Recommended Topic Related To:

Carbaglu

"Cerebral palsy (CP) is the most common motor disability in childhood, and these children and their families need support. Professionals who serve young children, from early childhood educators to pediatricians, play an important role in identifyi"...

Carbaglu

Indications
Dosage
How Supplied

INDICATIONS

Acute hyperammonemia in patients with NAGS deficiency

Carbaglu is indicated as an adjunctive therapy in pediatric and adult patients for the treatment of acute hyperammonemia due to the deficiency of the hepatic enzyme N-acetylglutamate synthase (NAGS). During acute hyperammonemic episodes concomitant administration of Carbaglu with other ammonia lowering therapies such as alternate pathway medications, hemodialysis, and dietary protein restriction are recommended.

Maintenance therapy for chronic hyperammonemia in patients with NAGS deficiency

Carbaglu is indicated for maintenance therapy in pediatric and adult patients for chronic hyperammonemia due to the deficiency of the hepatic enzyme Nacetylglutamate synthase (NAGS). During maintenance therapy, the concomitant use of other ammonia lowering therapies and protein restriction may be reduced or discontinued based on plasma ammonia levels.

DOSAGE AND ADMINISTRATION

Carbaglu treatment should be initiated by a physician experienced in metabolic disorders.

Adult Dosage and Administration

The recommended initial dose for acute hyperammonemia is 100 mg/kg/day to 250 mg/kg/day. Concomitant administration of other ammonia lowering therapies is recommended. Dosing should be titrated based on individual patient plasma ammonia levels and clinical symptoms.

The recommended maintenance dose should be titrated to target normal plasma ammonia level for age. Based on limited data in 22 patients receiving maintenance treatment with Carbaglu in a retrospective case series, maintenance doses were usually less than 100 mg/kg/day.

The total daily dose should be divided into 2 to 4 doses and rounded to the nearest 100 mg (i.e., half of a Carbaglu Tablet).

Preparation for Oral Administration in Adults

Carbaglu tablets should not be swallowed whole or crushed. Disperse Carbaglu tablets in water immediately before use.

Each 200 mg tablet should be dispersed in a minimum of 2.5 mL of water and taken immediately. Carbaglu tablets do not dissolve completely in water and undissolved particles of the tablet may remain in the mixing container. To ensure complete delivery of the dose, the mixing container should be rinsed with additional volumes of water and the contents swallowed immediately. USE IN OTHER FOODS AND LIQUIDS HAS NOT BEEN STUDIED CLINICALLY AND IS THEREFORE NOT RECOMMENDED.

Preparation for Nasogastric Tube Administration in Adults

For patients who have a nasogastric tube in place, Carbaglu should be administered as follows:

  • Mix each 200 mg tablet in a minimum of 2.5 mL of water. Shake gently to allow for quick dispersal.
  • Administer the dispersion immediately through the nasogastric tube
  • Flush with additional water to clear the nasogastric tube.

Pediatric Dosage and Administration

The recommended initial dose for acute hyperammonemia is 100 mg/kg/day to 250 mg/kg/day. Concomitant administration of other ammonia lowering therapies is recommended. Dosing should be titrated based on individual patient plasma ammonia levels and clinical symptoms.

The recommended maintenance dose should be titrated to target normal plasma ammonia level for age. Based on limited data in 22 patients receiving maintenance treatment with Carbaglu in a retrospective case series, maintenance doses were usually less than 100 mg/kg/day.

The total daily dose should be divided into 2 to 4 doses.

Preparation for Oral Administration Using an Oral Syringe in Pediatrics

For administration via oral syringe, Carbaglu should be administered as follows:

  • Mix each 200 mg tablet in 2.5 mL of water to yield a concentration of 80 mg/mL in a mixing container. Shake gently to allow for quick dispersal.
  • Draw up the appropriate volume of dispersion in an oral syringe and administer immediately. Discard the unused portion.
  • Refill the oral syringe with a minimum volume of water (1-2 mL) and administer immediately.

Preparation for Nasogastric Tube Administration in Pediatrics

For patients who have a nasogastric tube in place, Carbaglu should be administered as follows:

  • Mix each 200 mg tablet in 2.5 mL of water to yield a concentration of 80 mg/mL in a mixing container. Shake gently to allow for quick dispersal.
  • Draw up the appropriate volume of dispersion and administer immediately through a nasogastric tube. Discard the unused portion.
  • Flush with additional water to clear the nasogastric tube.

HOW SUPPLIED

Dosage Forms And Strengths

Carbaglu is a white and elongated 200 mg tablet, scored and coded “C” on one side.

Storage And Handling

Carbaglu is a white and elongated tablet, scored and coded “C” on one side.

Each tablet contains 200 mg of carglumic acid. Carbaglu is available in 5 or 60 tablets in a polypropylene bottle with polyethylene cap and desiccant unit.

NDC 52276-312-05 Bottles of 5 tablets
NDC 52276-312-60 Bottles of 60 tablets

Storage

Before opening, store refrigerated at 2 – 8 °C (36 – 46 °F).

After first opening of the container:

  • Do not refrigerate, do not store above 30 °C (86 °F).
  • Keep the container tightly closed in order to protect from moisture.
  • Write the date of opening on the tablet container.
  • Do not use after the expiration date stated on the tablet container.
  • Discard one month after first opening.

Manufactured For: Orphan Europe SARL, Paris, France. Distributed by: Accredo Health Group, Inc. 1680 Century Center Parkway Memphis, Tennessee 38134. Revised: August 2013

Last reviewed on RxList: 8/16/2013
This monograph has been modified to include the generic and brand name in many instances.

Indications
Dosage
How Supplied
A A A

Report Problems to the Food and Drug Administration

 

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit the FDA MedWatch website or call 1-800-FDA-1088.


WebMD Daily

Get breaking medical news.


NIH talks about Ebola on WebMD