What is Wilson's Disease?
Wilson’s disease is an inherited condition that causes copper to collect in your body. Wilson’s disease is autosomal recessive. This means you must have two copies of the gene to develop the condition. The genetic disorder causes an abnormal protein to struggle transporting copper in your body. This causes copper to build up in your body. Your body uses copper to build healthy bones and skin, but too much can damage your liver, brain, eyes, and other organs. Providers generally diagnose Wilson’s disease in people between the ages of 5–40. Wilson’s disease is uncommon, affecting only 1 out of every 30,000 people.
Wilson's Disease Symptoms
Wilson’s disease is congenital (present at birth), but you won’t experience symptoms until copper builds up in your organs.
Liver Symptoms
Liver symptoms are usually the first sign of Wilson’s disease in children and young adults:
- Abdominal swelling and pain
- Dark urine
- Fatigue
- Jaundice (yellowing of your skin or whites of your eyes)
- Leg swelling
- Loss of appetite
- Unexplained weight loss
Nervous System Symptoms
Adults with Wilson’s disease may also experience nervous system symptoms:
- Clumsiness
- Depression and anxiety
- Difficulty talking or walking
- Personality changes and mood swings
- Tremors in your head, arms, or legs
Other Symptoms Caused by Wilson's Disease
- Anemia (decreased healthy red blood cells)
- Arthritis (joint pain and swelling)
- Osteoporosis (weak bones)
Wilson’s Disease Eyes: Copper in the Eye
If you have Wilson’s disease, you may develop a Kayser-Fleischer ring around the colored part (iris) of your eye. Copper deposits on your cornea (front part of the eye) cause the brownish-green ring to appear as the disease progresses. These rings of copper are important in diagnosing Wilson’s disease. An eye specialist with a special lighted microscope can detect KF rings.
What Causes Wilson’s Disease?
A protein made by your liver removes the copper your body doesn’t need. People with Wilson’s disease have a gene mutation that stops this protein from working as it should. This causes extra copper to build up to toxic levels in your organs and tissues.
Genetic Testing for Wilson’s Disease
The gene mutation that causes Wilson’s disease passes through families. If both parents carry the mutation, their children are at greater risk of having Wilson’s disease. If one parent has the gene mutation, they can still pass it to their children and grandchildren. Genetic testing will tell you if your current or future children are at risk of developing the disorder.
Wilson’s Disease Treatments
Without treatment, copper can accumulate in your body, causing life-threatening organ damage. Wilson’s disease treatment focuses on lowering copper levels in your body to prevent organ damage and reduce symptoms. Treatment options include a combination of dietary changes and medication.
Wilson’s Disease Medication
Your provider will prescribe medications to make your organs release copper so your body can eliminate it as waste. You may also take medicines to treat tremors, muscle spasms, and other neurological symptoms associated with Wilson’s disease. You will need to take these medications for the rest of your life.
Diet for Wilson’s Disease
Avoiding the negative effects of Wilson’s disease requires reducing the amount of copper in your diet. You should eat a low-copper diet that avoids foods like mushrooms, shellfish, liver, dried fruits, and nuts. You should also avoid multivitamins with added copper. Your provider may also ask you to take zinc, which prevents your intestines from absorbing copper.
Wilson’s Disease Surgery
Untreated Wilson’s disease may cause severe liver damage that requires you to have a liver transplant. A surgeon will remove your damaged liver and replace it with a healthy liver from a donor. Our top-ranking liver transplant program has completed 600 adult liver transplants.
Wilson Disease Management
You’ll need to take medication to lower the amount of copper in your body and avoid eating foods or taking supplements that contain copper. With liver transplantation, you can live a normal life.
Additionally, you should see your provider regularly and keep your liver healthy, including getting vaccines for hepatitis A and B. If you’re pregnant or want to become pregnant, talk with your provider about changing your prenatal medicines.
Find a Wilson's Disease Specialist
Why Choose Â鶹ѧÉú¾«Æ·°æ?
The Liver Clinic at U of U Health treats common and rare liver conditions, such as Wilson’s disease. Board-certified hepatologists (liver specialists) and transplantation surgeons offer you comprehensive liver treatment, whether you live in the region or across the country. We also offer specialized treatment options for both pediatric (child) and adult patients.
Our team treats complex cases and combines the expertise of specialists in multiple areas:
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Hepatology
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Oncology
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Pathology
Make an Appointment
Please call 801-213-9797 to make an appointment. Providers should fax referrals to 801-581-2696.
