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 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 VII (MPS VII, also known as Sly Syndrome) confirmed by both of the following (as evidenced with submitted documentation):
- Deficiency of beta-glucuronidase enzyme
- Detection of pathogenic variants in the GUSB gene by molecular genetic testing; and
- The provider must submit documentation 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
- 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: 6 months
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 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:
- 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 6-minute walk test (6MWT); or
- Stability or improvement in Forced Vital Capacity (FVC) via Pulmonary Function Test.
Continuation Authorization: 12 months
The use of vestronidase alfa-vjbk (Mepsevii) for any other indication is considered experimental/investigational and therefore, non-covered. There is a lack of clinical data to support its safety and efficacy in the treatment of other conditions.