Proper Use

Drug information provided by: IBM 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.

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:

  • Take the oral liquid with or without food.
  • 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:

  • Take it with or without food.
  • If you cannot swallow the tablet, you can dissolve it in water and take the solution using an oral syringe. It can also be crushed and mixed with applesauce. Do not mix it with any other food or liquid.
    • To prepare for an applesauce mixture:
      • Clean your hands well before preparing the medicine.
      • Place the tablet onto a teaspoon and a second teaspoon on top of the tablet. Press and rotate the second teaspoon until the tablet is crushed into a fine powder. Crush only 1 tablet at a time.
      • Carefully place the powder into 1 teaspoon of applesauce. If more than 1 tablet is needed, repeat the previous steps. Mix well.
      • Swallow this mixture right away. Add about 1 more teaspoon of applesauce to the same container, mix, and swallow right away to get the full dose of this medicine.
      • If you can not take the mixture right away, you may store it within 2 hours after adding applesauce.
    • To prepare for an oral syringe:
      • Clean 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 tablets in the oral syringe. Add 5 mL of room temperature water.
      • Cap the syringe and let it sit for at least 60 minutes.
      • 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. Take this mixture right away.
      • If the tablet has not fully dissolved, let it sit for another 10 minutes. Then, turn the syringe up and down for at least 30 seconds. Do not take this medicine if the tablet has still not completely dissolved.
      • If you can not take the mixture right away, you may store it at room temperature in the capped oral syringe, protected from direct sunlight for up to 24 hours.
      • To make sure the full dose is given, refill the syringe with water. Use 2 mL of water and shake the syringe well for 10 seconds, then give the mixture.

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—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 mg per kg of body weight 2 times a 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 mg per 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 any mixture that is not used within 2 hours after mixing with applesauce or within 24 hours after mixing in the oral syringe.