CARBAGLU
Dosing and Administration

Including dosing adjustments for renal impairment

Propionic Acidemia and Methylmalonic Acidemia Patients

CARBAGLU® (carglumic acid) tablets for oral suspension 200mg should be initiated for the treatment of acute hyperammonemia in patients with a suspected or confirmed diagnosis of propionic acidemia (PA) or methylmalonic acidemia (MMA).1

Dosage for Acute Hyperammonemia due to Propionic Acidemia (PA) or Methylmalonic Acidemia (MMA):1

  1. The recommended pediatric and adult dosage is:
    • 150 mg/kg/day for patients less than or equal to 15 kg
    • 3.3 g/m2/day for patients greater than 15 kg
  2. Divide the daily dosage into 2 equal doses and round up to the next multiple of 50 mg (i.e., one-quarter of a CARBAGLU tablet). Administer doses 12 hours apart.
  3. Continue CARBAGLU treatment until the patient’s ammonia level is less than 50 micromol/L and for a maximum duration of 7 days.
  4. During acute hyperammonemic episodes, administer CARBAGLU with other ammonia lowering therapies, such as intravenous glucose, insulin, L-carnitine, protein restriction, and dialysis.

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

Dosing Carbaglu: Adjustments for Renal Impairment

NAGS Deficiency Patients

CARBAGLU® (carglumic acid) tablets for oral suspension 200mg 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 1

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 Acute Hyperammonemia due to NAGS Deficiency:1

  1. The recommended pediatric and adult dosage is 100mg/kg/day to 250 mg/kg/day.
  2. Divide the daily dosage into 2 to 4 doses and round to the nearest 100 mg (i.e., half of a CARBAGLU tablet).
  3. During acute hyperammonemic episodes, administer CARBAGLU with other ammonia lowering therapies, such as alternate pathway medications, hemodialysis, and protein restriction.

 

Dosage for Chronic Hyperammonemia due to NAGS Deficiency:1

  1. The recommended pediatric and adult maintenance dosage is 10 mg/kg/day to 100mg/kg/day.
  2. Divide the daily dosage into 2 to 4 doses and round to the nearest 100 mg (i.e., half of a CARBAGLU tablet).
  3. During maintenance therapy, the concomitant use of other ammonia lowering therapies and protein restriction may be needed based on plasma ammonia levels.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.).

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

Dosing Carbaglu: Adjustments for Renal Impairment

Administration

Based on patient age and condition, CARBAGLU can be administered:1

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

Preparation and Administration Overview1

  • Disperse CARBAGLU tablets in water. Do not swallow whole or crushed.
  • 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 and is not recommended.

Storage1

  • 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 after opening.

  • 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 dosing and administration of CARBAGLU as well as provides ordering information.

Download in English

STAT Delivery for a Hospital Emergency

CARBAGLU can be delivered to most hospitals within 6 hours of order placement (weather permitting). 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: Unopened 5-count 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.

Home orders and refills for NAGS deficiency patients

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.

Patients experiencing insurance changes?

Resources to support your practice

Appeal Tips and Checklist NAGSD Letter of Appeal Open Letter of Appeal

ORDERING CARBAGLU

Download the Ordering CARBAGLU brochure for acute and chronic hyperammonemia due to NAGS Deficiency & acute hyperammonemia due to Propionic Acidemia and Methylmalonic Acidemia

  • CARBAGLU clinical results

    CARBAGLU has been shown to normalize high plasma ammonia levels in NAGS deficiency patients.

    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.

    Identify symptoms and review diagnostic tests

  • Supporting your patients

    Help your patients access payment assistance and online resources.

    Access patient resources

REFERENCES

  1. CARBAGLU [Package Insert]. Lebanon, NJ: Recordati Rare Diseases; 2021

INDICATIONS AND USAGE

Carbaglu® (carglumic acid) tablets for oral suspension 200mg is a carbamoyl phosphate synthetase 1 (CPS 1) activator indicated in pediatric and adult patients as:

  • Adjunctive therapy to standard of care for the treatment of acute hyperammonemia due to N-acetylglutamate synthase (NAGS) deficiency.
  • Maintenance therapy for the treatment of chronic hyperammonemia due to N-acetylglutamate synthase (NAGS) deficiency.
  • Adjunctive therapy to standard of care for the treatment of acute hyperammonemia due to propionic acidemia (PA) or methylmalonic acidemia (MMA).

IMPORTANT SAFETY INFORMATION

  • Contraindications: None.
  • NAGS deficiency: Most common adverse reactions (≥13%) are: vomiting, abdominal pain, pyrexia, tonsillitis, anemia, diarrhea, ear infection, infections, nasopharyngitis, hemoglobin decreased, and headache.
  • PA and MMA: Most common adverse reactions (≥5%) are neutropenia, anemia, vomiting, electrolyte imbalance, decreased appetite, hypoglycemia, lethargy/stupor, encephalopathy and pancreatitis/lipase increased.
  • To report SUSPECTED ADVERSE REACTIONS, contact Recordati Rare Diseases Inc. at 1-888-575-8344, or FDA at 1‑800-FDA-1088 or www.fda.gov/medwatch.
  • If CARBAGLU is administered during pregnancy to women with NAGS deficiency, health care providers should report CARBAGLU exposure to the pregnancy pharmacovigilance program by calling 1-888-575-8344.
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