Proper Use

Drug information provided by: Micromedex

Take this medicine only 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.

This medicine comes with a patient information insert. Read and follow the instructions carefully. Ask your doctor if you have any questions.

If you vomit your dose of cysteamine within 20 minutes of taking it, take the dose again. However, if you vomit the dose a second time, do not repeat the dose but wait and take your next dose as scheduled. Also, if vomiting occurs more than 20 minutes after you take your dose, do not repeat the dose.

For children younger than 6 years of age, the capsule may be opened and the contents of the capsule sprinkled on food or mixed in formula.

Swallow the delayed-release capsule whole. Do not crush or chew the capsule or contents of the capsule.

Take the delayed-release capsule the same way every day. This means take it at the same time and take it consistently, either with or without food. High-fat meals may decrease absorption of this medicine.

  • Without food: Take it at least 30 minutes before meals or at least 2 hours after meals.
  • With food: You can eat a small amount of food (½ cup) between 1 hour before or after taking Procysbi™.

If you cannot swallow the capsule whole, you may open it and take the capsule contents with certain foods and juices:

  • Taking this medicine with applesauce or berry jelly: Open the capsule and sprinkle the granules on 4 ounces (½ cup) of applesauce or berry jelly. Swallow the entire mixture within 30 minutes.
  • Taking this medicine with apple juice or orange juice: Open the capsule and sprinkle the granules into 4 ounces (½ cup) of apple juice or orange juice. Shake it gently for 5 minutes. Drink it within 30 minutes.
  • If you are using a gastrostomy tube: Open the capsule and sprinkle the granules on 4 ounces (½ cup) of applesauce. Mix and give the medicine through the gastrostomy tube into the stomach within 30 minutes. Flush the tube with 8 ounces (1 cup) of apple juice or orange juice. Do not use any liquid to flush the tube.

It is important that you follow any special instructions from your doctor, such as taking dietary supplements. These supplements will replace minerals lost through the kidneys.

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.

  • To prevent buildup of cystine crystals in the kidneys:
    • For oral dosage form (capsules):
      • Adults and teenagers—The starting dose must be determined by your doctor. Your doctor may gradually increase your dose.
      • Children—The starting dose is based on body size and must be determined by your doctor. Your doctor may gradually increase your dose.
    • For oral dosage form (delayed-release capsules):
      • For patients not on cysteamine therapy:
        • Adults—The starting dose is based on body size and must be determined by your doctor. The maintenance dose is usually 1.3 gram per square meter of body size per day (gram/m[2]/day) divided in two equal doses and taken every 12 hours. Your doctor may adjust your dose as needed.
        • Children 6 years of age and older—Dose is based on body size and must be determined by your doctor. Your doctor may adjust your dose as needed.
        • Children younger than 6 years of age—Use and dose must be determined by your doctor.
      • For patients taking cysteamine immediate-release capsules:
        • Adults—The total dose is the same as the total dose that you are currently taking.
        • Children 6 years of age and older—The total dose is the same as the total dose that you are currently taking.
        • Children younger than 6 years of age—Use and dose must be determined by your doctor.

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.

If you missed a dose of the Procysbi™ and your next dose is less than 4 hours away, wait and take your next dose at the normal time.

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 the medicine in a closed container at room temperature, away from heat, moisture, and direct light. Keep from freezing.

Store the delayed-release capsules in its original container. Keep it in a dry place away from light.