Wilson's disease

Alternative names:

hepatolenticular degeneration

Treatment:

The goals of treatment are to reduce the amount of copper in the tissues and to manage the symptoms of the disorder. Treatment must be lifelong.

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.

Expectations (prognosis):

Lifelong treatment is required to control the disorder. The disorder may cause fatal effects, especially loss of liver function and toxic effects of copper on the nervous system. In cases where the disorder is not fatal, symptoms may be disabling.

Complications:

• acute hepatitis
• chronic hepatitis
• cirrhosis
• liver necrosis (death of liver tissues)
• spleen dysfunction
• anemia
• increased incidence of infections
• injury caused by falls
• loss of ability to interact with other people
• loss of ability to function at work and home
• loss of ability to care for self
• loss of muscle mass (atrophy)
• joint contractures or other deformity
• increased incidence of bone fractures
• side effects of penicillamine
• side effects of other medications used to treat the disorder

Calling your health care provider:

Call your health care provider if symptoms indicate Wilson's disease may be present.