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Generic name: carglumic acid  Kar-GLOO-mik-AS-id]

Class: Miscellaneous agents that are not classified

What is Carbaglu?

Carbaglu is a manufactured version of an enzyme that is naturally found inside the liver. This enzyme is essential for the processing of excess nitrogen created as the body processes proteins. In the absence of this enzyme, nitrogen accumulates into ammonia and is not eliminated from the human body. Ammonia can be extremely harmful when it circulates through tissues and the blood. It could cause permanent neurological damage, a coma, or even death.

Carbaglu is used to treat hyperammonemia (HYE-per-AM-moe-NEE-mee-a), a urea cycle disorder caused by a lack of a certain liver enzyme. Carbaglu is often prescribed in conjunction with other medications to treat this condition, which can last a lifetime.


Beware of eating foods high in protein as you begin taking Carbaglu. Follow the instructions of your physician regarding any other restrictions regarding drinks, food, or other activities. If you do not eat during eating time, do not take the dose of Carbaglu. Wait until your next meal. To make sure Carbaglu helps your condition and does not produce adverse consequences, the blood of your patient needs to be tested frequently. An accumulation of ammonia in your blood can result in brain damage or even death. Do not miss any follow-up visits to your doctor to have blood tests. Anyone suffering from a urea cycle disorder must be monitored by an expert. Inform your doctor about all medical conditions you suffer from.

Before you take this drug

Carbaglu is a small part of a full treatment program that could also comprise a specific diet as well as other medications. It is essential to follow the diet program that was developed for you by your physician or nutritionist. You must be acquainted with the list of food items you should avoid eating or not eat to manage your condition. Follow the instructions of your physician regarding taking Carbaglu if you are expecting. It is crucial to manage the levels of ammonia in your blood during pregnancy. Don't stop using If you're expecting and you are a registered mother, your name could be included on a registry for pregnant women to monitor any effects that carglumic acid has on the baby. It might not be safe to breastfeed when using Carbaglu. Talk to your doctor about any risks.

How to take Carbaglu?

Use Carbaglu exactly as directed by your physician. Follow all instructions on the prescription label and review all medication guides or instructions. The doctor might alter the dosage. Make sure you take the medication precisely as prescribed. Carbaglu is generally taken between 2 and 4 times daily, at the time of each meal or after a meal. Follow the directions of your physician.

Carglumic acid dosages are based on weight (especially for adolescents and children). Your dose needs may change if you gain or lose weight. The need for adjustments to doses is particularly critical as children grow. Do not crush or ingest these Carbaglu dispersible tablets whole. Place it in the glass of water and let it disperse (it is not going to dissolve completely). Drink the mixture immediately. Add a few drops of water to the glass, stir gently, and enjoy immediately. The tablet that is dispersed can be administered via an oral syringe or via an nasogastric (NG) tube for feeding. Take note of and adhere to any instructions for use that are included in your medication. Consult your physician or pharmacist if you don't understand these directions. Your blood should be checked frequently. An accumulation of ammonia in the blood can swiftly result in brain damage or even death. Even if you do not have any symptoms, testing can aid your doctor in determining the effectiveness of this treatment. Anyone suffering from an urea cycle disorder must be under the supervision of an expert.

Keep the unopened bottle filled with Carbaglu tablets in your refrigerator. Do not put them in the freezer. After opening the bottle, keep it at room temperature away from heat and moisture. Don't store unopened bottles in refrigerators. Keep the bottle shut when not being used. Once you have opened the bottle, note your name on it. Toss any tablets that have not been used for one month (30 days) from the date of opening or when you are unsure of the expiration date that is on the label.

Details on dosage

Usual Adult Dose of Carbaglu for Hyperammonemia:

The daily dose must be divided into 4 to 2 doses, then rounded to the nearest 100 mg.

Initial dosage: 100 to 250 mg/kg daily orally with 2 to 4 equal doses right prior to meals. Concurrent treatment with other ammonia-lowering treatments is recommended. Dosing must be titrated depending on the individual ammonia levels in the plasma and symptoms.

Maintenance doses are typically under 100 mg/kg daily in two to four divided doses right prior to eating. The recommended dose of maintenance should be titrated according to normal levels of ammonia in plasma for a person of a certain age.

Usual Pediatric Dose of Carbaglu for Hyperammonemia

The dose for the entire day should be divided into two to four doses.

Initial dosage: 100–250 mg/kg taken orally daily with 2–4 equal doses prior to meals or feedings.

In addition, the use of other treatments to reduce ammonia is suggested. Dosing should be adjusted according to individual ammonia levels in the plasma and clinical signs.

Maintenance doses are usually lower than 100 mg/kg every day, divided into 2 or 4 split doses prior to meals.

What happens If I miss a dose?

Start taking your dose as fast as you can, but only if you're making preparations for eating the meal. If you don't eat for a while, take the dose you missed and wait until the next meal. Don't take two doses at a time.

What happens If I overdose?

Get medical attention in an emergency or contact the poison help line at 1-800-222-1222.

The symptoms of an overdose can include fever, sweating heavily, a fast heart rate, coughing up mucus, and feeling agitated.

What should be avoided?

Avoid eating foods high in protein as you begin taking Carbaglu.

Follow the doctor's advice regarding any other restrictions regarding drinks, food, or other activities.

Side effects of Carbaglu

See a doctor immediately. If you are experiencing symptoms that you are experiencing an allergic reaction due to Carbaglu, such as hives, difficulty breathing, or swelling of your lips, face, and tongue,

Contact your doctor immediately in the event of serious side effects, for example:

  • Shifts in mental state—thoughts, sleepiness, problems with thinking, and loss of balance—eyes that move involuntarily;
  • Pancreatitis The pain is intense in your upper stomach, spreading into your back and causing nausea and vomiting.
  • Low blood sugar, a feeling of anxiety, sweating, irritability, a high heart rate, dizziness, and feeling nervous or shaky
  • Symptoms of an electrolyte imbalance, such as increased thirst or urination, constipation, muscle weakness, or pain in the legs, cramps sensation of numbness or tingling, being jittery, having irregular heartbeats, feeling fluttering around your chest, or a choke feeling
  • Symptoms for an infection in the ear, such as fever, hearing pain or fullness, difficulty hearing draining from your ear, and a fright in children;
  • Low red blood cells (anaemia): pale skin, unusual fatigue, feeling lightheaded or sluggish, cold feet and hands,
  • Lower white blood cell count (fever, lip sores, sores, sore throat, trouble breathing, cough).

Common Carbaglu side effects can include:

  • Low blood sugar levels;
  • Anaemia;
  • Electrolyte imbalance;
  • Low white blood cell count;
  • Hearing loss, ear pain, and drainage of the ear
  • Abnormal lab tests;
  • Diminished appetite, vomiting, stomach pain, and diarrhea;
  • Fever, infections;
  • Diminished alertness, feeling tired or weak;
  • Changes in mental state;
  • Headache Or
  • Stuffy nose, sore throat

This is not a comprehensive list of all the side effects. Other side effects could occur. Consult your physician for advice regarding medical adverse effects. You may report any adverse reactions to the FDA at 1-800-FDA-1088.

Interaction with other drugs

Other drugs can be incompatible with carglumic acid, which includes prescription and over-the-counter medicines, vitamins, and herbal products. Discuss with your doctor all the medicines you are currently taking and any medications you begin or stop taking.