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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.

The following medications may be used:

Sometimes, medications that chelate copper, especially penicillamine, can worsen the person's neurological function. There are other medications under investigation which will, hopefully, bind copper without risking possible worsening of neurological function.

In addition, 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. Patients are advised to 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 cases where the liver is severely damaged by the disease, liver transplantation might be considered by healthcare providers.

Support Groups:

Wilson disease support groups can be found at www.wilsonsdisease.org and www.geneticalliance.org .

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:

Calling your health care provider:

Call your health care provider if symptoms indicate Wilson's disease may be present. Call a genetic counselor if Wilson's disease is present in your family and you are planning to have children.

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