betaine anhydrous — CareFirst (Caremark)

Initial criteria

• Diagnosis of homocystinuria confirmed by enzyme assay or genetic testing demonstrating one of the following: cystathionine beta-synthase (CBS) deficiency, 5,10-methylenetetrahydrofolate reductase (MTHFR) deficiency, or cobalamin cofactor metabolism (cbl) defect
• If the member has CBS deficiency, plasma methionine concentrations are monitored and kept below 1,000 micromol/L through dietary modification, and if necessary, a reduction in dose for the requested medication
• Medication is prescribed by or in consultation with a physician who specializes in metabolic disease and/or lysosomal storage disorders
• For methylmalonic acidemia with homocystinuria: diagnosis of methylmalonic acidemia with homocystinuria

Reauthorization criteria

• For homocystinuria: total homocysteine level is undetectable or present only in small amounts OR there is a substantial decrease in homocysteine levels and the dose will be increased until maximum tolerability or plasma total homocysteine is undetectable or present in only small amounts
• If the member has CBS deficiency, plasma methionine concentrations are monitored and kept below 1,000 micromol/L through dietary modification, and if necessary, a reduction in dose for the requested medication
• For methylmalonic acidemia with homocystinuria: member is experiencing benefit from therapy as evidenced by disease stability or disease improvement

Approval duration

12 months