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PROCYSBI Dosing and Administration

Dosage Guide Administration Options

Start PROCYSBI immediately after diagnosis of nephropathic cystinosis1

PROCYSBI® (cysteamine bitartrate) delayed-release capsules and delayed-release oral granules should be initiated immediately following diagnosis of nephropathic cystinosis in patients aged ≥1 year.1 Early cystine-depleting therapy (CDT) treatment can delay or limit damage to the patient’s tissues and organs caused by rising cystine levels, but treatment cannot reverse damage that has already occurred.2-4

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To learn more about PROCYSBI, please see the Prescribing Information.

 

Determine dose by patient experience with cysteamine bitartrate1

Patients switching from immediate-release (IR) cysteamine bitartrate should start PROCYSBI by taking a total daily dose equal to their previous total daily dose.1
 

For cysteamine-naïve patients, PROCYSBI should be initiated with a dosage equal to one-sixth to one-fourth of the maintenance dose. The maintenance dosage after initial dose escalation is 1.3 g/m2 of body surface area (BSA) per day divided into 2 doses given every 12 hours.1
 

An Amgen Representative can provide more information on calculating dosage for weight above 51 kg (112 lb).

Starting and maintenance dosage of PROCYSBI by body weight for cysteamine-naïve patients1,*†
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*Higher dosages may be required to achieve target therapeutic WBC cystine concentration. The maximum dosage of PROCYSBI is 1.95 grams/m2 of body surface area per day.

†Dosage rounded using available strengths of capsules or packets of oral granules.

If you have questions or would like to learn more about appropriate dosing and administration, you may connect with a representative.

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Dosing Flashcard

For dosing calculations by patient weight, download the dosing flashcard.

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Starting low and slowly increasing the dose may reduce the risk of some adverse events1

  • Patients aged 1 year to <6 years: Increase the dosage in 10% increments to the maintenance dosage while monitoring WBC cystine concentrations. Allow a minimum of 2 weeks between dosage adjustments.
     
  • Patients aged ≥6 years: Gradually increase the dosage over 4 to 6 weeks until the maintenance dosage is achieved.

Administration Options

PROCYSBI offers flexibility in administration, with 3 different ways your patients can take it.1

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1 - Swallowed whole

 

PROCYSBI capsules can be swallowed whole with fruit juice (except grapefruit juice) or water. Do not crush or chew the capsules.1

Food---Liquids

2 - Opened and mixed with select foods or liquids

 

For patients who cannot tolerate PROCYSBI on an empty stomach or have difficulty swallowing, capsules or packets should be opened and the microbeads mixed with select foods or liquids. The microbeads may be mixed in about ½ cup (4 oz.) of applesauce, berry jelly, or fruit juice (except grapefruit juice). The mixture should be consumed within 30 minutes of mixing and should not be saved for later use.1

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3 - Through a G-tube

 

For individuals with a G-tube that is size 14 French or larger, PROCYSBI capsules or packets should be opened and the microbeads mixed with about ½ cup (4 oz.) of strained applesauce with no chunks. The mixture should be administered within 30 minutes of mixing and should be not saved for later use.1

Stomach acidity affects the release and absorption of PROCYSBI

To maintain the right stomach acid levels for continuous cystine control, patients should1:

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Stomach acidity affects the release and absorption of PROCYSBI

To maintain the right stomach acid levels for continuous cystine control, patients should1:

Take PROCYSBI at least 1 hour before or after they take medicines that contain bicarbonate or carbonate.

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Not eat for at least 2 hours before and 30 minutes after taking PROCYSBI.

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Take PROCYSBI with no more than ½ cup (4 oz) of food up to 1 hour before or after they take it, if they cannot tolerate PROCYSBI on an empty stomach.

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References

1. PROCYSBI (cysteamine bitartrate) delayed-release capsules and delayed-release oral granules [prescribing information] Amgen. 2. Nesterova G, Gahl WA. Cystinosis: the evolution of a treatable disease. Pediatr Nephrol. 2013;28(1):51-59. 3. Gahl WA, Balog JZ, Kleta R. Nephropathic cystinosis in adults: natural history and effects of oral cysteamine therapy. Ann Intern Med. 2007;147(4):242-250. 4. Elmonem MA, Veys KR, Soliman NA, van Dyck M, van den Heuvel LP, Levtchenko E. Cystinosis: a review. Orphanet J Rare Dis. 2016;11(47):1-17. 5. Mosteller RD. Simplified calculation of body-surface area. N Engl J Med. 1987;317(17):1098.

INDICATION and IMPORTANT SAFETY INFORMATION

INDICATION

PROCYSBI (cysteamine bitartrate) delayed-release capsules and delayed-release oral granules is a cystine-depleting agent indicated for the treatment of nephropathic cystinosis in adults and pediatric patients 1 year of age and older.

IMPORTANT SAFETY INFORMATION

CONTRAINDICATIONS

  • Patients with serious hypersensitivity reaction, including anaphylaxis to penicillamine or cysteamine.

WARNINGS AND PRECAUTIONS

  • Ehlers-Danlos-like Syndrome: Skin and bone lesions that resemble clinical findings for Ehlers-Danlos-like syndrome have been reported in patients treated with high doses of immediate-release cysteamine bitartrate or other cysteamine salts. Monitor patients for development of skin or bone lesions and reduce PROCYSBI dosing if patients develop these lesions.
  • Skin Rash: Severe skin rashes such as erythema multiforme bullosa or toxic epidermal necrolysis have been reported in patients receiving immediate-release cysteamine bitartrate. Discontinue use if severe skin rash occurs.
  • Gastrointestinal (GI) Ulcers and Bleeding: GI ulceration and bleeding have been reported in patients receiving immediate-release cysteamine bitartrate. Monitor for GI symptoms and consider decreasing the dose if severe symptoms occur.
  • Fibrosing Colonopathy: Fibrosing colonopathy has been reported with postmarketing use of PROCYSBI. Evaluate patients with severe, persistent, and/or worsening abdominal symptoms for fibrosing colonopathy. If the diagnosis is confirmed, permanently discontinue PROCYSBI and switch to immediate-release cysteamine bitartrate capsules.
  • Central Nervous System (CNS) Symptoms: CNS symptoms such as seizures, lethargy, somnolence, depression, and encephalopathy have been associated with immediate-release cysteamine. Monitor for CNS symptoms; interrupt or reduce the dose for severe symptoms or those that persist or progress.
  • Leukopenia and/or Elevated Alkaline Phosphatase Levels: Cysteamine has been associated with reversible leukopenia and elevated alkaline phosphatase levels. Monitor white blood cell counts and alkaline phosphatase levels; decrease or discontinue the dose until values revert to normal.
  • Benign lntracranial Hypertension: Benign intracranial hypertension (pseudotumor cerebri; PTC) and/or papilledema has been reported in patients receiving immediate-release cysteamine bitartrate treatment. Monitor for signs and symptoms of PTC; interrupt or reduce the dose for signs/symptoms that persist, or discontinue if diagnosis is confirmed.

ADVERSE REACTIONS

The most common adverse reactions reported in PROCYSBI clinical trials (≥ 5%): were:

  • Patients 2 years of age and older previously treated with cysteamine: vomiting, nausea, abdominal pain, headache, conjunctivitis, influenza, gastroenteritis, nasopharyngitis, dehydration, ear infection, upper respiratory tract infection, fatigue, arthralgia, cough, and pain in extremity.
  • Patients 1 year of age and older naïve to cysteamine treatment: vomiting, gastroenteritis/viral gastroenteritis, diarrhea, breath odor, nausea, electrolyte imbalance, headache.

DRUG INTERACTIONS

  • Drugs that increase gastric pH may alter the pharmacokinetics of cysteamine due to the premature release of cysteamine from PROCYSBI and increase WBC cystine concentration. Monitor WBC cystine concentration with concomitant use.
  • Consumption of alcohol with PROCYSBI may increase the rate of cysteamine release and/or adversely alter the pharmacokinetic properties, as well as the effectiveness and safety of PROCYSBI.
  • PROCYSBI can be administered with electrolyte (except bicarbonate) and mineral replacements necessary for management of Fanconi Syndrome as well as vitamin D and thyroid hormone.

USE IN SPECIFIC POPULATIONS

  • Lactation: Because of the potential risk for serious adverse reactions in breastfed children from cysteamine, breastfeeding is not recommended during treatment with PROCYSBI.

Please see Full Prescribing Information.

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