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.

Carefully follow your doctor's instructions about a special diet.

This medicine should come with a Medication Guide or patient instructions. Read and follow these instructions carefully. Ask your doctor if you have any questions.

You may take the oral liquid and tablet with or without food.

To use the capsule:

  • Take the capsule on an empty stomach at least 1 hour before or 2 hours after a meal.
  • If needed, you may open the capsule and put the contents in a small amount of water, formula, or applesauce. Give the medicine as soon as it is mixed.

To use the oral liquid:

  • Allow the bottle to warm to room temperature for 30 to 60 minutes.
  • Shake the bottle for at least 20 seconds until the solid cake at the bottom of the bottle is completely dissolved.
  • Insert the bottle adapter and measure the medicine with an oral syringe.
  • Shake the bottle for at least 5 seconds before measuring each dose to remove any particles at the bottom of the bottle.

To use the tablet:

  • If you are unable to swallow the tablet whole, you can mix the tablet with water in an oral syringe or crush the tablet and mix with applesauce. Do not mix it with any other food or liquid.
    • To prepare for an applesauce mixture:
      • Wash your hands well before preparing the medicine.
      • Measure about 1 teaspoon of applesauce and put it into a clean container (eg, glass or bowl).
      • Place the tablet onto a spoon. Crush one tablet at a time between 2 spoons until the tablet is crushed to a fine powder.
      • Carefully place the powder into the clean container of applesauce. Make sure that no tablet powder remains on the teaspoon. If more than 1 tablet is needed, repeat the previous steps. Stir the mixture well.
      • Swallow all of the mixture right away or within 2 hours of mixing. Repeat this step until no mixture is left in the container.
      • Measure another 1 teaspoon of applesauce and place it in the container. Stir the fresh applesauce with any remaining tablet powder until mixed well. Swallow this mixture right away. This will make sure any remaining medicine in the container is administered.
    • To prepare for an oral syringe:
      • Wash your hands well before preparing the medicine.
      • Use only 1 or 2 tablets in an oral syringe at a time.
      • For 1 tablet: place the tablet in the oral syringe. Add 2.6 milliliters (mL) of room temperature water and pull the plunger back to the 3 mL mark. For 2 tablets: place the 2 tablets in the oral syringe. Add 5 mL of room temperature water.
      • Replace the cap on the syringe and let it sit for at least 15 minutes for 2 milligrams (mg) tablets and at least 60 minutes for 5 mg or 10 mg tablets.
      • Turn the syringe up and down for at least 30 seconds.
      • Check the mixture in the syringe. The tablet should have broken down into small pieces and are evenly spread in the water (suspension). Do not take the suspension if there are any large pieces of tablet in it or if there are any tablet pieces that are not evenly spread through the water.
      • If the tablet is not fully dissolved, let it sit for another 10 minutes. Then, turn the syringe up and down for at least 30 seconds.
      • Place the tip of the syringe in the mouth. If you are giving it to a child, place the tip of the syringe along the inner cheek of the child's mouth.
      • Slowly push the plunger down until a small amount of air is left in the syringe, between the plunger and the tip. Do not press the plunger all the way down to the end of the syringe.
      • Draw up an additional 2 mL of water into the syringe. Replace the cap on the syringe. Shake well to spread the remaining tablet pieces evenly through the water. If particles are still present, repeat this step one more time.

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 hereditary tyrosinemia type 1:
    • For oral dosage forms (capsules or suspension):
      • Adults—Dose is based on body weight and must be determined by your doctor. The starting dose is usually 0.5 milligram (mg) per kilogram (kg) of body weight 2 times a day. Your doctor may adjust the dose as needed. However, the dose is usually not more than 1 or 2 mg per kg of body weight per day.
      • Children—Dose is based on body weight and must be determined by your doctor. The starting dose is usually 0.5 mg per kg of body weight 2 times a day. Your doctor may adjust the dose as needed. However, the dose is usually not more than 1 or 2 mg per kg of body weight per day.
    • For oral dosage form (tablets):
      • Adults and children—Dose is based on body weight and must be determined by your doctor. The starting dose is usually 0.5 milligrams per kilogram (mg/kg) of body weight 2 times a day. Your doctor may adjust the dose as needed. However, the dose is usually not more than 1 to 2 mg/kg of body weight 2 times a day.

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.

Store in the refrigerator. Do not freeze.

Once opened, you may store the bottle of oral liquid at room temperature for up to 60 days. Throw away any unused medicine after 60 days.

Store the tablets in its original container at room temperature. Keep the tablets and the medicine prepared in an oral syringe and with the applesauce away from direct light. Throw away unused mixture within 2 hours after mixing with applesauce or within 24 hours after mixing in the oral syringe.

Portions of this document last updated: April 01, 2024

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