Buphenyl — Blue Cross Blue Shield of Oklahoma

Preferred products

• generic sodium phenylbutyrate
• Pheburane

Initial criteria

• The patient has a diagnosis of hyperammonemia AND ALL of the following:
• - Elevated plasma ammonia levels according to age (Neonate ≥150 micromol/L; Older child/adult >100 micromol/L)
• - Normal anion gap
• - Normal blood glucose level
• The patient has a confirmed diagnosis by enzyme analysis or genetic testing of ONE of the following urea cycle disorders: carbamoyl phosphate synthetase I deficiency (CPSID), ornithine transcarbamylase deficiency (OTCD), argininosuccinic acid synthetase deficiency (ASSD), argininosuccinic acid lyase deficiency (ASLD), or arginase deficiency (ARG1D)
• The requested agent will NOT be used for acute hyperammonemia
• The patient is unable to maintain plasma ammonia within normal range using a protein-restricted diet and essential amino acid supplementation when appropriate
• The requested agent will be used as adjunctive therapy to dietary protein restriction
• If the requested agent is Buphenyl or Olpruva, ONE of the following applies: currently stable on therapy; inadequate response, discontinuation, or intolerance/hypersensitivity to generic sodium phenylbutyrate; contraindication; expected ineffectiveness or adherence/comorbidity barrier; not in patient’s best medical interest; failure of another drug in same class; or support for brand use over generic
• If the requested agent is Ravicti, ONE of the following applies: currently stable on therapy; inadequate response, discontinuation, or intolerance/hypersensitivity to BOTH generic sodium phenylbutyrate AND Pheburane; contraindication to both; expected ineffectiveness or adherence/comorbidity barrier of both; not in patient’s best medical interest; failure of another drug in same class; or support for brand use over both
• The prescriber is a specialist in metabolic disorders or has consulted one
• The patient has no FDA labeled contraindications to the requested agent
• The requested dose is within FDA labeled dosing for the indication

Reauthorization criteria

• The patient was previously approved for the requested agent through the plan's prior authorization process
• The patient has achieved clinical benefit (e.g., plasma ammonia within normal range)
• The requested agent will NOT be used for acute hyperammonemia
• The requested agent will be used as adjunctive therapy to dietary protein restriction
• Step-therapy criteria as listed for Buphenyl/Olpruva (vs generic sodium phenylbutyrate) or Ravicti (vs generic sodium phenylbutyrate AND Pheburane) remain satisfied
• The prescriber is a specialist in the area of the diagnosis or has consulted one
• The patient has no FDA labeled contraindications to the requested agent
• The requested dose is within FDA labeled limits

Approval duration

12 months