Two internationally recognised drugs for treatment exist: Zinc or Penicillamin and the treatment can be divided into two phases: a detoxification phase and a maintenance phase.
In the Penicillamin treatment the drug is used both for detoxication and for maintenance. Penicillamin binds the copper and leaves the body via the urine. During the treatment the content of copper in the body is reduced and as an effect the copper depositionings are diminishing or vanishing. The symptoms from the nerve system may worsen for a while, but the symptoms will vanish eventually. The on-going destruction of the liver will also stop, and the liver function can return to normal. Along with Penicillamin a supplement of vitamin B is to be taken.
The treatment with Penicillamin is unfortunately not without side effects. The most common side effects are skin eruption, kidney disturbances or disturbance in the number of blood cells. As a result the treatment is to be closely followed by a doctor. Due to the worsening in the detoxication phase and the side effects of the drug most of the Danish patients are treated with Zinc.
Zinc works by preventing absorption of copper via the intestine and by stimulating the production of a certain protein in the cells (metallothionin) that binds copper, especially in the intestinal mucosa. In the detoxication phase the Zinc treatment might be supplemented with a drug that eliminates the accumulated copper, e.g. Trientine or Tetrathiomolybdat. The use of these drugs requires a special permission.
In both treatments a supplement of vitamin E is recommended.
The drugs are to be taken for as long as the patients live, and it is paramount to observe the prescribed treatment. Sadly, a few patients stopped taking the prescribed drug, they experienced a serious recurrence and worsening of the disease and died.
Finally, it should be mentioned that a few patients have had a liver transplantation, but that is only in the cases where the liver is seriously damaged.
