carglumic acid (Rx)

Brand and Other Names:Carbaglu
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Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

dispersible tablet

  • 200mg

Hyperammonemia

Initial dose range: 100-250 mg/kg/day PO/NG divided in 2-4 doses before meals; round total daily dose to nearest 100 mg  

Maintenance: Generally <100 mg/kg/day in chronic hyperammonemia

Use of other ammonia-lowering therapies (eg, diet, hemodialysis) with carglumic acid during episodes of acute hyperammonemia is recommended

Administration

Disperse 200-mg dispersible tab in 2.5 mL water (resulting in 80 mg/mL) and administer immediately

Do not swallow whole or crush

Adjust dose according to ammonia levels and symptoms

Dosage Forms & Strengths

dispersible tablet

  • 200mg

Hyperammonemia

Initial dose range: 100-250 mg/kg/day PO/NG divided in 2-4 doses before meals; round total daily dose to nearest 100 mg  

Maintenance: Generally <100 mg/day in chronic hyperammonemia

Use of other ammonia-lowering therapies (eg, diet, hemodialysis) with carglumic acid during episodes of acute hyperammonemia is recommended

Other Information

Disperse 200-mg dispersible tab in 2.5 mL water (resulting in 80 mg/mL) and administer immediately

Do not swallow whole or crush

Adjust dose according to ammonia levels and symptoms

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Adverse Effects

Note: Adverse effects are derived from retrospective case series of 23 patients

>10%

Vomiting (26%)

Abdominal pain (17%)

Pyrexia (17%)

Tonsillitis (17%)

Anemia (13%)

Ear infection (13%)

Diarrhea (13%)

Nasopharyngitis (13%)

Headache (13%)

1-10%

Asthenia (9%)

Somnolence (9%)

Dysgeusia (9%)

Hyperhidrosis (9%)

Weakness (9%)

Pneumonia (9%)

Anorexia (9%)

Weight loss (9%)

Influenza (9%)

Rash (9%)

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Warnings

Contraindications

None

Cautions

Monitor serum ammonia levels and adjust dose to maintain within normal range for age

Treat acute hyperammonemia episode as life-threatening emergency

Acute symptomatic hyperammonemia should be treated as life-threatening emergency; treatment of severe hyperammonemia may require dialysis, preferably hemodialysis and/or hemofiltration, to reduce plasma ammonia concentration; untreated hyperammonemia can result in brain damage and death, and prompt use of all therapies necessary to reduce plasma ammonia level is essential

Protein restriction during an acute hyperammonemic episode is recommended for no longer than 12 to 36 hr while maximizing caloric supplementation to reverse catabolism; protein should be reintroduced as early as possible, following improvement of metabolic and clinical abnormalities in this setting; during long-term management, dietary protein restriction should be instituted to maintain blood ammonia level within an acceptable range for age

Ongoing monitoring of plasma ammonia level, neurological status, growth parameters, protein intake/nutritional status (both during acute hyperammonemic episodes and long-term), and relevant laboratory tests in patients receiving therapy should be part of evaluating clinical response to treatment

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Pregnancy & Lactation

Pregnancy

There are no adequate and well controlled studies or available human data in pregnant women; decreased survival and growth occurred in offspring born to animals that received carglumic acid at a dose approximately 38 times the maximum reported human maintenance dose; because untreated N-acetylglutamate synthase (NAGS) deficiency results in irreversible neurologic damage and death, women with NAGS must remain on treatment throughout pregnancy

Lactation

It is not known whether drug is excreted in human milk; it is excreted in rat milk, and an increase in mortality and impairment of body weight gain occurred in neonatal rats nursed by mothers receiving carglumic acid; because many drugs are excreted in human milk and because of potential for serious adverse reactions in nursing infants from therapy, breast-feeding is not recommended; treatment is continuous and life-long for NAGS deficiency patients

Pregnancy Categories

A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

NA: Information not available.

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Pharmacology

Mechanism of Action

Structural analogue of N-acetylglutamate, which enters cells and enables activation of CPS I (first enzyme of urea cycle) in vivo; therefore, decreases hyperammonemia by converting ammonia into urea

Pharmacokinetics

Half-Life: 5.6 hr

Bioavailability: Absolute biovailability is not known

Vd: 2657 L

Peak Plasma Time: 3 hr (range: 2-4 hr)

Metabolism: portion metabolized by intestinal bacterial flora; likely end product is carbon dioxide eliminated via lungs

Clearance: 5.7 L/min (total); 290 mL/min (renal)

Excretion: unchanged in feces (60%) and urine (9%)

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Formulary

FormularyPatient Discounts

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The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

Tier Description
1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
NC NOT COVERED – Drugs that are not covered by the plan.
Code Definition
PA Prior Authorization
Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
QL Quantity Limits
Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
ST Step Therapy
Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
OR Other Restrictions
Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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Medscape prescription drug monographs are based on FDA-approved labeling information, unless otherwise noted, combined with additional data derived from primary medical literature.