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Thiola
Thiola (generic name: tiopronin) is a medication used to prevent the formation of cystine kidney stones in individuals with cystinuria, a rare genetic disorder that causes excessive excretion of cystine in the urine. Thiola works by reducing the amount of cystine in the urine, helping to prevent the development of kidney stones. It is often prescribed for patients who have not responded well to other dietary or medical interventions aimed at reducing cystine levels.
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Frequently Asked Questions
How long does it take for Thiola to start working?
Thiola may take several weeks to months to significantly reduce cystine levels in the urine. Regular urine tests will be necessary to monitor its effectiveness.
Can I take Thiola with other medications for kidney stones?
Yes, Thiola can be combined with other medications like potassium citrate, but always consult your doctor before starting any new medications to avoid interactions.
What should I do if I miss a dose of Thiola?
If you miss a dose, take it as soon as you remember unless it is close to the time for your next dose. Do not double the dose to make up for a missed dose.
Can I stop taking Thiola if my symptoms improve?
No, you should not stop taking Thiola without consulting your doctor. Stopping the medication may lead to the formation of new cystine kidney stones.
How often will I need blood tests while on Thiola?
Your doctor may recommend regular blood tests every 3 to 6 months to monitor liver function, kidney function, and blood counts to ensure that Thiola is safe and effective for you.
Comprehensive Drug Guide
How Does Thiola Work?
Thiola works by binding to cystine in the urine, forming a more soluble compound known as tiopronin-cysteine disulfide, which is easier for the body to excrete. This action helps to reduce the concentration of cystine in the urine, thereby lowering the risk of cystine stone formation.
Common Dosages
- 100 mg tablets
- 300 mg tablets
Typical Dosing
- For cystinuria: The typical starting dose for adults is 800 mg to 1000 mg daily, divided into three doses. The dose may be adjusted based on the patient's cystine levels in the urine and response to therapy. Maximum doses can range from 15 mg/kg/day up to 2000 mg/day in severe cases.
Typical Dosing
FDA Approved Indications
- Cystinuria, to prevent the formation of cystine kidney stones in patients who do not respond adequately to other treatments such as increased fluid intake and dietary modifications.
Who Shouldn't Take Thiola?
- Known hypersensitivity to tiopronin or any of its components
- Severe liver or kidney disease, unless closely monitored by a healthcare provider
Advice From The Pharmacist
- Take Thiola exactly as prescribed, and be sure to take it consistently, as directed by your healthcare provider.
- Increase your fluid intake to at least 3 liters per day to help prevent kidney stones.
- Monitor your urine cystine levels regularly, as directed by your doctor, to ensure the medication is effective.
- Thiola can be taken with or without food, but if it causes stomach upset, try taking it with food.
- Be sure to attend follow-up appointments to monitor liver and kidney function, as Thiola can affect these organs.
Side Effects of Thiola
Common Side Effects
- Nausea or vomiting
- Diarrhea
- Stomach pain or upset
- Loss of appetite
- Skin rash
Uncommon/Severe Side Effects
- Severe allergic reactions (rash, swelling, difficulty breathing)
- Liver toxicity
- Kidney problems
- Bone marrow suppression (lowered blood cell counts)
Risks and Warnings of Thiola
- Liver and Kidney Monitoring: Thiola can affect liver and kidney function, so regular monitoring of liver enzymes and kidney function tests is necessary during treatment.
- Bone Marrow Suppression: In rare cases, Thiola can cause a decrease in the production of blood cells, leading to anemia, leukopenia, or thrombocytopenia. Regular blood tests are recommended to monitor blood counts.
- Hypersensitivity Reactions: Patients with a history of allergic reactions to thiol-containing medications should avoid Thiola, as it can cause severe allergic reactions.
Interactions with Thiola
Common Drug Interactions
- ACE inhibitors: Thiola may increase the risk of certain side effects, such as swelling or allergic reactions, when taken with medications like enalapril or lisinopril.
- Gold compounds: Concurrent use of gold-containing medications (used for rheumatoid arthritis) may increase the risk of bone marrow suppression and liver toxicity.
- Immunosuppressants: Taking Thiola with immunosuppressants like cyclosporine may increase the risk of infections and immune suppression.
Alternatives to Thiola
- Penicillamine: Another thiol-containing drug used for cystinuria and cystine stone prevention.
- Potassium citrate: Used to alkalinize the urine and reduce cystine stone formation.
- Increased fluid intake and dietary modifications: Often the first line of defense in reducing cystine levels in the urine and preventing stone formation.
