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.

Carglumic acid is used to treat hyperammonemia (HYE-per-AM-moe-NEE-mee-a), a urea cycle disorder caused by lack of a certain liver enzyme. Carglumic acid is usually given with other medications to treat this lifelong disorder.

Carglumic acid may also be used for purposes not listed in this medication guide.

Avoid eating foods that are high in protein when you first start taking carglumic acid. 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 carglumic acid. Wait until your next meal.

Your blood will need to be tested often. A buildup of ammonia in the blood can quickly cause brain injury or death. Every person with a urea cycle disorder should remain under the care of a 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.

Carglumic acid 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 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 carglumic acid 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.

Tell your doctor about all of your medical conditions.

Carglumic acid is only part of a complete program of treatment that may also include a special diet and other medications. Follow all instructions of your doctor or dietitian. Learn about the foods to eat or avoid to help control your condition.

Follow your doctor's instructions about taking carglumic acid if you are pregnant. It is very important to control your ammonia levels during pregnancy. Do not stop taking carglumic acid without your doctor's advice, and tell your doctor right away if you become pregnant.

If you are pregnant, your name may be listed on a pregnancy registry to track the effects of carglumic acid on the baby.

It may not be safe to breastfeed while using this medicine. Ask your doctor about any risk.

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.

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.

Avoid eating foods that are high in protein when you first start taking carglumic acid.

Follow your doctor's instructions about any other restrictions on food, beverages, or activity.

Get emergency medical help if you have signs of an allergic reaction: 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:

• changes in your mental status, thinking problems, drowsiness, loss of balance, involuntary eye movements;

• pancreatitis--severe pain in your upper stomach spreading to your back, nausea and vomiting;

• low blood sugar--headache, hunger, sweating, irritability, dizziness, fast heart rate, and feeling anxious or shaky;

• signs of an electrolyte imbalance--increased thirst or urination, constipation, muscle pain or weakness, leg cramps, numbness or tingling, feeling jittery, irregular heartbeats, fluttering in your chest, or a choking feeling;

• 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 side effects of carglumic acid may include:

• low blood sugar;

• anemia;

• electrolyte imbalance;

• low white blood cell counts;

• ear pain, trouble hearing, drainage from the ear;

• abnormal lab tests;

• decreased appetite, vomiting, diarrhea, stomach pain;

• fever, infections;

• decreased alertness, feeling very weak or tired;

• changes in mental status;

• headache; or

• 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.