carglumic acid — Blue Cross Blue Shield of Alabama

Initial criteria

• Patient has a diagnosis of N-acetylglutamate synthase (NAGS) deficiency confirmed by enzyme analysis (via liver biopsy) OR genetic testing
• Patient has a diagnosis of hyperammonemia AND ALL of the following:
• - Elevated ammonia levels according to age [neonate: plasma ammonia ≥150 micromol/L (≥260 micrograms/dL); older child or adult: plasma ammonia >100 micromol/L (175 micrograms/dL)]
• - Normal anion gap
• - Normal blood glucose level
• - Unable to maintain a plasma ammonia level within the normal range with a protein restricted diet and, when clinically appropriate, essential amino acid supplementation
• OR ALL of the following:
• - Diagnosis of methylmalonic acidemia (MMA) OR propionic acidemia (PA, PROP)
• - Drug will be used as adjunctive therapy to standard of care for acute hyperammonemia
• - Patient hospitalized with a plasma ammonia level ≥70 micromol/L
• If request is for brand agent when a generic equivalent exists (Carbaglu vs carglumic acid), ONE of the following:
• - Intolerance or hypersensitivity to the generic equivalent not expected to occur with the brand agent
• - FDA labeled contraindication to the generic equivalent not expected to occur with the brand agent
• - Support for use of brand over generic
• Prescriber is a specialist in the area of the patient’s diagnosis (e.g., nephrologist, metabolic disorders) or has consulted with such specialist
• Patient has no FDA labeled contraindications to the requested agent
• Requested dose/quantity is within FDA labeled dosing for the indication

Reauthorization criteria

• Patient has been previously approved for the requested agent through the plan’s Prior Authorization process
• Patient has had clinical benefit with the requested agent

Approval duration

MMA or PA: 1 month; NAGS deficiency: 12 months