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Buphenyl — Blue Cross Blue Shield of Texas

hyperammonemia due to urea cycle disorder (carbamoyl phosphate synthetase I deficiency, ornithine transcarbamylase deficiency, argininosuccinic acid synthetase deficiency, argininosuccinic acid lyase deficiency, arginase deficiency)

Preferred products

  • generic sodium phenylbutyrate
  • Pheburane

Initial criteria

  • Diagnosis of hyperammonemia AND elevated plasma ammonia levels according to age (neonate ≥150 micromol/L; older child/adult >100 micromol/L)
  • Normal anion gap AND normal blood glucose level
  • Diagnosis of one urea cycle disorder confirmed by enzyme analysis or genetic testing: carbamoyl phosphate synthetase I deficiency OR ornithine transcarbamylase deficiency OR argininosuccinic acid synthetase deficiency OR argininosuccinic acid lyase deficiency OR arginase deficiency
  • Requested agent will NOT be used for treatment of acute hyperammonemia
  • Unable to maintain normal plasma ammonia with protein restricted diet ± essential amino acid supplementation
  • Will use requested agent as adjunct to dietary protein restriction
  • If Buphenyl or Olpruva: patient stable on requested agent OR inadequate response/intolerance/contraindication/adverse event/medical necessity/expected inefficacy to generic sodium phenylbutyrate OR tried another agent in same class OR support for brand over generic
  • If Ravicti: patient stable on requested agent OR inadequate response/intolerance/contraindication/adverse event/medical necessity/expected inefficacy to both generic sodium phenylbutyrate AND Pheburane OR tried another agent in same class OR support for brand over generic sodium phenylbutyrate AND Pheburane
  • Prescriber is or has consulted a specialist in metabolic disorders
  • Patient has no FDA labeled contraindications to requested agent
  • Requested dose within FDA labeled dosing

Reauthorization criteria

  • Previously approved for requested agent through plan prior authorization
  • Clinical benefit shown (e.g., plasma ammonia within normal range)
  • Will not be used for acute hyperammonemia
  • Will be used as adjunct to dietary protein restriction
  • If Buphenyl or Olpruva: patient stable on requested agent OR inadequate response/intolerance/contraindication/adverse event/medical necessity/expected inefficacy to generic sodium phenylbutyrate OR tried another agent in same class OR support for brand over generic
  • If Ravicti: patient stable on requested agent OR inadequate response/intolerance/contraindication/adverse event/medical necessity/expected inefficacy to both generic sodium phenylbutyrate AND Pheburane OR tried another agent in same class OR support for brand over generic sodium phenylbutyrate AND Pheburane
  • Prescriber is or has consulted a specialist in metabolic disorders
  • No FDA labeled contraindications
  • Requested dose within FDA labeled dosing

Approval duration

12 months