Cysteamine Bitartrate (2 brand names, 3 dosage forms, 1 route)
Available dosage forms: GRANULE, DELAYED RELEASE, CAPSULE, DELAYED RELEASE, CAPSULE.
Routes of administration: ORAL.
Brand Names
- CYSTAGON
- PROCYSBI
Indications and Usage
PROCYSBI is indicated for the treatment of nephropathic cystinosis in adults and pediatric patients 1 year of age and older.
Contraindications
The use of PROCYSBI is contraindicated in patients with a serious hypersensitivity reaction, including anaphylaxis, to penicillamine or cysteamine.
Adverse Reactions
The following adverse reactions are also discussed in other sections of the labeling: Ehlers-Danlos-like Syndrome [see Warnings and Precautions (5.1)] Skin Rash [see Warnings and Precautions (5.2)] Gastrointestinal (GI) Ulcers and Bleeding [see Warnings and Precautions (5.3)] Fibrosing Colonopathy [see Warnings and Precautions (5.4)] Central Nervous System Symptoms [see Warnings and Precautions (5.5)] Leukopenia and/or Elevated Phosphatase Levels [see Warnings and Precautions (5.6)] Benign Intracranial Hypertension [see Warnings and Precautions (5.7)]
Mechanism of Action
Cysteamine is an aminothiol that participates within lysosomes in a thiol-disulfide interchange reaction converting cystine into cysteine and cysteine-cysteamine mixed disulfide, both of which can exit the lysosome in patients with cystinosis.
Overdosage
One case of overdosing with PROCYSBI has been reported. A 16-year-old male patient suffered nausea and vomiting after he mistakenly took a second dose of PROCYSBI 30 minutes after his usual dose. Two cases of overdosing with immediate-release cysteamine bitartrate have been reported in two patients. In the first case, the patient immediately vomited after ingesting an unknown dose and did not develop any symptoms. The second case involved an accidental ingestion of a 200 to 250 mg/kg dose by a healthy 13-month-old child. Vomiting and dehydration were experienced. The child was hospitalized and fluids were administered. The patient fully recovered from the overdosing. Should overdosing occur, the respiratory and cardiovascular systems should be supported appropriately. No specific antidote is known. Hemodialysis may be considered since cysteamine is poorly bound to plasma proteins.