Wilson’s disease can be cured with early diagnosis and effective treatment. The aim of treatment is to reduce the accumulation of copper in the body. For this purpose, excessive copper accumulation and toxicity in the tissues are tried to be reduced by using treatment methods that reduce the absorption of copper from the intestines and increase its excretion in the urine. In a patient diagnosed with Wilson’s disease, treatment is continued for life. Neurological damage is usually permanent. Siblings and children of Wilson’s patients should be screened and if there is evidence of copper accumulation in the body, treatment should be started even if there are no symptoms.
Penicillamine (Penicillamine) and trientine hydrochloride are drugs that bind copper in the body and allow it to be excreted in the urine. Clinical and biochemical improvement may occur if treatment with these drugs is started early. Drug doses can be reduced after 2-3 years from the start of treatment. Up to 10% of patients using penicillamine may experience serious drug-related side effects such as skin rashes, decrease in white blood cells (leukopenia) and kidney damage.
Zinc acetate (Zinc acetate) is a substance that acts by reducing the absorption of copper from the intestines and can be used instead of penicillamine because it has no obvious side effects. Trientine hydrochloride is the most preferred drug today due to its low side effects. It may be more effective if used with a zinc preparation. In patients with severe hepatic and neurological findings, the combined use of these 2 drugs is preferred in the first few months. Tetrathiomolybdolate is another drug that can be used in treatment.
Liver transplantation is the most effective treatment for Wilson’s disease in terms of providing definitive treatment. However, a limited number of applications can be made due to the lack of donor and the problems that may occur after transplantation. Fulminant liver failure or decompensated liver cirrhosis should be treated with liver transplantation.
In Wilson patients, consumption of certain foods with high copper content, such as giblets, lobster, mussels, crab, chocolate, soybeans, peas, mushrooms, dried kernels and peanuts, and beer and mineral drinks should be restricted. Since cooking foods in copper pots will increase the amount of copper taken, copper pots should not be used for cooking and storage.
If there is more than 0.1 ppm copper in drinking water, the use of this water may be risky for Wilson’s patients. Copper-containing intrauterine device (spirals) should not be used for birth control in women with Wilson’s disease.