What's the treatment for Wilson's disease?
The goals of treatment of Wilson's disease are to reduce the amount of copper in the tissues and to manage the symptoms of the disorder. Treatment must be lifelong. Treatment is started right after the diagnosis is made, even if there are no symptoms. It continues for the rest of the person's life. If treatment is stopped, the symptoms return. Treatment
includes avoiding foods rich in copper. Examples of such foods are dried beans, peas, whole wheat, chocolate, and organ meats.
Medicines are also needed. Penicillamine is usually used to treat Wilson's disease. It binds copper and allows it to be excreted in the urine. Pyridoxine, a component of vitamin B6, is also given. Other medicines such as trientine dihydrochloride and zinc may be used in some cases. Pyridoxine (vitamin B6) is used to counteract nervous tissue damage. Potassium or sodium supplements may be given before meals to reduce the amount of copper that is absorbed from foods. Penicillamine is a medication that helps move copper out of the tissues and causes it to be excreted from the body in the urine. Corticosteroids such as prednisone may be used if the person cannot tolerate penicillamine.
A low-copper diet may be recommended, including avoiding mushrooms, nuts, chocolate, dried fruit, liver, and shellfish. Distilled water may be suggested because most tap water flows through copper pipes. Avoid using copper cooking utensils.
Symptoms are treated as appropriate, including exercises or physical therapy, and protective measures for people who are confused or unable to care for themselves.
In rare cases, Wilson's disease may cause rapid, severe liver damage. The only option in such cases is a liver transplant. |