CARBAGLU DOSAGE AND ADMINISTRATION

Recommended Dosage

CARBAGLU should be initiated as soon as the diagnosis of NAGS deficiency is suspected, which may be as soon as at birth, and managed by a physician and medical team experienced in metabolic disorders.

Initial Dosage

The recommended initial daily dosage of CARBAGLU in pediatric and adult patients for acute hyperammonemia is 100 mg/kg to 250 mg/kg divided into 2 to 4 doses and rounded to the nearest 100 mg (i.e., half of a CARBAGLU tablet). Concomitant administration of other ammonia lowering therapies is recommended.

Maintenance Dosage

The recommended daily maintenance dosage of CARBAGLU in pediatric and adult patients is 10 mg/kg to 100 mg/kg divided into 2 to 4 doses and rounded to the nearest 100 mg (i.e., half of a CARBAGLU tablet).

Therapeutic Monitoring

Closely monitor plasma ammonia levels. Titrate the CARBAGLU dosage to maintain the plasma ammonia level within the normal range for the patient's age, taking into consideration their clinical condition (e.g., nutritional requirements, protein intake, growth parameters, etc.).

Preparation And Administration

    Disperse CARBAGLU tablets in water. Do not swallow whole or crushed.
    Mix each 200 mg tablet in a minimum of 2.5 mL of water to yield a concentration of 80 mg/mL.
    CARBAGLU tablets do not dissolve completely in water and undissolved particles of the tablet may remain in the mixing container.
    Take CARBAGLU immediately before meals or feedings.
    The CARBAGLU suspension has a slightly acidic taste.
    For all preparations, use in foods or liquids, other than water, has not been studied clinically and is not recommended.

Preparation For Oral Administration In Pediatric And Adult Patients

    Add about 2.5 mL of water into a small cup for each CARBAGLU tablet or each ½ CARBAGLU tablet needed for the prescribed dose.
    Add the CARBAGLU tablets to the water in the cup.
    Carefully stir the tablet and water mixture.
    Swallow the mixture immediately. Pieces of the tablet may remain in the cup.
    Rinse the cup with additional water and swallow the mixture immediately. Repeat as needed until no pieces of the tablet are left in the cup.

Preparation For Nasogastric Tube Administration In Pediatric And Adult Patients

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

    Add about 2.5 mL of water into a small cup for each CARBAGLU tablet or each ½ CARBAGLU tablet needed for the prescribed dose.
    Add the CARBAGLU tablets to the water in the cup.
    Carefully stir the tablet and water mixture.
    Draw up the mixture into a catheter-tip syringe.
    Administer the mixture immediately through the nasogastric (NG) tube. Pieces of the tablet may remain in the catheter-tip syringe or NG tube.
    Flush immediately with 1 to 2 mL of additional water to clear the NG tube.
    Flush the NG tube again, as needed, until no pieces of the tablet are left in the syringe or NG tube.

Preparation For Oral Administration Using An Oral Syringe In Pediatric Patients

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

    Add about 2.5 mL of water into a small cup for each CARBAGLU tablet or each ½ CARBAGLU tablet needed for the prescribed dose.
    Add the CARBAGLU tablets to the water in the cup.
    Carefully stir the tablet and water mixture.
    Draw up the mixture in an oral syringe and administer immediately. Pieces of the tablet may remain in the oral syringe.
    Refill the oral syringe with a minimum volume of water (1 to 2 mL) and administer immediately.
    Flush the oral syringe again, as needed, until no pieces of the tablet are left in the syringe.