Medical Policy

Effective Date:06/01/2011 Title:Carbaglu (Carglumic Acid)
Revision Date:10/01/2014 Document:BI294:00
CPT Code(s):None
Public Statement



 Effective Date:

a)    This policy will apply to all services performed on or after the above revision date which will become the new effective date.

b)    For all services referred to in this policy that were performed before the revision date, contact customer service for the rules that would apply.

1)    Carbaglu (carglumic acid) requires prior authorization.

2)    Carbaglu is considered a specialty medication and must be obtained through a contracted specialty pharmacy.

Medical Statement
Carbaglu is considered medically necessary for patients who meet the following criteria: 1) Diagnosis of hepatic-N-acetyl glutamate (NAGS) deficiency confirmed by genetic/mutational analysis AND 2) Patient is experiencing either acute or chronic hyperammonemia.


Carbaglu is limited to coverage under the pharmacy benefit through the contracted specialty pharmacy.



  1. Carbaglu Product Information.  Orphan Europe SAL.  March 2010
  2. Caldovic L, Ah Mew NA, Shi D, Morixono H, Yudkoff M, Tuchman M.  N-atetylglutamate synthase: structure, function, and defects [published online ahead of print February 26, 2010].  Mol Genet Metab. 2010; 100(suppl 1):S13-S19.  Doi:  10.1016/j.ymgme.2010.02.018
  3. Ah Mew N, Payan I, Daikhin Y, et al.  Effects of a single dose of N-carbmylglutamate on the rate of ureagenesis.  Mol Genet Metab.  2009;98(4):325-330.
  4. Belanger-Quintana A, Martinez-Pardo M, Garcia MJ, et al.  Hyperammonaemia as a cause of psychosis in an adolescent.  Eur J Pediatr.  2003;162(11):773-775
Application to Products
This policy applies to all health plans administered by QualChoice, both those insured by QualChoice and those that are self-funded by the sponsoring employer, unless there is indication in this policy otherwise or a stated exclusion in your medical plan booklet. Consult the individual plan sponsor Summary Plan Description (SPD) for self-insured plans or the specific Evidence of Coverage (EOC) for those plans insured by QualChoice. In the event of a discrepancy between this policy and a self-insured customer’s SPD or the specific QualChoice EOC, the SPD or EOC, as applicable, will prevail. State and federal mandates will be followed as they apply.

Changes: QualChoice reserves the right to alter, amend, change or supplement benefit interpretations as needed.