BlueCross BlueShield of Tennessee Medical Policy Manual

Velaglucerase Alfa

NDC CODE(S)

54092-0701-XX - Vpriv 400 unit powder for injection (Shire)

DESCRIPTION

Velaglucerase alfa is an enzyme replacement agent.  It is a hydrolytic lysosomal glucocerebroside-specific enzyme indicated for long-term enzyme replacement therapy (ERT) for individuals with type I Gaucher disease.  Gaucher disease generally refers to three forms of lysosomal storage disorders (LSD) resulting from the inherited deficiency of the enzyme beta-glucocerebrosidase which cleaves glucose residue from ceramide.  Without the natural enzyme, glucocerebroside accumulates in the body, primarily in circulating phagocytes but also in the central nervous system (CNS).  The overload of stored glucocerebroside and the attendant activation of macrophages with secretion of cytokines cause the pathology of Gaucher disease.

Type I Gaucher disease is the chronic, non-neuronopathic form of the disease and accounts for nearly 99% of cases.  The glucocerebroside storage is limited to phagocytes throughout the body without brain involvement and can manifest at any age.  Type II, or acute neuronopathic Gaucher disease, is the infantile cerebral pattern with progressive CNS involvement and early fatality.  Type III is subacute neuronopathic disease, an intermediate form between types I and II, with onset in adolescence or early adulthood.

REFER TO DECISION SUPPORT TREE

POLICY

MEDICAL APPROPRIATENESS

INITIAL APPROVAL

RENEWAL CRITERIA

INDICATION(S)

DOSAGE & ADMINISTRATION

Type 1 Gaucher Disease

Up to 60 units/kg every other week as a 60-minute IV infusion

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).

A review of the published literature found no controlled studies that validate the use of velaglucerase alfa in the treatment/prevention of any other conditions/diseases

SOURCES

Lexicomp Online. (2016). AHFS DI. Velaglucerase Alfa. Retrieved November 30, 2016 from Lexicomp Online with AHFS DI.

MICROMEDEX Healthcare Series. Drugdex Evaluations. (2016, August). Velaglucerase alfa. Retrieved November 30, 2016 from MICROMEDEX Healthcare Series.

U. S. Food and Drug Administration. (2015, May). Center for Drug Evaluation and Research. VPRIV™ (velaglucerase alfa). Retrieved November 30, 2016 from http://www.accessdata.fda.gov/drugsatfda_docs/label/2015/022575s017lbl.pdf.

ORIGINAL EFFECTIVE DATE:  9/12/2010

MOST RECENT REVIEW DATE:  2/27/2018

ID_MRx

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.

Pharmaceutical Decision Support Tree

Velaglucerase Alfa (Vpriv ®)

  1. Is this the initial request for this agent?

If yes, go to question #2

If no, go to question #7

  1. Is the individual 4 years of age or older with a documented diagnosis of type I Gaucher disease (non-neuronopathic form)?

If yes, go to question #3

If no, this does not meet medical necessity and/or medical appropriateness criteria

  1. Is the individual 18 years of age or older?

If yes, go to question #4

If no, go to question #5

  1. Does the disease result in a minimum of ANY ONE of the following?

If yes, go to question #5

If no, this does not meet medical necessity and/or medical appropriateness criteria

  1. Will treatment be as a single agent?

If yes, go to question #6

If no, this does not meet medical necessity and/or medical appropriateness criteria

  1. Is the request for 72 billable units every 14 days for dosage up to 60 units/kg every other week as a 60 minute IV infusion for an authorization period of 12 months with possible renewal?

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

  1. Does the individual continue to meet the initial approval criteria in questions 2 through 6? 

If yes, go to question #8

If no, this does not meet medical necessity and/or medical appropriateness criteria

  1. Is response to treatment indicated by ANY ONE of the following as compared to pre-treatment baseline?

If yes, go to question #9

If no, this does not meet medical necessity and/or medical appropriateness criteria

  1. Is there absence of unacceptable toxicity from the drug including hypersensitivity reactions?

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

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