sapropterin dihydrochloride — CareFirst (Caremark)
Biopterin metabolic defects (including GTP cyclohydrolase deficiency – autosomal dominant and recessive, 6-PTS deficiency, sepiapterin reductase deficiency, DHPR deficiency, pterin-4a-carbinolamine dehydratase deficiency)
Initial criteria
- For Phenylketonuria (PKU):
- • Member is age ≥ 1 month; AND
- • Member has been diagnosed with phenylketonuria and has a baseline phenylalanine level ≥ 360 micromol/L (6 mg/dL) despite dietary interventions; AND
- • Prescribed by or in consultation with a physician who specializes in the treatment of metabolic disease and/or PKU.
- Note: If Kuvan is initiated in a member currently receiving Palynziq for PKU, then Palynziq will be discontinued after an appropriate overlap period.
- For Biopterin Metabolic Defects (autosomal dominant/recessive GTP cyclohydrolase deficiency, 6-PTS deficiency, sepiapterin reductase deficiency, DHPR deficiency, or pterin-4a-carbinolamine dehydratase deficiency):
- • Member is age ≥ 1 month; AND
- • Diagnosis of one of the above conditions is supported by enzyme assay, genetic testing, or phenylalanine level results.
Reauthorization criteria
- For Phenylketonuria (PKU):
- • Member achieves or maintains ≥ 30% decrease in phenylalanine levels from baseline; OR
- • Phenylalanine levels are < 360 micromol/L (6 mg/dL); OR
- • Member demonstrates improvement in neuropsychiatric symptoms.
- Note: Kuvan should not be used concomitantly with Palynziq for PKU.
- For Biopterin Metabolic Defects:
- • Member is experiencing benefit from therapy as evidenced by disease stability or disease improvement.
Approval duration
Initial: 60 days for PKU; 6 months for Biopterin metabolic defects. Reauthorization: 6 months.