Understand the goals of cystinuria treatment to manage your condition
Cystinuria is a rare, lifelong, and serious genetic disorder that requires daily vigilance because of continuous buildup of cystine, an essential component of proteins, in the urine.
Even if you are not feeling any symptoms, you are at risk for stone formation. It is critical to work with your doctor to establish an optimal treatment plan focused on stone prevention.
A guide to your goals: help prevent the next cystine stone from forming
You can take an active role in cystine stone prevention by knowing the facts—and your cystine level.
Understand your levels
Prevent the next stone
Stay on track
Don’t wait until your next cystine stone to see your doctor. Take control of your cystinuria by staying in communication with your doctor and monitoring your cystine level.
Stone prevention begins with early recognition of cystine buildup
Regular monitoring enables early detection so you can take action at the first sign of cystine buildup. Look for these first signs of cystine buildup.
Sand/gravel in urine
24-hour urine test results showing elevated cystine levels
Imaging showing presence of stones
Kidney stone-related pain
If you are still forming or passing cystine stones, treatment adjustments may be necessary
THIOLA EC, along with increased fluids, alkali, and diet modification, may help prevent cystine stones
Increased fluid and alkali modification alone may not be enough to prevent stones from forming; 55% of patients continued forming stones on these methods alone.
In a clinical trial of 27 patients with cystinuria managed by high fluid intake and alkali modification, 15 patients failed to manage their condition on these methods alone.
Maximize cystine kidney stone prevention with a multifaceted approach
ACTION: Drink lots of fluids in order to produce at least 2.5 liters of urine daily. This goal requires drinking at least 4 liters a day.
WHY?: Increasing fluid intake helps to dilute cystine in the urine, making it harder for stones to form.
ACTION: Lower the amount of salt and animal protein in your diet. A reasonable goal for sodium intake is ≤2300 mg/day.
WHY?: Reducing sodium and animal protein in your diet reduces the amount of cystine in the urine.
ACTION: Increase urine pH level to 7.0 to help make your urine less acidic.
WHY?: Urine that is less acidic helps prevent cystine from binding together.
The right dosage of THIOLA EC helps remove cystine in the urine.
ACTION: Ask your doctor about finding the right THIOLA EC dosage to help reduce cystine below its solubility limit, generally <250 mg/L.
WHY?: Tiopronin, the active ingredient in THIOLA EC, attaches to cystine to help it dissolve more easily. Your THIOLA EC dosage may need to be adjusted to continue reducing the cystine below its solubility limit.
Download the enrollment form and talk to your doctor about how the right dose of THIOLA EC, in conjunction with increased fluids, alkali, and diet modification, may help you prevent cystine stones.
A personalized plan for managing cystinuria may help prevent your next stone
Partner with your doctor to find an individualized management plan that works for you. If you see an indicator of cystine buildup, don’t wait to talk to your doctor about making adjustments immediately.
Cystinuria management often requires a combination of measures to reduce cystine buildup and remove excess cystine from the body. Keep in mind that adjustments to your management plan should be individualized based on reducing cystine below its solubility limit, generally <250 mg/L.
Staying on track with managing your cystinuria is important
In an evaluation of medical files of 20 patients with cystinuria on a treatment plan, 11 compliant patients vs 9 non-compliant patients had greater success.*
- 73% of compliant patients stopped making stones (vs 33% non-compliant patients).
- An average of 1 surgery for compliant patients (vs 4 surgeries for non-compliant patients).
Those who follow their treatment plan as directed by their doctor may:
- Have fewer to no stones
- Have significantly lower urinary cystine levels
- Undergo significantly fewer surgical interventions
*Compliant patients defined as attending scheduled doctor appointments and reported compliance with their treatment plan.
Important Safety Information
What is THIOLA EC® (tiopronin) delayed-release tablets?
THIOLA EC delayed-release tablets is a prescription medicine. It is used in combination with high fluid intake, alkali (low acid), and dietary changes to help prevent the formation of one type (cystine) of kidney stones in certain adult and pediatric patients that weigh at least 44 lbs (20 kg) and who do not respond to these measures alone.
Who should not take THIOLA EC?
THIOLA EC should not be taken if you:
- are allergic to THIOLA EC, or any of the ingredients in THIOLA EC.
What should I tell my doctor before taking THIOLA EC?
THIOLA EC may not be right for you. Before taking THIOLA EC, tell your doctor about:
- all health conditions you may have and any medications you may be taking.
- if you are pregnant or plan to become pregnant.
- if you are breastfeeding or plan to breastfeed.
What should I avoid while taking THIOLA EC?
You should avoid drinking alcohol 2 hours before and 3 hours after taking THIOLA EC.
What are possible side effects of THIOLA EC?
Serious Side Effects: THIOLA EC can cause proteinuria (high amounts of protein in urine), including serious kidney problems, and hypersensitivity reactions (including fever, rash, joint pain and enlarged or swollen lymph nodes).
Most Common Side Effects: Nausea, diarrhea or soft stools, sores in the mouth, rash, fatigue, fever, muscle or joint pain, high amounts of protein in urine, vomiting.
These are not all the possible side effects of THIOLA EC. Call your doctor for medical advice about side effects.
You may report negative side effects to Retrophin® Medical Information at 1-877-659-5518, or to the FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.
Please see full THIOLA EC Prescribing Information.