Description
Cysteamine is used to prevent damage that may be caused by the buildup of cystine crystals in organs such as the kidneys (nephropathic cystinosis). Cystinosis is a hereditary disorder that causes an accumulation of the amino acid cystine within cells, forming crystals that can build up and damage 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.
This product is available in the following dosage forms:
- Capsule, Delayed Release
- Capsule
- Packet
Before Using
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 1 year of age and older. However, safety and efficacy have not been established in children younger than 1 year of age.
Geriatric
No information is available on the relationship of age to the effects of cysteamine in geriatric patients.
Breastfeeding
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 Interactions
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 Interactions
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 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 problems (eg, ulcers, bleeding)—Use with caution. May make these conditions worse.
Proper Use
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 leaflet. Read and follow the instructions carefully. Ask your doctor or pharmacist 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 1 year of age and older, the capsule may be opened and the contents of the capsule sprinkled on food or mixed in formula.
Swallow the delayed-release capsule or granules whole. Do not crush or chew the capsule or contents of the capsule or packet.
Take the delayed-release capsule or granules the same way every day. This means take it at the same time and take it consistently, either with or without food. Avoid high-fat meals close to the time that you will take 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 and after taking Procysbi®.
If you cannot swallow the capsule whole, you may open it and take the capsule or packet contents with certain foods and juices or water:
- Taking this medicine with applesauce or berry jelly: Open the capsule or packet 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 or packet 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 or packet 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 10 milliliters (mL) of fruit juice or water. 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, vitamin D, and thyroid hormone. 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.
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—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 or granules):
- 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 1 year 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 1 year 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. Divide the total daily dose by 2 and take it every 12 hours.
- Children 1 year of age and older—The total dose is the same as the total dose that you are currently taking.
- Children younger than 1 year of age—Use and dose must be determined by your doctor.
- For patients not on cysteamine therapy:
- For oral dosage form (capsules):
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.
You may take a missed dose of Procysbi® as soon as you can 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.
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 capsule in its original container. Keep it in a dry place away from direct light.
Precautions
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, including bone pain, tenderness, or aching, curved spine, loss of appetite, stretch marks, broken bones, joint problems, or unusual weight loss.
Serious skin reactions (eg, erythema multiforme bullosa, toxic epidermal necrolysis) can occur with this medicine. Check with your doctor right away if you have blistering, peeling, or loosening of the skin, chills, cough, diarrhea, itching, joint or muscle pain, red irritated eyes, red skin lesions, sore throat, sores, ulcers on the skin, or white spots in the mouth or on the lips, or unusual tiredness or weakness 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.
Check with your doctor right away if you have bloody or persistent diarrhea, loss of bowel control, stomach pain, or vomiting. These may be symptoms of fibrosing colonopathy.
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 bulging soft spot on the head of an infant, change in ability to see colors, especially blue or yellow, 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. These may be symptoms of benign intracranial hypertension.
This medicine will lower the number of some types of blood cells in your body. Because of this, you may get infections more easily. To help with these problems, avoid being near people who are sick or have infections. Wash your hands often.
This medicine may cause some people to become dizzy or drowsy. Do not drive or do anything else that could be dangerous until you know how this medicine affects you.
Do not drink alcohol while you are using this medicine.
It is important to tell your doctor right away if you are pregnant or have become pregnant while using this medicine.
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
- Diarrhea
- drowsiness
- fever
- loss of appetite
- nausea
- skin rash
- stomach pain
- vomiting
Less common
- Confusion
- dizziness
- headache
- mental depression
- sore throat
- trembling
Rare
- Increased thirst
- seizures
- unusual tiredness or weakness
Incidence not known
- Blistering, peeling, or loosening of the skin
- blurred or double vision
- bone lesions
- bulging soft spot on the 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 bowel control
- 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
- 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
- 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.
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