Wilson Disease

Description:

Wilson's disease is a rare inherited disorder that causes copper to accumulate in your liver, brain and
other vital organs. Most people with Wilson's disease are diagnosed between the ages of 5 and 35, but it
can affect younger and older people, as well.

Copper plays a key role in the development of healthy nerves, bones, collagen and the skin pigment
melanin. Normally, copper is absorbed from your food, and excess is excreted through a substance
produced in your liver (bile).

But in people with Wilson's disease, copper isn't eliminated properly and instead accumulates, possibly
to a life-threatening level. When diagnosed early, Wilson's disease is treatable, and many people with
the disorder live normal lives.

Treatment:

If you take medications for Wilson's disease, treatment is lifelong. Medications include:

 Penicillamine (Cuprimine, Depen). A chelating agent, penicillamine can cause serious side
effects, including skin and kidney problems, bone marrow suppression, and worsening of
neurological symptoms. Penicillamine should be used cautiously if you have a penicillin allergy.
It also keeps vitamin B-6 (pyridoxine) from working, so you'll need to take a supplement in small
doses.
 Trientine (Syprine). Trientine works much like penicillamine but tends to cause fewer side
effects. Still, neurological symptoms can worsen when taking trientine.
 Zinc acetate (Galzin). This medication prevents your body from absorbing copper from the food
you eat. It is typically used as maintenance therapy to prevent copper from building up again
after treatment with penicillamine or trientine. Note: Zinc acetate is currently the most common
form of medication used to treat Wilson disease.