Treatment of Wilson's Disease for Patients in the United Kingdom
Updated March 2021 by Rupert Purchase
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Copper is a small but essential part of our daily diet. In patients with Wilson's disease, the route of excretion of dietary copper is impaired and this results in an overload of copper in the body. The signs and symptoms of Wilson's disease are the consequence of this copper overload affecting various organs such as the liver and the brain. Wilson's disease can be treated by reducing the copper overload with the use of appropriate medication.
Drugs approved in the UK for Wilson's disease and how they work – March 2021
Currently, four drugs are approved for use in the UK for treating Wilson's disease: D-penicillamine, trientine in the form of trientine dihydrochloride or trientine tetrahydrochloride, and zinc acetate dihydrate. All four drugs are taken orally. D-Penicillamine and the two trientine formulations are "chelating agents", which attach to the copper in the body and remove it by urinary excretion. Zinc acetate dihydrate has a different mode of action and is known as an "inhibiting agent". It essentially limits the intestinal absorption of dietary copper, which leads to an overall decrease in copper overload in the body.
Taking the medication regularly
For patients newly diagnosed with Wilson's disease, the initial choice of one of these four drugs and the number of capsules or tablets to be taken each day will be determined by their consultant. In order to reduce copper overload, it is very important for patients to take their prescribed medication regularly, and at recommended times during the day. Treatment is lifelong. Taking the medication on an empty stomach will enhance the amount of these drugs which is absorbed by the body. Patients will be given advice about the best way to take their medication between meals.
Trientine and penicillamine are the generic (INN) names for these two drugs, while zinc acetate dihydrate is the accepted chemical name for this substance. But in addition, brand names are also used to identify penicillamine, the two trientine formulations and zinc acetate. In the UK, one of the brand names for penicillamine is Distamine, for trientine dihydrochloride (sold by Univar Solutions) it is Cufence, and for trientine tetrahydrochloride (sold by Orphalan UK) it is Cuprior. Zinc acetate has the brand name Wilzin and is marketed by Recordati Rare Diseases UK.
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Rupert Purchase, March 2021