Cystadane — CareFirst (Caremark)

Initial criteria

• Diagnosis of homocystinuria confirmed by enzyme assay or genetic testing showing CBS deficiency, MTHFR deficiency, or cobalamin cofactor metabolism (cbl) defect
• If the member has CBS deficiency, plasma methionine concentrations will be monitored and kept below 1,000 micromol/L through dietary modification and, if necessary, a reduction in dose for the requested medication
• Prescribed by or in consultation with a physician who specializes in the treatment of metabolic disease and/or lysosomal storage disorders

Reauthorization criteria

• 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 will be monitored and kept below 1,000 micromol/L through dietary modification and, if necessary, a reduction in dose for the requested medication

Approval duration

12 months