BlueCross BlueShield of Tennessee Medical Policy Manual

Idursulfase

NDC CODE(S)

54092-0700-xx Elaprase 6mg/3ml solution for injection Shire

DESCRIPTION

Idursulfase is a purified form of the lysosomal enzyme iduronate-2-sulfatase.  It is produced by recombinant DNA technology in a human cell line. This enzyme is required for systemic elimination of the glycosaminoglycans (GAGs) dermatan sulfate and heparan sulfate.  If the enzyme is missing or defective, GAGs progressively accumulate in the lysosomes of nearly all cell types, leading to cellular engorgement, organomegaly, tissue destruction and organ system dysfunction. This condition is known as Mucopolysaccharidosis II, MPS II or Hunter syndrome. A rare disease, it is the only known X-linked recessive mucopolysaccharidosis disorder.

MPS II is generally manifest in two forms:  Severe disease, affecting two-thirds of those diagnosed, in which death typically occurs in the mid-teenage years due to chronic progressive disease of neurological deterioration and cardiorespiratory failure.  The other third have attenuated disease with survival into adulthood, although death frequently occurs between the ages of 20 and 30 years from cardiac or respiratory disease.

While not curative, enzyme replacement therapy with idursulfase can improve quality of life if administered early in the disease state.  Additional supportive treatment is generally required for symptomatic control.

REFER TO DECISION SUPPORT TREE

POLICY

MEDICAL APPROPRIATENESS

INITIAL APPROVAL

RENEWAL CRITERIA

INDICATION(S) DOSAGE & ADMINISTRATION
Mucopolysaccharidosis II (i.e., MPS II, Hunter syndrome) 0.5mg/kg every week

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 dosage information, contraindications, precautions, warnings, and monitoring information, please refer to one of the standard reference compendia (e.g., The American Hospital Formulary Service Drug Information).

No controlled studies were found in the published literature that validate the use of idursulfase in the treatment/prevention of any other conditions/diseases.

SOURCES

Lexicomp Online. (2016, March). AHFS DI. Idursulfase. Retrieved June 21, 2016 from Lexicomp Online with AHFS.

MICROMEDEX  Healthcare Series.Drugdex Drug Evaluations. (2016). Idursulfase. Retrieved June 21, 2016 from MICROMEDEX  Healthcare Series.

U.S. Food and Drug Administration.  (2013, October). Center for Drug Evaluation and Research. Elaprase® (idursulfase). Retrieved June 21, 2016 from http://www.accessdata.fda.gov/drugsatfda_docs/label/2013/125151s0184lbl.pdf.

ORIGINAL EFFECTIVE DATE:  12/1/2016

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

Idursulfase (Elaprase®)

  1. Is this the initial request for this agent?

If yes, go to question #2

If no, go to question #5

  1. Is the individual 16 months of age or older diagnosed with mucopolysaccharidosis II (Hunter syndrome, MPS II)?

If yes, go to question #3

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

  1. Are there documented outcomes of ANY ONE of the following?

If yes, go to question #4

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

  1. Are the units requested 60 billable units or less (1 billable unit = 1 mg) every 7 days for dosage of 0.5mg/kg every week in an authorization of 6 months or less?

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 show improved documented outcomes in ANY ONE of the following?

If yes, go to question #6

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

  1. Does the individual show absence of unacceptable toxicity from the agent, including hypersensitivity reaction such as anaphylaxis

If yes, go to question #7

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

  1. Are the units requested 60 billable units or less (1 billable unit = 1 mg) every 7 days for dosage of 0.5mg/kg every week in an authorization of 6 months or less?

If yes, this satisfies medical necessity and medical appropriateness criteria

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

This document has been classified as public information.