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Specialty Drug
CYSTEAMINE breaks down cystine in the body. It is used to treat nephropathic cystinosis in children and adults. This medicine is not a cure. Compare cystine depleting agents.
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What is Procysbi?

Commonly Used Brand Name(s)Cystagon, Procysbi

Therapeutic ClassificationsRenal-Urologic Agent

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OverviewTOP

Cysteamine is used to prevent damage that may be caused by the buildup of cystine crystals in organs such as the kidneys. This medicine works by removing the extra cystine from the cells of the body.

This medicine is available only with your doctor's prescription.

Proper UseTOP

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 2 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. Mix the granules into the food. Swallow the entire mixture within 30 minutes. Do not chew the granules. Do not save the applesauce or berry jelly and granules for later use.
  • Taking this medicine with fruit juice (except grapefruit juice): Open the capsule and sprinkle the granules into 4 ounces (½ cup) of juice. Gently stir until mixed. Drink it within 30 minutes. Do not chew the granules. Do not save the fruit juice or granules for later use.
  • If you are using a gastrostomy tube: Open the capsule and sprinkle the granules on 4 ounces (½ cup) of applesauce. For children receiving 1 or 2 capsules, use a minimum of 1 ounce of applesauce. Use only strained applesauce with no chunks. Mix and give the medicine through the gastrostomy tube into the stomach within 30 minutes. Flush the tube with 8 ounces (1 cup) of fruit juice. Do not save the mixture for later use.

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.

If you are taking medicines containing bicarbonate or carbonate, take Procysbi® at least 1 hour before or after these medicines.

DosingTOP

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 capsules are taken every 12 hours. Your doctor may adjust your dose as needed.
        • Children 2 years of age and older— The starting dose is based on body size and must be determined by your doctor. The capsules are taken every 12 hours. Your doctor may adjust your dose as needed.
        • Children younger than 2 years of age—Use and dose must be determined by your doctor.
      • For patients switching from cysteamine immediate-release capsules:
        • Adults—The total dose is the same as the total dose that you are currently taking.
        • Children 2 years of age and older—The total dose is the same as the total dose that you are currently taking.
        • Children younger than 2 years of age—Use and dose must be determined by your doctor.

Missed DoseTOP

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.

You may take a missed dose of Procysbi® as soon as possible up to 8 hours after your scheduled dose. If you missed a dose of Procysbi® and your next dose is less than 4 hours away, wait and take your next dose at the normal time. Do not take 2 doses at one time to make up for a missed dose

Use & StorageTOP

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.

Before UsingTOP

In deciding to use a medicine, the risks of taking the medicine must be weighed against the good it will do. This is a decision you and your doctor will make. For this medicine, the following should be considered:

AllergiesTOP

Tell your doctor if you have ever had any unusual or allergic reaction to this medicine or any other medicines. Also tell your health care professional if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.

PediatricTOP

Appropriate studies performed to date have not demonstrated pediatric-specific problems that would limit the usefulness of cysteamine in children 2 years of age and older. However, safety and efficacy have not been established in children younger than 2 years of age.

GeriatricTOP

No information is available on the relationship of age to the effects of cysteamine in geriatric patients.

Breast FeedingTOP

There are no adequate studies in women for determining infant risk when using this medication during breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.

Drug InteractionsTOP

Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. Tell your healthcare professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicine.

Other InteractionsTOP

Certain medicines should not be used at or around the time of eating food or eating certain types of food since interactions may occur. Using alcohol or tobacco with certain medicines may also cause interactions to occur. The following interactions have been selected on the basis of their potential significance and are not necessarily all-inclusive.

Using this medicine with any of the following is usually not recommended, but may be unavoidable in some cases. If used together, your doctor may change the dose or how often you use this medicine, or give you special instructions about the use of food, alcohol, or tobacco.

  • Ethanol

Other Medical ProblemsTOP

The presence of other medical problems may affect the use of this medicine. Make sure you tell your doctor if you have any other medical problems, especially:

  • Blood problems, or history of or
  • Bone problems or
  • Brain disease or
  • Depression, history of or
  • Head injury, history of or
  • Increased pressure in the head or
  • Liver disease or
  • Seizures, history of or
  • Stomach or bowel ulcers or bleeding—Use with caution. May make these conditions worse.

PrecautionsTOP

It is important that your doctor check your progress at regular visits to make sure that this medicine is working properly. Blood tests and eye exams will be needed to check for unwanted effects.

Using high doses of this medicine may cause a condition called Ehler-Danlos like syndrome. Check with your doctor right away if you have unusual changes on your skin and bones, such as stretch marks, broken bones, or joint problems.

Serious skin reactions can occur with this medicine. Check with your doctor right away if you have blistering, peeling, or loosening of the skin, red skin lesions, severe skin rash, sores or ulcers on the skin, or chills while you are using this medicine.

This medicine may cause stomach or bowel ulcers or bleeding. Check with your doctor right away if you have severe stomach pain, nausea, vomiting, loss of appetite, or if you are vomiting blood that looks like coffee grounds.

This medicine may affect how your brain is working. Tell your doctor right away if you develop seizures, depression, or if you become very sleepy while taking this medicine.

Check with your doctor right away if you have dizziness, headache, hearing loss, continued ringing in the ears, nausea, blurred or double vision, loss of vision, pain behind the eyes, or pain with eye movement.

This medicine may cause some people to become dizzy or drowsy. Make sure you know how you react to this medicine before you drive, use machines, or do anything else that could be dangerous if you are dizzy or not alert .

Do not drink alcohol while you are using this medicine.

It is important to tell your doctor right away if you are pregnant or may become pregnant while using this medicine.

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