Manual of Clinical Nutrition Management III- 119 Copyright © 2013 Compass Group, Inc.
WILSON’S DISEASE
Discussion
Wilson’s disease is an inherited disorder of copper metabolism that is characterized by the abnormal
transport and storage of copper, resulting in hepatolenticular degeneration, neurologic damage, and damage
to the kidney, brain, and cornea. Onset may occur from 5 to 40 years of age. Liver disease is always present
when a patient with Wilson’s disease presents with any symptoms. Wilson’s disease is a highly treatable
condition; with proper therapy, the disease progress can be halted, and often symptoms can be improved.
Copper promotes iron absorption for hemoglobin synthesis, formation of bone and myelin sheath. Most of
copper in the copper-albumin complex is converted to ceruloplasmin in the hepatic tissues. In Wilson’s
disease, tissue deposition occurs instead of ceruloplasmin formation.
Approaches
Wilson’s disease is treated with a copper chelating agent, such as penicillamine (Cuprimine, Depen) or
trientine (Syprine), to keep the patient in a negative copper balance. The chelating agent bonds with copper
to form stable complexes that are excreted in the urine. A patient initially treated with a chelating agent may
receive maintenance therapy with zinc acetate (Galvin). This is particularly important for the patient who
has experienced adverse reactions to chelating agents. Zinc acetate acts by blocking the absorption of copper
in the intestinal tract, which results in both the depletion of accumulated copper and prevention of its
reaccumulation.
Chelating agents should be taken orally before meals. A vitamin B 6 supplement is needed with this
medication. Usually 25 mg/day of vitamin B 6 is adequate. Zinc may also be necessary. If the treatment with
penicillamine alone does not achieve a negative copper balance, a low-copper diet (1 to 2 mg/day) may be an
appropriate adjunct.
A normal diet provides 2 to 5 mg/day of copper. To achieve 1 to 2 mg/day of copper, limit intake of the
following foods:
Organ meats, such as liver, kidney, and brain
Shellfish, such as oysters, crab, and lobster
Dried legumes, except peas
Whole wheat and bran breads and cereals
Baked potato with skin
Sweet potato
Dried fruits, such as raisins, dates, and prunes
Mushrooms
Chocolate
Nuts and seeds
Wild game, such as duck and goose
Mineral water
No alcohol is permitted due to its hepatotoxic action.
Zinc acetate (Galvin) is given in 50-mg doses three times a day. Take separate from food and beverages
(other than water) by 1 hour. Avoid liver and limit shellfish (the only restrictions). Check the copper content
of drinking water. If copper is greater than 1 ppm, deionized water should be used.
Do not use copper or bronze cooking utensils.
Make sure supplements are copper free.
Bibliography
Escott-Stump S. Nutrition and Diagnosis-Related Care. 5th ed. Baltimore, Md: Lippincott, Williams & Wilkins; 2002.
Hesse JM, Matarese LE. Medical nutrition therapy for liver, bilary system, and exocrine pancreas disorders. In: Mahan KL, Escott-Stump S,
eds. Krause’s Food, Nutrition, and Diet Therapy. 10th ed. Philadelphia, Pa: WB Saunders; 2000: 703.
Skipper A, ed. Dietitian’s Handbook of Enteral and Parenteral Nutrition. 2nd ed. Gaithersburg, Md: Aspen Publishers; 1998.
Pennington J. Bowe’s and Church’s Food Values of Portions Commonly Used. 17th ed. City: Philadephia, Pa: Lippincott-Raven; 1998.
Wilson’s Disease Association. Available at: http://www.medhelp.org/wda/lit Accessed April 28, 1998.