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:
Tablet

• Take your medicine as directed. Your dose may need to be changed several times to find what works best for you.
• It is best to take this medicine on an empty stomach.
• Before you open the bottle, store it in the refrigerator. Do not freeze.
• After you open the bottle, 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 you wrote on the bottle.
• 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 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.
• 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.

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 immediate medical attention if you have symptoms such as nausea or vomiting, more frequent seizures, facial swelling, tiredness, weakness, or yellowing eyes or skin.
• Your doctor will check your progress and the effects of this medicine at regular visits. Keep all appointments. Blood tests may be needed to check for unwanted effects.
• 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, and body aches
• Increased sweating
• Shortness of breath or trouble breathing
• Swollen glands
• Unusual bleeding, bruising, or weakness

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

• Diarrhea, vomiting, stomach pain, or 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