Answers
What is Wilson's disease?
Short answer
Wilson's disease is an inherited condition in which copper builds up in the liver, brain and eyes. It usually shows up between the ages of 5 and 35 as unexplained liver disease, tremor, slurred speech or behaviour change. It is treatable with lifelong medicines that remove copper, and brothers and sisters must be tested.
Symptoms
- Liver: raised liver enzymes, jaundice, fluid in the belly, or cirrhosis at a young age; sometimes sudden liver failure
- Brain: tremor, stiffness, slurred speech, drooling or clumsy handwriting
- Mood and behaviour changes, or falling school performance
- A golden-brown ring around the coloured part of the eye (Kayser-Fleischer ring)
Who is at risk
It is inherited from both parents, who usually have no symptoms. It is more common when parents are related by blood. Any young person with unexplained liver disease should be checked for it.
Tests
Blood ceruloplasmin (usually low), a 24-hour urine copper test (usually high), an eye examination with a slit lamp for Kayser-Fleischer rings, and genetic testing. Sometimes a liver biopsy measures the copper in the liver.
Treatment
Stopping treatment can be dangerous. With regular treatment, most people lead a normal life. Brothers, sisters and children of the patient should be tested, even without symptoms.
- Lifelong medicines that remove copper or block its absorption, taken every day without breaks
- A low-copper diet, especially in the first year
- Regular blood and urine tests to check copper levels and side effects
- Liver transplant for sudden liver failure or advanced cirrhosis
Low-copper diet in Wilson's disease
Eat more
- Rice, wheat, milk, curd, paneer and eggs, which are lower in copper
- Most vegetables and fruits in normal amounts
- Filtered water from plastic or steel containers
Cut down
- Organ meats such as liver, and shellfish such as prawns
- Nuts, chocolate, cocoa, mushrooms and dried legumes in large amounts
- Water stored in copper vessels, and cooking in copper utensils
Good to know
- The diet helps, but medicines are the main treatment and must never be stopped.
- Some multivitamins contain copper; check with your doctor before taking any.
Common questions
Is Wilson's disease curable?
It is controlled, not cured. With daily lifelong treatment, copper stays under control and most people live normal lives. A liver transplant cures the liver problem in severe cases.
Should my brothers and sisters be tested?
Yes. Each brother or sister has a 1 in 4 chance of having it. Testing finds it before symptoms, when treatment works best.
Can people with Wilson's disease have children?
Yes. Treatment continues during pregnancy, with dose adjustments by the doctor. Plan the pregnancy with your specialist.