Proper Use

Drug information provided by: Merative, Micromedex®

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 (mL) or 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 (mL) or 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.

Dosing

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 caused by NAGS deficiency:
      • Adults and children—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.
    • For hyperammonemia caused by PA or MMA:
      • Adults and children—Dose is based on body weight or body surface area and must be determined by your doctor.
        • Weighing more than 15 kilograms (kg)—3.3 grams (g) per square meter (m2), divided and given into 2 equal doses (12 hours apart) for at least 7 days.
        • Weighing less than or equal to 15 kg—150 milligrams (mg) per kilogram (kg) per day, divided and given into 2 equal doses (12 hours apart) for at least 7 days.

Missed Dose

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.

Storage

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.

Portions of this document last updated: Feb. 01, 2024

Copyright: © Merative US L.P. 1973, 2024. All rights reserved. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes.