization, etc AND  Laboratory confirmed diagnosis . Low levels of functional C1-INH protein (below 67% or as defined by the laboratory reference values); OR . Lower than normal serum C4 levels (< 14 mg/dL or as defined by the laboratory reference values) AND lower than normal C1-INH level (< 19.9 mg/dL or as defined by the laboratory reference values)  Failure of, intolerance to or unable to receive treatment with attenuated androgens (e.g, danazol, stanozolol); AND  All other causes of acquired angioedema (e.g., medications, auto-immune diseases) have been excluded; AND  At least ONE of the following criteria . A history of one or more severe attack(s) per month (defined as an attack that significantly interrupts daily activities despite short-term treatment) . Disabling symptoms for at least 5 days per month . Laryngeal edema; AND  At least ONE of the following criteria . A history of self-limiting, non-inflammatory subcutaneous angioedema, without uticaria, which is recurrent and lasts >12 hours . Self-limiting, recurrent abdominal pain without a clear organic cause lasting >6 hours . A history of laryngeal edema

Appropriate  Dosing: 300 mg SC every 2 weeks Treatment  If patient is dosing every 2 weeks and has been attack free for 6 months, dosing will be Regimen & Other reduced to every 4 weeks Criteria:  Reauthorization requires documented clinical treatment success Exclusion Criteria:  Concurrent use of any long-term prophylactic therapy for HAE (such as Haegarda, Cinryze)  Concomitant diagnosis of another form of chronic, recurrent angioedema, such as acquired angioedema, idiopathic angioedema, or recurrent angioedema associated with urticaria.  Any of the following liver function test abnormalities o Alanine aminotransferase (ALT) > 3x upper limit of normal OR o Aspartate aminotransferase (AST) > 3x upper limit of normal OR

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o Total bilirubin > 2x upper limit of normal (unless the bilirubin elevation is a result of Gilbert's syndrome) Age Restriction:  Must be at least 12 years old Prescriber  Must be prescribed by, or in consultation with, an allergist/immunologist or physician Restrictions: that specializes in HAE or related disorders

Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

311

POLICY NAME: TARCEVA Affected Medications: TARCEVA (erlotinib)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, disease staging, all prior therapies used, and Information: prescribed treatment regimen  Documentation that Tarceva is being used as a NCCN 2A level of evidence regimen  Documentation of epidermal growth factor receptor (EGFR) exon 19 deletions or exon 21 (L858R) substitution mutations as detected by an FDA-approved test

Appropriate  Documentation of dose adjustment with strong CYP3A4 inducers or inhibitors Treatment  Reauthorization requires documentation of treatment success Regimen & Other Criteria: Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber  Oncologist Restrictions:

Coverage Duration:  Initial approval: 3 months, (2 week initial partial fill), unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: TARGRETIN Affected Medications: TARGRETIN (Bexarotene)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design,  Mycosis fungoides,  Sezary Syndrome (Capsules only),  Adult T-cell leukemia/lymphoma (Gel only), and  Primary cutaneous B-cell lymphoma (Gel only) Required Medical Targretin Capsule: Information:  Patient must meet one of following criteria: received prior systemic therapy for CTCL OR advanced-stage MF (stage IIB, III or IV) or SS OR  early-stage MF (stage IA, IB or IIA) with folliculotropic/large cell transformation OR  early-stage MF (stage IA, IB or IIA) refractory to skin directed therapy. Targretin Gel:  Patient must meet one of following criteria for CTCL: early-stage MF (stage IA, IB, or IIA) OR  stage IIB or III MF in combination with systemic therapy. Appropriate  Patient has been instructed on the importance and proper utilization of Treatment appropriate contraceptive methods. Regimen & Other Criteria: Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Pregnancy. Age Restriction: Prescriber Restrictions: Coverage Duration:  Initial approval: 2 weeks, unless otherwise specified  Subsequent approval: 12 months, unless otherwise specified.

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POLICY NAME: TASIGNA Affected Medications: TASIGNA (nilotinib)

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, all prior therapies used, and prescribed Information: treatment regimen  Documentation that Tasigna is being used as a NCCN 2A level of evidence regimen  Documentation Philadelphia chromosome-positive mutation, BCR-ABL1 positive mutation,  Documentation of smoking abstinence discussion of risks and acknowledgement from patient of anticipated nicotine reduction as able  For patients with low risk score, documented clinical failure with Imatinib

Appropriate  Documentation of dose adjustment with strong CYP3A4 inhibitors Treatment  Avoidance of strong CYP3A4 inducers Regimen & Other  Reauthorization requires documentation of treatment success (as applicable, BCR- Criteria: ABL1 transcript levels, cytogenetic response)

Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater  Hypokalemia, hypomagnesemia, or long QT syndrome

Age Restriction:

Prescriber  Oncologist Restrictions:

Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: TAVALISSE Affected Medications: Tavalisse (fostamatinib)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design

Required Medical  Complete blood count with differential and platelet count Information:  Liver function test

Thrombocytopenia in patients with Chronic Immune thrombocytopenia (ITP)  All therapies tried/failed  Documentation of splenectomy status

Appropriate Thrombocytopenia in patients with Chronic ITP Treatment  Documentation of platelet count less than 20,000/mcl and clinical bleeding Regimen & Other  Must fail at least 2 therapies for ITP – a thrombopoietin receptor agonist and another Criteria: including corticosteroids, immunoglobulins, immunosuppression, or splenectomy  Continuation of therapy requires response to treatment with platelet count of at least 50,000/mcl without significant liver function abnormalities  Discontinue therapy after 12 weeks if platelet count does not increase to a level sufficient to avoid clinically important bleeding

Exclusion Criteria:

Age Restriction:  18 years of age and older Prescriber  Prescribed by or consultation with hematologist Restrictions:

Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: TECENTRIQ Affected Medications: TECENTRIQ (atezolizumab)

Covered Uses:  NCCN (National Comprehensive Cancer Network) indications with evidence level of 2A or better For Triple Negative Breast Cancer (TNBC)  Used in combination with paclitaxel protein-bound (Abraxane) for the treatment of adult patients with unresectable locally advanced or metastatic TNBC whose tumors express PD-L1 (PD-L1 stained tumor-infiltrating immune cells of any intensity covering greater than or equal to 1% of the tumor area), as determined by an FDA approved test. Required  Documentation of performance status, disease staging, all prior therapies used, and Medical anticipated treatment course Information:

Appropriate Appropriate Treatment for Triple Negative Breast Cancer Treatment  Approved for first line therapy in combination with with Abraxane Regimen &  May be approved after first line if patient has not failed a taxane and provider wants to Other Criteria: combine with Abraxane.  Not approved for later use if patient has previously received Abraxane (or any taxane) and failed treatment.

Reauthorization: documentation of disease responsiveness to therapy Exclusion  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Criteria:

Age Restriction:

Prescriber  Oncologist Restrictions:

Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: TECFIDERA Affected Medications: Tecfidera (dimethyl fumarate)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design Required Medical  Diagnosis of relapsing forms of multiple sclerosis confirmed with MRI Information:  CBC with lymphocyte count (within 6 months) before initiating treatment, then annually and as clinically indicated

Appropriate Treatment  Initial dose of 120mg BID 7 days, then increasing to 240mg BID thereafter Regimen & Other  Hold therapy for four weeks if lymphocyte count is less than 500/mm3 for Criteria: greater than 6 months  No concurrent use of medications indicated for the treatment of relapsing- remitting multiple sclerosis  Not approved for primary and secondary progressive multiple sclerosis

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Pre-existing low lymphocyte counts (less than 500/mm3)

Age Restriction:

Prescriber Restrictions:  Prescribed by or after consultation with a neurologist or an MS specialist. Coverage Duration:  Approval: 12 months, unless otherwise noted.

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POLICY NAME: THALOMID Affected Medications: THALOMID (thalidomide)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.  NCCN indications with evidence level of 2A or higher Required Medical  Documentation of performance status, all prior therapies used, and prescribed Information: treatment regimen  Documented consideration of risk/benefit ratio for anticoagulation therapy  Baseline WBC count

Multiple Myeloma (MM):  Used in combination with dexamethasone in newly diagnosed MM

Erythema nodosum leprosum (ENL):  Thalomid is used for maintenance therapy or as part of a combination regimen in a patient with moderate to severe neuritis for acute therapy

Active/symptomatic myeloma or progressive solitary plasmacytoma:  Thalomid is warranted in any of the following settings: o Thalomid is used in combination with dexamethasone or both melphalan and prednisone as primary induction therapy o Thalomid is used as maintenance monotherapy for patients responding to primary induction therapy or for patients with stable or responsive disease following stem cell transplant, o Thalomid is used as salvage or palliative therapy.  Use for treatment of myelofibrosis with myeloid metaplasia.

Systemic light chain amyloidosis:  Thalomid is used as primary treatment in combination with dexamethasone  Documented tried/failed/contraindicated to alternative therapies

Waldenstrom’s macroglobulinemia:  Patient must not be a candidate for autologous hematopoietic cell transplantation  Thalomid is used as monotherapy and NOT recommended in combination with rituximab outside of clinical trials due to toxicity.

 Reauthorization: documentation of disease responsiveness to therapy Appropriate  All patients are monitored for signs and symptoms of thromboembolism Treatment  Patients of child-bearing potential are instructed on the importance and proper Regimen & Other utilization of appropriate contraceptive methods for Thalomid use. Criteria:

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Exclusion Criteria:  Pregnancy  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater  ANC less than 750/mm3 Age Restriction:  12 years of age or older Prescriber  Prescribed by or in consultation with an oncologist and must be registered with Restrictions: S.T.E.P.S program

Coverage  Initial approval: 3 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: THYMOGLOBULIN Affected Medications: THYMOGLOBULIN (antithymocyte globulin - rabbit derived)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design o Renal transplant acute rejection treatment and induction therapy  Off-label uses: o Heart transplant o Intestinal and multivisceral transplantation o Lung transplant o Chronic graft-versus-host disease prevention

Required  For prophylaxis: Patient must be considered high risk for acute rejection or delayed graft Medical function based on one or more of either the following donor/recipient risk factors: donor cold Information: ischemia for more than 24 hours, donor age older than 50 years old, donor without a heartbeat, donor with ATN, donor requiring high-dose inotropic support. Recipient risk factors include: repeated transplantation, panel-reactive antibody value exceeding 20% before transplant, black race, and one or more HLA antigen mismatches with the donor.

Appropriate Treatment of acute renal graft rejection – **No PA required for this diagnosis** Treatment • Prophylaxis: 1.5 mg/kg of body weight administered daily for 4 to 7 days. Regimen & • Clinical rationale for avoiding Simulect (Basiliximab) in prophylaxis Other Criteria:

Exclusion  Active acute or chronic infections that contraindicates any additional immunosuppression Criteria:

Age Restriction:

Prescriber  Physicians experienced in immunosuppressive therapy for the management of renal transplant Restrictions: patients.

Coverage  Initial approval: 1 Month, unless otherwise specified Duration:  Reauthorization: 1 Month, unless otherwise specified

320

POLICY NAME: TEGSEDI Affected Medications: TEGSEDI (inotersen sodium)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical  Documented pathogenic mutation in transthyretin (TTR; e.g. V30M mutation) Information:  Diagnosis of hereditary transthyretin (hATTR) amyloidosis with polyneuropathy  Documentation of baseline Neuropathy Impairment Score (NIS) of 10 to 130  Documented amyloid deposits determined on biopsy  Presence of clinical signs and symptoms of disease (e.g. peripheral/autonomic neuropathy, motor disability, cardiovascular dysfunction, and renal dysfunction)  Complete blood count, basic metabolic panel prior to start

Reauthorization:  Documentation of baseline Neuropathy Impairment Score (NIS) of 10 and 130 AND  Documentation of the patient experiencing positive clinical response to inotersen (e.g. improved neurologic impairment, motor function, cardiac function, quality of life assessment, serum TTR levels, etc) Appropriate Hereditary transthyretin-mediated (hATTR) amyloidosis Treatment  Tegsedi 284 mg injected subcutaneously once weekly Regimen & Other  During treatment, monitor platelets weekly during treatment if values are 75 x 109/L or Criteria: greater, and more frequently if values are less than 75 x 109/L  During treatment, monitor kidney function every 2 weeks

 Do not initiate if urinary protein to creatinine ratio (UPCR) is 1000 mg/g or higher Exclusion Criteria:  Platelet count less than 100 x 109/L prior to start of Tegsedi Age Restriction:  Adults 18 years and older Prescriber  Physicians experienced in the management of amyloidosis Restrictions:

Coverage  Initial approval: 4 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

321

POLICY NAME: TOBRAMYCIN INHALATION Affected Medications: TOBI PODHALER ® (tobramycin inhalation powder), tobramycin nebulized solution KITABIS PAK (tobramycin), BETHKIS (tobramycin), Tobi (tobramycin)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Medical  Diagnosis of Cystic Fibrosis (phenotyping not required). Information:  Culture and sensitivity report confirming presence of pseudomonas aeruginosa in the lungs  For Tobi Podhaler: Baseline forced expiratory volume in 1 second (FEV1) equal to or greater than 25% but equal to or less than 80%  For Bethkis: Baseline FEV1 equal to or greater than 40% but equal to or less than 80%  For Kitabis Pak: Baseline FEV1 equal to or greater than 25% but equal to or less than 75% Appropriate Treatment  Documentation of failure with nebulized tobramycin or clinical rationale for Regimen & Other avoidance Criteria:  Use is limited to a 28 days on and 28 days off regimen  Reauthorization requires documentation of improved respiratory symptoms and need for long-term use

Exclusion Criteria:  For Tobi Podhaler: Baseline FEV1 less than 25% or greater than 80%  For Bethkis: Baseline FEV1 less than 40% or greater than 80%  For Kitabis Pak: Baseline FEV1 less than 25% or greater than 75%

Age Restriction:  Age greater than or equal to 6 years Prescriber Restrictions:

Coverage Duration:  12 months, unless otherwise specified

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POLICY NAME: TESTOPEL AND TRANSDERMAL TESTOSTERONE Affected Medications: Testopel (testosterone pellets), Testosterone gel

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required Medical  All therapies tried/failed for indicated diagnosis Information:  Confirmed low testosterone level (total testosterone less than 300 ng/dl or morning free or bioavailable testosterone less than 5 ng/dL) or absence of endogenous testosterone  Testopel: dosage (in milligrams) or number of pellets to be administered and frequency  If age greater 65 years and older:  Yearly evaluation of need is completed discussing need for hormone replacement therapy.  Yearly documentation that provider has educated patient on risks of hormone replacement (heart attack, stroke)  Yearly documentation that provider has discussed limited efficacy and safety for hormone replacement in patients experiencing age related decrease in testosterone levels

Appropriate STEP 1 MEDICATIONS: Treatment  Testosterone injections Regimen & Other STEP 2 MEDICATIONS: Criteria:  Testosterone transdermal  Initial approval requires documented failure, intolerance, or clinical rationale for avoidance of the testosterone injections STEP 3 MEDICATIONS:  Testopel (Medical only)  Only approved if documented failure to generic transdermal testosterone AND testosterone injections  Maximum of 450 mg per treatment Reauthorization Criteria:  Documentation of recent testosterone level while on replacement therapy within normal limits Exclusion Criteria: Age Restriction:

Prescriber Restrictions:

Coverage Duration:  Testopel: Maximum of 4 treatments in 12 months, unless otherwise specified  Transdermal testosterone: 12 months, unless otherwise specified

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POLICY NAME: TRACLEER Affected Medications: TRACLEER (Bosentan)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical Pulmonary arterial hypertension (PAH) WHO Group 1 Information:  Documentation of PAH confirmed by right-heart catheterization  Etiology of PAH (idiopathic, heritable, associated with connective tissue disease, or associated with congenital heart disease with left-to-right shunts)  NYHA/WHO Functional Classification II, III or IV  Liver Function Tests within normal limits prior to initiation  Documentation of Acute Vasoreactivity Testing (positive result requires trial/failure to calcium channel blocker) Appropriate  Supportive care should be considered as first-line (anticoagulants, diuretics, oxygen, Treatment digoxin) Regimen & Other  Documentation of trial with at least 1 PDE5 inhibitor (unless contraindicated) OR Criteria: patient at high risk necessitating endothelin receptor antagonist.  Consider Tracleer as second-line therapy in patients with WHO-FC IV symptoms  Not recommended for patients with PAH secondary to heart failure with severe systolic dysfunction  Not recommended for patients with moderate to severe liver impairment  Subsequent approvals require documentation of treatment success such as improved walking distance or improvements in functional class Exclusion Criteria:  Pregnancy  Concomitant use with glyburide and cyclosporine Age Restriction:

Prescriber  Prescribed by or in consultation with a cardiologist or a pulmonologist Restrictions: Coverage Duration:  12 months, unless otherwise specified

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POLICY NAME: TRIPTROELIN Affected Medications: TRELSTAR, TRIPTODUR (triptorelin)

Covered Uses:  NCCN indications with evidence level of 2A or higher  All FDA approved indications not otherwise excluded by benefit design. (For non- cancer use only) Required Medical Prostate cancer, must meet one of the following: Information:  Documentation of performance status, disease staging, all prior therapies used, and prescribed treatment regimen  Documentation that Trelstar is being used as NCCN 2A level of evidence regimen

Central Precocious Puberty  documentation of central precocious puberty (CPP) confirmed by basal luteinizing hormone (LH), follicle-stimulating hormone (FSH), and either estradiol or testosterone concentrations  documented clinical rationale for avoiding Lupron depot-ped and Supprelin LA Appropriate  Triptroelin QL: 22.5 mg every 6 months Treatment Regimen & Other Reauthorization will require documentation of treatment success and a clinically Criteria: significant response to therapy

Exclusion Criteria:  Use as neoadjuvant ADT for radical prostatectomy Age Restriction: Prescriber  Oncology: prescribed by or in consultation with Oncologist Restrictions:  CPP: prescribed by or in consultation with pediatric endocrinologist Coverage Duration:  Oncology initial approval: 4 months, unless otherwise specified  CPP Approval/Oncology reauthorization: 12 months, unless otherwise specified

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POLICY NAME: TROGARZO Affected Medications: TROGARZO (ibalizumab-uiyk/IV infusion)

Covered Uses: ● All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Documentation of all prior therapies used Information:  Documentation of active antiretroviral therapy for at least 6 months  Documentation of multidrug resistant HIV-1 with resistance to at least one antiretroviral medication from each of the following classes: Nucleoside Reverse Trancriptase Inhibitors (NRTIs), Non-Nucleoside Reverse Transcriptase Inhibitors, and Protease Inhibitors (PIs).  Failure with current regimen or not on current antiretroviral therapy and failure with most recent regimen (viral load greater than 1,000 copies/mL) Appropriate ● Loading dose 2000mg Treatment ● Maintenance dose 800mg every 2 weeks Regimen & Other ● Initial reauthorization will require documentation of greater than or equal to a 0.5 Criteria: log10 reduction in viral load ● Reauthorization: Continued authorization will require undetectable viral load Exclusion Criteria: Age Restriction: ● 18 years and older Prescriber  Infectious Disease or specialist in HIV treatment Restrictions:

Coverage ● Initial approval: 3 months, unless otherwise specified Duration: ● Reauthorization 12 months, unless otherwise specified

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POLICY NAME: TYKERB Affected Medications: Tykerb (lapatinib)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  NCCN indications with evidence level of 2A or higher Required Medical  Documentation of performance status, disease staging, all prior therapies used, Information: and prescribed treatment regimen  Documentation that Tykerb is being used as a NCCN 2A level of evidence regimen  If applicable, documentation of HER2-positive cancer type Appropriate Treatment  Avoidance of strong CYP3A4 inducers or inhibitors Regimen & Other  Reauthorization requires documentation of treatment success Criteria: Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction:

Prescriber Restrictions:  Oncologist

Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

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POLICY NAME: TYSABRI Affected Medications: TYSABRI (natalizumab)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical Adults with Multiple Sclerosis (MS) Information:  Documentation of diagnosis relapsing forms of multiple sclerosis confirmed with MRI  Documentation of prior treatments with immunosuppressant and screening for seropositivity for anti-JC virus antibodies prior to Tysabri therapy Adults with Crohn disease (CD).  Patient has moderately to severely active CD with evidence of inflammation (eg, elevated C-reactive protein) Appropriate Adults with MS Treatment  Patient has a relapsing form of MS and has had an inadequate response to, or is unable to Regimen & Other tolerate, therapy with at least two of the following MS medications: interferon beta-1a Criteria: (Avonex, Rebif), interferon beta-1b (Betaseron, Extavia), glatiramer 20mg, glatiramer 40mg, glatopa 20mg, or fingolimod (Gilenya).  Exceptions to having tried an interferon beta-1a or -1b product (Avonex, Betaseron, Extavia, or Rebif) can be made if the patient has depression or a mood disorder.  In these cases, the patient should try glatiramer 20mg, glatiramer 40mg, glatopa 20mg, or fingolimod (Gilenya), but is not required to try an interferon beta-1a or -1b.  No concurrent use of medications indicated for the treatment of relapsing-remitting multiple sclerosis  Not approved for primary and secondary progressive multiple sclerosis  Reauthorization for patients with baseline positive JCV: documentation of response to therapy and testing for anti-JC virus antibodies after one year of natalizumab therapy

Adults with CD.  Patient has moderately to severely active CD with evidence of inflammation (eg, elevated C-reactive protein)  Documented clinical failure with at least 12 weeks of ONE oral immunosuppressive therapy such as corticosteroids, azathioprine, 6-mercaptopurine, or methotrexate AND  Documented clinical failure with at least 12 weeks of TWO TNF-inhibitors such as adalimumab, certolizumab pegol, or infliximab  Exception to the CD criteria of treatment with corticosteroids (systemic) are allowed if steroids are contraindicated or not desired, then azathioprine, 6-mercaptopurine, or methotrexate must be tried if they are not contraindicated. Exclusion  Concurrent use with immunosuppressants (eg, 6-mercaptopurine, azathioprine, Criteria: cyclosporine, methotrexate) or tumor necrosis factor (TNF) alfa inhibitors (eg, infliximab, adalimumab, certolizumab) Age Restriction:  Ages 18 and older

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Prescriber  MS prescribed by a neurologist or an MS specialist registered with the TOUCH prescribing Restrictions: program  CD prescribed by a physician registered with the TOUCH program

Coverage  Approval: 12 months, unless otherwise specified. Duration:

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POLICY NAME: TYVASO Affected Medications: TYVASO (treprostinil)

Covered Uses:  All FDA-approved indications not otherwise excluded from benefit design. Required Pulmonary arterial hypertension (PAH) WHO Group 1 Medical  Documentation of PAH confirmed by right-heart catheterization Information:  Etiology of PAH: idiopathic PAH, hereditary PAH, OR  PAH secondary to one of the following conditions: o Connective tissue disease o Human immunodeficiency virus (HIV) infection o Drugs o Congenital left to right shunts o Schistosomiasis o Portal hypertension  Documentation of acute vasoreactivity testing (positive result requires trial/failure to calcium channel blocker) o NYHA/WHO Functional Class III symptoms Appropriate  For initiation of therapy patient must have mean pulmonary artery pressure at least 25 Treatment mmHg at rest or at least 30 mmHg with exertion Regimen & AND Other Criteria:  The pulmonary hypertension has progressed despite maximal medical and/or surgical treatment of the identified condition  Treatment with oral calcium channel blocking agents has been tried and failed, or has been considered and ruled out  Documentation that treprostinil is used as a single route of administration (Remodulin, Tyvaso, Orenatriam should not be used in combination)  Subsequent approval requires documentation of treatment success: exercise endurance, echocardiographic testing, hemodynamic testing, BNP, functional class

Exclusion  PAH secondary to pulmonary venous hypertension (e.g., left sided atrial or ventricular Criteria: disease, left sided valvular heart disease, etc) or disorders of the respiratory system (e.g., chronic obstructive pulmonary disease, interstitial lung disease, obstructive sleep apnea or other sleep disordered breathing, alveolar hypoventilation disorders, etc.)

Age Restriction:  18 years of age and older

Prescriber  Prescribed by or in consultation with a cardiologist or pulmonologist Restrictions:

Coverage  Initial coverage: 3 months unless otherwise specified Duration:  Subsequent coverage: 12 months unless otherwise specified

330

POLICY NAME: ULTOMIRIS Affected Medications: ULTOMIRIS (ravulizumab-cwvz)

Covered Uses:  All food and Drug Administration (FDA) approved indications not otherwise excluded by plan design. Required Medical Paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis Information:  Complete blood count (CBC), reticulocyte count, lactate dehydrogenase (LDH), packed RBC transfusion requirement  Patients must be administered a meningococcal vaccine at least 2 weeks prior to initiation of Ultomiris therapy if have not received one in the past 3 years, and revaccinated according to current ACIP guidelines  LDH levels greater than or equal to 1.5 times the upper limit of normal range OR treated with eculizumab for PNH for at least 6 months prior to initiation of Ultomiris therapy  PNH diagnosis confirmed by documented by high-sensitivity flow cytometry evaluation of red blood cells and white blood cells with granulocyte or monocyte clone size of ≥ 5%  Platelet count of at least 30,000  At least 4 blood transfusions required in the previous 12 months for those not currently on Soliris Appropriate Paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis Treatment Documented treatment failure with Soliris, defined as ongoing need for transfusions Regimen & Other despite regular treatment for at least 6 months. Criteria: Dosing: Single loading dose on first day of therapy, followed by regular maintenance dosing beginning on 15th day of therapy, adjusted by weight:  ≥ 40 to <60 kg: 2400 mg loading, then 3000 mg every 8 weeks  ≥ 60 to <100 kg: 2700 mg loading, then 3300 mg every 8 weeks  ≥100 kg: 3000 mg loading, then 3600 mg every 8 weeks

Reauthorization requires documentation of treatment success  Serum LDH, hemoglobin, decrease in blood transfusions, infusion records

Exclusion Criteria:  Current meningitis infection  History of bone marrow transplantation Age Restriction:  18 years of age or older Prescriber Restrictions:  Hematologist

Coverage  Initial approval: 3 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

331

POLICY NAME: UNITUXIN Affected Medications: UNITUXIN (dinutuximab)

Covered Uses:  All FDA-approved indications not otherwise excluded from by plan design.  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, disease staging, all prior therapies used, Information: and prescribed dosing regimen  Documentation of Neuroblastoma, High risk, with at least a partial response to prior first-line multi-agent, multimodality therapy

Appropriate Treatment  Maximum duration: 5 cycles Regimen & Other  Must be used in combination with granulocyte-macrophage colony-stimulating Criteria: factor, interleukin-2, and 13-cis-retinoic acid

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber Restrictions:  Oncologist

Coverage Duration:  Approval: 5 months, unless otherwise specified

332

POLICY NAME: VAGINAL PROGESTERONE Affected Medications: FIRST-PROGESTERONE VGS 100,200, or 400MG (vaginal progesterone)

Covered Uses:  Prevention of preterm birth in pregnant women with a singleton pregnancy and prior history of preterm delivery before 37 weeks gestation or short cervical length Required Medical  Singleton pregnancy Information:  History of singleton spontaneous preterm birth before 37 weeks gestation or short cervical length defined as less than 20 mm Appropriate Treatment Regimen & Other Criteria: Exclusion Criteria:  Treatment of infertility Age Restriction: Prescriber  Prescribed by or in consultation with gynecologist or obstetrician Restrictions:

Coverage Duration:  20 weeks, unless otherwise specified

333

POLICY NAME: VALCHLOR Affected Medications: VALCHLOR (mechlorethamine hydrochloride)

Covered Uses:  All FDA-approved indications not otherwise excluded from benefit design.  NCCN indications with evidence level of 2A or higher Required Medical  Diagnosis of Stage IA or Stage IB mycosis fungoides-type cutaneous T-cell lymphoma Information: o Extent of skin involvement (limited/localized or generalized)

Appropriate  Documentation of all prior therapies used for the given indication Treatment  Documentation of counseling on applicable special handling procedure Regimen & Other Criteria: Limited/localized skin involvement  Documentation of failure or contraindication of ≥ 1 topical retinoid (tretinoin 0.05%, etc) AND topical corticosteroid

Generalized skin involvement  Documentation of failure or contraindication to at least ≥1 skin-directed therapy (topical corticosteroids, topical retinoids, phototherapy, topical chemotherapy [e.g. carmustine], topical imiquimod, local radiation)

Reauthorization:  Documentation of monitoring for non-melanoma skin cancer  Documentation of improvement with treatment based either on CAILS score or decrease in severity of scaling, plaque elevation or surface area

Exclusion Criteria:  Use in the management of onychomycosis,  treatment or prevention of vaginal or vulvovaginal candidiasis, tinea cruris, tinea manuum, tinea pedis, tinea faciei, tinea capitis, tinea barbae, tinea corporis, tinea versicolor (pityriasis versicolor), or other superficial fungal infections.  Coverage is not recommended for circumstances not listed in the Covered Uses. Age Restriction:  Age > 18 years.

Prescriber  Oncologist or Dermatologist Restrictions:

Coverage  Initial Approval: 3 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

334

POLICY NAME: VARIZIG Affected Medications: VARIZIG (varicella zoster immune globulin (human) IM injection)

Covered Uses:  All FDA-approved indications not otherwise excluded from from benefit design.  For postexposure prophylaxis of varicella in high-risk individuals Required Medical Documentation of immunocompromised patient, defined as: Information:  newborns of mothers with signs and symptoms of varicella shortly before or after delivery (eg five days before to two days after delivery),  Hospitalized premature infants born at at least 28 weeks of gestation who are exposed during their hospitalization and whose mothers do not have evidence of immunity,  Hospitalized premature infants less than 28 weeks of gestation or who weigh 1000 g or less at birth and were exposed to varicella during hospitalization, regardless of mothers immunity status to varicella  Immunocompromised children and adults who lack evidence of immunity to varicella,  Pregnant women who lack evidence of immunity to varicella . Lack evidence of immunity to varicella is defined as: those who are seronegative for varicella zoster antibodies OR those with unknown history of varicella Appropriate  If repeat dose is necessary due to re-exposure, use more than 3 weeks after initial Treatment administration. Regimen & Other Criteria:

Exclusion Criteria:  Coagulation disorders

Age Restriction: Prescriber Restrictions:

Coverage  Approval: 6 months, unless otherwise specified Duration:

335

POLICY NAME: VECTIBIX Affected Medication: VECTIBIX

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.  NCCN indications with evidence level of 2A or higher

Required  Documentation of performance status, disease staging, all prior therapies used, documentation: and prescribed treatment regimen  KRAS mutation status – wild type

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Appropriate Treatment Regimen: Exclusion Criteria:  Failure of previous EGFR inhibitor  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Provider Restriction:  Oncologist Approval Duration:  Initial approval: 4 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

336

POLICY NAME: VENTAVIS Drug Name: VENTAVIS (iloprost)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.

Required Pulmonary arterial hypertension (PAH) WHO Group 1 documentation:  Documentation of PAH confirmed by right-heart catheterization  Etiology of PAH: idiopathic PAH, hereditary PAH, OR  PAH secondary to one of the following conditions:  Connective tissue disease  Human immunodeficiency virus (HIV) infection  Drugs  Congenital left to right shunts  Shistosomiasis  Portal hypertension  Documentation of Acute Vasoreactivity Testing (positive result requires trial/failure to calcium channel blocker)  NYHA/WHO Functional Class III to IV symptoms

Appropriate  For initiation of therapy patient must have mean pulmonary artery pressure at least 25 Treatment mmHg at rest or at least 30 mmHg with exertion AND the pulmonary hypertension has Regimen: progressed despite maximal medical and/or surgical treatment of the identified condition  Treatment with oral calcium channel blocking agents has been tried and failed, or has been considered and ruled out  Subsequent approvals require documentation of treatment success such as improved walking distance or improvements in functional class Exclusion  PAH secondary to pulmonary venous hypertension (e.g., left sided atrial or ventricular Criteria: disease, left sided valvular heart disease, etc) or disorders of the respiratory system (e.g., chronic obstructive pulmonary disease, interstitial lung disease, obstructive sleep apnea or other sleep disordered breathing, alveolar hypoventilation disorders, etc.) Age Restriction:  18 years or older

Provider  Prescribed by or in consultation with a cardiologist or a pulmonologist Restriction:

Approval  12 months, unless otherwise specified Duration:

337

POLICY NAME: VERZENIO Drug Name: VERZENIO (abemaciclib tablet)

Covered Uses:  NCCN (National Comprehensive Cancer Network) indications with evidence level of 2a or higher

Required  Documentation of performance status, disease staging, all prior therapies used, documentation: and anticipated treatment course  For breast cancer – documentation of ER-positive and HER2-negative disease Appropriate  Reauthorization: documentation of disease responsiveness to therapy Treatment Regimen: Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater  Previous use of any agents within the class (such as Ibrance, Kisqali) Age Restriction:

Provider Restriction:  Oncologist Approval Duration:  Initial approval: 4 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

338

POLICY NAME: VFEND Affected Medications: VFEND (voriconazole)

Covered Uses:  All FDA-approved indications not otherwise excluded from benefit design.  Empiric treatment of presumed fungal infections in febrile neutropenic, high-risk patients pending susceptibility cultures.  Continuation therapy for patients started/stabilized on intravenous (IV) or oral voriconazole for a systemic infection. Required Medical  All indications: Information: o Susceptibility cultures matching voriconazole activity o Exceptions made for empiric therapy as long as treatment is adjusted when susceptibility cultures are available  Esophageal candidasis o Trial of one other systemic agent (eg., fluconazole, IV amphotericin B, itraconazole) Appropriate Treatment Regimen & Other Criteria: Exclusion  Concomitant use of carbamazepine, CYP3A4 substrates (terfenadine, astemizole, Criteria: cisapride, pimozide, or quinidine), high-dose ritonavir (400 mg every 12 hours), ergot alkaloids, long-acting barbiturates, rifabutin, rifampin, sirolimus, St. John's wort, or efavirenz at standard doses of 400 mg/day or higher Age Restriction:  12 years of age or older

Prescriber Restrictions: Coverage  Approval: 12 month, unless otherwise specified Duration:

339

POLICY NAME: VIMIZIM Affected Medications: VIMIZIM (elosulfase alfa)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Diagnosis of Mucopolysaccharidosis type IVA (MPS IVA; Morquio A syndrome) Information: confirmed by an enzyme assay  Medical history of musculoskeletal conditions such as knee deformity, kyphosis, hip dysplasia, prior spinal fusion surgery, and arthralgia  Baseline six minute walk test (6-MWT) Appropriate  Recommended dose is 2 mg per kg given intravenously over a minimum range of Treatment 3.5 to 4.5 hours, based on infusion volume, once every week Regimen & Other  Pre-treatment with antihistamines with or without antipyretics is recommended 30 Criteria: to 60 minutes prior to the start of the infusion  Reauthorization requires documentation of treatment success defined as improved six minute walk test Exclusion Criteria: Age Restriction:  5 years of age or older Prescriber Restrictions: Coverage Duration:  Initial approval: 6 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

340

POLICY NAME: VISTOGARD Affected Medications: VISTOGARD (uridine triacetate)

Covered  All FDA-approved indications not otherwise excluded by plan design Uses: Required  Documented therapy with fluorouracil OR Medical  Documented therapy with capecitabine OR Information:  Documented life-threatening adverse effects associated with fluorouracil or capecitabine treatment regimens Appropriate  To be used as antidote for fluorouracil or capecitabine overdose or to treat severe adverse- Treatment effects as consequence of fluorouracil/capecitabine treatment Regimen &  Ensure dosing according to FDA approved regimen Other  Ensure use is within 96 hours of fluorouracil/capecitabine treatment Criteria: Exclusion Criteria: Age Restriction: Prescriber  Oncologist Restrictions: Coverage  Approval: 5 days, unless otherwise specified Duration:

341

POLICY NAME: VIVITROL Affected Medications: VIVITROL (naltrexone for extended-release injectable suspension)

Covered  All FDA-approved indications not otherwise excluded by plan design Uses: Required  Documentation that member is part of a comprehensive management program that includes Medical psychosocial support Information:  Documentation of alcohol/benzodiazepine/opioid abstinence for a minimum 7-10 days prior to start of Vivitrol therapy AND  For opioid dependence, completion of opioid detoxification with negative urine drug screen OR successful passing of a naloxone challenge test Appropriate  Initial approval requires documented failure to minimum 1-month trial with oral naltrexone Treatment OR Regimen &  Contraindication to minimum 1-month trial with oral naltrexone Other  Subsequent approvals require documentation of treatment success defined as reduction in Criteria: days of heavy drinking or opioid-free days  Exclusion  Patient receiving opioid analgesics/current physiologic opioid dependence/acute opioid Criteria: withdrawal  Acute hepatitis Age  Age greater than or equal to 18 years Restriction: Prescriber Restrictions: Coverage  Approval: 12 months, unless otherwise specified Duration:

342

POLICY NAME: VOTRIENT Affected Medications: VOTRIENT (pazopanib)

Covered Uses:  NCCN indications with evidence level of 2A or higher Required Medical  Documentation of performance status, disease staging, all prior therapies used, Information: and prescribed treatment regimen.  Documentation that Votrient is being used as a NCCN 2A level of evidence regimen. Appropriate Treatment  Documentation of dose adjustment with strong CYP3A4 inhibitors. Regimen & Other  Avoidance of strong CYP3A4 inducers. Criteria:  Reauthorization required documentatin of treatment success Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber Restrictions:  Oncologist Coverage Duration:  Initial approval: 3 months (2 week initial partial fill), unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

343

POLICY NAME: VPRIV Affected Medications: VPRIV (velaglucerase alfa)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Patient has a diagnosis of type 1 Gaucher disease. Information:  Diagnosis of Gaucher disease is confirmed by an enzyme assay demonstrating a deficiency of beta-glucocerebrosidase enzyme activity.  Therapy is initiated for a patient with one or more of the following conditions: anemia, thrombocytopenia, bone disease, hepatomegaly, or splenomegaly. Appropriate Reauthorization will require documentation of treatment success and a clinically Treatment significant response to therapy Regimen & Other Criteria: Exclusion Criteria:  Concomitant therapy with miglustat Age Restriction: Prescriber Restrictions: Coverage Duration:  Approval: 12 months, unless otherwise specified.

344

POLICY NAME: XALKORI Affected Medications: XALKORI (crizotinib)

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, all prior therapies used, and prescribed Information: treatment regimen  Documentation of use with NCCN 2A or higher level of evidence regimen  Documentation of ROS1- or ALK-positive tumors, or IMT with ALK translocation

Appropriate Treatment  Avoid concurrent use with strong CYP3A inducers or inhibitors Regimen & Other Criteria:  Reauthorization requires documentation of disease responsiveness to therapy

345

POLICY NAME: XELJANZ Affected Medications: XELJANZ, XELJANZ XR (tofacitinib)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design. Required  Documentation of moderate to severe disease while on treatment Medical  Documented current level of disease activity/disease control Information:  Indication must be documented in chart notes within the last 6 months  Documentation of complete and current treatment course  Complete set of the following laboratory tests: basic metabolic panel, and liver function test, complete blood count Rheumatoid Arthritis (RA)  Documented current level of disease activity with one of the following:  The Disease Activity Score derivative for 28 joints (DAS-28) greater than 3.2  The Simplified Disease Activity Index (SDAI) greater than 11  The Clinical Disease Activity Index (CDAI) greater than 10  Laboratory test confirming diagnosis of rheumatoid arthritis (anti-CCP, RF) Psoriatic Arthritis (PsA)  Documentation of CASPAR criteria score greater than 3 based on chart notes  Skin psoriasis that is: . Present – two points, OR . Previously present by history – one point, OR . A family history of psoriasis, if the patient is not affected – one point  Nail lesions (onycholysis, pitting) – one point  Dactylitis (present or past, documented by a rheumatologist) – one point  Negative rheumatoid factor (RF) – one point  Juxtaarticular bone formation on radiographs (distinct from osteophytes) – one point Ulcerative Colitis (UC)  Diagnosis supported by endoscopy/colonoscopy/sigmoidoscopy or biopsy and a Mayo Clinic Score for Assessment of Ulcerative Colitis Activity

Appropriate Rheumatoid Arthritis (RA) Treatment  Documented clinical failure with at least 12 weeks of combination disease-modifying Regimen & antirheumatic drug (DMARD) therapy [Methotrexate plus sulfasalazine, methotrexate plus Other Criteria: hydroxychloroquine, sulfasalazine plus hydroxychloroquine, leflunamide plus sulfasalazine, or leflunomide plus hydrodroxychloroquine] AND  Has failed minimum 12 weeks of Humira AND minimum 12 weeks of Enbrel individually  Quantity Limit o QL #2 tablets per day (Xeljanz) o QL #1 tablet per day (Xeljanz XR)

346

Psoriatic Arthritis (PsA)  Failure of at least 12 weeks with methotrexate, or if unable to tolerate or contraindicated to methotrexate, another disease modifying antirheumatic drug sulfasalazine, cyclosporine, leflunomide) AND  Has failed minimum 12 weeks of Humira AND Enbrel individually  Quantity Limit o QL #2 tablets per day (Xeljanz) o QL #1 tablet per day (Xeljanz XR)

Ulcerative Colitis (UC)  Documented failure with at least two oral treatments for a minimum of 12 weeks: corticosteroids, sulfasalazine, azathioprine, mesalamine, balsalazide, cyclosporine, azathioprine, 6-mercaptopurine AND  Has failed minimum 12 weeks on Humira  Approval for Xeljanz immediate release only  Quantity Limit o QL #2 tablets per day (Xeljanz)

Reauthorization: documentation of treatment success and a clinically significant response to therapy

Exclusion  Concurrent use with potent immunosuppressants (azathioprine, cyclosporine), or other Criteria: biologic DMARDs.  Hepatitis B or Hepatitis C infection  ANC less than 1000 cells/mm3, lymphocytes less than 500 cells/mm3, Hgb less than 9 g/dL, CrCl less than 40 mL/min  History of diverticulitis or GI perforation, unresolved cytopenias, severe hepatic impairment, or severely uncontrolled hyperlipidemia  Evidence of active, latent, or inadequately treated tuberculosis or herpes zoster  Psoriatic arthritis with axial (spinal) involvement Age  18 years of age and older Restriction:

Prescriber  RA/PsA: Prescribed by or in consultation with a rheumatologist Restrictions:  UC: prescribed by or in consultation with a gastroenterologist specialist Coverage  Initial approval: 4 months, unless otherwise specified. Duration:  Reauthorization: 12 months, unless otherwise specified.

347

POLICY NAME: XENAZINE Affected Medications: XENAZINE (tetrabenazine)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.

Required Medical  Current complete medication list Information: Appropriate  Check for CYP2D6 interactions - strong CYP2D6 inhibitors (such as quinidine or Treatment antidepressants e.g., fluoxetine, paroxetine) significantly increase exposure therefore Regimen & Other the total daily dose should not exceed a maximum of 50 mg Criteria:  Reauthorization requires documentation of clinically significant response to therapy with no major adverse reactions to treatment

Exclusion Criteria:  Comorbid untreated or inadequately treated depression or actively suicidal  Combination use with an MAOI, or within a minimum of 14 days of discontinuing therapy with an MAOI  Combination use with reserpine. At least 20 days should elapse after stopping reserpine before starting Xenazine  Comorbid hepatic impairment, including mild impairment

Age Restriction: Prescriber  Prescribed by or after consultation with a neurologist. Restrictions: Coverage  Initial approval: 3 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified.

348

POLICY NAME: XEOMIN, DYSPORT and MYOBLOC Affected Medications: XEOMIN (incobotulinumtoxinA), DYSPORT (AbobotulinumtoxinA), MYOBLOC (RimabotulinumtoxinB)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design

Required Medical  Pertinent medical records and diagnostic testing Information:  Complete description of the site(s) of injection  Strength and dosage of botulinum toxin used Appropriate Dysport Treatment  Approved first-line for focal dystonia, hemifacial spasm, orofacial dyskinesia, Regimen & Other blepharospasm, upper or lower limb spasticity Criteria: Xeomin  For the uses of cervical dystonia and upper limb spasticity in adults documented failure with Botox and Dysport is required  In the treatment of blepharospasm, documented failure with Botox is required Myobloc  For the treatment of cervical dystonia documented failure with Botox and Dysport is required  For the treatment of overactive bladder, urinary incontinence due to spinal cord injury or axillary hyperhidrosis documented failure with Botox is required

Other criteria  Reauthorization requires documented treatment success  All indications not listed are considered experimental/investigational and are not a covered benefit  Maximum of 4 treatments per 12 months (2 treatments for Myobloc in overactive bladder)

Exclusion Criteria:  Cosmetic procedures  Headaches/Migraines  Hemifacial spasm: no longer above the line on the prioritized list  For intradetrusor injections: documented current/recent urinary tract infection or urinary retention  Current aminoglycoside use (or current use of other agents interfering with neuromuscular transmission)  Use in the treatment of sialorrhea

Age Restriction:  ≥18 years old for Xeomin and Myobloc Prescriber  Blepharospasm: ophthalmologist or optometrist Restrictions:  OAB or urinary incontinence due to neurologic condition: urologist or neurologist  Documentation of consultation with any of the above specialists mentioned 349

Coverage Duration: Overactive Bladder:  Initial approval: 3 months  Reauthorization: 12 months, unless otherwise specified All other indications  12 months, unless otherwise specified

350

POLICY NAME: XGEVA Affected Medications: XGEVA (denosumab)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  National Comprehensive Cancer Network (NCCN) indications with evidence level of 2A or higher. Required Medical  One of these diagnoses Information:  Giant Cell Tumor . Unresectable disease or surgical resection would likely result in severe morbidity  Bone Metastases from Solid Tumors  Hypercalcemia of Malignancy . Refractory to bisphosphonate therapy or contraindication  Multiple Myeloma  Requires failure of Zoledronic Acid or Pamidronate OR creatinine clearance less than 30mL/min

Appropriate  The patient will receive calcium and Vitamin D as needed to treat or prevent Treatment hypocalcemia Regimen:  For treatment of breast cancer with bony mets: Approval is limited to monthly dosage for the first 12 months of therapy followed by quarterly doses thereafter (not to exceed 4 doses within a 12 month period). Exclusion Criteria:

Age Restriction:  Giant Cell Tumor of the Bone: Age ≥ 13 years AND skeletally mature  Bone Metastases from Solid Tumor: Age ≥ 18 years Provider Restriction: Coverage  Approval: 12 months Duration:

351

POLICY NAME: XIAFLEX Affected Medications: XIAFLEX (collagenase clostridium histolyticum)

Covered Uses:  All FDA-approved indications not otherwise excluded plan design o Dupuytren’s contracture with a palpable cord o Peyronie’s disease Required Medical Peyronie’s Disease Information:  Documented diagnosis of Peyronie’s disease with a palpable plaque  Curvature deformity is at least 30 degrees at the start of therapy and results in pain  Symptoms have been present for at least 12 months Appropriate Dupuytren’s Treatment  Authorization will be limited per joint as follows: One injection per month for a Regimen: maximum of three injections per cord  Reauthorization will require documentation of treatment success and a clinically significant response to therapy Peyronie’s  One treatment cycle consisting of two Xiaflex injection procedures  Subsequent authorization(s) for additional treatment cycles may be given if the curvature deformity is more than 15 degrees after the first, second or third treatment cycle, or if the prescribing healthcare provider determines that further treatment is clinically indicated Exclusion  Prior intolerance or allergic reaction to requested medication Criteria: Age Restriction:

Provider  Peyronie’s: Urologist Restriction: Coverage  Dupuytren’s: 12 weeks, unless otherwise specified per impacted contracture (separate Duration: approval is required for each impacted cord for treatment of Dupuytren’s contracture)  Peyronie’s: 6 weeks, unless otherwise specified

352

POLICY NAME: XIFAXAN Affected Medications: XIFAXAN (rifaximin)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.  Treatment of complex Clostridium difficile infection in select populations Required Medical  Documentation of complete & current treatment course required. Information:  Patient Age  Previous antibiotic history and documented allergies/hypersensitivity Appropriate For C. difficile disease: Treatment  Patient must have failed 1 course of metronidazole and 2 courses of oral vancomycin Regimen & Other for coverage to be considered Criteria: For recurrent or persistent hepatic encephalopathy:  Patient has failed or has contraindication to 30 day attempt of lactulose therapy, with documentation of continued altered mental status or elevated ammonium levels despite adequate upward titration of lactulose.

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria: For C. difficile disease:  Xifaxan 200 mg tablets with a quantity supply exceeding 20 days of a quantity of 120 for C. diff infection. For recurrent or persistent hepatic encephalopathy:  Xifaxan exceeding the recommended dose of two 550 mg tablets daily for treatment / prevention of hepatic encephalopathy. Age Restriction:  12 years or older Prescriber Restrictions: Coverage  Clostridium difficile infection = 20 days, unless otherwise specified Duration:  Hepatic encephalopathy = 12 months, unless otherwise specified

353

POLICY NAME: XOFIGO Affected Medications: XOFIGO (Radium 223)

Covered Uses:  NCCN indications with evidence level of 2A or higher Required Medical  Diagnosis of castration-resistant prostate cancer (CRPC), symptomatic bone Information: metastases, and no known visceral metastatic disease and no pathologic adenopathy greater than 3 cm Appropriate  Baseline CBC Monitoring Treatment  ANC ≥ 1.5 × 109 /L Regimen & Other  Platelet count ≥ 100 × 109 /L Criteria:  Hemoglobin ≥ 10 g/dL

Reauthorization will require documentation of treatment success and a clinically significant response to therapy Exclusion Criteria:  Concomitant chemotherapy Age Restriction:  Age greater than 18 years Prescriber Restrictions: Coverage Duration:  Approval: 6 months, unless otherwise specified

354

POLICY NAME: XOLAIR Affected Medications: XOLAIR (omalizumab)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design. Required Medical Moderate to severe persistent allergic asthma Information:  patient has a positive skin test or in vitro test for 1 or more perennial aeroallergens (eg, house dust mite, animal dander [dog, cat], cockroach, feathers, mold spores) AND  documentation of total serum IgE level AND  Asthma symptoms not controlled for ≥ 2 months by concomitant use of high-dose inhaled corticosteroid AND long-acting beta agonist (LABA).

 If LABA contraindicated or not tolerated, sustained-release theophylline OR a leukotriene modifier must be tried AND  Inadequate control must be demonstrated by hospitalization for asthma OR  requirement of systemic corticosteroids for exacerbation OR  increasing need for short-acting beta agonist (SABA) as demonstrated by > 4 times a day use

Chronic idiopathic urticarial: Inadequate response or intolerance to  ≥ 2 high-dose (4x standard dose) H1 antihistamines (first or second generation), each given minimum 2-week trial AND  ≥ 4 week trial with addition of leukotriene modifier OR  H2 antihistamine (e.g. cimetidine, famotidine, or ranitidine) OR  brief burst of oral corticosteroids AND  ≥ 8 week trial with immunosuppressant (e.g. cyclosporine, tacrolimus) OR  anti-inflammatory agent (e.g. dapsone, sulfasalazine, 3-month trial if hydroxychloroquine)

Appropriate Dosing: Treatment  Allergic asthma: Regimen & Other o Dose based on pre-treatment Serum IgE (IU/mL), Weight (kg), and age. Criteria: o If weight and IgE levels are outside of recommended dosing schedule, use of Xolair is considered experimental and is not covered.  Chronic idiopathic urticaria o 150mg or 300mg subcutaneous injection every 4 weeks  Must provide documentation of removal of allergens before coverage is provided (pets, plants, etc.)  Chronic idiopathic urticaria continuation of treatment: documentation of improvement in weekly urticaria activity score (UAS7) by ≥ 50% at week 12

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

355

Exclusion Criteria: Ages 6 to <12 years old o Pre-treatment serum IgE >1300 IU/mL o See package insert for specific weight and serum IgE based dosing Ages >12 years old o Total serum IgE level < 30 or > 700 IU per mL  Not indicated for other allergic conditions or other forms of urticaria  Not indicated for acute bronchospasm or status asthmaticus Age Restriction: Moderate to severe persistent allergic asthma:  ≥ 6 years old Chronic idiopathic urticarial with inadequate response:  ≥12 years Prescriber  Moderate to severe persistent asthma: prescribed by or in consultation with an Restrictions: allergist, immunologist, or pulmonologist.  SAR/PAR: prescribed by an allergist, immunologist, or pulmonologist  EG/EE/EC: if prescribed or in consultation with an allergist, immunologist, or gastroenterologist. Coverage Duration:  12 months, unless otherwise specified

356

POLICY NAME: XTANDI Affected Medications: XTANDI (enzalutamide)

Covered Uses:  NCCN indications with evidence level of 2A or higher. Required Medical  Documentation of disease staging, all prior therapies used, and anticipated Information: treatment course AND  Documentation of Metastatic (M1) Castrate Recurrent Prostate Cancer  Documentation of ECOG performance status ≤ 2 Appropriate Treatment Reauthorization will require documentation of treatment success and a clinically Regimen & Other significant response to therapy Criteria: Exclusion Criteria: Age Restriction: Prescriber Restrictions: Coverage Duration:  Initial approval: 3 months, unless otherwise specified.  Reauthorization: 12 months, unless otherwise specified.

357

POLICY NAME: XURIDEN Affected Medications: XURIDEN (uridine triacetate)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Diagnosis of hereditary orotic aciduria Information:  Urine orotic acid levels  Patient weight Appropriate Treatment  Documentation of weight based dosing Regimen & Other Criteria:  Reauthorization requires documentation of treatment success based on laboratory values Exclusion Criteria:

Age Restriction:

Prescriber Restrictions:  In consultation with geneticist specialist

Coverage Duration:  Approval: 12 months, unless otherwise specified

358

POLICY NAME: XYREM Affected Medications: XYREM (sodium oxybate)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Patient has a diagnosis of narcolepsy and experiences episodes of cataplexy OR Information:  Patient has a diagnosis of narcolepsy and experiences excessive daytime sleepiness with symptoms that limit the ability to perform normal daily activities AND . An Epworth Sleepiness Scale score of at least 15 at baseline Appropriate  Documentation of alcohol abstinence Treatment  Documentation that at least two of the following were ineffective: Regimen & Other . modafinil or armodafinil Criteria: . methylphenidate or dextroamphetamine or lisdexamfetamine . venlafaxine or atomoxetine or fluoxetine  Re-authorization: If the patient has received prior treatment with Xyrem . Narcolepsy with cataplexy: clinically significant reduction in cataplexy episodes . Excessive daytime sleepiness: clinical significant improvement in activities of daily living and in Epworth Sleepiness Scale score

Exclusion Criteria:  If the patient is taking alcohol (ethanol), sedative/hypnotic drugs, or other CNS depressants. Age Restriction:  18 years of age or older Prescriber  Sleep specialist enrolled in Xyrem REMS program Restrictions:

Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Subsequent approval: 12 months, unless otherwise specified

359

POLICY NAME: YESCARTA Affected Medications: YESCARTA (axicabtagene ciloleucel)

Covered Uses:  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of disease staging, all prior therapies used, performance status of 0-1 Information:  Patient weight  Documentation of adequate organ and marrow function Appropriate  Dosing: single infusion only. Treatment Regimen & Other Criteria: Exclusion Criteria:  History of allogeneic HSCT or central nervous system lymphoma  Absolute lymphocyte count less than 100/ul, CrCl less than 60 mL/min, hepatic transaminases more 2.5x the upper limit of normal. Cardiac ejection fraction less than 50%, or active serious infection Age Restriction: Prescriber  Oncologist and Health care facilities must be enrolled and comply with the Risk Restrictions: Evaluation and Mitigation Strategies (REMS) requirement Coverage Duration:  Approval: 1 month, unless otherwise specified (one infusion only)

360

POLICY NAME: ZAFIRLUKAST Affected Medications: Zafirlukast

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design o Prophylaxis and chronic treatment of asthma in adults and children 5 years of age and older Required Medical  Documentation of current diagnosis of asthma OR exercise induced Information: bronchospasm AND  Treatment failure with montelukast  Reauthorization requires documentation of treatment success Appropriate Treatment Regimen & Other Criteria: Exclusion Criteria: Age Restriction:

Prescriber Restrictions:

Coverage Duration:  Approval: 12 months, unless otherwise specified

361

POLICY NAME: ZAVESCA Affected Medications: ZAVESCA (miglustat)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.

Required Medical  Diagnosis of Type 1 Gaucher disease Information:  Mild to moderate disease  Diagnosis of Gaucher disease confirmed by an enzyme assay demonstrating a deficiency of beta-glucocerebrosidase enzyme activity

Appropriate  Enzyme replacement therapy (ERT) is not a therapeutic option for the patient (e.g. Treatment due to allergy, hypersensitivity, or poor venous access) Regimen & Other  The patient will use adequate contraception throughout Zavesca therapy and for 3 Criteria: months thereafter

Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  Female of childbearing potential who is pregnant or planning a pregnancy

Age Restriction:  18 years of age and older

Prescriber Restrictions: Coverage Duration:  Initial Approval: 3 months, unless otherwise specified  Subsequent approval: 12 months, unless otherwise specified

362

POLICY NAME: ZEJULA Affected Medications: ZEJULA (naraparib)

Covered Uses:  National Comprehensive Cancer Network (NCCN) indications with evidence level of 2A or higher Required Medical  Documentation of performance status, disease staging, all prior therapies used, and Information: anticipated treatment course Appropriate  Maintenance therapy for Epithelial Ovarian Cancer/Fallopian Tube Cancer/Primary Treatment Peritoneal Cancer for those with platinum-sensitive disease who have completed two Regimen & Other or more lines of platinum-based therapy Criteria:  Reauthorization: documentation of disease responsiveness to therapy Exclusion Criteria:  Previous PARP inhibitor utilization  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber  Oncologist Restrictions:

Coverage  Initial approval: 3 months, unless otherwise specified Duration:  Reauthorization: 12 months, unless otherwise specified

363

POLICY NAME: ZELBORAF Affected Medications: ZELBORAF (vemurafenib)

Covered Uses:  All FDA-approved indications not otherwise excluded by plan design.  NCCN indications with evidence level of 2A or higher Required Medical  Documentation of performance status, disease staging, all prior therapies used, Information: and prescribed treatment regimen  Documentation that Zelboraf is being used as a NCCN 2A level of evidence regimen  If applicable, documentation of BRAF V600 mutant Appropriate Treatment  Avoidance of strong CYP3A4 inducers or inhibitors Regimen & Other  Reauthorization requires documentation of treatment success Criteria: Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater

Age Restriction: Prescriber Restrictions:  Oncologist Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

364

POLICY NAME: ZINPLAVA Affected Medications: ZINPLAVA (bezlotoxumab)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Documentation of diarrhea (at least 3 unformed stools in 24 hr) or radiographic evidence Information: of ileus or toxic megacolon  Stool positive for GDH antigen AND Toxin A & B OR PCR positive  If GDH positive/toxin negative OR GDH negative/toxin positive, PCR MUST be positive  Patient must be receiving concurrent treatment for Clostridium difficile: metronidazole (IV or PO), oral vancomycin, fidaxomicin

Appropriate  Patients at high risk for CDI recurrence (must have at least one risk factor): age >65, one Treatment or more episodes of Clostridium Difficile infection (CDI) in previous 6 months, Regimen & Other immunocompromised status, clinically severe CDI (as defined by Zar score ≥2). Criteria: Exclusion  Stool NEGATIVE for GDH and Toxin, or PCR negative if incongruent GDH/toxin Criteria:  Heart Failure Age Restriction:  Age 18 years or greater Prescriber Restrictions: Coverage  Approval: One treatment may be given while patient is receiving antibiotic therapy for Duration: treatment of C. difficile (usually 14 days)

365

POLICY NAME: ZOLINZA Affected Medications: Zolinza (vorinostat)

Covered Uses:  NCCN indications with evidence level of 2A or higher Required Medical  Documentation of disease staging, all prior therapies used, performance status, Information: and anticipated treatment course  Documentation to support anticipated regimen supported by NCCN evidence level 2A or higher

Appropriate Treatment  Reauthorization requires documentation of treatment success Regimen & Other Criteria: Exclusion Criteria:  Hold therapy if Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater.

Age Restriction: Prescriber Restrictions:  Oncologist

Coverage Duration:  Initial approval: 3 months (2 week initial partial fill), unless otherwise specified  Reauthorzation: 12 months, unless otherwise specified

366

POLICY NAME: ZONTIVITY Affected Medications: Zontivity (vorapaxar)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  History of MI or current diagnosis of peripheral artery disease Information:

Appropriate  Clinical documentation that treatment with clopidogrel or prasugrel (with or Treatment without concomitant aspirin) is not tolerated, is contraindicated or has failed Regimen & Other  Vorapaxar will be given with aspirin and/or clopidogrel unless contraindicated Criteria: Reauthorization will require documentation of treatment success and a clinically significant response to therapy

Exclusion Criteria:  History of stroke, TIA or intracranial hemorrhage; active pathological bleeding Age Restriction:  Adults Prescriber  Prescribed by a Cardiologist Restrictions: Coverage Duration:  Approval: 12 months, unless otherwise specified

367

POLICY NAME: ZORBTIVE Affected Medications: ZORBTIVE (somatropin)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Diagnosis of short bowel syndrome (SBS). Information:

Appropriate  Patients must be receiving specialized nutritional support (e.g., TPN, IPN, PPN, Treatment rehydration solutions, electrolyte replacement, high complex-carbohydrate, low-fat Regimen & Other diet) in conjunction with optimal management of SBS. Criteria:  For patients who have received at least 4 weeks of Zorbtive therapy, must show decrease in specialized nutritional support requirement as measured by total volume, total calories or infusion frequency and stabilized or increased in weight. Exclusion Criteria:  Active malignancy (newly diagnosed or recurrent).  Acute critical illness due to complications following open heart or abdominal surgery, accidental trauma or acute respiratory failure.

Age Restriction: Prescriber Restrictions: Coverage  Initial approval: 4 weeks, unless otherwise specified Duration:  Renewal: Up to maximum 8 weeks, unless otherwise specified

368

POLICY NAME: ZYDELIG Affected Medications: ZYDELIG (idelalisib)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design. Required Medical  Documentation of planned monitoring of the following lab values: liver function Information: tests, complete blood count  Documentation of risk/benefit ratio for use in combination with hepatotoxic medications, diarrhea inducing medications, strong CYP3A inducers, or CYP3A inhibitors

Appropriate Relapsed chronic lymphocytic leukemia Treatment o Documentation that rituximab monotherapy is clinically inappropriate Regimen & Other o Failure on one or more of equal placement NCCN recommended therapies Criteria: Relapsed follicular B-cell non-Hodgkin lymphoma OR relapsed small lymphocytic lymphoma o Failure on two or more of equal placement NCCN recommended therapies

 Documentation of ECOG performance status of 1 or 2 OR Karnofsky performance score greater than 50% Authorization will only be granted when used in an FDA approved or NCCN guideline supported regimen

Reauthorization: documentation of disease responsiveness to therapy

Exclusion Criteria: Age Restriction:  18 years and older Prescriber  Oncologist Restrictions:

Coverage Duration:  Initial coverage: 3 months unless otherwise specified  Subsequent coverage: 12 months, unless otherwise specified

369

POLICY NAME: ZYKADIA Affected Medications: ZYKADIA (ceritinib)

Covered Uses:  All FDA-approved indications not otherwise excluded by benefit design.  NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of performance status, disease staging, all prior therapies used, Information: and prescribed treatment regimen  Documentation that Zykadia is being used as a NCCN 2A level of evidence regimen If applicable, documentation of IMT with ALK translocation, ALK-positive mutation

Appropriate Treatment  Appropriate dose reduction with strong CYP3A4 inducers or inhibitors Regimen & Other  Reauthorization requires documentation of treatment success Criteria: Exclusion Criteria:  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater

Age Restriction:

Prescriber Restrictions:  Oncologist Coverage Duration:  Initial approval: 3 months, unless otherwise specified  Reauthorization: 12 months, unless otherwise specified

370

POLICY NAME: ZYTIGA Affected Medications: ZYTIGA (abiraterone)

Covered Uses:  All FDA approved indications not otherwise excluded by benefit design.  ● NCCN indications with evidence level of 2A or higher

Required Medical  Documentation of trial and failure to Yonsa or clinical reason for avoiding Information: Yonsa

Appropriate Treatment Reauthorization will require documentation of disease responsiveness to Regimen & Other Criteria: therapy

Exclusion Criteria:  Child-Pugh Class C  Karnofsky Performance Status 50% or less or ECOG performance score 3 or greater Age Restriction: Prescriber Restrictions: Coverage Duration:  Initial approval: 4 months (2 week initial partial fill), unless otherwise specified  Subsequent approval: 12 months, unless otherwise specified.

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