Criteria
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 FDA-approved label for use (e.g., use outside of studied population will be considered investigational);
and
-
Documentation of the member's diagnosis must be submitted, as evidenced by the following:
-
Deficiency of N-acetylgalactosamine 4-sufatase (arylsulfatase B or ASB) enzyme activity of <10% of the lower limit of normal;
and
-
Detection of pathogenic variants in the ARSB gene by molecular genetic testing;
and
-
Galsulfase (Naglazyme) must be prescribed by, or in consult with, an expert in lysosomal storage diseases;
and
-
Documentation of the following must be submitted:
-
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:
-
Six (6) or 12-minute walk test (6-MWT or 12-MWT);
or
-
Three (3)-minute stair climb test;
or
-
Forced Vital Capacity (FVC) via Pulmonary Function Test.
Initial Authorization: Six (6) months
Reauthorization Criteria
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 galsulfase (Naglazyme), as evidenced by medical documentation (e.g., chart notes) attached to the request (subject to clinical review) including maintenance or improvement in the one of the following scores and symptoms:
-
Reduction in urinary excretion of glycosaminoglycans (GAGs);
or
-
Stability or improvement in six (6) or 12-minute walk test (6-MWT or 12-MWT);
or
-
Stability or improvement in three (3)-minute stair climb test;
or
- Stability or improvement in Forced Vital Capacity (FVC) via Pulmonary Function Test
The use of galsulfase (Naglazyme) for all other indications not listed in this policy is considered experimental/investigational and therefore non-covered because the safety and/or effectiveness cannot be established by the available published peer-reviewed literature.
Procedure Code
