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Pheburane (sodium phenylbutyrate)Highmark

Chronic management of urea cycle disorders involving deficiencies of carbamylphosphate synthetase (CPS), argininosuccinic acid synthetase (AS), or ornithine transcarbamylase (OTC)

Preferred products

  • Generic sodium phenylbutyrate

Initial criteria

  • The member is using Buphenyl or Pheburane for chronic management of urea cycle disorders (ICD-10: E72.20)
  • The member is using Buphenyl or Pheburane involving deficiencies of carbamylphosphate synthetase (CPS), argininosuccinic acid synthetase (AS), or ornithine transcarbamylase (OTC)
  • The member is using Buphenyl or Pheburane as adjunctive therapy to dietary protein restriction
  • If the request is for brand Buphenyl, the member has experienced therapeutic failure, intolerance, or contraindication to generic sodium phenylbutyrate
  • If the request is for brand Pheburane, the member has experienced therapeutic failure, intolerance, or contraindication to plan-preferred generic sodium phenylbutyrate

Reauthorization criteria

  • The prescriber attests that the member has experienced positive clinical response to therapy (e.g., reduction in blood ammonia levels)
  • The member is continuing use of requested drug with standard of care (e.g., dietary protein restriction and/or amino acid supplementation)

Approval duration

12 months