From the creator of the Big Wow
Our three boys, Nicholas, Zach, and Joe were diagnosed with Wilson’s disease in July of 2013. In 2014, my wife Lisa and I attended the WDA conference in Ann Arbor Michigan. We were amazed by the stories we heard of those recently diagnosed or have lived with Wilson’s disease, showing remarkable strength in the face of great adversity. We realized that the Wilson’s community is filled with great people and that if we could somehow bring them all together, we could do great things. Hence, we set out on creating the first nationwide walk for Wilson’s disease. With the help of the WDA, we formed the Big WOW committee and we have all been working hard to make this event a reality. Thank you for joining us!
The Simpoulos Family
About Wilson's Disease
Wilson disease is a genetic disorder that is fatal unless detected and treated before serious illness from copper poisoning develops. Wilson disease affects approximately one in 20,000 people worldwide. The genetic defect causes excessive copper
accumulation in the liver or brain.
Small amounts of copper are as essential as vitamins. Copper is present in most foods and most people have much more copper than they need. Healthy people excrete copper they don't need but Wilson disease patients cannot.
Copper begins to accumulate immediately after birth. Excess copper attacks the liver or brain, resulting in hepatitis, psychiatric, or neurologic symptoms. The symptoms usually appear in late adolescence. Patients may have jaundice, abdominal swelling, vomiting of blood, and abdominal pain. They may have tremors and difficulty walking, talking and swallowing. They may develop all degrees of mental illness including homicidal or suicidal behavior, depression, and aggression. Women may have menstrual irregularities, absent periods, infertility, or multiple miscarriages. No matter how the disease begins, it is always fatal if it is not diagnosed and treated.
The first part of the body that copper affects is the liver. In about half of Wilson disease patients the liver is the only affected organ. The initial physical changes in the liver are only visible under the microscope. When hepatitis develops, patients are often thought to have infectious hepatitis or infectious mononucleosis when they actually have Wilson disease hepatitis. Testing for Wilson disease should be performed in individuals with unexplained, abnormal liver tests.
Wilson disease is a very treatable condition. With proper therapy, disease progress can be halted and oftentimes symptoms can be improved. Treatment is aimed at removing excess accumulated copper and preventing its reaccumulation. Treatment for Wilson disease is a lifelong process. Patients may become progressively sicker from day to day, so immediate treatment can be critical. Treatment delays may cause irreversible damage.
Chelation therapy drugs approved for treating Wilson disease work by binding of copper, causing its increased urinary excretion.
Zinc is a metallothionein inducer that acts by blocking the absorption of copper in the intestinal tract. This action both depletes accumulated copper and prevents its reaccumulation. Zinc's effectiveness has been shown by more than 30 years of considerable experience overseas. A major advantage of zinc therapy is its lack of side effects.
Patients with severe hepatitis or liver failure may require liver transplant.
For more in-depth information, please visit The Wilsons Disease Association.