a devoted [100:<100 mg/kg daily. 1 Round total daily dosage to nearest 100 mg (i.e., half-tablet dosage strength) and administer in 2 4 divided doses. 1 12 Adjust dosage to maintain a target normal plasma ammonia concentration. 1 3 Special Populations No special population dosage recommendations at this time. 1 Cautions for Carbaglu Contraindications Manufacturer states none known. 1 Warnings/Precautions Emergency Treatment of Hyperammonemia Risk of rapid brain damage or death if acute symptomatic hyperammonemia is left uncontrolled; treat as a life-threatening emergency. 1 3 4 5 8 9 Prompt use of all necessary therapies to reduce serum ammonia concentrations (e.g., dialysis [preferably hemodialysis]) is essential. 1 3 4 5 8 9 Caloric supplementation recommended to reverse catabolism and reduce protein turnover; complete restriction of dietary protein recommended for 24 48 hours. 1 4 5 8 9 (See Hyperammonemia under Uses.) Manage hyperammonemia due to inborn errors of metabolism in coordination with medical personnel familiar with such conditions; usually requires health-care facilities able to provide multidisciplinary treatment (e.g., hemodialysis, nutritional management, medical support). 1 4 5 8 9 Laboratory Monitoring Closely monitor plasma ammonia concentrations, neurologic status, laboratory tests, and clinical response during treatment. 1 Nutritional Management Protein restriction and hypercaloric intake recommended to block ammonia-generating catabolic pathways during acute hyperammonemic episodes. 1 4 5 8 9 Dietary protein intake may be increased when plasma ammonia concentrations normalize, with goal of unrestricted protein intake. 1 3 4 Specific Populations Pregnancy Category C. 1 Lactation Distributed into milk in rats; not known whether distributed into human milk. 1 Use not recommended because of potential risk to nursing infants. 1 3 Pediatric Use Efficacy evaluated in neonates, infants, and children. 1 No substantial differences in response relative to adults; however, data limited. 1 Geriatric Use Safety and efficacy not established. 1 Common Adverse Effects Infections (e.g., tonsilitis, ear infection, nasopharyngitis), vomiting, abdominal pain, diarrhea, fever, anemia, headache. 1 Interactions for Carbaglu No formal drug interaction studies to date. 1 Carbaglu Pharmacokinetics Absorption Bioavailability Peak plasma concentration usually attained within 2 4 hours. 1 Absolute bioavailability not determined. 1 Onset Ammonia concentrations decrease within 24 hours. 1 Distribution Distributed into milk in rats; not known whether distributed into human milk. 1 Elimination Metabolism Part of administered dose may be metabolized by intestinal bacteria to form carbon dioxide. 1 Elimination Route Excreted in feces ( 60%) and in urine (9%) as unchanged drug and through the lungs as carbon dioxide. 1 Half-life Median terminal half-life: 5.6 hours. 1 Stability Storage Oral Tablets Unopened container: 2 8 C. 1 Opened container: Store tightly closed at 30 C; do not refrigerate. 1 Discard 1 month after first opening container. 1 Actions Structurally similar to NAG, an activator of carbamoyl phosphate synthetase 1 (CPS 1), the first enzyme in the urea cycle. 1 3 Replaces NAG in the urea cycle. 1 3 Restores activity of the urea cycle, normalizes plasma ammonia concentrations, and increases urea production in patients with deficiency of NAGS (a mitochondrial enzyme that produces NAG). 1 3 Normal neurologic development may be possible if therapy is started early (before irreversible brain damage). 2 Advice to Patients Importance of instructing patients and/or their caregivers regarding proper dosage, preparation, and administration. 1 10 Importance of not crushing tablets or swallowing tablets whole. 1 Importance of dispersing each tablet in 2.5 mL of water and immediately swallowing or administering mixture. 1 Importance of informing patients and/or their caregivers that treatment is continuous and lifelong for patients with NAGS deficiency. 1 Importance of women informing clinicians if they are or plan to become pregnant or plan to breast-feed. 1 Importance of informing clinicians of existing or contemplated concomitant therapy, including prescription and OTC drugs and dietary or herbal supplements, as well as any concomitant illnesses. 1 Importance of informing patients and/or their caregivers of other important precautionary information. 1 (See Cautions.) Preparations Excipients in commercially available drug preparations may have clinically important effects in some individuals; consult specific product labeling for details. Please refer to the ASHP Drug Shortages Resource Center for information on shortages of one or more of these preparations. Distribution of carglumic acid is restricted. 11 (See Restricted Distribution under Dosage and Administration.) Carglumic Acid Routes Dosage Forms Strengths Brand Names Manufacturer Oral Tablets, dispersible, scored 200 mg Carbaglu Accredo AHFS DI Essentials. Copyright 2017, Selected Revisions May 1, 2013. American Society of Health-System Pharmacists, Inc., 4500 East-West Highway, Suite 900, Bethesda, Maryland 20814. References 1. Accredo Health Group, Inc. Carbaglu (carglumic acid) tablets prescribing information. Memphis, TN; 2012 May. 2. US Food and Drug Administration. Center for Drug Evaluation and Research. Application number 22-562: Medical review(s). From FDA website. 3. Häberle J. Role of carglumic acid in the treatment of acute hyperammonemia due to N-acetylglutamate synthase deficiency. Ther Clin Risk Manag . 2011; 7:327-32. [PubMed 21941437] 4. Häberle J, Boddaert N, Burlina A et al. Suggested guidelines for the diagnosis and management of urea cycle disorders. Orphanet J Rare Dis . 2012; 7:32. [PubMed 22642880] 5. Lanpher BC, Gropman A, Chapman KA et al. Urea cycle disorders overview. In: Pagon RA, Bird TD, Dolan CR et al., eds. GeneReviews [Internet]. Seattle: University of Washington, Seattle; 2011 Sep 1. 6. Food and Drug Administration. FDA Application: Search Orphan Drug Designations and Approvals. Rockville, MD. From FDA website. Accessed 2012 Nov 27. 7. Summar M, Tuchman M. Proceedings of a consensus conference for the management of patients with urea cycle disorders. J Pediatr . 2001; 138(1 Suppl):S6-10. 8. Summar M. Current strategies for the management of neonatal urea cycle disorders. J Pediatr . 2001; 138(1 Suppl):S30-9. 9. Urea Cycle Disorders Conference group.. Consensus statement from a conference for the management of patients with urea cycle disorders. J Pediatr . 2001; 138(1 Suppl):S1-5. 10. Orphan Europe. How to use Carbaglu. From Carbaglu website. Accessed 2013 Jan 15. 11. Orphan Europe. Ordering Carbaglu. From Carbaglu website. Accessed 2013 Jan 18. 12. Accredo Health Group. Carbaglu (carglumic acid). From Accredo website. Accessed Jan 18. Next Pregnancy Warnings Print this page Add to My Med List More about Carbaglu (carglumic acid) Side Effects During Pregnancy Dosage Information Support Group Pricing & Coupons En Español 0 Reviews Add your own review/rating Drug class: miscellaneous uncategorized agents Consumer resources Carbaglu Carbaglu (Advanced Reading) Professional resources Carbaglu (FDA) Carglumic Acid (AHFS Monograph) Related treatment guides Hyperammonemia> ] FEATURED: CAR-T Cell Therapy Overview Mechanism of Action KTE-C19 Studies KTE-C19 Cancer Targets Adverse Events Manufacturing Drug Status Rx Availability Prescription only C Pregnancy Category Risk cannot be ruled out N/A CSA Schedule Not a controlled drug 7 years Approval History FDA approved 2010 Drug Class Miscellaneous uncategorized agents Related Drugs Hyperammonemia sodium benzoate / sodium phenylacetate , carglumic acid , More... Carbaglu Rating No Reviews - Be the first! No Reviews - Be the first! Not Rated - Be the first! yuletide
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