Cysteamine (oral)

Cysteamine (oral)
Adult Indications & Dosage
Pediatric Indications & Dosage
Contraindications
Warnings & Precautions
Adverse Reactions
Drug Interactions
Use in Specific Populations
Administration & Monitoring
Overdosage
Pharmacology
Clinical Studies
How Supplied
Images
Patient Counseling Information
Precautions with Alcohol
Brand Names
Look-Alike Names

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];

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Overview

Cysteamine (oral) is a renal-urologic agent that is FDA approved for the treatment of nephropathic cystinosis in children and adults.. Common adverse reactions include Finding of odor of skin, rash, abdominal pain, breath smells unpleasant, diarrhea, loss of appetite, nausea, vomiting, dizziness, headache, lethargy and fever.

Adult Indications and Dosage

FDA-Labeled Indications and Dosage (Adult)

Indication

CYSTAGON® is indicated for the management of nephropathic cystinosis in children and adults.

Dosage

New patients should be started on ¼ to 1/6 of the maintenance dose of CYSTAGON®. The dose should then be raised gradually over four to six weeks to avoid intolerance.

The recommended CYSTAGON® maintenance dose for children up to age 12 years is 1.30 grams/m2/day of the free base, given in four divided doses. Intact CYSTAGON® capsules should not be administered to children under the age of approximately six years due to the risk of aspiration. CYSTAGON® capsules may be administered to children under the age of approximately six years by sprinkling the capsule contents over food. Patients over age 12 and over 110 pounds weight should receive 2.0 grams/day, divided four times daily.

If a dose is missed, it should be taken as soon as possible. If it is within two hours of the next dose, skip the missed dose and go back to the regular dosing schedule. Do not double dose.

When CYSTAGON® is well tolerated, the goal of therapy is to keep leukocyte cystine levels below 1 nmol/½ cystine/mg protein five to six hours following administration of CYSTAGON®. Patients with poorer tolerability still receive significant benefit if white cell cystine levels are below 2 nmol/½ cystine/mg protein. The CYSTAGON® dose can be increased to a maximum of 1.95 grams/m2/day to achieve this level. The dose of 1.95 grams/m2/day has been associated with an increased rate of withdrawal from treatment due to intolerance and an increased incidence of adverse events.

Cystinotic patients taking cysteamine hydrochloride or phosphocysteamine solutions may be transferred to equimolar doses of CYSTAGON® capsules.

The recommended maintenance dose of 1.30 grams/m2/day can be approximated by administering CYSTAGON® according to the following table, which takes surface area as well as weight into consideration.

This image is provided by the National Library of Medicine.

Off-Label Use and Dosage (Adult)

Guideline-Supported Use

There is limited information regarding Off-Label Guideline-Supported Use of Cysteamine in adult patients.

Non–Guideline-Supported Use

There is limited information regarding Off-Label Non–Guideline-Supported Use of Cysteamine in adult patients.

Pediatric Indications and Dosage

FDA-Labeled Indications and Dosage (Pediatric)

Indication

CYSTAGON® is indicated for the management of nephropathic cystinosis in children.

Dosage

The recommended CYSTAGON® maintenance dose for children up to age 12 years is 1.30 grams/m2/day of the free base, given in four divided doses. Intact CYSTAGON® capsules should not be administered to children under the age of approximately six years due to the risk of aspiration. CYSTAGON® capsules may be administered to children under the age of approximately six years by sprinkling the capsule contents over food. Patients over age 12 and over 110 pounds weight should receive 2.0 grams/day, divided four times daily.

Off-Label Use and Dosage (Pediatric)

Guideline-Supported Use

There is limited information regarding Off-Label Guideline-Supported Use of Cysteamine in pediatric patients.

Non–Guideline-Supported Use

There is limited information regarding Off-Label Non–Guideline-Supported Use of Cysteamine in pediatric patients.

Contraindications

CYSTAGON® is contraindicated in patients who have developed hypersensitivity to it or to cysteamine or penicillamine.

Warnings

If a skin rash develops, CYSTAGON® should be withheld until the rash clears. CYSTAGON® may be restarted at a lower dose under close supervision, then slowly titrated to the therapeutic dose. If a severe skin rash develops such as erythema multiforme bullosa or toxic epidermal necrolysis, CYSTAGON® should not be readministered.

CNS symptoms such as seizures, lethargy, somnolence, depression, and encephalopathy have been associated with cysteamine. If CNS symptoms develop, the patient should be carefully evaluated and the dose adjusted as necessary. Neurological complications have been described in some cystinotic patients not on cysteamine treatment. This may be a manifestation of the primary disorder. Patients should not engage in hazardous activities until the effects of CYSTAGON® on mental performance are known.

Gastrointestinal ulceration and bleeding have been reported in patients receiving cysteamine bitartrate. Physicians should remain alert for signs of ulceration and bleeding and should inform patients and/or guardians about the signs and symptoms of serious G.I. toxicity and what steps to take if they occur.

Adverse Reactions

Clinical Trials Experience

There is limited information regarding Cysteamine (oral) Clinical Trials Experience in the drug label.

Postmarketing Experience

Post marketing reports include one report of interstitial nephritis with early renal failure. A causal relationship between this event and cysteamine bitartrate therapy has not been established.

Drug Interactions

None have been described. CYSTAGON® can be administered with electrolyte and mineral replacements necessary for management of the Fanconi Syndrome as well as vitamin D and thyroid hormone.

Use in Specific Populations

Pregnancy

Pregnancy Category (FDA): C Teratology studies have been performed in rats at oral doses in a range of 37.5 to 150 mg/kg/day (about 0.2 to 0.7 times the recommended human maintenance dose on a body surface basis) and have revealed cysteamine bitartrate to be teratogenic and fetotoxic. Observed teratogenic findings were cleft palate, Kyphosis, heart ventricular septal defects, microcephaly and exencephaly. There are no adequate and well-controlled studies in pregnant women. CYSTAGON® should be used during pregnancy only if the potential benefit justifies the potential risk to the fetus.
Pregnancy Category (AUS): There is no Australian Drug Evaluation Committee (ADEC) guidance on usage of Cysteamine (oral) in women who are pregnant.

Labor and Delivery

There is no FDA guidance on use of Cysteamine (oral) during labor and delivery.

Nursing Mothers

It is not known whether cysteamine is excreted in human milk. Because many drugs are excreted in human milk and because of the manifested potential of cysteamine for developmental toxicity in suckling rat pups when it was administered to their lactating mothers at an oral dose of 375 mg/kg/day (2,250 mg/m2/day, 1.7 times the recommended human dose based on body surface area), a decision should be made whether to discontinue nursing or to discontinue the drug, taking into account the importance of the drug to the mother.

Pediatric Use

The safety and effectiveness of CYSTAGON® for cystinotic children have been established. Cysteamine therapy should be initiated as soon as the diagnosis of nephropathic cystinosis has been confirmed.

Geriatic Use

There is no FDA guidance on the use of Cysteamine (oral) in geriatric settings.

Gender

There is no FDA guidance on the use of Cysteamine (oral) with respect to specific gender populations.

Race

There is no FDA guidance on the use of Cysteamine (oral) with respect to specific racial populations.

Renal Impairment

There is no FDA guidance on the use of Cysteamine (oral) in patients with renal impairment.

Hepatic Impairment

There is no FDA guidance on the use of Cysteamine (oral) in patients with hepatic impairment.

Females of Reproductive Potential and Males

There is no FDA guidance on the use of Cysteamine (oral) in women of reproductive potentials and males.

Immunocompromised Patients

There is no FDA guidance one the use of Cysteamine (oral) in patients who are immunocompromised.

Administration and Monitoring

Administration

There is limited information regarding Cysteamine (oral) Administration in the drug label.

Monitoring

There is limited information regarding Cysteamine (oral) Monitoring in the drug label.

IV Compatibility

There is limited information regarding the compatibility of Cysteamine (oral) and IV administrations.

Overdosage

There is limited information regarding Cysteamine (oral) overdosage. If you suspect drug poisoning or overdose, please contact the National Poison Help hotline (1-800-222-1222) immediately.

Pharmacology

There is limited information regarding Cysteamine (oral) Pharmacology in the drug label.

Mechanism of Action

There is limited information regarding Cysteamine (oral) Mechanism of Action in the drug label.

Structure

There is limited information regarding Cysteamine (oral) Structure in the drug label.

Pharmacodynamics

There is limited information regarding Cysteamine (oral) Pharmacodynamics in the drug label.

Pharmacokinetics

There is limited information regarding Cysteamine (oral) Pharmacokinetics in the drug label.

Nonclinical Toxicology

There is limited information regarding Cysteamine (oral) Nonclinical Toxicology in the drug label.

Clinical Studies

There is limited information regarding Cysteamine (oral) Clinical Studies in the drug label.

How Supplied

There is limited information regarding Cysteamine (oral) How Supplied in the drug label.

Storage

There is limited information regarding Cysteamine (oral) Storage in the drug label.

Images

Drug Images

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Package and Label Display Panel

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Patient Counseling Information

There is limited information regarding Cysteamine (oral) Patient Counseling Information in the drug label.

Precautions with Alcohol

Alcohol-Cysteamine interaction has not been established. Talk to your doctor about the effects of taking alcohol with this medication.

Brand Names

There is limited information regarding Cysteamine (oral) Brand Names in the drug label.

Look-Alike Drug Names

There is limited information regarding Cysteamine (oral) Look-Alike Drug Names in the drug label.

Drug Shortage Status

Price

References

The contents of this FDA label are provided by the National Library of Medicine.