Wilson’s disease is an inherited disorder where copper builds up in the liver, brain, eyes, and other organs. When copper builds up in toxin amounts it damages organs. If not treated, copper spreads to other parts of the body leading to severe liver impairment, brain damage, and death.
Copper plays a key role in the development of healthy nerves, bones, collagen, and the skin pigment melanin. Copper is generally absorbed from your food, and the excess is excreted through bile, which is produced in your liver.
When copper isn’t eliminated properly it can build up into toxic amounts leading to organ damage. With early diagnosis, the disease is treatable and managed to allow people to live normal lives.
Causes
Wilson’s disease is an inherited trait, where a defective gene is passed from a parent. If you receive only one abnormal gene, you won’t become ill yourself, but as a carrier, you can pass the defective gene to your children.
Genetic testing is recommended to determine if you are a carrier or have more than one defective gene and begin treatment. Early diagnosis and treatment give a greater chance of success to manage the disease and lessen organ damage.
Symptoms
Symptoms generally don’t appear until there is a build-up of copper in the brain, liver, or other organs.
- Fatigue, lack of appetite and/or abdominal pain
- Yellowing of the skin and whites of the eyes (jaundice)
- Golden-brown eye discoloration (Kayser-Fleischer rings)
- Fluid buildup in the legs or abdomen
- Uncontrolled movements or muscle stiffness
- Problems with speech, swallowing, or physical coordination
Treatment
According to NORD, National Organization for Rare Disorders, “Treatment for Wilson disease is life-long and aimed at lowering copper levels to nontoxic levels, and prevent the progression of the disease and trying to reverse any signs and symptoms that appear because of copper accumulation in the body.”
Specific treatment is normally tailored with several medications and determined by various factors.
This entry was originally published in Life Beyond Hep C, and is reprinted with permission.
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