Recommended Dosage

Adults: The recommended initial dosage in adult patients is 800 mg/day. In clinical studies, the average dosage was about 1,000 mg/day.

Pediatrics: The recommended initial dosage in pediatric patients weighing 20 kg and greater is 15 mg/kg/day. Avoid dosages greater than 50 mg/kg per day in pediatric patients [see Warnings and Precautions (5.1), Use in Specific Populations (8.4)].

Administer THIOLA in 3 divided doses at the same times each day at least one hour before or 2 hours after meals.

Consider starting THIOLA at a lower dosage in patients with history of severe toxicity to d-penicillamine.

Monitoring

Measure urinary cystine 1 month after starting THIOLA and every 3 months thereafter. Adjust THIOLA dosage to maintain urinary cystine concentration less than 250 mg/L.

Assess for proteinuria before treatment and every 3 to 6 months during treatment [see Warnings and Precautions (5.1)].

Discontinue THIOLA in patients who develop proteinuria, and monitor urinary protein and renal function. Consider restarting THIOLA treatment at a lower dosage after resolution of proteinuria.