ph-0594
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Nulibry™ (fosdenopterin)

Policy Number: PH-0594

Intravenous

 

Last Review Date: 04/06/2021

Date of Origin: 04/06/2021                                                                                                                 

Dates Reviewed: 04/2021

 

FOR PEEHIP Members Only -Coverage excludes the provider-administered medication(s) outlined in this drug policy from being accessed through a specialty pharmacy. It must be obtained through buy and bill.

 

  1. Length of Authorization

Coverage will be provided initially for six months and may be renewed annually thereafter.

  1. Dosing Limits

A.  Quantity Limit (max daily dose) [NDC Unit]:

  • Nulibry 9.5 mg vial for injection: 10 vials daily

B.  Max Units (per dose and over time) [HCPCS Unit]:

  • 95 mg daily
  1. Initial Approval Criteria 1

Coverage is provided in the following conditions:

Universal Criteria 1,3

  • Will not be used in combination with other substrate replacement therapy (e.g., recombinant cyclic pyranopterin monophosphate, etc.); AND
  • Must be prescribed by, or in consultation with, a specialist in medical genetics or pediatric neurology; AND

Molybdenum Cofactor Deficiency Type A (MoCD Type A) † Ф 1-3

  • Patient has a diagnosis of MoCD Type A as confirmed, by molecular genetic testing, by a mutation in the MOCS1 gene suggestive of disease; OR
  • Patient has biochemical features suggestive of MoCD Type A (i.e., elevated sulfites in urine, low serum uric acid, elevated urinary xanthine and hypoxanthine) and will be treated presumptively while awaiting genetic confirmation; AND
  • Patient has a baseline value for the following:
  • Elevated urinary s-sulfocysteine (SSC) normalized to creatinine; AND
  • Clinical notes regarding signs and symptoms of disease which may include, but are not limited to, seizure frequency/duration, growth, and developmental milestones

FDA approved indication(s); Compendia recommended indication(s); Ф Orphan Drug

  1. Renewal Criteria 1

Authorizations can be renewed based on the following criteria:

  • Patient continues to meet universal and other indication-specific relevant criteria such as concomitant therapy requirements (not including prerequisite therapy), performance status, etc. identified in section III; AND
  • Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include severe phototoxicity, clinically significant infection, etc.; AND
  • Disease response compared to pre-treatment baseline as evidenced by the following:
  • Reduction in urinary SSC normalized to creatinine; AND
  • Stabilization or improvement in one or more signs and symptoms of disease including, but not limited to, seizure frequency/duration, growth, achievement of developmental milestones; OR
  • Patient initiated therapy as an inpatient based upon a presumptive diagnosis of MoCD Type A which was subsequently confirmed by genetic testing; AND
  •  Patient is responding to therapy compared to one or more pre-treatment baseline parameters which prompted the workup for MoCD
  1. Dosage/Administration 1

Indication

Dose

MoCD Type A

Age less than 1 year (Pre-Term neonates - gestational age <37 weeks)

  • Initial dosage: 0.4 mg/kg once daily
  • Dosage at 1 month: 0.7 mg/kg once daily
  • Dosage at 3 months: 0.9 mg/kg once daily

Age less than 1 year (Full-Term neonates - gestational age ≥37 weeks)

  • Initial dosage: 0.55 mg/kg once daily
  • Dosage at 1 month: 0.75 mg/kg once daily
  • Dosage at 3 months: 0.9 mg/kg once daily

Age at least 1 year

  • The recommended dosage is 0.9 mg/kg administered as an IV infusion once daily.

*Note all weights are based on Actual Body Weight (ABW)

Nulibry is administered intravenously by a healthcare provider or at home by the patient’s caregiver

  1. Billing Code/Availability Information

HCPCS Code:

  • J3490 – Unclassified drugs
  • C9399 – Unclassified drugs or biologicals (hospital outpatient use)

NDC:

  • Nulibry 9.5 mg single-dose vial as a lyophilized powder for injection: 73129-0001-xx
  1. References
  1. Nulibry [package insert].  Boston, MA; Origin Biosciences, Inc.; February 2021. Accessed March 2021.
  2. Origin Biosciences. A Phase 2, Multicenter, Multinational, Open-Label, Dose-Escalation Study to Evaluate the Safety and Efficacy of ORGN001 (Formerly ALXN1101) in Pediatric Patients With Molybdenum Cofactor Deficiency (MoCD) Type A Currently Treated With Recombinant Escherichia Coli-derived Cyclic Pyranopterin Monophosphate (rcPMP). Available from: https://clinicaltrials.gov/ct2/show/NCT02047461?term=NCT02047461&draw=2&rank=1. NLM identifier: NCT02047461. Accessed Marche 3, 2021.
  3. Origin Biosciences. A Phase 2/3, Multicenter, Multinational, Open Label Study to Evaluate the Efficacy and Safety of ORGN001 (Formerly ALXN1101) in Neonates, Infants and Children With Molybdenum Cofactor Deficiency (MOCD) Type A. Available from: https://clinicaltrials.gov/ct2/show/NCT02629393?term=NCT02629393&draw=2&rank=1. NLM identifier: NCT02629393. Accessed March 3, 2021.
  4. Reiss J, Hahnewald R. Molybdenum cofactor deficiency: Mutations in GPHN, MOCS1, and MOCS2. Hum Mutat. 2011 Jan;32(1):10-8.
  5. Veldman A, Santamaria-Araujo JA, Sollazzo S, Pitt J, Gianello R, Yaplito-Lee J, Wong F, Ramsden CA, Reiss J, Cook I, Fairweather J, Schwarz G. Successful treatment of molybdenum cofactor deficiency type A with cPMP. Pediatrics. 2010 May;125(5):e1249-54. doi: 10.1542/peds.2009-2192. Epub 2010 Apr 12.

Appendix 1 – Covered Diagnosis Codes

ICD-10

ICD-10 Description

E61.5

Molybdenum deficiency

E72.19

Other disorders of sulfur-bearing amino-acid metabolism

Appendix 2 – Centers for Medicare and Medicaid Services (CMS)

Medicare coverage for outpatient (Part B) drugs is outlined in the Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50 Drugs and Biologicals. In addition, National Coverage Determination (NCD), Local Coverage Articles (LCAs), and Local Coverage Determinations (LCDs) may exist and compliance with these policies is required where applicable. They can be found at: http://www.cms.gov/medicare-coverage-database/search/advanced-search.aspx. Additional indications may be covered at the discretion of the health plan.

Medicare Part B Covered Diagnosis Codes (applicable to existing NCD/LCD/LCA): N/A

 

 

Medicare Part B Administrative Contractor (MAC) Jurisdictions

Jurisdiction

Applicable State/US Territory

Contractor

E (1)

CA, HI, NV, AS, GU, CNMI

Noridian Healthcare Solutions, LLC

F (2 & 3)

AK, WA, OR, ID, ND, SD, MT, WY, UT, AZ

Noridian Healthcare Solutions, LLC

5

KS, NE, IA, MO

Wisconsin Physicians Service Insurance Corp (WPS)

6

MN, WI, IL

National Government Services, Inc. (NGS)

H (4 & 7)

LA, AR, MS, TX, OK, CO, NM

Novitas Solutions, Inc.

8

MI, IN

Wisconsin Physicians Service Insurance Corp (WPS)

N (9)

FL, PR, VI

First Coast Service Options, Inc.

J (10)

TN, GA, AL

Palmetto GBA, LLC

M (11)

NC, SC, WV, VA (excluding below)

Palmetto GBA, LLC

L (12)

DE, MD, PA, NJ, DC (includes Arlington & Fairfax counties and the city of Alexandria in VA)

Novitas Solutions, Inc.

K (13 & 14)

NY, CT, MA, RI, VT, ME, NH

National Government Services, Inc. (NGS)

15

KY, OH

                     

CGS Administrators, LLC

 

 


 

 

 Nulibry™ (fosdenopterin)  Prior Auth Criteria
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