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Buphenyl — Blue Cross Blue Shield of New Mexico

Urea cycle disorders (carbamoyl phosphate synthetase I deficiency [CPSID]; ornithine transcarbamylase deficiency [OTCD]; argininosuccinic acid synthetase deficiency [ASSD]; argininosuccinic acid lyase deficiency [ASLD]; arginase deficiency [ARG1D])

Preferred products

  • generic sodium phenylbutyrate
  • Pheburane

Initial criteria

  • 1. Patient has a diagnosis of hyperammonemia AND ALL of the following:
  • A. Elevated ammonia levels (Neonate: plasma ammonia ≥150 micromol/L (≥260 micrograms/dL); Older child/adult: plasma ammonia >100 micromol/L (>175 micrograms/dL)) AND
  • B. Normal anion gap AND
  • C. Normal blood glucose level
  • 2. Diagnosis of ONE of the following urea cycle disorders confirmed by enzyme analysis OR genetic testing: CPSID, OTCD, ASSD, ASLD, ARG1D
  • 3. Requested agent will NOT be used for acute hyperammonemia
  • 4. Unable to maintain plasma ammonia within normal range with protein‐restricted diet ± essential amino acid supplementation
  • 5. Requested agent used as adjunctive therapy to dietary protein restriction
  • 6. ONE of the following Brand/Step conditions:
  • A. If requested agent is Buphenyl or Olpruva, ONE of:
  • 1. Patient currently treated and stable on requested agent [chart notes required] OR
  • 2. Tried and inadequate response to generic sodium phenylbutyrate [chart notes required] OR
  • 3. Discontinued generic sodium phenylbutyrate due to lack of efficacy/effectiveness, diminished effect, or adverse event [chart notes required] OR
  • 4. Intolerance/hypersensitivity to generic sodium phenylbutyrate not expected with brand [chart notes required] OR
  • 5. FDA labeled contraindication to generic sodium phenylbutyrate not expected with brand [chart notes required] OR
  • 6. Generic sodium phenylbutyrate expected ineffective, causes adherence barrier, worsens comorbid condition, decreases functional ability, or causes harm [chart notes required] OR
  • 7. Generic sodium phenylbutyrate not in patient’s best interest based on medical necessity [chart notes required] OR
  • 8. Tried another drug in same class/mechanism and discontinued due to lack of efficacy/effectiveness, diminished effect, or adverse event [chart notes required] OR
  • 9. Support for use of requested brand agent over generic sodium phenylbutyrate
  • B. If requested agent is Ravicti, ONE of:
  • 1. Patient currently treated and stable on requested agent [chart notes required] OR
  • 2. Tried and inadequate response to BOTH generic sodium phenylbutyrate AND Pheburane [chart notes required] OR
  • 3. Discontinued generic sodium phenylbutyrate AND Pheburane due to lack of efficacy/effectiveness, diminished effect, or adverse event [chart notes required] OR
  • 4. Intolerance/hypersensitivity to generic sodium phenylbutyrate AND Pheburane [chart notes required] OR
  • 5. FDA labeled contraindication to generic sodium phenylbutyrate AND Pheburane [chart notes required] OR
  • 6. Generic sodium phenylbutyrate AND Pheburane expected ineffective, cause adherence barrier, worsen comorbid condition, decrease functioning, or cause harm [chart notes required] OR
  • 7. Generic sodium phenylbutyrate AND Pheburane not in best interest based on medical necessity [chart notes required] OR
  • 8. Tried another drug in same class/mechanism as generic sodium phenylbutyrate AND Pheburane and discontinued due to lack of efficacy/effectiveness, diminished effect, or adverse event [chart notes required] OR
  • 9. Support for use of requested brand agent over generic sodium phenylbutyrate AND Pheburane
  • 7. Prescriber is specialist in metabolic disorders or has consulted with one
  • 8. No FDA labeled contraindications to requested agent
  • 9. Requested dose within FDA labeled limits
  • Length of Approval: 12 months

Reauthorization criteria

  • 1. Patient previously approved for requested agent through plan’s PA process
  • 2. Patient has had clinical benefit (e.g., plasma ammonia within normal range)
  • 3. Requested agent not used for acute hyperammonemia
  • 4. Agent used as adjunctive therapy to dietary protein restriction
  • 5. ONE of the following Brand/Step conditions applies:
  • A. If requested agent is Buphenyl or Olpruva, same criteria as initial (current stable use OR inadequate response/intolerance/contraindication/etc. to generic sodium phenylbutyrate)
  • B. If requested agent is Ravicti, same criteria as initial (current stable use OR inadequate response/intolerance/contraindication/etc. to BOTH generic sodium phenylbutyrate AND Pheburane)
  • 6. Prescriber specialist or consult with specialist in diagnosis area
  • 7. No FDA labeled contraindications
  • 8. Dose within FDA labeling
  • Length of Approval: 12 months

Approval duration

12 months