Wilson’s disease

In Wilson’s disease your liver can’t process and remove copper from your body in the usual way. Copper is an important mineral that we all get from our food, but we only need small amounts. Too much copper is poisonous (toxic). In Wilson’s disease, copper builds up and can damage your liver, brain and other organs.

Copper builds up in your liver cells (hepatocytes) first. In around half of people with Wilson’s disease the liver is the only organ that shows signs of the disease. The copper causes inflammation, damage and in some cases scarring (fibrosis) in your liver. This is known as hepatitis. Some people with Wilson’s disease develop liver cirrhosis.

At the moment Wilson’s disease cannot be cured, but at most stages treatment is helpful and can stop more damage to your body. People with Wilson’s disease can enjoy a full and healthy life. However, if it is not treated people can die from Wilson’s disease.

The main treatments are medicines that stop copper building up, you will need to take these for the rest of your life. Some people may also need to change their diet, especially just after diagnosis, so they take in less copper from food.

Wilson’s disease only affects people who have two faulty genes, one inherited from each of their parents. You can have one faulty gene without having the disease. So the parents of people with Wilson’s disease are often not affected themselves and probably did not know they carried a faulty gene. The close relatives of people with Wilson’s disease can be checked for the faulty gene.

Wilson’s disease was named after the scientist Dr Samuel Kinnier Wilson, who identified it in 1912. It has also been known as ‘hepatolenticular degeneration’.