Procysbi

Generic Name: cysteamine (Oral route)

sis-TEE-a-meen

Commonly used brand name(s)

In the U.S.

  • Cystagon
  • Procysbi

Available Dosage Forms:

  • Capsule, Delayed Release
  • Capsule

Therapeutic Class: Renal-Urologic Agent

Uses For Procysbi

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.

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

Before Using Procysbi

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:

Allergies

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.

Pediatric

Appropriate studies performed to date have not demonstrated pediatric-specific problems that would limit the usefulness of cysteamine in children 6 years of age and older. However, safety and efficacy have not been established in children younger than 6 years of age.

Geriatric

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

Pregnancy

Pregnancy Category Explanation
All Trimesters C Animal studies have shown an adverse effect and there are no adequate studies in pregnant women OR no animal studies have been conducted and there are no adequate studies in pregnant women.

Breast Feeding

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.

Interactions with Medicines

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.

Interactions with Food/Tobacco/Alcohol

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. Discuss with your healthcare professional the use of your medicine with food, alcohol, or tobacco.

Other Medical Problems

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.

Proper Use of cysteamine

This section provides information on the proper use of a number of products that contain cysteamine. It may not be specific to Procysbi. Please read with care.

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.

Precautions While Using Procysbi

It is important that your doctor check your or your child's 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 or your child 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 or your child are using this medicine.

This medicine may cause stomach or bowel ulcers or bleeding. Check with your doctor right away if you or your child 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 or your child develop seizures, depression, or if you become very sleepy while taking this medicine.

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.

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

Procysbi Side Effects

Along with its needed effects, a medicine may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.

Check with your doctor immediately if any of the following side effects occur:

More common
  • Abdominal or stomach pain
  • diarrhea
  • drowsiness
  • fever
  • loss of appetite
  • nausea or vomiting
  • skin rash
Less common
  • Confusion
  • dizziness
  • headache
  • mental depression
  • sore throat
  • trembling
Rare
  • Convulsions
  • increased thirst
  • unusual tiredness or weakness
Incidence not known
  • Blistering, peeling, or loosening of the skin
  • blurred or double vision
  • bone lesions
  • bulging soft spot on head of an infant
  • change in the ability to see colors, especially blue or yellow
  • chills
  • continuing ringing or buzzing or other unexplained noise in the ears
  • cough
  • eye pain
  • hearing loss
  • itching
  • joint or muscle pain
  • loss of appetite
  • loss of vision
  • pain behind the eyes
  • pain in the legs
  • pain with eye movement
  • red skin lesions, often with a purple center
  • red, irritated eyes
  • reddish purple lines on the arms, face, legs, trunk, or groin
  • severe headache
  • sore throat
  • sores, ulcers, or white spots in the mouth or on the lips

Some side effects may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

Less common
  • Breath odor
  • constipation
  • hives or welts
  • redness of the skin
  • unusual drowsiness, dullness, tiredness, weakness, or feeling of sluggishness
  • weight loss

Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

See also: Side effects (in more detail)

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