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Thiola EC
Thiola EC (generic name: tiopronin) is an extended-release medication used to prevent the formation of cystine kidney stones in individuals with cystinuria, a rare genetic disorder that leads to high levels of cystine in the urine, promoting stone formation. Thiola EC helps to lower cystine levels in the urine by forming a more soluble compound, making it easier for the body to excrete. The extended-release formulation provides the benefit of once-daily dosing in many cases, which can improve treatment adherence.
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Frequently Asked Questions
How long does it take for Thiola EC to work?
Thiola EC may take several weeks to months to effectively reduce cystine levels in the urine. Regular urine tests will help determine its effectiveness.
Can I take Thiola EC with food?
Yes, Thiola EC can be taken with or without food. If you experience stomach upset, taking it with food may help alleviate this.
What should I do if I miss a dose of Thiola EC?
If you miss a dose, take it as soon as you remember, unless it’s close to the time for your next dose. Do not double the dose to make up for a missed one.
Can I stop taking Thiola EC if my symptoms improve?
No, you should not stop taking Thiola EC without consulting your doctor. Discontinuing the medication may result in the formation of new cystine stones.
How often will my blood and urine be monitored while on Thiola EC?
Your doctor will regularly check your urine cystine levels and may perform blood tests every 3 to 6 months to monitor liver and kidney function as well as blood counts.
Comprehensive Drug Guide
How Does Thiola EC Work?
Thiola EC works by binding to cystine in the urine, converting it into a more soluble compound, tiopronin-cysteine disulfide, which is more easily excreted by the body. This reduces the concentration of cystine in the urine and lowers the risk of cystine stone formation.
Common Dosages
- 100 mg enteric-coated tablets
- 300 mg enteric-coated tablets
Typical Dosing
- For cystinuria: The typical starting dose for adults is 800 mg to 1000 mg daily, divided into one or two doses, depending on the patient's urine cystine levels and treatment response. The dose may be adjusted based on the monitoring of cystine levels in the urine, with a maximum recommended dose of 2000 mg/day.
Typical Dosing
FDA Approved Indications
- Cystinuria, to prevent the formation of cystine kidney stones in patients who have not responded to other therapies such as dietary changes and increased fluid intake.
Who Shouldn't Take Thiola EC?
- Known hypersensitivity to tiopronin or any of its components
- Severe liver or kidney disease without close medical supervision
Advice From The Pharmacist
- Take Thiola EC as prescribed, and do not crush or chew the tablets. The enteric coating is designed to protect the stomach and ensure proper absorption.
- Increase fluid intake to at least 3 liters per day to help prevent cystine stones from forming.
- Regularly monitor urine cystine levels as directed by your healthcare provider to ensure the medication is working effectively.
- Store Thiola EC at room temperature, away from moisture and heat.
- Be sure to attend regular follow-up appointments to monitor kidney function, as Thiola EC can affect the kidneys over time.
Side Effects of Thiola EC
Common Side Effects
- Nausea or vomiting
- Stomach pain or upset
- Diarrhea
- Loss of appetite
- Skin rash
Uncommon/Severe Side Effects
- Severe allergic reactions (rash, swelling, difficulty breathing)
- Liver toxicity
- Kidney damage
- Bone marrow suppression (lowered blood cell counts)
Risks and Warnings of Thiola EC
- Liver and Kidney Monitoring: Thiola EC may affect liver and kidney function, so regular blood tests are required to monitor for any signs of organ damage.
- Bone Marrow Suppression: Prolonged use of Thiola EC can lead to a decrease in blood cell production, which may result in anemia, leukopenia, or thrombocytopenia. Regular blood tests are recommended to monitor blood counts.
- Hypersensitivity Reactions: Patients with known allergies to thiol-containing compounds should avoid using Thiola EC, as it can cause severe allergic reactions.
Interactions with Thiola EC
Common Drug Interactions
- ACE inhibitors: Thiola EC may increase the risk of swelling or allergic reactions when combined with medications like lisinopril or enalapril.
- Immunosuppressants: Combining Thiola EC with immunosuppressive medications like cyclosporine may increase the risk of infection and immune-related side effects.
- Gold compounds: Co-administration of gold-containing medications (used for rheumatoid arthritis) may increase the risk of bone marrow suppression and liver toxicity.
Alternatives to Thiola EC
- Penicillamine: Another thiol-containing medication used to reduce cystine levels in patients with cystinuria.
- Potassium citrate: Often used to alkalinize the urine and reduce the formation of cystine stones.
- Increased fluid intake and dietary modifications: A key part of managing cystinuria that can sometimes prevent the need for medication.
