Welcome to my Wilson's Disease Homepage

Purpose of Wilson's Disease Diet

     Wilson's Disease is a hereditary disease in the body when you are getting too much copper. Copper is a mineral in the body. It is essential for good health, but only a tiny amount is needed. When excess copper is taken in it is stored in the eyes, brain, kidneys, and liver. Too much copper in the liver causes cirrhosis of the liver, which is a serious, and life threatening. Wilson’s Disease is treatable. Copper is found in most foods. Dietary restrictions alone is usually not enough to control Wilson's Disease. The dietary intake of copper should be less than 1.0 mg per day.

Nutrition Facts

     A low copper diet is adequate in all nutrients necessary for good health. Patients taking Penicillamine may develop a loss of vitamin B-6, and the physician may prescribe a supplement of 25 mg per day.

Special Considerations

1. The content of copper in a specific food can vary by the number of factors. The copper content and where the food is produced, or the method used to process the food, can affect the amount of copper that is in the food when eaten. The diet is meant to restrict foods that are high in copper, especially organ meats, shellfish, dried beans, peas, whole wheat, and chocolate.

2. Drinking water should be tested for copper because it contains too much copper. If water contains more than 100 micrograms of copper per liter, then spring water should be used. Water should have 1 microgram of copper per liter. Spring water and distilled water are processed differently and contains less copper than drinking water.

3. Avoid alcohol. It can be harmful to the liver, and the liver is possibly already damaged from Wilson's Disease.

4. Always read food, mineral, and vitamins labels; some foods list the copper content.

5. For better copper control and intake, choose average portions of food. For example take 3-4 oz of meat, fish, or poultry; 1/2 cup of vegetables; 1 slice of bread.

6. Do not use copper cooking utensils.

7. Patients with Wilson's Disease should see a dietitian, not a doctor or a nurse, to make sure copper is being controlled.

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Kayser-Fleischer Rings

     Kayser-Fleischer rings are seen in 75% of children with only hepatic manifestations always present in those with neurological manifestations best seen with a slit lamp. The rings appear as a golden brown, brownish green, greenish yellow, bronze, or tarnish green within the limbic region of the cornea. Due to therapy the copper Kayser-Fleischer rings will disappear. Note: Rings will not harm eyesight.

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Penicillamine

     Penicillamine was first used in 1956 for a copper chelating agent. It increasing copper excretion and urinary copper excretion is a good method of monitoring the response of therapy. Also monitor liver function tests. It also helps with the disappearance of Kayser- Fleischer rings.

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Wilson's Disease Meanings:

1. Asymptomatic- some people with Wilson's Disease that never show symptoms are often called this.

2. Ceruloplasmin- a protein found in the blood which binds itself with copper. So the copper can be moved around the body by the circulatory system. Almost 90% of patients with Wilson's Disease have very low ceruloplasmin.

3. Jaundice- the yellowing of skin and whites of the eyes caused usually by disease of the liver or bile ducts.

4. Kayser- Fleischer Rings- discolouration of the outer membrane of the iris caused by copper deposits. Although the ring is not present in all the patients it is never presented in a person with Wilson's Disease.

5. Liver Biopsy- a procedure done while the patient is awake. A small piece of the liver is extracted by a special needle. (This can also be done in different ways)

6. Penicillamine- is the most common used drug with Wilson's Disease. It is a variant of penicillin. It's brand names are Cuprime and Depen.

7. Vitamin B-6- this is used to counteract some of the effects of the Penicillamine.

8. Bile Ducts- small tubes through which bile flows from the liver to the gall bladder and intestines.

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