Criteria
Coverage is subject to the specific terms of the member's benefit plan.
Velmanase alfa-tycv (Lamzede) may be considered medically necessary when an individual meets the following criteria:
- The individual must meet FDA-approved label for use (e.g., use outside of studied population will be considered investigational); and
- Documentation of the individual's diagnosis must be submitted, as evidenced by one of the following:
- Deficiency of alpha-mannosidase activity in leukocytes or fibroblasts less than 10% of normal activity; or
- Detection of biallelic pathogenic variants in the MAN2B1 gene by molecular genetic testing; and
- The requested medication must be prescribed by, or in consult with, a specialist in lysosomal storage diseases; and
- Documentation of all of the following must be submitted:
- Non-central nervous system manifestations (e.g., progressive motor function disturbances, physical disability, hearing and speech impairment, skeletal abnormalities, and immune deficiency); and
- Elevated level of serum oligosaccharide concentration, as defined by being above the upper limit of normal by the laboratory reference range; and
- If six (6) years of age or older, must be able to walk without support; and
- Motor function as measured by one of the following:
- Six (6)-minute walk test (6-MWT) for four (4) years of age and older; or
- Two (2)-minute walk test (2-MWT) for under four (4) years of age; or
- Three (3)-minute stair climb test; or
- Forced Vital Capacity (FVC) via Pulmonary Function Test.
Reauthorization Criteria
Continuation of therapy with velmanase alfa-tycv (Lamzede) 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 in medical documentation (e.g., chart notes) attached to the request (subject to clinical review) by both of the following:
- Reduction in serum oligosaccharide concentration; and
- Stability or improvement in the one of the following scores and symptoms:
- Six (6)-minute walk test (6-MWT) for four (4) years of age and older; or
- Two (2)-minute walk test (2-MWT) for under four (4) years of age; or
- Three (3)-minute stair climb test; or
- Forced Vital Capacity (FVC) via Pulmonary Function Test.
The use of velmanase alfa-tycv (Lamzede) 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.
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