Treatment for Wilson’s disease is aimed at removing the excess copper from your body and preventing it from building up again. This is primarily therapy with de-coppering (‘chelating’) agents.
With early detection and successful treatment it is possible for you to enjoy a healthy life. This type of treatment is known as maintenance therapy and is lifelong.
In the UK, copper-chelation therapy is most commonly used and is begun as soon as diagnosis is made. In chelation therapy the drugs D-penicillamine (cuprimine or depen) or trientine dihydrochloride (syprine) are given to remove copper from your body.
Penicillamine is a drug related to penicillin (but without the antibiotic properties) and is the first choice medication. It is generally successful in preventing or repairing the effects of copper overload unless your liver disease has become very severe. However, around 30% of people are resistant to penicillamine and therefore trientine may be used instead.
Taken in tablet or capsule form, these drugs work by binding with the copper which is then passed from your body in urine.
Medication with penicillamine may have common side effects such as diarrhoea, loss of appetite and nausea. If these persist, you must alert medical staff. Penicillamine can, however, have more serious side effects such as:
- joint pain
- fever
- skin rash, or itching (pruritus)
- swelling and/or pain in your glands
- losing your sense of taste
- unusual bleeding (clotting problems) or bruising.
These side effects occur in around one in ten people. Penicillamine is also used to treat rheumatoid arthritis, kidney stones and other conditions. Potential side effects should be known to medical staff who will closely monitor you. If necessary, dosage of the drug may be lowered. Trientine is likely to have fewer side effects but should also be monitored. A new chelating drug called tetrathiomolybdate is being trialled for future treatment.
It may take up to three months before you notice any improvement in your condition from chelation therapy.
Another therapy uses zinc acetate (galzin). Zinc is known to block the absorption of copper by inducing a protein that binds copper in your intestinal cells. The copper is then lost in the faeces (stools) as the intestinal cells are shed into the gut. Because zinc does not remove copper from the body but blocks the absorption of copper from the diet a course of chelation therapy is used to first lower copper levels.
This medication is also taken orally. It has the benefi t of having fewer (if any) side effects than chelation therapy and is becoming more common. However, it does take considerably longer for the zinc to become effective (four to eight weeks).
It is likely you will be advised to follow a diet that is low in copper. Normally a person absorbs about 4mg of copper daily. This may mean reducing your intake to less than 1mg a day.
Liver transplantation
When your life is threatened by end stage liver disease from cirrhosis, or in rare cases where a sudden and severe (fulminant) form of hepatitis may cause liver failure, a liver transplant is the only option to help you survive.
This is a major operation. If you are able, you will need to plan it carefully with your medical team, family and friends.
Who will be looking after me?
In hospital it is likely you will be treated either by a specialist in liver disease called a hepatologist, a specialist in digestive disorders called a gastroenterologist or a specialist in blood disorders called a haematologist.
Where you may have other conditions or problems caused by Wilson’s disease, additional specialists may be involved in your care. These are likely to be a neurologist (brain) or nephrologist (kidneys).