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Carbaglu (carglumic acid tablets for oral suspension)Cigna

N-acetylglutamate synthase (NAGS) deficiency with hyperammonemia

Initial criteria

  • Diagnosis is supported by ONE of the following (i or ii):
  • i. Genetic testing confirmed a mutation leading to N-acetylglutamate synthase deficiency; OR
  • ii. Patient has hyperammonemia diagnosed with an ammonia level above the upper limit of the normal reference range for the reporting laboratory;
  • Medication is prescribed in conjunction with a protein-restricted diet; AND
  • Medication is prescribed by or in consultation with a metabolic disease specialist (or specialist who focuses on the treatment of metabolic diseases).

Approval duration

1 year if genetic testing confirmed mutation; 3 months if diagnosis based on hyperammonemia