This medication is used to help treat certain inherited disorders that cause high levels of a certain substance (ammonia) to build up in your body. Carglumic acid works by helping your body remove ammonia. Too much ammonia in the body can cause brain damage and sometimes death.

Read the Patient Information Leaflet and Instructions for Use if available from your pharmacist before you start taking carglumic acid and each time you get a refill. If you have any questions, ask your doctor or pharmacist.

Take this medication by mouth right before meals or feedings as directed by your doctor, usually 2 to 4 times daily. The dosage is based on your medical condition, weight, and response to treatment.

This medication must be mixed in water before taking. Use at least one-half teaspoonful/2.5 milliliters water to mix with each tablet of carglumic acid. Place the water and tablet(s) in a cup and stir well until the tablet(s) break into small pieces. The tablet(s) will not completely dissolve. Drink all of the mixture right away. Add more water to the cup and drink all of the mixture to make sure you are getting all of the drug. Use only water for mixing, and do not prepare a supply ahead of time. Do not crush the tablets or swallow them whole.

The mixture may also be given with an oral syringe or through a tube into the stomach. If you are using either of these, ask your health care professional for detailed instructions on how to give it.

Use this medication regularly to get the most benefit from it. To help you remember, take it at the same times each day.

Do not increase your dose or use this drug more often or for longer than prescribed. Your condition will not improve any faster, and your risk of side effects will increase.

Tell your doctor if your condition does not get better or if it gets worse.

Upset stomach or vomiting may occur. If either of these effects lasts or gets worse, tell your doctor or pharmacist promptly.

Remember that this medication has been prescribed because your doctor has judged that the benefit to you is greater than the risk of side effects. Many people using this medication do not have serious side effects.

A very serious allergic reaction to this drug is rare. However, get medical help right away if you notice any symptoms of a serious allergic reaction, including:

• rash
• itching/swelling (especially of the face/tongue/throat)
• severe dizziness
• trouble breathing

This is not a complete list of possible side effects. If you notice other effects not listed above, contact your doctor or pharmacist.

In the US - Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088 or at www.fda.gov/medwatch.

In Canada - Call your doctor for medical advice about side effects. You may report side effects to Health Canada at 1-866-234-2345.

Before taking carglumic acid, tell your doctor or pharmacist if you are allergic to it; or if you have any other allergies. This product may contain inactive ingredients, which can cause allergic reactions or other problems. Talk to your pharmacist for more details.

Before using this medication, tell your doctor or pharmacist your medical history, especially of:

• kidney problems

Before having surgery, tell your doctor or dentist about all the products you use (including prescription drugs, nonprescription drugs, and herbal products).

During pregnancy, this medication should be used only when clearly needed. Discuss the risks and benefits with your doctor.

It is unknown if this medication passes into breast milk. Consult your doctor before breastfeeding.

Drug interactions may change how your medications work or increase your risk for serious side effects. This document does not contain all possible drug interactions. Keep a list of all the products you use (including prescription/nonprescription drugs and herbal products) and share it with your doctor and pharmacist. Do not start, stop, or change the dosage of any medicines without your doctor's approval.

If someone has overdosed and has serious symptoms such as passing out or trouble breathing, call 911. Otherwise, call a poison control center right away. US residents can call 1-800-222-1222. Canada residents can call 1-844-764-7669.

Do not share this medication with others.

Lab and/or medical tests (such as ammonia blood levels, kidney function, complete blood count) should be done while you are taking this medication. Keep all medical and lab appointments.

If you miss a dose, take it as soon as you remember. If it is near the time of the next dose, skip the missed dose. Take your next dose at the regular time. Do not double the dose to catch up.

Different brands of this medication have different storage needs. Check the product package for instructions on how to store your brand, or ask your pharmacist. Keep all medications away from children and pets.

Do not flush medications down the toilet or pour them into a drain unless instructed to do so. Properly discard this product when it is expired or no longer needed. Consult your pharmacist or local waste disposal company.