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
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.