ph-0346
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Mepsevii (vestronidase alfa-vjbk)

Policy Number: PH-0346

(Intravenous)

 

Last Review Date: 02/04/2020

Date of Origin: 12/12/2017

Dates Reviewed: 12/2017, 10/2018, 02/2019, 02/2020

  1. Length of Authorization

Coverage will be provided for twelve months and may be renewed.

  1. Dosing Limits

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

  • Mepsevii 10 mg vial: 46 vials per 14 days

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

  • 460 billable units (460 mg) every 14 days
  1. Initial Approval Criteria 1,2

Coverage is provided in the following conditions:

Universal Criteria

  • Patient aged 5 months or older; AND

Mucopolysaccharidosis VII (MPS VII; Sly syndrome) †

  • Patient has a definitive diagnosis of MPS VII confirmed by BOTH of the following:
    • Beta-glucuronidase enzyme deficiency in peripheral blood leukocytes; AND
    • Detection of pathogenic mutations in the GUSB gene by molecular genetic testing; AND
  • Documented baseline value for one or more of the following: six minute walk test (6MWT), motor function [i.e., Bruininks-Oseretsky Test of Motor Proficiency (BOT-2)], liver and/or spleen volume, urinary excretion of glycosaminoglycans (GAGs) such as chondroitin sulfate and dermatan sulfate, skeletal involvement (i.e. Z-score), pulmonary function tests, shoulder flexion, visual acuity, etc.

FDA-approved indication(s)

  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: anaphylaxis and severe allergic reactions, etc.; AND
  • Patient has responded to therapy compared to pretreatment baseline in one or more of the following:
    • Stability or improvement in 6MWT, shoulder flexion, visual acuity, and/or other motor functions
    • Reduction in liver and/or spleen volume
    • Reduction in urinary excretion of GAGs
    • Stability of skeletal disease (i.e. improvement in Z-score)
    • Stability or improvement in pulmonary function tests
  1. Dosage/Administration1

Indication

Dose

Mucopolysaccharidosis VII
(Sly syndrome)

4 mg/kg administered as an intravenous (IV) infusion once every 2 weeks

  1. Billing Code/Availability Information

HCPCS Code:

  • J3397 - Injection, vestronidase alfa-vjbk, 1 mg: 1 billable unit = 1 mg

NDC:

  • Mepsevii 10 mg/5 mL single-dose vial: 69794-0001-xx
  1. References
  1. Mepsevii [package insert]. Novato, CA; Ultragenyx Pharmaceutical Inc.; December 2019. Accessed January 2020.
  2. Montaño AM, Lock-Hock N, Steiner RD, et al. Clinical course of sly syndrome (mucopolysaccharidosis type VII). J Med Genet. 2016 Jun;53(6):403-18.
  3. Harmatz P, Whitley CB, Wang RY, et al. A novel, randomized, placebo-controlled, blind-start, single-crossover phase 3 study to assess the efficacy and safety of UX003 (rhGUS) enzyme replacement therapy in patients with MPS VII. Mol Genet Metab. 2017;120:S63.
  4. Qi Y, McKeever K, Taylor J, et al. Pharmacokinetic and Pharmacodynamic Modeling to Optimize the Dose of Vestronidase Alfa, an Enzyme Replacement Therapy for Treatment of Patients with Mucopolysaccharidosis Type VII: Results from Three Trials. Clin Pharmacokinet. 2019 May;58(5):673-683. doi: 10.1007/s40262-018-0721-y.

Appendix 1 – Covered Diagnosis Codes

ICD-10

ICD-10 Description

E76.29

Other mucopolysaccharidoses

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 Determinations (LCDs), and Articles 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/Article):  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

 

MEPSEVII™ (vestronidase alfa-vjbk) Prior Auth Criteria
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