Wilsons Disease Research Paper

Words: 1063
Pages: 5

Wilson’s Disease; Copper and its effect on the body
To stay healthy, the human body requires certain minerals and vitamins. However, too much or too little can be extremely harmful. Copper is one of these necessary substances. Normally, copper is regulated and the excess is removed via the liver. When the liver fails to process out the copper, it begins to build up in the eyes, liver, and brain. Wilson’s disease is an inherited genetic disorder that results in the buildup of copper.
The body uses copper for growth and development of bone, the heart, the brain, and other organs. It also helps form new red blood cells, and the production and release of enzymes and proteins. Copper also stimulates the immune system, promotes healing,
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Both parents must be carriers and each child has the potential to be a carrier, 50% chance, or affected with Wilson’s, 25% chance. It is recommended that each child be tested for Wilson’s, especially if a close relative has died of liver disease.
According to Ala, Walker, Ashkan, Dooley, Schilsky (2007), Wilson’s disease was originally discovered by Kinnier Wilson in 1912. The first real case was documented in India in 1968 (Raju, Bangalore, Thuruvekere, Pathavanalli, 2015). The symptoms vary case to case and in some instances the patient has no symptoms at all until it is too late. On average symptoms present as early as age 5 and as late as age 40. On average, one in every 30,000 is diagnosed with Wilson’s disease and an estimated one in ninety is identified as carriers (Alam, Biswas, Biswas, Rahman, Rahman, Das, Rahman, Kabir, 2012).
These symptoms include; fatigue, nausea, vomiting, weight loss, pain with bloating, swelling in the legs, feet, and ankles, itching, spider angiomas, muscle cramps, jaundice and acute liver failure. The disease is not limited to just liver related symptoms; it also affects the central nervous system and mental health. When copper builds up in the eyes it forms Kayser-Fleischer rings. The rings appear rusty brown around the iris and corneas (Schilsky,
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First there is medication. Two medication options are Chelating Agents and Zinc. Chelating agents remove copper through the kidneys and Zinc blocks the absorption of copper from food by the digestive tract. Penicillamine, the original chelating agent, binds copper and creates a water mixture that is passed through urine. Trientine, also a chelating agent, is less toxic than Penicillamine and doctors preferred choice. Zinc Acetate is the most common Zinc treatment and its slower acting than chelating agents. It is more commonly used for pregnant patients, patients with no symptoms, or those who have shone improvements (Ala et al., 2007). A doctor will also recommend a change in diet, limiting an intake of foods high in copper such as shellfish, liver, mushrooms, nuts, and chocolate. As well as tap water that runs through copper pipes or comes from wells. In extreme conditions, a liver transplant may be necessary. Wilson’s disease no matter when it is discovered is a lifelong disorder that requires ongoing treatment; there is no cure (Schilsky,