Thiola Ec

Do not take it if

Quoted from the official label, section “Contraindications”.

How to take it

These directions are for this exact strength and form. Another one is different.

• The recommended initial dosage in adult patients is 800 mg/day.
• In clinical studies, the average dosage was about 1,000 mg/day.
• ( 2.1 ) The recommended initial dosage in pediatric patients 20 kg and greater is 15 mg/kg/day.
• Avoid dosages greater than 50 mg/kg per day in pediatric patients.
• ( 5.1 , 8.4 ) Measure urinary cystine 1 month after initiation of THIOLA EC and every 3 months thereafter ( 2.3 ) 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.
• ( 2.1 ) THIOLA EC can be crushed and mixed with applesauce.
• For preparation and administration instructions, see the full prescribing information.
• ( 2.2 )
• 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.

Quoted from the official label, section “Dosage & Administration”.

Other warnings

Quoted from the official label, section “Warnings”.

Pregnancy and breastfeeding

Quoted from the official label, section “OTC — Pregnancy or Breast Feeding”.

Other medicines

Quoted from the official label, section “Drug Interactions”.

If you take too much

In an emergency, call your local emergency number or a poison control centre.

Quoted from the official label, section “Overdosage”.

Use in children

• THIOLA EC is indicated in pediatric patients weighing 20 kg or more with severe homozygous cystinuria, in combination with high fluid intake, alkali, and diet modification, for the prevention of cystine stone formation who are not responsive to these measures alone.
• This indication is based on safety and efficacy data from a trial in patients 9 years to 68 years of age and clinical experience.
• Proteinuria, including nephrotic syndrome, has been reported in pediatric patients.
• Pediatric patients receiving greater than 50 mg/kg tiopronin per day may be at greater risk [see Dosage and Administration ( 2.1 , 2.3 ), Warnings and Precautions ( 5.1 ) and Adverse Reactions ( 6.1 )] .
• THIOLA EC tablets are not approved for use in pediatric patients weighing less than 20 kg [see Dosage and Administration ( 2.1 )] .

Quoted from the official label, section “Pediatric Use”.

Use in older people

• This drug is known to be substantially excreted by the kidney, and the risk of adverse reactions to this drug may be greater in patients with impaired renal function.
• Because elderly patients are more likely to have decreased renal function, care should be taken in dose selection, and it may be useful to monitor renal function.

Quoted from the official label, section “Geriatric Use”.

Side effects

• The following adverse reactions are discussed in greater detail in other sections of the labeling:
• Proteinuria [see Warnings and Precautions ( 5.1 )] Hypersensitivity [see Warnings and Precautions ( 5.2 )] Most common adverse reactions (≥10%) are nausea, diarrhea or soft stools, oral ulcers, rash, fatigue, fever, arthralgia, proteinuria, and emesis.
• ( 6 ) To report SUSPECTED ADVERSE REACTIONS, contact Mission Pharmacal Company at toll-free phone # 1-800-298-1087 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.
• 6.1 Clinical Trials Experience Because clinical trials are conducted under widely varying conditions, the adverse reaction rates observed in the clinical trials of the drug cannot be directly compared to rates in the clinical trials of another drug and may not reflect the rates observed in practice.
• Adverse reactions occurring at an incidence of ≥5% in an uncontrolled trial in 66 patients with cystinuria age 9 to 68 years are shown in the table below.
• Patients in group 1 had previously been treated with d-penicillamine; those in group 2 had not.
• Of those patients who had stopped taking d-penicillamine due to toxicity (34 out of 49 patients in group 1), 22 were able to continue treatment with THIOLA.
• In those without prior history of d-penicillamine treatment, 6% developed reactions of sufficient severity to require THIOLA withdrawal.
• Table 1 presents adverse reactions ≥5% in either treatment group occurring in this trial.
• Table 1:
• Adverse Reactions Occurring in One or More Patients System Organ Class Adverse Reaction Group 1 Previously treated with d‑penicillamine (N = 49) Group 2 Naïve to d‑penicillamine (N = 17) Blood and Lymphatic System Disorders anemia 1 (2%) 1 (6%) Gastrointestinal Disorders nausea 12 (25%) 2 (12%) emesis 5 (10%) – diarrhea/soft stools 9 (18%) 1 (6%) abdominal pain – 1 (6%) oral ulcers 6 (12%) 3 (18%) General Disorders and Administration Site Conditions fever 4 (8%) – weakness 2 (4%) 2 (12%) fatigue 7 (14%) – peripheral (edema) 3 (6%) 1 (6%) chest pain – 1 (6%) Metabolism and Nutrition Disorders anorexia 4 (8%) – Musculoskeletal and Connective Tissue Disorders arthralgia – 2 (12%) Renal and Urinary Disorders proteinuria 5 (10%) 1 (6%) impotence – 1 (6%) Respiratory, Thoracic and Mediastinal Disorders cough – 1 (6%) Skin and Subcutaneous Tissue Disorders rash 7 (14%) 2 (12%) ecchymosis 3 (6%) – pruritus 2 (4%) 1 (6%) urticaria 4 (8%) – skin wrinkling 3 (6%) 1 (6%) Taste Disturbance A reduction in taste perception may develop.
• It is believed to be the result of chelation of trace metals by tiopronin.
• Hypogeusia is often self-limited.
• 6.2 Postmarketing Experience Adverse reactions have been reported from the literature, as well as during post-approval use of THIOLA.
• Because the post-approval reactions are reported voluntarily from a population of uncertain size, it is not always possible to reliably estimate their frequency or establish a causal relationship to THIOLA exposure.
• Adverse reactions reported during the postmarketing use of THIOLA are listed by body system in Table 2 .
• Table 2:
• Adverse Reactions Reported for THIOLA Pharmacovigilance by System Organ Class and Preferred Term System Organ Class Preferred Term Cardiac Disorders congestive heart failure Ear and Labyrinth Disorder vertigo Gastrointestinal Disorders abdominal discomfort; abdominal distension; abdominal pain; chapped lips; diarrhea; dry mouth; dyspepsia; eructation; flatulence; gastrointestinal disorder; gastroesophageal reflux disease; nausea; vomiting; jaundice; liver transaminitis General Disorders and Administration Site Conditions asthenia; chest pain; fatigue; malaise; pain; peripheral swelling; pyrexia; swelling Investigations glomerular filtration rate decreased; weight increased Metabolism and Nutrition Disorders decreased appetite; dehydration; hypophagia Musculoskeletal and Connective Tissue Disorders arthralgia; back pain; flank pain; joint swelling; limb discomfort; musculoskeletal discomfort; myalgia; neck pain; pain in extremity Nervous System Disorders ageusia; burning sensation; dizziness; dysgeusia; headache; hypoesthesia Renal and Urinary Disorders nephrotic syndrome; proteinuria; renal failure Skin and Subcutaneous Tissue Disorders dry skin; hyperhidrosis; pemphigus foliaceus; pruritus; rash; rash pruritic; skin irritation; skin texture abnormal; skin wrinkling; urticaria

Quoted from the official label, section “Adverse Reactions”.

What to discuss with your doctor

• Administration Instructions For patients who cannot swallow the tablet whole, the THIOLA EC tablets can be crushed and mixed with applesauce.
• See Dosage and Administration ( 2.2 ) for preparation and administration instructions.
• Lactation Advise women that breastfeeding is not recommended during treatment with THIOLA EC [see Use in Specific Populations ( 8.2 )] .
• Manufactured and packaged by Mission Pharmacal Company, San Antonio, TX 78230 1355 Distributed by Travere Therapeutics, Inc., San Diego, CA 92130 Copyright© 2024 Mission Pharmacal Company.
• All rights reserved.
• L090201R0824 THL_T13768R0924 Image

Quoted from the official label, section “Patient Counseling Information”.

Strengths and forms

• FORMS AND STRENGTHS Tablets for oral use:
• 100 mg tablets:
• round, white to off-white and imprinted in red with “T1” on one side 300 mg tablets:
• round, white to off-white and imprinted in red with “T3” on one side Tablets:
• 100 mg and 300 mg ( 3 )

Quoted from the official label, section “Dosage Forms & Strengths”.

What it looks like and how it is packed

• 100 mg delayed-release, round, white to off-white tablet imprinted with “T1” on one side with red ink and blank on the other side:
• Bottles of 300 NDC 0178-0902-01. 300 mg delayed-release, round, white to off-white tablet imprinted with “T3” on one side with red ink and blank on the other side:
• Bottles of 90 NDC 0178-0901-90.
• Store at 25°C (77°F); excursions permitted to 15-30°C (59-86°F) [see USP Controlled Room Temperature] .

Quoted from the official label, section “How Supplied”.

What is in it

• THIOLA EC (tiopronin) delayed-release tablets are a reducing and cystine-binding thiol drug (CBTD) for oral use.
• Tiopronin is N‑(2‑Mercaptopropionyl) glycine and has the following structure:
• Tiopronin has the empirical formula C 5 H 9 NO 3 S and a molecular weight of 163.20.
• In this drug product tiopronin exists as a dl racemic mixture.
• Tiopronin is a white crystalline powder, which is freely soluble in water.
• Each THIOLA EC tablet contains 100 or 300 mg of tiopronin.
• The inactive ingredients in THIOLA EC tablets include lactose monohydrate, hydroxypropyl cellulose, hydroxypropyl cellulose (low substitute), magnesium stearate, hydroxypropyl methylcellulose E5, methacrylic acid:
• ethyl acrylate copolymer (Eudragit L 100-55), talc, triethyl citrate.
• Tiopronin Chemical Structure

Quoted from the official label, section “Description”.

Ingredients people check for

Lactose, gluten, dyes, sugars and other ingredients that matter with an allergy, intolerance or diet — as this product’s FDA label lists them.

Details

Source: NDC Directory · 2026-09-13 · not reviewed by a clinician

Source: US Food and Drug Administration, NDC Directory. Reuse terms: US government work (FDA).

Report wrong or outdated information on this page

Other strengths and forms

2 products are sold under this name. Grouped by form; a number on a strength means several companies make it.

Same active substance

These contain the same substance. That does not mean one can replace another — ask a pharmacist.

See every product with Tiopronin