Coverage is subject to the specific terms of the member's benefit plan.
The use of galsulfase (Naglazyme) may be considered medically necessary when ALL of the following criteria are met:
- The individual must meet criteria as outlined in prescribing information (PI) including recommendations for diagnosis and age; and
- The prescriber is a, or in consult with, a metabolic or genetic specialist; and
- The individual must have a diagnosis mucopolysaccharidosis VI (MPS VI, also known as Maroteaux-Lamy syndrome) confirmed by both of the following (as evidenced with submitted documentation):
- Deficiency of N-acetylgalactosamine 4-sufatase (arylsulfatase B or ASB) enzyme activity of <10% of the lower limit of normal
- Detection of pathogenic variants in the ARSB gene by molecular genetic testing; and
- The provider must submit documentation both of the following:
- Elevated level of urinary excretion of glycosaminoglycans (GAGs) such as chondroitin sulfate and dermatan sulfate, as defined by being above the upper limit of normal by the laboratory reference range; and
- Motor function as measured by one of the following:
- 6 or 12-minute walk test (6-MWT or 12-MWT); or
- 3-minute stair claim test; or
- Forced Vital Capacity (FVC) via Pulmonary Function Test.
Initial Authorization: 6 months
Continuation of therapy with galsulfase (Naglazyme) may be considered medically necessary when the following is met:
- The individual must have experienced meaningful clinical benefit since starting treatment with the requested medication, as evidenced by medical documentation (e.g. chart notes) attached to the request (subject to clinical review) including improvement in the one of the following scores and symptoms:
- Reduction in urinary excretion of glycosaminoglycans (GAGs); or
- Stability or improvement in 6 or 12-minute walk test (6-MWT or 12-MWT); or
- Stability or improvement in 3-minute stair claim test; or
- Stability or improvement in Forced Vital Capacity (FVC) via Pulmonary Function Test.
Continuation Authorization: 12 months
The use of galsulfase (Naglazyme) for any other indication, is considered experimental/investigational and therefore, non-covered, due to lack of supporting published peer reviewed literature.