CARBAGLU Dosing and Administration

CARBAGLU® (carglumic acid) 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.

Patients should understand the importance of taking CARBAGLU exactly as prescribed—the right dose at the right time—to ensure a constant amount of medicine in their bodies.

Dosage for Pediatric and Adult Use

Acute Hyperammonemia:

  1. The recommended initial pediatric and adult dosage is 100mg/kg/day to 250 mg/kg/day.
  2. Titrate based on plasma ammonia level and clinical symptoms.

Maintenance for Chronic Hyperammonemia:

  1. The recommended pediatric and adult maintenance dose is 10 mg/kg/day to 100mg/kg/day.
  2. Titrate to target normal plasma ammonia level for age.

Divide the total daily dose into two to four doses.

Monitor plasma ammonia and adjust the dosage to maintain the level within normal range for age.

Please refer to Full Prescribing Information, including Instructions for Use for complete dosage instructions.


Based on patient age and condition, CARBAGLU can be administered via:

  • By mouth using a cup
  • By mouth using an oral syringe
  • Through a nasogastric (NG) tube

Important Information:

  • CARBAGLU tablet for oral suspension (CARBAGLU tablet) must be mixed in water before taking. CARBAGLU tablets should not be mixed in any other food or liquid.
  • Do not swallow CARBAGLU tablets whole.
  • Do not crush CARBAGLU tablets.
  • Take CARBAGLU right before meals or feedings.
  • The CARBAGLU tablet and water mixture has a slightly sour taste.
  • You may need to ask your healthcare provider or pharmacist for a medicine cup to measure the correct amount of water you will need to prepare your dose of CARBAGLU.


  • Before opening, store CARBAGLU in a refrigerator between 36°F to 46°F (2°C to 8°C) in the container it comes in.

  • After opening, store CARBAGLU at room temperature between 59°F to 86°F (15°C to 30°C). Do not store CARBAGLU in a refrigerator.

  • Keep CARBAGLU tablets in a tightly closed container to protect the tablets from moisture.

  • Write the date the CARBAGLU tablet container is opened on the container label. Throw away any unused tablets one month after opening the tablet container. Do not use CARBAGLU tablets after the expiration date on the tablet container.

    Keep CARBAGLU and all medicines out of the reach of children.

How to Use and Store CARBAGLU

This brochure explains and illustrates dosing of CARBAGLU in emergency and maintenance situations, methods of administration, and proper storage.

STAT Delivery for a Hospital Emergency

CARBAGLU can be delivered to most hospitals within 6 hours of order placement.
This service is provided 24/7.

  • Alert your hospital pharmacy that
    your patient requires CARBAGLU.
    Send an order for CARBAGLU to
    your hospital pharmacy and
    specify whether it is a STAT order
    (required in 6 hours or less).*

  • Ask your hospital pharmacy
    to call the wholesale department
    at Accredo at 1-877-900-9223
    to place the order.

  • The wholesale department at
    Accredo will work with your
    hospital pharmacy to obtain
    payment information, establish
    shipping timelines, and verify
    the delivery address.

  • The Accredo specialty
    pharmacy team will follow
    up in 1-2 days to help set up
    patient home delivery of
    CARBAGLU, if applicable.

*Delivery time frame is weather dependent and is not guaranteed.

Keep a 5-count bottle of CARBAGLU in stock to allow for immediate treatment.

Return policy: All new orders of 5-count unopened bottles of CARBAGLU can be returned for credit any time prior to the expiration date or up to 12 months past the expiration date.

Not applicable for CARBAGLU 60-count bottles. Product shipped as an emergency or STAT order is not eligible for credit unless damaged in transit. This policy covers products purchased after July 1, 2019. Unopened bottles have up to a 3-year expiration date and must be refrigerated. Other terms and conditions may apply.

The Key to Ordering CARBAGLU

Download the Key to Ordering CARBAGLU brochure for Acute and Chronic HYPERAMMONEMIA due to NAGS Deficiency.

Home orders and refills

For new patient enrollment, refill shipment, insurance/billing questions, or clinical support:

  • Download the CARBAGLU Prescription & Enrollment Form for Accredo.

  • Complete the CARBAGLU Prescription & Enrollment Form and fax it to the Accredo specialty pharmacy
    department at 1-888-454-8488.

  • A CARBAGLU specialty pharmacy representative may call to verify information and determine next steps.
    In some cases, prior authorization may be necessary.

  • Questions? Contact a CARBAGLU specialty pharmacy representative at 1-888-454-8860.
    Representatives are available 24/7 to assist you.

  • CARBAGLU clinical results

    CARBAGLU has been shown to normalize high blood ammonia levels.

    Review clinical data
  • Diagnosing hyperammonemia due to NAGSD

    Hyperammonemic crises can result in serious neurological complications and death. Clinicians must act quickly to diagnose and treat.3

    Identify symptoms and review diagnostic tests
  • Supporting your patients

    Help your patients access payment assistance and online resources.

    Access patient resources