Please ensure JavaScript is enabled for purposes of website accessibility Cystinosis Treatment | Staying on PROCYSBI® (cysteamine bitartrate)

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Staying on Treatment

HOW TO MAKE THE MOST OF TREATMENT

PROCYSBI® is designed to work over 12 hours. PROCYSBI (cysteamine bitartrate) delayed-release capsules or delayed-release oral granules should be taken every 12 hours at the same time every day as prescribed by the doctor. For example, a person might take PROCYSBI at 8:00 a.m. and again at 8:00 p.m.

Cystinosis Treatment Icon

Reminders about taking PROCYSBI:

  • Take PROCYSBI every 12 hours (2 times every 24 hours) at the same time every day
  • Take PROCYSBI at least 1 hour before or after you take medicines that contain bicarbonate or carbonate
  • Work with your healthcare team to schedule regular cystine level testing
  • Food and drinks can affect how the medicine in PROCYSBI is released, so it’s important to take PROCYSBI only as directed
  • Do not eat for at least 2 hours before taking PROCYSBI and at least 30 minutes after you take it
  • If you can't take PROCYSBI without eating, take it with no more than a ½ cup (4 oz.) of food up to 1 hour before or after you take PROCYSBI
  • Avoid high-fat foods (such as avocados, cheese, and nuts) right before and after dosing
  • Take PROCYSBI the same way each time

If you have questions about how to take PROCYSBI, review the Instructions for Use or contact your doctor or pharmacist.

Post-transplant: Staying on treatment

Staying on treatment after a kidney transplant is important. A new kidney does not end cystinosis because other organs are (or the rest of the body is) still affected, but CDT* can help delay or reduce new damage in other organs.

*CDT = Cystine-depleting therapy

Cystine levels facts icon

Tip:

Manage your levels by taking your medicine at the same time every day, even when you feel good. Your cystine levels can continue to rise and cause organ damage without you knowing. This may occur if your medicine is not taken as prescribed.

The role of cystine level testing

Your doctor will order cystine level tests to measure the amount of cystine in your white blood cells (WBCs) before and during treatment with PROCYSBI. Cystine level tests help your doctor determine:

  • The dose of PROCYSBI that is right for you
  • How well the medicine is working to manage your cystine levels
  • When and how much the dose should be increased in growing children

Types of cystine level tests

Your cystine levels can be measured by one of two tests. The tests measure your levels differently. Keep in mind, your target cystine level range will depend on the test you take.

TYPE OF TEST MIXED LEUKOCYTES TEST GRANULOCYTES TEST
TARGET CYSTINE
LEVEL RANGE		 Less than 1.0 nmol 1/2 cystine/mg protein Less than 1.9 nmol 1/2 cystine/mg protein
TESTING INSTITUTION Baylor Genetics University of California San Diego (UCSD)
WHEN TO GET THE TEST Your doctor will order WBC cystine level testing at the same point in your CDT dosing schedule.

These two tests are not interchangeable. It is important to have the same test every time so your doctor can compare the results every time.

How often to have cystine level tests

IF PROCYSBI IS YOUR FIRST CDT AGE FREQUENCY OF CYSTINE LEVEL TESTING
Children from age 1 year to younger than 6 years
  • 2 weeks after starting PROCYSBI
  • Continually as long as dose adjustments are needed
  • After target cystine levels are reached, at least once a month for 3 months, then every 3 months for a year, and then at least 2 times a year
Children and adults aged 6 years and older
  • 2 weeks after reaching the recommended long-term dose
  • Continually as long as dose adjustments are needed
  • After target cystine levels are reached, at least once a month for 3 months, then every 3 months for a year, and then at least 2 times a year
IF YOU’RE SWITCHING TO PROCYSBI FROM IMMEDIATE-RELEASE CDT Children and adults aged 1 year and older
  • 2 weeks after starting PROCYSBI
  • Continually as long as dose adjustments are needed
  • After that, every 3 months for 6 months and then at least 2 times a year

Work with your healthcare team to create a schedule for cystine level tests based on your treatment history and response to PROCYSBI. Your Patient Access Liaison (PAL) can tell you about cystine level test kits and why it's so important to be tested on schedule.

Helpful links

USE and IMPORTANT SAFETY INFORMATION

What is the most important safety information I should know about 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, such as 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 or problems with your bones or joints. 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.
  • Stomach and bowel (intestinal) problems. Some people who take other medicines that contain cysteamine bitartrate may develop ulcers and bleeding in their stomach or bowel. People treated with PROCYSBI may also develop abnormal swelling and narrowing of the large bowel which must be treated promptly. Tell your doctor right away if you get abdominal pain, bloody or persistent diarrhea, bloating, nausea, vomiting, loss of appetite, vomit blood, poor weight gain or weight loss.
  • 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.
  • 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.

What is PROCYSBI?

PROCYSBI (cysteamine bitartrate) delayed-release capsules and delayed-release oral granules is a prescription medicine used to treat nephropathic cystinosis in adults and children 1 year of age and older. It is not known if PROCYSBI is safe and effective in children under 1 year of age.

Do not take PROCYSBI if you are allergic to penicillamine or cysteamine.

Before taking PROCYSBI, tell your doctor about all your medical conditions, including if you:

  • drink alcohol.
  • have a skin rash or bone problems.
  • have or have had stomach or bowel (intestinal) problems including ulcers or bleeding.
  • have a history of seizures, lack of energy, unusual sleepiness, depression, or changes in your ability to think clearly.
  • 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 the medicines you take, including prescription and over the counter medicines, vitamins, dietary 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.

What should I avoid while taking PROCYSBI?

  • Do not drive or operate machinery until you know how PROCYSBI affects you. PROCYSBI can make you sleepy or less alert than normal.
  • Do not drink alcohol if you take PROCYSBI. Drinking alcohol while taking PROCYSBI may change how PROCYSBI works and may cause an increase in the amount of PROCYSBI in your blood that may cause serious side effects.

What are the possible side effects of PROCYSBI?

PROCYSBI can cause serious side effects, including:

  • See “What is the most important information I should know about PROCYSBI?”

The most common side effects of PROCYSBI include: vomiting, nausea, stomach (abdominal) pain, pink eye, diarrhea, cold, tiredness, flu, headache, problems with body salts or electrolytes, infection of ear, nose or throat, joint pain.

These are not all the possible side effects of PROCYSBI. Call your doctor for medical information about side effects. You may report side effects to FDA at 1-800-FDA-1088.

For additional important safety information, click here for the Patient Package Insert and discuss with your doctor.

USE and IMPORTANT SAFETY INFORMATION

What is the most important safety information I should know about 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, such as stretch marks, bone injuries (such as fractures), bone deformities, and joint problems. Check your skin while taking PROCYSBI. Tell your doctor if you notice