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Carglumic acid (By mouth)

Carglumic Acid (kar-GLOO-mik AS-id)

Treats and prevents high amounts of ammonia in the blood.

Brand Name(s):


There may be other brand names for this medicine.

When This Medicine Should Not Be Used:

This medicine is not right for everyone. Do not use it if you had an allergic reaction to carglumic acid.

How to Use This Medicine:


  • Take your medicine as directed. Your dose may need to be changed several times to find what works best for you.
  • Take this medicine right before meals or feedings.
  • Dissolve the tablet before use. Do not break, crush, chew, or swallow the tablet whole. Dissolve the tablet in a small container or medicine cup with at least 2.5 milliliters (one-half teaspoon) of water. Shake the container gently to dissolve the tablet.
  • Dissolved tablet: Drink the medicine right away. Some small particles or specks may be left at the bottom of the container. Rinse the mixing container with water and drink the water right away to make sure you take all of the medicine.
  • Nasogastric tube (NGT): Pour the medicine into the tube right away. Some small particles or specks may be left at the bottom of the container or in the tubing. Rinse the mixing container with water and pour into the tube right away. This will clear the particles from the tube and make sure you have given all of the medicine.
  • Oral syringe: Use the syringe to measure the correct amount for the dose and give it right away. Throw away any unused medicine in the container. Refill the oral syringe with 1 to 2 milliliters of water and give the water right away. This will clear the particles from the syringe and make sure you have given all of the medicine.
  • Missed dose: Take a dose as soon as you remember. If it is almost time for your next dose, wait until then and take a regular dose. Do not take extra medicine to make up for a missed dose.
  • Unopened bottle: Store in the refrigerator. Do not freeze.
  • Opened bottle: Store at room temperature. Keep the bottle tightly closed away from heat, moisture, and direct light. Do not refrigerate. Throw away any unused medicine 30 days after first opening the bottle.

Drugs and Foods to Avoid:

Ask your doctor or pharmacist before using any other medicine, including over-the-counter medicines, vitamins, and herbal products.

Warnings While Using This Medicine:

  • Tell your doctor if you are pregnant or breastfeeding.
  • Hyperammonemia (too much ammonia in the blood) is a life-threatening condition. Seek medical attention right away if you have symptoms including nausea or vomiting, more frequent seizures, facial swelling, tiredness, weakness, or yellowing eyes or skin.
  • Your doctor will do lab tests at regular visits to check on the effects of this medicine. Keep all appointments.
  • Keep all medicine out of the reach of children. Never share your medicine with anyone.

Possible Side Effects While Using This Medicine:

Call your doctor right away if you notice any of these side effects:

  • Allergic reaction: Itching or hives, swelling in your face or hands, swelling or tingling in your mouth or throat, chest tightness, trouble breathing
  • Fever, chills, cough, sore throat, body aches
  • Increased sweating
  • Swollen glands
  • Trouble breathing
  • Unusual bleeding, bruising, or weakness

If you notice these less serious side effects, talk with your doctor:

  • Diarrhea, vomiting, stomach pain, loss of appetite
  • Ear infection
  • Headache
  • Rash
  • Sleepiness or unusual drowsiness

If you notice other side effects that you think are caused by this medicine, tell your doctor

Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088

Last Updated: 3/19/2020

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites.

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