Prader Willi Syndrome Essay

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Prader-Willi Syndrome, also known as Prader-Labhart-Willi Syndrome or PWS, is a rare genetic disorder that has many physical and mental effects. It was first described in 1956 by Swiss doctors Andrew Prader, Alexis Labhart, and Heinrich Willi. They examined nine children and found that in infancy, when Prader-Willi Syndrome is usually diagnosed, babies will exhibit low muscle tone and strength, difficulties feeding because of a weak sucking reflex caused by underdeveloped muscles, and delayed mental and physical development (Foundation for Prader-Willi Research, 2014). It is best known for causing consistent hunger in those affected. This symptom appears between the ages of three and eight. An affected individual will feel hungry no matter …show more content…

Human Growth Hormone (HGH) is commonly administered to affected children. Approved by the Federal Drug Administration for use in people with Prader-Willi Syndrome in 200, HGH is effective in increasing height, muscle mass, bone density, and energy level. HGH also can decrease body fat, improve weight distribution, and has been linked to better development and behavior . Sex hormone replacement therapy is also often used to aid sexual development and reduce the risk of osteoporosis (Nordqvist, C., 2016). Behavioral issues associated with Prader-Willi Syndrome, like obsessive-compulsive tendencies and extreme stubbornness, can be treated with psychiatric therapy and sometimes antipsychotic drugs. Sleeping problems can be treated with medications, too. Daytime sleepiness can be treated with modafinil, a wake promoting drug. Breathing issues, like sleep apnea, that occur during sleep can be resolved with a Continuous Positive Airway Pressure (CPAP) machine (Forster, J., 2014). Back braces and surgery are used to treat scoliosis, which is more common in people with Prader-Willi Syndrome than the general population. While some of its symptoms can be treated, there no medications that alleviate the main effect of the syndrome: the constant hunger. Because of this, affected individuals often live in group homes where access to food is restricted for the rest of their lives after they leave the homes of their parents (Foundation for Prader-Willi Research, 2014). If obesity can be prevented and other symptoms of Prader-Willi Syndrome can be controlled, the lifespan of individuals with it is close to normal (Nordqvist, C., 2016), which is about 79 years in the United States (The World Bank, 2016). However, close medical supervision is recommended after age 40 because mental and physical issues become more common after this age (Sinnema, M., Schrander-Stumpel, C. T., Maaskant, M. A., Boer, H., & Curfs, L. M.,

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