What is Carbaglu?

Commonly Used Brand Name(s)Carbaglu

OverviewTOP

Carglumic acid is used together with diet and other medicines to treat and prevent hyperammonemia (too much ammonia in the blood) when it is caused by the lack of a liver enzyme called N-acetylglutamate synthase (NAGS).

Ammonia is formed in the body when we digest protein. A person without the NAGS enzyme cannot process ammonia properly, and too much ammonia will collect in the blood. This will cause serious unwanted effects. Carglumic acid acts by replacing the NAGS enzyme and helps reduce the amount of ammonia in the blood.

This medicine is available only with your doctor's prescription.

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Proper UseTOP

Take this medicine exactly as directed by your doctor. Do not take more of it, do not take it more often, and do not take it for a longer time than your doctor ordered. To do so may increase the chance of side effects.

It is important that you and your child follow any special diet instructions from your doctor, such as using a low-protein diet. If you have any questions about this, check with your doctor.

Do not swallow the tablet whole. Do not break, crush, or chew it. Also, it is best to take this medicine right away before meals or feedings.

To use:

Dissolve the tablet in a small container or medicine cup with at least 2.5 milliliters (one-half teaspoonful) of water. Shake the container gently to dissolve the tablet.
Drink the mixture 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.

To use with a nasogastric tube (NGT):

Dissolve the tablet in a small container or medicine cup with at least 2.5 milliliters (one-half teaspoonful) of water. Shake the container gently to dissolve the tablet.
Pour the mixture 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 the water into the tube right away. This will clear the particles from the tube and make sure you have given all of the medicine.

To use with an oral syringe:

Dissolve the tablet in a small container or medicine cup with at least 2.5 milliliters (mL) or one-half teaspoonful of water. Shake the container gently to dissolve the tablet.
Use an oral syringe to measure the correct amount of mixture for the dose and give it right away. Throw away any unused medicine in the container.
Some small particles or specks may be left in the oral syringe.
Refill the oral syringe with 1 to 2 mL 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.

DosingTOP

The dose of this medicine will be different for different patients. Follow your doctor's orders or the directions on the label. The following information includes only the average doses of this medicine. If your dose is different, do not change it unless your doctor tells you to do so.

The amount of medicine that you take depends on the strength of the medicine. Also, the number of doses you take each day, the time allowed between doses, and the length of time you take the medicine depend on the medical problem for which you are using the medicine.

For oral dosage form (tablets):
- For hyperammonemia:
  - Adults—Dose is based on body weight and must be determined by your doctor. The starting dose is usually 100 to 250 milligrams (mg) per kilogram (kg) of body weight per day. The total dose will be divided and given into 2 to 4 doses per day. Your doctor may adjust your dose to 10 mg/kg to 100 mg/kg divided into 2 to 4 doses per day.
  - Children—Dose is based on body weight and must be determined by your child's doctor. The starting dose is usually 100 to 250 milligrams (mg) per kilogram (kg) of body weight per day. The total dose will be divided and given into 2 to 4 doses per day. Your doctor may adjust your dose to 10 mg/kg to 100 mg/kg divided into 2 to 4 doses per day.

Missed DoseTOP

If you miss a dose of this medicine, take it as soon as possible. However, if it is almost time for your next dose, skip the missed dose and go back to your regular dosing schedule. Do not double doses.

Use & StorageTOP

Keep out of the reach of children.

Do not keep outdated medicine or medicine no longer needed.

Ask your healthcare professional how you should dispose of any medicine you do not use.

Before you open the bottle, store it in the refrigerator. Do not freeze.

After you open the bottle for the first time, store it at room temperature. Keep the bottle tightly closed, and away from heat, moisture, and direct light. Do not refrigerate the bottle. Write the date you used the first tablet on the bottle. Throw away any unused medicine 30 days after the date on the bottle.

Before UsingTOP

In deciding to use a medicine, the risks of taking the medicine must be weighed against the good it will do. This is a decision you and your doctor will make. For this medicine, the following should be considered:

AllergiesTOP

Tell your doctor if you have ever had any unusual or allergic reaction to this medicine or any other medicines. Also tell your health care professional if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.

PediatricTOP

Appropriate studies performed to date have not demonstrated pediatric-specific problems that would limit the usefulness of carglumic acid in children.

GeriatricTOP

Appropriate studies have not been performed on the relationship of age to the effects of carglumic acid in the geriatric population. Safety and efficacy have not been established.

PregnancyTOP

	Pregnancy Category	Explanation
All Trimesters	C	Animal studies have shown an adverse effect and there are no adequate studies in pregnant women OR no animal studies have been conducted and there are no adequate studies in pregnant women.

Breast FeedingTOP

There are no adequate studies in women for determining infant risk when using this medication during breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.

Drug InteractionsTOP

Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. Tell your healthcare professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicine.

Other InteractionsTOP

Certain medicines should not be used at or around the time of eating food or eating certain types of food since interactions may occur. Using alcohol or tobacco with certain medicines may also cause interactions to occur. Discuss with your healthcare professional the use of your medicine with food, alcohol, or tobacco.

PrecautionsTOP

It is very important that your doctor check your or your child's progress at regular visits to make sure that this medicine is working properly. Blood tests may be needed to check for unwanted effects.

Hyperammonemia (too much ammonia in the blood) is a life-threatening condition that requires immediate medical attention. Check with your doctor right away if you or your child have any of the following symptoms: nausea or vomiting that continues, an increase in the frequency of seizures, loss of appetite, swelling of the face, tiredness and weakness, or yellow eyes or skin.