Applies to: Nebraska Supplemental PDL Albenza (albendazole) Albenza (albendazole)

Covered uses All medically accepted indications

Exclusion Criteria See age restrictions

Required Medical Enterobiasis, ascariasis: Medical documentation indicating use Information for treatment for enterobiasis and previous trial and failure to ivermectin and Pin-X

Intestinal strongyloidiasis, cutaneous larva migrans, or infection by loa loa: Medical documentation indicating use for treatment for intestinal strongyloidiasis, cutaneous larva migrans, or infection by loa loa and previous trial and failure to ivermectin. Approve for use as empiric treatment for presumptive strongyloides infection in Sub-Saharan Africa refugees from LoaLoa endemic countries, regardless of previous ivermectin use.2

Echinococcosis (Hydatid disease), neurocysticercosis: Medical documentation indicating the use for treatment of echinococcosis or neurocysticercosis. Age restrictions 1 year of age or older Prescriber None Restrictions Coverage Duration 6 months (Hytadid) 1 month for other indications Other Criteria None

Reference 1. Albenza [package insert]. Amedra Pharmaceuticals. Horsham, PA. February, 2013 2. Centers for Disease Control and Prevention. Guidelines for Overseas Presumptive Treatment of Strongyloidiasis, Schistosomiasis, and Soil-Transmitted Helminth Infections. http://www.cdc.gov/immigrantrefugeehealth/guidelines/overseas/intestinalparasites- overseas.html. Accessed August 20, 2014.

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Applies to: Nebraska Supplemental PDL Makena (17 alpha-hydroxyprogesterone)

Makena (17 alpha-hydroxyprogesterone)

Covered uses All medically accepted indications

Exclusion Criteria <16 years of age >50 years of age

Do not use Makena in women with any of the following conditions: • Current or history of thrombosis or thromboembolic disorders • Known or suspected breast cancer, other hormone- sensitive cancer, or history of these conditions • Undiagnosed abnormal vaginal bleeding unrelated to pregnancy • Cholestatic jaundice of pregnancy • Liver tumors, benign or malignant, or active liver disease • Uncontrolled hypertension Required Medical Statement of need for preterm delivery prophylaxis in females Information with a singleton pregnancy who have a history of singleton spontaneous preterm birth:

1. Must be continued weekly until week 37 or delivery AND 2. Must be initiated between week 16 and week 27 of pregnancy

Age restrictions Women between 16 and 50 years of age

Prescriber N/A Restrictions Coverage Duration Up to 21 weeks Other Criteria Max Dose 250mg weekly Makena is not intended for use in women with multiple gestations

References: 1. Makena [package insert]. Chesterfield, MO: Ther-Rx Corporation; February 2014. 2. How H, Barton J, Istwan N, Prophylaxis with 17 alpha-hydroxyprogesterone caproate for prevention of recurrent preterm delivery: does gestational age ast initiation of treatment matter? American Journal of Obstetrics and Gynecology. 2007 September.

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Applies to: Nebraska Supplemental PDL Makena (17 alpha-hydroxyprogesterone)

3. Rebarber A, Roman A, Fox N, Recurrent Preterm Birth Prevention in Women Receiving Prophylactic 17P Experiencing Symptoms of Preterm Labor. Presented at 32nd Annual meeting of the Society for Maternal-Fetal Medicine, Dallas, Texas. February 2012. 4. Gonzalez-Quintero V, Istwan N, Rhea D, Gestational Age at Initiation of 17- hydroxyprogesterone caproate (17P) and recurrent preterm delivery. J Matern Fetal Neonatal Med. 2007 Mar; 20(3): 249-52.

Updated 01/2017 Page 2 of 2

Applies to: Aristada (aripiprazole lauroxil) Nebraska Supplemental PDL

Aristada (aripiprazole lauroxil, extended release)

Covered uses All medically accepted indications Exclusion Criteria Aristada should be avoided in populations with dementia-related psychosis. (Black Box warning) Required Medical 1. Medical diagnosis of schizophrenia Information 2. Must have failed at least 2 weeks of 2 oral atypical antipsychotics (aripiprazole, risperidone, paliperidone, ziprasidone, quetiapine, olanzapine, etc.) OR provide documentation of non-compliance, inability to swallow oral medications, or contraindication to oral atypical antipsychotics. 3. Requested dose is administered once monthly, every 6 weeks (882 mg only), or every 8 weeks (1064 mg only). Age restrictions 18 years or older Prescriber N/A Restrictions Coverage Duration 1 year Other Criteria Members currently taking this medication at the time of enrollment will not be required to meet prerequisites for authorization

References: 1) Aristada [package insert]. Alkermes, Inc. Waltham, MA. July 2016

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Applies to: Aripiprazole ER Susp (Abilify Maintena) Nebraska Supplemental PDL

Abilify Maintena (aripiprazole extended release suspension)

Covered uses All medically accepted indications Exclusion Criteria Abilify Maintena should be avoided in populations with dementia-related psychosis. (Black Box warning) Required Medical 1. Medical diagnosis of Schizophrenia or Bipolar I disorder Information 2. Must have failed at least 2 weeks of 2 oral atypical antipsychotics (aripiprazole, risperidone, paliperidone, ziprasidone, quetiapine, olanzapine, etc.) OR provide documentation of non-compliance, inability to swallow oral medications, or contraindication to oral atypical antipsychotics.

3. Requested maintenance dose is administered once monthly

Age restrictions 18 years of age and older Prescriber N/A Restrictions Coverage Duration 1 year Other Criteria Members currently taking this medication at the time of enrollment will not be required to meet prerequisites for authorization

The recommended starting and maintenance dose of Abilify Maintena is 400 mg monthly (no sooner than 26 days after the previous injection).

References: • Abilify Maintena (Aripiprazole). National Library of Medicine and National Institutes of Health. https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=ee49f3b1-1650-47ff- 9fb1-ea53fe0b92b6#S2.5. Updated February 23, 2017. Accessed August 8, 2017.

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Applies to: Nebraska Supplemental PDL Mepron (atovaquone7mg/ 5 mL)

Mepron (atovaquone 750 mg/ 5 mL)

Covered uses All FDA approved indications

Exclusion Criteria N/A

Required Medical Pneumocystis jirovecii pneumonia (PCP) Information 1. Atovaquone 750 mg/ 5 mL will be approved based on all of the following criteria: a. The patient has a diagnosis (i.e. HIV) warranting PCP infection prophylaxis. -AND- b. The patient has a documented intolerance to TMP-SMX and dapsone.

-OR

2. Atovaquone 750 mg/ 5 mL will be approved based on all of the following criteria: a. The patient has a diagnosis of acute mild to moderate pneumonia caused by P. jirovecii. -AND b. The patient has a documented intolerance to TMP-SMX.

Age restrictions N/A

Prescriber N/A Restrictions Coverage 6 months Duration Other Criteria N/A

REFERENCES

1 Mepron® Prescribing Information. GlaxoSmithKline, March 2014 2 Centers for Disease Control and Prevention. Guidelines for Prevention and Treatment of Opportunistic Infections in HIV-Infected Adults and Adolescents. MMWR 2009;58 (No. RR4): 6-10. 3 Atovaquone. Facts and Comparisons. Web. 26 July 2011.

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Applies to: Nebraska Supplemental PDL Mepron (atovaquone7mg/ 5 mL)

4 El-Sadr WM, Murphy RL, Yurik TM, et al. Atovaquone compared with dapsone for the prevention of Pneumocystis carinii pneumonia in patients with HIV infection who cannot tolerate trimethoprim, sulfonamides, or both. N Engl J Med 1998;339:1889-1895.

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Applies to: Nebraska Supplemental PDL Topical Santyl (collagenase)

Topical Santyl (collagenase)

Covered uses All medically accepted indications

Exclusion Criteria Stage 1 ulcers Wounds with healthy, non-necrotic tissue

Required Medical Use for treatment of: Information a) Burns- severe partial or full thickness wounds b) Decubitus ulcer c) Diabetic ulcer d) Varicose ulcer

Age restrictions 18 years of age and older

Prescriber N/A Restrictions Coverage Duration 3 months

Other Criteria Dosing: Apply once daily (or more frequently if the dressing becomes soiled).

References: 1. Product Information: COLLAGENASE SANTYL(R) topical ointment, collagenase topical ointment. Healthpoint Ltd. Fort Worth, TX 76107, 2013

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Applies to: Nebraska Supplemental PDL

Compounded Products

Covered uses All medically accepted indications Exclusion Criteria Compounded products ≥ $140 Certain cream, ointment, gel, and foam bases with GPI 98 Required Medical All individual ingredients within a compound, regardless of Information RX/OTC status, are covered if the aggregate cost of the compound is < $140 Age restrictions N/A Prescriber N/A Restrictions Coverage Duration Based on submitted request Other Criteria N/A

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Applies to: Jadenu (deferasirox) Nebraska Supplemental PDL

Jadenu (deferasirox) Covered uses All FDA approved indications Exclusion Criteria Serum creatinine greater than 2 times the age-appropriate upper limit of normal (ULN) OR CrCl less than 40 ml/min Poor performance status High-risk myelodysplastic syndromes or advanced malignancies Platelet count less than 50 x 109/L

Required Medical 1) Documentation of chronic iron overload due to blood transfusions Information (transfusional hemosiderosis) with: • Documentation of red blood cells transfusion of at least 100 mL/kg of packed red blood cells • Baseline serum ferritin level of 1,000 mcg/L prior to therapy • Baseline iron levels prior to therapy • Current serum ferritin levels greater 500 mcg/L • Current iron levels • Current weight • Initial dose: 14 mg/kg/day with dosage adjustments based on response 2) Documentation of chronic iron overload resulting from a genetic blood disorder called nontransfusion-dependent thalassemia. • Current serum ferritin levels greater 300 mcg/L • Liver iron concentration (LIC) of at least 5 milligrams of iron per gram of liver dry weight (mg Fe/g dw) • Current weight • Initial dose: 7 mg/kg/day with dosage adjustments based on response Age restrictions Age 2 or greater with chronic iron overload due to blood transfusions. Age 10 or greater with chronic iron overload resulting from a genetic blood disorder called non-transfusion dependent thalassemia. Prescriber Restrictions Hematologist Coverage Duration 6 months Other Criteria Max dose 28 mg/kg/day Reference:

Jadenu [package insert]. Novartis Pharmaceuticals. East Hanover, NJ. October 2015

Last updated 11/2015

Applies to: Nebraska Supplemental PDL Pulmozyme (dornase alfa)

Pulmozyme (dornase alfa)

Covered uses All medically accepted indications

Exclusion Criteria Required Medical Stated diagnosis from a pulmonologist of Cystic Fibrosis Information Stated diagnosis of atelectasis and/or pulmonary mucus plugging in non-cystic fibrosis disease states with trial and failure of preferred alternative

Age restrictions

Prescriber Pulmonologist Restrictions Coverage Duration 1 year

Other Criteria Safety and efficacy of daily administration have not been demonstrated in patients for longer than 12 months.

Preferred Alternative CF: approve Atelectasis and/or mucus plugging: acetylcysteine

References: 1. Product Information: Pulmozyme(R) inhalation solution, dornase alfa inhalation solution. Genentech, Inc., South San Francisco, CA, 2010.

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Applies to: Nebraska Supplemental PDL Multaq (dronedarone)

Multaq (dronedarone)

Covered uses All FDA approved indications Exclusion Criteria • Patients with PERMANENT atrial fibrillation who will not or cannot be cardioverted to normal sinus rhythm • Symptomatic heart failure with recent decompensation requiring hospitalization or NYHA Class IV symptoms • Concomitant use of a strong CYP3A inhibitor • Concomitant use of drugs or herbal products that prolong the QT interval and may induce Torsade de Pointes • Pregnant or nursing • Liver or lung toxicity related to the previous use of amiodarone • Severe hepatic impairment Required Medical • Diagnosis of one of the following: Information 1. Paroxysmal atrial fibrillation (AF) 2. Persistent AF defined as AF less than 6 months duration AND • One of the following: 1. Patient is in sinus rhythm 2. Patient is planned to undergo cardioversion to sinus rhythm AND • Patient receiving appropriate antithrombotic therapy AND • One of following: 1. Tried and failed amiodarone 2. Intolerant to or has a hypersensitivity to amiodarone

Age restrictions Adults ≥18 years old Prescriber Cardiologist Restrictions Coverage Duration 12 months Other Criteria N/A

References: 1. Multaq [Prescribing Information]. Sanofi Winthrop Industrie. Ambares, France. March 2014. 2. 2014 AHA/ACC/HRS Guideline for the Management of Patients With Atrial Fibrillation: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol. 2014;64(21):2246-2280

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Applies to: Nebraska Supplemental PDL

Early and Periodic Screening, Diagnostic, and Treatment (EPSDT)

Covered uses All medically necessary indications for members 21 years old or younger Exclusion Criteria N/A Required Medical A statement of medical necessity from the prescriber is required Information for FDA approved drugs including but not limited to those within the following classes:

• Agents used for anorexia, weight gain or weight loss • Agents used to promote fertility • Agents used for cosmetic purposes or hair growth • Drugs for the treatment of erectile dysfunction • DESI drugs or drugs that may have been determined to be identical, similar or related • Investigational or experimental drugs

Age restrictions Applies to members under 21 years of age

Prescriber Any qualified provider operating within the scope of his or her Restrictions practice, as defined by state law. Coverage Duration N/A Other Criteria N/A

References: 1) EPSDT- A Guide for States: Coverage in the Medicaid Benefit for Children and Adolescents. Center for Medicare and Medicaid Services. June 2014. Available at: http://www.medicaid.gov/Medicaid-CHIP-Program-Information/By- Topics/Benefits/Early-and-Periodic-Screening-Diagnostic-and-Treatment.html

Last updated 06/2017 Page 1 of 1

Applies to: Nebraska Supplemental PDL Entecavir

Entecavir

Covered All medically accepted indications uses Exclusion Criteria Required Criteria for approval: Medical • Must provide statement indicating FDA approved indication of hepatitis Information B AND • Trial and failure of preferred drug

Age Age 16 years or greater restrictions Prescriber Restrictions Coverage 6 months Duration Other Preferred Drugs: Criteria Lamivudine oral tablet 100 mg

Entecavir prescribing information contains the following boxed warning: Severe acute exacerbations of hepatitis have been reported in patients who have discontinued anti-Hepatitis B therapy including entecavir. Hepatic function should be monitored closely with both clinical and laboratory follow-up for at least several months in patients who discontinue anti-Hepatitis B therapy. If appropriate, initiation of anti-hepatitis B therapy may be warranted. Entecavir has not been evaluated in HIV/HBV co-infected patients who were not simultaneously receiving effective HIV treatment. Limited clinical experience suggests there is a potential for the development of resistance to HIV nucleoside reverse transcriptase inhibitors if entecavir is used to treat chronic hepatitis B virus infection in patients with HIV infection that is not being treated. Therefore, therapy with entecavir is not recommended for HIV/HBV co-infected patients who are not also receiving HAART. Before initiating entecavir therapy, HIV antibody testing should be offered to all patients. Entecavir has not been studied as a treatment for HIV infection and is not recommended for this use. Lactic acidosis and severe hepatomegaly with steatosis, including fatal cases, have been reported with the use of nucleoside analogue inhibitors, including entecavir, alone or in combination with antiretrovirals. A majority of these cases have been in women. Obesity and prolonged nucleoside inhibitor exposure may be risk factors. Particular caution should be exercised when administering nucleoside analogue inhibitors to any patient with known risk factors for liver disease; however, cases have also been reported in patients with no known risk factors. Lactic acidosis with entecavir use has been reported, often in association with hepatic decompensation, other serious medical conditions, or Updated 01/2017 Page 1 of 2

Applies to: Nebraska Supplemental PDL Entecavir

drug exposures. Patients with decompensated liver disease may be at higher risk for lactic acidosis. Treatment with entecavir should be suspended in any patient who develops clinical or laboratory findings suggestive of lactic acidosis or pronounced hepatotoxicity (which may include hepatomegaly and steatosis even in the absence of marked transaminase elevations).

References:

1. Baraclude [package insert]. Princeton, NJ: Bristol-Myers Squibb; August 2015 2. Entecavir [package insert.]. Dayton, NJ: Aurobindo Pharma USA, Inc.; April 2016. 3. Terrault NA, Bzowej NH, Chang K-M, Hwang JP, Jonas MM, Murad MH. AASLD guidelines for treatment of chronic hepatitis B. Hepatology 2016;63:261-283.

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Applies to: Nebraska Supplemental PDL Toposar (etoposide)

Toposar (etoposide)

Covered uses All medically accepted indications

Exclusion Criteria

Required Medical Stated diagnosis from oncologist of one of the following: Information 1. Refractory testicular tumors in combination with other approved chemotherapy a. Cisplatin + Bleomycin b. Carboplatin + Bleomycin c. Ifosfamide + Mensa + Cisplatin

2. Small cell lung cancer in combination with other approved chemotherapy a. Cyclophosphamide+ Doxorubicin b. Carboplatin c. Cisplatin

Age restrictions

Prescriber Oncologist Restrictions Coverage Duration 1 year Other Criteria Members currently taking this medication at time of enrollment will not be required to meet prerequisites for prior authorization.

References: 1. Product Information: TOPOSAR(TM) intravenous injection, etoposide intravenous injection. Teva Parenteral Medicines, Inc. (per DailyMed), Irvine, CA, 2011.

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Applies to: Nebraska Supplemental PDL Adrucil (fluorouracil)

Adrucil (fluorouracil)

Covered uses All medically accepted indications

Exclusion Criteria Therapy is contraindicated for patients in poor a nutritional state, those with depressed bone marrow function, or those with potentially serious infections.

Required Medical Stated diagnosis from oncologist for the palliative management of Information carcinoma of the colon, rectum, breast, stomach, or pancreas. -AND- Documentation of normal white blood cell count

Age restrictions Age 18 years or greater

Prescriber Oncologist Restrictions Coverage Duration 1 year

Other Criteria Members currently taking this medication at time of enrollment will not be required to meet prerequisites for prior authorization.

Reference Adrucil [package insert]. Teva Parenteral Medicines, Inc. Irvine, CA. August, 2012

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Applies to: Nebraska Supplemental PDL Fluorouracil topical

Fluorouracil topical cream 5%, solution 2%, 5%

Covered uses All medically accepted indications

Exclusion Criteria Pregnancy Patients with dihydropyrimidine dehydrogenase (DPD) enzyme deficiency Required Medical Stated diagnosis from dermatologist of multiple actinic/ solar Information keratoses or superficial basal cell carcinomas (sBCC).

Age restrictions Age 18 years or greater

Prescriber Dermatologist Restrictions Coverage Duration Actinic / solar keratoses:8weeks sBCC: 3 months Other Criteria Members currently taking this medication at time of enrollment will not be required to meet prerequisites for prior authorization.

References:

Efudex [package insert] Valeant Pharmaceuticals, Bridgewater, NJ. May 2014

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Applies to: Nebraska Supplemental PDL FIRAZYR (Icatibant) FIRAZYR® (Icatibant)

Covered uses All medically accepted indications

Exclusion Criteria N/A

Required Medical Statement of FDA approved indication Information Treatment of acute attacks of hereditary angioedema (HAE)

Documentation of number of attacks per month Age restrictions 18 years of age and older Prescriber Immunologist Restrictions Coverage Duration 3 month Other Criteria Maximum 3 (30mg) doses in 24 hours Reauthorization requires statement of reduction in number of attacks per month.

Reference Firazyr [package insert]. Shire Orphan Therapies, Inc. Lexington, MA. August 2013.

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Applies to: Nebraska Supplemental PDL Corlanor (ivabradine)

Corlanor (ivabradine)

Covered uses All FDA Approved Indications Exclusion Criteria • Acute decompensated heart failure • Severe hepatic impairment (Child-Pugh C) • Pregnancy • Concomitant use of strong cytochrome CYP3A4 inhibitors Required Medical Approve based on diagnosis of stable, symptomatic chronic heart Information failure with the following: • Left ventricular ejection fraction of 35% or less • Sinus rhythm with resting heart rate of 70 or greater BPM (before treatment initiation) o Dosage may be adjusted to achieve resting heart rate of 50-60 BPM after treatment has been initiated • Currently on maximally tolerated doses beta-blockers or contraindication to beta-blockers Age restrictions Age 18 years or greater Prescriber Cardiologist Restrictions Coverage Duration 1 year Other Criteria Recommended dosing: 5 mg twice daily Maximum dosing: 7.5 mg twice daily

References: 1. Corlanor (Ivabradine) package insert. Thousand Oaks, CA: Amgen Inc.; 2015 Apr.

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Applies to: Nebraska Supplemental PDL Kalydeco (ivacaftor)

Kalydeco (ivacaftor) tablets, granules

Covered uses All FDA approved indications

Exclusion Criteria Cystic Fibrosis patients w/o a G551 mutation in the CFTR gene Patients with a homozygous F508del mutation in the CFTR gene. Required Medical • Documentation of diagnosis of Cystic fibrosis Information • Statement that patient has a G551D, G1244E, G1349D, G178R, G551S, S1251N, S1255P, S549N, S549R, or R117H mutation in the CFTR gene

Age restrictions Tablets: 6 years of age or greater Granules: 2-5 years of age Prescriber Endocrinologist, pulmonologist, or immunologist Restrictions Coverage Duration 12 months Other Criteria

References: Kalydeco [Package Insert]. Boston, MA. Vertex Pharmaceuticals Incorporated; March 2015

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Applies to: Zyvox (linezolid) Nebraska Supplemental PDL

Zyvox (linezolid)

Covered uses All medically accepted indications

Exclusion Criteria Zyvox will not be approved in patients receiving any MAOI, or within two weeks of receiving an MAOI as evidenced by claims history Required Medical 1. Recent (within 30 days of request) Culture and Sensitivity Information report; AND 2. Documentation of one medically accepted indication (listed numerically below); AND 3. Vancomycin-resistant enterococcus faecium (VRE)

4. Nosocomial pneumonia (caused by MRSA)

5. Nosocomial or community acquired pneumonia (caused by MSSA or Streptococcus pneumonia): Trial and failure of two (2) preferred alternatives or resistance to all other appropriate therapies: Amoxicillin/clavulanic acid, Azithromycin, Cephalexin, Clarithromycin, Dicloxacillin, Doxycycline, Levofloxacin, Trimethoprim-sulfamethoxazole

6. Complicated skin / skin structure infections including diabetic foot infections, without osteomyelitis caused by MRSA Trial and failure of two (2) preferred alternatives or resistance to all other appropriate therapies: Doxycycline, Trimethoprim- sulfamethoxazole

7. Uncomplicated SSI caused by MRSA documented by C/S report or empirical treatment of patients with uncomplicated or community-acquired complicated SSI without osteomyelitis where MRSA infection is likely: Trial and failure of two (2) preferred alternatives or resistance to all other appropriate therapies: Trimethoprim-sulfamethoxazole, Dicloxacillin, Doxycycline, Minocycline, Clindamycin.

8. Uncomplicated or complicated SSI without osteomyelitis caused by MSSA, Streptococcus pyogenes, or Streptococcus agalactiae (complicated SSI only): Trial and failure of two (2) preferred alternatives or resistance to all other appropriate therapies: Amoxicillin/clavulanic acid, Cephalexin, Ciprofloxacin, Clindamycin, levofloxacin, Trimethoprim/Sulfamethoxazole, Dicloxacillin.

Age restrictions

Last updated 02/2017 Page 1 of 2

Applies to: Zyvox (linezolid) Nebraska Supplemental PDL

Prescriber Restrictions Coverage Duration 28 days for Vancomycin-resistant E. Faecium (VRE) 14 Days for all other infections Other Criteria Member must not currently be receiving therapy with any of the following medications: a) SSRIs b) SNRIs c) Triptans d) MAOIs e) Mirtazapine, Trazodone

References: 1. Zyvox [package insert]. New York, NY: Pfizer Pharmaceuticals LLC. 2013.

Last updated 02/2017 Page 2 of 2

Applies to: Nebraska Supplemental PDL

Nucala (mepolizumab)

Covered uses All FDA Approved Indications Exclusion Criteria • Use as treatment for acute asthma symptoms or acute exacerbations Required Medical Documentation of all of the following: Information • Diagnosis of severe asthma with an eosinophilic phenotype o Blood eosinophil count greater than or equal to 300 cells/mcL within the previous 12 months OR o Blood eosinophils count greater than or equal to 150 cells/mcL at initiation of therapy • Submitted notes or claims data indicate that Nucala will be used as add-on maintenance treatment with an inhaled corticosteroid, long-acting beta2 agonist, leukotriene receptor antagonist, and/or theophylline • Dosage is 100 mg once every 4 weeks • Member has been enrolled in WellCare’s Asthma disease management program for 6 months (or less depending on clinical situation)

Reauthorization Treatment with Nucala has resulted in clinical improvement as documented by one or more of the following: • Decreased utilization of rescue medications OR • Decreased frequency of exacerbations (defined as worsening of asthma that requires an increase in ICS dose or treatment with systemic corticosteroids) OR • Increase in predicted FEV1 from pretreatment baseline OR • Reduction in reported asthma-related symptoms, such as, asthmatic symptoms upon awakening, coughing, fatigue, shortness of breath, sleep disturbance, or wheezing. Age restrictions 12 years and older Prescriber Allergist, Pulmonologist, or Immunologist Restrictions Coverage Duration 1 year Other Criteria N/A

References: 1) Nucala [package insert]. Research Triangle Park, NC, GlaxoSmithKline

Last updated 05/2016 Page 1 of 1

Applies to: Nebraska Supplemental PDL Xolair (omalizumab)

Covered uses All FDA approved indications

Exclusion Criteria N/A Required Medical Documentation of the following: Information 1) Moderate to severe persistent asthma in adults and adolescents 12 years of age and older who have a positive skin test or in vitro reactivity to a perennial aeroallergen and whose symptoms are inadequately controlled with inhaled corticosteroids (ICS). a) IgE levels prior to initiation of therapy AND i. IgE level of 30 IU/ml to 700 IU/ml only b) RAST or Allergy skin test - Positive skin test or in vitro reactivity to a perennial aeroallergen AND c) Weight between 30 - 150kg (current weight must be provided) AND d) Peak flow rate less than 80% of predicted with at least a 30% variability AND e) Member has been enrolled in WellCare’s Asthma disease management program for 6 months (or less depending on clinical situation)

2) Moderate to severe persistent asthma in children 6 to <12 years of age and older who have a positive skin test or in vitro reactivity to a perennial aeroallergen and whose symptoms are inadequately controlled with inhaled corticosteroids (ICS). a. Xolair will be used as add-on therapy AND b. Member has been enrolled in WellCare’s Asthma disease management program for 6 months (or less depending on clinical situation)

3) Chronic Idiopathic Urticaria (CIU), refractory to all preferred agents: H1 antihistamines, corticosteroids and leukotriene modifiers, each used for a minimum of 30 days within the last 3 months AND for which allergic urticaria and other non- idiopathic urticaria is ruled out.

Reauthorization For asthma, treatment with Xolair has resulted in clinical improvement as documented by one or more of the following: • Decreased utilization of rescue medications OR • Decreased frequency of exacerbations (defined as worsening of asthma that requires an increase in ICS dose or treatment with

Last updated 07/2016 Page 1 of 2

Applies to: Nebraska Supplemental PDL

systemic corticosteroids) OR • Increase in predicted FEV1 from pretreatment baseline OR Reduction in reported asthma-related symptoms, such as, asthmatic symptoms upon awakening, coughing, fatigue, shortness of breath, sleep disturbance, or wheezing.

For Chronic Idiopathic Urticaria (CIU), treatment with Xolair has resulted in reduced itch or hive count.

Age restrictions Age 6 years or greater Prescriber Pulmonologist, Allergist, or Immunologist Restrictions Coverage Duration 1 year Other Criteria N/A

References: 1. Product Information: XOLAIR(R) subcutaneous injection, omalizumab subcutaneous injection. Genentech, Inc, South San Francisco, CA, 2010. 2. Product Information: XOLAIR(R) subcutaneous injection powder, omalizumab subcutaneous injection powder. Genentech, Inc. (per manufacturer), South San Francisco, CA, July 2016

Last updated 07/2016 Page 2 of 2

Applies to: Nebraska Supplemental PDL Oxandrin (oxandrolone)

Oxandrin (oxandrolone)

Covered uses All medically accepted indications

Exclusion Criteria Pregnancy X Required Medical Diagnosis of: Information • Bone pain: For the relief of the bone pain frequently accompanying osteoporosis Preferred Alternative(s): Alendronate calcitonin Evista Fortical NSAIDS (meloxicam, naproxen, ibuprofen, ketoprofen)

• Weight gain (Cachexia): Adjunctive therapy to promote weight gain in patients with AIDS associated wasting syndrome and after weight loss following extensive surgery, chronic infections or severe trauma Preferred Alternative: Megestrol

• To offset the protein catabolism associated with prolonged administration of corticosteroids Preferred Alternative: Megestrol

• Hereditary angioedema, for chronic prophylaxis Preferred Alternative: Danazol

Age restrictions

Prescriber Restrictions Coverage Duration 1 year Other Criteria Criteria for approval: Documentation of trial and failure of an adequate trial (1 month minimum) of at least one (1) preferred alternative.

References: 1. Product Information: OXANDRIN(R) tablets, oxandrolone tablets. Savient Pharmaceuticals, Inc., East Brunswick, NJ, 2005.

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Applies to: Nebraska Supplemental PDL Oxandrin (oxandrolone)

2. Caballero T , Baeza ML , Cabanas R , et al: Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part II. Treatment, follow-up, and special situations. J Investig Allergol Clin Immunol 2011; 21(6):422-441.

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Applies to: Invega Sustenna (paliperidone palmitate) Nebraska Supplemental PDL

Invega Sustenna (paliperidone palmitate)

Covered uses All medically accepted indications Exclusion Invega Sustenna should be avoided in populations with dementia- Criteria related psychosis. (Black Box warning) Required 1. Medical diagnosis of Schizophrenia or Schizoaffective disorder Medical Information 2. Must have failed at least 2 weeks of 2 oral atypical antipsychotics (aripiprazole, risperidone, paliperidone, ziprasidone, quetiapine, olanzapine, etc.) OR provide documentation of non-compliance, inability to swallow oral medications, or contraindication to oral atypical antipsychotics.

3. Requested maintenance dose is administered once monthly

Age restrictions 18 years of age and older Prescriber N/A Restrictions Coverage 1 year Duration Other Criteria Members currently taking this medication at the time of enrollment will not be required to meet prerequisites for authorization

Dosing for Schizophrenia and Schizoaffective disorder: • The recommended dosing of INVEGA SUSTENNA® for each approved indication is displayed in Table 1:

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Applies to: Invega Sustenna (paliperidone palmitate) Nebraska Supplemental PDL

Table 1: Recommended Dosing of INVEGA SUSTENNA® for Adults with Schizophrenia or Schizoaffective Disorder Initiation Monthly Maximum Monthly Dosing Maintenance Dose Indication (deltoid) Dose* Day Day (deltoid or 1 8 gluteal) 234 156 39–234 mg† Schizophrenia 234 mg mg mg Schizoaffective 234 156 78–234 mg‡ 234 mg disorder mg mg • *Administered 5 weeks after the first injection. • † The recommended maintenance dose for treatment of schizophrenia is 117 mg. Some patients may benefit from lower or higher maintenance doses within the additional available strengths (39 mg, 78 mg, 156 mg, and 234 mg). • ‡ Adjust dose based on tolerability and/or efficacy using available strengths. The 39 mg strength was not studied in the long-term schizoaffective disorder study.

References: • Invega Sustenna (Paliperidone palmitate). National Library of Medicine and National Institutes of Health. https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=1af14e42-951d-414d- 8564-5d5fce138554. Updated July 14, 2017. Accessed August 8, 2017. • Lehman AF, Lieberman JA, Dixon LB, McGlashan TH, Miller AL, Perkins DO, Kreyenbuhl J. “Treatment of patients with schizophrenia”. American Psychiatric Association. https://psychiatryonline.org/pb/assets/raw/sitewide/practice_guidelines/guidelines /schizophrenia.pdf. Published February 2004. Accessed July 8, 2017.

Created 08/2017 Page 2 of 2

Applies to: Invega Trinza (paliperidone palmitate) Nebraska Supplemental PDL

Invega Trinza (paliperidone palmitate)

Covered uses All medically accepted indications Exclusion Invega Trinza should be avoided in populations with dementia- Criteria related psychosis. (Black Box warning) Required 1. Medical diagnosis of Schizophrenia Medical Information 2. Must have failed at least 2 weeks of 2 oral atypical antipsychotics (aripiprazole, risperidone, paliperidone, ziprasidone, quetiapine, olanzapine, etc.) OR provide documentation of non-compliance, inability to swallow oral medications, or contraindication to oral atypical antipsychotics

3. Documentation of prior treatment with Invega Sustenna for at least 4 consecutive months

4. Requested maintenance dose is administered once every three months

Age restrictions 18 years of age and older Prescriber N/A Restrictions Coverage 1 year Duration Other Criteria Dosing for Schizophrenia: Treatment should be initiated when the next 1-month Invega Sustenna dose is scheduled with an Invega Trinza dose based on the previous 1-month injection dose, using the equivalent 3.5-fold higher dose as shown in Table 1.

Created 08/2017 Page 1 of 2

Applies to: Invega Trinza (paliperidone palmitate) Nebraska Supplemental PDL

Table 1. INVEGA TRINZA® Doses for Adult Patients Adequately Treated with INVEGA SUSTENNA® If the Last Dose of Initiate INVEGA INVEGA TRINZA® at the SUSTENNA® is: Following Dose: 78 mg 273 mg 117 mg 410 mg 156 mg 546 mg 234 mg 819 mg

References: • Invega Trinza (Paliperidone palmitate ER). National Library of Medicine and National Institutes of Health. https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=c39e65d7-fa44-4e4c- 8b12-a654d3ed0eae#S8.1.Updated March 31, 2017. Accessed August 8, 2017.

Created 08/2017 Page 2 of 2

Applies to: Nebraska Supplemental PDL Synagis (Palivizumab)

Synagis® (Palivizumab) 2016-2017 Season

Covered uses Respiratory syncytial virus (RSV) infection prophylaxis Exclusion Criteria Required Medical Dosing Chart information / Age restrictions Patient Group Age at the Start of RSV Season Maximum Number of Doses Premature, no CLD, no CHD ≤ 12 months 5 Doses* *** Gestational Age < 29 weeks

Chronic Lung Disease (CLD) In ≤ 12 months 5 Doses*** first year of life

Gestational Age < 32 weeks AND Required >21% oxygen for at least the first 28 days after birth Chronic Lung Disease (CLD) In 13 months to 24 months 5 Doses*** second year of life

Gestational Age < 32 weeks AND Required >21% oxygen for at least the first 28 days after birth AND Continue to require medical support (chronic corticosteroid therapy, diuretic therapy or supplemental oxygen) Hemodynamically significant ≤ 12 months 5 Doses*** (cyanotic or acyanotic) Congenital Heart Disease (CHD) ** Infant receiving medication to control congestive heart failure AND will require cardiac surgical procedures OR Infant with moderate to severe pulmonary hypertension Congenital Abnormalities of the ≤ 12 months 5 Doses*** Airway or Neuromuscular Condition that Compromises Handling of Respiratory Secretions

*Total number of Synagis® doses may vary based on birth month. Infants born during the RSV season require less than 5 monthly doses. See dosing chart.

Updated 01/2017 Page 1 of 3 Applies to: Nebraska Supplemental PDL Synagis (Palivizumab)

**Infants with CHD not at increased risk of RSV infection and generally should not receive immunoprophylaxis: • Infants and children with hemodynamically insignificant heart disease (eg. Secundum atrial septal defect, small ventricular septal defect, pulmonic stenosis, uncomplicated aortic stenosis, mild coarctation of the aorta, and patent ductus arteriosus) • Infants with lesions adequeately corrected by surgery unless they continue to require medication for CHF • Infants with mild cardiomyopathy who are not receiving medical therapy for the condition • Children in the second year of life ***5 monthly doses of palivizumab at 15mg/kg per dose will provide more than 6 months (>24weeks) of serum palivizumab concentrations above the desired level for most children.

Dose ♦ Infants and children <2 years of age at high risk of RSV disease: 15 mg/kg of body weight I.M. monthly (approximately every 30 days) with subsequent monthly doses (dependent on length of RSV season) up to the recommended number of doses. ♦ If the dose needed is less than 5 mg over the approved vial size, round down to the nearest vial size. If the dose needed is ≥ 5 mg over the approved vial size, then the new vial size will be approved. ♦ Administer prophylaxis throughout RSV season (which is dependent on state and region; see season chart). ♦ It is recommended that therapy begin prior to the onset of the RSV season. ♦ Cardio-pulmonary bypass: Synagis® serum levels are decreased after cardio-pulmonary bypass. For children who still require prophylaxis, a postoperative dose of palivizumab (15 mg/kg) should be considered as soon as the patient is medically stable. Thereafter, doses should be administered monthly. ♦ Prophylactic serum concentrations are reached within 48 hours of dosing (see pharmacokinetics). ♦ No data exist to support palivizumab use in controlling RSV outbreaks of nosocomial disease3

♦ NOTE: Patients who develop a RSV infection should continue to receive monthly prophylaxis for the recommended number of doses throughout the RSV season because there is more than one strain of RSV that concurrently circulates in a community. ♦ Monthly prophylaxis should be discontinued in any child who experiences a breakthrough RSV hospitalization. ♦ ♦ NOTE: If any infant or young child receiving monthly palivizumab prophylaxis experiences a breakthrough RSV hospitalization, monthly prophylaxis should be discontinued because of the extremely low likelihood of a second RSV hospitalization in the same season (<0.5%)

Summary of seasons and injection approval time frame based on AAP 2009 Red Book and National CDC RSV Surveillance reports may be accessed at this website: http://www.cdc.gov/surveillance/nrevss/rsv/index.htmland

Nebraska November through March

Updated 01/2017 Page 2 of 3 Applies to: Nebraska Supplemental PDL Synagis (Palivizumab)

Coverage Born before RSV season maximum of 5 doses Duration Born during RSV season maximum of 4 doses Other Criteria *Per American Academy of Pediatrics (AAP) guidelines, the last dose of Synagis should be administered one month prior to the end of the season. Doses will be approved during above timeframes.

References:

1. American Academy of Pediatrics. Respiratory Syncytial Virus. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2009 Report of the Committee on Infectious Diseases. 28th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2009.

2. Hawaii American Association of Pediatrics Web site. http://www.hawaiiaap.org/pdfs/Guidelines%20For%20Prophylaxis%20For%20RSV%20Infection s%20-%208.1.12.pdf Accessed September 17, 2012.

3. Summary of seasons and injection approval time frame based on AAP 2009 Red Book and National CDC RSV Surveillance reports accessed at this website: http://www.cdc.gov/surveillance/nrevss/rsv/index.html

4. American Academy of Pediatrics. Updated Guidance for Palivizumab Prophylaxis Among Infants and Young Children at Increased risk of Hospitalization for Respiratory Syncytial Virus Infection. Pediatrics. July 28, 2014.

Updated 01/2017 Page 3 of 3 Applies to: Nebraska Supplemental PDL Elmiron (pentosan polysulfate sodium)

Elmiron (pentosan polysulfate sodium)

Covered uses All medically accepted indications

Exclusion Criteria N/A

Required Medical A. Initial Authorization Information 1. Elmiron will be approved based on the following criteria: a. The patient has a documented diagnosis of bladder pain or discomfort associated with interstitial cystitis

B. Re-Authorization 1. Elmiron will be approved based on the following criteria: a. The patient’s pain or discomfort has improved and adverse events are not present

Age restrictions 16 years of age

Prescriber Restrictions Coverage Duration Initial authorization of therapy will be issued for 6 months. Re-Authorization of therapy will be issued for 12 months. Other Criteria Recommended dose: 300 mg/day taken as one 100 mg capsule orally three times daily

References ® 1) Elmiron [package insert]. Raritan, NJ: Ortho-McNeil-Janssen Pharmaceuticals Inc.; September 2014. 2) Jarrell JF, Vilos GA, Allaire C, et al. Consensus Guidelines for the Management of Chronic Pelvic Pain. JOGC. 2005; 164: 781-801. 3) Homma Y, Ueda T, Tomoe H, et al. Clinical guidelines for interstitial cystitis and hypersensitive bladder syndrome. Int J Urol. 2009: 16; 597-615. 4) Fall M, Baranwoski AP, Elneil S, et al. EAU Guidelines on Chronic Pelvic Pain. Eur Urol. 2010; 57: 35-48.

Updated 01/2017 Page 1 of 1

Applies to: Nebraska Supplemental PDL Biltricide (praziquantel) Biltricide (praziquantel)

Covered uses All medically accepted indications

Exclusion Criteria < 4 years of age

Required Medical Medical documentation indicating use for treatment for: Information FDA LABELED INDICATIONS • Clonorchiasis caused by Clonorchis sinensis • Infection by Opisthorchis viverrini, or • Schistosomiasis cause by Schistosoma hematobium, Schistosoma japonicum, Schistosoma mansoni, Schistosoma mekongi • NON-FDA LABELED INDICATIONS • Cysticercosis (Preffered agent: Albenza) • Infection by Taeni (Preffered agent: Albenza) • Hymenolepiasis • Infection by Fasciolopsis buski • Infection by Heterophyes heterophyes, Intestinal fluke • Infection by Metagonimus yokogawai • Infection by Metorchis conjunctus, North American liver fluke • Infection by Paragonimus Age restrictions ≥ 4 years of age Prescriber None Restrictions Coverage Duration 1 month Other Criteria Dosing • Clonorchiasis: 25 mg/kg ORALLY 3 times over 1 day (at 4 to 6 hr intervals) • Infection by Opisthorchis viverrini: 25 mg/kg ORALLY 3 times over 1 day (at 4 to 6 hr intervals) • Schistosomiasis: 20 mg/kg ORALLY 3 times over 1 day (at 4 to 6 hr intervals)

References: I. Biltricide [package insert]. Wayne, NJ: Bayer Healthcare Pharmaceuticals, Inc.; 2011.

Updated 01/2017 Page 1 of 1

Applies to: Lyrica (pregabalin) Nebraska Non-PDL Lyrica (pregabalin)

Covered uses All medically accepted indications

Exclusion Criteria Required Medical Treatment of Information 1. As adjunctive therapy for adults with partial-onset seizures o member must try and fail gabapentin 2. Neuropathy o member must try and fail at least two of the following : venlafaxine IR or ER, amitriptyline and gabapentin (1800 mg/day)

3. Postherpetic neuralgia o member must try and fail gabapentin. (1800 mg/day)

4. Fibromyalgia o member must try and fail at least two of the following: venlafaxine IR or ER, amitriptyline, gabapentin (1800 mg/day) and duloxetine.

5. Central pain o member must try and fail gabapentin. (1800 mg/day)

6. General Anxiety Disorder o member must try and fail at least two of the following: venlafaxine IR or ER, paroxetine, and sertraline

Age restrictions Age 18 years or greater Prescriber Restrictions Coverage Duration 1 year Other Criteria Preferred Alternative: Neuropathy: Gabapentin, venlafaxine IR or ER, amitriptyline Postherpetic neuralgia: Gabapentin Fibromyalgia: Gabapentin, venlafaxine IR or ER, amitriptyline, duloxetine Central Pain: Gabapentin Generalized Anxiety disorder: Venlafaxine IR or ER, paroxetine, sertraline Required dose of gabapentin tried and failed: 1800 mg/day (neuropathy, neuralgia, fibromyalgia, central pain)

Reference 1. Lyrica [package insert]. Pfizer, Inc. New York, Ny. December 2013

Last updated 04/2016 Page 1 of 1

Applies to: Nebraska Supplemental PDL Daraprim (pyrimethamine)

Daraprim (pyrimethamine)

Covered uses FDA Approved Indications: For the treatment of toxoplasmosis, acute malaria, and chemoprophylaxis of malaria due to susceptible strains of plasmodia. Exclusion Criteria Megaloblastic anemia due to folate deficiency Hypersensitivity to pyrimethamine Required Medical Criteria for approval: Information 1) Toxoplasmosis a. Documented diagnosis AND b. Denotation that agent will be used in conjunction with a sulfonamide (i.e. sulfadiazine, sulfamethoxazole) * Preferred regimens for prophylaxis/treatment of Toxoplasmosis gondii encephalitis in HIV-infected include sulfamethoxazole/trimethoprim or atovaquone monotherapy or atovaquone plus sulfadiazine.

2) Acute malaria: a. Documented diagnosis AND b. Denotation that agent will be used in conjunction with a sulfonamide (i.e. sulfadiazine, sulfamethoxazole) AND c. Previous T/F treatment with a preferred FDA indicated medication (i.e. chloroquine, hydroxychloroquine, mefloquine, etc.)

3) Chemoprophylaxis of malaria a. Documented diagnosis AND b. Submission of specific strain(s) in the endemic area member is traveling to. Daraprim must be appropriate treatment according to CDC prevalence/resistance statistics. Note: Daraprim is not effective against P. vivax. ** For a complete list of malaria information by country, please visit the CDC website. c. Previous T/F treatment of all preferred regimens (chloroquine, hydroxychloroquine, atovaquone/proguanil, mefloquine, and quinine) OR Must provide adequate rationale why preferred agents are unacceptable. Infants aged 2 months and above. Prescriber None Restrictions Coverage Duration 1) Toxoplasmosis treatment: a. Adults: 8 Weeks b. Pediatrics: 4 Weeks

Updated 01/2017 Page 1 of 2

Applies to: Nebraska Supplemental PDL Daraprim (pyrimethamine)

2) Acute Malaria treatment: 12 Weeks 3) Chemoprophylaxis treatment: Based on request Other Criteria In general, dosing for the elderly should be conservative. It is recommended to start at the lower end of the dosing range, taking into consideration the hepatic, renal, or cardiac functionality of the patient, and of concomitant disease or other drug regimens.

Dosing: Toxoplasmosis Adult: 50 – 75 mg daily (with 1 – 4 g of a sulfonamide daily). This dosage is continued for 1 – 3 weeks, contingent on patient response and tolerability. The two drugs can then be reduced to one half of the initial dose for an additional 4 -5 weeks.

Pediatric: 1mg/kg/day divided into 2 equal daily doses; after 2-4 days, the dose may be reduced to one half for approximately one month. (The typical sulfonamide dosage should be used in conjunction with Daraprim).

Malaria prophylaxis Adults, adolescents > 10 years of age: 25 mg po once weekly Pediatric (4-10 years of age): 12.5 mg po weekly

Malaria treatment 25 mg daily for 2 days; following clinical cure, administer a once-weekly chemoprophylaxis regimen for ≥10 weeks

References: 1) Daraprim [package insert]. Amedra Pharmaceuticals. Horsham, PA. Oct. 2014

Updated 01/2017 Page 2 of 2

Applies to: Nebraska Supplemental PDL Cinqair (reslizumab)

Covered uses All FDA Approved Indications Exclusion Criteria • Use as treatment for acute asthma symptoms or acute exacerbations Required Medical Documentation of all of the following: Information • Diagnosis of severe asthma with an eosinophilic phenotype o Blood eosinophil count greater than or equal to 400 cells/mcL within the previous 4 weeks • Submitted notes or claims data indicate that Cinqair will be used as add-on maintenance treatment with an inhaled corticosteroid, long- acting beta2 agonist, leukotriene receptor antagonist, and/or theophylline • Dosage is 3 mg/kg once every 4 weeks • Member has been enrolled in WellCare’s Asthma disease management program for 6 months (or less depending on clinical situation)

Reauthorization Treatment with Cinqair has resulted in clinical improvement as documented by one or more of the following: • Decreased utilization of rescue medications OR • Decreased frequency of exacerbations (defined as worsening of asthma that requires an increase in ICS dose or treatment with systemic corticosteroids) OR • Increase in predicted FEV1 from pretreatment baseline OR • Reduction in reported asthma-related symptoms, such as, asthmatic symptoms upon awakening, coughing, fatigue, shortness of breath, sleep disturbance, or wheezing. Age restrictions 18 years and older Prescriber Allergist, Pulmonologist, or Immunologist Restrictions Coverage Duration 1 year

Other Criteria N/A

References: 1) Cinqair [package insert]. Frazer, PA, Teva Respiratory, LLC

Last updated 05/2016 Page 1 of 1

Applies to: Nebraska Supplemental PDL Risperdal Consta

Risperdal Consta (risperidone, extended release)

Covered uses All medically accepted indications Exclusion Criteria Risperdal Consta should be avoided in populations with dementia-related psychosis. (Black Box warning) Required Medical 1. Medical diagnosis of schizophrenia or bipolar 1 disorder Information 2. Must have failed at least 2 weeks of 2 oral atypical antipsychotics (aripiprazole, risperidone, paliperidone, ziprasidone, quetiapine, olanzapine, etc.) OR provide documentation of non-compliance, inability to swallow oral medications, or contraindication to oral atypical antipsychotics.

3. Requested dose is administered every 2 weeks. Age restrictions 18 years or older Prescriber N/A Restrictions Coverage Duration 1 year Other Criteria Members currently taking this medication at the time of enrollment will not be required to meet prerequisites for authorization

References: 1) Risperdal Consta [package insert]. Janssen Pharmaceuticals, Inc. Horsham, PA. September 2016

Updated 08/2017 Page 1 of 1

Applies to: Nebraska Supplemental PDL Supartz (sodium hyaluronate/hyaluronic acid)

Supartz (sodium hyaluronate/hyaluronic acid)

Covered uses All FDA-approved indications

Exclusion Criteria Viscosupplementation is considered experimental and investigational for any indication other than osteoarthritis of the knee. These include, but are not limited to: • Chondromalacia patellae • Facet joint arthropathy • Osteochondritis dissecans • Patellofemoral arthritis • Patellofemoral syndrome • Plantar nerve entrapment syndrome • For use in joints other than the knee • Active synovitis

When a repeat series of injections is initiated prior to six months after completion of the previous course of treatment. Required Medical Treatment of pain in osteoarthritis (OA) of the knee Information Failure to respond adequately to two of the following for at least 3 consecutive months: 1. conservative nonpharmacologic therapy (low-impact aerobic exercises, neuromuscular education, weight loss programs, formal physical therapy) OR 2. simple analgesics (e.g., topical or oral NSAIDs, Tramadol) OR 3. intra-articular corticosteroid injection therapy Age restrictions 18 years or greater

Prescriber Orthopedic specialist Restrictions Interventional pain physician Rheumatologist Coverage Duration 6 weeks Other Criteria Quantity Limit: Supartz: 1 injection weekly for 5 weeks

References: 1. Product Information: Supartz(R), sodium hyaluronate injection. Smith & Nephew Inc., Memphis, TN, 2004. 2. Product Information: HYALGAN(R) injection, sodium hyaluronate injection. Sanofi-Synthelabo,Inc, New York, NY, 2001.

Updated 01/2017 Page 1 of 2

Applies to: Nebraska Supplemental PDL Supartz (sodium hyaluronate/hyaluronic acid)

3. Product Information: Orthovisc(R), high molecular weight hyaluronan. Ortho Biotech, Raritan, NJ, 2004. 4. Product Information: Hyalgan®, sodium hyaluronate. Sanofi-Synthelabo, New York, New York, 2000. 5. Product Information: Synvisc(R), Hylan G-F 20. Genzyme Corporation, Ridgefield, New Jersey, USA, 2004. 6. Viscosupplementation treatment of arthritis. American Academy of Orthopaedic surgeons. Website: http://orthoinfo.aaos.org/topic.cfm?topic=a00217. Published March 2014. Accesse May 2015.

Updated 01/2017 Page 2 of 2

Applies to: Nebraska Supplemental PDL Sivextro (tedizolid)

Sivextro (tedizolid)

Covered uses All medically accepted indications Exclusion Criteria Required Medical 1. Recent (within 30 days of request) Culture and Sensitivity Information report; AND 2. Documentation of an Acute Baterial Skin and Skin Structure Infection (listed numerically below); AND

3. Complicated skin / skin structure infections including diabetic foot infections, without osteomyelitis caused by MRSA: Trial and failure of two (2) preferred alternatives or resistance to all other appropriate therapies: Doxycycline, Trimethoprim-sulfamethoxazole

4. Uncomplicated SSI caused by MRSA documented by C/S report or empirical treatment of patients with uncomplicated or community-acquired complicated SSI without osteomyelitis where MRSA infection is likely: Trial and failure of two (2) preferred alternatives or resistance to all other appropriate therapies: Trimethoprim-sulfamethoxazole, Dicloxacillin, Doxycycline, Minocycline, Clindamycin.

5. Uncomplicated or complicated SSI without osteomyelitis caused by MSSA, Streptococcus pyogenes, or Streptococcus agalactiae (complicated SSI only), Streptococcus anginosus, and Enterococcus faecalis: Trial and failure of two (2) preferred alternatives or resistance to all other appropriate therapies: Amoxicillin/clavulanic acid, Cephalexin, Ciprofloxacin, Clindamycin, levofloxacin, Sulfamethoxazole/Trimethoprim, Dicloxacillin.

Age restrictions 18 years of age or older

Prescriber N/A Restrictions Coverage Duration 6 days Other Criteria Recommended dosage: 200 mg orally/IV for 6 days

When culture and susceptibility information are available, they should be considered in selecting or modifying antibacterial therapy. In the absence of such data, local epidemiology and

Updated 01/2017 Page 1 of 2

Applies to: Nebraska Supplemental PDL Sivextro (tedizolid)

susceptibility patterns may contribute to the empiric selection of therapy.

References: 1. Sivextro [package insert]. Lexington, MA: Cubist Pharmaceuticals Inc. 2014.

Updated 01/2017 Page 2 of 2

Applies to: Nebraska Supplemental PDL Tobacco Cessation Products

Tobacco Cessation Products Chantix, nicotine gum, lozenges, transdermal patch, inhaler, nasal solution

Covered uses Tobacco Cessation Products - Products are only available for clients 18 years of age or older who require this assistance. Coverage is limited to 90 days supply per session and is also dependent on the client being enrolled and actively participating with the Nebraska Tobacco Free Quitline.

Exclusion Criteria Under 18 years of age Required Medical Nebraska Tobacco Free Quitline - In order to receive drug Information products, clients must be enrolled in and actively participating with the Nebraska Tobacco Free Quitline. Clients can access the line directly or by referral from their medical provider. Use of the Quitline is not limited. The Quitline number is 1-800-QUIT- NOW (1-800-784-8669).

Age and QL apply (see Nebraska Supplemental Drug List)

Age restrictions 18 years

Prescriber none Restrictions Coverage Duration Smoking Cessation Agents: 180 days supply per rolling 365 days inclusive of all products

Other Criteria

References: . Nebraska Heritage Health Tobacco Quitline

Created 12/2016 Page 1 of 1

Applies to: Strensiq (alfotase alfa) Nebraska Non-PDL

Strensiq (alfotase alfa)

Covered uses All FDA Approved Indications Exclusion Criteria None Required Medical Strensiq will be approved based on the following criteria: Information • Documented diagnosis of hypophosphatasia (juvenile-onset, perinatal or infantile-onset) AND • Genetic testing confirming alkaline phosphatase gene (ALPL) mutation AND • Laboratory results of depressed phosphatase in serum and bone AND • Presence of periodontal disease AND • Documentation of patient’s weight

Reauthorization • Radiographical evidence of improvement in disease progression OR • Improvements in height and/or weight z-score OR • Documentation by treaing physician that patient is experiencing benefit from drug AND • Documentation or claims history indicative of compliance with drug

Age restrictions Maximum age of 17 years Prescriber None Restrictions Coverage Duration Initial authorization: 2 months Reauthorization: 1 year Other Criteria Perinatal/Infantile-Onset Hypophosphatasia • Recommended regimen is 2 mg/kg administered subcutaneously three times per week, or 1 mg/kg administered six times per week. • The dose may be increased to 3 mg/kg three times per week for insufficient efficacy. Juvenile-Onset Hypophosphatasia • Recommended regimen is 2 mg/kg administered subcutaneously three times per week, or 1 mg/kg administered six times per week.

Last updated 02/2016 Page 1 of 2

Applies to: Strensiq (alfotase alfa) Nebraska Non-PDL

References:

1) Stensiq [package insert] Alexion Pharmaceuticals, Inc. Cheshire, CT. October 2015. 2) Taillandier A, Lia-Baldini AS, Mouchard M, et al. Twelve novel mutations in the tissue-nonspecific alkaline phosphatase gene (ALPL) in patients with various forms of hypophosphatasia. Hum Mutat. 2001;18(1):83-4. PubMed PMID: 11438998 3) Whyte MP, Rockman-Greenberg C, Ozono K, et al. Asfotase Alfa Treatment Improves Survival for Perinatal and Infantile Hypophosphatasia. J Clin Endocrinol Metab. 2016 Jan;101(1):334-42. doi: 10.1210/jc.2015-3462. Epub 2015 Nov 3. PubMed PMID: 26529632; PubMed Central PMCID: PMC4701846. 4) US Food and Drug Administration. FDA approves new treatment for rare metabolic disorder.http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm4688 36.htm (Accessed on October 28, 2015). 5) Hypophosphatasia. (2007, September). Retrieved February 17, 2016 from http://ghr.nlm.nih.gov/condition/hypophosphatasia

Last updated 02/2016 Page 2 of 2

Applies to: Erwinaze Nebraska Non-PDL

ERWINAZE (Asparaginase Erwinia chrysanthemi)

Covered uses All medically accepted indications

Exclusion Criteria History of serious pancreatitis, serious thrombosis, or serious hemorrhagic events with prior L-asparaginase therapy Required Medical Documentation of: Information acute lymphoblastic leukemia (ALL) AND hypersensitivity to E. coli-derived asparaginase (Oncaspar) Age restrictions Age 2 or greater Prescriber Oncologist Restrictions Coverage Duration 2 weeks Other Criteria Recommended dose of ERWINAZE when substituting for Oncaspar is 25,000 International Units/m2 administered intramuscularly three times per week (Monday/Wednesday/Friday) for six doses

Reference Erwinaze [package insert]. Jazz Pharmaceuticals, Inc. Langhorne, PA. March 2014

Last Updated 05/2014 Page 1 of 1

Applies to: Adcetris (brentuximab vedotin) Nebraska Non-PDL Adcetris (brentuximab vedotin)

Covered uses All medically approved indications

Exclusion Criteria First line treatment of Hodgkin lymphoma or systemic anaplastic large cell lymphoma Concomitant treatment with bleomycin Required Medical Stated diagnosis of: Information 1) Hodgkin lymphoma (HL) after failure of autologous stem cell transplant (ASCT) or after failure of at least 2 prior multi agent chemotherapy regimens in patients who are not ASCT candidates. 2) Systemic anaplastic large cell lymphoma (sALCL) after failure of at least 1 prior multi-agent chemotherapy regimen. Age restrictions 18 years or older Prescriber Oncologist Restrictions Coverage Duration 1 year Other Criteria Members currently taking this medication at the time of enrollment will not be required to meet prerequisites for Prior Authorization.

References Adcetris [package insert]. Seattle Genetics, Inc., Bothell, WA. August, 2013.

Last updated 05/2014 Page 1 of 1

Applies to: Ilaris (canakinumab injection) Nebraska Non-PDL Ilaris (canakinumab injection)

Covered uses All FDA approved indications

Exclusion Criteria N/A

Required Medical Ilaris will be approved based on one of the following criteria: Information 1. Patients 4 years of age or older with confirmed diagnosis of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Autoinflammatory Syndrome (FCAS) and Muckle-Wells Syndrome, Systemic onset juvenile chronic arthritis -OR- 2. Patients 2 years of age or older with active Systemic Juvenile Arthritis (SJIA) AND treatment failure or intolerance to Glucocorticoid, or Methotrexate, or Leflunomide, or Actemra, or Kineret.

Age restrictions CAPS: 4 years of age or older SJIA: 2 years of age or older Prescriber Rhuematologist Restrictions Immunologist Coverage Duration Authorization for therapy will be issued for 12 months. Other Criteria Dosage: • Cryopyrin associated periodic syndrome: (4 years or older) greater than 40 kg, 150 mg SUBQ every 8 weeks • Cryopyrin associated periodic syndrome: (4 years or older) 15 to 40 kg, 2 mg/kg SUBQ every 8 weeks; may increase dose to 3 mg/kg every 8 weeks in children with an inadequate response • Familial cold urticaria: (4 years or older) greater than 40 kg, 150 mg SUBQ every 8 weeks • Familial cold urticaria: (4 years or older) 15 to 40 kg, 2 mg/kg SUBQ every 8 weeks; may increase dose to 3 mg/kg every 8 weeks in children with an inadequate response • Muckle-Wells syndrome: (4 years or older) greater than 40 kg, 150 mg SUBQ every 8 weeks • Muckle-Wells syndrome: (4 years or older) 15 to 40 kg, 2 mg/kg SUBQ every 8 weeks; may increase dose to 3 mg/kg every 8 weeks in children with an inadequate response • Systemic onset juvenile chronic arthritis: (2 years or older) body weight 7.5 kg or greater, 4 mg/kg SUBQ every 4 weeks; MAX 300 mg/dose

Last updated 04/2015 Page 1 of 2

Applies to: Ilaris (canakinumab injection) Nebraska Non-PDL

REFERENCES

1 Arcalyst™ (rilonacept) for Subcutaneous Injection Prescribing Information. Regeneron Pharmaceuticals, April 2010. 2 Ilaris™ (canakinumab) for Subcutaneous Injection Prescribing Information. Novartis Pharmaceutical Corporation, May 2013. 3 Hoffman HM et al. Durability of response to rilonacept (IL-1 Trap) in a phase 3 study of patients with cryopyrin-associated periodic syndromes (CAPS): familial cold autoinflammatory syndrome (FCAS) and Muckle-Wells syndrome. Journal of Allergy and Clinical Immunology. 2008; 121(2):S175. 4 Hoffman HM, Weinstein SP, Amar NJ, et al. The first placebo-controlled trial in cryopyrin¬associated periodic syndromes (CAPS): IL-1 trap (rilonacept) markedly reduces clinical and laboratory abnormalities in patients with familial cold autoinflammatory syndrome (FCAS) and Muckle-Wells syndrome (MWS) [abstract]. J Allergy Clin Immunol. 2007;119(2):524. 5 Lachmann HJ, Kone-Paut I, Kuemmerle-Deschner JB, et al. Canakinumab in CAPS Study Group. Use of canakinumab in the cryopyrin-associated periodic syndrome. N Engl J Med. 2009;360(23):2416-2425. 6 Kuemmerle-Deschner J, Blank N, Roesler J, et al. Canakinumab (ACZ885), a new IL- 1beta blocking monoclonal antibody, provides sustained remission in patients with cryopyrin associated periodic fever syndrome (CAPS): results of an open label phase II study [abstract]. Ann Rheum Dis. 2009;68(suppl 3):366. 7 Kuemmerle-Deschner J, Blank N, Roesler J, et al. Canakinumab (ACZ885), a new IL- 1beta blocking monoclonal antibody provides sustained remission in children with cryopyrin associated periodic fever syndrome (CAPS) [abstract]. Ann Rheum Dis. 2009;68(suppl 3):503. 8. AHFS. AHFS Drug Information 2008. Bethesda, MD: American Society of Health- System Pharmacists; 2008. 9 Ringold S, Weiss PF, Beukelman T, DeWitt EM, Ilowite NT, Kimura Y, Laxer RM, Lovell DJ, Nigrovic PA, Robinson AB, Vehe RK, American College of Rheumatology. 2013 update of the 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis: recommendations for the medical therapy of children with systemic juvenile idiopathic arthritis and tuberculosis screening among children receiving biologic medications. Arthritis Rheum. 2013 Oct;65(10):2499-512.

Last updated 04/2015 Page 2 of 2

Applies to: Brineura (cerliponase alfa) Nebraska Non-PDL

Brineura (cerliponase alfa)

Covered Uses All FDA approved indications

Exclusion Criteria Acute intraventricular access device/port complications (leakage, device failure, device-related infections) Patients with ventriculoperitoneal shunts Required Medical • Documented diagnosis of late infantile neuronal ceroid Information lipofuscinosis type 2 (CLN2) or tripeptidyl peptidase 1 (TPP1) deficiency. Diagnosis must be confirmed by genetic testing: o TPP1 enzyme activity assay via blood test or saliva showing deficient activity in leukocytes or fibroblasts OR o PPT1 enzyme assay OR o TPP1/CLN2 molecular test showing pathogenic variants/mutations in each allele of the TPP1/CLN2 gene OR o Neuronal ceroid lipofuscinosis (NCL) gene panel showing variants/mutation in each allele of the TPP1/CLN2 gene OR o Analysis of parental DNA samples showing TPP1/CLN2 gene on separate parental alleles in trans • Patient must exhibit symptomatic disease (new onset seizures, language delay, lack of motor control) • Submission of CLN2 Clinical Rating Scale assessment with a combined Motor plus Language score of 6 or less Age restrictions 3 years of age and older

Prescriber Pediatric Neurologist Restrictions Coverage Duration 1 year Other Criteria This is an intraventricular injection that must be administered under sterile conditions, via an intraventricular access device or port, by a health care professional The recommended dose is 300mg once every other week by intraventricular infusion followed by an infusion of electrolytes

Resources: 1. Brineura [package insert] BioMarin Pharmaceuticals Inc. Novato, CA. Apr 2017 2. Fietz M, Alsayed M, Burke D, et al. Diagnosis of neuronal ceroid lipofuscinosis type 2 (CLN2 disease): Expert recommendations for early detection and laboratory diagnosis. Mol Genet Metab. 2016;119(1-2):160-7.

Created: 03/2017 Page 1 of 1

Applies to: Sensipar (cinacalcet) Nebraska Non-PDL Sensipar (cinacalcet)

Covered uses All medically accepted indications Exclusion Criteria N/A Required Medical Trial and failure of or contraindication preferred products: Information calcitriol capsule or solution AND Stated diagnosis of: • Parathyroid Carcinoma • Secondary Hyperparathyroidism • Primary Hyperparathyroidism • Persistent Secondary Hyperparathyroidism after renal transplantation

Other Criteria For Secondary Hyperparathyroidism: • Patient has a diagnosis of chronic kidney disease and is on dialysis • Patient is concurrently receiving traditional therapy with vitamin D sterols and/or phosphate binders • Patient’s serum calcium level is ≥ 8.4 mg/dl

For Primary Hyperparathyroidism: • Patient has failed, or has a contraindication to parathyroidectomy • Patient has severe hypercalcemia defined as a serum calcium level of 12.5 mg/dl or greater

For Persistent Secondary Hyperparathyroidism after renal transplantation: • Patient has had a kidney transplantation • Patient’s serum calcium level is ≥ 8.4 mg/dl

Age restrictions The patient must be at least 18 years of age

Prescriber None Restrictions Coverage Duration Authorization of therapy will be issued for 6 months for Persistent Secondary Hyperparathyroidism after renal transplantation indication. For all other indications, Authorization of therapy will be issued for 12 months. Other Criteria

Last Updated 05/2014 Page 1 of 2

Applies to: Sensipar (cinacalcet) Nebraska Non-PDL

References 1. Sensipar [package insert]. Amgen: Thousand Oaks, CA; August 2011. 2. 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: 760-71. 3. 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: 36-45. 4. Block GA, Zeig S, Sugihara J, et al. Combined therapy with cinacalcet and low doses of vitamin D sterols in patients with moderate to severe secondary hyperparathyroidism. Nephrol Dial Transplant. 2008; 23: 2311-2318. 5. Bushinsky DA, Messa P. Efficacy of early treatment with calcimimetics in combination with reduced doses of vitamin D sterols in dialysis patients. Nephrol Dial Transplant. 2008; 1 (suppl 1) i18-i23. 6. Silverberg SJ, Rubin MR, Faiman C, et al. Cinacalcet Hydrochloride Reduces the Serum Calcium Concentration in Inoperable Parathyroid Carcinoma. J Clin Endocrinol Metab. 2007; 92(10): 3803-8. 7. Shane E. Clinical Review 122: Parathyroid Carcinoma. J Clin Endocrinol Metab. 2001; 86(2): 485-93. 8. National Kidney Foundation. K/DOQI Clinical Practice Guidelines for Bone Metabolism and Disease in Chronic Kidney Disease. Am J Kidney Dis. 2003; 42:S1-S202. 9. Leca N, Laftavi M, Gundroo A, et al. Early and Severe Hyperparathyroidism Associated with Hypercalcemia After Renal Transplant Treated with Cinacalcet. Amer J Transplant. 2006; 6: 2391-2395. 10. Bergua C, Torregrosa JV, Fuster D, et al. Effect of cinacalcet on hypercalcemia and bone mineral density in renal transplanted patients with secondary hyperparathyroidism. Transplantation. 2008; 86(3): 413-7. 11. El-Amm JM, Doshi MD, Singh A, et al. Preliminary experience with cinacalcet use in persistent secondary hyperparathyroidism after kidney transplantation. Transplantation. 2007; 83(5): 546-9. 12. Marcen R, Ponte B, Rodriguez-Mendiola N, Fernandez Rodriguez A, et al. Secondary hyperparathyroidism after kidney transplantation: a cross sectional study. 2009; 41(6): 2391¬3. 13. Beruga C, Torregrosa JV, Cofan F, et al. Cinacalcet for the treatment of hypercalcemia in renal transplanted patients with secondary hyperparathyroidism. Transplant Proc. 2007; 39(7): 2254-5. 14. Gomez Marques G, Obrador Mulet A, Vilar Gimeno A, et al. Treatment with cinacalcet of secondary hyperparathyroidism after renal transplantation. Transplant Proc. 2009; 41(6): 2139-43. 15. Apostolou T, Kollia K, Damianou L, et al. Hypercalcemia due to resistant hyperparathyroidism in renal transplant patients treated with the calcimimetic agent cinacalcet. Transplant Proc. 2006; 38(10): 3514-6.

Last Updated 05/2014 Page 2 of 2

Applies to: Xiaflex (collagenase clostridium histolyticum) Nebraska Non-PDL

Xiaflex (collagenase clostridium histolyticum)

Covered uses All FDA approved indications Exclusion Criteria The treatment of Peyronie’s plaques that involve the penile urethra due to potential risk to this structure. Required Medical Medical documentation indicating use for the treatment of Information 1) adult patients with Dupuytren’s contracture with a palpable cord OR 2) adult men with Peyronie’s disease with a palpable plaque and curvature deformity of at least 30 degrees at the start of therapy.

Reauthorization criteria Dupuytren's contracture: documentation of previous treatments and indication of use for treatment of an additional palpable cord. Peyronie's Disease: documentation of previous treatment cycles, indication of persistent curvature deformity of at least 15 degrees, and indication of use for treatment of an additional plaque. Age restrictions Age 18 years or greater Prescriber For Peyronie's Disease: Xiaflex should be administered by a Restrictions healthcare provider experienced in the treatment of male urological diseases, who has completed required training for use of Xiaflex in the treatment of Peyronie’s disease. Coverage Duration Dupuytren's contracture: 12 weeks per cord Peyronie's disease: 24 weeks per plaque Other Criteria Dosage and Duration Note: a single 0.58mg injection = a single 0.9mg vial. Dupuytren's contracture: 0.58 mg per injection into a palpable cord. May perform 2 injections in same hand (2 cords) per visit. If needed, may repeat treatment every 4 weeks, up to 3 injections per cord. Maximum of 2 vials every 4 weeks, for up to a total of 12 weeks per cord. Peyronie's disease: 0.58 mg per injection for 2 injections, separated by 1 to 3 days, followed by penile modeling procedure 1 to 3 days after 2nd injection. If needed, may repeat treatment cycle every 6 weeks, up to 4 treatment cycles (8 injections) per plaque. If after the first, second, or third treatment cycle, curvature deformity is less than 15 degrees or further treatment is not clinically indicated, subsequent treatment cycles should not be administered. Maximum of 2 vials every 6 weeks, for up to a total of 24 weeks.

Created 02/2017 Page 1 of 2

Applies to: Xiaflex (collagenase clostridium histolyticum) Nebraska Non-PDL

REFERENCES

1. XIAFLEX [package insert]. Auxilium Pharmaceuticals, Inc. Malvern, PA. August 2016 2. National Guideline Clearinghouse (NGC). Guideline summary: Peyronie's disease: AUA guideline. In: National Guideline Clearinghouse (NGC) [Web site]. Rockville (MD): Agency for Healthcare Research and Quality (AHRQ); 2015 Apr 01. [cited 2016 Nov 16]. Available: https://www.guideline.gov

Created 02/2017 Page 2 of 2

Applies to: H.P. Acthar Gel (Corticotropin) Nebraska Non-PDL

H.P. Acthar Gel (Corticotropin)

Covered uses All FDA-approved indications. 1) Monotherapy for the short-term treatment of infantile spasms (West syndrome) in infants and children under 2 years of age 2) Treatment of acute exacerbations of multiple sclerosis in adults. 3) Rheumatic Disorders a. Psoriatic arthritis b. Rheumatoid arthritis c. Ankylosing spondylitis 4) Collagen diseases a. Systemic lupus erythematosus b. Systemic dermatomyositis (polymyositis) 5) Dermatologic diseases a. Severe erythema multiforme b. Stevens-Johnson syndrome 6) Allergic states a. Serum sickness 7) Ophthalmic diseases a. Severe acute and chronic allergic and inflammatory processes involving the eye & its adnexa 8) Respiratory Diseases a. Symptomatic sarcoidosis 9) Edematous State a. Proteinuria in the nephrotic syndrome without uremia of the idiopathic type or that due to lupus erythematosus

Exclusion 1) Intravenous administration Criteria 2) Suspected congenital infections in infants 3) Administration of live or live attenuated vaccines in patients receiving immunosuppressive doses of H.P. Acthar gel. 4) H.P. Acthar gel is contraindicated in patients with: i. Scleroderma ii. Osteoporosis iii. Systemic fungal infections iv. Ocular herpes simplex v. Recent surgery vi. History of or the presence of a peptic ulcer vii. Congestive heart failure viii. Uncontrolled hypertension ix. Primary adrenocortical insufficiency x. Adrenocortical hyper-function or sensitivity to proteins of porcine origin

Required Criteria for approval for infantile spasms (West syndrome) Medical • Baseline 24 hour EEG displaying hypsarrythmia AND

Last Updated 07/2016 Page 1 of 5

Applies to: H.P. Acthar Gel (Corticotropin) Nebraska Non-PDL

Information • Age less than two AND • Documentation of current height and weight AND • Documentation of 2-week taper schedule

*Reauthorization for infantile spasms will require documentation of persistent hypsarrythmia on 24 hour EEG.

Criteria for approval for acute exacerbation of multiple sclerosis for adults • Documentation of the following: o FDA approved diagnosis AND o Age greater than or equal to 18 years old AND o Denotation of acute exacerbation lasting at least 24 hours AND o Indication that other exacerbation causes such as stress, pain, premenstrual syndrome, or infection have been ruled out AND • Immunomodulator use for at least the past 30 days unless adequate documentation reflects contraindication (such as Aubagio, Avonex, Glatiramer acetate, Betaseron, Rebif, Gilenya, and Tysabril) AND • Documented trial & failure of methylprednisolone 160mg daily for one week followed by 64mg every other day for one month OR • Medical reason indicating why the patient cannot use a formulary corticosteroid (IV methylprednisolone, IV dexamethasone, oral prednisolone, etc.) AND • Documentation of taper schedule

Criteria for approval for proteinuria in nephrotic syndrome: • Documented FDA approved diagnosis AND • Must have trial & failure or intolerance of at least 1 from the following categories (unless adequate documentation reflects contraindication): o Immunosuppressive agents: cyclophosphamide, mycophenolate mofetil, tacrolimus, or cyclosporine AND o Corticosteroid: Prednisolone, Dexamethasone IV AND o Methylprednisolone IM injection

Criteria for approval for Polymyositis/Dermatomyositis • Documented FDA approved diagnosis AND • Age greater than or equal to 18 years AND • Must have trial & failure or intolerance of at least 1 from each of the following groups (unless adequate

Last Updated 07/2016 Page 2 of 5

Applies to: H.P. Acthar Gel (Corticotropin) Nebraska Non-PDL

documentation reflects contraindication): o Corticosteroid: Prednisolone, Dexamethasone IV AND o Antimetabolite: azathioprine, methotrexate AND o Methylprednisolone IV

Criteria for approval for Rheumatoid Arthritis • Documented FDA approved diagnosis (including juvenile rheumatoid arthritis) AND • Age greater than or equal to18 years AND • Must have trial & failure or intolerance of at least 1 from each of the following groups (unless adequate documentation reflects contraindication): o Corticosteroid: Prednisolone, Dexamethasone IV AND o Non-biologic disease-modifying antirhuematic: azathioprine, methotrexate AND o Biologic disease-modifying antirheumatic: Humira, Enbrel AND o Methylprednisolone IM injection between 40 to 120mg or IV

Criteria for approval of Systemic Lupus Erythematosus • Documented FDA approved diagnosis AND • Must have trial & failure or intolerance of at least 1 from each of the following groups (unless adequate documentation reflects contraindication): o Corticosteroid: Prednisolone, Dexamethasone IV AND o Hydroxychloroquine, Benylsta, azathioprine, cyclosporine, cyclophosphamide, mycophenalate AND o Methylprednisolone IV

Criteria for approval of Ophthalmic Diseases • Documented FDA approved diagnosis (keratits, iritis, iridocylitis, diffuse posterior uveitis and choroidits, optic neuritis, chorioretinitis, and anterior segment inflammation) AND • Must have trial & failure or intolerance of a corticosteroid (such as oral Prednisolone, or Dexamethasone IV) AND • History of or concomitant use of: o A non-biologic disease-modifying anti-rheumatic drug (such as methotrexate or mycophenolic acid) AND

Last Updated 07/2016 Page 3 of 5

Applies to: H.P. Acthar Gel (Corticotropin) Nebraska Non-PDL

o A biologic disease-modifying anti-rheumatic drug (such as adalimumab) Criteria for approval of Sarcoidosis: • Documented FDA approved diagnosis AND • Disease severity must be denoted AND • Must have trial & failure or intolerance of at least 1 from each of the following groups (unless adequate documentation reflects contraindication): o Corticosteroid: IV methylprednisolone, IV dexamethasone, oral prednisone AND o Immunosuppressant: methotrexate, azathioprine

Criteria for approval for all other indications • Documented FDA approved diagnosis AND • Age greater than 18 years old AND • Documentation of taper schedule o Short courses of treatment may not require taper. Treatment is individualized based on severity of patient’s condition and expected response. Age Must be under 2 years of age for patients diagnosed with infantile spasms restrictions Must be at least 18 years of age for patients diagnosed with exacerbation of multiple sclerosis

Prescriber Specialist in the specific field of study Restrictions Coverage 1 month for all indications Duration Other Criteria • Recommended dosing for infantile spasms: 150 U/m2 (divided into twice daily intramuscular injections of 75 U/m2) administered over a 2 week period. Dosing should then be gradually tapered over a 2-week period to avoid adrenal insufficiency. One suggested tapering schedule: 30 U/m2 in the morning for 3 days; 15 U/m2 in the morning for 3 days; 10 U/m2 in the morning for 3 days; and10 U/m2 every other morning for 6 days.

( )× ( ) BSA (m2): 𝑤𝑤𝑤𝑤𝑤𝑤𝑤𝑤ℎ𝑡𝑡 𝑘𝑘𝑘𝑘 ℎ𝑒𝑒𝑒𝑒𝑒𝑒ℎ𝑡𝑡 𝑐𝑐𝑐𝑐 √ 3600 • Recommended dosing for the treatment of acute exacerbations of multiple sclerosis in adults: 80 – 120 units injected intramuscularly or subcutaneously daily for 2-3 weeks.

• Recommended dosing for other indications for adults and children over 2 years of age:

Last Updated 07/2016 Page 4 of 5

Applies to: H.P. Acthar Gel (Corticotropin) Nebraska Non-PDL

40 – 80 units given intramuscularly or subcutaneously every 24 – 72 hours

References: 1. Arnadottir M, Hultberg B, Berg AL. Plasma total homocysteine concentration in nephrotic patients with idiopathic membranous nephropathy. Nephrol Dial Transplant. 2001;16:45-47. 2. Baram TZ, Mitchell WG, Tournay A, Snead OC, Hanson RA, Horton EJ. High-dose corticotropin (ACTH) versus prednisone for infantile spasms: a prospective, randomized, blinded study. Pediatrics. 1996;97:375-379. 3. Brombeck AS, Canetta PA, Beck LH, Ayalon R, Radhakrishnan J, Appel GB. Treatment of resistant glomerular diseases with adrenocorticotropic hormone gel: a prospective trial. Am J Nephrol. 2012;36:58-67. 4. Brombeck AS, Tumlin JA, Baranski J, Bourdeau JE, Besarab A, Appel AS, Radhakrishnan J, Appel GB. Treatment of nephrotic syndrome with adrenocorticotropic hormone (ACTH) gel. Drug Des Devel Ther. 2011;5:147-153. 5. H.P. Acthar Gel [package insert and medication guide]. Hayward, CA: Questcor Pharmaceuticals, Inc.; Jun 2011. 6. Hrachovy RA, Frost JD, Jr., Glaze DG. High-dose, long duration versus low-dose, short duration corticotropin therapy for infantile spasms. J Pediatr. 1994;124:803-806. 7. Ponticelli C, Passerini P, Salvadori M, Manno C, Viola BF, Pasquali S, Mandolfo S, Messa P. A randomized pilot trial comparing methylprednisolone plus a cytotoxic agent versus synthetic adrenocorticotropic hormone in idiopathic membranous nephropathy. Am J Kidney Dis. 2006;47:233-240. 8. Simsarian JP, Saunders C, Smith DM. Five-day regimen of intramuscular or subcutaneous self-administered adrenocorticotropic hormone gel for acute exacerbations of multiple sclerosis: a prospective, randomized, open-label pilot trial. Drug Des Devel Ther. 2011;5:381-389.

Last Updated 07/2016 Page 5 of 5

Applies to: Cystaran Nebraska Non-PDL

Cystaran ophthalmic solution 0.44% (Cysteamine)

Covered uses All medically accepted indications

Exclusion Criteria Required Medical • Diagnosis of cystinosis, corneal cysteine crystal Information accumulation

Age restrictions

Prescriber • Nephrologist Restrictions • Ophthalmologist

Coverage 12 months Duration Other Criteria

REFERENCES

1. Cystaran [package insert]. Gaithersburg, MD: Sigma-Tau Pharmaceuticals Inc; October 2013.

Last updated 04/2015 Page 1 of 1

Applies to: Procysbi (cysteamine bitartrate) Nebraska Non-PDL

Procysbi (cysteamine bitartrate delayed release capsule)

Covered uses All FDA-approved indications Exclusion Criteria Members who are hypersensitive to penicillamine Required Medical Initial authorization Information • Documented diagnosis of nephropathic cystinosis based on leukocyte cysteine concentration assay or DNA testing • Documented trial and failure of Cystagon • Height and weight (required to estimate body surface area) Re-authorization requires the following: • Clinical benefit of Procysbi therapy is documented

Age restrictions Minimum of 2 years of age Prescriber Nephrologist Restrictions Coverage Duration Initial authorization of therapy will be issued for 3 months. Re-authorization of therapy will be issued for 12 months. Other Criteria Recommended maintenance dose is 1.3 gram/m2/day given in two divided doses every 12 hours. Requested dose should not exceed this.

Approximation of 1.3 gram/m2/day dose of Procysbi

Last updated 02/2017 Page 1 of 2

Applies to: Procysbi (cysteamine bitartrate) Nebraska Non-PDL

References: 1) PROCYSBI [package insert] Raptor Therapeutics, Inc. Novato, CA. April 2013.

Last updated 02/2017 Page 2 of 2

Applies to: Ferriprox (deferiprone) Nebraska Non-PDL

Ferriprox (deferiprone)

Covered uses All medically accepted indications

Exclusion Criteria

Required Medical Initial Authorization Information 1. Ferriprox will be approved based on all of the following criteria: a. The following diagnosis: transfusional iron overload due to thalassemia -AND b. Patient has serum ferritin levels consistently >1000 mcg/L prior to initiation of treatment with Ferriprox -AND c. Current chelation therapy is inadequate [eg, Desferal (deferoxamine) and Exjade (deferasirox)] -AND d. Prescribed by a hematologist/oncologist

Authorization will be issued for 12 months.

Reauthorization 1. Ferriprox will be approved based on the following criterion: a. Documentation of positive clinical response to Ferriprox therapy Authorization will be issued for 12 months.

Age restrictions

Prescriber Restrictions

Coverage Duration 12 months Other Criteria DOSING

Ferriprox tablets

Iron Overload: The recommended initial dose of deferiprone is 25 mg/kg, orally, three times per day for a total of 75 mg/kg/day. The maximum dose is 33 mg/kg, three times per day for a total of 99 mg/kg/day. Dose adjustments up to 33 mg/kg, orally, three times per day should be tailored to the individual patient’s response and therapeutic goals (maintenance or reduction of body iron burden).

Last updated 05/2014 Page 1 of 2

Applies to: Ferriprox (deferiprone) Nebraska Non-PDL

The maximum recommended total daily dose is 99 mg/kg/day. The dose should be rounded by the prescriber to the nearest 250 mg (half-tablet). Serum ferritin concentrations should be monitored every 2 to 3 months to assess the effects of deferiprone on body iron stores. Dose adjustments should be tailored to the individual patient’s response and therapeutic goals (maintenance or reduction of body iron burden). If the serum ferritin falls consistently below 500 µg/L, temporarily interrupting deferiprone should be considered.

REFERENCES

1 Ho PJ, Tay L, Linderman R, Catley L, Bowden DK. Australian guidelines for the assessment of iron overload and iron chelation in transfusion-dependent thalassaemia major, sickle cell disease and other congenital anaemias. Intern Med J. 2011;41(7):516-24. 2 Angelucci E, Barosi G, Camaschella C, et al. Italian Society of Hematology practice guidelines for the management of iron overload in thalassemia major and related disorders. Haematologica. 2008;93(5):741-52. 4. United Kingdom Thalassemia Society. Standards for the clinical care of children and adults with thalassemia in the UK. 2008. Available at http://www.ukts.org/pdfs/awareness/standards/ukts-standards-2008.pdf. Accessed December 27, 2012. 5 Nursing Practice Guidelines: Care of the Patient with Sickle Cell Disease and Iron Overlead. 2008 International Association of Sickle Cell Nurses and Physician Assistants. Available at: http://www.iascnapa.org/guidelines/Guidelines_IronOverload.pdf. Accessed on December 28, 2012. 6 Cavill I. Iron status as measured by serum ferritin: the marker and its limitations. Am J Kidney Dis. 1999 Oct;34(4 Suppl 2):S12-7. 7 Porter JB and Shah FT. Iron overload in thalassemia and related conditions: therapeutic goals and assessment of response to chelation therapies. 2010 Dec;24(6):1109-30 8 Ferriprox Prescribing Information. ApoPharma USA, Inc., April 2012.

Last updated 05/2014 Page 2 of 2

Applies to: Emflaza (deflazacort) Nebraska Non-PDL

Emflaza (deflazacort oral tablet and suspension)

Covered uses All FDA-approved indications Exclusion Criteria Patients taking rifampin, carbamazepine, phenytoin, efavirenz, moderate or strong CYPA4 inducers or other prescribed products known to significantly decrease deflazacort plasma concentrations Required Medical Initial authorization: Information • Documented diagnosis of Duchenne muscular dystrophy including: o Presence of abnormal dystrophin OR mutation of the dystrophin gene o Serum creatinine kinase activity of at least 10x the upper limit of normal (ULN) o Baseline muscle strength score from the 6-minute walk test • Documentation of prednisone trial (at least 0.75 mg/kg/day or 10 mg/kg/weekend) for at least 6 months AND experienced inadequate treatment response, intolerance, or contraindication Re-authorization: • Muscle strength score from the 6-minute walk test demonstrating clinical benefit of Emflaza Age restrictions 5 years of age and older Prescriber Neurologist who specializes in the treatment of Duchenne Restrictions Muscular Dystrophy Coverage Duration Initial authorization of therapy will be issued for 6 months. Re-authorization of therapy will be issued for 12 months. Other Criteria Recommended dosing is 0.9 mg/kg/day

References: 1.) EMFLAZA [package insert] Marathon Pharmaceuticals, LLC. Northbrook, IL. February 2017. 2.) Gloss D, Moxley RT, Ashwal S, Oskoui M. 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(5):465-72. 3.) Griggs, Robert C. et al. “Efficacy and Safety of Deflazacort vs Prednisone and Placebo for Duchenne Muscular Dystrophy.” Neurology 87.20 (2016): 2123– 2131. PMC. Web. 6 Apr. 2017.

Created 05/2017 Page 1 of 1

Applies to: Xgeva (denosumab) Nebraska Non-PDL

Xgeva (denosumab)

Covered uses All medically accepted indications

Exclusion Criteria Required Medical Medical documentation indicating diagnosis and meet the following Information criteria: 1) Bone metastasis, associated with solid tumors, prophylaxis 2) Giant cell tumor of bone, unresectable or where surgical resection is likely result is severe morbidity

Prior to initiation of therapy hypocalcemia must be corrected. 1Age restrictions 18 years or older except in adolescent patients who are mature skeletally and have giant cell tumor of bone

Prescriber Restrictions Coverage Through the benefit year Duration Other Criteria Dosing will be approved for the following: Bone metastases: 120 mg subcutaneously every 4 weeks Giant cell tumor of bone: 120 mg subcutaneously every 4 weeks with additional 120 mg doses on days 8 and 15 on the first month of therapy

Members who are taking this medication at the time of enrollment will not be required to meet the criteria for prior authorization.

References: 1. Product Information: Xgeva(R) subcutaneous injection, denosumab subcutaneous injection. Amgen Inc. (per FDA), Thousand Oaks, CA, 2013.

Updated 05/2014 Page 1 of 1

Applies to: DDAVP injectable (desmopressin) Nebraska Non-PDL

DDAVP injectable (desmopressin)

Covered uses All medically accepted indications

Exclusion Criteria Treatment in patients who have factor VIII antibodies, factor VIII coagulant activity levels equal to or less than 5%, or who have hemophilia B Required Medical Documented diagnosis of one of the following: Information 1) Central (cranial)diabetes insipidus and for the management of temporary polyuria and polydipsia (nasal solution, rhinal tube solution, injectable, tablet) 2) Hemophilia A with factor VIII coagulant activity levels greater than 5% (nasal solution, injectable) 3) von Willebrand’s disease Type I with factor VIII levels greater than 5% (nasal solution, rhinal tube solution, injectable) 4) Primary nocturnal enuresis (PNE) (tablets)

Age restrictions Age 3 months old or greater: injection von Willebrand’s, Hemophilia), nasal solution and rhinal tube (Diabetes insipidus) Age 11 months or greater: nasal solution (von Willebrand’s, Hemophilia) Age 4 years or greater: tablets (Diabetes insipidus) Age 6 years or greater: tablets (Nocturnal enuresis) Age 12 years or and greater: injection (Diabetes insipidus)

Prescriber Restrictions Coverage Duration 1 year Other Criteria Preferred Alternative(s): desmopressin tablets desmopressin nasal solution

References: 1. Product Information: DDAVP(R) injection, desmopressin acetate injection. Sanofi-Aventis US LLC, Bridgewater, NJ, 2007. 2. Product Information: DDAVP(R) nasal spray, desmopressin acetate nasal spray. Sanofi-Aventis US LLC, Bridgewater, NJ, 2007. 3. Product Information: DDAVP(R) rhinal tube, desmopressin acetate rhinal tube. Sanofi-Aventis US LLC, Bridgewater, NJ, 2007. 4. Product Information: DDAVP(R) oral tablets, desmopressin acetate oral tablets. Sanofi-Aventis US LLC, Bridgewater, NJ, 2007.

Last updated 04/2015 Page 1 of 1

Applies to: Radicava (edaravone) Nebraska Non-PDL

Radicava (edaravone)

Covered Uses All FDA approved indications Exclusion Criteria N/A Required Medical Initial Authorization Information • Documented diagnosis of amyotrophic lateral sclerosis (ALS, Lou Gehrig’s Disease) o Confirmed based on El Escorial criteria: . Signs of degeneration of lower motor neurons, which are in the spinal cord and brainstem, by clinical examination or specialized testing AND . Signs of degeneration of upper motor neurons, which are in the brain, by clinical examination AND . Progressive spread of signs within a region to other regions AND . The absence of evidence of other disease processes that might explain the observed clinical and electrophysiological signs. • Normal respiratory function: FVC ≥80% • Disease duration of 2 years or less • Retention of most activities of daily living – defined as scores of 2 or more points on each individual item of the ALS Functional Rating Scale-Revised (ALSFRS-R) • Will be used concurrently with riluzole Reauthorization • Physician statement of clinical improvement in symptoms Age restrictions 18 years of age and older

Prescriber Neurologist Restrictions Coverage Duration Initial authorization: 6 months Reauthorization: 12 months Other Criteria Dosing: • An initial treatment cycle with daily dosing for 14 days, followed by a 14-day drug-free period (Cycle 1) • Subsequent treatment cycles with daily dosing for 10 days out of 14-day periods, followed by 14-day drug-free periods (Cycles 2-6)

Resources: 1. Radicava [package insert]. MT Pharma America, Inc. Jersey City, NJ. May 2017

Created: 05/2017 Page 1 of 2

Applies to: Radicava (edaravone) Nebraska Non-PDL

2. Cedarbaum JM, Stambler N, Malta E, et al. The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci. 1999;169(1-2):13-21.

3. Abe K, Itoyama Y, Sobue G, et al. Confirmatory double-blind, parallel-group, placebo- controlled study of efficacy and safety of edaravone (MCI-186) in amyotrophic lateral sclerosis patients. Amyotroph Lateral Scler Frontotemporal Degener. 2014;15(7-8):610- 7. 4. Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial. Lancet Neurol. 2017;

Created: 05/2017 Page 2 of 2

Applies to: Invanz (ertapenem) Nebraska Non-PDL

Invanz (ertapenem)

Covered uses All medically accepted indications Exclusion Criteria Invanz will not be approved for the treatment of hospital-acquired or ventilator-associated pneumonia or infections caused by Pseudomonas spp. Required Medical 1. Recent (within 30 days of request) Culture and Information Sensitivity report; AND 2. Documentation of 1 of the following medically accepted indication with appropriate trial of alternative treatment

Community acquired bacterial pneumonia (caused Streptococcus pneumoniae (penicillin-susceptible isolates), including cases with concurrent bacteremia, Haemophilus influenzae (beta-lactamase negative isolates), or Moraxella catarrhalis: Trial and failure of two (2) preferred alternatives or resistance to all other appropriate therapies: Amoxicillin/clavulanic acid, Azithromycin, Cephalexin, Clarithromycin, Dicloxacillin, Doxycycline, Levofloxacin, Trimethoprim-sulfamethoxazole

Complicated skin / skin structure infections, including diabetic foot infections without osteomyelitis (caused by Staphylococcus aureus (methicillin susceptible isolates only), Streptococcus agalactiae, Streptococcus pyogenes, Escherichia coli, Klebsiella pneumoniae, Proteus mirabilis, Bacteroides fragilis, Peptostreptococcus species, Porphyromonas asaccharolytica, or Prevotella bivia.): Trial and failure of two (2) preferred alternatives or resistance to all other appropriate therapies: Doxycycline, Trimethoprim-sulfamethoxazole, Dicloxacillin, Minocycline, Clindamycin, Ampicillin, Amoxicillin/clavulanic acid, Cephalexin, Ciprofloxacin , Clindamycin, Levofloxacin

Complicated intra-abdominal infections (caused by Escherichia coli, Clostridium clostridioforme, Eubacterium lentum, Peptostreptococcus species, Bacteroides fragilis, Bacteroides distasonis, Bacteroides ovatus, Bacteroides thetaiotaomicron, or Bacteroides uniformis): Trial and failure of two(2) preferred alternatives or resistance to all other appropriate therapies: Ampicillin, Doxycycline, Trimethoprim- sulfamethoxazole, Dicloxacillin, Minocycline, Clindamycin, Amoxicillin/clavulanic acid, Cephalexin, Ciprofloxacin,

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Clindamycin, Levofloxacin

Complicated urinary tract infections (including pyelonephritis) (caused by Escherichia coli, including cases with concurrent bacteremia, or Klebsiella pneumoniae): Trial and failure of two (2) alternatives or resistance to all other appropriate therapies: Ampicillin, Amoxicillin/clavulanic acid, Doxycycline, Cephalexin, Cefepime, Ciprofloxacin, Levofloxacin, Trimethoprim-Sulfamethoxazole

Acute pelvic infections including (postpartum endomyometritis, septic absorption, and post-surgical gynecologic infection) (Caused by Streptococcus agalactiae, Escherichia coli, Bacteroides fragilis, Porphyromonas asaccharolytica, Peptostreptococcus species, or Prevotella bivia.): Trial and failure of two (2) alternatives or resistance to all other appropriate therapies: Ampicillin, Amoxicillin/clavulanic acid, Cephalexin, Ciprofloxacin, Levofloxacin, Clindamycin, Trimethoprim-Sulfamethoxazole, Doxycycline

Age restrictions Age 3 months or greater Prescriber Infectious Disease Specialist Restrictions Coverage Duration Maximum 14 days depending on indication Other Criteria Dosing must be in accordance with FDA approved labeling.

The concomitant use of ertapenem and valproic acid/divalproex sodium is generally not recommended.

References: 1. Invanz [package insert]. Whitehouse Station, NJ: Merck & Co., Inc.; 2011.

Last updated 11/2016 Page 2 of 2

Applies to: Exondys 51 (eteplirsen) Nebraska Non-PDL

Exondys 51 (eteplirsen)

Covered Uses All FDA approved indications

Exclusion Criteria Female patients Exondys 51 will not be covered for other forms of muscular dystrophy Required Medical Exondys will be approved based on the following criteria: Information Initial authorization: • Diagnosis of Duchenne Muscular Dystrophy (DMD) AND • Submission of laboratory results confirming mutation of the DMD gene that is amenable to exon 51 skipping documented by: o Multiplex ligation-dependent probe amplification (MLPA), OR o Array comparative genomic hybridization (array CGH) OR o DMD gene sequencing AND • Patient must be ambulatory (able to walk, not wheelchair dependent) AND • Submission of baseline six minute walk test (6MWT) of ≥300 meters AND • Submission of baseline BUN (blood urea nitrogen), SCr (serum creatinine), and urinalysis showing no proteinuria AND • Documentation of forced vital capacity (FVC) ≥30% AND • Documentation of prednisone trial (at least 0.75 mg/kg/day or 10 mg/kg/weekend) for at least 6 months AND experienced inadequate treatment response, intolerance, or contraindication • Prescribed dosing is no more than 30 mg/kg once weekly Reauthorization: • Patient continues to be ambulatory (able to walk) • Prescribed dosing is no more than 30 mg/kg once weekly Age restrictions Eteplirsen has been initiated in childhood (before 14 years of age)

Prescriber Neurologist who specializes in the treatment of Duchenne Muscular Restrictions Dystrophy Coverage Duration Initial authorization: 4 weeks Reauthorization: 4 months Other Criteria N/A

Resources: 1. Exondys 51 (eteplirsen) [package insert]. Sarepta Therapeutics. Cambridge, MA. Sept. 2016 2. Lim KR, Maruyama R, Yokota T. Eteplirsen in the treatment of Duchenne muscular dystrophy. Drug Des Devel Ther. 2017 Feb 28;11:533-545. doi: 10.2147/DDDT.S97635. eCollection 2017 Feb 28. Review. PubMed PMID: 28280301; PubMed Central PMCID: PMC5338848.

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Applies to: Exondys 51 (eteplirsen) Nebraska Non-PDL 3. Mendell JR, Rodino-Klapac LR, et.al. Eteplirsen for the treatment of Duchenne muscular dystrophy. Ann Neurol. 2013 Nov;74(5):637-47. doi: 10.1002/ana.23982. Epub 2013 Sep 10. PubMed PMID: 23907995.

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Applies to: Gaucher Disease Agents Nebraska Non-PDL

Agents used for Gaucher Disease (Vpriv, Cerezyme, Elelyso, Cerdelga, Zavesca)

Covered uses All FDA approved indications Exclusion Criteria N/A Required Medical 1. Medical diagnosis of Type 1 Gaucher disease Information 2. Enzyme replacement IV therapy (Vpriv, Cerezyme, and Elelyso) should be used before oral inhibitor therapy

For Cerdelga capsule or Zavesca capsule: a. Trial and failure of Vpriv, Cerezyme, or Elelyso OR b. Allergy, hypersensitivity, or poor venous access that limits the use of intravenous enzyme replacement therapies

Age restrictions N/A Prescriber Hematologist or neurologist Restrictions Coverage Duration 1 year Other Criteria

References: Vpriv [package insert]. Lexington, MA. Shire US Manufacturing. November 2013 Elelyso [package insert]. New York, New York. Pfizer laboratories. September 2014 Cerezyme [package insert]. Cambridge, MA. Genzyme Corporation. December 2012 Zavesca [package insert]. San Francisco, CA. Actelion Pharmaceuticals. February 2014 Cerdelga [package insert]. Waterford, Ireland. Genzyme Corporation. August 2014

Last updated 03/2015 Page 1 of 1

Applies to: Lupron (leuprolide acetate) Nebraska Non-PDL Lupron, Lupron Depot, Lupron Depot-PED (leuprolide acetate)

Covered uses All medically accepted indications, Recurrent Ovarian Cancer Exclusion Criteria None Required Medical Stated diagnosis of: Information • Central Precocious Puberty • Advanced prostatic cancer (palliative); must have tried and failed or have intolerance/contraindication to preferred agent Trelstar • Endometriosis: One of the following: o The patient did not exhibit an adequate response to a trial (at least 12 weeks) of treatment OR the patient experienced an intolerance/adverse reaction to previous therapy OR has a contraindication with one of the following: . Norethindrone acetate; OR . Progesterone; OR . Danazol; OR . An oral contraceptive • Anemia, Uterine leiomyomata (fibroids), preoperatively with iron therapy • Recurrent Ovarian Cancer o Defined as progression after two consecutive chemotherapy regimens without ever sustaining a clinical benefit, OR o Disease recurs in less than 6 months (platinum resistant) OR o Patient cannot tolerate or has been unsuccessful with cytotoxic regimens Age restrictions • For Central Precocious Puberty: Patient must be less than 11 years old if female, 12 years if male • For Endometriosis and Uterine Leiomyomata (fibroids): Patient must be 18 years or older Prescriber None Restrictions Coverage Duration • Authorization will be issued for 12 months for Advanced Prostatic Cancer, Central Precocious Puberty, and Recurrent Ovarian Cancer indications • Initial authorization for Endometriosis will be issued for 6 months; re-authorization will be issued for 6 months • Initial authorization for Uterine leiomyomata will be issued for 3 months; re-authorization will be issued for 3 months Other Criteria

Last Updated 04/2015 Page 1 of 3

Applies to: Lupron (leuprolide acetate) Nebraska Non-PDL

For Uterine leiomyomata • Re-authorization will be approved based on the following: o The patient has received less than 6 months of therapy with Lupron or Lupron Depot AND o The patient has not experienced side effects or adverse reactions to Lupron or Lupron Depot AND o The patient has had a bone density scan that was within normal limits. • Re-authorization for all other indications will approved based on the following: o The patient has received less than 12 months of therapy with Lupron or Lupron Depot AND o The patient has not experienced side effects or adverse reactions to Lupron or Lupron Depot AND o The patient has had a bone density scan that was with in normal limits.

References 1. Lupron DepotTM Prescribing Information. Abbott Laboratories, July 2010. 2. Lupron Depot-PED TM Prescribing Information. Abbott Laboratories, September 2010. 3. American Fertility Society. Revised American Fertility Society classification of endometriosis: 1985. Fertil Steril. 1985;43:351-352. 4. Balasch J, Creus M, Fábregues F, et al. Visible and nonvisible endometriosis at laparoscopy in fertile and infertile women and in patients with chronic pelvic pain: a prospective study. Hum Reprod. 1996;11:387-391. 5. Carter JE. Combined hysteroscopic and laparoscopic findings in patients with chronic pelvic pain. J Am Assoc Gynecol Laparosc. 1994;2:43-47. 6. Cornillie FJ, Oosterlynck D, Lauweryns JM, Koninckx PR. Deeply infiltrating pelvic endometriosis: Histology and clinical significance. Fertil Steril. 1990;53:978-983. 7. Fedele L, Parazzini F, Bianchi S, Arcaini L, Candiani GB. Stage and localization of pelvic endometriosis and pain. Fertil Steril. 1990;53:155-158. 8. Green TH. Conservative surgical treatment of endometriosis. Clin Obstet Gynecol. 1966;9:293-308. 9. Howard FM. The role of laparoscopy in chronic pelvic pain: Promises and pitfalls. Obstet Gynecol Survey. 1993;48:357-387. 10. Jacobson L. Differential diagnosis of acute pelvic inflammatory disease. Am J Obstet Gynecol. 1980;138:1006-1011. 11. King PM, Myers CA, Ling FW, et al. Musculoskeletal factors in chronic pelvic pain. J Psychosom Obstet Gynecol. 1991;12:87-98. 12. Koninckx PR, Lessaffre E, Meuleman C, Cornillie FJ, Demeyere S. Suggestive evidence that pelvic endometriosis is a progressive disease whereas deeply infiltrating endometriosis is associated with pelvic pain. Fertil Steril. 1991;55:759-765.

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Applies to: Lupron (leuprolide acetate) Nebraska Non-PDL

13. Lee NC, Dicker RC, Rubin GL, Ory HW. Confirmation of the preoperative diagnosis for hysterectomy. Am J Obstet Gynecol. 1984;150:283-287. 14. Levitan Z, Eibschitz I, Devries K. The value of laparoscopy in women with chronic pelvic pain and a “normal pelvis.” Int J Gynecol Obstet. 1985;23:71-74. 15. Martin DC, Hubert GD, Vander Zwaag R, el-Zeky FA. Laparoscopic appearances of peritoneal endometriosis. Fertil Steril. 1989;51:63-67. 16. Mathias SD, Kuppermann M, Lieberman RF, Lipschutz RC, Steege JF. Chronic pelvic pain: prevalence, health-related quality of life, and economic correlates. Obstet Gynecol. 1996;87:321-327. 17. Sutton C, Hill D. Laser laparoscopy in the treatment of endometriosis. A 5-year study. Br J Obstet Gynaecol. 1990;97:181-185. 18. Waller KG, Shaw RW. Gonadotropin-releasing hormone analogues for the treatment of endometriosis: Long-term follow-up. Fertil Steril. 1993;59:511-515. 19. Wheeler JM, Malinak LR. Recurrent endometriosis: incidence, management, and prognosis. Am J Obstet Gynecol. 1983;146:247-50. 20. Wheeler JM, Malinak LR. Recurrent endometriosis. Contrib Obstet Gynecol. 1987;16:13-21. 21. Winkel CA, Bray M. Treatment of women with endometriosis using excision alone, ablation alone, or ablation in combination with leuprolide acetate. Proceedings of the 4th World Congress on Endometriosis, Yokohama. 1996:55. Journal-Less than 6 authors Finkelstein JS, Hayes A, Hunzelman JL, Wyland JJ, Lee H, Neer RM. The effects of parathyroid hormone, alendronate, or both in men with osteoporosis. N Eng J Med 2003;349:1216-26. 22. Facts and Comparisons, 4.0; 2010. 23. American Society for Reproducticve Medicine. Treatment of pelvic pain associated with endometriosis. Fertil Steril. 2006;84 (Suppl 4):S18-S27. 24. Eligard TM Prescribing Information. Tolmar, Inc., November 2009. 25. Clinical Practice Guidelines in Oncology™. The National Comprehensive Cancer Network (NCCN) Drugs & Biologics. Available at www.nccn.org. 26. Rao GG, Miller DS. Hormonal therapy in epithelial ovarian cancer. Expert Rev Anticancer Ther. 2006; 6: 43-7.

Last Updated 04/2015 Page 3 of 3

Applies to: Diabetic Macular Edema Agents Nebraska Non-PDL

Diabetic Macular Edema Agents/ Ophthalmic agent-vascular endothelial growth factor: Avastin (bevacizumab), Ozurdex (dexamethasone), Eylea (aflibercept), Lucentis (ranibizumab), Illuvien (fluocinolone acetonide intravitreal implant)

Covered uses Diabetic Macular Edema (DME)

Exclusion Criteria Eylea, Iluvien, Lucentis, Ozurdex: Contraindicated in patients with active or suspected ocular or periocular infections including most viral disease of the cornea and conjunctiva including active epithelial herpes simplex keratitis (dendritic keratitis), vaccinia, varicella, mycobacterial infections or fungal diseases.

Required Medical Avastin: diagnosis of DME Information Iluvien: diagnosis of DME and t/f of Avastin and corticosteroid

Lucentis: diagnosis of DME and t/f of Avastin

Eylea: 1. diagnosis of DME and t/f of Avastin AND Lucentis OR 2. diagnosis of DME with diabetic retinopathy: Approve

Ozurdex: diagnosis of DME and t/f of Avastin

Age Restriction 18 years of age or older Prescriber Ophthalmologist Restriction Coverage Through benefit year Duration Other Criteria Preferred agent: Avastin (bevacizumab) 1.25 mg intravitreal injection every 6 weeks (max 9 injections in first 12 months). Reauthorization for 12 months will be approved for continuation of therapy based on documentation of clinical improvement from ongoing therapy with the requested medication.

Ozurdex: 0.7 mg intravitreal implant every 6 months.

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Applies to: Diabetic Macular Edema Agents Nebraska Non-PDL

Eylea: 2 mg (0.05 mL) administered by intravitreal injection every 4 weeks (monthly) for the first 5 injections followed by 2 mg (0.05 mL) via intravitreal injection once every 8 weeks (2 months). Although EYLEA may be dosed as frequently as 2 mg every 4 weeks (monthly), additional efficacy was not demonstrated when EYLEA was dosed every 4 weeks compared to every 8 weeks.

Lucentis: 0.3 mg (0.05 mL of 6 mg/mL LUCENTIS solution) by intravitreal injection once a month (approximately 28 days).

Iluvien: 0.19 mg intravitreal implant lasting 36 months.

REFERENCES 1. Eylea (aflibercept) [prescribing information]. Tarrytown, NY: Regeneron Pharmaceuticals Inc; October 2014. 2. Korobelnik JF, Do DV, Schmidt-Erfurth U, et al. Intravitreal aflibercept 3. for diabetic macular edema. Ophthalmology. 2014 4. Avastin (bevacizumab) [prescribing information]. South San Francisco, CA: Genentech Inc; November 2014. 5. Product Information: ILUVIEN(R) intravitreal implant, fluocinolone acetonide intravitreal implant. Alimera Sciences, Inc. (per Manufacturer), Alpharetta, GA, 2014. 6. Rajendram R, Fraser-Bell S, Kaines A, et al: A 2-year prospective randomized controlled trial of intravitreal bevacizumab or laser therapy (BOLT) in the management of diabetic macular edema: 24-month data: report 3. Arch Ophthalmol 2012.

Last Updated 11/2015 Page 2 of 2

Applies to: Increlex (mecasermin) Nebraska Non-PDL

Increlex (mecasermin)

Covered uses All medically accepted indications Exclusion Criteria N/A Required Medical Stated diagnosis of: Information Insulin-Like-Growth Factor (IGF-1) deficiency • Evidenced by low IGF-1 (IGF-1/Somatomedin-C) level matched by age and gender (see table below for normal reference values) Growth hormone deletion with antibodies to growth hormone

Age restrictions Patient must be less than 14 years of age, unless epiphyses have been confirmed open through a wrist film evaluation Prescriber Prescriber must be an endocrinologist or a pediatric Restrictions endocrinologist. Coverage Duration Initial authorization of therapy will be issued for 12 months. Re-authorization of therapy will be issued for 12 months. Other Criteria For diagnosis of IGF-1 Deficiency: • The patient has a height standard deviation score of ≤ -3.0 • The patient has normal or elevated growth hormone levels. For re-authorization: • Confirmed diagnosis of one of the following: o Primary IGF-1 deficiency; OR o Growth hormone deletion with antibodies to growth hormone; AND The patient is less than 14 years of age, unless epiphyses have been confirmed open through a wrist film evaluation.

Normal IGF-1 level reference ranges based on gender

References 1. IncrelexTM Prescribing Information. Tercica, Inc., September 2012.

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Applies to: Increlex (mecasermin) Nebraska Non-PDL

2. Chernausek SD, Backeljauw PF, Frane J, Kuntze J, Underwood LE. Long-term treatment with recombinant insulin-like growth factor (IGF-1) in children with severe IGF-1 deficiency due to growth hormone insensitivity. J Clin Endocrinol Metab 2007;92(3):902-910. 3. Rosenbloom AL. Insulin-like growth factor (rhIGF-1) therapy of short statute. J Pediatr Endocrinol Metab 2008;21:301-315. 4. Collett-Solberg PF, Misra M, Drug and Therapeutics Committee of the Lawson Wilkins Pediatric Endocrine Society. The role of recombinant insulin-like growth factor-I in treating children with short stature. J Clin Endocrinol Metab 2008;93(1):10-8. 5. Facts and Comparisons 4.0; 2012. 6. Clinical Pharmacology Gold Standard. 2012.

Last updated 04/2015 Page 2 of 2

Applies to: Korlym (mifepristone) Nebraska Non-PDL

Korlym (mifepristone)

Covered uses All medically accepted indications Exclusion Criteria Pregnancy Category X Required Medical Stated diagnosis of: Information Stated diagnosis to control hyperglycemia secondary to hypercortisolism in adult patients with endogenous Cushing’s syndrome who have type 2 diabetes mellitus or glucose intolerance and have failed surgery or are not candidates for surgery.

Korlym should not be used in the treatment of patients with type 2 diabetes unless it is secondary to Cushing’s syndrome.

Age restrictions 18 years and older

Prescriber None Restrictions Coverage Duration Initial authorization will be issued for 6 months. Re-authorization will be issued for 6 months. Other Criteria Do not exceed 20 mg/kg per day.

Re-authorization will be approved with documentation of improved glucose tolerance while on Korlym therapy

References 1. Korlym Prescribing Information. Corecept Therapuetics, June 2013. 2. Nathan DM, Buse JB, Davidson MB, et al. Medical management of hyperglycemia in type 2 diabetes: A consensus algorithm for the initiation and adjustment of therapy. A consensus statement of the American Diabetes Association and the European Association for the Study of Diabetes. Diabetes Care 2009;32(1):193-203. 3. Rodbard HW, Jellinger PS, Davidson JA, et al. Statement by an American Association of Clinical Endocrinologists/American College of Endocrinology consensus panel on type 2 diabetes mellitus: an algorithm for glycemic control. Endocr Pract. 2009; 15(6):540-559. 4. Clinical Pharmacology Gold Standard 2012.

Last updated 05/2014 Page 1 of 1

Applies to: Vivitrol Nebraska Non-PDL

Vivitrol (naltrexone extended-release injectable)

Covered uses All FDA Approved Indications • For the treatment of alcohol dependence in patients who are able to abstain from alcohol in an outpatient setting prior to Vivitrol initiation • For the prevention of relapse to opioid dependence, following opioid detoxification Exclusion Criteria Patients who may not take Vivitrol include: • Patients with acute hepatitis/ liver failure • Patients currently taking opioid analgesics (past 7-10 days) • Patients with current physiologic opioid dependence • Patients in acute opioid withdrawal • Patients who have a positive urine drug screen for opioids • Patients who failed the naloxone challenge test • Patients who display hypersensitivity to naltrexone, PLG, carboxymethylcellulose, or any other ingredient Required Medical Criteria for approval for treatment of alcohol dependence: Information • Documentation of the following: o Trial and failure on oral naltrexone . Failure will be defines as prescriber-based determination of inefficacy or non- adherence of the treatment o Enrollment in a substance abuse treatment program or community support program (counseling, 12-step program, etc.) o Attestation indicating the member is not actively consuming alcohol at the time of therapy initiation o Determination of adequate liver function that is WNL

Criteria for approval for treatment of opioid dependence: • Documentation of the following o Trial and failure on oral naloxone (i.e. Zubsolv) . Failure will be defined as prescriber- based determination of inefficacy or non-adherence of the treatment o Enrollment in a substance abuse treatment program or community support program (counseling, 12-step program, etc.) o Documentation is submitted indicating member has been opioid-free for a minimum of 7-10 days prior to therapy initiation including

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Applies to: Vivitrol Nebraska Non-PDL

pertinent lab testing (e.g. a recent urine drug screen for opioids, naloxone challenge test) o Determination of adequate liver function that is WNL

Reauthorization Criteria: • If prescribed for opioid dependence, documentation of a recent random urine drug screen negative for opioids • Documentation of compliance (per claims history) • Patient must continue to participate in a counseling or support program throughout the duration of time they are taking Vivitrol

Age restrictions Age minimum of 18 years old Prescriber Prescriber should be a specialist in the field of study- namely, Restrictions addiction or detoxification, or may be a primary care provider who is working in consultation with a specialist Coverage Duration Initial authorization: 3 months Subsequent authorization: 6 months Other Criteria Vivitrol must be prepared and administered by a healthcare provider.

Recommended dosing is 380 mg of Vivitrol delivered as an IM gluteal injection every 4 weeks (or once monthly), alternating buttocks for each injection and using only the customized needles provided in the carton.

References: 1) Prescribing Information (PDF) - VIVITROL. Retrieved August, 2016, from https://www.vivitrol.com/Content/pdf/prescribing_info.pdf 2) Esti, V. S. (2013). Predictors of Self-Reported Adherence to Naltrexone Medication in an Outpatient Treatment for Problem Drinking. J Addict Res Ther Journal of Addiction Research & Therapy, 04(04). doi:10.4172/2155-6105.1000159 Retrieved August, 2016, from http://www.omicsonline.org/predictors-of-selfreported- adherence-to-naltrexone-medication-in-an-outpatient-treatment-for-problem- drinking-2155-6105.1000159.php?aid=18432 3) Center for Substance Abuse Treatment. Incorporating Alcohol Pharmacotherapies Into Medical Practice. Rockville (MD): Substance Abuse and Mental Health Services Administration (US); 2009. (Treatment Improvement Protocol (TIP) Series, No. 49.) Chapter 5—Extended-Release Injectable Naltrexone. Retrieved August, 2016, from http://www.ncbi.nlm.nih.gov/books/NBK64031/ 4) Excerpt from Helping Patients Who Drink Too Much, A Clinician's Guide, Updated 2008 Edition. National Institutes of Health. Retrieved August, 2016, from http://pubs.niaaa.nih.gov/publications/Practitioner/CliniciansGuide2005/Prescribing Meds.pdf

Created 08/2016 Page 2 of 2

Applies to: Spinraza (nusinersen) Nebraska Non-PDL

Spinraza (nusinersen)

Covered Uses All FDA approved indications

Exclusion Criteria N/A

Required Medical Initial Authorization: Information 1. Member must have one of the following: a. Diagnosis of spinal muscular atrophy type I, II, or III by a neurologist trained in diagnosing SMA; OR b. Diagnosis of spinal muscular atrophy type I, II, or III by a physician in consultation with a neurologist trained in diagnosing SMA. AND 2. Submission of medical records (e.g., chart notes, laboratory values) confirming both of the following: a. The mutation or deletion of genes in chromosome 5q resulting in one of the following: i. Homozygous gene deletion or mutation (e.g., homozygous deletion of exon 7 at locus 5q13); OR ii. Compound heterozygous mutation (e.g., deletion of SMN1 exon 7[allele 1] and mutation of SMN1 [allele 2]) AND b. Patient has at least 2 copies of SMN2. AND 3. Patient is not dependent on either of the following: a. Invasive ventilation or tracheostomy b. Non-invasive ventilation for at least 6 hours per day6,7 AND 4. Submission of medical records (e.g., chart notes, laboratory results) of the baseline exam of at least one of the following exams (based on patient age and motor ability) to establish baseline motor ability: a. Hammersmith Infant Neurological Exam (HINE) (infant to early childhood); OR b. Hammersmith Functional Motor Scale Expanded (HFMSE); OR c. Upper Limb Module (ULM) Test (Non ambulatory); OR d. Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND).6,8 AND 5. Member’s baseline labs were submitted and member has no evidence of thrombocytopenia or renal impairment.

AND

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Applies to: Spinraza (nusinersen) Nebraska Non-PDL

6. One of the following: a. Spinraza is being prescribed by a neurologist trained in diagnosing SMA; OR b. Spinraza is prescribed by a physician in consultation with a neurologist trained in diagnosing SMA. AND 7. Spinraza will be administered intrathecally by, or under the direction of, healthcare providers experienced in performing lumbar punctures. AND 8. Spinraza dosing for SMA is in accordance with the United States Food and Drug Administration approved labeling (maximum dosing of 12mg for each loading dose). AND 9. Initial authorization will be for no more than 4 loading doses.

Reauthorization: 1. Patient is not dependent on either of the following: c. Invasive ventilation or tracheostomy d. Non-invasive ventilation for at least 6 hours per day6,7 AND 2. Submission of medical records (e.g., chart notes, laboratory values) with the most recent results (< 1 month prior to request) documenting a positive clinical response from pretreatment baseline status to Spinraza therapy as demonstrated by at least one of the following exams: a. HINE milestones: i. One of the following: (i) Improvement or maintenance of previous improvement of at least 2 point (or maximal score) increase in ability to kick; OR (ii) Improvement or maintenance of previous improvement of at least 1 point increase in any other HINE milestone (e.g., head control, rolling, sitting, crawling, etc.), excluding voluntary grasp. AND ii. One of the following: (i) The patient exhibited improvement, or maintenance of previous improvement in more HINE motor milestones than worsening, from pretreatment baseline (net positive improvement); OR (ii) Achieved and maintained any new motor milestones when they would otherwise be unexpected to do so (e.g., sit unassisted, stand, walk). OR one of the following (b, c, or d): b. HFMSE: One of the following: i. Improvement or maintenance of previous improvement of Created 03/2017 Page 2 of 4

Applies to: Spinraza (nusinersen) Nebraska Non-PDL at least a 3 point increase in score from pretreatment baseline; OR ii. Patient has achieved and maintained any new motor milestone from pretreatment baseline when they would otherwise be unexpected to do so. OR c. ULM: One of the following: i. Improvement or maintenance of previous improvement of at least a 2 point increase in score from pretreatment baseline; OR ii. Patient has achieved and maintained any new motor milestone from pretreatment baseline when they would otherwise be unexpected to do so. OR d. CHOP INTEND: One of the following: i. Improvement or maintenance of previous improvement of at least a 4 point increase in score from pretreatment baseline; OR ii. Patient has achieved and maintained any new motor milestone from pretreatment baseline when they would otherwise be unexpected to do so. AND 3. Member’s baseline labs were submitted and member has no evidence of thrombocytopenia or renal impairment. AND 4. One of the following: a. Spinraza is prescribed by a neurologist trained in diagnosing SMA; OR b. Spinraza is prescribed by a physician in consultation with a neurologist trained in diagnosing SMA. AND 5. Spinraza is to be administered intrathecally by, or under the direction of, healthcare professionals experienced in performing lumbar punctures. AND 6. Spinraza dosing for SMA is in accordance with the United States Food and Drug Administration approved labeling (maximum dosing of 12mg every 4 months, starting 4 months after the last loading dose). AND 7. Reauthorization will be for no more than 3 maintenance doses (12 months).

Age restrictions N/A

Prescriber Neurologist Restrictions

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Applies to: Spinraza (nusinersen) Nebraska Non-PDL Coverage Duration Initial authorization: 3 months Reauthorization: 12 months Other Criteria Nebraska In addition to meeting to above criteria the following must also be met: 1. Member must be 15 years of age or younger at time of initiation of Spinraza therapy.

Resources: 1. Bodamer, OA. Spinal muscular atrophy (SMA). www.uptodate.com (subscriber based). Published February 2017. Accessed March 7, 2017 2. Nusinersen: drug information. www.uptodate.com (subscriber based). Published 2017. Accessed March 7, 2017. 3. Nusinersen: patient drug information. www.uptodate.com (subscriber based). Published 2017. Accessed March 7, 2017. 4. Spinraza (nusinersen): a guide to Spinraza reimbursement. 5. Spinraza (nusinersen): summary of relevant codes for Spinraza. 6. Ionis Pharmaceuticals, Inc. A study to assess the efficacy and safety of IONIS-SMN rx in infants with Spinal Muscular Atrophy. In: ClinicalTrials.gov [Internet]. Bethesda (MD): National Library of Medicine (US). 2000. Available from: https://clinicaltrials.gov/show/NCT02193074 NLM Identifier: NCT02193074. Accessed March 23, 2017. 7. Ionis Pharmaceuticals, Inc. A Study to Assess the Efficacy and Safety of IONIS-SMN Rx in Patients With Later-onset Spinal Muscular Atrophy. In: ClinicalTrials.gov [Internet]. Bethesda (MD): National Library of Medicine (US). 2000. Available from: https://clinicaltrials.gov/show/NCT02292537 NLM Identifier: NCT02292537. Accessed March 23, 2017. 8. Spinraza [package insert]. Cambridge, MA: Biogen, Inc, December 2016.

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Applies to: Ocrevus (ocrelizumab) Nebraska Non-PDL

Ocrevus (ocrelizumab)

Covered Uses All FDA approved indications

Exclusion Criteria Active Hepatitis B infection

Required Medical Initial authorization: Information Ocrevus will be approved based on the following criteria • Documentation confirming diagnosis of: o Relapsing multiple sclerosis OR o Primary progressive multiple sclerosis • Negative Hepatitis B screening • Baseline Expanded Disability Status Scale (EDSS) score

Reauthorization • Documentation of clinical benefit and improvement o Decrease in EDSS score by 1 or more points if baseline score was <5.5 or decrease in EDSS score by 0.5 or more points if baseline score was >5.5 Age restrictions 18 years or older

Prescriber Neurologist Restrictions Coverage Duration 12 months Other Criteria Ocrevus will be administered in a medical facility Starting dose: 300 mg IV infusion followed two weeks later by a second 300mg IV infusion Subsequent doses: 600mg infusion every 6 months Ensure patient is current with recommended vaccines Require Hepatitis B vaccine counseling

Resources: 1. Ocrevus (ocrelizumab) [package insert]. Genetech, Inc. San Francisco, CA. March 2017 2.Montalban X, Hauser SL, Kappos L, et al. Ocrelizumab versus Placebo in Primary Progressive Multiple Sclerosis. N Engl J Med. 2016 3.Hauser SL, Bar-or A, Comi G, et al. Ocrelizumab versus Interferon Beta-1a in Relapsing Multiple Sclerosis. N Engl J Med. 2017;376(3):221-234. 4.Kurtzke JF. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology. 1983;33(11):1444-52.

Created 05/2017 Page 1 of 1

Applies to: BOTOX (onabotulinumtoxinA) Nebraska Non-PDL

BOTOX (onabotulinumtoxinA)

Covered uses 1. All medically accepted indications Exclusion Criteria Cosmetic Purposes: Uses for cosmetic purposes are not a covered benefit.

Infection at the injection site.

For bladder dysfunction, patients with urinary tract infections at the time of treatment should not be treatment.

Not proven effective for episodic migraines (those occurring 14 days or fewer per month). Required Medical Stated diagnosis of: Information Chronic Migraines: Documented history of migraine on 15 or more days of the month with headaches lasting four hours a day or longer. AND Documented treatment failure or intolerable side effects with at least 3 classes of migraine headache prophylaxis medications of at least 2 months (60 days) duration for each medication: a. Angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers (e.g., losartan, valsartan, lisinopril); b. Anti-depressants (e.g., amitriptyline, clomipramine, doxepin, mirtazapine, nortryptiline, protriptyline); c. Anti-epileptic drugs (e.g., gabapentin, topiramate, valproic acid); d. Beta blockers (e.g., atenolol, metoprolol, nadolol, propranolol, timolol); e. Calcium channel blockers (e.g., diltiazem, nifedipine, nimodipine, verapamil); f. Alpha agonist (e.g., clonidine, guanfacine); g. Antihistamine (e.g., cyproheptadine).

Upper or Lower Limb Spasticity: Documented history of treatment failure or intolerable side effects from treatment with 2 of the preferred medications: baclofen, cyclobenzaprine, tizanidine or chlorzoxazone.

Cervical Dystonia: Documented history of treatment failure or intolerable side effects from treatment with 1 of the preferred medications from 2 different classes of medications: anticholinergic agents (trihexyphenidyl or benztropine) and skeletal muscle relaxants (chlorzoxazone, methocarbamol, cyclobenzaprine or carisoprodol).

Last updated 02/2017 Page 1 of 2

Applies to: BOTOX (onabotulinumtoxinA) Nebraska Non-PDL

Severe Primary Axillary Hyperhidrosis: Documented history of treatment failure or intolerable side effects from treatment with prescription Aluminum Chloride antiperspirant

Detrusor Overactivity (associated with a neurologic condition): Documented history of trial and failure or intolerable side effects with 2 of the preferred medications: oxybutynin, tamsulosin, or trospium

Overactive Bladder: Documented history of trial and failure or intolerable side effects with 2 of the preferred medications: oxybutynin, tamsulosin, or trospium

Blepharospasm or Strabismus: Documented diagnosis of blepharospasm or strabismus

Re-authorization requires the following: If the prescriber notates efficacy to a medically accepted indication, then reauthorize for an additional 12 weeks Age restrictions 18 years or greater (chronic migraine, upper limb spasticity, primary axillary hyperhidrosis, overactive bladder or detrusor overactivity [associated with a neurologic condition]) 16 years or greater (cervical dystonia) 12 years or greater (blepharospasm and strabismus) Prescriber Neurologist (Chronic Migraines) Restrictions Coverage Duration Initial authorization will be issued for 12 weeks Re-authorization will be issued for 12 weeks

Other Criteria

Reference 1. Botox [package insert]. Allergan, Inc. Irvine, CA. February, 2016

Last updated 02/2017 Page 2 of 2

Applies to: Aloxi (palonosetron) Nebraska Non-PDL

Aloxi (palonosetron)

Covered uses All FDA approved indications

Exclusion Criteria . Member has received, or will receive chemotherapy regimen with low or minimal emetogenic risk. . Treatment for active nausea and vomiting . Treatment after day 1 of chemotherapy (should only be administered once)

Required Medical . Member has received, or will receive chemotherapy regimen Information with moderate to high emetogenic risk . Long-acting anti-emetic therapy must be initiated on day 1, 30 minutes before start of chemotherapy.

Must be for FDA-approved indications: . Moderately emetogenic cancer chemotherapy -- prevention of acute and delayed nausea and vomiting associated with initial and repeat courses (adults and pediatrics) o Failure, contraindication, or clinically significant adverse event to a serotonin (5-HT3) antagonist PO/IV ± steroid PO/IV is required . Highly emetogenic cancer chemotherapy -- prevention of acute nausea and vomiting associated with initial and repeat courses (adults and pediatrics) . Prevention of postoperative nausea and vomiting (PONV) for up to 24 hours following surgery (adults only) o Failure, contraindication, or clinically significant adverse events to ondansetron is required

Age restrictions 1 month or older

Prescriber Oncologist Restrictions Coverage Duration 1 year

References

1. Product Information: ALOXI® intravenous injection. Eisai, Inc., Woodcliff Lake, NJ. Updated 9/2014. 2. Antiemesis. The NCCN Guidelines. Version 2.2015. Updated 9/2015.

Last updated: 05/2017 Page 1 of 1

Applies to: Keytruda (pembrolizumab) Nebraska Non-PDL

Keytruda (pembrolizumab)

Covered uses All medically accepted indications Exclusion Criteria N/A Required Medical Stated diagnosis from oncologist of one of the following: Information 1) Unresectable or metastatic melanoma

2) Metastatic non-small cell lung cancer (NSCLC) • High PD-L1 expression (Tumor Proportion Score [TPS] …... ≥50%), with no EGFR or ALK tumor aberrations and no ……prior systemic chemotherapy for metastatic NSCLC OR • PD-L1 expression (TPS ≥1%), with disease progression on …...or after platinum-based chemotherapy

3) Recurrent or metastatic head and neck squamous cell carcinoma (HNSCC), with disease progression on or after platinum-based chemotherapy Age restrictions 18 years or older Prescriber Oncologist Restrictions Coverage Duration 1 year Other Criteria Recommended Dosing: • Melanoma: 2 mg/kg every 3 weeks • NSCLC: 200 mg every 3 weeks • HNSCC: 200 mg every 3 weeks

References: 1) Keytruda [Package Insert] Merck & Co., Inc. Whitehouse Station, NJ. October 2016

Last updated 02/2017 Page 1 of 1

Applies to: Mozobil (plerixafor) Nebraska Non-PDL

Mozobil (plerixafor)

Covered uses All medically accepted indications

Exclusion Criteria N/A

Required Medical Mozobil will be approved based on all of the following criteria: Information 1. One of the following: a. Patients with non-Hodgkin’s lymphoma (NHL) who will be undergoing autologous hematopoietic stem cell transplantation; OR b. Patients with multiple myeloma (MM) who will be undergoing autologous hematopoietic stem cell transplantation -AND- 2. Used in combination with granulocyte-colony stimulating factor (G-CSF)

Age restrictions N/A

Prescriber Hematologist/oncologist Restrictions Coverage Duration 1 course (up to four days of therapy) 2, 3, 4, (The duration of treatment for Mozobil in both the pivotal studies and compassionate use data was limited to one course of therapy.) Other Criteria Recommended dose is 0.24 mg/kg body weight by subcutaneous (SC) injection.

REFERENCES

1 Mozobil® Prescribing Information. Genzyme Corporation, August 2010. 2 DiPersio JF, Micallef I, Stiff P, et al. Months report from the phase 3 study of plerixafor + G-CSF vs. placebo + G-CSF for mobilization of hematopoietic stem cell for autologous transplant in patients with NHL. [abstract]. Blood. 2008;112:Abstract 1136. 3 DiPersio JF, Stadtmauer E, Nademanee A, et al. Months report from a phase 3 study of plerixafor + G-CSF vs. placebo + G-CSF for mobilization of hematopoietic stem cell for autologous transplant in patients with multiple myeloma. [abstract]. Blood. 2008;112:Abstract 3312. 4 Calandra G, McCarty J, McGuirk J, et al. AMD3100 plus G-CSF can successfully mobilize CD34+ cells from non-hodgkin’s lymphoma, hodgkin’s disease and multiple myeloma patients previously failing mobilization with chemotherapy and/or cytokine treatment: compassionate use data. Bone Marrow Transplant. 2008;41:331-38.

Last updated 03/2015 Page 1 of 1

Applies to: Kuvan (sapropterin dihydrochloride) Nebraska Non-PDL

Kuvan (sapropterin dihydrochloride)

Covered uses All medically accepted indications Exclusion Criteria None Required Medical Medical statement indicating treatment to reduce blood Information phenylalanine (Phe) levels in patients with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin-(BH4-) responsive Phenylketonuria (PKU). AND Documentation of baseline blood Phe levels

Reauthorization: Documentation of blood Phe levels after 1 month of therapy indicating 30% reduction in Phe levels from baseline.

Age restrictions None

Prescriber None Restrictions Coverage Duration Initial authorization of therapy will be issued for 2 months. Re-Authorization of therapy will be issued for 12 months. Other Criteria This medication is to be used in conjunction with a Phe-restricted diet. Discontinue sapropterin therapy if blood Phe concentrations do not decrease after 1 month of treatment at 20 mg/kg/day.

References 1. Kuvan® Prescribing Information. BioMarin Pharmaceticals, June 2013. 2. Facts and Comparisons 4.0; 20012. 3. National Institutes of Health. Phenylketonuria (PKU): Screening and Management. NIH Consensus Statement 2000 October 16-18;17(3):1-33. Available at: http://consensus.nih.gov/2000/2000Phenylketonuria113Program.pdf.

Last updated 04/2015 Page 1 of 1

Applies to: Xyrem (sodium oxybate) Nebraska Non-PDL

Xyrem (sodium oxybate)

Covered uses All medically accepted indications Exclusion Criteria Patients concurrently using sedative hypnotics or alcohol Required Medical Stated diagnosis of: Information • Narcolepsy confirmed by a sleep study o Not necessary if prescriber provides justification confirming that a sleep study would not be feasible o Trial and failure or contraindication to one of the preferred stimulants: dextroamphetamine tablets, dexmethylphenidate tablets, methylin chewables, methyphenidate IR tablet, methyphenidate ER tablet (10mg or 20 mg) AND modafinil • Cataplexy; approve Other Criteria • Reauthorization will occur if: o Patient experienced a reduction in the frequency of cataplexy attacks associated with Xyrem therapy OR o Patient experienced a reduction in symptoms of excessive daytime sleepiness associated with Xyrem therapy.

Age restrictions 18 years of age and older

Prescriber None Restrictions Coverage Duration Original authorization will be issued for 3 months. Reauthorization will be issued for 12 months. Other Criteria If concurrently using with divalproex sodium, an intitial reduction of 20% of Xyrem dose is recommended.

References: 1. Product Information: Xyrem(R) oral solution, sodium oxybate oral solution. Jazz Pharmaceuticals (per FDA), Palo Alto, CA, 2012. 2. Xyrem Prescribing Information. Jazz Pharmaceuticals, Inc. November 2005. 3. US Xyrem Multicenter Study Group. A randomized, double blind, placebo- controlled multicenter trial comparing the effects of three doses of orally administered sodium oxybate with placebo for the treatment of narcolepsy. Sleep. 2002;25:42-49.

Last Updated 05/2014 Page 1 of 2

Applies to: Xyrem (sodium oxybate) Nebraska Non-PDL

4. US Xyrem Multicenter Study Group. Sodium oxybate demonstrates long-term efficacy for the treatment of cataplexy in patients with narcolepsy. Sleep Med. 2004;5(2):119-23. 5. US Xyrem Multicenter Study Group. A 12-month, open-label, multicenter extension trial of orally administered sodium oxybate for the treatment of narcolepsy. Sleep. 2003;26(1):31- 35. 6. Morgenthaler TI, Kapur VK, Brown TM, Swick TJ, Alessi C, Aurora N, et al. Practice parameters for the treatment of narcolepsy and other hypersomnias of central origin: An American Academy of Sleep Medicine report. Sleep. 2007;30(12):1705-1711. 7. The Xyrem International Study Group. A double-blind, placebo-controlled study demonstrates sodium oxybate is effective for the treatment of excessive daytime sleepiness in narcolepsy. J Clin Sleep Med. 2005;1:391-397. 8. American Academy of Sleep Medicine. International Classification of Sleep Disorders: Diagnostic and Coding Manual. 2nd ed. Westchester, IL: American Academy of Sleep Medicine; 2005. 9. Billiard M, Dauvilliers Y, Dolenc-Groselj L, et al. Chapter 38 : Management of narcolepsy in adults. In: Gilhus NE, Barnes MP, Brainin M, eds. European Handbook of Neurological Management. 2nd ed. Oxford England: Wiley- Blackwell; 2011:513-28. 10. Per clinical consultation with neurologist/sleep specialist. October 9, 2012.

Last Updated 05/2014 Page 2 of 2

Applies to: Gattex (Teduglutide) Nebraska Non-PDL

Gattex (Teduglutide)

Covered uses All FDA approved indications

Exclusion Criteria N/A

Required Medical A. Initial Authorization Information 1. Gattex will be approved based on both of the following criteria: a. Diagnosis of short bowel syndrome (SBS) -AND b.Dependent on parenteral nutrition/intravenous (PN/I.V.) support for at least 12 consecutive months

B. Reauthorization 1. Gattex will be approved based on the following criterion: a. Documentation of positive clinical response to Gattex therapy Age restrictions

Prescriber Restrictions Coverage Duration 6 months Other Criteria The recommended daily dose of GATTEX is 0.05 mg/kg body weight administered by subcutaneous injection once daily.

References Gattex [package insert] McPherson, KS Hospira, Inc. January 2013

Last updated 11/2015 Page 1 of 1

Applies to: Xenazine (tetrabenazine) Nebraska Non-PDL

Xenazine (tetrabenazine)

Covered uses All FDA approved indications

Exclusion Criteria Patients who are actively suicidal Patients with untreated or inadequately treated depression Hepatic impairment Taking MAOIs or reserpine Required Medical Stated diagnosis of: Information Chorea associated with Huntington’s Disease

Re-authorization Submission of both of the following: • The patient is being treated for chorea associated with Huntington disease • The patient’s chorea has not progressed to rigidity and bradykinesia

Age restrictions 18 years or older

Prescriber Neurologist Restrictions Coverage Duration Initial authorization of therapy will be issued for 3 months. Re- authorization of therapy will be issued for 12 months. Other Criteria

References: 1. Xenazine® Prescribing Information. Ovation Pharmaceuticals, September 2012. 2. Frank, S. Ondo W, Fahn S, et al. A study of chorea after tetrabenazine withdrawal in patients with Huntingon disease. Clinical Neuropharmacology. 2008;31(3):127-133. 3. Rosenblatt A, Ranen NG, Nance MA, et al. Huntington’s Disease Society of America. A physician’s guide to the management of Huntington’s disease. 2nd ed. Available at http://www.hdsa.org/images/content/1/1/11289.pdf.

Last Updated 05/2016 Page 1 of 1

Applies to: Tygacil (tigecycline) Nebraska Non-PDL

Tygacil (tigecycline)

Covered uses All medically accepted indications Exclusion Criteria Tygacil will not be approved for the treatment of diabetic foot infections or for the treatment of hospital-acquired or ventilator- associated pneumonia. Required Medical 1. Recent (within 30 days of request) Culture and Sensitivity report; Information AND 2. Documentation of 1 of the following medically accepted indication with appropriate trial of alternative treatment.

Community acquired bacterial pneumonia (caused Streptococcus pneumoniae (penicillin-susceptible isolates), including cases with concurrent bacteremia, Haemophilus influenzae (beta-lactamase negative isolates), and Legionella pneumophila): Trial and failure of linezolid and two (2) preferred alternatives or resistance to all other appropriate therapies: Amoxicillin/clavulanic acid, Azithromycin, Cephalexin, Clarithromycin, Dicloxacillin, Doxycycline, Levofloxacin, Trimethoprim-sulfamethoxazole.

Complicated skin / skin structure infections (caused by Escherichia coli, Enterococcus faecalis (vancomycin-susceptible isolates), Staphylococcus aureus (methicillin-susceptible and -resistant isolates), Streptococcus agalactiae, Streptococcus anginosus grp. (includes S. anginosus, S. intermedius, and S. constellatus), Streptococcus pyogenes, Enterobacter cloacae, Klebsiella pneumoniae, and Bacteroides fragilis): Trial and failure of linezolid and two (2) preferred alternatives or resistance to all other appropriate therapies: Doxycycline, Trimethoprim-sulfamethoxazole, Dicloxacillin, Minocycline, Clindamycin, Amoxicillin/ clavulanic acid, Cephalexin, Ciprofloxacin , Clindamycin, levofloxacin

Complicated intra-abdominal infections (caused by Citrobacter freundii, Enterobacter cloacae, Escherichia coli, Klebsiella oxytoca, Klebsiella pneumoniae, Enterococcus faecalis (vancomycin-susceptible isolates), Staphylococcus aureus (methicillin-susceptible and -resistant isolates), Streptococcus anginosus grp. (includes S. anginosus, S. intermedius, and S. constellatus), Bacteroides fragilis, Bacteroides thetaiotaomicron, Bacteroides uniformis, Bacteroides vulgatus, Clostridium perfringens, and Peptostreptococcus micros): Trial and failure of two (2) preferred alternatives or resistance to all other appropriate therapies: Doxycycline, Trimethoprim-sulfamethoxazole, Dicloxacillin, Minocycline, Clindamycin, Amox/clavulanic acid, Cephalexin, Ciprofloxacin , Clindamycin , levofloxacin

Age restrictions Age 18 years or greater

Last updated: 02/2017 Page 1 of 2

Applies to: Tygacil (tigecycline) Nebraska Non-PDL

Prescriber Infectious Disease Specialist Restrictions Coverage Duration 14 Days

Other Criteria Dosing must be in accordance with FDA approved labeling

References

1. Tygacil [package insert]. Philadelphia, PA: Pfizer Injectables; 2014

Last updated: 02/2017 Page 2 of 2

Applies to: Samsca (tolvaptan) Nebraska Non-PDL

Samsca (tolvaptan)

Covered uses All medically accepted indications

Exclusion Criteria • Use > 30 days of treatment • Underlying liver disease (ALT/AST levels >3 x ULN; Bilirubin level > 2 x ULN) including cirrhosis • Not started in a hospital setting • Use to raise serum sodium urgently to prevent or to treat serious neurological symptoms. • Hypovolemic hyponatremia • Doses greater than 60mg/day • Concomitant use of strong CYP 3A inhibitors (e.g., ketoconazole, clarithromycin, itraconazole, ritonavir, indinavir, nelfinavir, saquinavir, nefazadone, telithromycin) Required Medical • Documentation that the current treatment course was initiated in a Information hospital setting by confirming that initiation of outpatient treatment is less than 7 days post hospital discharge • Baseline liver enzyme and bilirubin levels • Documentation of significant hypervolemic or euvolemic hyponatremia: Serum sodium <125 mEq/L OR less marked hyponatremia that is symptomatic and has resisted correction with fluid restriction o This includes patients with heart failure and syndrome of inappropriate antidiuretic hormone (SIADH)

Age restrictions 18 years or older Prescriber Restrictions Coverage Duration 30 days Other Criteria

References: 1. Product Information: SAMSCA(R) oral tablets, tolvaptan oral tablets. Otsuka America Pharmaceutical, Inc. (per FDA), Rockville, MD, 2013.

Last updated 05/2014 Page 1 of 1

Applies to: Lysteda (tranexamic acid) Nebraska Non-PDL

Lysteda (tranexamic acid)

Covered uses Cyclic Heavy Menstrual Bleeding Exclusion Criteria

Required Medical Lysteda will be approved based on one of the following Information criteria: 1. The patient did not exhibit an adequate response to treatment with a hormonal contraceptive/hormone replacement product. -OR 2. The patient experienced an intolerance / adverse reaction to previous therapy with a hormonal contraceptive/hormone replacement product. -OR- 3. The patient has a documented contraindication to treatment with hormonal a contraceptive/hormone replacement product.

Age restrictions N/A

Prescriber N/A Restrictions Coverage Duration 12 months Other Criteria N/A

REFERENCES

1 Lysteda [package insert]. Newport, KY: Xanodyne Pharmaceuticals; October 2013. 2 National Collaborating Centre for Women's and Children's Health. Heavy menstrual bleeding. London (UK): Royal College of Obstetricians and Gynaecologists (RCOG); 2007 Jan. 164 p. 3 American College of Obstetricians and Gynecologists (ACOG). Noncontraceptive uses of hormonal contraceptives. Washington (DC): American College of Obstetricians and Gynecologists (ACOG); 2010 Jan. 13 p. (ACOG practice bulletin; no. 110). 4 American College of Obstetricians and Gynecologists (ACOG). Intrauterine device. Washington (DC): American College of Obstetricians and Gynecologists (ACOG); 2005 Jan. 10 p. (ACOG practice bulletin; no. 59).

Last updated 04/2015 Page 1 of 1

Applies to: Ingrezza (valbenazine) Nebraska Non-PDL

Ingrezza (valbenazine)

Covered uses All FDA approved indications

Exclusion Criteria N/A

Required Medical Initial Authorization: Information . Confirm the length of antipsychotic drug exposure (three or more months of drug exposure if under 60 years of age OR four or more weeks of drug exposure if over the age of 60; exposure does not need to be constant) . Documented diagnosis of Tardive Dyskinesia by Abnormal Involuntary Movement Scale (AIMS) score of 9 or higher . Trial and failure or contraindication to two of the following preferred agents: o Clonazepam, Amantadine, Risperidone

Reauthorization: . Decrease in AIMS score or documented improvement of symptoms

Age restrictions 18 years of age and older

Prescriber Neurologist, Movement Disorder Specialist Restrictions Coverage Duration Initial authorization: 3 months Reauthorization: 12 months

Other Criteria Quantity Limit: 60 capsules/30 days

References

1. Lerner PP, Miodownik C, Lerner V. Tardive dyskinesia (syndrome): Current concept and modern approaches to its management. Psychiatry Clin Neurosci. 2015;69(6):321-34. 2. Pappa S, Tsouli S, Apostolou G, Mavreas V, Konitsiotis S. Effects of amantadine on tardive dyskinesia: a randomized, double-blind, placebo-controlled study. Clin Neuropharmacol. 2010;33(6):271-5. 3. Ondo WG, Hanna PA, Jankovic J. Tetrabenazine treatment for tardive dyskinesia: assessment by randomized videotape protocol. Am J Psychiatry. 1999;156(8):1279-81. 4. Cloud LJ, Zutshi D, Factor SA. Tardive dyskinesia: therapeutic options for an increasingly common disorder. Neurotherapeutics. 2014;11(1):166-76. 5. Bhidayasiri R, Fahn S, Weiner WJ, et al. Evidence-based guideline: treatment of tardive syndromes: report of the Guideline Development Subcommittee of the American Academy of Neurology. Neurology. 2013;81(5):463-9.

Last updated: 02/2017 Page 1 of 2

Applies to: Ingrezza (valbenazine) Nebraska Non-PDL

6. Bai YM, Yu SC, Lin CC. Risperidone for severe tardive dyskinesia: a 12-week randomized, double-blind, placebo-controlled study. J Clin Psychiatry. 2003;64(11):1342-8 7. Summary of Evidence-based Guideline for PATIENTS and their FAMILIESTREATING AND MANAGING TARDIVE SYNDROMES. American Academy of Neurology. https://www.aan.com/Guidelines/Home/GetGuidelineContent/614 8. Hauser RA, Factor SA, Marder SR, et al. KINECT 3: A Phase 3 Randomized, Double-Blind, Placebo-Controlled Trial of Valbenazine for Tardive Dyskinesia. Am J Psychiatry. 2017;:appiajp201716091037.

Last updated: 02/2017 Page 2 of 2