Criteria
Coverage is subject to the specific terms of the member's benefit plan.
The use of vestronidase alfa-vjbk (Mepsevii) 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
-
Vestronidase alfa-vjbk (Mepsevii) must be prescribed by, or in consult with, an expert in lysosomal storage diseases;
and
-
Documentation of the member's diagnosis must be submitted, as evidenced by the following:
- Deficiency of beta-glucuronidase enzyme
-
Detection of pathogenic variants in the GUSB gene by molecular genetic testing;
and
-
The provider must submit documentation of one or more of the following:
- Skeletal abnormalities
- 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
- Liver and/or spleen volume
- Six (6)-minute walk test (6MWT)
- Motor function test (e.g., Bruininks-Oseretsky Test of Motor Proficiency (BOT-2))
- Forced Vital Capacity (FVC) via Pulmonary Function Test.
Initial Authorization: Six (6) months
Reauthorization Criteria
Continuation of therapy with vestronidase alfa-vjbk (Mepsevii) may be considered medically necessary when the following is met:
-
The individual must have experienced meaningful clinical benefit since starting treatment with vestronidase alfa-vjbk (Mepsevii), 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:
-
Stability or improvement in Skeletal abnormalities shown on x-ray, short stature, macrocephaly;
or
-
Reduction in urinary excretion of glycosaminoglycans (GAGs);
or
-
Reduction in liver and/or spleen volume;
or
-
Stability or improvement in six (6)-minute walk test (6MWT);
or
- Stability or improvement in Forced Vital Capacity (FVC) via Pulmonary Function Test.
The use of vestronidase alfa-vjbk (Mepsevii) 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
