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

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Taking PROCYSBI

DESIGNED TO DELIVER CONTINUOUS CONTROL

For PROCYSBI® to continuously control cystine levels, it must be taken 2 times a day—once every 12 hours. For example, a person might take PROCYSBI at 7:00 a.m. and again at 7:00 p.m. every day. This dosing allows people with cystinosis to fit PROCYSBI into their daily routines, even with school and work.

PROCYSBI Prescribing Information Icon

With PROCYSBI, you can create an every-12-hour dosing schedule that may fit into your daily routine.

See the role of cystine level testing.

One medical study showed that people who took their doses of cysteamine therapy on time had lower average cystine levels compared with people who missed a dose or took their medicine late.

PROCYSBI can be taken by mouth and by G-tube.

PROCYSBI for cystinosis

Talking to your doctor about PROCYSBI for cystinosis

It’s important to understand your treatment for cystinosis. If you have questions about your treatment, use this informational download to start a conversation with your provider.

Download the Resource
PROCYSBI Label

How to take PROCYSBI by mouth

PROCYSBI® (cysteamine bitartrate) delayed-release capsules are available in 2 strengths: 25 mg and 75 mg. PROCYSBI delayed-release oral granules are available in 2 strengths: 75 mg and 300 mg. Capsules may be swallowed whole with select liquids, or opened and the microbeads mixed with select foods or liquids. Packets should be opened and the microbeads mixed with select foods or liquids.

Important notes about taking PROCYSBI delayed-release capsules and delayed-release oral granules

  • Take PROCYSBI the same way each time
  • Do not eat for at least 2 hours before taking PROCYSBI and at least 30 minutes after you take it
  • Take PROCYSBI at least 1 hour before or after you take medicines that contain bicarbonate or carbonate
  • 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
PROCYSBI Package Insert Step 1
Whole capsules

STEPS REQUIRED

  1. Swallow with water or fruit juice (except grapefruit juice). Do not crush or chew the capsules.
PROCYSBI Package Insert Step 2
Mix microbeads with select foods

STEPS REQUIRED

  1. Open the capsule(s) or packet(s). See instructions below.
  2. Gently mix the contents with about ½ cup (4 oz.) of applesauce or berry jelly. Do not crush or chew the microbeads. Do not mix with any food other than applesauce or berry jelly.
  3. Eat the applesauce or berry jelly mixture within 30 minutes of mixing. Do not save the applesauce or berry jelly mixture for later use.
PROCYSBI Package Insert Step 2
Mix microbeads with select liquids

STEPS REQUIRED

  1. Open the capsule(s) or packet(s). See instructions below.
  2. Gently mix the contents with about ½ cup (4 oz.) of any fruit juice (except grapefruit juice). Do not crush or chew the microbeads.
  3. Drink all of the fruit juice mixture within 30 minutes of mixing. Do not save the fruit juice mixture for later use.

How to open PROCYSBI capsules:

  • Use dry hands to hold each end of the capsule
  • Using one hand on each end, gently twist the 2 ends in opposite directions to open
  • Do not pinch the capsule in the center
  • Do not crush or chew the capsule

 
To open PROCYSBI packets:

  • Tear the packet open along the perforated line

How to take PROCYSBI via G-tube

  • Read video transcript

    You or your loved one may have decided with your doctor to take PROCYSBI, cysteamine bitartrate, delayed-release capsules and delayed-release oral granules through a gastrostomy tube, or G-tube. This video will walk you through the process from preparing PROCYSBI for administration to giving the medicine through a G-tube.

    If you have any questions about PROCYSBI or taking PROCYSBI through a G-tube, contact your doctor or your patient access liaison, your PAL. You should also look at the instructions for use, which are included when your PROCYSBI is sent to you and can also be found at PROCYSBI.com. Let's get started.

    Before you gather your materials and PROCYSBI capsules or packets, always wash your hands to reduce the spread of germs. Now, you're ready to gather your materials. Here's what you'll need: catheter-tips syringes and a feeding tube for a G-tube that is size 14 French or larger. A straight or bolus feeding tube is best. Strained applesauce, do not use chunky applesauce. A clean bowl and measuring cup, water, any fruit juice except grapefruit juice, and the prescribed dose of PROCYSBI.

    Now you're ready to start the preparation process. Unclasp the G-tube button and attach the feeding tube. Flush with five milliliters of water to clear the button. Measure the strained applesauce and put it in a clean bowl. Make sure your hands are dry before opening PROCYSBI capsules. To open a capsule, grab each end and twist slowly. Be careful not to pinch the capsule in the center or crush it. Sprinkle the microbeads on top of the applesauce. To open a packet, tear open the packet along the perforated line, sprinkle the microbeads on top of the applesauce.

    Use a syringe to gently stir the PROCYSBI microbeads into the applesauce. Draw the mixture up into the syringe. Drop at least 10 milliliters for children, and about 40 milliliters for adults. Next, connect the syringe to the feeding tube. Fill the feeding tube with a mixture. Hold the syringe and feeding tube in a horizontal position straight across, to administer the mixture. Give the mixture at a quick and steady pace. Repeat as needed to give all of the mixture.

    All of the mixture should be given within 30 minutes of mixing. None of the mixture should be saved. To be sure all of the mixture has been given, pour some liquid into the bowl and swirl it around to get the remaining applesauce. You can use water or juice, but do not use grapefruit juice. Draw the liquid up into another syringe, gently swirl the syringe and flush the G-tube to be sure that none of the applesauce and microbead mixture remains.

    You're finished. Although there are a number of steps in the process, it becomes more natural over time. Be sure to clean all your equipment for your next use. Use this video to go through the steps whenever you need to. If you have any questions about giving PROCYSBI through a G-tube, see the PROCYSBI instructions for use or contact your doctor or a PAL at Amgen. Support is always available.

    USE and IMPORTANT SAFETY INFORMATION
    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 one year of age and older. It is not known if PROCYSBI is safe and effective in children under one year of age.

    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. 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. Tell your doctor right away if you get a skin rash.
    • 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.
    • 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.
    Who should not take PROCYSBI?

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

    What should I tell my doctor before taking PROCYSBI?

    Tell your doctor if you have any other 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.
    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?

    • 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 of the possible side effects of PROCYSBI. Call your doctor for medical information about side effects.

    You are encouraged to report all the possible side effects of prescription drugs to the FDA. Visit http://www.fda.gov/medwatch to call 1-800-FDA-1088.

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

For individuals with a gastrostomy tube (G-tube) that is size 14 French or larger, PROCYSBI® (cysteamine bitartrate) delayed-release capsules or delayed-release oral granules packets may be opened and their contents mixed with strained applesauce with no chunks.

STEPS REQUIRED
  1. Flush the G-tube button with 5 mL of water to clear the button
  2. Open the PROCYSBI capsule(s) or packet(s). See instructions above
  3. In a clean container, sprinkle the contents of the PROCYSBI capsule(s) or packet(s) on about ½ cup (4 oz.) of strained applesauce with no chunks, and mix gently*
  4. Place the tip of a catheter tip syringe at the bottom of the container of the mixture. For an adult dose, draw up about 40 mL of the mixture. When giving to a child, draw up at least 10 mL of the mixture
  5. Place the tip of the catheter tip syringe into a feeding tube, and fill it with the mixture
  6. Holding the feeding tube in a horizontal position, push the mixture through the G-tube at a quick and steady rate of 10 mL over 10 seconds. Repeat until all the mixture is given
  7. Be sure to push all of the mixture through the G-tube within 30 minutes of mixing. Do not save the mixture for later use
  8. Draw up at least 10 mL of fruit juice (except grapefruit juice) or water into another catheter tip syringe. Gently swirl the syringe. Flush the G-tube with the fruit juice or water until there is no mixture left in the G-tube

* A minimum of 1/8 cup (1 oz.) of applesauce may be used for children who are 55 lbs (25 kg) or less and starting PROCYSBI at a dose of 1 or 2 capsules.

For best results, administer the applesauce and microbeads mixture through a G-tube that is size 14 French or larger and use a straight (bolus) feeding tube.

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

or delayed-release oral granules packets

What to do if you miss a dose

If you realize you missed a dose of PROCYSBI... Then...
Within 8 hours of when you were scheduled to take it Take it as soon as possible
When your next dose is due in less than 4 hours Take the next dose at your regularly scheduled time

Do not take 2 doses at one time to make up for a missed dose.

What you eat and drink can affect acid levels. If acid levels are too low, it can result in the medicine releasing too soon (in the stomach). Talk to your doctor about how to take PROCYSBI with highly acidic foods to ensure that it doesn’t dissolve in the stomach.

To maintain acid levels, you should:

  • Take PROCYSBI at least 1 hour before or 1 hour after you take medicines that contain bicarbonate or carbonate
  • If you can’t take PROCYSBI without eating, take it with no more than 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

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.

taking-mobile
Cysteamine capsules with 12 hour dosing schedule image

How to fill a prescription for PROCYSBI

PROCYSBI is only available through a specialty pharmacy. Your PROCYSBI prescription is filled and sent to your home through a package delivery service. Before you start taking PROCYSBI, your doctor will submit a form to enroll you in Amgen By Your Side, a patient support program. An Amgen By Your Side representative will help you connect with the specialty pharmacy.

To get started with Amgen By Your Side, call 1-855-888-4004.

Talk to your doctor about a PROCYSBI prescription. The Patient Enrollment Form found here is required, and your doctor will assist you in filling it out.

Amgen By Your Side offers access and reimbursement support for eligible patients

Storing PROCYSBI

Keep PROCYSBI and all medicines out of the reach of children.

Capsules

  • Keep capsules tightly closed in the original bottle
  • Store at room temperature, 68°F-77°F (20°C-25°C) in a dry place away from light
  • To keep the medicine dry, capsules are bottled with a small canister that absorbs moisture. Keep the canister in the bottle with the capsules. Do not eat or throw it away

Packets

  • Keep packets closed in the original box
  • Store at room temperature, 68°F-77°F (20°C-25°C) in a dry place away from light

Disposing of unused or expired PROCYSBI

  • Discard opened packets after taking your dose. Do not store or save opened packets of PROCYSBI for your next dose
  • PROCYSBI may not work as intended after the discard date written by your pharmacist. Dispose of any unused PROCYSBI after the expiration date on the bottle or box
  • Ask your pharmacist or contact the Drug Enforcement Administration at deadiversion.usdoj.gov/drug_disposal/index.html or 1-800-882-9539 to find out how to properly dispose of PROCYSBI in your area

Possible side effects of PROCYSBI

Side effects may be dose-related. The most common side effects with PROCYSBI include:

  • Vomiting
  • Stomach (abdominal) pain
  • Diarrhea
  • Tiredness
  • Headache
  • Nausea
  • Breath odor
  • Skin odor
  • Skin rash
  • Problems with electrolytes (body salts)

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