PRADER - WILLI SYNDROME

WHAT IS PRADER - WILLI SYNDROME?

Prader-Willi Syndrome is a genetic disorder and in most cases it is not inherited. It affects the individual's appetite, growth, metabolism, cognitive function and behavior. Prader-Willi syndrome is caused by the loss of function of genes in a particular region of chromosome 15. This occurrence is due to the absences of genes on the individuals two chromosome 15 which usually occurs on the chromosome that is received from the father. In about 70 % of cases the critical genes are lost from the chromosome and in 25% of cases the individual is missing the chromosome from the father completely and instead has two chromosomes 15 from the mother. (This is called uniparental disomy it is when a person receives two copies of a chromosome, or part of one, from one parent and does not receive any chromosomes form the other.) In the remaining 5% the entire chromosome 15 comes from the father but the imprinting on the chromosome is faulty. About 1 in every 10,000 to 30,000 people word wide is estimated to be living with Prader-Willi Syndrome. Prader-Willi Syndrome symptoms can also occur after birth if the hypothalamus portion of the brain is damaged due to injury.

STATISTICS
350,000- 400,000 people worldwide live with Prader- Willi Syndrome Affects males and female with equal frequency There is no known cure for Pader-Willi Syndrome Life expectancy can be normal if weight is controlled The risk of a family having another child with Prader-Willi Syndrome varies widely from zero to 50 %. Affects all races and ethnicities

SYMPTOMS AND BEHAVIOR

Common symptoms of Prader-Willi Syndrome are: low muscle tone, short stature, small feet and hands compared to the rest of the body, incomplete sexual development, slow metabolism, and cognitive disabilities. They also feel an involuntary and uncontrollable chronic feeling of hunger which is why many of the individuals who have Prader-Willi Syndrome are overweight or obese. Also individuals with Prader-Willi Syndrome tend to be stubborn, argumentative, and do not like changes. Other behaviors include skin picking, tantrums, obsessive compulsive behavior, and sleep disturbance. performance, increase height and growth rate, and increase hand and foot size to normal proportions. It also helps to increase muscle development and helps control weight of individuals with Prader-Willi Syndrome.

TREATMENTS
The best way to diagnose Prader-Willi Syndrome is to get genetic testing. Even though there is no known cure for Prader- Willi Syndrome there has been a new treatment released which can help with symptoms. It is a growth hormone treatment. In recent studies it has been shown to help improve physical

"We worry about what a child will become tomorrow, yet we forget that he is someone today." Stacia Tauscher

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CURRENT RESEARCH
In November of 2009, approximately 65 physicians

CURRENT LAWS
Individuals with Disabilities Act (IDEA) - entitles all children to a free public education. The Americans with Disabilities Act Amendments Act (ADAAA) - gives a broader coverage of what is a disability. Rosa's Law - replaced the phrase "mental retardation" with "intellectual disability" in laws. Section 504 - anyone with a disability will not be denied benefits or services.

and scientists from eight different countries gathered in Bethesda, Maryland for the Prader-Willi Syndrome Research Strategy Workshop. Also in 2013 the Prader-Willi Syndrome Research Program outlined research priorities and initiatives that will pursue advancements in treatment and research of Prader-Willi Syndrome.

FAMILY RESOURSES
Prader-Willi Syndrome Association(USA) 5700 Midnight Pass RD Sarasota, FL 34242 800-926-4797 www.pwsausa.org Mt. Washington Pediatric Hospital 1708 West Roger Ave. Baltimore, MD 21209 410-367-2222 www.mwph.org Division of Special Edu./Early intervention Maryland State Department of Education 200 West Baltimore Street Baltimore, MD 21201 410-767-0863 www.marylandpublicschools.org

"No kid is unsmart. Every kid's a genius at something. Our job is to find it. And then encourage it." Robin Sharma

TEACHING STRATEGIES
Consistency of a routine is very important for a child with Parder-Willi Syndrome and if there is a change in that routine the child needs to be warned in advance. Many children with Prader-Willi Syndrome tire easily so short lesson plans are needed and new materials are best introduced in the morning. When dealing with behavior issues a positive reward system works best. Visual cues are needed because many students with Parder-Willi Syndrome have poor auditory processing skills. Repetition and reteaching learning objectives is important for children with Prader-Willi Syndrome who have poor short term memory.

Computers with Alternative Keyboards- keyboards that make it easier to work on a computer for people with disabilities. Smart Boards- an interactive white board that uses touch detection and is used for visual learning.

REFERENCES
Dimitropoulos, A., Feldman, B., Alan, H. (2012). Social Responsiveness and Competence in Prader-Willi Syndrome. Springer Science and Business Media, 43,103-113. Prader-Willi Syndrome Association. (2006, March).PraderWilli Syndrome Fact Sheet. Retrieved from http://www.pwsausa.org/awareness/FactSheet.pdf Raskind,M., Stanberry.K.(n.d.).Assistive Technology for Kids with LD: An Overview. Retrieved February 1, 2014, from http://www.greatschools.org/specialeducation/assistive-technology/702-assistivetechnology-for-kids-with-learning-disabilities-an-overview.gs. Reddy,L. A.,Pfeiffer,S.I.(2006).Behavioral and Emotional Symptoms of Children and Adolescents with Prader-Willi Syndrome. SpringerScience and Business Media, 37,830-839. Sandie, W. B. (2013). Diagnosis and Management of Prader-Willi Syndrome. Learning Disability Practice , 16(5), 26-28.

TECHNOLOGY INNOVATIONS
Sounding Board- an iPad touch app that allows students to turn the device into a story board communicator. This helps students with writing and communication disabilities to write easily and with a limitless supply of symbols.

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