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Ravicti (glycerol phenylbutyrate)Highmark

Chronic management of urea cycle disorders that cannot be managed by dietary protein restriction and/or amino acid supplementation alone

Preferred products

  • Generic sodium phenylbutyrate
  • Pheburane

Initial criteria

  • The member is using Ravicti for the chronic management of UCDs (ICD-10: E72.20)
  • Documentation of specific protein deficiency subtype (e.g., carbamylphosphate synthetase [CPS], ornithine transcarbamylase [OTC], or argininosuccinic acid synthetase [AS])
  • The UCD cannot be managed by dietary protein restriction and/or amino acid supplementation alone
  • The member is using Ravicti as adjunctive therapy to dietary protein restriction
  • If the member has a deficiency of CPS, OTC, or AS, the member has experienced therapeutic failure, intolerance, or contraindication to both of the following plan-preferred medications: generic sodium phenylbutyrate and Pheburane

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 Ravicti with standard of care (e.g., dietary protein restriction and/or amino acid supplementation)
  • If the member has a deficiency of CPS, OTC, or AS, the member has experienced therapeutic failure, intolerance, or contraindication to both of the following plan-preferred medications: generic sodium phenylbutyrate and Pheburane

Approval duration

12 months