Select therapeutic use:
Indications for THIOLA:
To prevent kidney stone formation in patients with severe homozygous cystinuria with urinary cystine >500mg/day, who are resistant to treatment with conservative measures of high fluid intake, alkali and diet modification, or who have adverse reactions to d-penicillamine.
See full labeling. Give in divided doses 3 times/day at least 1 hour before or 2 hours after meals. Initially 800mg/day; usual dose 1000mg/day. Measure urinary cystine at 1 month after initiation, and then every 3 months. Adjust dose based on urinary cystine levels. Severe toxicity to d-penicillamine: may start with lower dose.
See full labeling. <9yrs: not established. ≥9yrs: Give in divided doses 3 times/day at least 1 hour before or 2 hours after meals. Initially 15mg/kg/day. Measure urinary cystine at 1 month after initiation, and then every 3 months. Adjust dose based on urinary cystine levels. Severe toxicity to d-penicillamine: may start with lower dose.
History of agranulocytosis, aplastic anemia, thrombocytopenia with prior treatment. Pregnancy (Cat.C). Nursing mothers.
Monitor for signs/symptoms of hematological abnormalities. Obtain blood counts, platelets, hemoglobin, serum albumin, liver function tests, 24-hour urinary protein and routine urinalysis at 3–6 month intervals during treatment. Do urinary cystine analysis frequently during the first 6 months when the optimum dose schedule is being determined, and at 6-month intervals thereafter. Assess abdominal roentogenogram (KUB) once a year.
Drug fever (discontinue until subsides, may restart at a small dose), rash, pruritus, lupus-like syndrome, hypogeusia, skin wrinkling/friability, GI upset; hematologic abnormalities (eg, leukopenia, thrombocytopenia); proteinuria; pulmonary manifestations; rare: Goodpasture's syndrome, myasthenia gravis, pemphigus-type reactions: discontinue if occurs.