What is it?
Prader-Willi Syndrome (PWS) is a genetic abnormality of chromosome 15 and involves two typical stages. The first stage is referred to as the ‘failure to thrive’ phase and is characterized in infancy by a weak and floppy body, lethargy, and having a poor sucking reflex, resulting in the need for many to receive nutrients through a feeding tube. According to the Utah Prader-Willi Syndrome Association, these children often have fairer-hair and skin than their siblings and a good-natured and easy-going temperament. It usually takes these babies twice as long to learn to roll over, sit up, crawl, walk and talk.
The second stage is characterized by excessive obesity due to an insatiable appetite (hyperphagia) and over-eating. “The hypothalamus of the brain does not register the sensation of being full properly and this causes the child with PWS to have an uncontrollable urge to eat all the time” (Mason, 2011). This stage generally starts after the age of 2 and before 6, and will last for the rest of their lives. If a person with PWS is left unsupervised, it is possible that their high pain tolerance and appetite will cause them to over-eat so drastically that their stomach could rupture. Individuals with PWS also develop sex hormones at a very low level, and as a result may result as stunted growth, delays in cognitive and oral development, or other disabilities.
Known Causes/National Occurrence Rates
Although much is left undiscovered about the causes of this particular syndrome, what is known is that it is generally (about 70%) a result of some loss of unidentified genes on chromosome 15 given by the father (Goldstone & Holland, 2008).
The occurrence rate of this syndrome is one in every 12 to 15 thousand, which is relatively low. However, it is one of the most common disorder seen by geneticists. According to the Utah Prader-Willi Syndrome Association “approximately 61 people in Utah are diagnosed with PWS.” Based on the national average, 80% of people who have the syndrome are left undiagnosed (Mason, 2011).
Although this is a very difficult disorder to manage effectively. It is absolutely possible. There are three specific strategies that have been researched and tested over the years that will help individuals with PWS successfully navigate into their later years of high school and into adulthood. These strategies involve creating and upholding an effective behavior management plan, planning and preparing current and future educational goals and safeguards, and providing prevocational and vocational training when students transition out of the school setting. This consistency, close monitoring, and an effective system can serve to offset many of the common behavior problems such as moodiness, impulsivity, non-compliance, tantrums, and more.
In order to provide an appropriate education for students with PWS, classroom placement should be based on intellectual functioning and behavioral needs of the individual (Hanchett & Greenswag, 2010). Classroom aids, self-contained classrooms, and outside consultation are all useful and common strategies for helping students with Prader-Willi syndrome to succeed in their education.
It is essential, as with any transition program, that prevocational training is provided for the individual according to their abilities and interests. Individual Transition Plans (ITPs) are mandated by law to “help prepare the student for appropriate vocational, residential, and recreational options in young adulthood” (Hanchett & Greenswag, 2010). Due to the nature of the disability, on-site community vocational settings are inappropriate when they include food-related or centered work. Even with continuous and close supervision, individuals with PWS will take every opportunity possible and may go to many extreme lengths to get food. It is critical that transition programs include instruction in social skills and community living//success skills, always keeping in mind the need for these individuals to be in a completely supervised setting.
Goldstone, A.P., & Holland, B.B. (2008). Recommendations for the Diagnosis and Management ofPrader-Willi Syndrome. The Journal of Clinical Endocrinology & Metabolism, val. 93 no. II. Retrieved from http://jcem.endojournals.org/content/93/11/4183.full
Hachett, Jeanne, & Greenswag, Louise (2010). Health Care Guidelines for Individuals with Prader-Willi Syndrome. The Scientific Advisory Board of the Prader-Willi Syndrome Association (USA). Retrieved from http://www.pwsausa.org/
Mason, Debbie (2011). Management of Prader-Willi Syndrome, Basic Information and Medical Concerns, Third Edition. Retrieved January 29, 2012
Prader-Willi Syndrome (n.d.). Retrieved January 29, 2012 from the Prader-Willi Syndrome Wiki:http://en.wikipedia.org/wiki/Prader-Willi_syndrome
Prader-Willi Association USA http://www.pwsausa.org/