Getting PROCYSBI

How to start treatment with PROCYSBI® (cysteamine bitartrate) delayed-release capsules

Follow these easy steps to get started on PROCYSBI.

Get ready for your visit

Be prepared for your visit with your doctor. Remember, your doctor is there to help you, so it’s important to talk openly and honestly about your health. Be ready to talk about topics like:

  • Any prescriptions, over-the-counter medications, and dietary or herbal supplements you are currently taking
  • Diet
  • Allergies to medication
  • Any experience with cysteamine, including side effects and the size of your current dose, if you are currently taking cysteamine
  • Tell your doctor if you miss a dose or do not take your immediate-release cysteamine every 6 hours, as directed

Fill out the Prescription Enrollment Form

Download and print the PROCYSBI Prescription Enrollment Form (PEF), and complete the sections that require your information. Make sure you sign the form when you are finished. Leave the “Clinical Information” section blank; your doctor will fill it out.
Download the PEF ›

Meet with your doctor

Talk with your doctor and ask if PROCYSBI is right for you. Your doctor will then complete your PEF and submit it to TranscendRare™ to be processed.
Learn more ›

Prevent delays in getting your PROCYSBI prescription

After your doctor submits your PROCYSBI Prescription Enrollment Form (PEF), you will receive a call from a TranscendRare representative to confirm some of your personal information or request additional insurance details. Accredo Specialty Pharmacy will call you shortly afterward to schedule a delivery of PROCYSBI. To prevent delays in getting your prescription, make sure you:

  • Call back a TranscendRare representative and Accredo Specialty Pharmacy as soon as you can
  • Provide any additional information needed to help process your prescription
    • TranscendRare will call you to discuss your insurance benefits and discuss your copay
    • Accredo Specialty Pharmacy will call you to schedule your first shipment and your refills
    • Accredo Specialty Pharmacy will also discuss the opportunity to enroll in the PROCYSBI text-message refill reminder program. The text program is optional
TranscendRare™ offers support services for people living with nephropathic cystinosis and their caregivers Learn more ›

How to take PROCYSBI

Dosing instructions1

PROCYSBI can be taken in several different ways. It can be swallowed, mixed with approved foods or drinks, or administered through a G-tube. You should take PROCYSBI the same way each time; for example, swallowed whole on an empty stomach or sprinkled on a similar type and amount of food (applesauce or berry jelly). Your doctor can help you choose the best way for you to take PROCYSBI.

Taking PROCYSBI with foods and juices not listed below is not recommended.

PROCYSBI works to control cystine concentration continuously over 12 hours, so patients and caregivers can choose a schedule that fits their lifestyle.

You can swallow PROCYSBI whole1

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

  • Take PROCYSBI capsules with water or fruit juice (except for grapefruit juice)

Opening PROCYSBI capsules1

  • To open a PROCYSBI capsule, using both hands, hold each capsule end between your thumb and index finger and gently twist the ends in opposite directions
  • Do not pinch the capsule in the center or crush the capsule
  • You may need to open more than one capsule, depending on the dose prescribed by your doctor

You can mix PROCYSBI with certain foods or juices1

Do not crush or chew PROCYSBI or the capsule contents. If you have difficulty swallowing you may mix PROCYSBI in a clean container with one of the options listed below:

You can mix PROCYSBI with applesauce or berry jelly1

  • Open and sprinkle capsule contents onto ½ cup (4 ounces) of applesauce or berry jelly and gently mix*
  • Swallow the applesauce or berry jelly mixture within 30 minutes. Do not save the mixture for later use

You can mix PROCYSBI with fruit juice (except grapefruit juice)1

  • Open and sprinkle capsule contents into ½ cup (4 ounces) of fruit juice and stir gently until mixed
  • Drink all the fruit juice mixture within 30 minutes. Do not save the fruit juice mixture for later use

 
 
Taking PROCYSBI with foods and juices not listed above is not recommended.1

You can take PROCYSBI through a G-tube1

Use only strained applesauce with no chunks.
It is best to use a straight (bolus) feeding tube. For people who have a gastrostomy tube (G-tube) that is size 14 French or larger, PROCYSBI may be given as follows:

  • Flush the G-tube button with 5 mL of water to clear the button
  • Open the capsule and empty the granules into a clean container with approximately 4 ounces (1/2 cup) of applesauce.
  • Use only strained applesauce with no chunks
    • A minimum of 1 ounce (1/8 cup) of applesauce may be used for children ≤ 25 kg starting PROCYSBI at a dose of 1 or 2 capsules
  • Gently mix and place the tip of a catheter-tip syringe at the bottom of the container
    • Adult: Draw up about 40 mL of the mixture and repeat until full dose is administered. If the entire half a cup (118 mL) of applesauce is used to mix with the dose, then it may take 3 syringes to administer all of the mixture
    • Child: Draw up at least 10 mL of the mixture
  • Place the tip into the feeding tube and fill the feeding tube with the mixture
  • Hold the feeding tube in a horizontal (straight across) position and give the mixture through the G-tube at a quick and steady rate of 10 mL over 10 seconds
  • Give all the mixture through the G-tube within 30 minutes of mixing. Do not save the applesauce mixture for later use
  • Draw up at least 10 mL of fruit juice (except grapefruit) into another catheter-tip syringe. Flush the G-tube using enough fruit juice so that there is no mixture left

 

*Volume of applesauce needed may depend upon patient size and medication amount. Please consult your doctor.

Talk to your doctor about the best way for you to take PROCYSBI. Always take PROCYSBI exactly as discussed.1 Be sure to talk to your doctor if you have any questions.

Download the Instructions for Use

Eating and drinking with PROCYSBI1

Foods and beverages can affect how much cysteamine your body absorbs. For this reason, it is recommended that you do not eat for 2 hours before taking PROCYSBI and for 30 minutes after taking PROCYSBI. However, it is OK to take PROCYSBI with certain foods and beverages, if needed. Do not drink alcohol if you are taking PROCYSBI.

Whether you take PROCYSBI with or without food, it is very important to take PROCYSBI with a similar type and amount of food every time. Do not eat for at least 2 hours before and for at least 30 minutes after. If you are unable to take PROCYSBI without eating, take it with food, and limit the amount of food to approximately 4 ounces (1∕2 cup) within 1 hour before taking PROCYSBI through 1 hour after taking PROCYSBI. Take PROCYSBI in a consistent manner in regard to food. Avoid eating high-fat food close to the time when you take PROCYSBI. These highlights do not include all the information needed to use taking PROCYSBI. Please see the Full Prescribing Information, Patient Package Insert, and Instructions for Use for more complete information.

Be sure to check in with your doctor as you are getting used to your new medicine. Your doctor may reduce your dose to help you manage any side effects. Foods and beverages not listed have not been studied.

Avoid eating high-fat foods close to when you take PROCYSBI1

Managing missed doses1

  • It is important to take PROCYSBI every 12 hours to maintain continuous cystine control
  • If a dose is missed, take the dose as soon as possible up to 8 hours after the scheduled time
  • If a dose is missed and the next scheduled dose is due in less than 4 hours, do not take the missed dose, and take the next dose at the usual scheduled time
  • DO NOT take 2 doses at once to make up for a missed dose

Being disciplined and consistent with your cystine-depleting therapy is the only way to prevent irreversible damage caused by elevated cystine concentrations. This is true even if you’ve had a kidney transplant and look or feel fine. Do not stop taking PROCYSBI without talking to your doctor first.2-6

What to Expect

Possible side effects1

PROCYSBI can cause serious side effects, including the following:

  • Skin, bone, and joint problems. People treated with high doses of cysteamine bitartrate may develop abnormal changes in 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 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 develop ulcers and bleeding in their stomach or bowel. Tell your doctor right away if you get stomach-area pain, nausea, vomiting, or loss of appetite, or if you vomit blood.
  • Central nervous system symptoms. Some people who take other medicines that contain cysteamine bitartrate develop seizures or depression or 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.
  • A low white blood cell count and certain abnormal liver-function blood test results. 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 in which 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.

In clinical trials, some people experienced side effects while taking PROCYSBI.

  • The most common side effects (5%) in patients 6 years of age and older who were previously treated with cysteamine were: vomiting, nausea, abdominal pain, breath odor, diarrhea, skin odor, fatigue, rash, and headache.1
  • The most common side effects (>10%) in patients 1 year to less than 6 years who never took cysteamine medicine before were: vomiting, gastroenteritis/viral gastroenteritis, diarrhea, breath odor, nausea, electrolyte imbalance, and headache.1

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. Call your doctor for medical advice about side effects.

References

1. PROCYSBI [package insert]. Lake Forest, IL: Horizon Pharma USA, Inc; 2017.

2. Brodin-Sartorius A, Tète M-J, Niaudet P, et al. Cysteamine therapy delays the progression of nephropathic cystinosis in late adolescents and adults. Kidney Int. 2012;81:179-189.

3. Gahl WA, Thoene JG, Schneider JA. Cystinosis. N Engl J Med. 2002;347(2):111-121.

4. Gahl WA, Balog JZ, Kleta R. Nephropathic cystinosis in adults: Natural history and effects of oral cysteamine therapy. Ann Intern Med. 2007;147:242-250.

5. Levtchenko EN, van Dael CM, de Graaf-Hess AC, et al. Strict cysteamine dose regimen is required to prevent nocturnal cystine accumulation in cystinosis. Pediatr Nephrol. 2006;21:110-113.

6. Nesterova G, Gahl WA. Cystinosis: The evolution of a treatable disease. Pediatr Nephrol. 2013;28:51-59.

What is PROCYSBI (Pro-CIS-bee)?

PROCYSBI® (cysteamine bitartrate) delayed-release capsules is a prescription medicine used to treat a medical condition called 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.

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?

Before you take PROCYSBI, tell your doctor 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
  • have any other medical conditions
  • 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.

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.

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:

  • 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 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) 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.

The most common side effects with PROCYSBI include vomiting, nausea, stomach (abdominal) pain, breath odor, diarrhea, skin odor, tiredness, skin rash, headache, and problems with body salts or electrolytes.

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. Call your doctor for medical advice about side effects.

You may report side effects to the FDA at 1-800-FDA-1088.

Issued: 12/2017                    Piece number: P-PYB-00032                    Derived from Patient Information approved 12/2017