Idursulfase (Elaprase®), is a purified form of human iduronate-2-sulfatase used for the treatment of Hunter syndrome (Mucopolysaccharidosis II, MPS II) a rare, genetic disease which can lead to premature death. Hunter syndrome is characterized by glycosaminoglycan accumulation due to deficiency in the enzyme iduronate 2-sulfase, which is needed to adequately break down complex sugars produced in the body. Hunter syndrome usually becomes apparent in children one (1) to three (3) years of age. Symptoms include cognitive deterioration, growth delay, joint stiffness, and coarsening of facial features. In severe cases, individuals experience respiratory and cardiac problems, neurological deficits, and death. Individuals with the syndrome do not produce enough of a functional lysosomal enzyme called iduronate-2-sulfatase (IDS) because of this genetic defect.
Coverage is subject to the specific terms of the member's benefit plan.
Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the www.fepblue.org website.
Idursulfase (Elaprase) may be considered medically necessary for use in individuals with Hunter syndrome (Mucopolysaccharidosis II, MPS II) when the following criteria are met.
Continuation therapy with Idursulfase (Elaprase) may be considered medically necessary when ANY of the following are met:
The use of idursulfase (Elaprase) for any indication other than Hunter syndrome is considered experimental/investigational, and therefore, not covered. The safety and efficacy for any other indication has not been established.
NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines. Blue Cross Blue Shield of North Dakota may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.
Original Effective Date July 1, 2018
Internal Medical Policy Committee 5-19-2020 Updated description, added additional criteria, added reauthorization criteria and change to precertification drug
Current medical policy is to be used in determining a Member's contract benefits on the date that services are rendered. Contract language, including definitions and specific inclusions/exclusions, as well as state and federal law, must be considered in determining eligibility for coverage. Members must consult their applicable benefit plans or contact a Member Services representative for specific coverage information. Likewise, medical policy, which addresses the issue(s) in any specific case, should be considered before utilizing medical opinion in adjudication. Medical technology is constantly evolving and the Company reserves the right to review and update medical policy periodically.