Select therapeutic use:
Indications for PROCYSBI:
Management of nephropathic cystinosis.
Adults and Children:
<6yrs: not established. Take on an empty stomach. Swallow whole or sprinkle contents of cap on 4oz of food (eg, applesauce or berry jelly) or mix in 4oz of liquids (eg, orange or apple juice); also, may administer via feeding tube after mixing with 4oz of applesauce. ≥6yrs: New patients: start on ⅙ to ¼ of the maintenance dose; increase gradually over 4–6 weeks. Maintenance: 1.3g/m2/day, in 2 divided doses given every 12 hrs. Max 1.95g/m2/day. Goal of therapy: to maintain WBC cystine level <1nmol ½ cystine/mg protein or a plasma cysteamine concentration >0.1mg/L. Switching from immediate-release cysteamine: give previous total daily dose of immediate-release caps. Dose titration, measuring WBC cystine levels: see full labeling.
Monitor for development of skin or bone lesions and interrupt dosing if occur; may restart at a lower dose. Discontinue if severe skin rash develops. Consider lowering the dose if severe GI symptoms develop. Evaluate and monitor for CNS symptoms; interrupt or adjust dose if severe symptoms, persist or progress. Monitor blood counts and alkaline phosphatase levels. Monitor for signs/symptoms of benign intracranial hypertension. Pregnancy (Cat.C). Nursing mothers: not recommended.
Vomiting, abdominal pain/discomfort, headaches, nausea, diarrhea, anorexia/decreased appetite, breath odor, fatigue, dizziness, skin odor, rash; skin or bone lesions, leukopenia, CNS effects (eg, somnolence, encephalopathy, seizures), GI ulceration/bleeding, interstitial nephritis, elevated alkaline phosphatase, pseudotumor cerebri, papilledema.
Caps 25mg—60; 75mg—250