THIOLA EC Delayed-release tablet Ref.[50386] Active ingredients: Tiopronin

2.1 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 EC in 3 divided doses at the same times each day, with or without food. Maintain a routine pattern with regard to meals.

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

2.2 Preparation and Administration Instructions

For patients who cannot swallow the tablet whole, THIOLA EC can be crushed and mixed with applesauce. Administration of THIOLA EC with other liquids or foods has not been studied and is not recommended.

Preparation and Administration of THIOLA EC Mixed in Applesauce

For patients who can swallow semi-solid food, THIOLA EC can be crushed and mixed with applesauce:

• Crush the THIOLA EC tablet in a clean pill crusher or mortar and pestle. Always crush one tablet at a time.
• Measure approximately one tablespoon of applesauce and transfer it into a container with the crushed THIOLA EC tablet.
• Mix the crushed THIOLA EC tablet in the applesauce until the powder is well dispersed.
• Administer the entire THIOLA EC-applesauce mixture to the patient’s mouth immediately. (However, if this is not possible, the mixture can be stored in a refrigerator for up to 2 hours after adding the crushed tablet to the applesauce. Discard any mixture that has not been given within 2 hours.)
• To assure that any leftover applesauce mixture from the container is recovered, add tap water to the same container, mix, and have the patient drink the water.

2.3 Monitoring

Measure urinary cystine 1 month after starting THIOLA EC and every 3 months thereafter. Adjust THIOLA EC 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 EC in patients who develop proteinuria, and monitor urinary protein and renal function. Consider restarting THIOLA EC treatment at a lower dosage after resolution of proteinuria.

There is no information on overdosage with tiopronin.

Store at 25°C (77°F); excursions permitted to 15-30°C (59-86°F) [see USP Controlled Room Temperature].