Generic Name: carglumic acid (kar GLOO mik AS id)
Brand Names: Carbaglu
What is Carbaglu?
Carbaglu (carglumic acid) is a man-made form of an enzyme that occurs naturally in the liver. This enzyme is necessary for processing excess nitrogen produced when the body metabolizes proteins. Without this enzyme, nitrogen builds up in the form of ammonia and is not removed from the body. Ammonia is very toxic when it circulates in blood and tissues and can cause permanent brain damage, coma, or death.
Carbaglu is used to treat hyperammonemia (HYE-per-AM-moe-NEE-mee-a), a urea cycle disorder caused by lack of a certain liver enzyme.
Carbaglu is usually given with other medications to treat this lifelong disorder.
Avoid eating foods that are high in protein when you first start taking Carbaglu. Follow your doctor's instructions about any other restrictions on food, beverages, or activity. If you skip a meal, do not take your dose of Carbaglu. Wait until your next meal.
To be sure Carbaglu is helping your condition and is not causing harmful effects, your blood will need to be tested often. A buildup of ammonia in the blood can quickly cause brain injury or death. Do not miss any follow up visits to your doctor for blood tests. Every person with a urea cycle disorder should remain under the care of a doctor.
Tell your doctor about all of your medical conditions.
Before taking this medicine
Carbaglu is only part of a complete program of treatment that may also include a special diet and other medications. It is very important to follow the diet plan created for you by your doctor or nutrition counselor. You should become very familiar with the list of foods you should eat or avoid to help control your condition.
Follow your doctor's instructions about taking Carbaglu if you are pregnant. It is very important to control your ammonia levels during pregnancy. Do not stop taking this medicine without your doctor's advice, and tell your doctor right away if you become pregnant.
You should not breast-feed while you are using Carbaglu. Talk with your doctor about other medications that can be more safely used to control your ammonia levels while you are nursing a baby.
How should I take Carbaglu?
Take Carbaglu exactly as prescribed by your doctor. Follow all directions on your prescription label and read all medication guides or instruction sheets. Your doctor may occasionally change your dose. Use the medicine exactly as directed.
Carbaglu is usually taken 2 to 4 times each day, just before each meal or feeding. Follow your doctor's instructions.
Carglumic acid doses are based on weight (especially in children and teenagers). Your dose needs may change if you gain or lose weight. Dose adjustments are especially important in children who are growing.
Do not crush or swallow the Carbaglu dispersible tablet whole. Place it into a glass of water and allow the tablet to disperse (it will not dissolve completely). Drink this mixture right away. Add a little more water to the glass, swirl gently and drink right away.
The dispersed tablet may also be taken with an oral syringe or through a nasogastric (NG) feeding tube. Read and carefully follow any Instructions for Use provided with your medicine. Ask your doctor or pharmacist if you do not understand these instructions.
Your blood will need to be tested often. A buildup of ammonia in the blood can quickly cause brain injury or death. Even if you have no symptoms, tests can help your doctor determine if this medicine is effective. Every person with a urea cycle disorder should remain under the care of a doctor.
Store unopened bottles of Carbaglu tablets in the refrigerator, do not freeze.
After opening the bottle, store at room temperature away from moisture and heat. Do not store opened bottles in the refrigerator. Keep the bottle tightly closed when not in use.
When you open the bottle, write the date on the bottle. Throw away any unused tablets 1 month (30 days) after the date of opening, or if the expiration date printed on the label has passed.
Carbaglu dosing information
Usual Adult Dose of Carbaglu for Hyperammonemia:
The total daily dose should be divided into 2 to 4 doses and rounded to the nearest 100 mg.
Initial dose: 100 to 250 mg/kg orally per day in 2 to 4 divided doses immediately prior to meals. Concomitant administration of other ammonia lowering therapies is recommended. Dosing should be titrated based on individual patient plasma ammonia levels and clinical symptoms.
Maintenance dose: Usually less than 100 mg/kg orally per day in 2 to 4 divided doses immediately prior to meals. The recommended maintenance dose should be titrated to target normal plasma ammonia level for age.
Usual Pediatric Dose of Carbaglu for Hyperammonemia:
The total daily dose should be divided into 2 to 4 doses.
Initial dose: 100 to 250 mg/kg orally per day in 2 to 4 divided doses immediately prior to meals or feedings.
Concomitant administration of other ammonia lowering therapies is recommended. Dosing should be titrated based on individual patient plasma ammonia levels and clinical symptoms.
Maintenance dose: Usually less than 100 mg/kg orally per day in 2 to 4 divided doses immediately prior to meals or feedings.
What happens if I miss a dose?
Take your dose as soon as you can, but only if you are getting ready to eat a meal. If you skip a meal, skip the missed dose and wait until your next meal. Do not take two doses at one time.
What happens if I overdose?
Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.
Overdose symptoms may include fever, heavy sweating, fast heart rate, coughing up mucus, and feeling restless.
What should I avoid while taking Carbaglu?
Avoid eating foods that are high in protein when you first start taking Carbaglu.
Follow your doctor's instructions about any other restrictions on food, beverages, or activity.
Carbaglu side effects
Get emergency medical help if you have signs of an allergic reaction to Carbaglu: hives; difficult breathing; swelling of your face, lips, tongue, or throat.
Call your doctor at once if you have a serious side effect such as:
signs of an ear infection - fever, ear pain or full feeling, trouble hearing, drainage from the ear, fussiness in a child;
low red blood cells (anemia) - pale skin, unusual tiredness, feeling light-headed or short of breath, cold hands and feet; or
low white blood cell counts - fever, mouth sores, skin sores, sore throat, cough, trouble breathing.
Common Carbaglu side effects may include:
vomiting, diarrhea, stomach pain;
stuffy nose, sore throat.
This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
What other drugs will affect Carbaglu?
Other drugs may interact with carglumic acid, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell your doctor about all your current medicines and any medicine you start or stop using.
Remember, keep this and all other medicines out of the reach of children, never share your medicines with others, and use Carbaglu only for the indication prescribed.
Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.
Editorial References and Review
Medically reviewed by USARx EDITORIAL TEAM Last updated on 1/1/2020.
Source: Drugs.com Carbaglu (www.drugs.com/carbaglu.html).