Procysbi

Procysbi treats cystinosis of the kidneys. Avoid eating foods that are high in fat close to the time that you will take a dose of Procysbi.

Procysbi Overview

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Procysbi is a prescription medication used to treat cystinosis of the kidneys. Cystinosis is a rare condition that causes cystine, a protein building block, to build up in the cells of the body. Procysbi belongs to a class of medications called anti-cystine agents, which help by decreasing the levels of cystine in the body.

This medication comes as a delayed-release capsules to be taken by mouth or by gastric tube (feeding tube inserted into the stomach) every 12 hours.

Swallow Procysbi capsules whole. Do not crush or chew Procysbi or the capsule contents. 

The most common side effects with Procysbi include vomiting, stomach pain or discomfort, and headache. Procysbi capsules may also cause dizziness. Do not drive or operate heavy machinery until you know how this medication will affect you.

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Procysbi Cautionary Labels

precautionsprecautionsprecautionsprecautionsprecautionsprecautionsprecautions

Uses of Procysbi

Procysbi is a prescription medication used to treat cystinosis of the kidneys in adults and children 6 years and older. Cystinosis is a rare condition that causes cystine, a protein building block, to build up in the cells of the body.

This medication may be prescribed for other uses. Ask your doctor or pharmacist for more information.

Manufacturer

Procysbi Drug Class

Procysbi is part of the drug class:

Side Effects of Procysbi

Serious side effects have been reported with Procysbi. See “Drug Precautions” section.

Common side effects of Procysbi include the following:

  • vomiting
  • stomach (abdominal) pain or discomfort
  • headache
  • nausea
  • diarrhea
  • loss of appetite or decreased appetite
  • breath odor
  • tiredness
  • dizziness
  • skin odor
  • skin rash

This is not a complete list of Procysbi side effects. Ask your doctor or pharmacist for more information.

Tell your doctor if you have any side effect that bothers you or that does not go away. 

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

 

Procysbi Interactions

Tell your doctor about all the medicines you take including prescription and non-prescription medicines, vitamins, and herbal supplements. Especially tell your doctor if you take bicarbonate.

This is not a complete list of Procysbi drug interactions. Ask your doctor or pharmacist for more information.

Procysbi Precautions

Serious side effects have been reported with Procysbi including:

  • Skin, bone, and joint problems: People treated with high doses of this medication may develop problems such as stretch marks, bone injuries (such as fractures), bone deformities, and joint problems. Check your skin while taking cysteamine. 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 and may sometimes be severe. Tell your doctor right away if you get a skin rash.
  • Central nervous system symptoms: Some people who take other medicines that contain cysteamine 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 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. 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 cysteamine: 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.

Procysbi can make you sleepy, less alert than normal, and dizziness. Do not drive or operate heavy machinery until you know how Procysbi affects you.

Do not take Procysbi if you have an allergic or hypersensitive reaction to Procysbi, its ingredients, or penicillamine.

Procysbi Food Interactions

Medicines can interact with certain foods. In some cases, this may be harmful and your doctor may advise you to avoid certain foods. In the case of Procysbi, there are no specific foods that you must exclude from your diet when receiving Procysbi.

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.
  • When sprinkling Procysbi on food, only use applesauce or berry jelly. 
  • When sprinkling Procysbi in juice, only use apple juice or orange juice. 

Inform MD

Before taking Procysbi, tell your doctor about all of your medical conditions. Especially tell your doctor if you:

  • are allergic to Procysbi, its ingredients, or penicillamine
  • 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
  • are breastfeeding or plan to breastfeed

Tell your doctor about all the medicines you take including prescription and non-prescription medicines, vitamins, and herbal supplements.

Procysbi and Pregnancy

Tell your doctor if you are pregnant or plan to become pregnant.

The FDA categorizes medications based on safety for use during pregnancy. Five categories - A, B, C, D, and X, are used to classify the possible risks to an unborn baby when a medication is taken during pregnancy.

Procysbi falls into category C. There are no adequate and well-controlled studies in pregnant women. It is not known if Procysbi will harm your unborn baby. Procysbi should only be used during pregnancy if the potential benefit justifies the potential risk to the fetus.  Talk with your doctor about the benefits and risks of taking Procysbi during pregnancy.

Procysbi and Lactation

Tell your doctor if you are breastfeeding or plan to breastfeed.

It is not known if Procysbi is excreted in human breast milk or if it will harm your nursing baby. Because many drugs are present in human milk and because of the potential for serious adverse reactions in nursing infants from Procysbi, you should not breastfeed during treatment with Procysbi. Talk with your doctor about the best way to feed your baby if you take Procysbi.

Procysbi Usage

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 (feeding tube inserted into the stomach), 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.

Procysbi Dosage

Take this medication exactly as prescribed by your doctor. Follow the directions on your prescription label carefully.

The dose your doctor recommends may be based on the following:

  • the state of the condition being treated
  • how you respond to this medication
  • if you have or have not received cysteamine containing medicines

The recommended total daily dose of Procysbi to treat cystinosis of the kidneys is 1.3 gram/m2/day, in two divided doses given every 12 hours.

  • Patients who have not received cysteamine containing medicines before will be started on a lower dose. Then the doctor will increase the dose slowly over 4 to 6 weeks to help reduce the risk of side-effects. 

Procysbi Overdose

If you take too much Procysbi call your healthcare provider or local Poison Control Center, or seek emergency medical attention right away.

 

Other Requirements

  • 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 this and all medications out of the reach of children.