Within the next few years, most of the specific gene defects should be identified. Effective treatment is available even in advanced stages. The toxic concentration of copper in the body must be removed and its re-accumulation prevented. This is done with the use of a de-coppering agent such as penicillamine or zinc. Treatment might also involve oral medication to increase copper excretion in the urine - like diuretics.
Treatment also includes the elimination of copper containing foods such as chocolate and mushrooms.
The treatment does not correct the fundamental flaw in liver function. Therefore, to prevent re-accumulation of copper in the body, treatment must be continued throughout the patient's lifetime. Therefore, when a new case is diagnosed, all children should be screened for the disease. Foods high in copper, such as organ meats and oysters, should be eliminated from the diet. 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.