BlueCross BlueShield of Tennessee Medical Policy Manual

Idursulfase

NDC CODE(S)

54092-0700-XX Elaprase 6MG/3ML SOLN (SHIRE US INC)

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.

POLICY

MEDICAL APPROPRIATENESS

INITIAL APPROVAL

RENEWAL CRITERIA

INDICATION(S) DOSAGE & ADMINISTRATION
Mucopolysaccharidosis II (i.e., MPS II, Hunter syndrome) 0.5mg/kg 0.5 mg/kg of body weight administered once weekly as an intravenous infusion

LENGTH OF AUTHORIZATION

Coverage will be provided for 12 months and may be renewed

Refer to DOSAGE LIMITS below

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

Lexicomp Online. (2018). AHFS DI. Idursulfase. Retrieved November 5, 2018 from Lexicomp Online with AHFS.

MICROMEDEX  Healthcare Series. Drugdex Drug Evaluations. (2018, September).Idursulfase.Retrieved November 5, 2018 from MICROMEDEX  Healthcare Series.

Scarpa, M. (2007, November Updated 2018, October). Mucopolysaccharidosis Type II. In: Adam, M. P., Ardinger, H. H., Pagon, R. A., et al., eds., GeneReviews® (Internet). Seattle (WA): University of Washington, Seattle; 1993-2018. Retrieved November 2, 2018 from https://www.ncbi.nlm.nih.gov/books/NBK1274/.  

U.S. Food and Drug Administration.  (2013, June). Center for Drug Evaluation and Research. Elaprase® (idursulfase). Retrieved November 5, 2018 from https://www.accessdata.fda.gov/drugsatfda_docs/label/2013/125151s0184lbl.pdf.

ORIGINAL EFFECTIVE DATE: 12/1/201

MOST RECENT REVIEW DATE:  4/9/2019

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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.

This document has been classified as public information.

 

 

 

DOSAGE LIMITS

Maximum billable units per dose and over time by indication as a Medical Benefit; 1 billable unit = 1 mg

DIAGNOSIS

MAXIMUM UNITS

Hunter Syndrome, MPS-II

60 billable units every 7 days