BlueCross BlueShield of Tennessee Medical Policy Manual

Vestronidase alfa-vjbk (Mepsevii®)

NDC CODE(S)

69794-0001-XX MEPSEVII 2MG/ML Solution (ULTRAGENYX PHARMACEUTICAL)

DESCRIPTION

Vestronidase alfa-vjbk is a recombinant human lysosomal beta glucuronidase which is a purified human enzyme produced by recombinant DNA technology.  Its amino acid sequence is the same as that of naturally occurring human beta-glucuronidase (GUS).

Mucopolysaccharidosis VII (MPS VII or Sly syndrome) is a lysosomal disorder characterized by the deficiency of GUS which results in the accumulation of glycosaminoglycans (GAGs) throughout the body leading to multisystem tissue and organ damage.  Enzyme replacement therapy with vestronidase alfa-vjbk has been shown to reduce urinary excretion of GAGs including chondroitin sulfate and dermatan sulfate, which was sustained with continued treatment.

POLICY

MEDICAL APPROPRIATENESS

INITIAL APPROVAL

Mucopolysaccharidosis VII (MPS VII; Sly syndrome)

RENEWAL CRITERIA

DOSAGE/ADMINISTRATION

INDICATION

DOSE

Mucopolysaccharidosis VII (Sly syndrome)

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

LENGTH OF AUTHORIZATION

Coverage will be provided for 12 months and may be renewed.

DOSAGE LIMITS

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

APPLICABLE TENNESSEE STATE MANDATE REQUIREMENTS

BlueCross BlueShield of Tennessee’s Medical Policy complies with Tennessee Code Annotated Section 56-7-2352 regarding coverage of off-label indications of Food and Drug Administration (FDA) approved drugs when the off-label use is recognized in one of the statutorily recognized standard reference compendia or in the published peer-reviewed medical literature.

IMPORTANT REMINDER

We develop Medical Policies to provide guidance to Members and Providers.  This Medical Policy relates only to the services or supplies described in it.  The existence of a Medical Policy is not an authorization, certification, explanation of benefits or a contract for the service (or supply) that is referenced in the Medical Policy.  For a determination of the benefits that a Member is entitled to receive under his or her health plan, the Member's health plan must be reviewed.  If there is a conflict between the Medical Policy and a health plan, the express terms of the health plan will govern.

ADDITIONAL INFORMATION 

For appropriate chemotherapy regimens, dosage information, contraindications, precautions, warnings, and monitoring information, please refer to one of the standard reference compendia (e.g., the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) published by the National Comprehensive Cancer Network®, Drugdex Evaluations of Micromedex Solutions at Truven Health, or The American Hospital Formulary Service Drug Information).

SOURCES

1.     Mepsevii [package insert]. Novato, CA; Ultragenyx Pharmaceutical Inc.; December 2020. Accessed December 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, blindstart, 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.

5.     Lexicomp Online. (2021). AHFS DI. Vestronidase alfa-vjbk. Retrieved February 10, 2021 from Lexicomp Online with AHFS.

6.     MICROMEDEX Healthcare Series. Drugdex Evaluations. (2019, December). Vestronidase alfa-vjbk. Retrieved February 10, 2021 from MICROMEDEX Healthcare Series.

ORIGINAL EFFECTIVE DATE:  1/18/2018

MOST RECENT REVIEW DATE:    3/9/2021

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Policies included in the Medical Policy Manual are not intended to certify coverage availability. They are medical determinations about a particular technology, service, drug, etc. While a policy or technology may be medically necessary, it could be excluded in a member's benefit plan. Please check with the appropriate claims department to determine if the service in question is a covered service under a particular benefit plan. Use of the Medical Policy Manual is not intended to replace independent medical judgment for treatment of individuals. The content on this Web site is not intended to be a substitute for professional medical advice in any way. Always seek the advice of your physician or other qualified health care provider if you have questions regarding a medical condition or treatment.

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