Wilson Disease (WD)

Wilson disease is a rare inherited condition that affects how the body processes and removes excess copper. In Wilson disease, excess copper accumulates in the organs and tissues, especially the liver, brain, kidneys, and eyes. At first, dietary copper is transported to the liver after it is absorbed by the digestive system. Excess copper accumulates in the liver until it spills over into the bloodstream, where it travels to other sites throughout the body.¹

Two main types of medications prevent further accumulation of copper in the body. Chelators like penicillamine and trientine aid in binding excess copper and removing it from the body through the urine. Zinc prevents dietary copper from being absorbed by the digestive tract.^2,3

Low-Copper Diet

Another way to prevent the body from becoming overloaded with excess copper is to limit the amount of copper that is consumed in food. It is not possible to control Wilson disease through diet alone, but dietary restriction of copper is a helpful addition to the management of the condition. Many guidelines recommend following a low-copper diet for life, and it is especially important when first starting treatment for Wilson disease to allow the medications to start reducing copper levels to manageable levels.^4,5 

Foods With High Copper Content 

Copper concentrations in specific foods depend on many variables, including the copper content of the soil in which the food was grown, the methods used to process the food, the natural copper levels within the food itself, and even the use of copper cooking utensils, which may leave traces of copper behind in the food.⁵

Many foods are high in copper, especially shellfish, organ meats such as liver, chocolate, mushrooms, and nuts.^3,5-7 Other foods high in copper include cashews, black-eyed peas, vegetable juice, soy products, avocados, nectarines, dried beans and peas, lentils, barley, millet, wheat, bran, commercially dried fruits, fresh sweet potatoes, brewer’s yeast, instant breakfast beverages, and various meats and seafood such as lamb, pork, pheasant, duck, goose, salmon, and squid.^3,5 

Cereals should contain no more than 0.2 mg of copper per serving, so labels should be checked prior to eating. Labels should also be inspected for multivitamins or other dietary supplements to make sure that copper is not included. Prenatal vitamins tend to be high in copper, so these should be avoided prior to and during pregnancy. Consulting with an obstetrician and a liver specialist can help to determine which prenatal vitamins are safest to use.^3-5

It may be difficult for vegetarian patients to adhere to a low-copper diet because many of the protein alternatives used in a vegetarian diet are high in copper. Consulting with a dietitian to create a low-copper meal plan that works best within a vegetarian diet is very important.⁵

Foods With Low Copper Content

Several foods have low copper content, including eggs, beef, white meat such as turkey and chicken, rice, most vegetables, regular oatmeal, oils, most dairy products (such as butter, cream, and cheeses), fruit beverages, tea, coffee, and soups made with low-copper ingredients.⁵

Water

It is important that patients have copper levels checked in their water supply, especially if the water runs through or rests within copper plumbing. Copper levels in water should not be above 0.1 ppm. If copper levels in the water are higher than this, letting the water run for a while when it is first turned on or running tap water through a filter that removes copper might be necessary.^1,5-7

Alcohol

It is recommended that alcohol consumption is avoided, as this can hasten damage to hepatocytes, further complicating symptoms caused by Wilson disease.⁵

Following Initial Treatment

Once treatment has been established and has effectively lowered copper levels, it may be possible to consume small or moderate amounts of foods containing copper. This should only be done after consulting with a physician to best manage Wilson disease in the long term. It is also important that patients discuss any complementary or alternative medicines or supplements they take with their doctor, as these may contain copper.⁷

The Wilson Disease Association is currently undertaking a project that will provide comprehensive dietary and nutrition materials in a series of video webinars aimed at assisting patients with Wilson disease to make health-informed decisions when grocery shopping, eating out, and preparing food.⁶

References

1. Gilroy RK. Wilson disease: etiology. Medscape. Updated February 14, 2019. Accessed September 16, 2022.
2. Shah I. Wilson’s disease: patient education. Pediatric Oncall Child Health Care. April 2, 2001. Updated August 1, 2015. Accessed September 16, 2022.
3. Johnson LE. Wilson disease. MSD Manual Consumer Version. Updated December 2021. Accessed September 16, 2022.
4. Wilson disease. MedlinePlus. Updated April 6, 2016. Accessed September 16, 2022.
5. Low copper diet for Wilson’s disease. Arizona Digestive Health. Accessed September 16, 2022.
6. Copper conscious eating. Wilson Disease Association. Accessed September 16, 2022.
7. Eating, diet, & nutrition for Wilson disease. National Institute of Diabetes and Digestive and Kidney Diseases. Accessed September 16, 2022.

Reviewed by Kyle Habet, MD, on 9/27/2022.

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Maria Arini Lopez, PT, DPT, CSCS, CMTPT, CIMT

Maria Arini Lopez, PT, DPT, CSCS, CMTPT, CIMT is a freelance medical writer and Doctor of Physical Therapy from Maryland. She has expertise in the therapeutic areas of orthopedics, neurology, chronic pain, gastrointestinal dysfunctions, and rare diseases especially Ehlers Danlos Syndrome.