Wilson's disease

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Central nervous system

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.

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The following medications may be used:

Zinc acetate (Galzin), which blocks the absorption of copper in the intestinal tract.
Trientine (Syprine), which binds or chelates the copper and leads to increased urinary excretion of the metal.
Penicillamine (Cuprimine, Depen), which also binds or chelates copper and leads to increased urinary excretion.

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

Support Groups:

Wilson disease support groups can be found at 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:

acute hepatitis
chronic hepatitis
cirrhosis
death of liver tissues
spleen dysfunction
anemia
increased number 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. Call a genetic counselor if Wilson's disease is present in your family.