Prior Authorization Criteria April 2020

Prior Authorization Criteria

The following is the listing of Prescryptive Health prior authorization criteria that will be used to evaluate prior authorization requests. Prescryptive Health’s prior authorization criteria are based on clinical monographs and National Pharmacy and Therapeutics guidelines. Prior Authorization Criteria will be updated regularly to reflect ongoing changes and is subject to change without notice.

Prior Authorization Requests for Tier 4 Medications and Non-Preferred Medications

Tier 4 and non-preferred medications may be authorized when there is clinical justification for doing so. Clinicians can submit a prior authorization (PA) request to initiate a review with the following steps:

1. Download the Prior Authorization Request Form. This form can be found at: www.prescryptive.com/prescriber

2. Fax the completed form with supporting documentation to 1- (848) 456-5463 for both standard and urgent requests.

Note: Urgent requests should be clearly labeled “URGENT” at the top of the prior authorization request form

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 1 Prior Authorization Criteria April 2020 Contents Blanket Criteria ...... 7 OFF-LABEL USES ...... 7 STEP THERAPY EXCEPTION ...... 8 QUANTITY LIMIT EXCEPTION ...... 9 SAFETY EDIT EXCEPTION ...... 10 ORAL and SELF INJECTABLE ONCOLYTICS ...... 11 COMPOUNDED MEDICATIONS ...... 13 SHORT-ACTING ANALGESIC NARCOTICS ...... 14 LONG-ACTING OPIOIDS ...... 17 STADOL NS® (BUTORPHANOL) ...... 19 LOW MOLECULAR WEIGHT HEPARIN/FACTOR XA INHIBITOR INJECTABLES ...... 20 PCSK-9 INHIBITORS ...... 21 PULMONARY HYPERTENSION ...... 24 Dupixent® (dupilumab) ...... 27 SORIATANE® (ACITRETIN) ...... 29 CHEMET® (SUCCIMER) ...... 30 DEFERASIROX, JADENU, FERRIPROX, EXJADE ...... 31 WILSON’S DISEASE ...... 32 TESTOSTERONE REPLACEMENT ...... 34 HP ACTHAR® (CORTICOTROPIN) 80 UNITS/ML GEL ...... 36 SENSIPAR® (CINACALCET) ...... 38 NON-FORMULARY TEST STRIPS/QUANTITY LIMIT EXCEPTION ...... 39 NON-FORMULARY BLOOD GLUCOSE METERS ...... 41 GROWTH HORMONE ...... 42 EGRIFTA® (TESAMORELIN INJECTION) ...... 45 OCTREOTIDE AND SOMAVERT® ...... 47 FORTEO® (TERIPARATIDE) ...... 49 PROLIA® AND XGEVA® (DENOSUMAB) ...... 50 EVENITY (ROMOSOZUMAB) ...... 51

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 2 Prior Authorization Criteria April 2020 ZAVESCA® (MIGLUSTAT) AND CERDELGA® (ELIGLUSTAT TARTRATE) ...... 52 EMFLAZA® (DEFLAZACORT) ...... 53 OXANDROLONE (OXANDRIN®) ...... 55 CONSTIPATION AGENTS ...... 56 CHOLBAM® (CHOLIC ACID) ...... 57 OCALIVA® (OBETICHOLIC ACID) ...... 60 THROMBOCYTOPENIA ...... 62 WHITE BLOOD CELL STIMULATORS ...... 63 ERYTHROPOIETIN STIMULATING AGENTS (ESAs) ...... 65 HEREDITARY ANGIOEDEMA ...... 67 ORFADIN® AND NITYR® (NITISINONE) ...... 70 DARAPRIM® (PYRIMETHAMINE) ...... 72 NEBUPENT® (PENTAMIDINE ISETHIONATE) ...... 73 AZOLE ANTIFUNGALS ...... 74 HEPATITIS B ...... 76 HEPATITIS C ...... 77 SIRTURO® (BEDAQUILINE) ...... 80 MULTIPLE SCLEROSIS ...... 82 XYREM® (SODIUM OXYBATE) ...... 83 DRUGS FOR MOVEMENT DISORDERS ...... 85 NUEDEXTA® (dextromethorphan/quinidine) ...... 87 PHOSPHATE BINDERS ...... 88 ENDARI™ (L-GLUTAMINE) ...... 89 MAKENA® ...... 91 IDIOPATHIC PULMONARY FIBROSIS ...... 92 CYSTIC FIBROSIS ...... 93 LEUKOTRIENE RECEPTOR ANTAGONISTS ...... 95 NARCOTIC WITHDRAWAL THERAPY AGENTS ...... 96 DISEASE MODIFYING BIOLOGICS ...... 98 CALCITONIN GENE-RELATED PEPTIDE (CGRP) RECEPTOR ANTAGONISTS ...... 105

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 3 Prior Authorization Criteria April 2020 SYNAREL (Histrelin) & ORILISSA (elagolix) ...... 107 PICATO (INGENOL) ...... 109 STRENSIQ (ASFOTASE ALFA) ...... 109 GATTEX (TEDUGLITIDE) ...... 111 RAYOS (PREDNISONE DELAYED RELEASE TABLET) ...... 112 EVZIO (NALOXONE AUTO-INJECTOR) ...... 113 NORTHERA (DROXIDOPA) ...... 114 NATPARA (PARATHYROID HORMONE) ...... 116 MYALEPT (METRELEPTIN) ...... 117 KORLYM (MIFEPRISTONE) ...... 119 INTERLEUKIN 1 ANTAGONISTS ...... 120 FAMILIAL HYPERCHOLESTEROLEMIA ...... 123 HEMLIBRA (EMICIZUMAB-KXWH) ...... 125 GALAFOLD (MIGALASTAT) ...... 128 UREA CYCLE DISORDERS ...... 130 EPIDIOLEX ...... 131 BENLYSTA (BELIMUMAB) ...... 132 APOKYN (APOMORPHINE) ...... 134 ARIKAYCE (AMIKACIN) ...... 135 CARBAGLU (CARGLUMIC ACID) ...... 136 CHENODAL (CHENODIOL) ...... 137 CRYSVITA (BUROSUMAB-TWZA) ...... 138 CYSTADANE (BETAINE) ...... 139 CYSTARAN (CYSTEAMINE) (Cysteamine ophthalmic) (Cystaran) ...... 139 RUZURGI (AMIFAMPRIDINE)/FIRDAPSE (AMIFAMPRIDINE) ...... 140 INCRELEX (MECASERMIN) ...... 141 KUVAN (SAPROPTERIN) ...... 142 LOKELMA (SODIUM ZIRCONIUM CYCLOSILICATE) ...... 143 LUCEMYRA (LOFEXIDINE) ...... 144 IL 5 ANTAGONISTS NUCALA (MEPOLIZUMAB) ...... 145

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 4 Prior Authorization Criteria April 2020 OXERVATE (CENEGERMIN-BKBJ) ...... 146 PALYNZIQ (PEGVALIASE-PQPZ) ...... 147 RAYALDEE (CALDIFEDIOL) ...... 148 TEGSEDI (INOTERSEN) ...... 149 TAFAMIDIS (VYNDAMAX, VYNDAQEL) ...... 150 VECAMYL (MECAMYLAMINE) ...... 151 VELTASSA (PATIROMER) ...... 152 VIBERZI (ELUXADOLINE) ...... 153 LOTRONEX (ALOSETRON) ...... 154 XADAGO (SAFINAMIDE) ...... 155 XERMELO (TELOSTRISTAT ETHYL) ...... 156 XOLAIR (OMALIZUMAB) ...... 157 NEUMEGA (OPRELVEKIN) ...... 158 CYSTEAMINE (CYSTAGON, PROCYSBI) ...... 160 ELMIRON (PENTOSAN POLYSULFATE SODIUM) ...... 161 ZELAPAR (SELEGILINE) ...... 162 XURIDEN (URIDINE TRIACETATE) ...... 163 SIKLOS (HYDROXYUREA) ...... 163 THIOLA (TIOPRONIN) ...... 164 SPRIX (KETOROLAC) ...... 165 SUCRAID (SACROSIDASE) ...... 166 INBRIJA (Levodopa) ...... 167 JYNARQUE (TOLVAPTAN) ...... 168 KEVEYIS (Dichlorphenamide) ...... 171 MOZOBIL (Plerixafor) ...... 172 NUPLAZID (Pimavanserin) ...... 173 PANRETIN (Alitretinoin) ...... 174 SAMSCA (Tolvaptan) ...... 174 SANCUSO (Granisetron) ...... 176 XENLETA (Lefamulin) ...... 177

Blanket Criteria

OFF-LABEL USES Formulary Status: Formulary, PA or Non-formulary Coverage Duration: 1 year Diagnosis Considered for Coverage: • Off-Label indications (medically accepted indications are defined using the following sources: American Hospital Formulary Service-Drug Information (AHFS-DI), Truven Health Analytics Micromedex DrugDEX (DrugDEX), National Comprehensive Cancer Network (NCCN) Drugs and Biologics Compendium, Wolters Kluwer Lexi-Drugs, and Elsevier/Gold Standard Clinical Pharmacology and/or positive results from two peer-reviewed published studies. Prescribing Restriction • Quantity Limit*: not to exceed common off-label dose or dose used in published trials Clinical Information required for Review • Diagnosis • Previous therapy • Supporting documentation Coverage Criteria: I. Initiation of Therapy: • Approve if: o No other formulary medication has a medically accepted use for the patient’s specific diagnosis as referenced in the medical compendia AND o Medication is being requested for an accepted off-label use and is listed in the standard clinical decision support resources (as noted in Diagnosis section above) OR o Requested use can be supported by at least two published peer reviewed clinical studies II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication References: N/A Last review/revision date: 2/2020

STEP THERAPY EXCEPTION Formulary Status: Formulary, Non-Formulary, PA/step required *For drugs without specific criteria Coverage Duration: 1 year Diagnosis Considered for Coverage: • FDA approved indications • See off-label criteria Prescribing Restriction: • Quantity Limit*: As requested not to exceed FDA approved or off-label dose *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Previous therapy • Supporting documentation Coverage Criteria: I. Initiation of Therapy, approve if: o Provider has demonstrated knowledge of step therapy requirements AND o Medical justification why required step therapy drug(s) would be ineffective or have the potential to cause harm or deterioration of the member’s condition OR o Medical justification why the requested drug would be superior to the required prerequisite trail(s) with formulary drug(s) II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication References: N/A Last review/revision date: 02/2020

QUANTITY LIMIT EXCEPTION Formulary Status: Formulary, PA or Non-formulary Coverage Duration: 1 year Diagnosis Considered for Coverage: • FDA approved indications • See off-label criteria Prescribing Restriction: • Quantity Limit: N/A Clinical Information required for Review: • Diagnosis • Previous therapy • Supporting documentation Coverage Criteria: I. Initiation of Therapy, approve if: o Member has a documented treatment failure with the drug prescribed at the quantity limit OR o Member requires a dose within prescribing guidelines that exceeds the quantity limit AND o Medical justification why the plan’s quantity limit will be inadequate based on the member’s condition and treatment history AND o Dose requested is supported by Medical Compendia or current treatment guidelines II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medical justification for continuation of therapy III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Medical justification for continuation of therapy References: N/A Last review/revision date: 02/2020

SAFETY EDIT EXCEPTION Formulary Status: Formulary, PA or Non-formulary *For drugs without specific criteria Coverage Duration: 1 year* *One month approval for duplication of therapy when transitioning from one agent to another. Diagnosis Considered for Coverage: • FDA approved indications • See off-label criteria Prescribing Restriction: • Quantity Limit: N/A Clinical Information required for Review: • Diagnosis • Previous therapy • Concurrent therapy • Dose and duration of therapy • Supporting documentation Coverage Criteria: I. Initiation of Therapy: • For requests exceeding the FDA or compendia max dose, administration frequency or duration of therapy recommendations, approve if: o Patient has documented treatment failure with the drug at the maximum tolerated dose or maximum dose (whichever is the lesser dose), administration frequency or duration of therapy AND o Medical justification why the maximum dose, administration frequency or duration of therapy needs to be exceeded based on the member’s condition or treatment history AND o Patient has tried other therapeutic options which have also failed or are inappropriate AND o Dose requested is supported by the Medical Compendia or current treatment guidelines • For requests for a duplication of therapy o Transition from one agent to another (one month only), approve if: § Provider has outlined a plan to transition member to a similar drug OR § Provider has provided a dose titration schedule o Ongoing concurrent therapy with two similar agents, approve if: § Medical justification why treatment with more than one drug in the same class is required based on the patient’s condition and treatment history OR § Provider has submitted disease state specific standard of care guidelines supporting concurrent therapy • For requests exceeding an age restriction, approve if: o Medical justification why the drug is needed outside age limit o Indication and dose requested are supported by the Medical Compendia or current treatment guidelines II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Medical justification for continuation of therapy

SAFETY EDIT EXCEPTION III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Medical justification for continuation of therapy References: N/A Last review/revision date: 03/2019

ORAL and SELF INJECTABLE ONCOLYTICS Category: Policy Formulary Status: Formulary, PA Drugs included: abiraterone, Afinitor, Afinitor Disperz, Alecensa, Alferon N, Alunbrig, Balversa, bexarotene, Bosulif, Braftovi, Cabometyx, Calquence, Caprelsa, Cometriq, Copiktra, Cotellic, Daurismo, Demser, Emcyt, Erivedge, Erleada, erlotinib, etoposide, Farydak, Gilotrif, Hycamtin, Ibrance, Iclusig, Idhifa, imatinib, Imbruvica, Inrebic, Inlyta, Intron A, Iressa, Jakafi, Kisqali, Lenvima, Lonsurf, Lorbrena, Lynparza, Lysodren, Matulane, Mekinist, Mektovi, Mesnex, Nerlynx, Nexavar, Ninlaro, Nubeqa, Odomzo, Ofev, Piqray, Pomalyst, Rinvoq, Revlamid, Rozlytrek, Rubraca, Rydapt, Sprycel, Stivarga, Sutent, Sylatron, Synribo, Tafinlar, Tagrisso, Targretin, Talzenna, Tasigna, Thalomid, Tibsovo, Tiglutik, tretinoin, Turalio, Tykerb, Tymlos, Valchlor, Venclexta, Verzenio, Vitrakvi, Vizimpro, Votrient, Xalkori, Xospata, Xpovio, Xtandi, Zejula, Zelboraf, Zolinza, Zydelig, Zykadia Coverage Duration: Indefinite Diagnosis Considered for Coverage: • FDA approved indications • Off-Label indications (medically accepted indications are defined using the following sources: American Hospital Formulary Service-Drug Information (AHFS-DI), Truven Health Analytics Micromedex DrugDEX (DrugDEX), National Comprehensive Cancer Network (NCCN) Drugs and Biologics Compendium (evidence rating 2b or greater), Wolters Kluwer Lexi-Drugs, Elsevier/Gold Standard Clinical Pharmacology and/or positive results from two peer-reviewed published studies) Prescribing Restriction • Authorized quantity: 30 day supply • Prescriber restriction: Prescriber must be oncologist or hematologist Clinical Information required for Review • Diagnosis • Dose • Prescriber specialty Coverage Criteria: I. Initiation of Therapy:

ORAL and SELF INJECTABLE ONCOLYTICS Category: Policy Formulary Status: Formulary, PA Drugs included: abiraterone, Afinitor, Afinitor Disperz, Alecensa, Alferon N, Alunbrig, Balversa, bexarotene, Bosulif, Braftovi, Cabometyx, Calquence, Caprelsa, Cometriq, Copiktra, Cotellic, Daurismo, Demser, Emcyt, Erivedge, Erleada, erlotinib, etoposide, Farydak, Gilotrif, Hycamtin, Ibrance, Iclusig, Idhifa, imatinib, Imbruvica, Inrebic, Inlyta, Intron A, Iressa, Jakafi, Kisqali, Lenvima, Lonsurf, Lorbrena, Lynparza, Lysodren, Matulane, Mekinist, Mektovi, Mesnex, Nerlynx, Nexavar, Ninlaro, Nubeqa, Odomzo, Ofev, Piqray, Pomalyst, Rinvoq, Revlamid, Rozlytrek, Rubraca, Rydapt, Sprycel, Stivarga, Sutent, Sylatron, Synribo, Tafinlar, Tagrisso, Targretin, Talzenna, Tasigna, Thalomid, Tibsovo, Tiglutik, tretinoin, Turalio, Tykerb, Tymlos, Valchlor, Venclexta, Verzenio, Vitrakvi, Vizimpro, Votrient, Xalkori, Xospata, Xpovio, Xtandi, Zejula, Zelboraf, Zolinza, Zydelig, Zykadia • Requested indication must be supported by NCCN category 2b or greater evidence rating. If the request is for a lower level of evidence rating, then medical documentation has been provided as to why member is unable to utilize a treatment regimen with a higher level of evidence (e.g. allergic reaction, contraindication) AND • Documentation provided of results of genetic testing where required per drug package insert AND • Documentation provided of results of all required laboratory values and patient specific information (e.g. weight, ALT/AST, creatinine kinase, etc.) when recommended/required per drug package insert AND • Requested quantity does not exceed FDA approved or compendia supported dose AND II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Requested quantity does not exceed FDA approved or standard off-label dose References: • NCCN Guidelines® & Clinical Resources. Development and Update of the NCCN Guidelines® Available at: Last review/revision date: 02/2020

COMPOUNDED MEDICATIONS Formulary Status: Non-Formulary/Prior Authorization required Coverage Duration: Initial: Not to exceed 3 months Reauthorization: 6 months Diagnosis Considered for Coverage: • Diagnosis appropriate for medications contained in the compounded product. Prescriber Restriction • Quantity Limit* 30 day supply *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Current therapy • Other medications that have been used for diagnosis • Comorbidities Coverage Criteria: The plan may authorize coverage of compounded prescription medications with an ingredient cost greater than or equal to $200 when ALL of the following criteria are met: • The indication, therapeutic amount, and route of administration of each of the active ingredients in the coumpound is FDA-approved, or CMS-recognized compendia supported, AND • All of the active ingredients included in the compound are FDA-approved medications (bulk chemicals are not FDA approved), AND • If there are existing clinical coverage criteria for any of the active ingredients, those criteria must also be met for these ingredients, AND • Any one (1) of the following: o There is a current supply shortage of the commercial product, OR o The Member has a medical need for a dosage form or dosage strength that is not commercially available, OR o The Member had a trial and intolerance to or contraindication to the commercially available product (e.g. allergen/preservative/dye-free, palatability for pediatrics, adverse effects to binders/fillers/other active ingredients), OR o The commercial product has been discontinued by the pharmaceutical manufacturer for reasons other than lack of safety or effectiveness Note: All of the active ingredients included in the compound need to be included on the request for authorization References: N/A Last review/revision date: 07/2019

SHORT-ACTING ANALGESIC NARCOTICS Standard/Specific Therapeutic Class: Narcotic Analgesics Formulary Status: • Formulary o codeine tablet (age minimum, 12 yo) o hydromorphone (Dilaudid® ) tablet o morphine sulfate (MS-IR®) tablet o oxycodone (Roxicodone®) tablet o tramadol (Ultram®) 50 mg tablet (age minimum, 18 yo) o codeine phosphate/ acetaminophen (Tylenol w/codeine®) tablet (age minimum, 12 yo) o hydrocodone/acetaminophen (Vicodin®) 2.5-325, 5-325, 7.5-325, 10-325 mg tablet o oxycodone/acetaminophen (Percocet®) 2.5-325, 5-325, 7.5-325, 10-325 mg tablet o oxycodone/aspirin (Percodan®) 4.8355-325 mg tablet o acetaminophen with codeine (Tylenol-Codeine #3®) 300-30 mg tablet (age minimum, 12 yo) o acetaminophen with codeine (Tylenol-Codeine #4®) 300-60 mg tablet (age minimum, 12 yo) o acetaminophen with codeine (Capital with codeine®) 300-15 mg tablet (age minimum, 12 yo) o tramadol/acetaminophen (Ultracet®) 37.5-325 mg tablet (age minimum, 18 yo) o oxymorphone o oxycodone/acetaminophen 5-325 mg/5 ml solution o morphine sulfate 10, 20, 100 mg/5 ml solution o oxycodone 5 mg/5 ml solution o oxycodone 20 mg/ml oral concentrate o morphine sulfate 5, 20, 20, 30 mg suppository o acetaminophen with codeine 120-12 mg/5ml solution (age minimum, 12 yo) o acetaminophen with codeine 120-12 mg oral suspension (age minimum, 12 yo) • Non-formulary o oxycodone/APAP 5/300, 7.5/300, 10/300 mg tab (Primlev®) o hydrocodone/acetaminophen (Xodol®) 5-300, 7.5-300, 10-300 mg tablet; oral solution Coverage Duration: Initial days supply up to 7 days: one-time only Subsequent quantity max #120 per 30 days: for duration requested up to one year Non-formulary drug: for duration requested up to one year Diagnosis Considered for Coverage: • Acute pain • Chronic pain • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit* o Initial fill day supply limit for new starts (no previous opioid claim in the past 180 days): 7 days

SHORT-ACTING ANALGESIC NARCOTICS o Subsequent fill quantity limit: #120 units per 30 days for products listed below: § Codeine tablet (age minimum, 12 yo) § Hydromorphone (Dilaudid®) tablet § Morphine sulfate (MS-IR®) tablet § Oxycodone (Roxicodone®) tablet § Codeine phosphate/ acetaminophen (Tylenol w/codeine®) tablet § Hydrocodone/acetaminophen (Vicodin®) 2.5-325, 5-325, 7.5-325, 10-325 mg tablet § Oxycodone/acetaminophen (Percocet®) 2.5-325, 5-325, 7.5-325, 10-325 mg tablet § Oxycodone/aspirin (Percodan®) 4.8355-325 mg tablet § Acetaminophen with codeine (Tylenol-Codeine #3®) 300-30 mg tablet § Acetaminophen with codeine (Tylenol-Codeine #4®) 300-60 mg tablet § Acetaminophen with codeine (Capital with codeine®) 300-15 mg tablet § Tramadol/acetaminophen (Ultracet®) 37.5-325 mg tablet *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review: • Diagnosis • Dose • Previous therapy Coverage Criteria: I. Initiation of Therapy: • If request is for management of pain due to terminal illness and medication and dose requested is appropriate based on nature and severity of the diagnosis and not likely to cause harm, approve • For requests for short-acting opioid medication over the initial day supply limit of 7, approve if: o Medication is prescribed by a practitioner involved with care of the diagnosis provided AND o If quantity requested exceeds subsequent fill quantity limit, criteria for such a quantity are met: o One of the following: § Member has history of opioid use within the last 180 days OR § Documented by requesting physician if member was on opioids out of state OR § Indication of cancer pain OR § Indication of palliative care OR § Indication of acute pain from a chronic diagnosis (i.e., sickle cell disease) OR § Expected duration of treatment is greater than 7 days based on indication, with documentation of indication and expected duration • For requests for formulary medication over subsequent fill quantity limit, approve if: o Use is short-term (i.e. less than 6 months requested) for post-operative or acute injury pain OR o Indication of chronic cancer pain OR o There is failure with or inability to use long-acting opiates (e.g. morphine sulfate ER tablets) OR

SHORT-ACTING ANALGESIC NARCOTICS o Higher dose is needed as part of a protocol to taper to a lower dose or off long-acting opiates II. Continuation of Therapy for NEW Members (within the last 6 months), refer to "Initiation of Therapy" section III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • For dose increases from previous approval to quantity > #120 per 30 days, criteria for subsequent fill quantity limit are met: o One of the following: § Member has history of opioid use within the last 180 days OR § Documented by requesting physician if member was on opioids out of state OR § Indication of cancer pain OR § Indication of palliative care OR § Indication of acute pain from a chronic diagnosis (i.e., sickle cell disease) OR § Expected duration of treatment is greater than 7 days based on indication, with documentation of indication and expected duration References: N/A Last review/revision date: 07/2019

LONG-ACTING OPIOIDS Therapeutic Class: Analgesics: Opiates, Long-Acting Formulary Status: Formulary: o morphine sulfate ER tablet (MS Contin®) o fentanyl transdermal (Duragesic®) 12, 25, 37.5, 50, 62.5, 75, 87.5, 100 mcg/h o oxycodone ER (Oxycontin®) o morphine sulfate ER caps (Kadian®) o oxymorphone ER o methadone o Nucynta ER® (tapentadol) o Hydrocodone Bitartate ER (Zohydro) • Non-formulary: o Exalgo® (hydromorphone ER) ® o Xtampza ER (oxycodone ER) Coverage Duration: 1 year Diagnosis Considered for Coverage: • Chronic pain • Other diagnoses: follow off-label criteria Prescriber Restriction • Quantity Limit:* o fentanyl: #15 patches per 30 days o morphine sulfate caps, oxycodone ER (Oxycontin® and Xtampza®), oxymorphone ER, Nucynta ER®: #60 per 30 days o methadone: #180 per 30 days (up to 60 mg/day) o hydromorphone ER: #30 tablets per 30 days *NOTE: doses above quantity limits are allowed for cancer pain Clinical Information required for Review • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • If request is for management of pain due to terminal illness and medication and dose requested is appropriate based on nature and severity of the diagnosis and not likely to cause harm, approve • For fentanyl patches, morphine sulfate ER caps, oxycodone ER, approve if: o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use morphine sulfate ER tablets at an adequate (equianalgesic) dose OR

LONG-ACTING OPIOIDS o There is documentation of pain caused by active cancer; AND o For Xtampza ER®, there is documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use Oxycontin® • For methadone, approve if: o Diagnosis of pain o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use the following alternatives AND § short-acting opiates AND § morphine sulfate ER tablets AND one other long-acting opioid at an adequate (equianalgesic) dose o Naloxone has been prescribed for the member • For hydromorphone ER, Nucynta ER®, or oxymorphone ER, approve if: o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use ALL of the following alternatives at an adequate (equianalgesic) dose § Oxymorphone immediate release AND § Morphine sulfate ER tablets or capsules AND § Fentanyl patches AND Oxycodone ER II. Continuation of Therapy for NEW Members (within the last 6 months): • Refer to "Initiation of Therapy" section but allow up to 2 months to transition to preferred agents III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if patient is stable and continuing the medication References: • CDC Guideline for Prescribing Opioids for Chronic Pain — United States, 2016. Recommendations and Reports / March 18, 2016 / 65(1);1–49. Accessed at http://www.cdc.gov/mmwr/volumes/65/rr/rr6501e1.htm • Whalen, J. FDA Advisory Panel Says Benefits of Painkiller Opana ER No Longer Outweigh Risks. Wall Street Journal. March 14, 2017. Available at https://www.wsj.com/articles/fda-advisory-panel-says-benefits-of-painkiller-opana-er-no-longer-outweigh-risks- 1489524063 Last review/revision date: 02/2020

STADOL NS® (BUTORPHANOL) Standard/Specific Therapeutic Class: Narcotic Analgesics Formulary Status: Non-formulary Coverage Duration: 1 year Diagnosis Considered for Coverage: • Acute pain (moderate to severe) or migraine • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit*: #1 unit/30 days • Prescriber restriction: Migraine specialist, neurologist, or pain management specialist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Dose • Previous therapy • Prescriber specialty Coverage Criteria: I. Initiation of Therapy: • For diagnosis of acute pain (moderate to severe) or migraine, approve if: o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e drug interaction, allergy, adverse reaction, etc.) to use the 2 or more pain medications (See lists above in short-acting opioids) AND o Followed by a migraine specialist, neurologist, or pain management specialist II. Continuation of Therapy for NEW Members (within the last 6 months): • Refer to "Initiation of Therapy" section III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication References: • Hoffert MJ, Couch JR, Diamond S, et al. Transnasal Butorphanol in the Treatment of Acute Migraine. Headache. 1995; 35: 65-69. • Silberstein SD. Practice parameter: evidence-based guidelines for migraine headache (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2000;55:754–762. • Stadol Drug Information. Micromedex 2016 • Butorphanol Tartrate Nasal Spray Prescribing Information. Roxane Laboratories, Inc. Revised September 2014. Last review/revision date: 02/2020

LOW MOLECULAR WEIGHT HEPARIN/FACTOR XA INHIBITOR INJECTABLES Standard/Specific Therapeutic Class: Anticoagulant, Heparin and Related, LMWH, Factor Xa Inhibitor Formulary Status: • Formulary o enoxaparin (Lovenox®) o Fragmin® (dalteparin) o fondaparinux (Arixtra®) o Iprivask (desirudin) Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Deep vein thrombosis (DVT), pulmonary embolism (PE), VTE, All FDA indications Prescribing Restriction: • Quantity Limit* o 30 days per fill; quantity is variable depending on patient’s weight and FDA approved dosing guidelines • Prescriber restriction: N/A *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review: • Diagnosis • Dose • Pregnancy status Coverage Criteria: § Initiation of Therapy: o For Fragmin®, fondaparinux, Iprivask: There is documentation of trial and failure, intolerance, contraindication, or inability (i.e drug interaction, allergy, adverse reaction, etc.) to use the enoxaparin

§ Continuation of Therapy for NEW Members (within the last 6 months), approve if: o Patient is stable and continuing the medication AND o Medication is used for appropriate indication and at appropriate dose

§ Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: o Patient is stable and continuing the medication o Dose is appropriate References: N/A Last review/revision date: 02/2020

PCSK-9 INHIBITORS Standard/Specific Therapeutic Class: Lipotropics, Antihyperlipidemic – PCSK-9 inhibitors Formulary Status: • Formulary: Praluent® (alirocumab) Repatha® (evolocumab) Coverage Duration: Initial: 6 months Continuation: Indefinite Diagnosis Considered for Coverage: • Heterozygous Familial Hypercholesterolemia (HeFH), primary hyperlipidemia, homozygous familial hypercholesterolemia (HoFH), primary hyperlipidemia Prescribing Restriction: • Quantity Limit* o Praluent® #2mL per 30 days o Repatha® #2mL per 28 days (140 mg mg/ml every 2 weeks) • Prescriber restriction: Prescriber must be cardiologist or specialist in treatment of lipid disorders *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review: • Diagnosis • Previous therapy • Concurrent therapy • Dose • Lipid levels Coverage Criteria: I. Initiation of Therapy: • For diagnosis of familial hypercholesterolemia (FH), approve if: o 2 fasting lipid panel labs within the past 12 months with abnormal LDL levels ≥190mg/dL for FH in adults or ≥160mg/dL for FH in children; AND o Documentation submitted indicates the patient is a non-smoker; AND o Documented claim history or chart notes showing consistent therapy and trial with one high-intensity statin regimen (atorvastatin 40-80mg or rosuvastatin 20-40mg) with inadequate response still requiring additional LDL lowering, or a documented medical reason (e.g. intolerance, hypersensitivity) for not utilizing high-dose statin; AND

PCSK-9 INHIBITORS o If request indicates that the patient is “statin intolerant”, documentation was provided including description of the side effects, duration of therapy, “wash out”, re-trial, and then change of agents. Patient should have documentation of trial and failure of at least two statin therapies; AND o One of the following applies: - LDL > 400mg/dL with documented strong (1st and 2nd degree relatives) family history of high levels of LDL and/or heart attack and relationship to member; OR - Documented chart notes of clinical manifestations of FH such as xanthomas or aortic valve disease at <20 years of age; OR - Autosomal Dominant Hypercholesterolemia Genetic Testing Reflex Panel (ADHP Panel) with positive genetic testing for LDL raising gene defect or autosomal-recessive FH; OR - Premature coronary artery disease • For diagnosis of primary hyperlipidemia, approve if: o Two fasting lipid panel labs within the past 12 months demonstrate abnormal LDL levels > 70mg/dL; AND o Documentation submitted indicates the patient is a non-smoker; AND o Documented claim history or chart notes showing consistent therapy and trial with one high-intensity statin regimen (atorvastatin 40-80mg or rosuvastatin 20-40mg) with inadequate response still requiring additional LDL lowering, or a documented medical reason (e.g. intolerance, hypersensitivity) for not utilizing high-dose statin; AND o If request indicates that the patient is “statin intolerant”, documentation was provided including description of the side effects, duration of therapy, “wash out”, re-trial, and then change of agents. Patient should have documentation of trial and failure of at least two statin therapies; AND o If ezetimibe is indicated below, it should be tried prior to PCSK9 inhibitor per table below, documentation of trial and failure, intolerance, contraindication, or inability to use ezetimibe Member Age Co-Morbidities LDL Level Treatment Regimen ≥ 21 years old • Stable Clinical ASCVD >70-189mg/dL 1. Add EZETIMIBE to current statin • NO other co-morbidities therapy first 2. Add PCSK9 inhibitor OR replace with PCSK9 inhibitor second ≥ 21 years old • With or Without ASCVD ≥190mg/dL 1. Add EZETIMIBE OR PCSK9 inhibitor • NO other co-morbidities ≥ 21 years old • Clinical ASCVD >70-189mg/dL 1. Add EZETIMIBE OR PCSK9 inhibitor • WITH co-morbidities that increase likelihood of cardiovascular event [Diabetes Mellitus (DM), daily smoker, metabolic syndrome, etc.] 40-75 years old • Diabetes (DM) and without 70-189mg/dL 1. Add EZETIMIBE to current statin ASCVD therapy*

PCSK-9 INHIBITORS • No diabetes with ≥ 7.5% estimated *May also consider bile acid sequestrant 10 year risk for ASCVD NO RECOMMENDATION TO USE PCSK9 inhibitors as they do not have an established role for primary prevention of ASCVD

References: N/A Last review/revision date: 02/2020

PULMONARY HYPERTENSION Standard/Specific Therapeutic Class: Other Antihypertensives, Pulmonary Anti-Hypertension, Endothelin Receptor Antagonists, Prostacyclin-type, Selective C-GMP Phosphodiesterase T5 Inhibitors, Soluble Guanylate Cyclase Stimulators Formulary Status: Formulary: • tadalafil (Adcirca) 20mg oral tablet • Adempas® (riociguat) oral tablet • ambrienstan (Letairis) oral tablet • Opsumit® (macitentan) oral tablet • Remodulin® (treprostinil) vial for infusion • sildenafil (Revatio) 20mg oral tablet • Tyvaso® ampule for nebulized inhalation starter and refill kits • Uptravi® (selexipag) oral tablet and initial titration pack • Ventavis® (iloprost) ampule for nebulized inhalation • epoprostenol • Orenitram® (treprostinil) ER oral tablet • Revatio® (sildenafil) 10mg/mL oral suspension • bosentan (Tracleer) oral tablet • Tyvaso® (treprostinil) 1.74mg/2.9mL amp for nebulizer Coverage Duration: Indefinite Diagnosis Considered for Coverage: • World Health Organization (WHO) group 1 pulmonary hypertension (PAH) and documented functional class II-IV • WHO group 4 pulmonary hypertension (CTEPH) and documented functional class II-IV (Adempas® only) • Other diagnoses, including PH groups 2, 3 and 5: see off-label criteria Prescribing Restriction: • Quantity Limit* o tadalafil: #60 tablets per 30 days o Adempas®: #90 tablets per 30 days o Letairis®: #30 tablets per 30 days o Opsumit®: #30 tablets per 30 days o Remodulin® solution for injection: weight-based dosing dependent on previous treatment o sildenafil: #360 tablets per 30 days (up to 80mg TID) o Tyvaso® Inhalation Starter Kit (NDC 66302-0206-01): #81.2 mL per 28 days, 1 fills only o Tyvaso® Inhalation Refill Kit (NDC 66302-0206-02): #81.2 mL per 28 days o Uptravi® titration pack: § 200mg #140 tablets (first pack, NDC 66215-0602-14) for 28 days

PULMONARY HYPERTENSION § 200-800mg #200 tablets (second pack, NDC 66215-0628-20) for 28 days o Uptravi® tablet (all strengths): #60 per 30 days o Ventavis® neb ampule: #270 mL per 30 days o epoprostenol weight-based dosing, no defined maximum o Orenitram® ER: #60 tablets per 30 days o Revatio® oral suspension: #180 mL per 30 days o Tracleer®: #60 tablets per 30 days o Tyvaso® 1.74mg/2.9mL neb ampule: #81.2 mL per 28 days • Prescriber restriction: Cardiologist or pulmonologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review: • Diagnosis • Previous therapy • Concurrent therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For World Health Organization (WHO) Group 1 pulmonary hypertension, or pulmonary arterial hypertension (PAH), approve if: o Confirmed diagnosis and documentation of functional class (II-IV) by a cardiologist or pulmonologist AND o Request is for initial monotherapy with preferred formulary agent or initial dual therapy with preferred formulary agents of different mechanism of action AND o Other criteria: - If the provider is requesting to switch between formulary agents, then documentation is submitted of intolerance or ineffectiveness of prior/current therapy - If the provider is requesting combination therapy with three agents, then documentation is submitted of an adequate trial of dual therapy with two agents of different mechanism, and patient has been compliant with dual therapy - If the request is for Tracleer®, documentation is submitted of trial and failure, intolerance of, or contraindication to Letairis® AND Opsumit® - If request is for Adcirca®, documentation is submitted of trial and failure, intolerance of, or contraindication to sildenafil oral tablets - If the request is for Revatio® oral suspension, documentation submitted as to why patient cannot use sildenafil oral tablets (i.e., difficulty swallowing)

PULMONARY HYPERTENSION - If the request is for Orenitram®, the patient must have documented failure or inability to use other formulary prostanoids • For WHO Group 4 pulmonary hypertension, or chronic thromboembolic pulmonary hypertension (CTEPH) approve if: o Request is for Adempas® (for other medications, refer to off-label criteria) AND o Confirmed diagnosis and documentation of functional class (II-IV) by a cardiologist or pulmonologist AND o Recurrent or persistent CTEPH following pulmonary thromboendarterectomy OR inoperable CTEPH II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • If dose is being increased, document compliance to prior dose AND • The mediation is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines AND • For Uptravi® continuation requests, documentation is submitted of current dosing and titration schedule III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • The mediation is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines AND • For dose increases, documentation is submitted of patient compliance with prior dose • For Uptravi® continuation requests, documentation is submitted of current dosing and titration schedule References: N/A Last review/revision date: 7/2019

Dupixent® (dupilumab) Specific Therapeutic Class:, systemic interleukin-4 receptor antagonist monoclonal antibody Formulary Status: • Formulary, PA required o Dupixent® (dupilumab) 300 mg/3 ml syringe Coverage Duration: Dupixent®: Initial: 6 months; Renewal: indefinite Diagnosis Considered for Coverage: • Atopic dermatitis (mild, moderate, or severe eczema) • Eosinophilic asthma for add on maintenance therapy of moderate to severe corticosteroid dependent asthma • Rhinosinusitis (chronic) with nasal polyposis as add on therapy • Other diagnoses: see off-label criteria Prescribing Restriction: • Quantity Limit*: Dupixent®: • Atopic dermatitis: #2 syringes for initiation and #1 syringe every other week (300mg syringe) • Asthma: #2 syringes for initiation and #1 syringe every other week (of 200 or 300mg syringes) • Rhinosinusitis: #1 syringe every other week (300mg syringe) • Prescriber restriction: pediatrician or dermatologist (for atopic dermatitis only) Clinical Information Required for Review: • Diagnosis • Previous therapy Coverage Criteria: I.Initiation of Therapy: • For Atopic Dermatitis, approve if: o Age > 18 years AND o Diagnosis of moderate to severe atopic dermatitis AND o Body surface area (BSA) involvement > 10% o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, areas involving face, neck flexural, genital, or intertriginous areas etc.) to use at least 1 medium to high potency topical corticosteroid AND topical calcineurin inhibitor o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use Eucrisa®. o Prescribed dose is 600mg (2 syringes) at initiation and 300mg (1 syringe) every other week • For Asthma, approve if: o Diagnosis of asthma; AND

Dupixent® (dupilumab) o Member has an absolute blood eosinophil count ≥150 cells/mcL within the past 3 months; AND o Prescribed by or in consultation with a pulmonologist, immunologist, or allergist; AND o Age ≥ 12 years; AND o Member is currently corticosteroid dependent o Prescribed dose is 400mg (2 syringes) at initiation and 200mg (1 syringe) every other week OR 600mg (2 syringes) at initiation and 300mg (1 syringe) every other week

• For Rhinosinusitis, approve if: o Age > 18 years AND o Diagnosis of chronic rhinosinusitis AND o Documented presence of nasal polyposis AND o Documented trial and failure or inadequate response to intranasal and oral corticosteroids AND o Prescribed dose is 300mg (1 syringe) every other week

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose AND • If for atopic dermatitis, documentation of improvement in BSA involvement from baseline

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing medication • If for atopic dermatitis, documentation of improvement in BSA involvement from baseline References: N/A Last review/revision date: 02/2020

SORIATANE® (ACITRETIN) Standard/Specific Therapeutic Class: All Other Dermatologicals, Systemic Antipsoriatic Agents Mechanism of Action: Interleukin-6 (IL-6) Inhibitor Formulary Status: • Formulary Soriatane (acitretin) Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Plaque psoriasis, moderate to severe psoriasis • Other diagnoses: see off-label criteria Prescribing Restriction • Quantity Limit*: # 90 per 90 days • Prescriber restriction: Prescriber must be a dermatologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Previous therapy • Concurrent therapy • Dose • Prescriber specialty Coverage Criteria: I. Initiation of Therapy: • For diagnosis of moderate to severe plaque psoriasis, approve if: o Member is 18 years of age or older AND o Prescribed by a dermatologist AND o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e drug interaction, allergy, adverse reaction, etc.) to use topical steroids AND calcipotriene, tazarotene, anthraln, or coal tar OR Failure of cyclosporine, methotrexate or UVB or PUVA therapy II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication References:

SORIATANE® (ACITRETIN) • Menter, Alan et al. Guidelines of care for the management of psoriasis and psoriatic arthritis. Journal of the American Academy of Dermatology, Volume 58 , Issue 5 , 826 – 850 Last review/revision date: 03/2019

CHEMET® (SUCCIMER) Standard/Specific Therapeutic Class: Antidotes; Metallic Poison, Agents to Treat Mechanism of Action: Heavy Metal Chelator Formulary Status: Formulary, PA required Coverage Duration: up to 19 days Diagnosis Considered for Coverage: • Lead poisoning • Other Diagnoses: Follow off-label criteria Prescribing Restriction • Quantity: not to exceed maximum dose (>= 12months Children < 18 years;10 mg/kg/dose (or 350 mg/m2/dose) every 8 hours for 5 days followed by 10 mg/kg/dose (or 350 mg/m2/dose) every 12 hours for 14 days. Maximum: 500 mg/dose) ; (Adults>= 18 years; 10mg-30mg/kg/day for 5 days) Clinical Information required for Review • Diagnosis • Dose

Coverage Criteria: I. Initiation of Therapy: • Diagnosis of lead poisoning and treatment plan by or in consultation with a toxicologist or clinician who has experience with chelating agents AND • Blood lead level (BLL) > 45 mcg/dL in children and > 50 mcg/dL with significant symptoms or > 100 mcg/dL with or without symptoms in adults

II. Continuation of Therapy for NEW or EXISTING members: • Repeat treatment course is required per PA request References: • Centers for Disease Control and Prevention. Managing elevated blood lead levels among young children: Recommendations from the Advisory Committee on Childhood Lead Poisoning Prevention. Atlanta, GA, Centers for Disease Control and Prevention, 2002. http://www.cdc.gov/nceh/lead/CaseManagement/caseManage_main.htm • Lexicomp Online®, Dimercaptosuccinic acid (succimer): Drug information, Hudson, Ohio: Lexi-Comp, Inc. Last review/revision date: 03/2019

DEFERASIROX, JADENU, FERRIPROX, EXJADE Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Metallic Poison Formulary Status: Formulary, PA required Coverage Duration:1 year Diagnosis Considered for Coverage: • Chronic iron overload due to blood transfusions or non-transfusion dependent thalassemia syndromes • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit*: FDA approved dose based on weight • Prescriber restriction: Initially prescribed or being followed by a hematologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Dose • Labs (e.g. serum ferritin level) • Concurrent therapy

DEFERASIROX, JADENU, FERRIPROX, EXJADE Coverage Criteria: I. Initiation of Therapy: • For diagnosis of chronic iron overload due to blood transfusion, approve if: o Patient is between 2 and 65 years of age AND o Patient is transfusion dependent AND o Initially prescribed or being followed by a hematologist AND o Serum ferritin consistently greater than 1000 mcg/L AND o Not being used in combination with other chelator therapies • For diagnosis of chronic iron overload in non-transfusion dependent thalassemia syndromes, approve if: o Patient is 10 years of age or older AND o Diagnosis of thalassemia syndrome AND o Liver iron content (LIC) by liver biopsy of ≥ 5 mg Fe/g dry weight AND o Serum ferritin level on ≥ 2 measurements one month apart of > 300 mcg/L AND o Not being used in combination with other chelator therapies II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Diagnosis of chronic iron overaload due to blood transfusion AND • Serum ferritin is NOT consistently below 500 mcg (if consistently < 500 mcg/L, therapy must be discontinued) III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Diagnosis of chronic iron overaload due to blood transfusion AND • Serum ferritin is NOT consistently below 500 mcg (if consistently < 500 mcg/L, therapy must be discontinued) References: • UCSF Benioff Children’s Hospital. Thallasemia Standard-ofCare Practice Guidelines (2012). Accessed at http://thalassemia.com/treatment-guidelines-5.aspx#gsc.tab=0 • Thalassemia Foundation of Canada and Anemia Institute for Research & Education. Guidelines for the Clinical Care of Patients with Thalassemia in Canada. 2009. Accessed at http://www.thalassemia.ca/wp-content/uploads/Thalassemia-Guidelines_LR.pdf. • Thalassaemia International Foundation. Guidelines for the Management of Non Transfusion Dependent Thalassaemia (NTDT). 2013. Accessed at http://thalassemia.com/documents/NTDT-TIF-guidelines.pdf. Last review/revision date: 03/2019

WILSON’S DISEASE Standard/Specific Therapeutic Class: • Antiarthritics, Anti-Arthritic and Chelating Agent (Cuprimine) • Antidotes; Metallic Poison, Agents to Treat (Syprine, Galzin)

WILSON’S DISEASE Mechanism of Action: Chelating Agents Formulary Status: Formulary, PA required • D-Penicillamine (Cuprimine, Depen, D-Penamine) • Syprine, Clovique (Trientine) • Galzin (Zinc Acetate) Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Wilson’s Disease • Other diagnoses: follow off-label criteria Prescribing Restrictions • Quantity Limits: o Cuprimine (D-Penicillamine) § 125mg: 6 capsules/day (1500 mg/day divided) § 250mg: 6 capsules/day (1500 mg/day divided) o Syprine (Trientine): up to 8 capsules/day (2 g/ day divided) o Galzin (Zinc Acetate): § 25mg: up to 6 capsules/day § 50 mg: up to 3 capsules/day Clinical Information required for Review • Diagnosis • Dose Coverage Criteria: I. Initiation of Therapy, approve if: • Diagnosis is Wilson’s disease AND • For Galzin (Zinc Acetate), patient is undergoing maintenance therapy II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication and medication AND • Medication is used for appropriate indication and at appropriate dose References: • Roberts EA, Scilsky ML. Diagnosis and Treatment of Wilson Disease: An Update. AASLD PRACTICE GUIDELINES. Hepatology June 2008. https://www.aasld.org/sites/default/files/guideline_documents/Wilson%20Disease2009.pdf • http://www.crlonline.com.ucsf.idm.oclc.org/lco/action/doc/retrieve/docid/sfcomb_f/5385704?hl=Chelating%20Agent#f_preparat ions Last review/revision date: 03/2019

TESTOSTERONE REPLACEMENT Standard/Specific Therapeutic Class: Endocrine/Metabolism, Androgens Mechanism of Action: Testosterone analog/replacement Formulary Status: • Formulary, PA required: o Xyosted o Natesto 5.5mg/0.122g nasal gel pump o Striant 30mg buccal tablet o Methyltestosterone (Testred, Android) 10mg cap o Methitest 10mg tablet, Androxy 10mg tablet Coverage Duration • Primary hypogonadism and gender dysphoria (formerly termed gender identity disorder (GID): indefinite • Secondary hypogonadism: o Initial: 6 months o Re-authorization: 1 year Diagnosis Considered for Coverage: • Primary (testicular) hypogonadism (i.e. Klinefelters syndrome, primary failure due to radiation, 5-alpha reductase deficiency, myotonic dystrophy, cryptorchidism, hemochromatosis, mumps orchitis) • Secondary (hypogonadotropic) hypogonadism (i.e. hx of pituitary tumor, panhypopituitarism, d/t high dose opiate use (hypothalamic dysfunction), obesity, Kallmann syndrome, fertile eunuch syndrome) • Gender dysphoria • Other Diagnoses: Follow off-label criteria Prescribing Restriction • Provider Restriction: none • Quantity Limits*: o Topical products to 30 day supply o Methyltestosterone/Methitest/Androxy #30 per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Previous therapy • Dose Coverage Criteria: • Initiation of Therapy: o For diagnosis of hypogonadism approve if:

§ Documentation of low testosterone level on at least 2 blood samples before 10 am (e.g. total testosterone level below lower limit of normal as defined by the laboratory where the test was done OR total testosterone level below 280 ng/dL (9.7 nmol/L) for younger men or below 200 ng/dL (6.9 nmol/L) for symptomatic older men (> 40 y/o)) AND § There is documentation of trial and failure, intolerance, contraindication, or inability to use the 1% and 2% gel products and patches (e.g. Androgel 1.62% is requested) o For diagnosis of Gender Dysphoria (previously termed GID), approve if: § There is documentation of trial and failure, intolerance, contraindication, or inability to use the 1% and 2% gel products and patches (e.g. Androgel 1.62% is requested) • Continuation of Therapy for NEW Members (within the last 6 months), approve if: o For diagnosis of primary hypogonadism and gender dysphoria approve if: § Patient is stable and continuing the medication AND § Medication is used at appropriate dose o For diagnosis of secondary hypogonadism, approve if: § Medication is used at an appropriate dose AND § Testosterone level is within therapeutic range as defined by the laboratory where the test was done OR total testosterone level is above 280 ng/dL (9.7 nmol/L) for younger men or above 200 ng/dL (6.9 nmol/L) for symptomatic older men (> 40 y/o) • Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication): o For diagnosis of primary hypogonadism and gender dysphoria (GID) approve if: § Patient is stable and continuing the medication o For diagnosis of secondary hypogonadism, approve if: § Testosterone level is within therapeutic range as defined by the laboratory where the test was done OR total testosterone level above 280 ng/dL (9.7 nmol/L) for younger men or above 200 ng/dL (6.9 nmol/L) for symptomatic older men (> 40 y/o) References: • Goodman Neil, et al. American Association of Clinical Endocrinologists and American College of Endocrinology Position Statement On The Association Testosterone and Cardiovascular Risk. Endocrine Practice Vol 21 No.9 September 2015. Accessed at http://journals.aace.com/doi/pdf/10.4158/EP14434.PS Last review/revision date: 03/2019

HP ACTHAR® (CORTICOTROPIN) 80 UNITS/ML GEL Standard/Specific Therapeutic Class: Corticotropins, Adrenocorticotrophic Hormones Formulary Status: Formulary, PA required Coverage Duration: Infantile spasms (West syndrome): 4 weeks Acute exacerbation of multiple sclerosis: 3 weeks Nephrotic syndrome: 4 weeks All other FDA approved indications: 4 weeks Diagnosis Considered for Coverage: • Infantile Spasms (West Syndrome) • Acute exacerbation of multiple sclerosis • Nephrotic syndrome • Other diagnoses (e.g. OMS): see off-label criteria Prescribing Restriction • Quantity Limit*: o Infantile spasm (West syndrome): up to 15 ml per 14 days (three 5 ml vials (~80 units per day)) o Nephrotic syndrome: up to 10 ml per 30 days (two 5 ml vials; 80 units twice weekly) o Acute exacerbation of multiple sclerosis: 80 to 120 units/day for 2 to 3 weeks • Diagnosis by a nephrologist for Nephrotic Syndrome • Diagnosis by a neurologist or neonatologist for infantile spasms (West Syndrome) • Diagnosis by a neurologist for multiple sclerosis *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Infantile Spasms (West Syndrome), approve if: o Patient is < 2 years of age (Medi-Cal only) AND o Documentation of patient’s current weight (in kg) and height/length (in cm) or body surface area (BSA) AND o Dose is not to exceed 150 units/m2/day for 2 weeks, followed by a 2-week taper • For diagnosis of Idiopathic or Lupus Erythematosus associated nephrotic syndrome, approve if: o There is documentation of intolerance/side effects with oral corticosteroids that would not also be expected with ACTH AND o There is documentation of trial and failure of or inability to use all other standard therapies AND o Dose does not exceed 80 units per day • For diagnosis of acute exacerbation of multiple sclerosis, approve if: o Documentation patient is currently receiving maintenance treatment for MS AND

HP ACTHAR® (CORTICOTROPIN) 80 UNITS/ML GEL o Documentation was submitted that patient is having acute attack, with neurologic symptoms and increased disability or impairments in vision, strength or cerebellar function, and has failed therapy with corticosteroids, has intolerable side effect or contraindication to corticosteroids not expected to be seen with HP Achtar, or a medical reason has been submitted why patient is unable to use corticosteroids AND o Dose does not exceed 120 units/day for 3 weeks per exacerbation episode. • For all other FDA approved indications, approve if: o There is documentation of intolerable side effect or contraindication to corticosteroids that is not also expected with HP Acthar AND o Documentation was provided that ALL other standard therapies have been used to treat the member’s condition as described in the medical compendium (Micromedex, AHFS, Drug Points, and package insert) as defined in the Social Security Act and/or per recognized standard of care guidelines OR there is a documented medical reason (i.e. medical intolerance, treatment failure, etc.) for why all other standard therapies could not be used to treat the member’s condition AND o Prescriber is a specialist in the condition they are treating. II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • For diagnosis of infantile spasms, documented confirmation of diagnosis of infantile spasm via EEG and prescribed dose follows FDA approved dosing guidelines. • For diagnosis of Idiopathic or Lupus Erythematosus associated Nephrotic Syndrome, provider attestation patient is responding to therapy and prescribed dose follows FDA approved dosing guidelines. • For diagnosis of acute exacerbation of multiple sclerosis, see initiation of therapy criteria. • For all other FDA approved indications, provider attestation patient is responding to therapy and prescribed dose follows FDA approved dosing guidelines. III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • For diagnosis of infantile spasms, documented confirmation of diagnosis of infantile spasm via EEG and prescribed dose follows FDA approved dosing guidelines. • For diagnosis of Idiopathic or Lupus Erythematosus associated Nephrotic Syndrome, provider attestation patient is responding to therapy and prescribed dose follows FDA approved dosing guidelines. • For diagnosis of acute exacerbation of multiple sclerosis, see initiation of therapy criteria. • For all other FDA approved indications, provider attestation patient is responding to therapy and prescribed dose follows FDA approved dosing guidelines. References: • Gipson DS, Massengil SF, Yao L, Nagaraj S, Smoyer WE, Mahan JD, Wigfall D, Miles P, Powell L, Lin JJ, Trachtman H, Greenbaum LA. Management of childhood onset nephrotic syndrome. Pediatrics. 2009 Aug;124(2):747-57. doi: 10.1542/peds.2008-1559. Epub 2009 Jul 27. • Go CY, Mackay MT, Weiss SK, et al. Evidence-based guideline update: medical treatment of infantile spasms. Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2012;78(24):1974. PubMed.

HP ACTHAR® (CORTICOTROPIN) 80 UNITS/ML GEL • Hancock EC, Osborne JP, Edwards SW. Treatment of infantile spasms. Cochrane Database Syst Rev. 2008; PubMed • Niaudet P. Etiology, clinical manifestations, and diagnosis of nephrotic syndrome in children. UpToDate. 7 January 2015.

Last review/revision date: 03/2019

SENSIPAR® (CINACALCET) Standard/Specific Therapeutic Class: Miscellaneous, Calcimimetic, Parathyroid Calcium Enhancer Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Secondary hyperparathyroidism (HPT) in post renal transplant patients OR in patients with chronic kidney disease (CKD) on dialysis • Hypercalcemia in patients with parathyroid carcinoma (PC) • Severe hypercalcemia in patients with primary HPT who are unable to undergo parathyroidectomy • Other indications: see off-label criteria Prescribing Restriction • Quantity Limit*: up to 120 per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Dose • Serum calcium and iPTH levels where appropriate Coverage Criteria: I. Initiation of Therapy: a. For diagnosis of secondary hyperparathyroidism, approve if: i. Patient has CKD and is on dialysis (hemodialysis or peritoneal dialysis) or has posttransplant secondary hyperparathyroidism AND ii. Current serum calcium ≥ 8.4 mg/dL AND iii. Current iPTH levels ≥ 300 pg/ml AND iv. There is documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use phosphate binders (e.g. calcium acetate) AND calcitriol or another Vitamin D analog b. For diagnosis of hypercalcemia with parathyroid carcinoma, approve if serum calcium level ≥ 10.2 mg/dL c. For diagnosis of hypercalcemia in primary hyperparathyroidism, approve if: i. Patient is unable to undergo parathyroidectomy AND

ii. There is documentation of severe hypercalcemia and current serum calcium levels >12.5 mg/dL II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: a. Patient has had response to therapy AND b. Patient does not have hypocalcemia (serum calcium less than the lower limit of normal range) AND c. Medication is used for an appropriate indication and at an appropriate dose References: • Moe SM, Chertow GM, Coburn JW, et al. Achieving NKF-K/DOQI bone metabolism and disease treatment goals with cinacalcet HCl. Kidney Int. 2005;67(2):760 PubMed • Messa P, Macario F, Yaqoob M, et al. The OPTIMA study: assessing a new cinacalcet (Sensipar/Mimpara) treatment algorithm for secondary hyperparathyroidism. Clin J Am Soc Nephrol. 2008;3(1):36. PubMed • Arenas MD, Alvarez-Ude F, Gil MT, et al. Implementation of 'K/DOQI Clinical Practice Guidelines for Bone Metabolism and Disease in Chronic Kidney Disease' after the introduction of cinacalcet in a population of patients on chronic haemodialysis. Nephrol Dial Transplant. 2007;22(6):1639. PubMed Last review/revision date: 03/2020

NON-FORMULARY TEST STRIPS/QUANTITY LIMIT EXCEPTION Standard/Specific Therapeutic Class: Medical Supplies/Diabetic Supplies Formulary Status: • Formulary: o One Touch • Non-Formulary, PA required: o Breeze and Contour o FreeStyle Libre reader and sensor o All other brands of Blood Glucose Meetings Coverage Criteria: I. Initiation of Therapy: • For One Touch, If over formulary quantity limit approve if: o If over formulary quantity limit, approve if there is medical need for glucose monitoring more frequent than 4 times daily, or 8 times daily in the case of gestational diabetes. Eg. Frequent hospitalizations, incidents of hypoglycemia, DKA hospitalizations etc. • For Breeze or Contour test strips, approve if: o There is documented trial and failure with One Touch branded test strips manufactured by LifeScan OR o If the patient is stabilized on an insulin pump where it is medically necessary to use a nonpreferred diabetic test strip. AND o If over formulary quantity limit, approve if there is medical need for glucose monitoring more frequent than 4 times daily, or 8 times daily in the case of gestational diabetes. Eg. Frequent hospitalizations, incidents of hypoglycemia, DKA hospitalizations etc. • For FreeStyle Libre reader/sensor system, approve if: o There is documented trial and failure with One Touch branded test strips manufactured by LifeScan OR o If the patient is stabilized on an insulin pump where it is medically necessary to use a nonpreferred diabetic test strip. AND o If over formulary quantity limit, approve if there is medical need for glucose monitoring more frequent than 4 times daily, or 8 times daily in the case of gestational diabetes. Eg. Frequent hospitalizations, incidents of hypoglycemia, DKA hospitalizations etc.

• For All other strips, approve if: o There is documented trial and failure with One Touch branded test strips manufactured by LifeScan OR o If the patient is stabilized on an insulin pump where it is medically necessary to use a nonpreferred diabetic test strip. AND o If over formulary quantity limit, approve if there is medical need for glucose monitoring more frequent than 4 times daily, or 8 times daily in the case of gestational diabetes. Eg. Frequent hospitalizations, incidents of hypoglycemia, DKA hospitalizations etc.

NON-FORMULARY BLOOD GLUCOSE METERS Standard/Specific Therapeutic Class: Medical Supplies/Diabetic Supplies Formulary Status: • Formulary: o One Touch • Non-formulary: all other blood glucose meters Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Diabetes Prescribing Restriction: • Quantity Limit*: 1 unit per year (365 days) *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review: • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • Approve if there is documentation of trial and failure of both Formulary options or inability to use a formulary blood glucose meter (e.g. Prodigy Voice Blood Glucose Meter is needed due to visual impairment) References: N/A Last review/revision date: 03/2020

GROWTH HORMONE Standard/Specific Therapeutic Class: Other Hormones, Growth Hormones Formulary Status: • Formulary, PA required: Norditropin®, Norditropin Flexpro®, Norditropin NordiFlex® • Non-formulary, PA required: Genotropin®, Humatrope®, Nutropin®, Nutropin AQ®, Nutropin AQ NuSpin®, Omnitrope®, Saizen®, Serostim®, Zorbtive®, Zomacton® Coverage Duration: Indication Initial Therapy Re-authorization Pediatric growth hormone deficiency (GHD) 6 months 1 year Growth Failure due to Chronic Renal Insufficiency 1 year 1 year Short stature associated with Turner Syndrome and Prader-Willi Syndrome 6 months 1 year HIV/AIDS-wasting syndrome 6 months 1 year Short Bowel Syndrome 4 weeks Not approvable Diagnosis Considered for Coverage: • Pediatric growth hormone deficiency (GHD) • Growth Failure due to Chronic Renal Insufficiency • Short stature associated with Turner Syndrome and Prader-Willi Syndrome • Adult growth hormone deficiency • HIV/AIDS-wasting syndrome • Short Bowel Syndrome • Other Diagnoses: follow off-label criteria Excluded Diagnoses: • Idiopathic Short Stature (i.e. not GH-deficient short stature) Prescriber Restriction • Endocrinologist or nephrologist for certain indications. See diagnosis specific criteria below Clinical Information required for Review • Diagnosis • Relevant labs (height, weight, etc.) Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Pediatric growth hormone deficiency (GHD) approve if: o Medication is being prescribed by an endocrinologist or pediatric endocrinologist AND o The diagnosis has been confirmed as subnormal GH response < 10 mg/mL by at least one provocative stimulation test (i.e., insulin-induced hypoglycemia, arginine, ARG-GHRH, ARG-LDOPA, GHRH) AND o Diagnosis has been confirmed by one of the following:

GROWTH HORMONE - Severe short stature (defined as patient’s height at ≥ 2 standard-deviation [SD] below the population mean) OR - Height velocity < 25th percentile OR - Patient’s height ≥ 1.5 SD below the midparental height (avg of mother’s and father’s heights) OR - Patient’s height ≥ 2 SD below the mean and a 1-year height velocity more than 1 SD below the mean for chronologic age or (in children 2 years of age or older) a 1- year decrease of more than 0.5 SD in height OR - In the absence of short stature, a 1-year height velocity more than 2 SD below the mean or a 2-year height velocity more than 1.5 SD below the mean (may occur in GHD manifesting during infancy or in organic, acquired GHD) OR - Signs indicative of an intracranial lesion OR - Signs of multiple pituitary hormone deficiencies OR - Neonatal symptoms and signs of GHD AND o Patient’s epiphysis has NOT closed (as confirmed by radiograph of the wrist and hand) or patient has NOT reached final height AND o Weight-based dosing within FDA approved range (weight must be provided on PA request) AND o If the request is not for Norditropin, the provider submitted a documented medical reason (i.e. intolerance) why it is medically necessary to utilize some other brand of growth hormone. • For diagnosis of Growth Failure due to Chronic Renal Insufficiency approve if: o Medication is being prescribed by a nephrologist or endocrinologist AND o Patient’s epiphysis has NOT closed (as confirmed by radiograph of the wrist and hand) AND o Patient’s height at is ≥ 2 standard-deviations (SD) below the mean height for normal children of the same age and gender AND o Weight-based dosing within FDA approved range (weight must be provided on PA request) • For diagnosis of Short stature associated with Turner Syndrome and Prader-Willi Syndrome approve if: o Medication is being prescribed by an endocrinologist or pediatric endocrinologist AND o Patient has short stature as defined as ONE of the following: - For Turner’s Syndrome, height is below the 5th percentile of normal growth curve OR - For Prader Willi Syndrome, height at ≥ 2 standard-deviation (SD) below the mean height for normal children of the same age and gender AND o Patient’s epiphysis has NOT closed (as confirmed by radiograph of the wrist and hand) OR the patient has NOT reached final height AND o Weight-based dose within FDA approved range (weight must be provided on PA request) AND

GROWTH HORMONE o If the request is for Turner Syndrome and is not for Norditropin, the provider submitted a documented medical reason (i.e. intolerance) why it is medically necessary to utilize some other brand of growth hormone. • For diagnosis of Adult Growth Hormone Deficiency approve if: o Medication is being prescribed by an endocrinologist AND o For hypopituitarism due to pituitary disease, hypothalamic disease, surgery, radiation therapy or trauma, diagnosis has been confirmed by at least one subnormal provocative stimulation test (i.e., insulin-induced hypoglycemia, arginine, ARG-GHRH, ARG-LDOPA) OR o For childhood-onset growth hormone deficiency (GHD), patient has childhood-onset growth hormone deficiency (GHD) due to organic diseases (e.g. craniopharyngioma) AND o Weight-based dose within FDA approved range (weight must be provided on PA request) AND o If the request is for Turner Syndrome and is not for Norditropin, the provider submitted a documented medical reason (i.e. intolerance) why it is medically necessary to utilize some other brand of growth hormone. • For diagnosis of HIV/AIDS-wasting syndrome approve if: o Patient is on antiviral therapy AND o Patient meets at least one of the following: - 10% unintentional weight loss over 12 months - 7.5% unintentional weight loss over 6 months - 5% body cell mass (BCM) loss within 6 months - In men: BCM < 35% of total body weight and body mass index (BMI) < 27kg/m2 - In women: BCM < 23% of total body weight and BMI < 27 kg/m2 - BMI < 20kg/m2 AND o Patient has had an inadequate response to previous therapy (i.e., exercise training, nutritional supplements, appetite stimulants or anabolic steroids) AND o Weight-based dose within FDA approved range (weight must be provided on PA request) • For diagnosis of Short Bowel Syndrome approve if: o Patient is currently on specialized nutritional support (i.e. consisting of high carbohydrate, low-fat diet) AND o Weight-based dose within FDA approved range (weight must be provided on PA request) II. Continuation of Therapy for NEW Members (within the last 6 months) or EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • For pediatric growth hormone deficiency (GHD), Growth Failure due to Chronic Renal Insufficiency, Short stature associated with Turner Syndrome and Prader-Willi Syndrome, approve if: o There is documented response to growth hormone therapy (e.g. IGF-1 level normalization, increase in height velocity defined by >2cm/year compared to that of previous year) AND o Weight-based dose within FDA approved range (weight must be provided on the PA request)

GROWTH HORMONE • For HIV/AIDS-wasting syndrome, approve if: o Therapeutic response per PA request with attached clinic notes showing an increase in muscle mass and weight from growth hormone replacement therapy AND o Weight-based dose within FDA approved range (weight must be provided on the PA request) • For Short Bowel Syndrome, approve if: o Not approvable (administration for more than 4 weeks has not been adequately studied). References: • Bang P, Bjerknes R, Dahlgren J, et al. A comparison of different definitions of growth response in short prepubertal children treated with growth hormone. Hormone Research in Paediatrics. 2011; 75:335–45. • Bondy CA. Clinical Practice Guideline: Care of Girls and Women with Turner Syndrome: A Guideline of the Turner Syndrome Study Group. Journal of Clinical Endocrinology & Metabolism. 2007; 92(1):10-25. • Cayir A, Kosan C. Growth Hormone Therapy in Children with Chronic Renal Failure. Eurasian J Med. 2015 Feb; 47(1): 62–65. • Cook DM, Yuen KCJ, Biller BMK, et al. American association of clinical endocrinologists medical guidelines for clinical practice for growth hormone use in growth hormone-deficient adults and transition patients – 2009 update. Endocrine Practice. 2009; 15(2):1-29. • Haymond M, Kappelgaard AM, Czernichow P, et al. Early recognition of growth abnormalities permitting early intervention. Acta Paediatr. 2013 Aug; 102(8): 787–796. • Mahesh S, Kaskel F. Growth hormone axis in chronic kidney disease. Pediatr Nephrol. 2008 Jan; 23(1): 41–48. • Murray PG, Dattani MT, Clayton PE. Controversies in the diagnosis and management of growth hormone deficiency in childhood and adolescence. Archives of Disease in Childhood. 2016; 101:96-100. • Nwosu B, Lee M. Evaluation of Short and Tall Stature in Children. Am Fam Physician. 2008 Sep 1; 78(5):597-604. • Rose SR, Cook DM, Fine MJ. Growth Hormone Therapy Guidelines: Clinical and Managed Care Perspectives. American Journal of Pharmacy Benefits. 2014;6(5):e134-e146. • Savage MO, Bang P. The variability of responses to growth hormone therapy in children with short stature. Indian J Endocrinol Metab. 2012 Dec; 16(Suppl 2): S178–S184. • Wit JM, Ranke MB, Albertsson-Wikland K, et al. Personalized Approach to Growth Hormone Treatment: Clinical Use of Growth Prediction Models. Hormone Research in Paediatrics. 2013; 79(5):257-70. Last review/revision date: 03/2019

EGRIFTA® (TESAMORELIN INJECTION) Standard/Specific Therapeutic Class: Other Hormones, Growth Hormone Releasing Hormone (GNRH) and Analogs Formulary Status: Non-formulary Coverage Duration: 6 months Diagnosis Considered for Coverage: • HIV-Associated Visceral Adipose Tissue (VAT) Lipodystrophy

EGRIFTA® (TESAMORELIN INJECTION) Prescribing Restriction • Prescriber restriction: endocrinologist or HIV specialist • Quantity Limit: 60 vials of 1mg Egrifta OR 30 vials of 2mg EgriftaTM per 30 day supply Clinical Information Required for Review • Diagnosis • Previous therapy • Concurrent therapy • Dose Coverage Criteria: I. Initiation of Therapy: a. For the reduction of excess abdominal fat in patients diagnosed with HIV-associated lipodystrophy, approve if: i. Patient is 18 years of age or older AND ii. Documentation of CT scan indicating excess visceral fat accumulation OR waist circumference ≥ 95cm (37.4 in) for men or ≥ 94cm (37.0 in) for women AND a waist-to hip ratio of ≥ 0.94 for men or ≥ 0.88 for women AND iii. Physician attests patient does not currently have active malignancy and does not have disruption of the hypothalamic-pituitary axis AND iv. Drug is being requested at an FDA approved dose II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: i. Documentation of clinical response based on decrease in waist circumference OR reduction in visceral adipose tissue on CT scan AND ii. Patient is adherent to therapy AND iii. Drug is being requested at an FDA approved dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: i. Documentation of clinical response based on decrease in waist circumference OR reduction in visceral adipose tissue on CT scan AND ii. Patient is adherent to therapy AND iii. Drug is being requested at an FDA approved dose References: • Egrifta [package insert]. Rockland, MA: EMD Serono, Inc.; December 2014. • Falutz J, Potvin D, Mamputu JC, et al. Effects of Tesamorelin, a Growth Hormone-Releasing Factor, in HIV-Infected Patients With Abdominal Fat Accumulation: A Randomized Placebo Controlled Trial With a Safety Extension. J Acquir Immune Defic Syndr. 2010; 53(3):311. • Stanley TK, Falutz J, Marsolais C, et al. Reduction in visceral adiposity is associated with an improved metabolic profile in HIV- infected patients receiving tasmorelin. Clinical Infectious Diseases. 2012; 54(11):1642-51. Last review/revision date: 03/2019

OCTREOTIDE AND SOMAVERT® Standard/ Specific Therapeutic Class: Other Hormones, Somatostatic Agents Formulary Status: • Formulary, PA required o Octreotide acetate (Sandostatin®) 200, 1000 mcg/ml vial o Octreotide acetate 50, 100, 500 mcg/ml syringe o Octreotide acetate 50, 100, 500 mcg/ml vial o Octreotide acetate 50, 100, 500 mcg/ml ampule o Somavert® Coverage Duration • Initial: 6 months • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Acromegaly • Metastatic carcinoid tumors, management of symptoms associated (diarrhea and flushing) • Vasoactive intestinal peptide-secreting tumors (VIPomas), treatment of profuse watery diarrhea associated with VIPomas • Other indications: see off-label criteria Prescribing Restriction • Quantity Limit* up to 1,500 mcg daily • Prescriber restriction: Prescriber is an endocrinologist or oncologist. *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Dose Coverage Criteria: I. Initiation of Therapy: a. For diagnosis of acromegaly and request is for octreotide, approve if, documentation of baseline IGF-1 is provided with PA request b. For diagnosis of acromegaly and request is for Somavert, approve if, i. Documentation of failure to respond to surgery or radiation OR patient is not a candidate for surgery or radiation AND

ii. Documentation of trial and failure to respond to or intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use octreotide. c. For diagnosis of metastatic carcinoid tumors and associated symptoms of diarrhea and flushing, approve. d. For diagnosis of VIPomas and associated profuse watery diarrhea, approve. II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: a. For acromegaly, patient is stable, tolerating and responding to medication and IGF-1 level has decreased or normalized from baseline OR b. For all other diagnoses, patient is stable, tolerating and responding to medication and there is continued medical justification for continuation of therapy. III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: a. For acromegaly, patient is stable, tolerating, and IGF-1 level has decreased or normalized from baseline. b. For all other diagnoses, patient is stable, tolerating and responding to medication and there is continued medical justification for continuation of therapy. References: • Katznelson L, Atkinson JLD, Cook DM, Ezzat SZ, Hamarahian AH, Miller KK. American Association of Clinical Endocrinologists medical guidelines for clinical practice for the diagnosis and treatment of acromegaly – 2011 update. Endocr Pract.2011;17(Suppl 4):1-44. Available at: https://www.aace.com/publications/guidelines Last review/revision date: 03/2019

FORTEO® (TERIPARATIDE) Standard/Specific Therapeutic Class: Miscellaneous, Bone Formation Stimulation Agents, Parathyroid Hormone Formulary Status: Non-formulary, PA Required Coverage Duration: 2 years Diagnosis Considered for Coverage: • Osteoporosis • Other diagnoses: see off-label criteria Prescribing Restriction • Quantity Limit*: #2.4 ml per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Previous therapy • Dose • T-score • Fracture history Coverage Criteria: I. Initiation of Therapy: • Approve if: o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e. inability to swallow, drug interaction, allergy, adverse reaction, etc.) to use at least one bisphosphonate AND o T-score < 2.5 OR T-score -1.0 and -2.5 with high risk of facture or history of fracture II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose AND • Total length of therapy does not exceed 2 years III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Medical justification provided for continuation of therapy beyond 2 years References: • Camacho PM, Petak SM, Binkley N, et al. American association of clinical endocrinologists and American college of endocrinology clinical practice guidelines for the diagnosis and treatment of postmenopausal osteoporosis – 2016. Endocrine Practice. 2016;22:Suppl 4;1-42. Available at: https://www.aace.com/publications/guidelines Last review/revision date: 03/2019

PROLIA® AND XGEVA® (DENOSUMAB) Standard/Specific Therapeutic Class: Miscellaneous, Bone Resorption Inhibitors Formulary Status: Formulary, Prior Authorization Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Prolia®: Osteoporosis; bone loss due to medication therapy (e.g. androgen deprivation medications or aromatase inhibitors) • Xgeva®: hypercalcemia of malignancy, prevention of skeletal-related events in bone metastases from solid tumors, treatment of giant cell tumor of the bone Prescribing Restriction • Quantity Limit*: o Prolia: #1ml per 180 days (6 months) o Xgeva: #1.7ml per 28 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Previous therapy • Dose • T-score • Fracture history Coverage Criteria: I. Initiation of Therapy: • For Prolia®, approve if: o Diagnosis is FDA approved (see “Diagnosis Considered for Coverage” above) AND o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e. inability to swallow, drug interaction, allergy, adverse reaction, etc.) to use bisphosphonates AND o T-score < 2.5 OR T-score -1.0 and -2.5 with high risk of fracture or history of fracture • For Xgeva®, approve if: o Diagnosis is FDA approved indication (see “Diagnosis Considered for Coverage” above) II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: • Camacho PM, Petak SM, Binkley N, et al. American association of clinical endocrinologists and American college of endocrinology clinical practice guidelines for the diagnosis and treatment of postmenopausal osteoporosis – 2016. Endocrine Practice. 2016;22:Suppl 4;1-42. Available at: https://www.aace.com/publications/guidelines Last review/revision date: 03/2019

EVENITY (ROMOSOZUMAB) Standard/Specific Therapeutic Class: Sclerostin Inhibitor, Monoclonal Antibody Formulary Status: Formulary, Prior Authorization Coverage Duration: 12 months Diagnosis Considered for Coverage: • Evenity®: Osteoporosis in postmenopausal females at high risk for fractures, patients who have failed or are intolerant to other osteoporosis therapies. Prescribing Restriction • Quantity Limit*: o Evenity: #2.34ml (as #2 1.17ml Syringes) per 30 days (1 months)

*Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Previous therapy • Dose • T-score • Fracture history Coverage Criteria: III. Initiation of Therapy: • For Evenity®, approve if: o Osteoporosis in postmenopausal females at high risk for fractures (defined as a history of osteoporotic fracture or multiple risk factors for fracture) OR o Patients who have failed or are intolerant to other osteoporosis therapies - There is documentation of trial and failure, intolerance, contraindication, or inability (i.e. inability to swallow, drug interaction, allergy, adverse reaction, etc.) to use bisphosphonates and denosumab AND o T-score < 2.5 OR T-score -1.0 and -2.5 with high risk of fracture or history of fracture IV. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: • Langdahl BL, Libanati C, Crittenden DB, et al. Romosozumab (sclerostin monoclonal antibody) versus teriparatide in postmenopausal women with osteoporosis transitioning from oral bisphosphonate therapy: a randomised, open-label, phase 3 trial. Lancet 2017; 390:1585. • Cosman F, Crittenden DB, Adachi JD, et al. Romosozumab Treatment in Postmenopausal Women with Osteoporosis. N Engl J Med 2016; 375:1532. • Saag KG, Petersen J, Brandi ML, et al. Romosozumab or Alendronate for Fracture Prevention in Women with Osteoporosis. N Engl J Med 2017; 377:1417. • Camacho PM, Petak SM, Binkley N, et al. American association of clinical endocrinologists and American college of endocrinology clinical practice guidelines for the diagnosis and treatment of postmenopausal osteoporosis – 2016. Endocrine Practice. 2016;22:Suppl 4;1-42. Available at: https://www.aace.com/publications/guidelines Last review/revision date: 02/2020

ZAVESCA® (MIGLUSTAT) AND CERDELGA® (ELIGLUSTAT TARTRATE) Standard/Specific Therapeutic Class: Miscellaneous, Drugs To Tx Gaucher Dx-Type 1, Substrate Reducing Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Gaucher disease, type 1 • Other indications: see off-label criteria Prescribing Restriction • Prescriber restriction: endocrinologist • Quantity Limit: o Zavesca®: up to #90 per 30 days o Cerdelga®: #60 per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information Required for Review • Diagnosis • Dose • For Zavesca®: previous therapy • For Cerdelga®: CYP2D6 genotype Coverage Criteria: I. Initiation of Therapy: a. For Zavesca®, approve if: i. Diagnosis is Gaucher disease, type 1 AND ii. Therapy is prescribed or recommended by an endocrinologist AND iii. There is documentation of trial and failure, intolerance, contraindication, or inability (e.g. due to allergy, hypersensitivity, or poor venous access, etc.) to use enzyme replacement therapy (ERT) (e.g. Cerezyme®) b. For Cerdelga®, approve if: i. Diagnosis is Gaucher disease, type 1 AND ii. Therapy is prescribed or recommended by an endocrinologist AND

ZAVESCA® (MIGLUSTAT) AND CERDELGA® (ELIGLUSTAT TARTRATE) iii. There is documentation of an FDA-cleared test indicating that member is CYP2D6 extensive metabolizers (EMs), intermediate metabolizers (IMs), or poor metabolizer (PMs) II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: a. Patient is stable and continuing the medication AND b. Medication is used for appropriate indication and at appropriate dose References: • Egrifta [package insert]. Rockland, MA: EMD Serono, Inc.; December 2014. • Falutz J, Potvin D, Mamputu JC, et al. Effects of Tesamorelin, a Growth Hormone-Releasing Factor, in HIV-Infected Patients With Abdominal Fat Accumulation: A Randomized Placebo Controlled Trial With a Safety Extension. J Acquir Immune Defic Syndr. 2010; 53(3):311. • Stanley TK, Falutz J, Marsolais C, et al. Reduction in visceral adiposity is associated with an improved metabolic profile in HIV- infected patients receiving tasmorelin. Clinical Infectious Diseases. 2012; 54(11):1642-51. Last review/revision date: 03/2019

EMFLAZA® (DEFLAZACORT) Standard Therapeutic Class, Specific Therapeutic Class: Glucocorticoids Formulary Status: Formulary, PA required Coverage Duration: 6 months Diagnosis Considered for Coverage: • Duchenne Muscular Dystrophy • Other indications – follow off-label criteria Prescribing Restriction • Quantity Limit*: up to 0.9 mg/kg daily • Prescriber restriction: Prescriber must a neurologist. *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: a. For diagnosis of Duchenne Muscular Dystrophy, approve if: i. Patient is 5 years of age or older AND ii. Documented mutation of dystrophin gene and copies of testing were submitted with request AND

EMFLAZA® (DEFLAZACORT) iii. Patient has onset of weakness before 5 years of age, and serum creatinine kinase activity of at least 10 times the upper limit of normal (ULN) at some stage in their illness AND iv. Patient is ambulatory AND v. Patient has had a baseline eye examination AND vi. Patient has had a baseline behavioral health evaluation AND vii. Patient had a baseline bone mineral density (BMD) screening completed (including date and results) AND viii. Patient is or will be taking adequate calcium and vitamin D supplementation AND ix. If patient has been previously established on deflazacort before available in the U.S., provider has submitted detailed chart notes including dates of therapy and response AND x. There is documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use the following formulary alternatives: prednisone or prednisolone for at least 12 months AND xi. Documented medical reason provided why prednisone or prednisolone are not able to be continued, and Emflaza would be medically necessary and not have the same side effect as the preferred agents AND xii. The request is for an FDA approved dose. II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: a. The patient is ambulatory AND b. Physician attests that the patient’s muscle strength has stabilized or improved since the starting treatment AND c. Patient’s prior claim history shows consistent therapy (monthly fills) AND d. Physician attests patient has had repeat eye and BMD screening as appropriate AND e. The request is for an FDA approved dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: a. The patient is ambulatory AND b. Physician attests that the patient’s muscle strength has stabilized or improved since the starting treatment AND c. Patient’s claim history shows consistent therapy (monthly fills) AND d. Physician attests patient has had repeat eye and BMD screening as appropriate AND e. The request is for an FDA approved dose. References: • Gloss D, et al. Practice guideline update summary: Corticosteroid treatment of Duchenne muscular dystrophy. Report of the Guideline Development Subcommittee of the American Academy of Neurology. Neurology. 2016;86:465-472 • Emflaza® [package insert] Northbrook, IL: Marathon Pharmaceuticals; 2017. Last review/revision date: 03/2019

OXANDROLONE (OXANDRIN®) Standard/Specific Therapeutic Class: Anabolics, Androgenic agents Mechanism of Action: Synthetic testosterone derivative with similar androgenic and anabolic actions Formulary Status: • Formulary, PA required: oxandrolone (Oxandrin®) Coverage Duration: 4 weeks Diagnosis Considered for Coverage: • Weight loss following extensive surgery, chronic infections, or severe trauma • To offset protein catabolism with prolonged corticosteroid administration • Relief of bone pain associated with osteoporosis • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit*: Up to 2.5-20 mg in divided doses 2-4 times daily based on individual response. *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Dose Coverage Criteria: I. Initiation of Therapy: • For oxandrolone, approve if: o Must have one of the following diagnosis: - Used as an adjunctive therapy to promote weight gain following: o Extensive Surgery o Chronic Infection o Severe Trauma OR - Therapy to offset protein catabolism associated with long-term use of corticosteroids, OR - Treatment of bone pain associated with osteoporosis • Continuation of Therapy for NEW Members (within the last 6 months), approve if: o For diagnosis of extensive surgery, chronic infection, severe trauma, or bone pain associated with osteoporosis, approve if there is documented therapeutic response and continued medical need per PA request o For diagnosis of therapy to offset catabolism associated with long-term use of corticosteroids, approve if there is documented response to therapy AND patient is still on corticosteroids II. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if:

OXANDROLONE (OXANDRIN®) o For diagnosis of extensive surgery, chronic infection, severe trauma, or bone pain associated with osteoporosis, approve if there is documented therapeutic response and continued medical need per PA request o For diagnosis of therapy to offset catabolism associated with long-term use of corticosteroids, approve if there is documented response to therapy AND patient is still on corticosteroids References: N/A Last review/revision date: 3/2019

CONSTIPATION AGENTS Standard/Specific Therapeutic Class: Laxatives/Laxatives and Cathartics, Antidotes/Mu-opioid Receptor Peripherally Acting Formulary Status: • Formulary, PA required: o Relistor® (methylnaltrexone) tablet, SQ injection Coverage Duration: 1 year Diagnosis Considered for Coverage: • Relistor®: opioid induced constipation (OIC) with chronic non-cancer pain, OIC with advanced illness Prescribing Restriction: • Quantity Limit*: o Relistor®: § For OIC with chronic non-cancer pain: #90 tabs per 90 days, 54 ml per 90 days (12 mg/0.6 ml daily) § For OIC with advanced illness: #36-54 ml per 90 days (8 mg/0.4 ml or 12 mg/0.6 ml daily) *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review: • Diagnosis • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • Approve if: o Diagnosis is appropriate for the medication (see “Diagnosis Considered for Coverage” section above) AND

CONSTIPATION AGENTS o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use at least 2 laxatives from different classes (e.g. fiber supplements, stimulants, osmotic laxatives, etc.) AND o For Relistor®, there is documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use of all three: Movantik®, Symproic® and Amitiza® II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication References: N/A Last review/revision date: 03/2019

CHOLBAM® (CHOLIC ACID) Standard Therapeutic Class, Specific Therapeutic Class: Bile therapy, bile salts Mechanism of Action enhances bile flow and provides the physiologic feedback inhibition of bile acid synthesis to maintain bile acid homeostasis. Formulary Status: • Formulary, PA required o Cholbam® Coverage Duration Initial: 3 months Re-authorization: 6 months Diagnosis Considered for Coverage: • Bile acid synthesis disorder due to single enzyme defect, peroxisomal disorders including Zellweger spectrum disorders in patients that exhibit manifestations of liver disease, steatorrhea or complications from decreased fat- soluble vitamin absorption. • All other medically accepted indications (medically accepted indications are defined using the following sources: The Food and Drug Administration (FDA), Micromedex, American Hospital Formulary Service (AHFS), United States Pharmacopeia Drug Information for the Healthcare Professional (USP DI), and the Drug Package Insert)

CHOLBAM® (CHOLIC ACID) Prescribing Restriction • Quantity Limit*: Up to 10 to 15 mg/kg (once daily or in 2 divided doses) or up to 11 to 17 mg/kg (once daily or in 2 divided doses) in patients with concomitant familial hypertriglyceridemia. • Prescriber restriction: prescriber must be hepatologist or gastroenterologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review (to be used as a check list for providers for needed information or guidance for verbal PA requests) • Diagnosis • Labs

Coverage Criteria: • Initiation of Therapy: • For confirmed diagnosis of bile acid synthesis disorder due to single enzyme defect (SEDs) or peroxisomal disorders including Zellweger spectrum disorders in patients that exhibit manifestations of liver disease, steatorrhea or complications from decreased fat soluble vitamin absorption, approve if: o Current labs (within 30 days of request) have been submitted for the following: 1. ALT/AST 2. GGT (serum gamma glutamyltransferase) 3. ALP (alkaline phosphatase) 4. Bilirubin 5. INR • Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Documentation has been submitted indicating clinical benefit/liver function has improved since beginning treatment. * *TREATMENT SHOULD BE DISCONTINUED IF LIVER FUNCTION DOES NOT IMPROVE WITHIN 3 MONTHS OF STARTING TREATMENT, IF COMPLETE BILIARY OBSTRUCTION DEVELOPS OR CHOLESTASIS • Current labs (within 30 days of request) have been submitted for the following: o ALT/AST o GGT (serum gamma glutamyltransferase) o ALP (alkaline phosphatase) o Bilirubin o INR

CHOLBAM® (CHOLIC ACID) • Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Documentation has been submitted indicating clinical benefit/liver function has improved since beginning treatment. * *TREATMENT SHOULD BE DISCONTINUED IF LIVER FUNCTION DOES NOT IMPROVE WITHIN 3 MONTHS OF STARTING TREATMENT, IF COMPLETE BILIARY OBSTRUCTION DEVELOPS OR CHOLESTASIS • Current labs (within 30 days of request) have been submitted for the following: o ALT/AST o GGT (serum gamma glutamyltransferase) o ALP (alkaline phosphatase) o Bilirubin o INR References: N/A Last review/revision date: 03/2019

OCALIVA® (OBETICHOLIC ACID) Standard Therapeutic Class, Specific Therapeutic Class: Bile therapy, farnesoid X receptor (FXR) agonist, bile acid analog Mechanism of Action: farnesoid X receptor agonist Formulary Status: • Formulary, PA required o Ocaliva® Coverage Duration Initial: 6 months Re-authorization: 1 year Diagnosis Considered for Coverage: • Primary biliary cholangitis (PBC) • All other medically accepted indications (medically accepted indications are defined using the following sources: The Food and Drug Administration (FDA), Micromedex, American Hospital Formulary Service (AHFS), United States Pharmacopeia Drug Information for the Healthcare Professional (USP DI), and the Drug Package Insert) Prescribing Restriction • Quantity Limit*: Up to 5 mg once daily (initial) and 10 mg once daily for re-authorization • Prescriber restriction: prescriber must be hepatologist or gastroenterologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review (to be used as a check list for providers for needed information or guidance for verbal PA requests) • Diagnosis • Previous therapy • Concurrent therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For diagnosis of primary biliary cholangitis, approve if: o Patient is taking Ocaliva in addition to usodeoxycholic acid (UDCA) due to inadequate response to UDCA for at least 1 year OR o Patient is unable to tolerate UDCA and is taking Ocaliva as monotherapy

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND

OCALIVA® (OBETICHOLIC ACID) • Medication is used for appropriate indication and at appropriate dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication References: N/A Last review/revision date: 03/2019

THROMBOCYTOPENIA Standard/Specific Therapeutic Class: Hemostatics, Thrombopoietin Receptor Agonists and Miscellaneous, Spleen Tyrosine Kinase Inhibitors Formulary Status: • Formulary, PA required: o Promacta® (eltrombopag) • Non-formulary: o Nplate® (romiplostim) o Doptelet® (avatrombopag) o Mulpleta® (lusutrombopag) o Tavalisse® (fostamatinib) Coverage Duration: • Indefinite for Promacta®, Nplate®, and Tavalisse® • 5 days for Doptelet® • 7 days for Mulpleta® Diagnosis Considered for Coverage: • Promacta®: chronic immune (idiopathic) thrombocytopenia (ITP), severe aplastic anemia, thrombocytopenia associated with hepatitis C infection • Nplate®: chronic immune (idiopathic) thrombocytopenia (ITP) • Doptelet® and Mulpleta®: thrombocytopenia associated with chronic liver disease in patients requiring elective surgery • Tavalisse®: Chronic or refractory immune (idiopathic) thrombocytopenia (ITP) • Off-label uses: medically accepted indications are defined using the following sources: American Hospital Formulary Service-Drug Information (AHFS-DI), Truven Health Analytics Micromedex DrugDEX (DrugDEX), National Comprehensive Cancer Network (NCCN) Drugs and Biologics Compendium, Wolters Kluwer Lexi-Drugs, and Elsevier/Gold Standard Clinical Pharmacology and/or positive results from two peer-reviewed published studies Prescribing Restriction: • Quantity Limit*: o Promacta®: #30 per 30 days o Nplate®: dose appropriate based on patient’s weight o Doptelet®: #15 per 5 days o Mulpleta®: #7 per 7 days o Tavalisse®: #60 per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis

Clinical Information Required for Review: • Diagnosis, dose • Prior therapy • Platelet level Coverage Criteria: I. Initiation of Therapy: • For diagnosis of chronic immune (idiopathic) thrombocytopenia (ITP), approve if: o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e drug interaction, allergy, adverse reaction, etc.) to use ONE of the following: glucocorticoids, intravenous immune globulin (IVIG), Rituxan® (if appropriate) or splenectomy AND o Platelet level < 20,000 mm3 OR < 30,000 mm3 with bleeding AND o For Nplate® or Tavalisse®, approve if there is a documentation of trial and failure, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use Promacta® References: N/A Last review/revision date: 03/2019

WHITE BLOOD CELL STIMULATORS Standard/Specific Therapeutic Class: Hematinics & Blood Cell Stimulators, Leukocyte (WBC) Stimulants Formulary Status: • Formulary, PA required: ZarxioTM (filgrastim-sndz) Granix® (TBO-filgrastim) Nivestym (filgrastim-aafi) Fulphila® (pegfilgrastim) Udenyca (pegfilgrastim-cbqv) Leukine (sargramostin) Non-formulary: Neupogen® (filgrastim) Neulasta® (pegfilgrastim) Coverage Duration: 6 months or duration of chemotherapy Diagnosis Considered for Coverage: • Febrile neutropenia treatment or prevention • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit*

WHITE BLOOD CELL STIMULATORS o Granix®,Zarxio, Nivestym § 300/0.5 ml syringe: up to 7 ml per 28 days (should be billed in increments of 0.5 ml) § 480/0.8 ml syringe: up to 11.2 ml per 28 days (should be billed in increments of 0.8 ml) o Neupogen®: § 300/0.5 ml syringe: up to 7 ml per 28 days (should be billed in increments of 0.5 ml) § 300/ml vial: up to 14 ml per 28 days (should be billed in increments of 1 ml) § 480/0.8 ml syringe: up to 11.2 ml per 28 days (should be billed in increments of 0.8 ml) § 480/1.6 ml vial: up to 22.4 ml per 28 days (should be billed in increments of 1.6 ml) o Fulphila, Udenyca: 0.6 ml per chemotherapy cycle, #4 vials or syringes per 30 days o Neulasta 0.6 ml per chemotherapy cycle, #4 vials or syringes per 30 days o Leukine 250mcg injection • Prescriber restriction: Prescription written or currently being supervised by a hematologist or an oncologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Dose • Prior therapy Coverage Criteria: I. Initiation of Therapy: • Approve if: o Patient is 18 years of age or older AND o Prescription written or currently being supervised by a hematologist or oncologist AND o Drug is being used for an FDA-approved indication at an FDA-approved dose AND o For Neupogen®, patient has documented treatment failure (i.e, failure to reach and/or maintain target ANC, prolonged febrile neutropenia, unplanned hospitalization, infection requiring prolonged anti-infectives) with an adequate trial (including dates, doses of therapy) of Zarxio™ and Granix®or has a documented medical reason (intolerance, hypersensitivity, dose dense chemotherapy, or stem cell collection, etc.) for not using Zarxio™ and Granix® o For Neulasta®, patient has documented treatment failure (i.e, failure to reach and/or maintain target ANC, prolonged febrile neutropenia, unplanned hospitalization, infection requiring prolonged anti-infectives) with an adequate trial (including dates, doses of therapy) of Fulphila®or has a documented medical reason (intolerance, hypersensitivity, dose dense chemotherapy, or stem cell collection, etc.) for not using Fulphila® II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is 18 years of age or older AND • Patient is still receiving chemotherapy

WHITE BLOOD CELL STIMULATORS III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if patient is still receiving chemotherapy References: • National Comprehensive Cancer Network. Myeloid Growth Factors (Version 2.2016). https://www.nccn.org/professionals/physician_gls/pdf/myeloid_growth.pdf . Accessed November 4, 2016. • Pappas AL, PharmD, Hanna S. TBO-Filgrastim (Granix). Pharmacy Times. March 12, 2014. • Freifeld AG, Bow EJ, Sepkowitz KA, et al. Clinical practice guideline for the use of antimicrobial agents in neutropenic patients with cancer: 2010 update by the infectious diseases society of America. Clin Infect Dis. 2011;52(4):e56. • Smith TJ, Bohlke K, Lyman GH, et al. Recommendations for the Use of WBC Growth Factors: American Society of Clinical Oncology Clinical Practice Guideline Update. J Clin Oncol. 2015;33(28):3199-212. • National Comprehensive Cancer Network (NCCN) Guidelines v1.2017: Myelodysplastic Syndromes – https://www.nccn.org/professionals/physician_gls/pdf/mds.pdf. Accessed November 7, 2016 • National Comprehensive Cancer Network (NCCN) Guidelines v2.2016: Acute Myeloid Leukemia – https://www.nccn.org/professionals/physician_gls/pdf/aml.pdf. Accessed November 10, 2016 • Guidelines for the Use of Anitretroviral Agents in Pediatric HIV infection - Antiretroviral-therapy-associated Adverse Effects and Management Recommendations—Hematologic Effects. https://aidsinfo.nih.gov/contentfiles/lvguidelines/pediatricguidelines.pdf. Accessed November 4, 2016 • National Comprehensive Cancer Network (NCCN) Guidelines v2.2017: Multiple Myeloma. https://www.nccn.org/professionals/physician_gls/pdf/myeloma.pdf. Accessed November 10, 2016 Last review/revision date: 03/2019

ERYTHROPOIETIN STIMULATING AGENTS (ESAs) Standard/Specific Therapeutic Class: Hematinics & Blood Cell Stimulators, Erythropoiesis-stimulating Agents Formulary Status: Formulary, PA required o Retacrit (epoetin alfa-epbx) o Epogen®, Procrit® (epoetin alfa) o Aranesp® (darbepoetin alfa) Non-Formulary: o Mircera® (methoxy peg-epoetin beta) Coverage Duration: • ESRD: indefinite • Chemotherapy: Duration of chemotherapy • Other: 12 months Diagnosis Considered for Coverage: Anemia due to:

ERYTHROPOIETIN STIMULATING AGENTS (ESAs) • Chronic Kidney Disease (CKD)/ End Stage Renal Disease (ESRD): All • Cancer / Chemotherapy-induced, Myelodysplastic syndrome (MDS), hepatitis C treatment: Epogen®/Procrit®, Aranesp® • Zidovudine therapy: Epogen®/Procrit® only • EXCLUDED DIAGNOSES: Mircera® for chemotherapy induced anemia Prescribing Restriction • Quantity Limit*: o Aranesp®: § 10mcg, 25mcg, 40mcg, 60mcg, 100mcg, 150mcg, 200mcg: #4 vials or prefilled syringes per 28 days § 300mcg: #2 vials or prefilled syringe per 28 days § 500mcg: 1 vial or prefilled syringe per 21 days o Epogen®/Procrit®: § 2,000U/mL, 3,000U/mL, 4,000U/mL and 10,000U/mL vials: #12 vials per month § 20,000U/mL, 20,000U/mL vials and 40,000U/mL vials: #4 vials per month o Mircera®: #2 syringes per 30 days • Prescriber: Hematologist/Oncologist, Nephrologist, Hepatologist, or Infectious Disease physician *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Hemoglobin level • Dose Coverage Criteria: I. Initiation of Therapy: • For anemia due to CKD/ESRD, MDS, Hepatitis C treatment, and zidovudine therapy, approve if: o Hemoglobin < 10 g/dL AND o Drug is FDA approved for the requested diagnosis (see “Diagnosis Considered for Coverage” above) • For cancer/chemotherapy-induced anemia: o Requested drug is NOT Mircera® AND o Hemoglobin < 10 g/dL AND o Patient is undergoing palliative treatment, OR on myelosuppressive chemotherapy without other identifiable cause of anemia, OR refusing blood transfusions AND o Patient does NOT meet one of the following: - Patient with cancer not receiving chemotherapy

ERYTHROPOIETIN STIMULATING AGENTS (ESAs) - Patients on non-myelosuppressive chemotherapy (e.g. NOT breast, non–small cell lung, head and neck, lymphoid, and cervical cancers), AND - Patients receiving myelosuppressive chemotherapy with curative intent II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Hemoglobin < 12 g/dL AND • Medication is used for appropriate indication and at appropriate dose AND • Patient is stable and continuing the medication AND • If taking for chemotherapy induced anemia, patient is still receiving chemotherapy III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Hemoglobin < 12 g/dL AND • If taking for chemotherapy induced anemia, patient is still receiving chemotherapy References: • NCCN: Cancer- and Chemotherapy-Induced Anemia. Ver 2.2017 – Nov 6 2016. Accessed Nov 29 2016. Access at: https://www.nccn.org/professionals/physician_gls/pdf/anemia.pdf Last review/revision date: 07/2019

HEREDITARY ANGIOEDEMA Standard/Specific Therapeutic Class: Miscellaneous/C1 Esterase Inhibitors Formulary Status: Formulary: • Danazol 50mg Formulary, PA required: • Haegarda (C1 esterase inhibitor) • Kalbitor® (ecallantide) • Takhzyro™ (lanadelumab-flyo) Non-formulary: • Firazyr® (C1 Esterase Inhibitor Subcutaneous [Human]) Coverage Duration: • Initial: 3 months • Re-authorization: 1 year

Diagnosis Considered for Coverage: • Hereditary Angioedema (HAE) • Off-label uses: medically accepted indications are defined using the following sources: American Hospital Formulary Service-Drug Information (AHFS-DI), Truven Health Analytics Micromedex DrugDEX (DrugDEX), National Comprehensive Cancer Network (NCCN) Drugs and Biologics Compendium, Wolters Kluwer Lexi-Drugs, and Elsevier/Gold Standard Clinical Pharmacology and/or positive results from two peer-reviewed published studies Prescribing Restriction: • Quantity*: o Haegarda®: #16 vials per 28 days o Firazyr®: #30 syringes per 30 days o Kalbitor®: #30 vials per 30 days o Takhzyro™: #4 mL (2 vials) per 28 days • Prescriber Restriction: restricted to allergy specialist Clinical Information Required for Review: • Diagnosis • Dose • Weight • Quantity Coverage Criteria: I. Initiation of Therapy: • For diagnosis of hereditary angioedema: o For Takhzyro™, approve if: § Medication is used for prevention of HAE attacks AND § Documentation of patient’s weight and quantity/dose requested AND § Documentation of at least one HAE attack per month o For Haegarda®, approve if: § Documentation meets criteria above for Takhzyro™ AND § Documentation of trial and failure, intolerance, contraindication or inability to use Takhzyro™ o For Kalbitor®, approve if medication is used for on-demand treatment of acute HAE attacks o For Firazyr®, approve if: § Medication is used for on-demand treatment of acute HAE attacks § Documentation of trial and failure, intolerance, contraindication, or inability to use: Kalbitor® • For off-label indications or dosing, approve if: o No other formulary medication has a medically accepted use for the patient’s specific diagnosis as referenced in the medical compendia AND o Medication is being requested for an accepted off-label use and is listed in the standard clinical decision support resources (as noted in Diagnosis section above) OR o Requested use can be supported by at least two published peer reviewed clinical studies

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if:

• Prescriber attests that member has been on this medication continuously before joining Prescryptive AND • The diagnosis and dosage provided meets FDA labeling and/or drug-specific criteria or off-label criteria

II. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Documented response to therapy AND o Patient is stable and continuing the medication

Refrences: N/A Last review/revision date: 7/2019

ORFADIN® AND NITYR® (NITISINONE) Standard/Specific Therapeutic Class: Drugs to Treat Hereditary Tyrosinemia Formulary Status: • Formulary, PA required: Nityr® (nitisinone) • Non-formulary: Orfadin® (nitisinone) Coverage Duration: Initial: 6 months Renewal: 1 year Diagnosis Considered for Coverage: • Hereditary tyrosinemia type 1 (HT-1) • Other diagnoses: see off-label criteria Prescribing Restriction: • Quantity*: o Up to 2 mg/kg/day • Prescriber: Specialist in inherited metabolic disorders *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information required for Review: • Diagnosis • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For diagnosis of hereditary tyrosinemia type 1, approve if: o Diagnosis confirmed by one of the following: 1. DNA testing OR 2. Detection of succinylacetone (SA) in urine AND o Documentation provided attesting to diet restricting tyrosine and phenylalanine AND o If request is for Orfadin® capsule or suspension, documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use Nityr® tablet

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable or improving and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if:

ORFADIN® AND NITYR® (NITISINONE) • Patient is stable or improving and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 03/2019

DARAPRIM® (PYRIMETHAMINE) Standard/Specific Therapeutic Class: Antiparasitics Formulary Status: Formulary, PA required Coverage Duration: 1 year Diagnosis Considered for Coverage: • Toxoplasma gondii (Toxoplasmosis) • Other diagnoses: see off-label criteria Prescribing Restriction • Quantity: #90 per 30 days* • Prescriber restriction: HIV specialist, infectious disease specialist, internal medicine specialist OR oncologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Prescriber Coverage Criteria: I. Initiation of Therapy: • For treatment of Toxoplasma gondii (Toxoplasmosis), approve if: o Diagnosis of Toxoplasmosis AND o Documented immunosuppression (i.e. CD4+ count ≤ 200/mm3) II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Medical justification provided for continuation of therapy. III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Medical justification provided for continuation of therapy. References: N/A Last review/revision date: 07/2019

NEBUPENT® (PENTAMIDINE ISETHIONATE) Standard/Specific Therapeutic Class: Antiparasitics/Antiprotozoal Drugs, Misc Formulary Status: • Formulary, PA required o Nebupent® (pentamidine isethionate) 300 mg vial for nebulization Coverage Duration: 1 year Diagnosis Considered for Coverage: • pneumocystis jirovecii pneumonia (PCP) • Other diagnoses: see off-label criteria Prescribing Restriction • Quantity Limit*: 1 vial per 28 days • Prescriber restriction: HIV specialist, infectious disease specialist, internal medicine specialist OR oncologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Previous therapy • Prescriber Coverage Criteria: I. Initiation of Therapy: • For prevention of pneumocystis jirovecii pneumonia (PCP), approve if: o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e drug interaction, allergy, adverse reaction, etc.) to use sulfamethoxazole/trimethoprim AND o Diagnosis of HIV AND o Documented history of one or more episodes of PCP OR documented CD4+ count ≤ 200/mm3 II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Medical justification provided for continuation of therapy. III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Medical justification provided for continuation of therapy. References: N/A Last review/revision date: 03/2019

AZOLE ANTIFUNGALS Standard/Specific Therapeutic Class: Antifungals, antifungal agents Mechanism of Action: 14-alpha-lanosterol Demethylation Inhibitor, Inhibiting Fungal Cell Membrane Formation. Formulary Status: • Formulary: fluconazole • Formulary, PA required o Voriconazole (Vfend) o Posaconazole (Noxafil) o Isavuconazonium Sulfate (Cresemba) Coverage Duration Drug Initial Re-authorization Isavuconazonium 6 months Up to 1 year Voriconazole 6 months Up to 1 year Posaconazole 6 months Up to 1 year

Diagnosis Considered for Coverage: • Blastomycosis, histoplasmosis, sporotrichosis • For onychomychosis: refer to “onychomycosis” criteria • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit* • Voriconazole 50 mg: #360 per 90 days • Voriconazole 200 mg: #180 per 90 days • Posaconazole (Nofaxil) 300mg: #30 per 30 days • Isavuconazonium Sulfate (Cresemba) 186mg: #60 per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For voriconazole, approve if: o Diagnosis if one of the following OR - Fungal infection by Scedosporium apiospermum or Fusarium species - Treatment of invasive candidasis in critically ill patients

AZOLE ANTIFUNGALS - Invasive pulmonary aspergillus infections - Primary prophylaxis for aspergillus infections for special populations such as lung transplant, Acute Myleoid Leukemia, Allo-stem cell transplant with prolonged neutropenia from chemotherapy AND high risk for infection o For esophageal candidiasis or candidemia in nonneutropenic patients, or treatment of disseminated Candida infections of the skin and abdomen, kidney, bladder wall, and wounds: documentation of trial and failure, intolerance, contraindication, or inability (i.e drug interaction, allergy, adverse reaction, etc.) to use fluconazole OR o For blastomycosis, histoplasmosis or sporotrichosis: documentation of trial and failure, intolerance, contraindication, or inability (i.e drug interaction, allergy, adverse reaction, etc.) to use itraconazole • For Posaconazole, approve for: o For prophylaxis of invasive aspergillus or candida in patients at high risk of developing invasive aspergillus or candida due to being severely immunocompromised: trial and failure or inability to use voriconazole OR o For orophangeal candidiasis: trial and failure or inability to use fluconazole • For Isavuconazonium sulfate (Cresemba), approve for: o Diagnosis of invasive mucormycosis in adults OR o For invasive aspergillosis in adults: trial and failure or inability to use to voriconazole II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Documented response to therapy AND • Additional therapy is medically necessary and clinically appropriate III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Documented response to therapy AND • Additional therapy is medically necessary and clinically appropriate References: • IDSA guidelines for aspergillus: http://cid.oxfordjournals.org/content/46/3/327.1.full.pdf+html • IDSA guidelines for blastomycosis: http://cid.oxfordjournals.org/content/46/12/1801.full.pdf+html • IDSA guidelines for Coccidioidomycosis: http://cid.oxfordjournals.org/content/41/9/1217.full.pdf+html • IDSA guidelines for Cryptococcal Disease: http://cid.oxfordjournals.org/content/50/3/291.full.pdf+html • IDSA guidelines for candidiasis: http://cid.oxfordjournals.org/content/48/5/503.1.full.pdf+html • IDSA guidelines for histoplasmosis: http://cid.oxfordjournals.org/content/45/7/807.full.pdf+html • IDSA guidelines for Sporotrichosis: http://cid.oxfordjournals.org/content/45/10/1255.full.pdf+html • Rex JH et al. Practice guidelines for the treatment of candidiasis. Clin Infect Dis 2000;30:662-78. • National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Prevention and treatment of cancer- related infections. V.2.2009. Available at: www.nccn.org. • Lexi-comp: Last updated on 11/07/15 Last review/revision date: 03/2019

HEPATITIS B Standard/Specific Therapeutic Class: Antivirals/Hepatitis B Treatment Agents and Antivirals, HIV Specific, Nucleotide Analog, RTI Formulary Status: • Formulary o Entecavir (Baraclude®) tablet, Baraclude® (entecavir) solution o Viread® (tenofovir disoproxil fumarate) o Lamivudine HBV (Epivir HBV®) tablet, Epivir HBV® (lamivudine) solution o Adefovir (Hepsera®) o Vemlidy® (tenofovir alafenamide) o Telbivudine Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Hepatitis B • Other diagnoses: see off-label criteria Prescribing Restriction • Quantity*: o Liquids: 600 mg per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For Hepatitis B, approve II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: • AASLD Guidelines for Treatment of Chronic Hepatitis B. Hepatology. 2016;63(1):261-283. • Asia-Pacific Clinical Practice Guidelines on the Management of Hepatitis B: A 2015 Update. Hepatol Int 2016;10:1-98. • Hepatitis B (Chronic): Diagnosis and Management. National Institute for Health and Care Excellence. 2013. • EASL Clinical Practice Guidelines: Management of Chronic Hepatitis B. Journal of Hepatology. 2012;57:167-185. Last review/revision date: 03/2019

HEPATITIS C Standard/Therapeutic Class: Antivirals/Hepatitis C Virus- NS5A Replication Complex Inhibitor; NS3/4A Serine Protease Inhibitor; Nucleotide Analog NS5B Polymerase Inhibitor; NS5B Polymerase and NS5A Inhibitor Combination; NS5A and NS3/4A Inhibitor Combination; NS5B Polymerase and NS5A Inhibitor Combination; NS5A, NS3/4A, Nucleotide NS5B Inhibitor Combination; Hepatitis C Treatment Agents Formulary Status: Formulary: ribavirin 200mg capsules and tablets Formulary, PA required Mavyret™ (glecaprevir/pibrentasvir) Vosevi™ (sofosbuvir/velpatasvir/voxilaprevir) ledipasvir/sofosbuvir (Authorized Generic) sofosbuvir/velpatasvir (Authorized Generic) ribavirin 400 mg tab ribavirin 600 mg tab ribavirin 200-400 mg tab/600-400 mg tab (Ribapak®) peginterferon Alfa-2a (Pegasys®, Pegasys Proclick®) interferon Alfa 2B (Intron) Non-formulary Zepatier® (elbasvir/grazoprevir) Harvoni® (ledipasvir/sofosbuvir) Epclusa® (sofosbuvir/velpatasvir) Daklinza® (daclatasvir) Olysio® (simeprevir) Sovaldi® (sofosbuvir) peginterferon Alfa-2B (PegIntron) Technivie® (ombitasvir/paritaprevir/ritonavir) Viekira Pak® and Viekira XR® ER (ombitasvir/paritaprevir/ritonavir and dasabuvir) Coverage Duration: Full course of therapy (8, 12, 16, or 24 weeks depending on therapy) Diagnosis Considered for Coverage: • Hepatitis C Viral Infection (HCV) Prescribing Restriction: • Quantity Limit: 8 weeks 12 weeks 16 weeks 24 weeks Mavyret™ #84/ + 1 refill 84/ + 3 refills #84/ + 4 refills n/a

Vosevi™ n/a #28 + 3 refills n/a n/a Epclusa or n/a #28 + 3 refills n/a #28 + 11 refills authorized generic® Zepatier™ n/a #28 + 3 refills #28 + 3 refills n/a

HEPATITIS C Harvoni or n/a #28 + 3 refills #28 + 5 refills #28 +11 refills authorized generic Daklinza® Sovaldi® Viekira Pak® n/a 1 pack (#112) + 2 n/a 1 pack (#112) + 5 refills refills Technivie® n/a 1 pack (#56) + 2 n/a n/a refills *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis

Clinical Information required for Review • Diagnosis • Dose • Genotype • Stage of liver disease • HIV and/or Hepatitis B Co-Infection • Duration of therapy • Concurrent medications for drug-interaction assessment • Difficulty managing blister pack (use or storage) or swallowing 3 large pills (if assessed/anticipated) • Prior treatment status (i.e. naïve vs experienced) and prior therapies • If cirrhosis, compensated or decompensated Coverage Criteria: For diagnosis of HCV, approve if: o Patient is ≥ 13 years old with life expectancy ≥ 12 months Treatment-naïve Treat with ledipasvir/sofosbuvir, sofosbuvir/velpatasvir, Mayveret, or Vosevi as follows:

HCV Genotype No Cirrhosis Compensated Cirrhosis (Child-Pugh A) 1-6 8 weeks 12 weeks

Treatment Experienced, Treat with ledipasvir/sofosbuvir, sofosbuvir/velpatasvir, Mayveret, or Vosevi when appropriate as follows. Use any non-formulary product(s) when the above formulary products are not appropriate.

HCV Genotype Prior Regimen No Cirrhosis Compensated Cirrhosis (Child-Pugh A)

HEPATITIS C 1 NS5A containing regimen 16 weeks 16 weeks without prior NS3/4A protease inhibitor experience 1 NS3/4A containing regimen 12 weeks 12 weeks without prior NS5A protease inhibitor experience 1,2,4,5,6 Interferon, pegylated 8 weeks 12 weeks interferon, ribavirin and/or sofosbuvir 3 Interferon, pegylated 16 weeks 16 weeks interferon, ribavirin and/or sofosbuvir

Unique Populations Population Genotype Preferred Regimen Decompensated All RBV eligible: prefer 12 weeks sofosbuvir/velpatasvir cirrhosis RBV ineligible: prefer 24 weeks sofosbuvir/velpatasvir Prior SOF or NS5A failure: prefer 24 weeks sofosbuvir/velpatasvir + RBV Recurrent HCV With or without compensated cirrhosis: prefer 12 weeks Mavyret™ post-transplant All Decompensated cirrhosis: prefer 12 weeks sofosbuvir/velpatasvir + RBV ESRD/eGFR <30* All Prefer 8-16 weeks Mavyret™, duration based on presence of cirrhosis and prior Tx experience (see tables above) Kidney transplant* All Prefer 12 weeks Mavyret™ Children* Treatment-naïve: Prefer 12 weeks ledipasvir/sofosbuvir Treatment-experienced: 1 -No cirrhosis: prefer 12 weeks ledipasvir/sofosbuvir -Compensated cirrhosis: prefer 24 weeks ledipasvir/sofosbuvir 2 Prefer 12 weeks Sovaldi® + RBV 3 Prefer 24 weeks Sovaldi® + RBV 4-6 Prefer 12 weeks Harvoni® *with and without compensated cirrhosis Non-preferred agents (Epclusa®, Harvoni®, Zepatier®) may be used for patients unable to use preferred agents above due to failure/intolerance/contraindication.

HEPATITIS C I. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Requested regimen and duration is appropriate per AASLD/IDSA guidelines II. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Extension of therapy is supported by AASLD/IDSA guidelines References: N/A Last review/revision date: 08/2019

SIRTURO® (BEDAQUILINE) Standard/Specific Therapeutic Class: TB Preparations, Antitubercular Antibiotics Formulary Status: Formulary, PA required Coverage Duration: Up to 2 years Diagnosis Considered for Coverage: • Laboratory-confirmed pulmonary multi-drug resistant (MDR) tuberculosis • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit*: 100 mg tablet: #56 per 14 days for 1 fill (400 mg once daily for 2 weeks), then #504 per 84 days for 2 years (200 mg three times weekly) *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis confirmed by laboratory results • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • Approve if: o Diagnosis is laboratory confirmed pulmonary multi-drug resistant (MDR) tuberculosis (TB) with an isolate showing genotypic or phenotypic resistance to both INH and RIF AND o Effective treatment regimen cannot otherwise be provided (e.g. fluoroquinolones, linezolid, capreomycin/amikacin) II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

SIRTURO® (BEDAQUILINE) III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Medical justification provided for continuation of therapy beyond 2 years References: • Provisional CDC Guidelines for the Use and Safety Monitoring of Bedaquiline Fumarate (Sirturo) for the treatment of Multidrug-Resistant Tuberculosis. Last review/revision date: 03/2019

MULTIPLE SCLEROSIS Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Formulary, PA required: • glatiramer acetate (Copaxone®, Glatopa®) • Tecfidera® (dimethyl fumarate) • Gilenya® (fingolimod) • Avonex® (interferon beta-1a) • Rebif® (interferon beta-1a) • Plegridy® (peginterferon beta-1a) • Mavenclad® (cladribine) Non-formulary: Betaseron® (interferon beta-1b), Extavia® (interferon beta-1b) Aubagio® (teriflunomide) Mayzent® (siponimod) Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Multiple Sclerosis Prescriber Restriction • Quantity Limit * o glatiramer acetate 20 mg: #30 mL/30 days, 40 mg: #12 ml/28 days o Tecfidera®: #60 per 30 days o Gilenya®: #30 per 30 days o Avonex®: #30mcg weekly (IM) o Rebif®: #6 mL (12 syringes) per 30 days o Betaseron®: #1 kit (14 vials) per 30 days o Extavia®: #1 kit (15 syringes) per 30 days o Plegridy® syringe, pen: #2 syringes or pens per 30 days o Aubagio®: #28 per 28 days o Mavenclad: #20 per year o Mayzent: #30 per 30 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Previous therapy

MULTIPLE SCLEROSIS Coverage Criteria: I. Initiation of Therapy: • Approve if: o Patient has relapsing/remitting MS (RRMS) or secondary progressive MS (SPMS) with a relapsing element o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o The preferred agents are glatiramer, Rebif®, Plegridy®, Avonex®, Gilenya® , Mavenclad, or Tecfidera®. • For Betaseron®, Extavia®, Aubagio®, Mayzent, the member has: o Documented treatment failure to 6 months of therapy with 2 preferred agents OR o Medical reason for not taking for a minimum of 6 months (e.g. intolerance, hypersensitivity, contraindication, etc.) of preferred agents

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication References: N/A Last review/revision date: 07/2019

XYREM® (SODIUM OXYBATE) Standard/Specific Therapeutic Class: Sedative Non-barbituate, Anti-narcolepsy & Anti-cataplexy Sefative Type Agonist Formulary Status: Non-formulary, PA Required Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Narcolepsy, Treatment of cataplexy and excessive daytime sleepiness in patients with narcolepsy • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit*: Quantity within FDA-approved dosing limits (maximum 9 g per night) • Prescriber restriction: Prescriber is a neurologist or sleep specialty, or documentation has been provided that provider has consulted with a neurologist or sleep specialist. *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis

XYREM® (SODIUM OXYBATE) Clinical Information required for Review: • Diagnosis • Previous therapy • Concurrent therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For diagnosis of narcolepsy with exceesive daytime sleepiness, approve if: o Patient is ≥ 7 years of age AND o Sleep study has been done to confirm diagnosis of narcolepsy AND o If the patient has a history of substance abuse, documentation has been provided that provider has referred the patient for substance abuse disorder treatment AND o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e drug interaction, allergy, adverse reaction, etc.) to use the formulary alternatives: at least two formulary stimulants AND o Medication is being prescribed at an FDA approved dose • For diagnosis of narcolepsy with cataplexy, approve if: o Patient is ≥ 7 years of age AND o Sleep study has been done to confirm diagnosis of narcolepsy AND o If the patient has a history of substance abuse, documentation has been provided that provider has referred the patient for substance abuse disorder treatment AND o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e drug interaction, allergy, adverse reaction, etc.) to use the formulary alternatives: at least two formulary stimulants AND o At least two of the following: formulary SSRI, formulary TCA, or venlafaxine AND o Medication is being prescribed at an FDA approved dose II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Medical justification provided for continuation of therapy III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Documentation has been submitted indicating patient has clinically benefited from treatment (i.e. improvement on Epworth Sleepiness score or other documentation) AND • Medication is being prescribed at an FDA approved dose AND • For cataplexy, documentation has been provided that there has been a reduction in frequency of cataplexy attacks.

XYREM® (SODIUM OXYBATE) References: N/A Last review/revision date: 07/2019

DRUGS FOR MOVEMENT DISORDERS Standard/Specific Therapeutic Class: Miscellaneous, Drugs to Treat Movement Disorders Formulary Status: Formulary, PA required • Tetrabenazine (Xenazine®) • Austedo® (deutetrabenazine) • Ingrezza™ (valbenazine) Coverage Duration Initial: 6 months; Re-authorization: 1 year Diagnosis Considered for Coverage: • Chorea associated with Huntington’s disease (tetrabenazine, Austedo®) • Moderate to severe tardive dyskinesia (Austedo®, Ingrezza™) • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit*: o tetrabenazine, Austedo®: #120/30 days o Ingrezza™: #30/30 days • Prescriber restriction: neurologist or psychiatrist (if patient has Hx of depression, documentation of psychiatrist consult) *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information Required for Review • Diagnosis, dose • Previous therapy • History of depression, congenital long QT syndrome or cardiac arrhythmias

DRUGS FOR MOVEMENT DISORDERS Coverage Criteria: I. Initiation of Therapy: • For diagnosis of chorea associated with Huntington’s disease: o For tetrabenazine, approve if § Physician attests that patient has had a baseline electrocardiogram (EKG) and is aware of the possible risk of QT prolongation AND § Documentation of baseline Total Maximal Chorea (TMC) score ≥ 8, or Total Functional Capacity (TFC) score ≥ 5 from UHDRS has been provided with the request AND § Dose is within FDA approved limits o For Austedo®, approve if: § Physician attests that patient has had a baseline electrocardiogram (EKG) and is aware of the possible risk of QT prolongation AND § Documentation of baseline Total Maximal Chorea (TMC) score ≥ 8, or Total Functional Capacity (TFC) score ≥ 5 from UHDRS has been provided with the request AND § Documentation of trial and failure of, intolerance of, contraindication to, or inability to use tetrabenazine, AND § Dose is within FDA approved limits • For diagnosis of moderate to severe tardive dyskinesia: o For Austedo®, approve if: § Documented baseline evaluation with one of the following scoring tools: Abnormal Involuntary Movement Scale (AIMS) > 10 OR Extrapyramidal Symptom Rating Scale (ESRS) > 20 AND § Dose is within FDA approved limits o For Ingrezza™, approve if: § Documented baseline evaluation with one of the following scoring tools: Abnormal Involuntary Movement Scale (AIMS) > 10 OR Extrapyramidal Symptom Rating Scale (ESRS) > 20 AND § Documentation of trial and failure of, intolerance of, contraindication to, or inability to use Austedo® AND § Dose is within FDA approved limits

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • For diagnosis of chorea associated with Huntington’s disease: o Documentation was provided that demonstrates clinical symptom improvement (i.e. reduction of total chorea score from UHDRS) AND o Dose is appropriate • For diagnosis of moderate to severe tardive dyskinesia:

DRUGS FOR MOVEMENT DISORDERS o Documentation was provided that demonstrates improvement in AIMS or ESRS scores AND o Dose is appropriate

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • For diagnosis of chorea associated with Huntington’s disease: o Documentation was provided that demonstrates clinical symptom improvement (i.e., reduction of total chorea score from UHDRS) AND o Dose is appropriate • For diagnosis of moderate to severe tardive dyskinesia: o Documentation was provided that demonstrates improvement in AIMS or ESRS scores AND o Dose is appropriate

References: N/A Last review/revision date: 03/2019

NUEDEXTA® (dextromethorphan/quinidine) Standard/Specific Therapeutic Class: NMDA receptor antagonist and sigma-1 agonist/CYP450 2D6 inhibitor Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Pseudobulbar Affect (PBA) secondary to amyotrophic lateral sclerosis (ALS) or multiple sclerosis (MS) • Other diagnoses: see off-label criteria Prescribing Restriction: • Quantity*: o 60 capsules/30 days • Prescriber: Restricted to neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review: • Diagnosis • Dose Coverage Criteria:

NUEDEXTA® (dextromethorphan/quinidine) • Initiation of Therapy: o For diagnosis of Pseudobulbar Affect (PBA) due to ALS or MS, approve o For all other diagnoses, use off label criteria • Continuation of Therapy for NEW Members (within the last 6 months), approve if: o Patient is stable and continuing the medication AND o Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 03/2019

PHOSPHATE BINDERS Standard/Specific Therapeutic Class: Electrolytes & Miscellaneous Nutrients, Electrolyte Depleters Formulary Status: • Formulary, PA required o Fosrenol® (lanthanum) chewable tablets o Auryxia® (ferric citrate) 210mg tablets o Velphoro® (sucroferric oxyhydroxide) 500 mg chewable tablet o Renagel® (sevelamer chloride) 400, 800 mg tabs Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Hyperphosphatemia with end stage renal disease • Other diagnoses: see off-label criteria Prescribing Restriction • Quantity Limit* o Fosrenol®: 500 mg #90 per 30 days; 750 mg #90 per 30 days; 1000 #90 per 30 days o Renagel®: #270 per 30 days o Auryxia®: #360 per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis, dose, previous therapy Coverage Criteria: I. Initiation of Therapy, approve if: • Patient meets ONE of the following AND o Phosphate level > 5.5 mg/dl on Renvela (or its generic) OR

PHOSPHATE BINDERS o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e drug interaction, allergy, adverse reaction, etc.) to use Renvela (or its generic) OR • For Renagel®: trial and failure or inability to use Renvela® 800 mg tablet (or its generic) AND Fosrenol® II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication References: • K/DOQI Clinical Practice Guidelines for Bone Metabolism and Disease in Chronic Kidney Disease. American Journal of Kidney Diseases. Vol 24, No 4, Suppl 3, October 2013. Available at https://www.kidney.org/sites/default/files/docs/boneguidelines.pdf. • KDIGO Clinical Practice Guidelines for the Diagnosis, Evaluation, Prevention, and Treatment of Chronic Kidney Disease- Mineral and Bone Disorder (CKD-MBD). Kidney Disease: Improving Global Outcomes (KDIGO) CKD-MBD Work Group. Kidney Int Suppl. 2009. Last review/revision date: 03/2019

ENDARI™ (L-GLUTAMINE) Standard/Specific Therapeutic Class: Protein lysates, Nutritional therapy, medical condition special formulation Formulary Status: • Formulary, prior authorization required o Endari™ (l-glutamine) Coverage Duration: Initial: 12 months; Renewal: 12 months Diagnosis Considered for Coverage: • Sickle cell disease • Other diagnoses: see off-label criteria Prescribing Restriction • Quantity*: o Weight < 30 kg: 60 packets per 30 days o Weight 30 to 65 kg: 120 packets per 30 days o Weight > 65 kg: 180 packets per 30 days • Prescriber: Restricted to hematologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis • Previous therapy

ENDARI™ (L-GLUTAMINE) • Dose Coverage Criteria: I. Initiation of Therapy: • For diagnosis of sickle cell disease, approve if: • Documentation provided that patient had 2 or more crises in the last 12 months • Documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use hydroxyurea at the maximum tolerated dose with compliance per submitted documentation or refill history within the last 6 months (or medical reason was provided why patient is unable to use hydroxyurea) • Request is for an FDA approved dose. II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Prescriber attests member had reduction in the number of sickle cell crises • Medication is used for appropriate indication and at appropriate dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Prescriber attests member had reduction in the number of sickle cell crises • Request is for an FDA approved dose References: N/A Last review/revision date: 03/2019

MAKENA® (HYDROXYPROGESTERONE CAPROATE) Therapeutic Class: Obstetrics/ Gynecology: Labor Suppression/Tocolytics Formulary Status: Formulary, PA required Coverage Duration: Up to 37 weeks of gestation Diagnosis Considered for Coverage: • Preterm birth prevention Prescribing Restriction: • Quantity Limit*: not to exceed #5 ml per 35 days (5 ml vial) or #4 ml (4 x 1 ml vial) per 28 days • Prescriber Restriction: None Clinical Information required for Review: • Past medical history of preterm birth • Time of treatment initiation (i.e. gestation age) Coverage Criteria: I. Initiation of Therapy: • For diagnosis of preterm birth prevention, approve if: o Patient is 16 years of age or older AND o History of previous spontaneous preterm birth before 37 weeks gestation AND o Treatment to be started between 16 weeks 0 days gestation and 20 weeks 6 days gestation AND o Documented expected delivery date or current gestational age provided with request AND o Quantity not to exceed #5 ml per 35 days (5 ml vial) or #4 ml (4 x 1 ml vial) per 28 days II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication started prior to joining Prescryptive AND • Duration not to exceed 37 weeks of gestation III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Therapy past 37 weeks of gestation is not covered References: • González-Quintero VH, Istwan NB, Rhea DJ, Smarkusky L, Hoffman MC, Stanziano GJ. Gestational age at initiation of 17- hydroxyprogesterone caproate (17P) and recurrent preterm delivery. J Matern Fetal Neonatal Med. 2007 Mar;20(3):249-52. PMID: 17437227. • Makena® [package insert]. AMAG Pharmaceuticals, Inc. Waltham, MA 02451 • How, HY, et al. Prophylaxis with 17 alpha-hydroxyprogesterone caproate for prevention of recurrent preterm delivery: does gestational age at initiation of treatment matter? American Journal of Obstetrics & Gynecology, September 2007. 260e1. Last review/revision date: 07/2019

IDIOPATHIC PULMONARY FIBROSIS Standard/Specific Therapeutic Class: Miscellaneous/Pulmonary Fibrosis – Systemic Enzyme Inhibitors, Antifibrotic Therapy – Pyridone Analogs Formulary Status: • Formulary, PA required: Esbriet® (pirfenidone) • Non-formulary, PA required: Ofev® (nintedanib) Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Idiopathic Pulmonary Fibrosis • Systemic sclerosis-associated interstitial lung disease (ILD) • Other diagnoses: see off-label criteria Prescribing Restriction: • Quantity*: o Esbriet®: § 267mg tablet/capsule: #180/30 days § 801mg tablet: #90/30 days o Ofev®: #60/30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information Required for Review: • Diagnosis • Dose Coverage Criteria: I. Initiation of Therapy: Idiopathic Pulmonary Fibrosis: • For Esbriet®, approve if: o FVC 50% – 80% (mild to moderate impairment in PFTs) AND o Medication is used for appropriate indication and at appropriate dose that is within FDA approved guidelines • For Ofev®, approve if: o FVC 50% – 80% (mild to moderate impairment in PFTs) AND o Documentation of trial and failure, intolerance, contraindication, or inability to use Esbriet AND o Medication is used for appropriate indication and at appropriate dose that is within FDA approved guidelines

Systemic sclerosis-associated interstitial lung disease (ILD) • For Ofev®, approve if: o FVC ≥ 40% of predicted AND o Prescribed by or recommended by a pulmonologist or rheumatologist AND o Medication is used for appropriate indication and at appropriate dose that is within FDA approved guidelines

IDIOPATHIC PULMONARY FIBROSIS II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose that is within FDA approved guidelines References: N/A Last review/revision date: 03/2019

CYSTIC FIBROSIS Standard/Specific Therapeutic Class: Mucolytics, Cystic Fibrosis CFTR Potentiators, Aminoglycosides, Betalactams Formulary Status: • Formulary, PA required: o CFTR agents: § Kalydeco® (ivacaftor) 150mg tablet; 50mg, 75mg granules § Orkambi® (lumacaftor/ivacaftor) 200mg-125mg, 100-125mg tab; 100-125mg, 150-188mg granule pack § Symdeko™ (tezacaftor/ivacaftor) 100mg-150mg/150mg tab therapy pack § TRIKAFTA™ (Elexacaftor, Tezacaftor, and Ivacaftor) o Inhaled antibiotics: § Cayston® (aztreonam) § Tobramcyin (TOBI®) 300 mg/5 ml solution, 300 mg/5ml solution with nebulizer (KITABISTM PAK) § Bethkis® (tobramycin 300 mg/4 ml nebulization solution) § TOBI PodHaler® (tobramycin 28 mg caps) o Mucolytics: Pulmozyme® (dornase alfa) Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Cystic Fibrosis • Non-cystic fibrosis bronchiectasis (for tobramycin only; off-label) • Other diagnoses: follow off-label criteria Prescribing Restriction • Authorized quantity*: o Kalydeco®: 56 tablets or granules per 28 days (1 tablet every 12 hours; granules for kids 2-6 y/o) o Orkambi®: 112 tablets per 28 days (2 tablets every 12 hours; 200/125 mg for adults, 100 mg/125 for pediatrics) or 56 packets per 28 days (1 packet every 12 hours, weight-based dosing)

CYSTIC FIBROSIS o Symdeko™: 56 tablets per 28 days (1 tablet tezacaftor/ivacaftor 100mg-150mg qAM, 1 tablet ivacaftor 150mg qPM) o TRIKAFTA™: oral therapy pack 84 tablets per 28 days (2 tablets each containing elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg in the morning and 1 150mg ivacaftor tablet in the evening) o TOBI PodHaler®: 224 caps per 56 days (4 caps twice daily; 28 days on, 28 days off therapy) o Pulmozyme: 75 ml per 30 days o Cayston® (75 mg/mL): 84 ml per 56 days (75 mg/ml TID; 28 days on, 28 days off therapy) o BETHKIS®: 224 ml per 56 days (300 mg/4ml twice daily; 28 days on, 28 days off therapy) o Tobramycin (TOBI®): 280 ml per 56 days (300 mg/5ml twice daily; 28 days on, 28 days off therapy) o Tobramycin Pak (KITABISTM PAK): 280 ml per 56 days (300 mg/5ml twice daily; 28 days on, 28 days off therapy) • Prescriber restriction: Pulmonologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review • Diagnosis, dose • Mutation • Prescriber specialty: pulmonologist Coverage Criteria: I. Initiation of Therapy: • For Kalydeco®, approve if: o The medication is for the treatment of a CF patient who has an FDA approved indication for treatment of the patient’s genotype (FDA cleared CF mutation test can be used to determine genotype if unknown) AND o MD is pulmonologist AND o Copy of the FDA-cleared CF mutation test has been provided with request AND o The patient is NOT homozygous for the F508del mutation in the CFTR gene AND o The medication is being prescribed at a dose that is within FDA approved guidelines AND o For patients 2-6 years of age: documentation of weight is required to determine appropriate dosing • For Orkambi®, approve if: o The patient is homozygous for the F508del mutation in the CFTR gene AND o Copy of the FDA-cleared CF mutation test has been provided with request AND o The medication is being prescribed at a dose that is within FDA approved guidelines • For Symdeko™, approve if: o The patient is homozygous for the F508del mutation in the CFTR gene OR o The patient has a tezacaftor/ivacaftor-responsive mutation in the CFTR gene AND o Copy of the FDA-cleared CF mutation test has been provided with request AND

CYSTIC FIBROSIS o The medication is being prescribed at a dose that is within FDA approved guidelines • For Trikafta™, approve if: o The patient is 12 years of age or older AND o The patient is homozygous for the F508del mutation in the CFTR gene OR o The patient has a tezacaftor/ivacaftor-responsive mutation in the CFTR gene AND o Copy of the FDA-cleared CF mutation test has been provided with request AND o The medication is being prescribed at a dose that is within FDA approved guidelines • For tobramycin 300 mg/5 ml solution (TOBI®, KITABISTM PAK), TOBI PodHaler®, BETHKIS® approve if: o Diagnosis is cystic fibrosis patient with P. aeruginosa or non-cystic fibrosis bronchiectasis AND o The medication is being prescribed at a dose that is within FDA approved guidelines AND o MD is pulmonologist AND o For TOBI PodHaler® or BETHKIS®: trial with or inability to use tobramycin 300 mg/5 ml solution • For Pulmozyme®, approve if: o The patient is 5 years or older AND o The medication is not being used as monotherapy AND o MD is pulmonologist AND o The medication is being prescribed at a dose that is within FDA approved guidelines • For Cayston®, approve if: o The medication is being prescribed for a cystic fibrosis patient colonized with P. aeruginosa AND o MD is pulmonologist AND o The medication is being prescribed at a dose that is within FDA approved guidelines II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 03/2019

LEUKOTRIENE RECEPTOR ANTAGONISTS Standard/Specific Therapeutic Class: Bronchial Dilators, Leukotriene Receptor Antagonists Formulary Status: o Zyflo (zileuton) 600 mg tablet o zileuton ER 600 mg CR tablet Coverage Duration:

LEUKOTRIENE RECEPTOR ANTAGONISTS • Indefinite Diagnosis Considered for Coverage: • Asthma • Other diagnoses: follow off-label criteria Prescribing Restriction: None *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review: • Previous therapy Coverage Criteria: I. Initiation of Therapy, approve if: • There is documentation of trial and failure, intolerance, contraindication, or inability (i.e drug interaction, allergy, adverse reaction, etc.) to use: montelukast tablet or chewable tablet (e.g. request is for granules in member 6-23 months old) II. Continuation of Therapy approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 07/2019

NARCOTIC WITHDRAWAL THERAPY AGENTS Standard/Specific Therapeutic Class: Miscellaneous, Narcotic Withdrawal Therapy Agents Mechanism of Action: Opiate Partial Agonist Formulary Status: • Formulary PA required, Quantity Limit #120 per 30 days o Buprenorphine hydrochloride (Subutex®) o Buprenorphine hydrochloride/naloxone (Suboxone®) SL tabs o Suboxone® (buprenorphine/naloxone) film • Non-Formulary, PA required o Zubsolv® (buprenorphine/naloxone) o Bunavail™ (buprenorphine/naloxone) Coverage Duration: Indefinite

NARCOTIC WITHDRAWAL THERAPY AGENTS Diagnosis Considered for Coverage: • Opioid Dependence or Opioid Addiction (requests for the diagnosis of pain will be denied) • Other Diagnoses: Follow off-label criteria Prescribing Restriction: • Prescriber restriction: Physician meets all qualifications to prescribe buprenorphine/naloxone (Federal, State, and Local) and has a valid DEA X number • Quantity limit: #120 per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information required for Review: • Diagnosis • Dose Coverage Criteria: I. Initiation of Therapy, approve if: o Physician meets all qualifications to prescribe buprenorphine/naloxone (Federal, State, and Local) and has a valid DEA X number AND o Patient is diagnosed with opioid dependence and/or opioid addiction (requests for the diagnosis of pain will be denied) AND o For requests for Zubsolv and Bunavail documentation of trial and failure, intolerance, contraindication, or inability (i.e drug interaction, allergy, adverse reaction, etc.) to use generic buprenorphine/naloxone sublingual tablets, buprenorphine sublingual tablet, or Suboxone II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Refer to Initiation of Therapy criteria III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Refer to Initiation of Therapy criteria References: N/A Last review/revision date: 03/2019

DISEASE MODIFYING BIOLOGICS Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: • Formulary, PA required o Humira® (adalimumab) o Enbrel® (etanercept) o Cosentyx® (secukinumab) o Skyrizi® (risankizumab-rzaa) • Non-formulary o Siliq® (brodalumab) o Tremfya® (guselkumab) o Kevzara® (sarilumab) o Ilumya (tidrakizumab-asmn) o Actemra® (tocilizumab) o Xeljanz®, Xeljanz XR® (tofacitinib) o Orencia® (abatacept) o Olumiant (baricitinib) o Kineret® (anakinra) o Otezla® (apremilast) o Cimzia® (certolizumab) o Simponi® (golimumab) o Stelara® (ustekinumab) o Taltz® (ixekizumab) Coverage Duration Initial: 1 year (8 weeks for ulcerative colitis) Re-authorization: Indefinite Diagnosis Considered for Coverage: • Rheumatoid arthritis, ankylosing spondylitis, polyarticular juvenile idiopathic arthritis, systemic juvenile idiopathic arthritis, psoriasis, psoriatic arthritis, Crohn’s disease, ulcerative colitis, guttate psoriasis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit*:

Drug Indication Dose Enbrel® Rheumatoid arthritis • 25 mg #8 (syringes) or 4.08 ml (8 vials) per 28 days (25 Juvenile Idiopathic mg 2x/week dosing) Arthritis* OR Psoriatic arthritis • 50 mg 3.92 ml per 28 days (1 kit, 4 syringes/pen Ankylosing spondylitis injectors) (50 mg once weekly dosing)

*NOTE: for Juvenile Idiopathic Arthritis, dose should be 0.8 mg/kg once weekly (max 50 mg/dose) or 0.4 mg/kg 2x/week (max 25 mg/dose) Plaque Psoriasis • Up to 50 mg 7.84 ml per 28 days (2 kits, 8 syringes/pen injectors) for the first 3 months (50 mg 2x/week dosing) • Then 50 mg 3.92 ml per 28 days (1 kit, 4 syringes/pen injectors) (50 mg once weekly dosing) Humira® Rheumatoid Arthritis • #2 per 28 days (1 kit or #2 syringes/vials) Ankylosing Spondylitis OR Psoriatic Arthritis • #4 per 28 days (2 kits or #4 syringes/vials) with documented treatment failure of 40 mg every other week (16 weeks of continuous therapy) AND medical reason for not using methotrexate (NOTE: dose of #4 per 28 days is NOT approvable if methotrexate is already being used in combination with Humira). Suggest Enbrel, Stelara or Taltz, depending on diagnosis, before increasing to weekly Humira. Juvenile Idiopathic #2 per 28 days (1 kit or 2 syringes, 20mg/0.4ml if 15-30kg in Arthritis weight or 40mg/0.8ml if >=30kg weight) Plaque Psoriasis • #4 per 28 days x 1 month (Psoriasis starter package, 4 x 40mg syringes) • Then #2 per 28 days (#1 kit/#2 syringes/pens) Crohn’s Disease • 40 mg #6 per 28 days x 1 month (Crohn’s Disease Ulcerative Colitis starter package, contains 6 x 40mg syringes) • Then 40 mg #2 per 28 days (#1 kit, #2 syringes/vials) Hidradenitis Suppurativa • #4 (160mg) on day 1, then 80mg on day 15 • Then 40mg weekly (#4 per 28 days) Cimzia® Ankylosing Spondylitis Initial: #3 per 28 days (400 mg weeks 0, 2, 4; dosed with starter kit of 3 sets of 2 syringes 200 ml each) Maintenance #1 per 28 days (200 mg every 2 weeks or 400 mg every 4 weeks; dosed with 1 set of 2 vials or 2 syringes 200 mg each) Crohn’s Disease Initial: #3 per 28 days (400 mg at weeks, 0, 2 and 4) Maintenance: #2 per 28 days (400 mg every 28 days) Plaque psoriasis #2 per 28 days (400 mg every14 days)

If ≤90kg, Initial: #3 per 28 days (400 mg at weeks, 0, 2 and 4). Maintenance: #2 per 28 days (400 mg every 28 days) Psoriatic Arthritis Initial: #3 per 28 days (400 mg at weeks, 0, 2 and 4) Rheumatoid Arthritis Maintenance: 200 mg (#1) every other week or 400 mg (#2) every 28 days Actemra® Rheumatoid Arthritis < 100 kg: #1.8 ml (2 syringes) per 28 days Juvenile Idiopathic ≥ 100 kg: #3.6 ml (4 syringes) per 28 days Arthritis Giant Cell Arteritis #3.6 ml (4 syringes) per 28 days Xeljanz® Rheumatoid Arthritis 60 tablets per 30 days Psoriatic Arthritis Ulcerative Colitis

Xeljanz XR® Rheumatoid Arthritis 30 tablets per 30 days Psoriatic Arthritis Ulcerative Colitis Cosentyx® Ankylosing spondylitis #4/28 days x 1 fill, then #1/28 days Psoriatic arthritis Taltz® Plaque Psoriasis Initial: #2 (160 mg) once for 14 days, then #1 (80 mg) week Psoriatic Arthritis 2, 4, 6, 8, 10 and 12 Ankylosing spondylitis Maintenance #1 per 28 days Siliq® Plaque Psoriasis Initial: 210 mg at week 0, 1, and 2 Maintenance 210 mg be every 2 weeks Tremfya® Plaque Psoriasis Initial: 100 mg (#1) weeks 0 and 4 Maintenance: 100 mg (#1) every 8 weeks Kevzara® Rheumatoid Arthritis Up to 200 mg (#1.140 ml) every 2 weeks Kineret® Rheumatoid Arthritis Up to 8 mg/kg daily Otezla® Plaque Psoriasis Initial: 10 mg on day 1, 10 mg twice daily day 2, 10 mg in Psoriatic Arthritis morning 20 mg in evening day 3, 20 mg twice daily day 4, 20 mg in morning and 30 mg in evening day 5 Maintenance: 30 mg twice daily starting on day 6 Olumiant Rhematoid Arthritis 30 tablets per 30 days Orencia® Psoriatic Arthritis Psoriatic Arthritis and Rheumatoid Arthritis: 125 mg once Rheumatoid Arthritis weekly Juvenile Idiopathic Juvenile Idiopathic Arthritis: 10 to <25 kg: 50 mg weekly Arthritis 25 to <50 kg: 87. 5 mg weekly 50 kg or more: 125 mg once weekly Simponi® Ankylosing Spondylitis Ankylosing spondylitis, psoriatic arthritis, rheumatoid Psoriatic Arthritis arthritis: 50 mg per 28 days Rheumatoid Arthritis Ulcerative colitis: Initial: 200 mg at week 0, 200 mg at week Ulcerative Colitis 2; Maintenance: 100 mg every 28 days Skyrizi® Plaque Psoriasis Initial: 2x75mg at Week 0 and Week 4

DISEASE MODIFYING BIOLOGICS Maintenance: 2x75mg every 12 weeks Stelara® Crohn’s Disease Crohn’s disease: Maintenance only, 90 mg every 8 weeks Plaque Psoriasis Plaque psoriasis: 100 kg or less: 45 mg at week 0, 4 and Psoriatic Arthritis then 45 mg every 12 weeks; >100 kg: 90 mg at week 0, 4, Ulcerative Colitis and then 90 mg every 12 weeks Psoriatic arthritis: 45 mg at week 0, 4 and then 45 mg every 12 weeks; coexistent plaque psoriasis and >100 kg: 90 mg at week 0, 4, and then 90 mg every 12 weeks

• Prescriber restriction: rheumatologist, dermatologist, or gastroenterologist (see specific diagnosis in Coverage Criteria) Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of moderate to severe rheumatoid arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) (except for Xeljanz and Xeljanz XR) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o Documented trial and failure with at least ONE DMARD (e.g. methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent OR early RA [less than 6 months from diagnosis] with poor prognosis (e.g. boney erosions, rheumatoid nodules, positive rheumatoid factor, and severe functional limitation) AND o For non-preferred medications Actemra®, Orencia®, Xeljanz®, Xeljanz XR®, Cimzia®, Kineret®, Olumiant®, Siliq®, Simponi®: trial and failure, intolerance, contraindication, or inability (e.g. inability to self-inject for Xeljanz® or Xeljanz® XR requests, drug interaction, allergy, adverse reaction, etc.) to use the following alternatives: Enbrel® and Humira® • For diagnosis of Ankylosing Spondylitis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of ankylosing spondylitis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND

DISEASE MODIFYING BIOLOGICS o Trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use at least ONE NSAID AND o For non-preferred medications Cimzia®, Simponi®,Taltz®, or Cosentyx®: trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use Enbrel® and Humira® • For diagnosis of Polyarticular Juvenile Idiopathic Arthritis, approve if: o Patient is 17 years of age or younger AND o Patient has diagnosis of juvenile idiopathic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed or is currently being supervised by a rheumatologist. AND o Documented trial and failure with at least ONE DMARD (e.g. methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent. AND o For non-preferred medications Actemra® and Orencia®: trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use Enbrel® and Humira® • For diagnosis of Systemic Juvenile Idiopathic Arthritis, approve if: o Patient is 17 years of age or younger AND o Patient has documented clinical diagnosis of juvenile clinical diagnosis of juvenile idiopathic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed or is currently being supervised by a rheumatologist AND o For non-preferred medications Actemra® and Orencia®: trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use Enbrel® and Humira® • For diagnosis of Psoriasis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of chronic moderate to severe plaque psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a dermatologist AND o Trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use at least 3 of the following alternatives - Topical steroids - Topical medications [i.e. Dovonex® (calcipotriene), Tazorac® (tazarotene), anthralin or a coal tar preparation] - Methotrexate (inability to use examples include but not limited to history of liver or kidney disease, pregnancy, severe cytopenia, alcoholism) - Cyclosporine - Acitretin (Soriatane®)

DISEASE MODIFYING BIOLOGICS - UVB phototherapy or PUVA (psoralen – oral or topical methoxsalen plus UVA therapy) (inability to use examples include but not limited to pregnancy, skin cancer, hypersensitivity due to preexisting disease state - e.g. systemic lupus erythematus, cataracts) - Prior trial of disease modifying biologic o For non-preferred medications Kevzara®, Otezla®, Siliq®, Stelara®, Taltz®,Cimzia®, or Tremfya®: trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following Enbrel®, Humira®, Cosentyx®, and Skyrizi® • For diagnosis of guttate psoriasis, approve if: o Patient has moderate to severe guttate psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Medication is being prescribed by a dermatologist AND o There is documentation of trial and failure, intolerance or inability (e.g. drug interaction, allergy, adverse reaction, etc.) to treatment with ALL of the following alternatives: There is documentation of trial and failure, intolerance or inability (e.g. drug interaction, allergy, adverse reaction, etc.) to use the following: 1. Medium to high potency steroid or another topical medication (e.g. calcipotriene, tazorotene, anthralin, or a coal tar preparation) 2. Ultraviolet (UV) phototherapy 3. Oral DMARDs (e.g. methotrexate) o For non-preferred medications Taltz®, Otezla®, or Stelara®: trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following Enbrel, Humira, Cosentyx®, and Skyrizi ® • For diagnosis of Psoriatic Arthritis, approve if: o Patient is 18 years of age or older AND o Diagnosis of psoriatic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) (Except Xeljanz and Xeljanz XR) o Drug is being prescribed by a rheumatologist or dermatologist AND o Documented trial and failure, intolerance, contraindication with at least one DMARD (e.g. methotrexate) or inability to use DMARD (e.g. liver toxicity with methotrexate) OR predominantly axial symptoms ( i.e. spinal column or sacral involvement) or active enthesitis (tendon swelling) and/or dactylitis (toe/finger swelling) with trial and failure of NSAIDS or steroids AND o For non-preferred medications Cimzia®, Cosentyx®, Simponi®, Otezla®, Orencia®, Xeljanz®, Xeljanz XR® or Taltz®: trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following Enbrel, Humira, Cosentyx®, and Skyrizi ® • For diagnosis of Crohn’s Disease, approve if:

DISEASE MODIFYING BIOLOGICS o Patient is 6 years of age or older AND o Patient has a diagnosis of moderate to severely active Crohn’s Disease AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a gastroenterologist or rheumatologist AND o Patient has documented trial and failure of one or more conventional therapies for Crohn’s Disease such as corticosteroids, azathioprine, mercaptopurine, methotrexate, or mesalamine. AND o For non-preferred medication Cimzia® trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use Humira® • For diagnosis of Hidradenitis Suppurativa, approve if: o Patient is 12 years of age or older AND o Patient has a diagnosis of moderate to severe hidradenitis suppurativa (Hurlet stage II and III) o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a Dermatologist AND o Patient has documented trial and failure of one or more conventional therapies for hidradenitis suppurativa such as acitrentin, isotretinoin, alitretinoin, hormonal therapy, and systemic antibiotics • For diagnosis of Ulcerative Colitis, approve if: o Patient is 6 years of age or older AND o Request is for subcutaneous administration (self-administration or by caregiver at home) (Except Xeljanz and Xeljanz XR) o Drug is being prescribed by a gastroenterologist AND o Trial and failure or inability (i.e. drug interaction, allergy, adverse reaction, GI intolerance, etc.) to use sulfasalazine, mesalamine, azathioprine, 6-mercaptopurine or oral corticosteroids AND o For non-preferred medication Stelera or Simponi®: trial and failure, intolerance, contraindication or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use Humira® • For diagnosis of Giant Cell Arteritis, approve if: o Patient is 18 years of age or older AND o Documented trial and failure to at least 3 months of glucocorticoids

DISEASE MODIFYING BIOLOGICS • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 07/2019

CALCITONIN GENE-RELATED PEPTIDE (CGRP) RECEPTOR ANTAGONISTS Standard/Specific Therapeutic Class: Non-narcotic analgesics/Antimigraine preparations Formulary Status: • Formulary, PA required: o Emgality™ (galcanezumab) o Aimovig™ (erenumab) • Non-formulary, PA required: o Ajovy™ (fremanezumab) Coverage Duration: Initial: 6 months Renewal: Indefinite Diagnosis Considered for Coverage: • Migraine headache (episodic or chronic) • Cluster headaches (Emgality™ (galcanezumab) only) • Off-label uses: medically accepted indications are defined using the following sources: American Hospital Formulary Service-Drug Information (AHFS-DI), Truven Health Analytics Micromedex DrugDEX (DrugDEX), National Comprehensive Cancer Network (NCCN) Drugs and Biologics Compendium, Wolters Kluwer Lexi- Drugs, and Elsevier/Gold Standard Clinical Pharmacology and/or positive results from two peer-reviewed published studies Prescribing Restriction: • Quantity*: o Aimovig™: 2 auto-injectors (140mg) per 30 days o Ajovy™: 3 syringes (675mg) per 90 days o Emgality™: 1 auto-injector or syringe (120mg) per 30 days • Prescriber: Prescribed by a neurologist or in consultation with a neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis

Clinical Information required for Review: • Diagnosis • Previous therapy • Dose 1. For migraine headache, approve if: • Patient must have at least 4 migraine days per month or one or more severe migraines lasting for greater than 12 hours despite use of abortive therapy (e.g. triptan or NSAIDs) AND • There is documentation of trial and failure, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least one drug from two categories below for at least 4 weeks EACH, at minimum effective doses, AND: • Beta-adrenergic blockers • Topiramate or divalproex ER or DR • Amitriptyline or venlafaxine • Frovatriptan, zolmitriptan or naratriptan (for menstrual migraine prophylaxis) • For Ajovy, documentation of trial and failure, intolerance, contraindication, or inability (i.e drug interaction, allergy, adverse reaction, etc.) to use Aimovig and Emgality 2. Continuation of Therapy for NEW Members (within the last 6 months), approve if: Prescriber attests that member has been on this medication continuously before joining AND Request is for generic or single source brand AND The diagnosis and dosage provided meets FDA labeling and/or drug-specific criteria or off-label criteria 3. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: Documentation supporting re-evaluation of patient and a reduction in number of headache days by at least 1 day per month during the initial authorization period References: N/A Last review/revision date: 8/2019

SYNAREL (Histrelin) & ORILISSA (elagolix) Standard/Specific Therapeutic Class: LHRH (GNRH) Agonist Analogue Pituitary Suppressants, LHRH (GNRH) Agonist Analogue And Progestin Combinations, Androgens Formulary Status: • Formulary, PA required: o Synarel® (histrelin) nasal spray o Orilissa™ (elagolix) tablets Coverage Duration: 6 months Diagnosis Considered for Coverage: • Endometriosis/uterine fibroids • Central Precocious Puberty (Synarel® only) • Off-label uses: medically accepted indications are defined using the following sources: American Hospital Formulary Service-Drug Information (AHFS-DI), Truven Health Analytics Micromedex DrugDEX (DrugDEX), National Comprehensive Cancer Network (NCCN) Drugs and Biologics Compendium, Wolters Kluwer Lexi- Drugs, and Elsevier/Gold Standard Clinical Pharmacology and/or positive results from two peer-reviewed published studies (for pediatric indications and products, see GONADOTROPIN RELEASING HORMONE (GnRH) AGONISTS- PEDIATRIC Criteria) Prescribing Restriction: • Quantity Limit: o Orilissa™: § 150mg tablet: #30 tablets per 30 days § 200mg tablet: #60 tablets per 30 days o Synarel®: 12mL per 30 days or 27ml per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case by case basis Clinical Information Required for Review: • Diagnosis • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For endometriosis, approve if: o There is documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use combined oral contraceptive or progestin AND o For Synarel®, there is documentation of trial and failure, intolerance, contraindication, or inability (i.e. drug interaction, allergy, adverse reaction, etc.) to use an injectable GnRH agonist

• For Central Precocious Puberty (Synarel®only) approve if:

o Diagnosis of central Precocius Puberty confirmed by: 1. Elevated basal luteinizing hormone (LH) level > 0.2 -0.3 mlU/L OR elevated leuprolide-stimulated LH level >3.3-5 IU/l AND 2. Difference between bone age and chronological age was > 1 year AND 3. Age at onset of sex characteristics is <8 years if female or <9 years if male AND o Prescribed by or in consultation with a pediatric endocrinologist AND o If female, member is 2-11 years of age AND o If male, member is 2-12 years of age AND o Member is not pregnant o Dose is no more than 1800mcg per day

• For off-label indications or dosing, approve if: o No other formulary medication has a medically accepted use for the patient’s specific diagnosis as referenced in the medical compendia AND o Medication is being requested for an accepted off-label use and is listed in the standard clinical decision support resources (as noted in Diagnosis section above) OR o Requested use can be supported by at least two published peer reviewed clinical studies

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Prescriber attests that member has been on this medication continuously before joining AND • Request is for generic or single source brand AND • The diagnosis and dosage provided meets FDA labeling and/or drug-specific criteria or off-label criteria AND • For Orilissa™ 150mg QD dose only, maximum 24 months total duration • For Orilissa™ 200mg BID dose only, maximum 6 months total duration

PICATO (INGENOL) Standard/Specific Therapeutic Class: Dermatological Fomulary Status: Formulary, PA required Coverage Duration: 1 year Diagnosis Considered for Coverage: Actinic Keratosis Prescribing Restriction: • 0.015% Gel: 3 tubes/90 days • 0.05% Gel: 2 tubes/90 days Clinical Information required for Review: • Diagnosis Coverage Criteria: a. The patient’s medication history includes generic fluorouracil cream (5%) or generic imiquimod cream (5%) in the past 90 days OR b. The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to an available generic fluorouracil cream (5%) or generic imiquimod cream (5%) References: N/A Last Review/Revision date: 5/2019

STRENSIQ (ASFOTASE ALFA) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents Fomulary Status: Formulary, PA required Coverage Duration: Initial: 1 year, Renewal: Indefinite Diagnosis Considered for Coverage: Perinatal/infantile OR juvenile-onset hypophosphatasia (HPP) Prescribing Restriction: • See quantity in “j” Clinical Information required for Review: • Diagnosis • Appropriate laboratory values • Patient weight Coverage Criteria: Initiation of Therapy: a. The patient was ≤ 18 years of age at onset AND b. The patient has/had clinical manifestations consistent with hypophosphatasia at the age of onset prior to age 18 (e.g. vitamin B6- dependent seizures, skeletal abnormalities: such as rachitic chest deformity leading to respiratory problems or bowed arms/legs, “failure to thrive”) AND c. The patient has/had radiographic imaging to support the diagnosis of hypophosphatasia at the age of onset prior to age 18 (e.g. infantile rickets, Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 109 Prior Authorization Criteria April 2020

alveolar bone loss, craniosynostosis) AND d. Molecular genetic test has been completed confirming mutations in the ALPL gene that encodes the tissue nonspecific isoenzyme of ALP (TNSALP) AND e. Reduced activity of unfractionated serum alkaline phosphatase (ALP) in the absence of bisphosphonate therapy (i.e. below the normal lab reference range for age and sex) AND f. ONE of the following: i. Elevated urine concentration of phosphoethanolamine (PEA) OR ii. Elevated serum concentration of pyridoxal 5'-phosphate (PLP) in the absence of vitamin supplements within one week prior to the test OR iii. Elevated urinary inorganic pyrophosphate (PPi) AND g. The prescriber is a specialist in the area of the patient’s diagnosis (e.g. endocrinologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND h. The patient does not have any FDA labeled contraindication(s) to the requested agent AND i. The prescriber has provided the patient’s weight AND j. The requested quantity is within FDA labeled dosing (based on patient’s weight) Continuation of Therapy: 1. The patient has been previously approved for the requested agent through the PA process AND 2. The prescriber is a specialist in the area of the patient’s diagnosis (e.g. endocrinologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 3. The patient has shown clinical improvement with the requested agent as evidenced by an improvement and/or stabilization (upon subsequent renewals) respiratory status, growth, or radiographic findings from baseline [documentation from the medical record is required to be submitted] AND 4. The prescriber has provided the patient’s weight AND 5. The requested quantity is within FDA labeled dosing (based on patient’s weight) References: N/A Last Review/Revision date: 7/2019

GATTEX (TEDUGLITIDE) Standard/Specific Therapeutic Class: Gastrointestinal Agents Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months, Renewal: Indefinite Diagnosis Considered for Coverage: Short Bowel Syndrome Prescribing Restriction: • See quantity in “3 or 4” Clinical Information required for Review: • Diagnosis • Medical Record confirmations (see below) • Patient weight Coverage Criteria: Initiation of Therapy: 1. • The patient is an adult with less than 200 cm of bowel remaining [medical records including chart notes are required] AND • ONE of the following: • The patient has had an inadequate response to maximal use of two anti-diarrheal agents (i.e. loperamide AND diphenoxylate) taken in conjunction with oral rehydration solution [medical records including chart notes are required] OR • The patient has a documented intolerance to loperamide, diphenoxylate, and oral rehydration solution AND • The patient is currently receiving parenteral nutrition/intravenous fluids (PN/IV) at least 3 days per week [medical records are required] AND • BOTH of the following: • The patient has had a colonoscopy within the last 6 months [medical records are required] AND • If polyps were present at this colonoscopy, the polyps were removed AND 2. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., gastroenterologist) or has consulted with a specialist in the area of the patient’s diagnosis AND 3. The dose is within the FDA-labeled dose for the requested diagnosis Continuation of Therapy: 1. The patient has been previously approved forthe requested agent through the PA process AND 2. The prescriber is a specialist in the area of the patient’s diagnosis (e.g.gastroenterologist) or has

consulted with a specialist in the area of the patient’s diagnosis AND 3. If the patient is using parenteral nutrition/intravenous fluids (PN/IV), the patient has had at least a 20% reduction from baseline in PN/IV fluid volume [medical records including home parenteral nutrition (HPN) order sheet are required] AND 4. The dose is within the FDA-labeled dose for the requested diagnosis References: N/A Last Review/Revision date: 5/2019

RAYOS (PREDNISONE DELAYED RELEASE TABLET) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents, Corticosteroids Fomulary Status: Non-Formulary, PA required Coverage Duration: 6 months Diagnosis Considered for Coverage: Rheumatoid Arthritis, COPD Prescribing Restriction: • Quantity Limit: 30 tablets/30 days Clinical Information required for Review: • Diagnosis Coverage Criteria: 1. The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to generic oral prednisone AND at least 1 other different generic oral corticosteroids (e.g. dexamethasone, methylprednisolone, prednisolone) that would not be not expected to occur with the requested agent AND 2. The prescriber has submitted documentation regarding the medical necessity of the requested agent over generic prednisone AND 3. ONE of the following: a. The requested quantity (dose) does not exceed the program quantity limit OR b. ALL of the following i. The requested quantity (dose) is greater than the quantity limit AND ii. The requested quantity (dose) does not exceed the maxiumum FDA labeled dose AND iii. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit OR c. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit OR ii. The requested quantity (dose) is greater than the maximum FDA labeled dose AND iii. The prescriber has submitted documentation in support of therapy with a higher dose for the requested indication References: N/A Last Review/Revision date: 7/2019

EVZIO (NALOXONE AUTO-INJECTOR) Standard/Specific Therapeutic Class: Antidotes and Specific Antagonists Fomulary Status:Non-Formulary, PA required Coverage Duration: 6 months Diagnosis Considered for Coverage: Risk for Opioid Overdose Prescribing Restriction: • Quantity Limit: 1 autoinjector/30days Clinical Information required for Review: • Diagnosis • Medical Record confirmations (see below) Coverage Criteria: Initiation of Therapy: 1. Patient has ONE of the following risk factors for opioid overdose: a. History of opioid overdose OR b. History of substance use disorder OR c. The patient is receiving ≥ 50 morphine milligram equivalents (MME) per day OR d. The patient is concomitantly using opioids with another Central Nervous System (CNS) depressant (e.g. benzodiazepines, alcohol, or muscle relaxants) OR e. The prescriber has provided documentation of another risk factor AND 2. ONE of the following: a. The patient has a documented inability to use generic naloxone injection AND Narcan Nasal Spray OR b. The patient has an FDA labeled contraindication, intolerance, or hypersensitivity to generic naloxone injection AND Narcan Nasal Spray that is not expected to occur with the requested agent AND 3. ONE of the following: A. The requested quantity (dose) is NOT greater than the program quantity limit OR B. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) is less than or equal to the FDA labeled dose AND iii. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit OR C. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) is greater than the FDA labeled dose AND iii. The prescriber has submitted documentation in support of therapy with a higher dose for the intended diagnosis (must be reviewed by the Clinical Review pharmacist) References: N/A Last Review/Revision date: 5/2019 Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 113 Prior Authorization Criteria April 2020

NORTHERA (DROXIDOPA) Standard/Specific Therapeutic Class: Vasopressors Fomulary Status: Formulary, PA required Coverage Duration: Initial: 1 month Renewal: 3 months Diagnosis Considered for Coverage: Neurogenic Orthostatic Hypotension (NOH) Prescribing Restriction: Quantity Limits: • 100mg: 15 Caps/day • 200mg or 300mg: 6 Caps/day Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. ALL of the following • The patient is 18 years of age or over AND • The prescriber has performed baseline blood pressure readings while the patient is sitting or in supine position AND also within 3 minutes of standing from a supine (lying face up) position AND • The patient has a decrease of at least 20 mmHg in systolic blood pressure or 10 mmHg diastolic blood pressure within three minutes after standing AND • The patient has persistent and consistent symptoms of neurogenic orthostatic hypotension (NOH) caused by ONE of the following: • Primary autonomic failure [Parkinson's disease (PD), multiple system atrophy, or pure autonomic failure] OR • Dopamine beta-hydroxylase deficiency OR • Non-diabetic autonomic neuropathy AND • The prescriber has assessed the severity of the patient’s baseline symptoms of dizziness, lightheadedness, feeling faint, or feeling like the patient may black out AND • The prescriber has assessed and adjusted, if applicable, any medications known to exacerbate orthostatic hypotension (e.g., diuretics, vasodilators, beta-blockers) AND • The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to midodrine OR • The patient has another FDA approved diagnosis for the requested agent AND 2. The prescriber is a specialist in the area of practice related to the patient’s diagnosis (e.g. cardiologist, neurologist) or the prescriber has consulted with a specialist in the area of practice related to the patient’s diagnosis AND

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 114 Prior Authorization Criteria April 2020 3. ONE of the following: • The requested quantity (dose) is NOT greater than the program quantity limit OR • ALL of the following: • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is less than or equal to the FDA labeled dose AND • The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit OR • ALL of the following: • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is greater than the FDA labeled dose AND • The prescriber has submitted documentation in support of therapy with a higher dose for the intended diagnosis (must be reviewed by the Clinical Review pharmacist)

Continuation of Therapy: 1. The patient has been previously approved for the requested agent through the prior authorization process AND 2. ONE of the following: • The patient has a diagnosis of neurogenic orthostatic hypotension (NOH) AND BOTH of the following: • The patient has demonstrated improvement in severity from baseline symptoms of dizziness, lightheadedness, feeling faint, or feeling like the patient may black out AND • The patient had an increase in systolic blood pressure from baseline of at least 10 mmHg upon standing from a supine (laying face up) position OR • The patient has another FDA approved diagnosis for the requested agent AND 3. The prescriber is a specialist in the area of practice related to the patient’s diagnosis (for example: cardiologist, neurologist) or the prescriber has consulted with a specialist in the area of practice related to the patient’s diagnosis AND 4. ONE of the following: • The requested quantity (dose) is NOT greater than the program quantity limit OR • ALL of the following • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is less than or equal to the FDA labeled dose AND • The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit OR • ALL of the following: • The requested quantity (dose) is greater than the program quantity Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 115 Prior Authorization Criteria April 2020 limit AND • The requested quantity (dose) is greater than the FDA labeled dose AND • The prescriber has submitted documentation in support of therapy with a higher dose for the intended diagnosis References: N/A Last Review/Revision date: 7/2019

NATPARA (PARATHYROID HORMONE) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Hypocalcemia Associated with Hypoparathyroidism Prescribing Restriction: • Quantity Limit: 2 Cartridges/28 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. ONE of the following: A. The patient has the diagnosis of AND ALL of the following: a. The patient has baseline vitamin D levels above the lower limit of normal AND b. The patient has baseline serum calcium levels above 7.5 mg/dL AND c. The patient has had an inadequate response to maximally tolerated calcium AND vitamin D supplements (e.g., calcitriol, ergocalciferol, cholecalciferol) AND d. The patient will continue calcium and vitamin D supplementation with the requested agent AND e. The patient does NOT have hypoparathyroidism caused by calcium- sensing receptor mutations AND f. The patient does NOT have acute post-surgical hypoparathyroidism OR B. The patient has another FDA approved indication for the requested agent AND 2. The patient is NOT at an increased risk for osteosarcoma (including those with Paget’s disease of bone or unexplained elevations of alkaline phosphatase, pediatric and young adult patients with open epiphyses, patients with hereditary disorders predisposing to osteosarcoma or patients with a history of prior external beam or implant radiation therapy involving the skeleton) AND 3. ONE of the following: A. The patient is NOT currently being treated with alendronate OR B. The patient is currently being treated with alendronate AND will discontinue prior

to initiating the requested agent AND 4. The prescriber is a specialist (e.g. endocrinologist) in the area of the patient’s diagnosis or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 5. The patient does NOT have any FDA labeled contraindication(s) to the requested agent AND 6. ONE of the following: A. The requested quantity (dose) is NOT greater than the program quantity limit OR B. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) is less than or equal to the maximum FDA labeled dose AND iii. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit OR C. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) is greater than the maximum FDA labeled dose AND iii. The prescriber has submitted documentation in support of therapy with a higher dose for the requested indication References: N/A Last Review/Revision date: 5/2019

MYALEPT (METRELEPTIN) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Congenital Generalized Lipodystrophy (CGL) Or Acquired Generalized Lipodystrophy (AGL) Prescribing Restriction: Quantity Limits: 1 month based on body weight Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. The patient has a diagnosis of leptin deficiency AND 2. The patient does NOT have any of the following: partial lipodystrophy, liver disease (non- alcoholic steatohepatitis), HIV-related lipodystrophy, or generalized metabolic disease without generalized lipodystrophy AND 3. The prescriber is a specialist in the area of the patient’s diagnosis (e.g. endocrinologist) Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 117 Prior Authorization Criteria April 2020

or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 4. The patient has complications related to lipodystrophy [e.g. diabetes mellitus, hypertriglyceridemia (≥200 mg/dL), and/or high fasting insulin (≥30µU/mL)] AND 5. The patient has had an inadequate response to maximally tolerable conventional agent for complications related to lipodystrophy AND 6. The patient has had an inadequate response to lifestyle modification (i.e. diet modification and exercise) and will continue lifestyle modifications with the requested agent AND 7. The dose is within FDA labeled dose References: N/A Last Review/Revision date: 7/2019

KORLYM (MIFEPRISTONE) Standard/Specific Therapeutic Class: Antidiabetics Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Cushing’s Syndrome Prescribing Restriction: Quantity Limits: 120 tablets/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: 1. ONE of the following: • The patient has type 2 diabetes mellitus OR • The patient has glucose intolerance as defined as a 2-hr glucose tolerance test glucose value of 140-199 mg/dL AND 2. ONE of the following: • The patient has had an inadequate response to surgical resection OR • The patient is NOT a candidate for surgical resection AND 3. The patient does NOT have any FDA labeled contraindication(s) to the requested agent AND 4. The requested dose does NOT exceed 20 mg/kg/day AND 5. ONE of the following: • The requested quantity (dose) is less than or equal to the program quantity limit OR • ALL of the following: • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is less than or equal to the maximum FDA labeled dose AND • The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit References: N/A Last Review/Revision date: 5/2019

INTERLEUKIN 1 ANTAGONISTS Standard/Specific Therapeutic Class: Analgesics-Antiinflammatories Fomulary Status: Formulary, PA required ARCALYST (RILONACEPT) ILARIS (CANAKINUMAB) Coverage Duration: Initial: 3 months Renewal: 12 months Diagnosis Considered for Coverage: See Below Prescribing Restriction: Quantity Limits: • Arcalyst: 4 vials /28 days after 320mg loading dose • Ilaris: 2 vials /28 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: Arcalyst (rilonacept) will be approved when ALL of the following are met: 1. The patient has ONE Of the following diagnoses • Cryopyrin Associated Periodic Syndrome (CAPS) • Familial Cold Auto-Inflammatory Syndrome (FCAS) • Muckle-Wells Syndrome (MWS) • Another FDA approve diagnosis AND 2. The patient is 12 years of age and over AND 3. The prescriber is a specialist in the area of the patient’s requested indication or has consulted with a specialist in the area of the patient’s requested indication (e.g. allergist, autoimmune specialist, immunologist, pediatrician) AND 4. ONE of the following: • The patient is NOT currently being treated with another biologic immunomodulator OR • The patient is currently being treated with another biologic immunomodulator and it will be discontinued prior to starting the requested agent AND 5. The patient does NOT have any FDA labeled contraindication(s) to the requested agent AND 6. ONE of the following: • The quantity (dose) requested is less than or equal to the program quantity limit OR • ALL of the following: • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is less than or equal to the FDA labeled

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 120 Prior Authorization Criteria April 2020 dose AND • The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit • ALL of the following: • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is greater than the FDA labeled dose AND • The prescriber has submitted documentation in support of therapy with a higher dose for the intended diagnosis (must be reviewed by the Clinical Review pharmacist) Ilaris (canakinumab) will be approved when ALL of the following are met: 1. The patient has ONE of the following diagnoses: • Cryopyrin Associated Periodic Syndrome (CAPS) OR • Familial Cold Auto-Inflammatory Syndrome (FCAS) OR • Muckle-Wells Syndrome (MWS) OR • Familial Mediterranean Fever (FMF) AND ONE of the following: • The patient has tried and had an inadequate response to colchicine for at least 6 months OR • The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to colchicine OR • Hyperimmunoglobulin D Syndrome (HIDS) or Mevalonate Kinase (MKD) AND ONE of the following: • The patient has tried and had an inadequate response to BOTH NSAIDs and glucocorticosteroids (e.g. prednisone, prednisolone) OR • The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to BOTH NSAIDs and glucocorticosteroids OR • Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) OR • Active systemic Juvenile Idiopathic Arthritis (SJIA) AND BOTH of the following: • The patient has documented active systemic features (e.g. ongoing fever for at least 2 weeks, anemia, rash, C-Reactive Protein levels >50 mg/L, ≥1 joint with active arthritis, hepatomegaly, splenomegaly, etc) AND • ONE of the following: • The patient has tried and had an inadequate response to TWO of the following: methotrexate, leflunomide, or systemic glucocorticoids (oral or IV), for at least 3 month trial each, or NSAID 1 month trial is accepted OR • The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to ALL prerequisite agents OR • Another FDA approved diagnosis AND 2. The patient is within the FDA labeled age for the requested indication AND Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 121 Prior Authorization Criteria April 2020

3. The prescriber is a specialist in the area of the patient’s requested indication or has consulted with a specialist in the area of the patient’s requested indication (e.g. immunologist, pediatrician, rheumatologist) AND 4. ONE of the following: • The patient is NOT currently being treated with another biologic immunomodulator OR • The patient is currently being treated with another biologic immunomodulator and it will be discontinued prior to starting the requested agent AND 5. The patient does NOT have any FDA labeled contraindication(s) to the requested agent AND 6. ONE of the following: • The quantity (dose) requested is less than or equal to the program quantity limit OR • ALL of the following: • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is less than or equal to the FDA labeled dose AND • The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit OR Continuation of Therapy: The targeted agent will be approved when ALL of the following are met: 1. The patient has been previously approved for the requested agent through Prior Authorization process AND 2. The patient has shown clinical improvement with the requested agent (i.e. improvement in serum levels of C-Reactive Protein (CRP), improvement in Serum Amyloid A (SAA), slowing of disease progression, decrease in symptom severity and/or frequency) AND 3. The prescriber is a specialist in area of the patient’s requested indication or has consulted with a specialist in the area of the patient’s requested indication (e.g. immunologist, pediatrician, rheumatologist) AND 4. The patient is NOT currently being treated with another biologic immunomodulator agent AND 5. ONE of the following: a. The quantity (dose) requested is less than or equal to the program quantity limit OR b. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) is less than or equal to the FDA labeled dose AND iii. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit References: N/A Last Review/Revision date: 7/2019

FAMILIAL HYPERCHOLESTEROLEMIA Standard/Specific Therapeutic Class: Antihyperlipidemics Fomulary Status: Formulary, PA required JUXTAPID (LOMITAPIDE) KYNAMRO (MIPOMERSEN) Coverage Duration: Initial: 6 months Renewal: Indefinite Diagnosis Considered For Coverage: Homozygous Familial Hypercholesterolemia (HoFH) Prescribing Restriction: Quantity Limits: • Juxtapid: 30 caps/30days • Kynamro: 4 injections/28 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: Target agents will be approved when ALL of the following are met: 2. ONE of the following: a. The patient has the diagnosis of and ALL of the following: i. The patient has a confirmed diagnosis of homozygous familial hypercholesterolemia (HoFH), through ONE of the following: • Genetic confirmation of two mutant alleles at the LDLR, Apo- B, PCSK9, ARH adaptor protein 1/LDLRAP1 gene locus OR • History of untreated LDL-C >500 mg/dL (>13 mmol/L) or treated LDL-C ≥300 mg/dL (≥7.76 mmol/L) with ONE of the following: • The patient had cutaneous or tendon xanthoma before age 10 years OR • Untreated elevated cholesterol levels consistent with heterozygous FH in both parents [untreated LDL-C >190 mg/dL (>4.9 mmol/L) or untreated total cholesterol greater than 290 mg/dL (>7.5 mmol/L)] AND ii. ONE of the following: • The patient is on a maximally tolerated statin containing lipid- lowering regimen (i.e. rosuvastatin in combination with ezetimibe or atorvastatin in combination with ezetimibe) OR • The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to ALL of these therapies (i.e. rosuvastatin in combination with ezetimibe and atorvastatin in combination with ezetimibe) AND iii. ONE of the following: • The patient has tried with demonstrated adherence for at least Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 123 Prior Authorization Criteria April 2020 3 months and had an inadequate response to a PCSK9 inhibitor (e.g. Repatha, Praluent) OR • The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to ALL PCSK9 inhibitors AND iv. If Juxtapid (lomitapide) is requested, the patient is taking daily vitamin E, linoleic acid, alpha-linolenic acid (ALA), eicosapentaenoic acid (EPA), and docosahexaenoic acid (DHA) supplements AND vi. If Kynamro (mipomersen) is requested, the patient will NOT be receiving apheresis while on therapy with mipomersen OR b. The patient has another FDA approved diagnosis AND 3. The prescriber is a specialist in the area of the patient’s diagnosis (e.g. cardiologist, endocrinologist, lipid specialist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis 4. ONE of the following: I. The requested quantity (dose) does NOT exceed the program quantity limit OR II. ALL of the following: a. The requested quantity (dose) is greater than the program quantity limit AND b. The requested quantity (dose) does NOT exceed the maximum FDA labeled dose (for the requested indication) AND c. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit

Continuation of Therapy: 1. The patient has been previously approved for therapy with the requested agent through Prior Authorization process AND 2. The patient has shown clinical benefit with the requested agent AND 3. If Juxtapid (lomitapide) is requested, the patient is taking daily vitamin E, linoleic acid, alpha- linolenic acid (ALA), eicosapentaenoic acid (EPA), and docosahexaenoic acid (DHA) supplements AND 4. If Kynamro (mipomersen) is requested, the patient will NOT be receiving apheresis while on therapy with mipomersen AND 5. The prescriber is a specialist in the area of the patient’s diagnosis (e.g. cardiologist, endocrinologist, lipid specialist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 6. ONE of the following: a. The requested quantity (dose) does NOT exceed the program quantity limit OR b. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) does NOT exceed the maximum FDA labeled dose (for the requested indication) AND iii. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit References: N/A Last Review/Revision date: 5/2019 Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 124 Prior Authorization Criteria April 2020

HEMLIBRA (EMICIZUMAB-KXWH) Standard/Specific Therapeutic Class: Hemotological Agents-Mescellaneous Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Renewal: Indefinite Diagnosis Considered For Coverage: Hemophilia A with or without Factor VIII Inhibitors Prescribing Restriction: Quantity Limits: • Weight Based. • Smallest number of vials to meet the dosing needs. • No more than a 30 days supply. Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. ONE of the following: A. The patient has a diagnosis of hemophilia A with Factor VIII inhibitors (hemophilia A with inhibitors) AND ALL of the following i. The requested agent is prescribed for prophylactic use AND ii. ONE of the following: 1. The patient’s inhibitor level is ≥ 5 BU OR 2. The patient has tried and had an inadequate response to Immune Tolerance Therapy (ITT) [Immune Tolerance Induction (ITI)] OR 3. The patient is using a bypassing agent (Feiba, NovoSeven) for on- demand treatment and is not adequately controlled OR 4. The patient is using a bypassing agent for prophylaxis and had an inadequate response OR 5. The patient is using more than 5 doses per week of a bypassing agent OR 6. The patient has a documented intolerance, FDA labeled contraindication or hypersensitivity to a bypassing agent AND iii. If the patient is receiving Feiba [activated prothrombin complex concentrate (aPCC)] for breakthrough bleeds, and BOTH of the following: 1. The patient will be monitored for thrombotic microangiopathy and thromboembolism AND 2. The prescriber has counseled the patient on the maximum dosages of Feiba to be used (i.e., no more than 100 u/kg/24 hours) OR ii. The patient has a diagnosis of hemophilia A without Factor VIII Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 125 Prior Authorization Criteria April 2020 inhibitors (hemophilia A) AND BOTH of the following: a. The requested agent is being prescribed for prophylactic use AND b. The prescriber has submitted documentation supporting ONE of the following: 1. The patient has tried and failed to be adequately controlled on prophylaxis with a Factor VIII clotting factor agent (e.g., Advate, Adynovate, Eloctate, Nuwiq, Recombinate, Xyntha) after at least 50 exposure days. Documentation including chart notes and/or treatment logs must be submitted OR 2. The patient has poor venous access. Documentation including chart notes must be submitted OR 3. The patient failed to achieve an adequate trough level while on clinically optimal dose and frequency of a Factor VIII clotting factor agent. Documentation including chart notes must be submitted OR 4. The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to prophylaxis with a Factor VIII clotting factor agent v. The prescriber has submitted documentation supporting ONE of the following: 1. The patient has tried and failed to be adequately controlled on prophylaxis with a Factor VIII clotting factor agent (e.g., Advate, Adynovate, Eloctate, Nuwiq, Recombinate, Xyntha) after at least 50 exposure days. Documentation including chart notes and/or treatment logs must be submitted OR 2. The patient has poor venous access. Documentation including chart notes must be submitted OR 3. The patient failed to achieve an adequate trough level while on clinically optimal dose and frequency of a Factor VIII clotting factor agent. Documentation including chart notes must be submitted OR 4. The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to prophylaxis with a Factor VIII clotting factor agent 2. The prescriber has discussed with the patient that a treatment log, documenting at least 6 months of bleeds prior to starting the requested agent, and which includes ALL of the following must be maintained and a copy will be submitted (via prescriber or pharmacy) for renewal purposes *note if a historical bleed log is unavailable, a new log must be started and submitted for renewal A. Date of the bleed AND B. The treatment used (include the brand name and number of units administered) AND C. The number of doses required to treat the bleed AND 3. The prescriber is a specialist in the area of the patient’s diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or has consulted with a specialist in the area of the patient’s diagnosis AND Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 126 Prior Authorization Criteria April 2020 4. If the patient is using a Factor VIII product (e.g., Advate, Adynovate, Eloctate, Nuwiq, Recombinate, Xyntha) or a bypassing agent (e.g., Feiba, NovoSeven) for prophylaxis treatment, the prophylaxis with the Factor VIII product or bypassing agent will be discontinued (on-demand treatment is acceptable to continue) AND 5. The prescriber has provided the patient’s weight AND 6. The requested dose is within the FDA labeled dosing based on the patient’s weight AND 7. ONE of the following: A. The patient is requesting induction therapy and maintenance therapy and the requested quantity for maintenance therapy is NOT greater than the allowed quantity OR 8. The patient is requesting maintenance therapy only and the requested quantity is NOT greater than the allowed 9. The prescriber has discussed with the patient that a treatment log, documenting at least 6 months of bleeds prior to starting the requested agent, and which includes ALL of the following must be maintained and a copy will be submitted (via prescriber or pharmacy) for renewal purposes *note if a historical bleed log is unavailable, a new log must be started and submitted for renewal A. Date of the bleed AND B. The treatment used (include the brand name and number of units administered) AND C. The number of doses required to treat the bleed AND 10. The prescriber is a specialist in the area of the patient’s diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or has consulted with a specialist in the area of the patient’s diagnosis AND 11. If the patient is using a Factor VIII product (e.g., Advate, Adynovate, Eloctate, Nuwiq, Recombinate, Xyntha) or a bypassing agent (e.g., Feiba, NovoSeven) for prophylaxis treatment, the prophylaxis with the Factor VIII product or bypassing agent will be discontinued (on-demand treatment is acceptable to continue) AND 12. The prescriber has provided the patient’s weight AND 13. The requested dose is within the FDA labeled dosing based on the patient’s weight AND 14. ONE of the following: A. The patient is requesting induction therapy and maintenance therapy and the requested quantity for maintenance therapy is NOT greater than the allowed quantity OR B. The patient is requesting maintenance therapy only and the requested quantity is NOT greater than the allowed quantity Continuation of Therapy: 1. The patient has been previously approved for the requested agent through the Prior Authorization process AND 2. The prescriber is a specialist in the area of the patient’s diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or has consulted with a specialist in the area of the patient’s diagnosis AND 3. The prescriber has provided a copy of the patient’s treatment logs for bleeds that includes ALL of the following: Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 127 Prior Authorization Criteria April 2020

A. Date of bleed AND B. The treatment used (include the brand name and number of units administered) AND C. The number of doses required to treat the bleed AND 4. ONE of the following: A. The patient has shown clinical benefit since starting the requested agent (i.e., less breakthrough bleeds as documented in the treatment log) OR B. The prescriber has submitted documentation supporting the continued use of the requested agent AND 5. If the patient is receiving Feiba [activated prothrombin complex concentrate (aPCC)] for breakthrough bleeds, the patient will be monitored for thrombotic microangiopathy and thromboembolism AND 6. The patient does not have any FDA labeled contraindication(s) to therapy with the requested agent AND 7. The prescriber has provided the patient’s weight AND 8. The requested dose is within the FDA labeled dosing based on the patient’s weight References: N/A Last Review/Revision date: 5/2019

GALAFOLD (MIGALASTAT) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneout Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Renewal: Indefinite Diagnosis Considered For Coverage: Fabry Disease Prescribing Restriction: Quantity Limits: 14 caps/28 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. The patient has a diagnosis of Fabry disease AND BOTH of the following: i. The diagnosis was confirmed by mutation of alpha-galactosidase A (alpha-Gal A) gene AND ii. The patient has a confirmed amenable variant mutation (a complete list of amenable variants is available in the Galafold prescribing information or a specific variant can be verified as amenable at http://www.fabrygenevariantsearch.com) AND 2. The prescriber is a specialist (e.g. endocrinologist, geneticist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 3. The prescriber has assessed current levels of ALL of the following: kidney function Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 128 Prior Authorization Criteria April 2020

(proteinuria, GFR), cardiac function (left ventricular hypertrophy, conduction or rhythm, mitral or aortic insufficiency), optic neuropathy, neuropathic pain, and gastrointestinal symptoms AND 4. ONE of the following: A. The patient is NOT currently being treated with enzyme replacement therapy (ERT) (e.g., Fabrazyme) OR B. The patient is currently being treated with ERT and will discontinue prior to starting the requested agent AND 5. ONE of the following: A. The requested quantity (dose) does not exceed the program quantity limit OR B. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) does not exceed the maximum FDA labeled dose for the requested indication AND iii. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit

Continuation of Therapy: 1. The patient has been previously approved for the requested agent through the Prior Authorization Review process AND 2. The patient has had improvements and/or stabilization of at least ONE of the following while being treated with the requested agent: A. Proteinuria OR B. GFR OR C. Left ventricular hypertrophy OR D. Cardiac conduction or rhythm OR E. Mitral or aortic insufficiency OR F. Optic neuropathy OR G. Neuropathic pain OR H. Gastrointestinal symptoms AND 3. The prescriber is a specialist (e.g. endocrinologist, geneticist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 4. The patient is NOT currently being treated with enzyme replacement therapy (ERT) (e.g., Fabrazyme) AND 5. ONE of the following: A. The requested quantity (dose) does not exceed the program quantity limit OR B. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) does not exceed the maximum FDA labeled dose for the requested indication AND Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 129 Prior Authorization Criteria April 2020 6. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit References: N/A Last Review/Revision date: 5/2019

UREA CYCLE DISORDERS Standard/Specific Therapeutic Class: Antihyperlipidemics Fomulary Status: Formulary, PA required SODIUM PHENYLBUTYRATE (BUPHENYL) RAVICTI (GLYCEROL PHENYLBUTYRATE) Coverage Duration: Indefinite Diagnosis Considered For Coverage: See below Prescribing Restriction: Quantity Limits: • Amount sufficient to meet current dose • Maximum 30 day supply Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. The patient has a diagnosis of hyperammonemia AND ALL of the following: a. The patient has elevated ammonia levels according to the patient’s age [Neonate: plasma ammonia level 150 µmol/L (>260 µg/dL) or higher; Older child or adult: plasma ammonia level >100 µmol/L (>175 µg/dL)] AND b. The patient has a normal anion gap AND c. The patient has a normal blood glucose level AND 2. The patient has a diagnosis of ONE of the following urea cycle disorders confirmed by enzymatic OR genetic testing: a. carbamylphosphate synthetase I deficiency [CPSID] b. ornithine transcarbamylase deficiency [OTCD] c. argininosuccinic acid synthetase deficiency [ASSD] d. argininosuccinic acid lyase deficiency [ASLD] e. arginase deficiency [ARGD] AND 3. The patient does not have acute hyperammonemia AND 4. The patient is unable to maintain a plasma ammonia level within the normal range with the use of a protein restricted diet and, if clinically appropriate, essential amino acid supplementation AND 5. The patient will be using the requested agent as adjunctive therapy to dietary protein restriction AND 6. If the requested agent is Ravicti, one of the following:

a. The patient has tried and had an inadequate response to generic sodium phenylbutyrate OR b. The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to generic sodium phenylbutyrate AND 7. The prescriber is a specialist in metabolic diseases or a geneticist or the prescriber has consulted with a specialist in metabolic diseases or a geneticist AND 8. The requested dose is within FDA approved labeling for the requested indication References: N/A Last Review/Revision date: 5/2019

EPIDIOLEX (CANNABIDIOL) Standard/Specific Therapeutic Class: Neuromuscular Agents, Anticonvulsants Fomulary Status: Formulary, PA required

Coverage Duration: Indefinite Diagnosis Considered For Coverage: See below Prescribing Restriction: Quantity Limits: • Amount sufficient to meet current dose • Maximum 30 day supply Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. The patient has a diagnosis of ONE of the following: I. Seizure associated with Lennox-Gastaut syndrome and the requested agent will not be used as monotherapy for seizure management OR II. Seizure associated with Dravet syndrome and the requested agent will not be used as monotherapy for seizure management AND 2. The patient is 2 years of age or greater AND 3. The patient’s medication history includes the use of an anticonvulsant in the past 90 days AND 4. The prescriber is a specialist in the area of the patient’s diagnosis (e.g. neurologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 5. The prescriber has provided the patient’s weight AND 6. The requested quantity (dose) does not exceed the maximum FDA labeled dose (for the requested indication) References: N/A Last Review/Revision date: 5/2019

BENLYSTA (BELIMUMAB) Standard/Specific Therapeutic Class: Neuromuscular Agents, Anticonvulsants Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered For Coverage: Systemic Lupus Erythematosis (SLE) Prescribing Restriction: Quantity Limits: • 4 autoinjectors/28 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. ONE of the following: A. There is documentation that the patient is currently being treated with the requested agent within the past 90 days OR B. The prescriber states that the patient is currently being treated with the requested agent within the past 90 days AND is at risk if therapy is changed OR C. The patient has a diagnosis of active systemic lupus erythematosus (SLE) disease AND ALL of the following: i. The patient has a history of positive antinuclear antibody (ANA) and/or positive anti-dsDNA results AND ii. The patient has a history of 3 other SLE diagnostic criteria (i.e., malar rash, discoid rash, photosensitivity, oral ulcers, nonerosive arthritis, serositis (e.g., pleuritis/pericarditis), renal disorder (e.g., persistent proteinuria >0.5 grams/day or cellular casts), hematologic disorder (e.g., hemolytic anemia with reticulocytosis, leukopenia, lymphopenia, or thrombocytopenia), and/or immunologic disorder (e.g., positive finding of antiphospholipid antibodies or anti-Sm antibodies) AND iii. ONE of the following: A. BOTH of the following: a. The patient has tried and had an inadequate response to TWO of the following classes: corticosteroids, antimalarials (e.g., hydroxychloroquine, chloroquine), nonsteroidal anti- inflammatory drugs (NSAIDS), aspirin, and/or immunosuppressives (e.g., azathioprine, methotrexate, cyclosporine, oral

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 132 Prior Authorization Criteria April 2020 cyclophosphamide, or mycophenolate AND b. The patient is currently being treated with at least ONE of the following classes: corticosteroids, antimalarials (e.g., hydroxychloroquine, chloroquine), nonsteroidal anti-inflammatory drugs (NSAIDS), aspirin, and/or immunosuppressives (e.g., azathioprine, methotrexate, cyclosporine, oral cyclophosphamide, or mycophenolate) within the past 90 days OR B. The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to ALL of the following classes: corticosteroids, antimalarials (e.g., hydroxychloroquine, chloroquine), nonsteroidal anti- inflammatory drugs (NSAIDS), aspirin, and/or immunosuppressives (e.g., azathioprine, methotrexate, cyclosporine, oral cyclophosphamide, or mycophenolate) AND 2. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., rheumatologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 3. The patient does NOT have severe active lupus nephritis AND 4. The patient does NOT have severe active central nervous system lupus AND 5. ONE of the following: a. The patient is NOT currently being treated with another biologic agent OR intravenous cyclophosphamide within the past 30 days OR b. The patient is currently being treated with another biologic agent or intravenous cyclophosphamide within the past 30 days AND will discontinue prior to starting the requested agent AND 6. The requested quantity (dose) does NOT exceed the program quantity limit OR 7. The requested quantity (dose) is greater than the program quantity limit AND a. The requested quantity (dose) does NOT exceed the maximum FDA labeled dose for the requested indication AND b. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit References: N/A Last Review/Revision date: 5/2019

APOKYN (APOMORPHINE) Standard/Specific Therapeutic Class: Antiparkinson Therapy and Related Agents Fomulary Status: Formulary, PA required

Coverage Duration: Indefinite Diagnosis Considered For Coverage: Parkinson’s Disease Prescribing Restriction: Quantity Limits: • 5 cartidges/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. The requested agent will be used to treat acute, intermittent hypomobility, “off” episodes (muscle stiffness, slow movements, or difficulty starting movement) associated with advanced Parkinson’s disease AND 2. There is evidence of a claim that the patient is receiving concurrent therapy for Parkinson’s disease (e.g., levodopa, dopamine agonist, or monoamine oxidase B inhibitor) within the past 30 days AND 3. Prescriber is a neurologist or the prescriber has consulted with a neurologist AND 4. Patient is not receiving a 5-HT3 antagonist (e.g., ondansetron, granisetron, dolasetron, palonosetron, alosetron) concomitantly with the requested agent References: N/A Last Review/Revision date: 5/2019

ARIKAYCE (AMIKACIN) Standard/Specific Therapeutic Class: Aminoglycosides Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuintg: 12 months Diagnosis Considered For Coverage: Treatment Of Mycobacterium Avium Complex (MAC) Prescribing Restriction: Quantity Limits: • 30 vial/ 30day Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of MAC; AND 2. Prescribed by or in consultation with an infectious disease specialist or pulmonologist; AND 3. Age ≥ 18 years; AND 4. Failure, as evidenced by positive sputum culture, of at least a 6-month trial of a multidrug background regimen therapy at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced; AND 5. Dose does not exceed one vial per day. Continuation of Therapy: 1. Currently receiving medication as member has previously met initial approval criteria; AND 2. Documentation of at least 3 consecutive negative monthly sputum cultures in the first 6 months of therapy or at least 2 consecutive negative monthly sputum cultures in the last 2 months of therapy; AND 3. If request is for a dose increase, new dose does not exceed one vial per day. References: N/A Last Review/Revision date: 5/2019

CARBAGLU (CARGLUMIC ACID) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered For Coverage: N-acteylglutamate synthase (NAGS) deficiency Prescribing Restriction: Quantity Limits: Quantity sufficient for only 30-day supply Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: Urea Cycle Disorder: NAGS 1. Diagnosis of a urea cycle disorder (UCD) caused by NAGS deficiency; AND 2. NAGS deficiency is confirmed by enzymatic, biochemical or genetic analysis; AND 3. Prescribed by or in consultation with a physician experienced in treating metabolic disorders. References: N/A Last Review/Revision date: 5/2019

CHENODAL (CHENODIOL) Standard/Specific Therapeutic Class: Gastrointestinal Agents-Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Initial: 12 months Continuing: 12 months No more than a total of 24 months Diagnosis Considered For Coverage: Gallstones Prescribing Restriction: • Quantity Limits: Quantity sufficient for only 30-day supply Clinical Information required for Review: • Diagnosis • Medical Records • Patient Weight Coverage Criteria: Initiation of Therapy: Radiolucent Gallstones 1. Presence of radiolucent stones in well-opacifying gallbladders; AND 2. Age ≥ 18 years; AND 3. Failure of a 6-month trial of ursodiol, unless contraindicated or clinically significant adverse effects are experienced; AND 4. Member is not a candidate for surgery (e.g., due to systemic disease or age); AND 5. Dose does not exceed 18 mg/kg/day. Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy; AND 3. Total treatment duration does not exceed 24 months; AND 4. If request is for a dose increase, new dose does not exceed 18 mg/kg/day References: N/A Last Review/Revision date: 5/2019

CRYSVITA (BUROSUMAB-TWZA) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered For Coverage: X-linked hypophosphatemia (XLH) Prescribing Restriction: • Quantity Limits: 30 day supply • Pediatrics: 90mg q 2 weeks • Adults: 90mg q 4 weeks Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of XLH confirmed by one of the following: a) DNA testing confirms the presence of mutations in the PHEX gene; b) Elevated serum fibroblast growth factor 23 (FGF23) levels; AND 2. Prescribed by or in consultation with an endocrinologist or metabolic disease specialist; AND 3. Age ≥ 1 year; AND 4. Current (within the last 30 days) serum phosphorus level is below the reference range for age and gender (use laboratory-specific reference ranges if available); AND 5. Presence of clinical signs and symptoms of the disease (e.g., rickets, growth impairment, musculoskeletal pain, bone fractures); AND 6. Failure of calcitriol (Rocaltrol®) with an oral phosphate agent (K-Phos®, K-Phos Neutra®), unless contraindicated or adverse effects are experienced; AND 7. Dose does not exceed 90 mg every two weeks (pediatrics) or 90 mg every four weeks (adults). Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; and 2. Member is responding positively to therapy as evidenced by both of the following: a) An increase in serum phosphorus levels from baseline and/or maintenance within the normal range for age and gender, not to exceed the upper limit of that normal range (use laboratory-specific reference ranges if available); b) A positive clinical response including any of the following: enhanced height velocity, improvement in skeletal deformities, reduction of

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 138 Prior Authorization Criteria April 2020 fractures, reduction of generalized bone pain; AND 3. If request is for a dose increase, new dose does not exceed 90 mg every two weeks (pediatrics) or 90 mg every four weeks (adults). If request is for a dose increase, new dose does not exceed 18 mg/kg/day References: N/A Last Review/Revision date: 7/2019

CYSTADANE (BETAINE) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered For Coverage: Homocystinuria Prescribing Restriction: • Quantity Limits: 30 day supply • <= 20gm/day Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of homocystinuria associated with one of the following: a. Cystathionine beta-synthase (CBS) deficiency; OR b. 5,10-methylenetetrahydrofolate reductase (MTHFR) deficiency; OR c. Cobalamin cofactor metabolism (cbl) defect; AND 2. Prescribed by or in consultation with metabolic or genetic disease specialist; AND 3. Dose does not exceed 20 g per day. References: N/A Last Review/Revision date: 5/2019

CYSTARAN (CYSTEAMINE) (Cysteamine ophthalmic) (Cystaran) Standard/Specific Therapeutic Class: Ophthalmic Agents Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered For Coverage: Corneal cystine crystal accumulation in patients with cystinosis. Prescribing Restriction: • Quantity Limits: 4 bottles/28 days Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 139 Prior Authorization Criteria April 2020 Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of cystinosis; AND 2. Prescribed by or in consultation with an ophthalmologist; AND 3. Presence of corneal cystine accumulation; AND 4. Dose does not exceed 1 drop in each eye every hour while awake (1 bottle/week). References: N/A Last Review/Revision date: 5/2019

RUZURGI (AMIFAMPRIDINE)/FIRDAPSE (AMIFAMPRIDINE) Standard/Specific Therapeutic Class: Antimyasthenic/Cholinergic Agents Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered For Coverage: Lambert-Eaton myasthenic syndrome (LEMS) Prescribing Restriction: • Quantity Limits 10mg tabs: 240/30days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of LEMS; AND 2. Prescribed by or in consultation with a neurologist; AND 3. Age ≥ 18 years; AND 4. Documentation of a baseline clinical muscle strength assessment (examples may include but are not limited to the Quantitative Myasthenia Gravis (QMG) score, triple-timed up-and-go test (3TUG), Timed 25-foot Walk test (T25FW)); AND 5. Ruzurgi is preferred. Firdapse should only be approved if patient has tried Ruzurgi and has a demonstrated intolerance to one of its inert ingredients 6. Dose does not exceed 80 mg per day. Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy as evidenced by clinical muscle strength assessments (examples may include but are not limited to the QMG score, 3TUG test, T25FW test); AND 3. If request is for a dose increase, new dose does not exceed 80 mg per day References: N/A Last Review/Revision date: 7/2019 Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 140 Prior Authorization Criteria April 2020

INCRELEX (MECASERMIN) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Up to age 18 Diagnosis Considered For Coverage: Treatment of growth failure in children with severe primary IGF-1 deficiency or with growth hormone (GH) gene deletion who have developed neutralizing antibodies to GH. Prescribing Restriction: • Quantity Limits: 30 day supply based on dose. Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: Severe Primary IGF-1 Deficiency: 1. Diagnosis of severe primary IGF-1 deficiency (IGFD) (i.e., inherited growth hormone insensitivity) and associated growth failure as evidenced by all of the following: 1. Basal IGF-1 is ≥ 3 standard deviations (SD) below the mean; AND 2. Normal or elevated GH level; AND 3. Height is ≥ 3 SD below the mean; AND 2. Prescribed by or in consultation with an endocrinologist; AND 3. Age ≥ 2 and < 18 years; AND 4. Documentation of baseline height at the time of request; AND 5. Somatropin (recombinant human GH) is not prescribed concurrently with Increlex; AND 6. Dose does not exceed 0.12 mg/kg twice daily. Growth Hormone Insensitivity: 2. Diagnosis of acquired GH insensitivity as evidenced by both of the following: 1. Documentation of genetic GH deficiency due to a GH gene deletion; AND 2. Documentation of presence of neutralizing GH antibodies; AND 3. Age ≥ 2 and < 18 years; AND 4. Prescribed by or in consultation with an endocrinologist; AND 5. Documentation of growth failure as indicated by any of the following: 1. Height > 3 SD below the mean; OR 2. Height > 2 SD below the mean and one of the following: a. Height velocity > 1 SD below the mean over 1 year; OR b. Decrease in height SD > 0.5 over 1 year in children > 2 years of age; OR

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 141 Prior Authorization Criteria April 2020 3. Height > 1.5 SD below midparental height; OR 4. Height velocity > 2 SD below the mean over 1 year; OR 5. Height velocity > 1.5 SD below the mean over 2 years; AND 6. Documentation of baseline height at the time of request; AND 7. Somatropin (recombinant human GH) is not prescribed concurrently with Increlex; AND 8. Dose does not exceed 0.12 mg/kg twice daily. Diagnoses for which Increlex will NOT be approved: Severe primary IGF-1 deficiency or growth hormone insensitivity in patients with any 1 of the following: 1. Closed epiphyses; 2. Active or suspected neoplasm; 3. Hypothyroidism; 4. Malnutrition; 5. Chronic treatment with pharmacologic doses of anti-inflammatory steroids. References: N/A Last Review/Revision date: 5/2019

KUVAN (SAPROPTERIN) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered For Coverage: Phenylketonuria (PKU) with hyperphenylalanimeia Prescribing Restriction: • Quantity Limits: 30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of hyperphenylalanimeia due to PKU; AND 2. Prescribed by or in consultation with a metabolic or genetic disease specialist; AND 3. Recent (within 90 days) Phe blood level is > 360 µmols/L; AND 4. Not to be used in conjunction with Palynziq: AND 5. Dose does not exceed 20 mg/kg per day Continuation of Therapy: 1. Currently receiving medication or member has previously met all initial approval criteria; 2. Member is responding positively to therapy as demonstrated by a reduction in Phe (phenylalanine) blood levels since initiation of therapy; 3. If request is for a dose increase, new dose does not exceed 20 mg/kg per day References: N/A Last Review/Revision date: 7/2019

LOKELMA (SODIUM ZIRCONIUM CYCLOSILICATE) Standard/Specific Therapeutic Class: Miscellaneous Therapeutic Class Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered For Coverage: Hyperkalemia Prescribing Restriction: • Quantity Limits: 30 day supply; 15gm/day • Initial 48 hours may have up to 30gm/day Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: Severe Primary IGF-1 Deficiency: 1. Diagnosis of hyperkalemia; AND 2. Age ≥ 18 years; AND 3. Failure of sodium polystyrene sulfonate at up to maximally tolerated doses, unless contraindicated or clinically significant adverse effects are experienced; AND 4. Dose does not exceed the following: a) Initial dose: 30 g per day for up to 48 hours; b) Maintenance dose: 15 g per day. Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; 2. Member is responding positively to therapy; 3. If request is for a dose increase, new dose does not exceed 15 g per day References: N/A Last Review/Revision date: 5/2019

LUCEMYRA (LOFEXIDINE) Standard/Specific Therapeutic Class: Psychotherapeutic and Neurological Agents - Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Initial: 7 days Continuing: 7 days No more than 14 days total treatment Diagnosis Considered For Coverage: Mitigation of opioid withdrawal symptoms to facilitate abrupt opioid discontinuation. Prescribing Restriction: • Quantity Limits: 16 tablets/day Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of opioid dependence (may be limited to physiologic dependence/tolerance) or opioid use disorder; AND 2. Prescribed by or in consultation with a physician specializing in one of the following areas: emergency medicine/inpatient care, pain management, addiction psychiatry; AND 3. Age ≥ 18 years; AND 4. Member is currently or will be undergoing abrupt opioid discontinuation within the next seven days and one of the following: a) Has taken one or more opioids for at least the last three weeks; OR b) Has been or will be administered an opioid antagonist (e.g., naltrexone) after a period of opioid use; AND 5. Medical justification supports why an opioid taper (e.g., with buprenorphine, methadone or other opioid) cannot be used; AND 6. One of the following: a) Failure of clonidine unless contraindicated or clinically significant adverse effects are experienced; OR b) Lucemyra has already been initiated (e.g., in an inpatient/ER setting); AND 7. Lucemyra has not been prescribed for a prior opioid withdrawal event within the last 30 days or medical justification supports retreatment; AND 8. Dose does not exceed 2.88 mg (16 tablets) daily Continuation of Therapy: 1. Currently receiving medication, or documentation supports that member is currently receiving Lucemyra for a covered indication and has received this medication for less than 14 days; 2. Member is responding positively to therapy; 3. If request is for a dose increase, new dose does not exceed 2.88 mg (16 tablets) daily References: N/A Last Review/Revision date: 5/2019

IL 5 ANTAGONISTS NUCALA (MEPOLIZUMAB) Standard/Specific Therapeutic Class: Antiasthmatic And Bronchodilator Agents Fomulary Status: Formulary, PA required Drugs included: Fasenra (Benralizumab), Nucala (Mepolizumab) Coverage Duration: Initial: 6 months Continuing: 12 months Diagnosis Considered For Coverage: Add on maintenance treatment for asthma Prescribing Restriction: • Quantity Limits: • Fasenra: 30mg q4 weeks x doses then 30mg q8 weeks • Nucala: Asthma: 100mg q4 weeks, EPGA: 300mg q 4 weeks Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Severe Asthma (Fasenra and Nucala): 1. Diagnosis of asthma; AND 2. Member has an absolute blood eosinophil count ≥150 cells/mcL within the past 3 months; AND 3. Prescribed by or in consultation with a pulmonologist, immunologist, or allergist; AND 4. Age ≥ 12 years; AND 5. Member has experienced ≥ 2 exacerbations with in the last 12 months, requiring any of the following despite adherent use of controller therapy (i.e., medium- to high-dose inhaled corticosteroid (ICS) plus either a long acting beta-2 agonist (LABA) or leukotriene modifier (LTRA) if LABA contraindication/intolerance): A. Oral/systemic corticosteroid treatment (or increase in dose if already on oral corticosteroid); B. Urgent care visit or hospital admission; C. Intubation; AND 6. Nucala is prescribed concomitantly with an ICS plus either a LABA or LTRA; AND 7. Dose does not exceed 100 mg every 4 weeks.

2. Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss; Nucala only): 1. Diagnosis of EGPA (Churg-Strauss); AND 2. Member has an absolute blood eosinophil count ≥150 cells/mcL within the last 3 months; AND 3. Prescribed by or in consultation with a pulmonologist, rheumatologist, immunologist, or nephrologist; AND 4. Age ≥ 18 years; AND Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 145 Prior Authorization Criteria April 2020 5. Failure of a 3-month trial of a glucocorticoid, unless contraindicated or clinically significant adverse events are experienced; AND 6. Dose does not exceed 300 mg every 4 weeks.

Continuation of Therapy: Severe Asthma 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Demonstrated adherence to asthma controller therapy that includes an ICS plus either an LABA or LTRA; AND 3. Member is responding positively to therapy (examples may include but are not limited to a reduction in exacerbations or corticosteroid dose, improvement in forced expiratory volume over one second since baseline; reduction in the use of rescue therapy); AND 4. If request is for a dose increase, new dose does not exceed 100 mg every 4 weeks. Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss; Nucala only): 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy; AND 3. If request is for a dose increase, new dose does not exceed 300 mg every 4 weeks References: N/A Last Review/Revision date: 7/2019

OXERVATE (CENEGERMIN-BKBJ) Standard/Specific Therapeutic Class: Ophthalmic Agents Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered For Coverage: Neurotrophic Keratitis Prescribing Restriction: • Quantity Limits: 30 vials/30days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of neurotrophic keratitis; AND 2. Prescribed by or in consultation with an ophthalmologist; AND 3. Age ≥ 2 years; AND 4. Dose does not exceed 1 vial per affected eye per day

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 146 Prior Authorization Criteria April 2020 Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy; AND 3. If request is for a dose increase, new dose does not exceed 1 vial per affected eye per day References: N/A Last Review/Revision date: 5/2019 PALYNZIQ (PEGVALIASE-PQPZ) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered For Coverage: Phenylketonuria (PKU) Prescribing Restriction: • Quantity Limits: • Initial: 20mg/day • Continuing: 40mg/day Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of PKU; AND 2. Prescribed by or in consultation with an endocrinologist, metabolic disease specialist, or genetic disease specialist; AND 3. Age ≥ 18 years; AND 4. Recent (within 90 days) phenylalanine (Phe) blood level is > 600 µmols/L; AND 5. Palynziq is not prescribed concurrently with Kuvan; AND 6. Dose does not exceed 20 mg per day Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member meets one of the following: • Blood Phe level has decreased by ≥ 20% from pre-treatment baseline; OR • Blood Phe level is ≤ 600 µmol/L; OR • Member has been using 20 mg per day for at least 6 months, but a dose titration to 40 mg per day is being requested after failure to meet therapeutic targets (a or b above) [only the 40 mg per day dose will be approved]; AND 3. If request is for a dose increase, new dose does not exceed 40 mg per day. References: N/A Last Review/Revision date: 5/2019 Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 147 Prior Authorization Criteria April 2020

RAYALDEE (CALDIFEDIOL) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Initial: 12 months Continuing: Indefinite Diagnosis Considered For Coverage: Secondary Hyperparathyroidism In Adult Patients With Stage 3 Or 4 Chronic Kidney Disease (CKD) Prescribing Restriction: • Quantity Limits: 60mcg/day (2 caps) Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of secondary hyperparathyroidism; AND 2. Age ≥ 18 years; AND 3. Member has stage 3 or 4 chronic kidney disease defined by eGFR of 15-59 mL/min; AND 4. Current (within the last 30 days) serum total 25-hydroxyvitamin D level is less than 30 ng/mL; AND 5. Failure of ergocalciferol or cholecalciferol, at up to maximally indicated doses, unless both are contraindicated, or clinically significant adverse effects are experienced; AND 6. Lab results over the previous 3-6 months show trending increase in iPTH level or current (within the last 30 days) labs show iPTH above the normal levels; AND 7. Dose does not exceed 60 mcg per day (2 capsules per day). Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy (suspend dosing if intact PTH is persistently abnormally low, serum calcium is consistently above the normal range or serum 25- hydroxyvitamin D is consistently above 100 ng/mL); AND 3. If request is for a dose increase, new dose does not 60 mcg per day (2 capsules per day).

References: N/A Last Review/Revision date: 5/2019

TEGSEDI (INOTERSEN) Standard/Specific Therapeutic Class: Psychotherapeutic and Neurological Agents - Miscellaneuous Fomulary Status: Formulary, PA required Coverage Duration: Initial: 12 months Continuing: Indefinite Diagnosis Considered For Coverage: Polyneuropathy Of Hereditary Transthyretin-Mediated Amyloidosis (hATTR) Prescribing Restriction: • Quantity Limits: 4 syringes/28 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of hATTR with polyneuropathy; AND 2. Documentation confirms presence of a transthyretin (TTR) mutation; AND 3. Biopsy is positive for amyloid deposits or medical justification is provided as to why treatment should be initiated despite a negative biopsy or no biopsy; AND 4. Prescribed by or in consultation with a neurologist; AND 5. Age ≥ 18 years; AND 6. Member has not had a liver transplant; AND 7. Dose does not exceed 284 mg (1 syringe) per week.

Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy – including but not limited to improvement in any of the following parameters: a. Neuropathy (motor function, sensation, reflexes, walking ability); b. Nutrition (body mass index); c. Cardiac parameters (Holter monitoring, echocardiography, electrocardiogram, plasma BNP or NT-proBNP, serum troponin); d. Renal parameters (creatinine clearance, urine albumin); e. Ophthalmic parameters (eye exam); AND 3. Member has not had a liver transpant; AND 4. If request is for a dose increase, new dose does not exceed 284 mg (1 syringe) per week.

References: N/A Last Review/Revision date: 5/2019

TAFAMIDIS (VYNDAMAX, VYNDAQEL) Standard/Specific Therapeutic Class: Transthyretin Stabilizer Fomulary Status: Formulary, PA required Coverage Duration: Initial: 12 months Continuing: Indefinite Diagnosis Considered For Coverage: Cardiomyopathy of wild type or hereditary transthyretin-mediated amyloidosis (ATTR-CM) Prescribing Restriction: • Quantity Limits: Vyndamax: 30 capsules/30days, Vyndaqel: 120 capsules/30days Clinical Information required for Review: • Diagnosis • Medical Records • Current therapy Coverage Criteria: Initiation of Therapy: 1. Diagnosis of cardiomyopathy of wild type or hereditary transthyretin-mediated amyloidosis (ATTR- CM); AND 2. Age ≥ 18 years; AND 3. Documentation of amyloid deposits on biopsy analysis from cardiac or non-cardiac sites; AND 4. Documentation of cardiac involvement confirmed by echocardiography or cardiac magnetic resonance imagining: AND 5. For members with hereditary ATTR-CM there is documentation of of a mutation of the TTR gene; AND 6. For members with wild type ATTR-CM there is documentation of transthyretin precursor proteins confirmed by immunohistochemical analysis, scintigraphy, or mass spectrometry; AND 7. Member has documented clinical symptoms of New York Heeart Association Class I, II, or III heart failure (ex-dyspnea, fatigue, perpherial edema); AND 8. Prescriber has attested member is not and will not be prescribed tetramer stabilizers; AND 9. Prescription is written by or in consultation with a cardiologist or a physician who specializes in the treatment of amyloidosis; AND 10. Member does not have a history of heart or liver transplant; AND 11. Dose does not exceed 61mg per day for Vyndamax OR 80mg per day for Vyndaqel

Continuation of Therapy: 1. Currently receiving therapy or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy including but not limited to improvement in any of the following parameters: f. Improved 6-minute walk test g. Improvement in Kansas City Cardiomyopathy Questionnaire-Overall Summary h. Decreased cardiovascular related hospitalizations i. Improvement in NYHA classification j. Improved ventricular stroke volume

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 150 Prior Authorization Criteria April 2020 k. Improved NT-proBNP level AND 5. Member has not had a liver transpant; AND 6. Dose does not exceed 61mg per day for Vyndamax OR 80mg per day for Vyndaqel

References: N/A Last Review/Revision date: 2/2020

VECAMYL (MECAMYLAMINE) Standard/Specific Therapeutic Class: Psychotherapeutic and Neurological Agents - Miscellaneuous Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered For Coverage: Moderately Severe To Severe Essential Hypertension Or Uncomplicated Malignant Hypertension Prescribing Restriction: • Quantity Limits: Sufficient tablets for 30 days supply by sig. No more than 300 tabs/month Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of hypertension; 2. Failure of a combination of 3 formulary antihypertensive agents from different classes, at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced; 3. Age ≥ 18 years References: N/A Last Review/Revision date: 7/2019

VELTASSA (PATIROMER) Standard/Specific Therapeutic Class: Miscellaneous Therapeutic Class Fomulary Status: Formulary, PA required Coverage Duration: Initial: 12 months Continuing: Indefinite Diagnosis Considered For Coverage: Hyperkalemia Prescribing Restriction: • Quantity Limits: 30 packets/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of hyperkalemia; AND 2. Age ≥ 18 years; AND 3. Failure of sodium polystyrene sulfonate powder at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced; AND 4. Dose not to exceed 25.2gm/day

Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Patient still has hyperkalemia diagnosis; AND 3. Member is responding positively to therapy; AND 4. If request is for a dose increase, new dose does not exceed 25.2 gm/day References: N/A Last Review/Revision date: 5/2019

VIBERZI (ELUXADOLINE) Standard/Specific Therapeutic Class: Gastrointestinal Agents - Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Initial: 12 months Continuing: Indefinite Diagnosis Considered For Coverage: Irritable Bowel Syndrome With Diarrhea (IBS- D) Prescribing Restriction: • Quantity Limits: 60 tablets/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of IBS-D; AND 2. Age ≥ 18 years; AND 3. Failure of an anti-diarrheal agent (e.g., loperamide, diphenoxylate w/atropine) at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced; AND 4. Failure of an antispasmodic (e.g., dicyclomine) at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced; AND 5. Dose does not exceed 200 mg per day (2 tablets per day). Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy; AND 3. If request is for a dose increase, new dose does not exceed 200 mg per day (2 tablets per day). References: N/A Last Review/Revision date: 5/2019

LOTRONEX (ALOSETRON) Standard/Specific Therapeutic Class: Selective 5-HT3 Receptor Antagonist Fomulary Status: Non-Formulary, PA required Coverage Duration: Initial: 12 months Continuing: Indefinite Diagnosis Considered For Coverage: Irritable Bowel Syndrome With Diarrhea (IBS- D) Prescribing Restriction: • Quantity Limits: 60 tablets/30 days Clinical Information required for Review: • Diagnosis • Medical Records • Previous therapy Coverage Criteria: Initiation of Therapy: 1. Diagnosis of IBS-D; AND 2. Age ≥ 18 years; AND 3. Patient is female; AND 4. Patient has had anatomic or biochemical abnormalities of the GI track excluded; AND 5. Documented Failure of an anti-diarrheal agent (e.g., loperamide, diphenoxylate w/atropine) at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced; AND 6. Failure of an antispasmodic (e.g., dicyclomine) at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced; AN 7. Patient and prescriber have met all requirement of the alosetron REMS program; AND 8. Dose does not exceed 1mg twice daily (2 tablets per day) Continuation of Therapy: 3. Currently receiving medication or member has previously met initial approval criteria; AND 4. Member is responding positivitely to therapy; AND 5. Patient and prescriber have both met atll REMS program requirements 6. Requested dose does not exceed 1mg twice daily (2 tablets per day) References: American Gastroenterological Association (AGA), “American Gastroenterological Association Institute Guideline on the Pharmacological Management of Irritable Bowel Syndrome,” 2014 Last Review/Revision date: 2/2020

XADAGO (SAFINAMIDE) Standard/Specific Therapeutic Class: Antiparkinson And Related Therapy Agents Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered For Coverage: Adjunctive Treatment To Levodopa/Carbidopa In Patients With Parkinson’s Disease (PD) Experiencing “Off” Episodes Prescribing Restriction: • Quantity Limits: 30 tablets/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of idiopathic Parkinson’s disease (PD); AND 2. Member is experiencing “off” time on levodopa/carbidopa therapy; AND 3. Failure of two drugs, as specified below, unless contraindicated or clinically significant adverse effects are experienced: a. Rasagiline (Azilect); AND b. One of the following drugs: entacapone (Comtan/Stalevo), ropinirole/ropinirole ER (Requip/Requip XL), pramipexole/pramipexole ER (Mirapex/Mirapex ER), Neupro (ritigotine); AND 4. Xadago is prescribed in combination with carbidopa/levodopa; AND 5. Dose does not exceed 100 mg once daily

References: N/A Last Review/Revision date: 7/2019

XERMELO (TELOSTRISTAT ETHYL) Standard/Specific Therapeutic Class: Gastrointestinal Agents - Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: 12 months Diagnosis Considered For Coverage: Carcinoid Syndrome Diarrhea In Combination With Somatostatin Analog (SSA) Therapy In Adults Inadequately Controlled By SSA Therapy Prescribing Restriction: • Quantity Limits: 90 tablets/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of carcinoid syndrome diarrhea; AND 2. Failure of a one-month trial of an SSA (e.g., octreotide, lanreotide) at up to maximally indicated doses unless contraindicated or clinically significant adverse effects are experienced; AND 3. Xermelo is prescribed in combination with an SSA unless contraindicated or clinically significant adverse effects are experienced; AND 4. Dose does not exceed 750 mg/day (3 tablets/day). Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy; AND 3. Member continues to have diarrhea; AND 4. Xermelo is prescribed in combination with an SSA unless contraindicated or clinically significant adverse effects are experienced; AND 5. If request is for a dose increase, new dose does not exceed 750 mg/day (3 tablets/day). References: N/A Last Review/Revision date: 5/2019

XOLAIR (OMALIZUMAB) Standard/Specific Therapeutic Class: Antiasthmatic And Bronchodilator Agents Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered For Coverage: • Moderate to severe persistent asthma in patients 6 years of age and older with a positive skin test or in vitro reactivity to a perennial aeroallergen and symptoms that are inadequately controlled with inhaled corticosteroids (ICS). • Chronic idiopathic urticaria (CIU) in adults and adolescents 12 years of age and older. Prescribing Restriction: • Quantity Limits: 30 day supply: o Asthma: 6 vials/month o CIU: 2 vials/month Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: A. Moderate to Severe Persistent Asthma: 1. Diagnosis of moderate to severe persistent asthma; AND 2. Prescribed by or in consultation with an allergist, immunologist, or pulmonologist; AND 3. Age ≥ 6 years; AND 4. Member has experienced ≥ 2 exacerbations, within the last 12 months, requiring any of the following despite adherent use of controller therapy for at least 6 months (i.e., medium- to high-dose inhaled corticosteroid (ICS) plus either a long acting beta-2 agonist (LABA) or leukotriene modifier (LTRA) if LABA contraindicated/intolerance): a. Oral/systemic corticosteroid treatment (or increase in dose if already on oral corticosteroid); AND/OR b. Urgent care visit or hospital admission; AND/OR c. Intubation; AND 5. Positive skin test or in vitro reactivity to a perennial aeroallergen; 6. Immunoglobulin E (IgE) level ≥ 30 IU/mL; AND 7. Xolair is prescribed concomitantly with an ICS plus either a LABA or LTRA; AND 8. Dose does not exceed 375 mg administered every 2 weeks.

B. Chronic Idiopathic Urticaria (CIU): 1. Diagnosis of CIU; AND 2. Prescribed by or in consultation with a dermatologist, immunologist, or allergist; AND 3. Age ≥ 12 years; AND 4. Failure of both of the following unless contraindicated or clinically significant adverse effects are experienced:

a. Two antihistamines (including one second generation antihistamine – e.g., cetirizine, levocetirizine, fexofenadine, loratadine, desloratadine) at maximum indicated doses, each used for ≥ 2 weeks; AND b. A LTRA in combination with an antihistamine at maximum indicated doses for ≥ 2 weeks; AND 5. Dose does not exceed 300 mg every 4 weeks Continuation of Therapy: A. Moderate to Severe Persistent Asthma): 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Demonstrated continued adherence to asthma controller therapy that includes an ICS plus either an LABA or LTRA; AND 3. Member is responding positively to therapy (examples may include but are not limited to a reduction in exacerbations or corticosteroid dose, improvement in forced expiratory volume over one second) since baseline; reduction in the use of rescue B. therapy); AND 4. If request is for a dose increase, new dose does not exceed 375 mg administered every 2 weeks for dosing based on pre-treatment IgE level, weight, and age). Chronic Idiopathic Urticaria: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy; AND 3. If request is for a dose increase, new dose does not exceed 300 mg every 4 weeks References: N/A Last Review/Revision date: 5/2019

NEUMEGA (OPRELVEKIN) Standard/Specific Therapeutic Class: Hematopoetic Agents Fomulary Status: Formulary, PA required Coverage Duration: 3 months Diagnosis Considered For Coverage: Prevention Of Thrombocytopenia in Adults Prescribing Restriction: • Quantity Limits: 30 vials/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Patient is 18 years old or older; AND 2. Patient is receiving chemotherapy to treat a non-myeloid malignancy; AND 3. Patient is at high risk for developing severe thrombocytopenia; AND 4. Patient does not have preexisting atrial arrhythmias or fluid retention.

5. Dose does not exceed 50mcg/kg/day. References: N/A Last Review/Revision date: 7/2019

CYSTEAMINE (CYSTAGON, PROCYSBI) Standard/Specific Therapeutic Class: Genitourinary Agents - Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Initial: 3 months Continuing: Indefinite Diagnosis Considered For Coverage: Management Of Nephropathic Cystinosis Prescribing Restriction: • Quantity Limits: 30 day supply Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of nephropathic cystinosis. The diagnosis must be confirmed by having all of the following : a. Elevated baseline white blood cell (WBC) cystine levels > 2 nmol/1/2cystine/mg protein; AND b. Laboratory result confirming CTNS gene mutation; AND c. Clinical symptoms consistent with nephropathic cystinosis including electrolyte imbalances (e.g. Fanconi Syndrome) and polyuria; AND 2. Must be prescribed by or in consultation with a physician who specializes in the treatment of inherited metabolic disorders AND 3. Must use Cystagon as preferred therapy and must provide the following chart documentation when requesting Procysbi: a. Chart documentation of an adequate trial of immediate-release cysteamine (Cystagon) with an inadequate response despite dose titration and compliance with therapy demonstrated by inadequately controlled WBC cystine levels OR b. The physician must provide relevant written documentation of laboratory and/or objective values [e.g., WBC cysteine levels, physician progress notes; or Subjective, Objective, Assessment, and Plan (SOAP note) information representing the physician’s interaction with the member] as well as clinical rationale explaining why Cystagon has not produced the same clinical results as would be expected with the use of Procysbi (They are the same chemical entity). Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND

2. Chart documentation that the member’s condition has improved based upon the prescriber’s assessment while on therapy and reduction in WBC cystine levels since starting treatment with cysteamine (Cystagon) or cysteamine delayed-release capsule (Procysbi) References: N/A Last Review/Revision date: 7/2019

ELMIRON (PENTOSAN POLYSULFATE SODIUM) Standard/Specific Therapeutic Class: Genitourinary Agents - Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: 12 months Diagnosis Considered For Coverage: Interstitial Cystitis Prescribing Restriction: • Quantity Limits: 90 capsules/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of bladder pain or discomfort associated with interstitial cystitis; AND 2. Prescribed by or in consultation with a Urologist; AND 3. Patient has not gotten relief from an adequate trial of NSAIDs and tricyclic antidepressants unless contraindicated or clinically significant adverse effects are experienced; AND 4. Dose does not exceed 300 mg/day (3 capsules/day). Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Improvement in symptoms (for example: pelvic/bladder pain, urinary frequency/urgency); AND 3. If request is for a dose increase, new dose does not exceed 300 mg/day (3 capsules/day). References: N/A Last Review/Revision date: 5/2019

ZELAPAR (SELEGILINE) Standard/Specific Therapeutic Class: Antiparkinson And Related Therapy Agents Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered For Coverage: Parkinson’s Disease Prescribing Restriction: • Quantity Limits: 60 tablets/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of Parkinson’s disease; AND 2. Selegiline concomitantly with carbidopa/levodopa; AND 3. One of the following: 1. The member is new to the plan and is already stabilized on the medication; OR 2. There is difficulty with the administration of, an intolerance to one of the preferred monoamine oxidase inhibitors agents, selegiline (oral), or rasagiline (oral) 4. Dose does not exceed 1.25 mg/day (1 tablet/day). Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Improvement in symptoms ; AND 3. If request is for a dose increase, new dose does not exceed 2.5 mg/day (2 tablets/day). References: N/A Last Review/Revision date: 5/2019

XURIDEN (URIDINE TRIACETATE) Standard/Specific Therapeutic Class: Endocrine And Metabolic Agents - Miscellaneous Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered For Coverage: Hereditary Orotic Aciduria Prescribing Restriction: • Quantity Limits: 120 packs/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Medication is prescribed by or in consultation with specialist in genetic metabolic diseases; AND 2. Patient is 2 months of age or older; AND 3. A confirmed diagnosis of hereditary orotic aciduria with all of the following features: • Megaloblastic anemia unresponsive to iron, folic acid, or vitamin B12 • Excessive urinary excretion of orotic acid; AND 4. All of the following baseline tests have been completed before initiation of treatment: • Complete blood count with differential • Urinalysis for orotic acid and orotidine levels References: N/A Last Review/Revision date: 5/2019

SIKLOS (HYDROXYUREA) Standard/Specific Therapeutic Class: Hematologic Agents; Hematopoietic Agents Fomulary Status: Formulary, PA required Coverage Duration: Indefinite until age 18 Diagnosis Considered For Coverage: Reduce The Frequency Of Painful Crises And To Reduce The Need For Blood Transfusions In Pediatric Patients, 2 Years Of Age And Older, With Sickle Cell Anemia Prescribing Restriction: • Quantity Limits: Sufficient qty for 30 day supply Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Medication is prescribed by or in consultation with a Hematologist; AND 2. Patient is between 2 and 18 years of age; AND 3. Documentation the Member requires a dispersible tablet due to a clinical Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 163 Prior Authorization Criteria April 2020

condition such as but not limited to: a. Dysphagia b. Member age; AND 4. Member is not over age 19 References: N/A Last Review/Revision date: 7/2019

THIOLA (TIOPRONIN) Standard/Specific Therapeutic Class: Hematologic Agents; Hematopoietic Agents Fomulary Status: Formulary, PA required Coverage Duration: Indefinite until age 18 Diagnosis Considered For Coverage: Prevention Of Cystine (Kidney) Stone Formation In Patients With Severe Homozygous Cystinuria Prescribing Restriction: • Quantity Limits: Sufficient qty for 30 day supply Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Prescriber is a Nephrologist or Urologist or is prescribed in consultation with a Nephrologist or Urologist 2. Patient is 9 years of age or older 3. A confirmed diagnosis of severe homozygous cystinuria with all of the following i. 24-hour urine collection with urinary cystine > 500 mg/day; AND ii. Individual is resistant to treatment with all of the following conservative measures 1. High fluid intake of at least 3 L/day; AND 2. Urinary alkalinization with potassium citrate to keep urine above pH 7; AND 3. Diet modification to restricted sodium and protein intake; AND Continuation of Therapy: 1. Individual continues to be seen by Nephrologist or Urologist or is prescribed in consultation with a Nephrologist or Urologist: AND 2. Individual’s condition has responded while on therapy a. Response is defined as either: i. Urinary cysteine concentration is < 250 mg/L; OR ii. Reduction in cysteine stone production; AND 3. Individual has been adherent with the medication

References: N/A Last Review/Revision date: 7/2019

SPRIX (KETOROLAC) Standard/Specific Therapeutic Class: Analgesics - Anti-Inflammatory Fomulary Status: Formulary, PA required Coverage Duration: 5 days Diagnosis Considered For Coverage: Short term (up to 5 days) management of moderate to severe acute pain that requires analgesia at the opioid level Prescribing Restriction: • Quantity Limits: 5 bottles, 1 bottle / day (8 actuations per bottle 15.75 mg/actuations) Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Inadequate treatment response or intolerance to oral ketorolac tablets OR 2. Inadequate treatment response, intolerance, or contraindication to 2 prescription strength oral generic NSAID's, one of which is celecoxib OR 3. Patient has dysphagia, esophagitis, mucositis, or uncontrollable nausea/vomiting References: N/A Last Review/Revision date: 5/2019

SUCRAID (SACROSIDASE) Standard/Specific Therapeutic Class: Digestive Aids Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered For Coverage: Sucrase-Isomaltase Deficiency (CSID) Prescribing Restriction: • Quantity Limits: 240ml/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Patient is age 5 months or older; AND 2. Drug is prescribed by, or in consultation with, a gastroenterologist; AND 3. Must have a diagnosis of congenital sucrose-isomaltase deficiency confirmed by: • Duodenal biopsy showing low sucrose activity and normal amounts of other disaccharides OR • Meeting all of the following criteria: • Stool pH <6 • Increase in breath hydrogen of >10ppm when challenged with sucrose after fasting • Negative lactose breath test 4. Patient is age 5 months or older; AND 5. Drug is prescribed by, or in consultation with, a gastroenterologist; AND 6. Must have a diagnosis of congenital sucrose-isomaltase deficiency confirmed by: • Duodenal biopsy showing low sucrose activity and normal amounts of other disaccharides OR • Meeting all of the following criteria: • Stool pH <6 • Increase in breath hydrogen of >10ppm when challenged with sucrose after fasting • Negative lactose breath test References: N/A Last Review/Revision date: 5/2019

INBRIJA (Levodopa) Standard/Specific Therapeutic Class: Antiparkinson Therapy and Related Agents Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered For Coverage: Parkinson’s Disease Prescribing Restriction: Quantity Limits: • 300 inhalations/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. The requested agent will be used to treat acute, intermittent hypomobility, “off” episodes (muscle stiffness, slow movements, or difficulty starting movement) associated with advanced Parkinson’s disease AND 2. There is evidence of a claim that the patient is receiving concurrent therapy for Parkinson’s disease (e.g., levodopa, dopamine agonist, or monoamine oxidase B inhibitor) within the past 30 days AND 3. Prescriber is a neurologist or the prescriber has consulted with a neurologist References: N/A Last Review/Revision date: 6/2019

JYNARQUE (TOLVAPTAN) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents Fomulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered For Coverage: Slow kidney function decline in adults at risk of rapidly progressing autosomal dominant polycystic kidney disease Prescribing Restriction: • Quantity Limits: • 15mg: 60/30 days • 30mg: 30/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. The patient has a diagnosis of autosomal dominant polycystic kidney disease (ADPKD) confirmed by ONE of the following: a. Ultrasonography OR b. MRI or CT scan OR c. Genetic testing AND 2. ONE of the following: a. The patients has had a sequential increase of >5% annually in TKV on imaging OR b. Total kidney volume (TKV) >750 mL OR c. Kidney length (KL) >16.5 cm OR d. The patient has typical (Class 1) ADPKD and ONE of the following: i. The patient has been classified as 1C, 1D, or 1E using the Mayo ADPKD Classification assessment OR ii. The prescriber has provided documentation indicating the patient’s ADPKD is rapidly progressing OR e. The patient has atypical (Class 2) ADPKD and the prescriber has provided documentation indicating the patient’s ADPKD is rapidly progressing AND 3. ONE of the following: a. The patient is initiating therapy and ONE of the following: i. The patient’s ALT, AST, bilirubin has been measured within the past 3 months prior to initiating therapy with the requested agent AND ONE of the following: 1. The patient’s most recent ALT, AST, and bilirubin levels are under the upper limit of normal (ULN) OR 2. The patient’s most recent ALT, AST, and bilirubin levels are above the ULN and the prescriber has provided documentation in support of use with the requested agent with the elevated levels OR Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 168 Prior Authorization Criteria April 2020

b. The patient is continuing therapy and BOTH of the following: i. The patient has had ALT, AST, and bilirubin assessed in the past 3 months AND ONE of the following: 1. The patient’s most recent levels have NOT exceeded EITHER of the following: a. ALT or AST 2 times ULN OR b. ALT or AST 2 times the patient’s baseline prior to initiating therapy with the requested agent OR 2. BOTH of the following: a. The most recent ALT or AST levels have exceeded 2 times ULN or 2 times the patient’s baseline prior to initiating therapy with the requested agent AND b. The prescriber has provided documentation in support of use with the requested agent while at these elevated levels AND ii. ONE of the following: 1. The patient has never experienced ALT or AST levels exceeding 3 times ULN while on therapy with the requested agent OR 2. BOTH of the following: a. The prescriber has provided documentation indicating that the ALT or AST levels exceeding 3 times ULN while on therapy with the requested agent were due to cause unrelated to therapy with the requested agent 3. The patient’s current ALT and AST levels have stabilized and are now below 3 times ULN AND 4. The patient will continue to receive routine ALT, AST, and bilirubin monitoring at least every 3 months AND 5. ONE of the following: a. The patient is not currently on therapy with another tolvaptan agent OR b. The other tolvaptan agent will be discontinued prior to starting therapy with the requested agent AND 6. The prescriber is a specialist (e.g. nephrologist) or has consulted with a specialist in the area of the patient’s diagnosis AND 7. The patient does NOT have any FDA labeled contraindications to the requested agent AND 8. ONE of the following: a. The requested quantity (dose) is NOT greater than the program quantity limit OR b. ALL of the following: i. The requested quantity is greater than the program quantity limit AND ii. The requested quantity (dose) is less than or equal to the maximum iii. FDA labeled dose AND iv. The requested quantity (dose) cannot be achieved with a lower v. quantity of a higher strength that does not exceed the limit Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 169 Prior Authorization Criteria April 2020

Continuation of Therapy 1. The patient has been previously approved for the requested agent through Prescryptive AND 2. The prescriber has indicated that the patient has received benefit from the requested agent AND 3. The patient has had ALT, AST, and bilirubin assessed in the past 3 months AND ONE of the following: a. The patient’s most recent levels have NOT exceeded EITHER of the following: i. ALT or AST 2 times ULN OR ii. ALT or AST 2 times the patient’s baseline prior to initiating therapy with the requested agent OR b. BOTH of the following: i. The most recent ALT or AST levels have exceeded 2 times ULN or 2 times the patient’s baseline prior to initiating therapy with the requested agent AND ii. The prescriber has provided documentation in support of use with the requested agent while at these elevated levels AND 4. ONE of the following: a. The patient has never experienced ALT or AST levels exceeding 3 times ULN while on therapy with the requested agent OR b. BOTH of the following: i. The prescriber has provided documentation indicating that the ALT or AST levels exceeding 3 times ULN while on therapy with the requested agent were due to cause unrelated to therapy with the requested agent AND ii. The patient’s current ALT and AST levels have stabilized and are now below 3 times ULN AND 5. The patient will continue to receive routine ALT, AST, and bilirubin monitoring at least every 3 months AND 6. ONE of the following: a. The patient is not currently on therapy with another tolvaptan agent OR b. The other tolvaptan agent will be discontinued prior to starting therapy with the requested agent AND 7. The patient does NOT have any FDA labeled contraindications to the requested agent AND 8. ONE of the following: a. The requested quantity (dose) is NOT greater than the program quantity limit OR b. ALL of the following: i. The requested quantity is greater than the program quantity limit AND ii. The requested quantity (dose) is less than or equal to the maximum FDA labeled dose AND iii. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit References: N/A Last Review/Revision date: 7/2019 Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 170 Prior Authorization Criteria April 2020

KEVEYIS (Dichlorphenamide) Standard/Specific Therapeutic Class: Diuretics Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered For Coverage: Primary Periodic Paralysis Prescribing Restriction: • Quantity Limits: 120 tablets/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Patient is age 18 years or older; AND 2. Patient must have one of the following a) Primary hyperkalemic periodic paralysis and related variants OR b) Primary hypokalemic periodic paralysis and related variants AND 3. Baseline and periodic monitoring of serum potassium and bicarbonate levels AND 4. Diagnosis confirmed by ONE of the following: a. Genetic testing OR b. Provocative testing OR c. Electromyography OR d. Muscle biopsy AND 5. Documentation that lifestyle modifications, dietary restrictions and exercise restrictions have been maximally challenged 6. Inadequate treatment response, intolerance, or contraindication to acetazolamide AND 7. Patient has no signs of hepatic impairment AND 8. Patient does not have severe pulmonary disease AND 9. Patient is not using of high-dose aspirin References: N/A Last Review/Revision date: 7/2019

MOZOBIL (Plerixafor) Standard/Specific Therapeutic Class: Hematopoetic Agents Fomulary Status: Formulary, PA required Coverage Duration: 4 days Diagnosis Considered For Coverage: Mobilize Hematopoetic Stem Cells for Autologous Transplantation in non-Hodgkins Lymphoma or multiple myeloma Prescribing Restriction: • Quantity Limits: 8 vials/4 day treatment Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Patient is at least 18 years old; AND 2. Prescriber is a bone marrow transplant specialist, hematologist or oncologist; AND 3. Patient had been diagnosed with non-Hodgkin’s lymphoma (NHL) or multiple myeloma; AND 4. Patient is scheduled for autologous transplantation AND 5. Must be used in combination with one of the following: filgrastim or its biosimilars References: N/A Last Review/Revision date: 7/2019

NUPLAZID (Pimavanserin) Standard/Specific Therapeutic Class: Antipsyctics/Antimanic Agents Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered For Coverage: Hallucinations and/or delusions associated with Parkinson’s disease psychosis Prescribing Restriction: • Quantity Limits: 30/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Submission of medical records documenting the presence of hallucinations or delusions (which may include illusions or a false sense of presence) on a recurrent or continuous basis for at least 1 month AND 2. Submission of medical records documenting the prescribing physician has attempted to adjust Parkinson’s disease medications in order to reduce psychosis without worsening motor symptoms prior to requesting Nuplazid AND 3. Used in combination with another Parkinson’s disease medication

References: N/A Last Review/Revision date: 7/2019

PANRETIN (Alitretinoin) Standard/Specific Therapeutic Class: Dermatological Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered For Coverage: Kaposi’s Sarcoma in HIV infected patients Prescribing Restriction: • Quantity Limits: 60gm/30 days Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Must be prescribed by or in consultation with a dermatologist or an HIV specialist AND 2. Must be age 18 years or older AND 3. Must have a diagnosis of cutaneous lesions in patients with AIDS-related Kaposi’s sarcoma AND 4. Must be on an antiretroviral regimen

References: N/A Last Review/Revision date: 7/2019

SAMSCA (Tolvaptan) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents Fomulary Status: Formulary, PA required Coverage Duration: Initial: 30 days Continuing: 30 days Diagnosis Considered For Coverage: Euvolemic or hypervolemic hyponatremia Prescribing Restriction: • Quantity Limits: 120 tablets/30 days Clinical Information required for Review: • Diagnosis • Medical Records

Coverage Criteria: Initiation of Therapy: 1. Medication HAS or WILL BE initiated in the hospital where serum sodium can be monitored closely AND 2. Is not used for the treatment of autosomal dominant polycystic kidney disease (ADPKD) AND 3. Is not used for hypovolemic hyponatremia AND 4. Patient does not have significant liver disease (including cirrhosis) AND 5. Patient is not anuric AND 6. May not be used in combination with Jynarque (tolvaptan)

Continuing Therapy: 1. There has been at least a 30 day lapse between previous treatment. AND 2. Medication HAS or WILL BE initiated in the hospital where serum sodium can be monitored closely AND 3. Is not used for the treatment of autosomal dominant polycystic kidney disease (ADPKD) AND 4. Is not used for hypovolemic hyponatremia AND 5. Patient does not have significant liver disease (including cirrhosis) AND 6. Patient is not anuric AND 7. May not be used in combination with Jynarque (tolvaptan)

References: N/A Last Review/Revision date: 7/2019

SANCUSO (Granisetron) Standard/Specific Therapeutic Class: Antiemetic Fomulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered For Coverage: Prevention of nausea and vomiting in patients receiving moderately and/or highly emetogenic chemotherapy regimens of up to 5 consecutive day duration Prescribing Restriction: • 1 patch/cycle Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Patient is receiving moderately to highly ematogentic chemotherapy in a cycle that is at least 2 consecutive days; AND 2. Patient has tried and failed oral ondansetron and granisetron

References: N/A Last Review/Revision date: 7/2019

XENLETA (Lefamulin) Standard/Specific Therapeutic Class: Antibiotic, Pleuromutilin Fomulary Status: Formulary, PA required Coverage Duration: 5 days Diagnosis Considered For Coverage: Community acquired bacterial pneumonia caused by Streptococcus pneumoniae, Staphylococcus aureus (methicillin-susceptible isolates), Haemophilus influenzae, Legionella pneumophila, Mycoplasma pneumoniae and Chlamydophila pneumoniae). Prescribing Restriction: • 10 tablets per 5 days Clinical Information required for Review: • Diagnosis • Medical Records • Culture and sensitivity results • Previous therapy • Concurrent therapy Coverage Criteria: Initiation of Therapy: Approve if: 1. Diagnosis of community acquired bacterial pneumonia; AND 2. Member is ≥ 18 years of age; AND 3. Prescribed by or in consultation with an infectious disease specialist; AND 4. Culture and Sensitivity (C&S) testing shows isolated pathogen that is susceptible to lefamulin [documentation required]: S. pneumoniae, S, aureus (methicillin-susceptible isolates), H. influenzae, L. pneumophila, M. pneumoniae and C. pneumoniae; AND 5. The C&S report shows resistance of the isolated pathogen to all formulary antibiotics approved for community acquired bacterial pneumonia

References: N/A Last Review/Revision date: 2/2020

VIRAZOLE (Ribavirin inh) Standard/Specific Therapeutic Class: Inhaled Antiviral Agent Fomulary Status: Formulary, PA required Coverage Duration: 1 year Diagnosis Considered For Coverage: Severe lower resipiratory tract infection due to respiratory syncytial virus in hospitalized infants or young children Prescribing Restriction: • 6 grams per day Clinical Information required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: Approve if: 1. Patient is an infant or young child; AND 2. Diagnosis of severe lower respiratory tract infection due to respiratory syncytial virus; AND 3. Treatment will occur during a hospitalization;

References: N/A Last Review/Revision date: 2/2020

PALFORZIA (Peanut Allergen Powder) Standard/Specific Therapeutic Class: Allergen-Specific Immunotherapy Fomulary Status: Formulary, PA required Coverage Duration: Initial: 1 year Diagnosis Considered For Coverage: Peanut Allergy Prescribing Restriction: • Initial dose escalation and up-dosing: Max of 6 capsules/14 days • Maintenance: #30 Packets per 30 days Clinical Information required for Review: • Diagnosis • Allergies • Medical Records Coverage Criteria: I. Initiation of Therapy: Approve if: 1. Member is between 4 and 17 years of age if in initiation phase OR 2. Member is at least 4 years of age if in maintenance phase 3. Member does not have Uncontrolled asthma AND 4. Member does not have a history of eosinophilic esophagitis or other eosinophilic gastrointestinal disease AND 5. Dose is appropriate for members stage in therapy

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: 1. Patient is stable and continuing the medication AND 2. Medication is used for appropriate indication and at an appropriate dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: 1. Patient is stable and continuing the medication AND 2. Medication is used for appropriate indication and at an appropriate dose

• References: Palforzia (peanut [Arachis hypogaea] Allergen Powder-dnfp) [prescribing information]. Brisbane, CA: Aimmune Therapeutics, Inc; January 2020.

Last Review/Revision date: 2/2020

BELIMUMAB ...... 132 A BENLYSTA ...... 132 Benralizumab ...... 145 abatacept ...... 98 BETAINE ...... 139 abiraterone ...... 11 Betaseron® ...... 82 acetaminophen with codeine ...... 14 Bethkis® ...... 93 acitretin ...... 29 bexarotene ...... 11 Actemra® ...... 98, 100 bosentan ...... 24 adalimumab ...... 98 Bosulif ...... 11 Adcirca ...... 24 Braftovi ...... 11 Adefovir ...... 76 Breeze and Contour ...... 41 Adempas® ...... 24 brodalumab ...... 98 Afinitor Disperz ...... 11 Bunavail™ ...... 96 Afinitor, ...... 11 BUPHENYL ...... 130 Aimovig™ ...... 105 Buprenorphine hydrochloride ...... 96 Ajovy™ ...... 105 Buprenorphine hydrochloride/naloxone ...... 96 Alecensa ...... 11 buprenorphine/naloxone ...... 96 Alferon N ...... 11 BUROSUMAB-TWZA ...... 138 alirocumab ...... 21 BUTORPHANOL ...... 19 Alitretinoin ...... 174 Alunbrig ...... 11 C ambrienstan ...... 24 AMIKACIN ...... 135 C1 esterase inhibitor ...... 67 anakinra ...... 98 C1 Esterase Inhibitor Subcutaneous ...... 67 Android ...... 34 Cabometyx ...... 11 APOKYN ...... 134 CALDIFEDIOL ...... 148 APOMORPHINE ...... 134 Calquence ...... 11 apremilast ...... 98 CANAKINUMAB ...... 120 Aranesp® ...... 65 CANNABIDIOL ...... 131 ARCALYST ...... 120 Capital with codeine® ...... 14 ARIKAYCE ...... 135 Caprelsa ...... 11 Arixtra® ...... 20 CARBAGLU ...... 136 ASFOTASE ALFA ...... 109 CARGLUMIC ACID ...... 136 Aubagio® ...... 82 Cayston® ...... 93 Auryxia® ...... 88 CENEGERMIN-BKBJ ...... 146 Austedo® ...... 85 CERDELGA® ...... 52 avatrombopag ...... 62 certolizumab ...... 98 Avonex® ...... 82 CHEMET® ...... 30 aztreonam ...... 93 CHENODAL ...... 137 CHENODIOL ...... 137 B Cholbam® ...... 57 CHOLIC ACID ...... 57 Balversa ...... 11 Cimzia® ...... 98, 99 Baraclude® ...... 76 CINACALCET ...... 38 baricitinib ...... 98 cladribine ...... 82 BEDAQUILINE ...... 80 codeine ...... 14

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 180 Prior Authorization Criteria April 2020 Codeine ...... 14 E codeine phosphate/ acetaminophen ...... 14 Cometriq ...... 11 ecallantide ...... 67 Copaxone® ...... 82 EGRIFTA® ...... 45 Copiktra ...... 11 elagolix ...... 107 CORTICOTROPIN ...... 36 elbasvir/grazoprevir ...... 77 Cosentyx® ...... 98, 100 ELIGLUSTAT TARTRATE ...... 52 Cotellic ...... 11 ELMIRON ...... 161 Cresemba ...... 74 eltrombopag ...... 62 CRYSVITA ...... 138 ELUXADOLINE ...... 153, 154 Cuprimine ...... 33 Emcyt ...... 11 CYSTADANE ...... 139 EMFLAZA® ...... 53 CYSTAGON, PROCYSBI ...... 160 Emgality™ ...... 105 Cystaran ...... 139 EMICIZUMAB-KXWH ...... 125 CYSTARAN ...... 139 Enbrel® ...... 98, 99 CYSTEAMINE ...... 139, 160 ENDARI™ ...... 89 Cysteamine ophthalmic ...... 139 enoxaparin ...... 20 entecavir ...... 76 D Entecavir ...... 76 Epclusa® ...... 77 daclatasvir ...... 77 EPIDIOLEX ...... 131 Daklinza® ...... 77 Epivir HBV® ...... 76 dalteparin ...... 20 epoetin alfa ...... 65 Danazol ...... 67 epoetin alfa-epbx ...... 65 DARAPRIM® ...... 72 Epogen® ...... 65 darbepoetin alfa ...... 65 epoprostenol ...... 24 Daurismo ...... 11 erenumab ...... 105 DEFERASIROX ...... 31 Erivedge ...... 11 DEFLAZACORT ...... 53 Erleada ...... 11 Demser ...... 11 erlotinib ...... 11 DENOSUMAB ...... 50, 51 Esbriet® ...... 92 Depen ...... 33 etanercept ...... 98 desirudin ...... 20 etoposide ...... 11 deutetrabenazine ...... 85 evolocumab ...... 21 dextromethorphan/quinidine ...... 87 EVZIO ...... 113 Dichlorphenamide ...... 171 Exalgo® ...... 17 Dilaudid® ...... 14 Extavia® ...... 82 dimethyl fumarate ...... 82 Doptelet® ...... 62 F dornase alfa ...... 93 D-Penamine ...... 33 Farydak ...... 11 D-Penicillamidne ...... 33 Fasenra ...... 145 DROXIDOPA ...... 114 fentanyl transdermal ...... 17 dupilumab ...... 27 ferric citrate ...... 88 Dupixent® ...... 27 FERRIPROX ...... 31 Duragesic® ...... 17 filgrastim ...... 63 filgrastim-aafi ...... 63 filgrastim-sndz ...... 63

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 181 Prior Authorization Criteria April 2020 fingolimod ...... 82 I Firazyr® ...... 67 FIRDAPSE (AMIFAMPRIDINE) ...... 140 Ibrance ...... 11 fondaparinux ...... 20 Iclusig ...... 11 FORTEO® ...... 49 Idhifa ...... 11 Fosrenol® ...... 88 ILARIS ...... 120 fostamatinib ...... 62 iloprost ...... 24 Fragmin® ...... 20 Ilumya ...... 98 FreeStyle Libre reader and sensor ...... 39 imatinib ...... 11 fremanezumab ...... 105 Imbruvica ...... 11 Fulphila® ...... 63 INBRIJA ...... 167 INCRELEX ...... 141 G INGENOL ...... 109 Ingrezza™ ...... 85 GALAFOLD ...... 128 Inlyta ...... 11 galcanezumab ...... 105 INOTERSEN ...... 149, 150 Galzin ...... 33 interferon Alfa 2B ...... 77 GATTEX ...... 111 interferon beta-1a ...... 82 Genotropin® ...... 42 interferon beta-1b ...... 82 Gilenya® ...... 82 Intron ...... 77 Gilotrif ...... 11 Intron A ...... 11 glatiramer acetate ...... 82 Iprivask ...... 20 Glatopa® ...... 82 Iressa ...... 11 glecaprevir/pibrentasvir ...... 77 Isavuconazonium Sulfate ...... 74 GLYCEROL PHENYLBUTYRATE ...... 130 ivacaftor ...... 93 golimumab ...... 98 ixekizumab ...... 98 Granisetron ...... 176, 177, 178, 179 Granix® ...... 63 J guselkumab ...... 98 JADENU ...... 31 H Jakafi ...... 11 JUXTAPID ...... 123 Haegarda ...... 67 JYNARQUE ...... 168 Harvoni® ...... 77 HEMLIBRA ...... 125 K Hepsera® ...... 76 histrelin ...... 107 Kadian® ...... 17 HP ACTHAR® ...... 36 Kalbitor® ...... 67 Humatrope® ...... 42 Kalydeco® ...... 93 Humira® ...... 98, 99 KETOROLAC ...... 165 Hycamtin ...... 11 KEVEYIS ...... 171 hydrocodone/acetaminophen ...... 14 Kevzara® ...... 98, 100 hydromorphone ...... 14, 17 Kineret® ...... 98, 100 HYDROXYPROGESTERONE CAPROATE ...... 91 Kisqali ...... 11 HYDROXYUREA ...... 163 KITABISTM PAK ...... 93 KORLYM ...... 119 KUVAN ...... 142 KYNAMRO ...... 123 Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 182 Prior Authorization Criteria April 2020 L MOZOBIL ...... 172 MS Contin® ...... 17 lamivudine ...... 76 MS-IR® ...... 14 Lamivudine HBV ...... 76 Mulpleta® ...... 62 lanadelumab-flyo ...... 67 MYALEPT ...... 117 lanthanum ...... 88 ledipasvir/sofosbuvir ...... 77 N Lenvima ...... 11 Letairis ...... 24 NALOXONE AUTO-INJECTOR ...... 113 Levodopa ...... 167 Natesto ...... 34 L-GLUTAMINE ...... 89 NATPARA ...... 116 LOFEXIDINE ...... 144 Nebupent® ...... 73 LOKELMA ...... 143 Nerlynx ...... 11 LOMITAPIDE ...... 123 Neulasta® ...... 63 Lonsurf ...... 11 NEUMEGA ...... 158 Lorbrena ...... 11 Neupogen® ...... 63 Lovenox® ...... 20 Nexavar ...... 11 LUCEMYRA ...... 144 Ninlaro ...... 11 lumacaftor/ivacaftor ...... 93 nintedanib ...... 92 lusutrombopag ...... 62 nitisinone ...... 70 Lynparza ...... 11 Nityr® ...... 70 Lysodren ...... 11 Nivestym ...... 63 Norditropin Flexpro® ...... 42 M Norditropin NordiFlex® ...... 42 Norditropin® ...... 42 macitentan ...... 24 NORTHERA ...... 114 MAKENA® ...... 91 Noxafil ...... 74 Matulane ...... 11 Nplate® ...... 62 Mavenclad® ...... 82 Nucala ...... 145 Mavyret™ ...... 77 Nucynta ER® ...... 17 Mayzent® ...... 82 NUEDEXTA® ...... 87 MECAMYLAMINE ...... 151 NUPLAZID ...... 173 MECASERMIN ...... 141 Nutropin AQ NuSpin®, ...... 42 Mekinist ...... 11 Nutropin AQ®, ...... 42 Mektovi ...... 11 Nutropin® ...... 42 Mepolizumab ...... 145 Mesnex ...... 11 O methadone ...... 17, 144 methoxy peg-epoetin beta ...... 65 OBETICHOLIC ACID ...... 60 methylnaltrexone ...... 56 Ocaliva® ...... 60 Methyltestosterone ...... 34 OCTREOTIDE ...... 47 METRELEPTIN ...... 117 Odomzo ...... 11 MIFEPRISTONE ...... 119 Ofev ...... 11, 92 MIGALASTAT ...... 128 Olumiant ...... 98, 100 MIGLUSTAT ...... 52 Olysio® ...... 77 MIPOMERSEN ...... 123 OMALIZUMAB ...... 157 Mircera® ...... 65 ombitasvir/paritaprevir/ritonavir ...... 77 morphine sulfate ...... 14, 17 ombitasvir/paritaprevir/ritonavir and dasabuvir ...... 77

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 183 Prior Authorization Criteria April 2020 Omnitrope® ...... 42 Primlev® ...... 14 OPRELVEKIN ...... 158 Procrit® ...... 65 Opsumit® ...... 24 PROLIA® ...... 50 ORAL and SELF INJECTABLE ONCOLYTICS ...... 11 Promacta® ...... 62 Orencia® ...... 98, 100 Pulmozyme® ...... 93 Orenitram® ...... 24 PYRIMETHAMINE ...... 72 Orfadin® ...... 70 Orilissa™ ...... 107 R Orkambi® ...... 93 Otezla® ...... 98, 100 RAVICTI ...... 130 Oxandrin® ...... 55 RAYALDEE ...... 148 oxandrolone ...... 55 RAYOS ...... 112 OXERVATE ...... 146 Rebif® ...... 82 oxycodone ...... 14, 17 Relistor® ...... 56 oxycodone/acetaminophen ...... 14 Remodulin® ...... 24 oxycodone/APAP ...... 14 Renagel® ...... 88 oxycodone/aspirin ...... 14 Repatha® ...... 21 Oxycontin® ...... 17, 18 Retacrit ...... 65 oxymorphone ...... 14, 17 Revatio ...... 24 Revatio® ...... 24 P Revlamid ...... 11 Ribapak® ...... 77 PALYNZIQ ...... 147 ribavirin ...... 77 PANRETIN ...... 174 RILONACEPT ...... 120 PARATHYROID HORMONE ...... 116 riociguat ...... 24 PATIROMER ...... 152 risankizumab-rzaa ...... 98 Pegasys Proclick®) ...... 77 romiplostim ...... 62 Pegasys® ...... 77 Roxicodone® ...... 14 pegfilgrastim ...... 63 Rubraca ...... 11 pegfilgrastim-cbqv ...... 63 RUZURGI (AMIFAMPRIDINE) ...... 140 peginterferon Alfa-2a ...... 77 Rydapt ...... 11 peginterferon Alfa-2B ...... 77 peginterferon beta-1a ...... 82 S PegIntron ...... 77 PEGVALIASE-PQPZ ...... 147 SACROSIDASE ...... 166 pentamidine isethionate ...... 73 SAFINAMIDE ...... 155 PENTOSAN POLYSULFATE SODIUM ...... 161 Saizen® ...... 42 Percocet® ...... 14 SAMSCA ...... 174 Percodan® ...... 14 SANCUSO ...... 176 PICATO ...... 109 SAPROPTERIN ...... 142 Pimavanserin ...... 173 sarilumab ...... 98 Piqray ...... 11 secukinumab ...... 98 pirfenidone ...... 92 SELEGILINE ...... 162 Plegridy® ...... 82 selexipag ...... 24 Pomalyst ...... 11 SENSIPAR® ...... 38 Posaconazole ...... 74 Serostim® ...... 42 Praluent® ...... 21 sevelamer chloride ...... 88 PREDNISONE DELAYED RELEASE TABLET ...... 112 sildenafil ...... 24

Version 202002.1 Copyright 2020 Prescryptive Health, Inc. 184 Prior Authorization Criteria April 2020 Siliq® ...... 98, 100 Technivie® ...... 77 simeprevir ...... 77 TEDUGLITIDE ...... 111 Simponi® ...... 98, 100 TEGSEDI ...... 149, 150 siponimod ...... 82 telbivudine ...... 76 SIRTURO ...... 80 TELOSTRISTAT ETHYL ...... 155 Skyrizi® ...... 98, 100 tenofovir alafenamide ...... 76 SODIUM OXYBATE ...... 83 tenofovir disoproxil fumarate ...... 76 SODIUM PHENYLBUTYRATE ...... 130 teriflunomide ...... 82 SODIUM ZIRCONIUM CYCLOSILICATE ...... 143 TERIPARATIDE ...... 49 sofosbuvir ...... 77 TESAMORELIN INJECTION ...... 45 sofosbuvir/velpatasvir ...... 77 Testred ...... 34 sofosbuvir/velpatasvir/voxilaprevir ...... 77 Tetrabenazine ...... 85 SOMAVERT® ...... 47 tezacaftor/ivacaftor ...... 93 Soriatane ...... 29 Thalomid ...... 11 Sovaldi® ...... 77 THIOLA ...... 164 SPRIX ...... 165 Tibsovo ...... 11 Sprycel ...... 11 tidrakizumab-asmn ...... 98 STADOL NS® ...... 19 Tiglutik ...... 11 Stelara® ...... 98, 101 TIOPRONIN ...... 164 Stivarga ...... 11 TOBI PodHaler® ...... 93 STRENSIQ ...... 109 TOBI® ...... 93 Striant ...... 34 Tobramcyin ...... 93 Suboxone® ...... 96 tobramycin ...... 93 Subutex® ...... 96 tocilizumab ...... 98 SUCCIMER ...... 30 tofacitinib ...... 98 SUCRAID ...... 166 Tolvaptan ...... 174 sucroferric oxyhydroxide ...... 88 TOLVAPTAN ...... 168 Sutent ...... 11 Tracleer ...... 24 Sylatron ...... 11 tramadol ...... 14 Symdeko™ ...... 93 tramadol/acetaminophen ...... 14 Synarel® ...... 107 Tremfya® ...... 98, 100 Synribo ...... 11 treprostinil ...... 24 Syprin ...... 33 tretinoin ...... 11 Trienterine ...... 33 T Tykerb ...... 11 Tylenol w/codeine® ...... 14 tadalafil ...... 24 Tylenol-Codeine #3® ...... 14 Tafinlar ...... 11 Tylenol-Codeine #4® ...... 14 Tagrisso ...... 11 Tymlos ...... 11 Takhzyro™ ...... 67 Tyvaso® ...... 24 Taltz® ...... 98, 100 Talzenna ...... 11 U tapentadol ...... 17 Targretin ...... 11 Udenyca ...... 63 Tasigna ...... 11 Ultracet® ...... 14 Tavalisse® ...... 62 Ultram® ...... 14 TBO-filgrastim ...... 63 Uptravi® ...... 24 Tecfidera® ...... 82 URIDINE TRIACETATE ...... 163

ustekinumab ...... 98 Xeljanz XR® ...... 98, 100 Xeljanz® ...... 98, 100 V Xenazine® ...... 85 XERMELO ...... 155 valbenazine ...... 85 XGEVA® ...... 50, 51 Valchlor ...... 11 Xodol® ...... 14 VECAMYL ...... 151 XOLAIR ...... 157 Velphoro® ...... 88 Xospata ...... 11 VELTASSA ...... 152 Xtandi ...... 11 Vemlidy® ...... 76 XURIDEN ...... 163 Venclexta ...... 11 XYREM® ...... 83 Ventavis® ...... 24 Xysoted ...... 34 Verzenio ...... 11 Vfend ...... 74 Z VIBERZI ...... 153, 154 Vicodin® ...... 14 ZarxioTM ...... 63 Viekira Pak® ...... 77 ZAVESCA® ...... 52 Viekira XR® ...... 77 Zejula ...... 11 Viread® ...... 76 ZELAPAR ...... 162 Vitrakvi ...... 11 Zelboraf ...... 11 Vizimpro ...... 11 Zepatier® ...... 77 Voriconazole ...... 74 zilueton ...... 95 Vosevi™ ...... 77 Zinc Acetate ...... 33 Votrient ...... 11 Zolinza ...... 11 Zomacton® ...... 42 X Zorbtive® ...... 42 Zubsolv® ...... 96 XADAGO ...... 155 Zydelig ...... 11 Xalkori ...... 11 Zyflo ...... 95 Zykadia ...... 11