Cysteamine Bitartrate
Drug class: Antineoplastic Agents
Usage of Cysteamine Bitartrate
Cystinosis
Treatment of nephropathic cystinosis (designated an orphan drug by FDA for this use).
Immediate-release cysteamine bitartrate capsules (Cystagon) are indicated for use in children and adults. Delayed-release capsules and oral granules (Procysbi) are indicated for use in adults and pediatric patients ≥1 year of age.
Early and long-term oral cysteamine therapy has been shown to preserve renal glomerular function and reduce or prevent extra-renal complications such as growth retardation and hypothyroidism.
Cysteamine therapy should be considered for all patients with nephropathic cystinosis regardless of age and transplantation status, and such therapy should be continued lifelong.
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How to use Cysteamine Bitartrate
General
Patient Monitoring
Administration
Oral Administration
Cysteamine bitartrate is administered orally. The drug is commercially available as immediate-release capsules or delayed-release capsules or oral granules.
Immediate-release CysteamineImmediate-release capsules (Cystagon) are administered every 6 hours; the capsules may be swallowed whole or the entire contents of the capsule(s) may be sprinkled on food. The manufacturer recommends that intact cysteamine capsules should not be administered to children under the age of approximately 6 years because of the risk of aspiration; in these patients, the capsules may be administered by sprinkling the capsule contents over food.
Delayed-release CysteamineDelayed-release cysteamine bitartrate capsules (Procysbi) are administered every 12 hours. The capsules should be swallowed whole with fruit juice (except grapefruit juice) or water; do not crush or chew the capsules or contents. For patients who cannot swallow the capsules, the capsules can be opened and the capsule contents sprinkled on and mixed in applesauce, berry jelly, or fruit juice (except grapefruit juice) and administered orally. For patients with a gastrostomy tube, the capsules can be opened and the capsule contents mixed in applesauce and administered.
Cysteamine bitartrate delayed-release oral granules should be sprinkled and mixed in applesauce, berry jelly, or fruit juice (except grapefruit juice) and administered orally; do not crush or chew the oral granules. For patients with a gastrostomy tube, the oral granules can be mixed in applesauce and administered.
Do not eat for at least 2 hours before taking delayed-release cysteamine and for at least 30 minutes after to maximize absorption. If patients are unable to take the drug without eating, take with food but limit the amount to approximately 4 ounces (½ cup) within 1 hour before to 1 hour after taking cysteamine. Administer the drug in a consistent manner with regard to food and avoid consumption of high fat foods close to dosing. Administer cysteamine at least 1 hour before or 1 hour after drugs containing bicarbonate or carbonate, and avoid alcohol while taking cysteamine.
Dosage
Available as cysteamine bitartrate; dosage is expressed in terms of cysteamine.
Initiate cysteamine therapy promptly once the diagnosis of nephropathic cystinosis is confirmed.
Pediatric Patients
Nephropathic Cystinosis (Immediate-release Cysteamine Capsules) OralInitial dosage in treatment-naive patients should be 1/6–¼ of the maintenance dosage; increase dosage gradually to minimize adverse effects. The maintenance dosage should be reached after 4 to 6 weeks of incremental dosage increases.
Patients who are currently taking cysteamine hydrochloride or phosphocysteamine may be transitioned to equimolar doses of immediate-release cysteamine bitartrate.
If patient is unable to tolerate cysteamine initially due to adverse effects, temporarily withhold therapy, then re-institute at a lower dosage and gradually increase to appropriate dosage.
The recommended maintenance dosage of immediate-release cysteamine in treatment-naive pediatric patients ≤12 years of age is 1.3 g/m2 daily administered in 4 divided doses given every 6 hours. (See Table 1.)
Table 1. Immediate-release Cysteamine Maintenance Dosage of 1.3 g/m2 Daily1Weight in Pounds
Dosage of Cysteamine Every 6 hours (mg)
0–10
100
11–20
150
21–30
200
31–40
250
41–50
300
51–70
350
71–90
400
91–110
450
over 110
500
The recommended maintenance dosage of immediate-release cysteamine in treatment-naive pediatric patients >12 years of age weighing >110 pounds is 2 g daily administered in 4 divided doses.
After maintenance dosage is achieved, obtain leukocyte cystine measurements 5 to 6 hours after dose administration in treatment-naive patients. Patients being transferred from cysteamine hydrochloride or phosphocysteamine to immediate-release cysteamine bitartrate should have their WBC cystine levels measured in 2 weeks, and every 3 months thereafter to assess optimal dosage.
Titrate dosage to achieve WBC cystine goal <1 nmol ½ cystine/mg protein. Determine WBC cystine concentration and increase dosage if WBC cystine >2 nmol ½ cystine/mg protein. May increase dosage to a maximum of 1.95 g/m2 daily to achieve this level; however, some patients may not tolerate this dosage.
If a dose is missed, take the dose as soon as possible, unless it is within 2 hours of the next dose in which case the dose should be skipped and the regular dosing schedule resumed. Do not double a dose to make up for a missed dose.
Nephropathic Cystinosis (Delayed-release Cysteamine Capsules or Oral Granules) OralTreatment-naive pediatric patients ≥1 year of age: recommended initial dosage should be 1/6–¼ of the maintenance dosage; increase dosage gradually to a maintenance dosage of 1.3 g/m2 daily administered in 2 divided doses every 12 hours. (See Table 2.)
In patients 1 year to <6 years of age, increase dosage in 10% increments to maintenance dosage while monitoring WBC cystine concentrations; allow a minimum of 2 weeks between dosage adjustments.
In patients ≥6 years of age, gradually increase dosage over 4–6 weeks until maintenance dosage is achieved.
If patient is unable to tolerate cysteamine initially because of adverse effects, decrease dosage and then gradually increase back to the maintenance dosage.
Table 2. Delayed-release Cysteamine Maintenance Dosage of 1.3 g/m2 Daily.2Weight in kilograms
Dosage of Cysteamine Delayed-Release Capsules Every 12 hours (mg)
≤5
200
6–10
300
11–15
400
16–20
500
21–25
600
26–30
700
31–40
800
41–50
900
≥51
1000
Determine WBC cystine concentrations and adjust dosage accordingly; consider other factors such as medication adherence, dosing interval, timing between the last dose and blood draw for laboratory measurement, and other administration factors before adjusting the dosage. In treatment-naive patients 1 year to less than 6 years of age, obtain a measurement 2 weeks after initiation of cysteamine treatment and continue monitoring during the dosage titration period until the therapeutic target WBC cystine concentration is achieved. Once this is achieved, continue monitoring monthly for 3 months, then quarterly for 1 year, and then twice-yearly, at a minimum. In treatment-naive patients ≥6 years of age, obtain a measurement after reaching the maintenance cysteamine dosage, then monitor monthly for 3 months, quarterly for 1 year, and then twice-yearly, at a minimum.
After maintenance dosage is achieved, further titrate dosage to achieve a WBC cystine <1 nmol ½ cystine/mg protein. If a dosage adjustment is required, increase dosage by 10%, rounded to nearest dosage that can be administered using the available strengths of capsules or packets of oral granules, up to a maximum dosage of 1.95 g/m2 daily. For patients 1 year to less than 6 years of age, allow a minimum of 2 weeks between dosage increments.
If a dose is missed, take the dose as soon as possible up to 8 hours after the scheduled time. If the next scheduled dose is due in less than 4 hours, skip the missed dose and take the next dose at the usual scheduled time. Do not take a double dose to make up for a missed dose.
Adults
Nephropathic Cystinosis (Immediate-release Cysteamine Capsules) OralInitial dosage in treatment-naive patients should be 1/6–¼ of the maintenance dosage; increase dosage gradually to minimize adverse effects. The maintenance dosage should be reached after 4 to 6 weeks of incremental dosage increases.
Patients who are currently taking cysteamine hydrochloride or phosphocysteamine may be transitioned to equimolar doses of immediate-release cysteamine bitartrate.
If patient is unable to tolerate cysteamine initially due to adverse effects, temporarily withhold therapy, then re-institute at a lower dosage and gradually increase to appropriate dosage.
The recommended maintenance dosage of immediate-release cysteamine in treatment-naive adults weighing >110 pounds is 2 g daily administered in 4 divided doses.
After maintenance dosage is achieved, obtain leukocyte cystine measurements 5 to 6 hours after dose administration in treatment-naive patients. Patients being transferred from cysteamine hydrochloride or phosphocysteamine to immediate-release cysteamine bitartrate should have their WBC cystine levels measured in 2 weeks, and every 3 months thereafter to assess optimal dosage.
Titrate dosage to achieve WBC cystine goal <1 nmol ½ cystine/mg protein. Determine WBC cystine concentration and increase dosage if WBC cystine >2 nmol ½ cystine/mg protein. May increase dosage to a maximum of 1.95 g/m2 daily to achieve this level; however, some patients may not tolerate this dosage.
If a dose is missed, take the dose as soon as possible, unless it is within 2 hours of the next dose in which case the dose should be skipped and the regular dosing schedule resumed. Do not double a dose to make up for a missed dose.
Nephropathic Cystinosis (Delayed-release Cysteamine Capsules or Oral Granules) OralTreatment-naive patients: recommended initial dosage should be 1/6–¼ of the maintenance dosage; increase dosage gradually to a maintenance dosage of 1.3 g/m2 daily administered in 2 divided doses every 12 hours. (See Table 2.)
Gradually increase dosage over 4–6 weeks until maintenance dosage is achieved.
If patient is unable to tolerate cysteamine initially because of adverse effects, decrease dosage and then gradually increase back to the maintenance dosage.
Determine WBC cystine concentrations and adjust dosage accordingly; consider other factors such as medication adherence, dosing interval, timing between the last dose and blood draw for laboratory measurement, and other administration factors before adjusting the dosage. In treatment-naive patients, obtain a measurement after reaching the maintenance cysteamine dosage, then monitor monthly for 3 months, quarterly for 1 year, and then twice-yearly, at a minimum.
After maintenance dosage is achieved, further titrate dosage to achieve a WBC cystine <1 nmol ½ cystine/mg protein. If a dosage adjustment is required, increase dosage by 10%, rounded to nearest dosage that can be administered using the available strengths of capsules or packets of oral granules, up to a maximum dosage of 1.95 g/m2 daily.
If a dose is missed, take the dose as soon as possible up to 8 hours after the scheduled time. If the next scheduled dose is due in less than 4 hours, skip the missed dose and take the next dose at the usual scheduled time. Do not take a double dose to make up for a missed dose.
Prescribing Limits
Pediatric Patients
Nephropathic Cystinosis Oral1.95 g/m2 daily.
Adults
Nephropathic Cystinosis Oral1.95 g/m2 daily.
Special Populations
Hepatic Impairment
No special population dosage recommendations at this time.
Renal Impairment
No special population dosage recommendations at this time.
Geriatric Patients
No special population dosage recommendations at this time.
Warnings
Contraindications
Warnings/Precautions
General Precautions
Ehlers-Danlos-like SyndromeSkin and bone lesions that resemble Ehlers-Danlos-like syndrome reported in patients taking high doses of cysteamine. Clinical findings included molluscoid pseudotumors (purplish hemorrhagic lesions), skin striae, bone lesions (e.g., osteopenia, compression fractures, scoliosis, and genu valgum), leg pain, and joint hyperextension.
Monitor for skin and bone abnormalities: if any abnormalities occur, reduce dosage of cysteamine.
Dermatologic ReactionsSevere skin reactions such as erythema multiforme bullosa and toxic epidermal necrolysis reported.
Routinely monitor the skin and bones of patients. If skin rash develops, discontinue cysteamine until rash resolves; may reinitiate drug at a lower dosage under close supervision and slowly titrate to the therapeutic dosage. Permanently discontinue therapy in patients who develop severe skin rash (e.g., erythema multiforme bullosa and toxic epidermal necrolysis).
GI Ulcers and BleedingGI symptoms (e.g., nausea, vomiting, diarrhea) are common adverse effects of cysteamine. GI bleeding and ulcers also reported.
Consider dosage reduction if severe GI symptoms occur.
Fibrosing ColonopathyFibrosing colonopathy, including colonic stricture formation, reported during postmarketing experience with delayed-release cysteamine bitartrate (Procysbi). Reported symptoms includes abdominal pain, vomiting, bloody or persistent diarrhea, and fecal incontinence.
Evaluate patients with severe, persistent, and/or worsening abdominal symptoms for fibrosing colonopathy. If a diagnosis is confirmed, permanently discontinue delayed-release cysteamine and switch to immediate-release capsules. An association between methacrylic acid-ethyl acrylate copolymer (an inactive ingredient in delayed-release cysteamine) and fibrosing colonopathy cannot be ruled out.
CNS EffectsCNS symptoms (e.g., seizures, lethargy, somnolence, depression, encephalopathy) reported. Neurological complications have also been described in some patients with cystinosis not receiving cysteamine therapy.
Monitor patients for CNS symptoms; reduce dosage if severe or persistent CNS symptoms occur.
Patients should not drive, operate machinery, or engage in other dangerous activities until they know how the drug will affect them.
LeukopeniaReversible leukopenia and elevated alkaline phosphatase levels reported occasionally; monitor blood counts during cysteamine therapy.
Monitor WBC counts and alkaline phosphatase levels. If test values remain elevated, consider decreasing the dosage or discontinuing the drug until values return to normal.
Benign Intracranial HypertensionBenign intracranial hypertension (or pseudotumor cerebri [PTC]) and/or papilledema reported. A causal relationship to the drug has not been established.
Monitor for symptoms of PTC including headache, tinnitus, dizziness, nausea, diplopia, blurry vision, loss of vision, pain behind the eye, or pain with eye movement. Perform periodic eye examinations; initiate treatment promptly to prevent vision loss in patients who develop PTC.
Specific Populations
PregnancyNo available data on cysteamine use in pregnant women to inform any drug-associated risks for birth defects or miscarriage. Cysteamine (administered as cysteamine bitartrate) was teratogenic and fetotoxic in rats at doses less than the recommended human maintenance dose.
LactationNot known whether oral cysteamine is distributed into human milk or whether the drug has any effects on the breast-fed infant or on milk production. Breastfeeding is not recommended.
Pediatric UseSafety and efficacy established in pediatric patients; the drug is approved for use in such patients.
Safety and effectiveness of delayed-release cysteamine not established in patients <1 year of age.
Geriatric UseNo studies conducted in geriatric patients.
Common Adverse Effects
Common adverse effects with immediate-release cysteamine (>5%): vomiting, anorexia, fever, diarrhea, lethargy, rash.
Common adverse effects with delayed-release cysteamine in previously-treated patients ≥6 years of age (≥5%): vomiting, nausea, abdominal pain, breath odor, diarrhea, skin odor, fatigue, rash, headache.
Common adverse effects with delayed-release cysteamine in treatment-naive patients 1 year to <6 years of age (>10%): vomiting, gastroenteritis/viral gastroenteritis, diarrhea, breath odor, nausea, electrolyte imbalance, headache.
What other drugs will affect Cysteamine Bitartrate
Does not inhibit CYP1A2, 2A6, 2B6, 2C8, 2C9, 2C19, 2D6, 2E1, or 3A4.
Drugs that Increase Gastric pH
Drugs that increase gastric pH (e.g., medications containing bicarbonate or carbonate) may alter pharmacokinetics of cysteamine due to the premature release of cysteamine from the delayed-release preparations and increase WBC cystine concentration.
Monitor WBC cystine concentration when drugs that increase the gastric pH are used concomitantly with delayed-release cysteamine.
Alcohol
Consumption of alcohol with delayed-release cysteamine may increase the rate of cysteamine release and/or adversely alter the pharmacokinetics, effectiveness, and safety of cysteamine.
Avoid consumption of alcoholic beverages during treatment with cysteamine.
Other Medications Used in Fanconi Syndrome
Cysteamine may be administered with electrolyte and mineral replacements necessary for management of Fanconi Syndrome, vitamin D, and thyroid agents.
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