Ravicti — Blue Cross Blue Shield of Montana

Preferred products

• generic sodium phenylbutyrate
• Pheburane

Initial criteria

• Patient has a diagnosis of hyperammonemia AND ALL of the following: (A) elevated ammonia levels according to age [neonate ≥150 micromol/L; older child/adult >100 micromol/L]; (B) normal anion gap; (C) normal blood glucose level
• 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)
• Requested agent NOT used for treatment of acute hyperammonemia
• Patient unable to maintain plasma ammonia level within normal range with protein restricted diet and, when clinically appropriate, essential amino acid supplementation
• Requested agent used as adjunctive therapy to dietary protein restriction
• If requested agent is Buphenyl or Olpruva, ONE of the following: (1) currently treated and stable on agent; OR (2) inadequate response to generic sodium phenylbutyrate; OR (3) discontinued generic sodium phenylbutyrate due to lack of efficacy/effectiveness/adverse event; OR (4) intolerance or hypersensitivity to generic sodium phenylbutyrate; OR (5) FDA labeled contraindication to generic sodium phenylbutyrate; OR (6) generic sodium phenylbutyrate expected to be ineffective, cause adherence barrier, worsen comorbid condition, reduce functional ability, or cause harm; OR (7) not in best interest based on medical necessity; OR (8) tried another prescription drug in same pharmacologic class/mechanism with poor outcome; OR (9) support for use of requested brand agent over generic sodium phenylbutyrate
• If requested agent is Ravicti, ONE of the following: (1) currently treated and stable on agent; OR (2) inadequate response to BOTH generic sodium phenylbutyrate AND Pheburane; OR (3) BOTH agents discontinued due to lack of efficacy/effectiveness/adverse event; OR (4) intolerance or hypersensitivity to BOTH agents; OR (5) FDA labeled contraindication to BOTH agents; OR (6) BOTH agents expected to be ineffective, cause adherence barrier, worsen comorbid condition, reduce functional ability, or cause harm; OR (7) BOTH agents not in best interest based on medical necessity; OR (8) tried another prescription drug in same pharmacologic class/mechanism with poor outcome; OR (9) support for use of requested brand agent over BOTH agents
• Prescriber is a specialist in metabolic disorders or has consulted with such a specialist
• No FDA labeled contraindications to the requested agent
• Requested dose within FDA labeled dosing for indication

Reauthorization criteria

• Previously approved for the requested agent through plan’s prior authorization process
• Clinical benefit with the requested agent (e.g., plasma ammonia level within normal range)
• Requested agent NOT used for treatment of acute hyperammonemia
• Requested agent used as adjunctive therapy to dietary protein restriction
• If requested agent is Buphenyl or Olpruva, ONE of the following: currently treated and stable on agent; inadequate response or discontinuation/adverse event/intolerance/FDA contraindication/expected ineffectiveness/medical necessity vs generic sodium phenylbutyrate; tried another same-class drug with poor outcome; OR support for brand agent over generic
• If requested agent is Ravicti, ONE of the following: currently treated and stable on agent; inadequate response or discontinuation/adverse event/intolerance/FDA contraindication/expected ineffectiveness/medical necessity vs BOTH generic sodium phenylbutyrate AND Pheburane; tried another same-class drug with poor outcome; OR support for use of brand agent over both agents
• Prescriber is specialist in metabolic disorders or has consulted with such a specialist
• No FDA labeled contraindications to requested agent
• Requested dose within FDA labeled dosing

Approval duration

12 months