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Wilson Disease
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Treatment for Wilson Disease
Wilson disease requires lifelong treatment to reduce and control the amount of copper in the body.
Initial therapy includes the removal of excess copper, a reduction of copper intake, and the treatment of any liver or central nervous system damage.
The drugs d-penicillamine (Cuprimine) and trientine hydrochloride (Syprine) release copper from organs into the bloodstream. Most of the copper is then filtered out by the kidneys and excreted in urine.
A potential major side effect of both drugs is that neurologic symptoms can become worse - a possible result of the newly released copper becoming reabsorbed by the central nervous system. About 20 to 30 percent of patients using d-penicillamine will also initially experience other reactions to the medication, including fever, rash, and other drug-related effects on the kidneys and bone marrow. The risk of drug reaction and neurologic worsening appears to be lower with trientine hydrochloride, which should be the first choice for the treatment of all symptomatic patients.
Pregnant women should take a lower dose of d-penicillamine or trientine hydrochloride during pregnancy to reduce the risk of birth defects. A lower dose will also help reduce the risk of slower wound healing if surgical procedures are performed during childbirth.
Zinc, administered as zinc salts such as zinc acetate (Galzin), blocks the digestive tract's absorption of copper from food. Zinc removes copper too slowly to be used alone as an initial therapy for people who already have symptoms, but it is often used in combination with d-penicillamine or trientine hydrochloride. Zinc is safe to use at full dosage during pregnancy.
Maintenance therapy begins when symptoms improve and tests show that copper has been reduced to a safe level. Maintenance therapy typically includes taking zinc and low doses of either d-penicillamine or trientine hydrochloride. Blood and urine should be monitored by a health care provider to ensure treatment is keeping copper at a safe level.
People with Wilson disease should reduce their dietary copper intake. They should not eat shellfish or liver, as these foods may contain high levels of copper. Other foods high in copper - including mushrooms, nuts, and chocolate - should be avoided during initial therapy but, in most cases, may be eaten in moderation during maintenance therapy.
People with Wilson disease should have their drinking water checked for copper content and should not take multivitamins that contain copper.
If the disorder is detected early and treated effectively, people with Wilson disease can enjoy good health.
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References:
National Digestive Diseases Information Clearinghouse (NDDIC)
July 2008
digestive.niddk.nih.gov
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