"The U.S. Food and Drug Administration today approved Procysbi (cysteamine bitartrate) for the management of nephropathic cystinosis in children and adults. Procysbi was granted orphan product designation because it is intended to treat a rare dis"...
(cysteamine bitartrate) Delayed-release Capsules
What is PROCYSBI?
PROCYSBI is a prescription medicine used to manage a medical condition called nephropathic cystinosis, in adults and children 6 years of age and older.
It is not known if PROCYSBI is safe and effective in children under 6 years of age.
Who should not take PROCYSBI?
Do not take PROCYSBI if you are allergic to penicillamine.
What should I tell my doctor before taking PROCYSBI?
Before you take PROCYSBI, tell your doctor if you:
- have a skin rash or bone problems
- have a history of seizures, exhaustion, sleepiness, depression or other nervous system problems
- have or have had stomach or bowel (intestinal) problems including ulcers or bleeding
- have liver or blood problems
- are pregnant or plan to become pregnant. It is not known if PROCYSBI will harm your unborn baby. Tell your doctor right away if you think that you are pregnant. Talk with your doctor about the benefits and risks of taking PROCYSBI during pregnancy.
- are breastfeeding or plan to breastfeed. You should not breastfeed during treatment with PROCYSBI. Talk with your doctor about the best way to feed your baby if you take PROCYSBI.
Tell your doctor about all medicines that you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements.
Know the medicines you take. Keep a list of them to show your doctor and pharmacist when you get a new medicine.
How should I take PROCYSBI?
- Take PROCYSBI exactly as your doctor tells you to.
- Your doctor may start you on a low dose of PROCYSBI, and slowly increase your dose to help avoid side effects, especially if you have not taken a medicine that contains cysteamine bitartrate before.
- Do not change your dose of PROCYSBI unless your doctor tells you to.
- PROCYSBI is usually taken 2 times each day, every 12 hours.
- Take PROCYSBI the same way each time, either without
eating or with a small amount of food, as follows:
- If you take PROCYSBI without eating, take PROCYSBI at least 30 minutes before you eat or at least 2 hours after you eat.
- If you are not able to take PROCYSBI without eating, you can eat a small amount of food (½ cup) between 1 hour before you take PROCYSBI to 1 hour after you take it.
- Avoid eating foods that are high in fat close to the time that you will take a dose of PROCYSBI.
- Talk to your doctor or pharmacist if you have questions about how to take PROCYSBI.
- Swallow PROCYSBI capsules whole.
- Do not crush or chew PROCYSBI or the capsule contents.
- If you cannot swallow the whole capsule, you can open it and take the capsule contents with certain foods and juices. PROCYSBI can also be given through a gastrostomy tube, 12 French or larger.
Taking PROCYSBI with certain foods:
You can only use applesauce or berry jelly.
- Open the capsule.
- Sprinkle the granules on 4 ounces (½ cup) of applesauce or berry jelly. Swallow mixture within 30 minutes.
Taking PROCYSBI with certain juices:
You can only use apple juice or orange juice.
- Open the capsule.
- Sprinkle the granules into 4 ounces (about ½ cup) of apple juice or orange juice.
- Shake gently for 5 minutes.
- Drink within 30 minutes.
Taking PROCYSBI through a gastrostomy tube, 12 French or larger:
You can only use applesauce.
- Open the capsule.
- Sprinkle the granules on 4 ounces (½ cup) of applesauce. Mix the granules with the applesauce.
- Give the medicine through the gastrostomy tube into the stomach within 30 minutes.
- After giving the mixture, flush the gastrostomy tube with 8 ounces (about 1 cup) of apple juice or orange juice to clear the tube. Do not use other liquids to flush the tube.
- If you miss a dose, take it as soon as possible. If it is within 4 hours of the time the next dose is due, skip the missed dose. Take the next dose at your regularly scheduled time. Do not take 2 doses at one time to make up for a missed dose.
- If you take too much PROCYSBI, call your doctor or go to the nearest hospital emergency room right away.
- Your doctor should do blood tests to measure the amount of cystine inside your white blood cells or to measure the amount of PROCYSBI in your blood before you start taking it and regularly during treatment with PROCYSBI. This will help your doctor to prescribe the dose of PROCYSBI that is right for you.
- Take supplements if prescribed by your doctor, such as salt or mineral replacement supplements, vitamin D, or thyroid hormone.
What should I avoid while taking PROCYSBI?
Do not drive or operate heavy machinery until you know how PROCYSBI affects you. PROCYSBI can make you sleepy or less alert than normal.
What are the possible side effects of PROCYSBI?
PROCYSBI can cause serious side effects, including:
- Skin, bone, and joint problems. People treated with high doses of cysteamine bitartrate may develop abnormal changes of their skin and bones. These changes may include stretch marks, bone injuries (such as fractures), bone deformities, and joint problems. Check your skin while taking PROCYSBI. Tell your doctor if you notice any skin changes. Your doctor will check you for these problems.
- Skin rash. Skin rash is common with cysteamine bitartrate and may sometimes be severe. Tell your doctor right away if you get a skin rash. Your dose of PROCYSBI may need to be decreased until the rash goes away. If the rash is severe, your doctor may tell you to stop taking PROCYSBI.
- Central nervous system symptoms. Some people who take other medicines that contain cysteamine bitartrate develop seizures, depression, and become very sleepy. The medicine may affect how your brain is working (encephalopathy). Tell your doctor right away if you develop any of these symptoms.
- Stomach and bowel (intestinal) problems. Some people who take other medicines that contain cysteamine bitartrate develop
ulcers and bleeding in their stomach or bowel.
Tell your doctor right away if you get stomach-area pain, nausea, vomiting, loss of appetite, or vomit blood.
- Low white blood cell count and certain abnormal liver function blood tests. Your doctor should check you for these problems.
- Benign intracranial hypertension (pseudotumor cerebri) has happened in some people who take immediate-release cysteamine bitartrate. This is a condition where there is high pressure in the fluid around the brain. Your doctor should do eye examinations to find and treat this problem early.
Tell your doctor right away if you develop any of the following symptoms while taking PROCYSBI: headache, buzzing or “whooshing” sound in the ear, dizziness, nausea, double vision, blurry vision, loss of vision, pain behind the eye or pain with eye movement.
The most common side effects with PROCYSBI include:
- abdominal pain or discomfort
- loss of appetite or decreased appetite
- breath odor
- skin odor
- skin rash
Tell your doctor if you have any side effect that bothers you or that does not go away.
These are not all of the possible side effects of PROCYSBI. For more information, ask your doctor or pharmacist.
Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
How should I store PROCYSBI?
- Store PROCYSBI at room temperature between 68° to 77°F (20° to 25°C).
- Store PROCYSBI in a dry place away from light.
- Keep PROCYSBI tightly closed in the original bottle.
- The PROCYSBI bottle contains a desiccant packet to help keep your medicine dry. Do not eat or throw away the desiccant packet.
Keep PROCYSBI and all medicines out of the reach of children.
General information about PROCYSBI
Medicines are sometimes prescribed for purposes other than those listed in a Patient Information leaflet. Do not use PROCYSBI for a condition for which it was not prescribed. Do not give PROCYSBI to other people, even if they have the same symptoms that you have. It may harm them.
If you would like more information about PROCYSBI, talk with your doctor. You can ask your doctor or pharmacist for information about PROCYSBI that is written for health professionals. For more information call 1-855-888-4004.
What are the ingredients in PROCYSBI?
Active ingredients: cysteamine bitartrate
Inactive ingredients: microcrystalline cellulose, Eudragit ® L 30 D-55, hypromellose, talc, triethyl citrate, sodium lauryl sulfate, and purified water.
Capsule shell ingredients: gelatin, titanium dioxide, blue ink and white ink.
This Patient Information has been approved by the U.S. Food and Drug Administration.
Last reviewed on RxList: 5/13/2013
This monograph has been modified to include the generic and brand name in many instances.
Additional Procysbi Information
Report Problems to the Food and Drug Administration
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit the FDA MedWatch website or call 1-800-FDA-1088.
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