Prevents buildup of cystine crystals in the kidneys.
There may be other brand names for this medicine.
When This Medicine Should Not Be Used
Do not use this medicine if you have had an allergic reaction to cysteamine or penicillamine.
How to Use This Medicine
Capsule, Delayed Release Capsule
- Your doctor will tell you how much of this medicine to use and how often. Your dose may need to be changed several times in order to find out what works best for you. Do not use more medicine or use it more often than your doctor tells you to.
- This medicine comes with patient instructions. Read and follow these instructions carefully. Ask your doctor or pharmacist if you have any questions.
- Immediate-release capsule (Cystagon®):
- If your child is using this medicine, you may open the immediate-release capsule and mix it in your child's food.
- Delayed-release capsule (Procysbi?)::
- Swallow the delayed-release capsule whole. Do not crush or chew the capsule or contents of the capsule.
- Take this medicine the same way every day. This means take it at the same time and take it consistently, either with or without food. Do not eat high-fat food near the time that you take this medicine.
- Without food: It is best to take this medicine on an empty stomach. Take it at least 30 minutes before a meal or at least 2 hours after a meal.
- With food: If you must eat, you can eat a small amount of food (1/2 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 only with certain foods and juices.
- Applesauce or berry jelly: Open the capsule and sprinkle the granules on 4 ounces (1/2 cup) of only applesauce or berry jelly. Swallow the entire mixture within 30 minutes.
- Apple juice or orange juice: Open the capsule and sprinkle the granules into 4 ounces (1/2 cup) of only apple juice or orange juice. Shake this mixture gently for 5 minutes. Drink it within 30 minutes.
- Feeding tube: Open the capsule and sprinkle the granules on 4 ounces (1/2 cup) of applesauce. Mix and give the medicine through the gastrostomy (feeding) tube into the stomach within 30 minutes. Flush the tube with 8 ounces (1 cup) of apple juice or orange juice. Do not use other liquids to flush the tube.
- You may have to take supplements while using this medicine. Talk to your doctor about this.
If a dose is missed:
- Immediate-release (Cystagon®): If you miss a dose or forget to use your medicine and it is less than 2 hours until your next dose, wait until then and take your normal dose at the normal time. If it is more than 2 hours, take the missed dose as soon as you can. Never take extra medicine to make up for the missed dose.
- Delayed-release (Procysbi?): If you miss a dose or forget to use your medicine and it is less than 4 hours until your next dose, wait until then and take your normal dose at the normal time. If it is more than 4 hours, take the missed dose as soon as you can. Never take extra medicine to make up for the missed dose.
How to Store and Dispose of This Medicine
- Store the medicine in a closed container at room temperature, away from heat, moisture, and direct light. Store the delayed-release capsules in the original container. Keep it in a dry place away from light.
- Ask your pharmacist, doctor, or health caregiver about the best way to dispose of any outdated medicine or medicine no longer needed.
- Keep all medicine away from children and never share your medicine with anyone.
Drugs and Foods to Avoid
Ask your doctor or pharmacist before using any other medicine, including over-the-counter medicines, vitamins, and herbal products.
Warnings While Using This Medicine
- Make sure your doctor knows if you are pregnant or breastfeeding, or if you have liver disease, ulcers or bleeding in your stomach or intestines, or a history of head injury, depression, or seizures.
- Your doctor may need to check your skin, joints, and bones for any problems that may be caused by this medicine. You are more likely to have these problems if you take high doses of this medicine. Tell your doctor right away if you have changes or problems with your skin, bones, or joints, such as red-purple rash, stretch marks, pain, bruises, or loose joints.
- This medicine may make you dizzy or drowsy. Avoid driving, using machines, or doing anything else that could be dangerous until you know how this medicine affects you.
- Rarely, this medicine could cause higher pressure inside your head. You may need to be examined by an eye doctor to detect this problem. Tell your doctor if you have an unusual headache, trouble seeing, pain when you move your eyes, double vision, blurry vision, or buzzing in your ears.
- Your doctor will need to check your blood at regular visits while you are using this medicine. Be sure to keep all appointments.
Possible Side Effects While Using This Medicine
Call your doctor right away if you notice any of these side effects:
- Allergic reaction: Itching or hives, swelling in your face or hands, swelling or tingling in your mouth or throat, chest tightness, trouble breathing
- Blistering, peeling, or red skin rash
- Bloody vomit or black, tarry stools
- Bone, joint, or leg pain
- Depression, severe sleepiness or weakness
- Headache, ringing in your ears, dizziness, nausea, blurred or double vision, or pain when you move your eyes
- Fever, chills, cough, sore throat, and body aches
- Red-purple lines on your arms, face, legs, trunk, or groin
- Severe stomach pain, nausea, vomiting, loss of appetite
- Unusual bleeding, bruising, or weakness
If you notice these less serious side effects, talk with your doctor:
- Mild headache
- Mild nausea, vomiting, constipation, diarrhea, stomach upset
- Mild skin rash or itching
- Mild tiredness