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Wilson Disease

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Treatments

There is no way to prevent or cure Wilson disease, but it can be successfully managed. Symptoms, complications, and response to treatment will vary from person to person, even within families who have the same genetic mutations.

In those with symptomatic Wilson disease, the goals of treatment are to decrease excess copper stores, prevent their recurrence, preserve liver, neurological, and kidney function, and to minimize complications associated with the condition and associated with the medications used to treat it.

People who are asymptomatic but have been diagnosed as having Wilson disease, such as siblings of affected people, will usually be treated to decrease any excess copper that is present and to prevent its buildup. Those who are carriers of Wilson disease should receive genetic counseling but do not usually require any treatment.

Most people with Wilson disease are treated first with one of two chelating agents, D-penicillamine or trientine (triethylene tetramine dihydochloride), to increase urinary excretion of copper and decrease copper stores. They must be monitored for side effects as the medications can decrease red and white blood cells and platelets and can cause nausea, fever, and skin conditions. Some people must take these medications long-term; others can switch to zinc therapy once copper stores have normalized. High doses of zinc inhibit the absorption of copper.

Those affected are put on a low copper diet. Copper dietary restriction and treatment must be continued throughout a person's life. Treatments may be changed but should never be discontinued. Untreated Wilson disease is eventually fatal, and organ damage is usually permanent. In some severe cases, liver transplantation may be necessary.

Antioxidants such as vitamin E may be recommended to help prevent liver and other tissue damage.

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