| Wilson's disease causes the body to retain
copper. The liver of a person who has Wilson's disease does not release
copper into bile as it should. Bile is a liquid produced by the liver that
helps with digestion. As the intestines absorb copper from food, the
copper builds up in the liver and injures liver tissue. Eventually, the
damage causes the liver to release the copper directly into the
bloodstream, which carries the copper throughout the body. The copper
buildup leads to damage in the kidneys, brain, and eyes. If not treated,
Wilson's disease can cause severe brain damage, liver failure, and death.
Wilson's disease is hereditary. Symptoms usually appear between the
ages of 6 and 20 years, but can begin as late as age 40. The most
characteristic sign is the Kayser-Fleischer ring--a rusty brown ring
around the cornea of the eye that can be seen only through an eye exam.
Other signs depend on whether the damage occurs in the liver, blood,
central nervous system, urinary system, or musculoskeletal system. Many
signs would be detected only by a doctor, like swelling of the liver and
spleen; fluid buildup in the lining of the abdomen; anemia; low platelet
and white blood cell count in the blood; high levels of amino acids,
protein, uric acid, and carbohydrates in urine; and softening of the
bones. Some symptoms are more obvious, like jaundice, which appears as
yellowing of the eyes and skin; vomiting blood; speech and language
problems; tremors in the arms and hands; and rigid muscles.
Wilson's disease is diagnosed through tests that measure the amount of
copper in the blood, urine, and liver. An eye exam would detect the
Kayser-Fleischer ring.
The disease is treated with lifelong use of D-penicillamine or
trientine hydrochloride, drugs that help remove copper from tissue.
Patients will also need to take vitamin B6
and follow a low-copper diet, which means avoiding mushrooms, nuts,
chocolate, dried fruit, liver, and shellfish. Taking extra zinc may be
helpful in blocking the intestines' absorption of copper.
Wilson's disease requires lifelong treatment. If the disorder is
detected early and treated correctly, a person with Wilson's disease can
enjoy completely normal health.
More information is available from
Wilson's Disease Association
4 Navaho Drive
Brookfield, CT
06804
Phone: 1-800-399-0266 or (203) 775-9666
Email: hasellner@worldnet.att.net
Internet: http://www.wilsonsdisease.org/
American Liver Foundation
75 Maiden Lane, Suite 603
New York, NY
10038
Phone: 1-800-GO-LIVER (465-4837)
Email: info@liverfoundation.org
Internet:
http://www.liverfoundation.org/
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