Generic Name and Formulations:
Cysteamine bitartrate 50mg, 150mg; caps.
Management of nephropathic cystinosis.
New patients: start on ¼ to ⅙ of the maintenance dose; increase gradually over 4–6 weeks. Maintenance: >12yrs and >110lbs: 2g/day divided 4 times daily. Max 1.95g/m2/day. Goal of therapy to keep leukocyte cystine levels below 1nmol/½ cystine/mg protein 5–6 hours after administration. Patients taking cysteamine HCl or phosphocysteamine solutions may be transferred to equimolar doses of cysteamine bitartrate.
1.3g/m2/day in 4 divided doses. Max 1.95g/m2/day. If <6yrs old: do not give intact caps; may open and sprinkle capsule contents over food.
Withhold dose if skin rash develops, may be restarted at a lower dose then titrated slowly; if severe, do not readminister. Monitor blood counts, LFTs, vision, skin, bones. Measure leukocyte cystine levels every 3 months, more frequently when transferring from cysteamine HCl or phosphocysteamine solutions. Pregnancy (Cat.C). Nursing mothers: not recommended.
Vomiting, anorexia, fever, diarrhea, lethargy, rash; skin or bone lesions (reduce dose if appears), leukopenia, abnormal LFTs, CNS effects, GI ulceration/bleeding, pseudotumor cerebri, papilledema; rare: interstitial nephritis with early renal failure.
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