Pharmacy Prior Authorization Criteria

Carbaglu (carglumic acid)

Indications for Prior Authorization

Carbaglu (carglumic acid) tablets for oral suspension

• For diagnosis of Acute Hyperammonemia due to N-acetylglutamate Synthase (NAGS) Deficiency
  Indicated in pediatric and adult patients as adjunctive therapy to standard of care for the treatment of acute hyperammonemia due to NAGS deficiency.

• For diagnosis of Chronic Hyperammonemia due to N-acetylglutamate Synthase (NAGS) Deficiency
  Indicated in pediatric and adult patients as maintenance therapy for the treatment of chronic hyperammonemia due to NAGS deficiency.

• For diagnosis of Acute Hyperammonemia due to Propionic Acidemia (PA) or Methylmalonic Acidemia (MMA)
  Indicated in pediatric and adult patients as adjunctive therapy to standard of care for the treatment of acute hyperammonemia due to propionic acidemia (PA) or methylmalonic acidemia (MMA).

Criteria

Brand Carbaglu, Generic carglumic acid

Prior Authorization

Length of Approval: 3 Month(s)
For diagnosis of Acute Hyperammonemia due to N-acetylglutamate Synthase (NAGS) Deficiency

• Diagnosis of acute hyperammonemia due to N-acetylglutamate synthase (NAGS) deficiency
AND
• Medication will be used as adjunctive therapy to other ammonia lowering therapies (e.g., protein restriction, ammonia scavengers, dialysis)
AND
• Prescribed by or in consultation with a specialist focused in the treatment of metabolic disorders

Brand Carbaglu, Generic carglumic acid

Prior Authorization

Length of Approval: 1 Month(s)
For diagnosis of Acute Hyperammonemia due to Propionic Acidemia (PA) or Methylmalonic Acidemia (MMA)

• Diagnosis of acute hyperammonemia due to propionic acidemia (PA) or methylmalonic acidemia (MMA)
AND
• Medication will be used as adjunctive therapy to other ammonia lowering therapies (e.g. intravenous glucose, insulin, protein restriction, dialysis)
AND
• Patient’s plasma ammonia level is greater than or equal to 50 micromol/L
AND
• Medication will be used for a maximum duration of 7 days
AND
• Prescribed by or in consultation with a specialist focused in the treatment of metabolic disorders

Brand Carbaglu, Generic carglumic acid

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)
For diagnosis of Chronic Hyperammonemia due to N-acetylglutamate Synthase (NAGS) Deficiency

• Diagnosis of chronic hyperammonemia due to N-acetylglutamate synthase (NAGS) deficiency
AND
• NAGS deficiency has been confirmed by genetic/mutational analysis
AND
• Medication will be used as maintenance therapy
AND
• Prescribed by or in consultation with a specialist focused in the treatment of metabolic disorders

Brand Carbaglu, Generic carglumic acid

Prior Authorization (Reauthorization)

Length of Approval: 12 Month(s)
For diagnosis of Chronic Hyperammonemia due to N-acetylglutamate Synthase (NAGS) Deficiency

• Documentation of a positive clinical response to therapy (e.g., plasma ammonia level within the normal range)

P & T Revisions

2024-01-29, 2023-01-20, 2022-04-19, 2022-04-19, 2022-01-31

References

1. Carbaglu tablet, for suspension. Recordati Rare Diseases Inc , Lebanon, NJ, September 2021.
2. Kenneson, A., Singh, R.H. Presentation and management of N-acetylglutamate synthase deficiency: a review of the literature. Orphanet J Rare Dis 15, 279 (2020).
3. Baumgartner MR, Hörster F, Dionisi-Vici C, et al. Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia. Orphanet J Rare Dis. 2014;9:130.

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Revision History

• 2024-01-29: Annual Review - No criteria changes
• 2023-01-20: Annual Review - No criteria changes
• 2022-04-19: GPI - reclassification
• 2022-04-19: GPI - reclassification
• 2022-01-31: New UM Program for generic carglumic acid

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