Criteria
Coverage is subject to the specific terms of the member's benefit plan.
The use of idursulfase (Elaprase) may be considered medically necessary when ALL of the following criteria are met:
- The individual has Hunter Syndrome (MPS II) confirmed by one of the following:
- Deficiency in iduronate-2sulfatase (I2S) enzyme activity in white cells, fibroblasts, or plasma in the presence of normal activity of at least one other sulfatase; or
- Genetic testing confirming pathogenic mutations in the IDS gene; and
- The individual age must be 5 years of age or older; and
- The prescriber must be, or in consult with an expert in lysosomal storage diseases; and
- The individual does not have severe cognitive or neurologic impairment (e.g., inability to swallow); and
- Documentation of one of the following must be submitted:
- The Forced Vital Capacity (FVC) via Pulmonary Function Test; or
- Urinary glycosaminoglycan (uGAG) levels are elevated defined by laboratory reference range; or
- 6-minute walk test (6MWT); or
- Hepatomegaly (liver size 1.25 or more times normal); or
- Splenomegaly (spleen size five (5) or more times normal).
Initial Authorization: 6 months
Reauthorization Criteria
Continuation of therapy with idursulfase (Elaprase) may be considered medically necessary when ALL of the following are met:
- The individual does not have severe cognitive or neurologic impairment (e.g. inability to swallow); and
- Documentation must be submitted confirming improvement of one of the following:
- The Forced Vital Capacity (FVC) via Pulmonary Function Test relative improvement of 10% over baseline; or
- Urinary glycosaminoglycan (uGAG) levels normalization defined by laboratory reference range; or
- 6-minute walk test (6MWT) increase; or
- Reduction in liver volume to normal size or by 10%; or
- Reduction in spleen volume by 15%.
Continuation Authorization: 12 months
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.
Procedure Codes
