Prader-Willi Syndrome

Learning Objectives
A. Identify and understand the causes of Prader-Willi B. C.

D. E.

Syndrome. Identify characteristics and behaviors of individuals with Prader-Willi Syndrome. Describe the recourses, support services and technology that is available to individuals with this syndrome. Identify appropriate teaching strategies. Discuss current research findings and laws that can help individuals with Prader-Willi Syndrome.

Prader-Willi Syndrome

What is Prader-Willi Syndrome?

What causes individuals to be born with this syndrome?

Facts About Prader-Willi Syndrome

Prader-Willi Syndrome is a genetic disorder and in most

cases it is not inherited. 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.

Facts Continued
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. In the remaining 5% the entire chromosome 15 comes from the father but the imprinting on the chromosome is faulty. It affects the individual's appetite, growth, metabolism, cognitive function and behavior. About 1 in every 10,000 to 30,000 people word wide is estimated to be living with Prader-Willi Syndrome

Symptoms
Low muscle tone

Short stature
Small feet and hands compared to the rest of the

body Incomplete sexual development Slow metabolism Cognitive disabilities Uncontrollable feeling of hunger

Behaviors of Individuals with Prader-Willi Syndrome

Stubbornness

Argumentative
They do not like changes Skin picking

Tantrums
Obsessive compulsive behavior Sleep disturbance

Over eating

Testing for Prader-Willi Syndrome

The best way to diagnose Prader-Willi Syndrome is to get genetic testing.

Treatments
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 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.

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 Prader-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

Current Research
In November of 2009, approximately 65 physicians

and scientists from eight different countries gathered in Bethesda, Maryland for the Prader-Willi Syndrome Research Strategy Workshop.
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 Resources

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

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. with "intellectual disability" in laws. denied benefits or services.

Rosa's Law - replaced the phrase "mental retardation"

Section 504 - anyone with a disability will not be

Teaching Strategies
Consistency of a routine is very important for a child with

Prader-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 PraderWilli 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.

More Information
For more information on teaching strategies watch this informative video. https://www.youtube.com/watch?v=Y5LhSePDvqk

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. Computers with Alternative Keyboardskeyboards 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.

Check Your Understanding

What causes Prader-Willi Syndrome?

What are some symptoms and behaviors of Prader-

Willi Syndrome? Who can be affected by Prader-Willi Syndrome? Can you think of other teaching strategies that could be beneficial for students with Prader-Willi Syndrome? What other technology is available to help students with Prader-Willi Syndrome?

Prader-Willi Syndrome

ANY QUESTIONS ?

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).Prader-Willi 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-assistive-technology-for-kids-with- learningdisabilities-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.