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 experiencing pain, you are at risk for stone formation. It is critical to work with your doctor to establish an optimal treatment plan focused on becoming stone-free by managing your cystine levels.

A guide to your goals: help prevent the next cystine stone from forming

You can take an active role in achieving the goal of stone-free without surgery by knowing the facts—and your cystine level.

1

Understand your levels

Cystine kidney stones can form when your cystine level is above the line of solubility (generally <250 mg/L).

2

Prevent the next stone

Keeping your cystine level below this solubility line can help prevent the next cystine stone from forming.

3

Stay on track

A 24-hour urine test is the best way of knowing if your cystine level is below the line and in turn, on the optimal treatment plan.

Talk to your doctor

Don’t wait until the next stone forms to see your doctor. Take control of your cystinuria by staying in communication with your doctor and monitoring your cystine level.


Here are 4 ways to get your cystine level below the line:

Hydration Hydration

Consume at least 3 liters of fluid per day (10 glassfuls, 10 ounces each) to achieve a minimum urine output of 2 liters per day on a consistent basis.

Urinary pH Urinary pH

Maintain a urinary pH level between 6.5 and 7.0, often made possible by taking potassium alkali.

Dietary modifications Dietary modifications

Eat a diet low in protein and sodium, and high in fruits and vegetables.

Thiol-binding medication Thiol-binding medication

Use cystine-binding thiol drugs, like THIOLA® (tiopronin), in conjunction with therapeutic lifestyle changes.

While procedures exist to help remove cystine kidney stones, they do not treat the underlying disease and are not effective at preventing stone formation.

Dosage and treatment plan

The best way to get to stone-free is to stay on track with your treatment plan and regularly monitor your cystine level so that your doctor can optimize your THIOLA dosage and treatment plan.
See Important Safety Information below.

Have more questions? Connect to more resources and get the support you need to reach your treatment goals.


Talk to your doctor about establishing a treatment plan focused on preventing your next stone. Stone prevention results in:

Surgery

Avoiding surgery whenever possible, as surgery does not prevent cystine stones from recurring

Kidney

Keeping your kidneys working normally to prevent long-term damage

In addition to speaking with your doctor, learn more by connecting to educational resources.

Getting cystine under 250 mg/L can help you become stone-free 

People with cystinuria cannot process cystine properly, leading to excess levels of cystine in the urine (generally >250 mg/L) and, ultimately, the continual formation of cystine kidney stones.

Understand the science behind cystinuria

Cystine kidney stones (pictured below) are different from other kinds of kidney stones—they are often larger, harder, occur more frequently, and often require surgery to remove. Pain and disruption of normal daily life are often unfortunate consequences of living with cystinuria.

Close up picture of cystine stones
Picture of 6 cystine stones

Photo credit: Florian Buchkremer

Below the line

Manage your cystine stones by keeping cystine levels below the line of solubility (generally <250 mg/L).

Fast facts about living with cystinuria

Cystinuria can pose significant challenges. Read some quick facts below to help you better understand your condition.

1 in 10,000

About 1 in 10,000 people in the United States live with cystinuria.

80%

More than 80% of people with cystinuria develop cystine kidney stones by age 20.

10%

Cystinuria is the cause of approximately 10% of all kidney stones in children.

1/yr

It has been reported that people with cystinuria experience an average of 1 stone formation per year.

70%

Up to 70% of people with cystinuria experience long-term kidney disease.

every 3yrs

People with cystinuria undergo an average of 1 surgical procedure every 3 years, totaling an average of 7 surgical procedures by middle age.

The higher your cystine levels, the more likely you are to form stones

Your doctor diagnosed you with cystinuria because you have excess cystine in your urine and are at risk of chronic stones.

Time

The American Urological Association emphasizes the importance of using a 24-hour urine test not only to diagnose cystinuria, but as a way for you and your doctor to monitor your cystine level.

Doctor

Your doctor may also perform a stone analysis or order imaging tests to help understand more about your condition.

A 24-hour urine test is important for evaluation and proper management of cystinuria. Regular monitoring of cystine via 24-hour urine collection can provide your doctor with important information for optimizing your treatment plan and THIOLA tablets dose.

Paul’s 20-year journey to stone-freea

After years of stone events, countless visits to the emergency department, and chronic pain, Paul finally found the management plan that was right for him—and he has been stone-free for more than 3 years.

Can you commit to becoming stone-free? Watch Paul’s story to learn how he did.

aIndividual patient response. Not all patients will respond similarly to the same medical treatment.

For more information on Paul’s journey, as well as cystinuria management tips and insights, download this guide. If you have questions about your own journey to stone-free, bring this guide to your next doctor’s appointment to get the conversation started.

THIOLA® (tiopronin)

Important Safety Information

What is THIOLA® (tiopronin) tablets?

THIOLA 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 pounds (20 kg) and who do not respond to these measures alone. 

Who should not take THIOLA?

THIOLA should not be taken if you:

  • Are allergic to THIOLA, or any of the ingredients in THIOLA.

What should I tell my doctor before taking THIOLA?

THIOLA may not be right for you.  Before taking THIOLA, 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 are possible side effects of THIOLA?

Serious Side Effects: THIOLA 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. 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 Prescribing Information

 

THIOLA® EC (tiopronin)

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 pounds (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