Wilson’s Disease

Also indexed as: Copper Overload, Hepatolenticular Degeneration

Wilson’s disease is a genetic disorder that results in excessive accumulation of copper in many parts of the body, particularly the liver.

This condition is readily treatable, but if Wilson’s disease is left untreated, it can be fatal.

Checklist for Wilson’s Disease

Rating Nutritional Supplements Herbs
3Stars

Zinc

  
3Stars Reliable and relatively consistent scientific data showing a substantial health benefit.
2Stars Contradictory, insufficient, or preliminary studies suggesting a health benefit or minimal health benefit.
1Star For an herb, supported by traditional use but minimal or no scientific evidence. For a supplement, little scientific support and/or minimal health benefit.

What are the symptoms of Wilson’s disease?

Wilson’s disease is initially silent and may first be noticed as fatigue, absent menstrual periods in premenopausal women, or repeated and unexplained spontaneous abortions. In more advanced stages, there may be headaches, tremors, uncoordinated limb movements, unsteady gait, drooling, difficulty swallowing, and joint pain. There also may also be strange thought patterns with unusual behaviors.

Medical treatments

Over the counter zinc supplements are sometimes used, but not in combination with the prescription medications used to treat Wilson’s disease, due to interactions.

The prescription drug trientine (Syprine®) is commonly used to remove excess copper. Oral penicillamine (Depen®), given with vitamin B6 to avoid depletion of the vitamin, is also used.

Healthcare providers also recommend a low copper diet.

Dietary changes that may be helpful

Most foods contain at least some copper, so it is not possible to avoid the metal completely. Foods high in copper, such as organ meats and oysters, should be eliminated from the diet. Some foods are relatively high in copper but are quite nutritious (e.g., nuts and legumes)—these foods should be eaten in moderation by people with Wilson’s disease. Grains contain significant amounts of copper but are important components of a healthful diet, and dietary restriction may be neither wise nor necessary, particularly if zinc is supplemented.

Nutritional supplements that may be helpful

Zinc is known for its ability to reduce copper absorption and has been used successfully in patients with Wilson’s disease,1 with some trials lasting for years years.2 3 Researchers have called zinc a “remarkably effective and nontoxic therapy for Wilson’s disease.”4 The U.S. Food and Drug Administration has approved the use of zinc to treat Wilson’s disease for maintenance therapy following drug therapy, although some scientists recommend that it be considered for initial therapy as well.5

Zinc has also been used to keep normal copper levels from rising in people with Wilson’s disease who had previously been treated successfully with prescription drugs.6 Zinc (50 mg taken three times per day) has been used for such maintenance therapy,7 though some researchers have used the same amount of zinc to successfully treat people with Wilson’s disease who had not received drug therapy.8

Zinc is so effective in lessening the body’s burden of copper that a copper deficiency was reported in someone with Wilson’s disease who took too much (480 mg per day) zinc.9 Nonetheless, zinc may not help everyone with Wilson’s disease. Sometimes increased copper levels can occur in the liver after zinc supplementation;10 however, leading researchers believe this increase is temporary and may not be not harmful.11

Zinc supplementation (25 mg or 50 mg three times daily) has also been used to successfully treat pregnant women with Wilson’s disease.12 Management of Wilson’s disease with zinc should only be undertaken with the close supervision of a doctor.

Copper is present in several dietary supplements, especially multimineral and multivitamin-mineral preparations. Supplements containing even small amounts of copper should be avoided by virtually all Wilson’s disease patients.

Are there any side effects or interactions?
Refer to the individual supplement for information about any side effects or interactions.

References

1. Hoogenraad TU, Van den Hammer CJA, Van Hattum J. Effective treatment of Wilson’s disease with oral zinc sulphate: two case reports. Br Med J 1984;289:273–6.

2. Cossack ZT. The efficacy of oral zinc therapy as an alternative to penicillamine for Wilson’s disease. N Engl J Med 1988;318:322–3 [letter/review].

3. Brewer GJ, Dick RD, Johnson VD, et al. Treatment of Wilson’s disease with zinc: XV long-term follow-up studies. J Lab Clin Med 1998;132:264–78.

4. Brewer GJ, Yuzbasiyan-Gurkan V. The use of zinc-copper metabolic interactions in the treatment of Wilson’s disease. J Am Coll Nutr 1989;8:452 [abstract 103].

5. Hoogenraad TU. Zinc treatment of Wilson’s Disease. J Lab Clin Med 1998;132:240–1 [editorial].

6. Brewer GJ, Hill GM, Dick RD, et al. Treatment of Wilson’s disease with zinc. III. Prevention of reaccumulation of hepatic copper. J Lab Clin Med 1987;109:526–31.

7. Brewer GJ, Yuzbasiyan-Gurkan V. Use of zinc-copper metabolic interactions in the treatment of Wilson’s disease. J Am Coll Nutr 1990;9:487–91.

8. Brewer JG, Yuzbasiyan-Gurkan V, Lee D-Y, Appelman H. Treatment of Wilson’s disease with zinc. VI. Initial treatment studies. J Lab Clin Med 1989;114:633–8.

9. Van den Hamer CJA, Hoogenraad TU. Copper deficiency in Wilson’s disease. Lancet 1989;ii:442 [letter].

10. Van Caillie-Bertrand M, Degenhart HJ, Visser HKA, et al. Oral zinc sulphates for Wilson’s disease. Arch Dis Child 1985;60:656–9.

11. Brewer JG, Yuzbasiyan-Gurkan V, Lee D-Y, Appelman H. Treatment of Wilson’s disease with zinc. VI. Initial treatment studies. J Lab Clin Med 1989;114:633–8.

12. Brewer GJ, Johnson VD, Dick RD, et al. Treatment of Wilson’s disease with zinc. XVII: treatment during pregnancy. Hepatology 2000;31:364–70.