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Wilson's disease

  • Alternative Names

    Hepatolenticular degeneration


    Treatment

    The goal of treatment is to reduce the amount of copper in the tissues. This is done by a procedure called chelation -- certain medications can bind to copper and help remove it through the kidneys or gut. Treatment must be lifelong.

    The following medications may be used:

    • Penicillamine (Cuprimine, Depen) binds to copper and leads to increased release of copper in the urine.
    • Trientine (Syprine) binds (chelates) the copper and increases its release through the urine.
    • Zinc acetate (Galzin) blocks copper from being absorbed in the intestinal tract.

    Vitamin E supplements may also be used.

    Sometimes, medications that chelate copper (especially penicillamine) can affect the function of the brain and nervous system (neurological function). Other medications under investigation may bind copper without affecting neurological function.

    A low-copper diet may also be recommended. Foods to avoid include:

    • Chocolate
    • Dried fruit
    • Liver
    • Mushrooms
    • Nuts
    • Shellfish

    You may want to drink distilled water because most tap water flows through copper pipes. Avoid using copper cooking utensils.

    Symptoms may be treated with exercise or physical therapy. People who are confused or unable to care for themselves may need special protective measures.

    A liver transplant may be considered in cases where the liver is severely damaged by the disease.


    Support Groups

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


    Expectations (prognosis)

    Lifelong treatment is needed to control Wilson's disease. 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
    • Anemia ( hemolytic anemia is rare)
    • Central nervous system complications
    • Cirrhosis
    • Death of liver tissues
    • Fatty liver
    • Hepatitis
    • Increased number of bone fractures
    • Increased number of infections
    • Injury caused by falls
    • Jaundice
    • Joint contractures or other deformity
    • Loss of ability to care for self
    • Loss of ability to function at work and home
    • Loss of ability to interact with other people
    • Loss of muscle mass (muscle atrophy)
    • Psychological complications
    • Side effects of penicillamine and other medications used to treat the disorder
    • Spleen problems

    Liver failure and damage to the central nervous system (brain, spinal cord) are the most common and dangerous effects of the disorder. If not caught and treated early, Wilson's disease is fatal.


    Calling your health care provider

    Call your health care provider if you have symptoms of Wilson's disease. Call a genetic counselor if you have a history of Wilson's disease in your family and you are planning to have children.