Buphenyl — Blue Cross Blue Shield of Kansas

Preferred products

• generic sodium phenylbutyrate
• Pheburane

Initial criteria

• 1. The patient has a diagnosis of hyperammonemia AND ALL of the following:
• A. The patient has elevated ammonia levels according to age: Neonate plasma ammonia ≥150 micromol/L (≥260 micrograms/dL); Older child/adult plasma ammonia >100 micromol/L (>175 micrograms/dL) AND
• B. The patient has a normal anion gap AND
• C. The patient has a normal blood glucose level AND
• 2. The patient has a diagnosis of one of the following urea cycle disorders confirmed by enzyme analysis OR genetic testing: carbamoyl phosphate synthetase I deficiency (CPSID), ornithine transcarbamylase deficiency (OTCD), argininosuccinic acid synthetase deficiency (ASSD), argininosuccinic acid lyase deficiency (ASLD), or arginase deficiency (ARG1D) AND
• 3. The requested agent will NOT be used for the treatment of acute hyperammonemia AND
• 4. The patient is unable to maintain a normal plasma ammonia level with protein-restricted diet and appropriate essential amino acid supplementation AND
• 5. The requested agent will be used as adjunctive therapy to dietary protein restriction AND
• 6. ONE of the following:
• A. If the requested agent is Buphenyl or Olpruva, ONE of the following:
• 1. The patient has an intolerance or hypersensitivity to generic sodium phenylbutyrate not expected with the brand agent OR
• 2. The patient has an FDA labeled contraindication to generic sodium phenylbutyrate not expected with the brand agent OR
• 3. There is clinical support for the use of the requested brand agent over generic sodium phenylbutyrate OR
• B. If the requested agent is Ravicti, ONE of the following:
• 1. The patient has tried and had an inadequate response to generic sodium phenylbutyrate AND Pheburane OR
• 2. The patient has an intolerance or hypersensitivity to generic sodium phenylbutyrate AND Pheburane OR
• 3. The patient has an FDA labeled contraindication to generic sodium phenylbutyrate AND Pheburane OR
• 4. There is clinical support for use of the requested brand agent over generic sodium phenylbutyrate AND Pheburane AND
• 7. The prescriber is a specialist in metabolic disorders or has consulted with such a specialist AND
• 8. The patient has no FDA labeled contraindications to the requested agent AND
• 9. Requested dose is within FDA labeled limits

Reauthorization criteria

• 1. The patient has been previously approved for the requested agent through the plan’s Prior Authorization process AND
• 2. The patient has had clinical benefit (e.g., plasma ammonia level within normal range) AND
• 3. The agent will NOT be used to treat acute hyperammonemia AND
• 4. The agent will be used as adjunctive therapy to dietary protein restriction AND
• 5. ONE of the following:
• A. If Buphenyl or Olpruva, ONE of:
• 1. Intolerance/hypersensitivity to generic sodium phenylbutyrate not expected with brand agent OR
• 2. FDA labeled contraindication to generic sodium phenylbutyrate not expected with brand agent OR
• 3. Clinical support for brand agent over generic sodium phenylbutyrate OR
• B. If Ravicti, ONE of:
• 1. Tried and inadequate response to generic sodium phenylbutyrate AND Pheburane OR
• 2. Intolerance/hypersensitivity to generic sodium phenylbutyrate AND Pheburane OR
• 3. FDA labeled contraindication to generic sodium phenylbutyrate AND Pheburane OR
• 4. Clinical support for use of brand agent over generic sodium phenylbutyrate AND Pheburane AND
• 6. Prescriber is specialist in metabolic disorders or has consulted with such a specialist AND
• 7. Patient has no FDA labeled contraindications to the requested agent AND
• 8. Requested dose is within FDA labeled limits

Approval duration

12 months