Chelsi Anderson Brad Solberg Prader Willi Syndrome Chelsi Anderson Brad Solberg

Definition Rare disorder present at birth that results in a number of physical, mental and behavior problems (Mayo Clinic)

Causes of Prader Willi Caused by an error in a gene or genes Particularly in the region of chromosome 15 Chromosome15 is missing---70% of cases Two copies of the chromosome 15-- 25% of cases Or defect of chromosome 15--- Less than 5% Due to the deficiency of chromosome 15, the hypothalamus is defected. Hypothalamus controls hunger & thirst reflexes, sexual development, and stunted growth

Characteristics of Prader Willi Constant hunger that is apparent early in the person’s life Eat constantly Difficulty controlling weight Often suffer from obesity Notice: narrow temples, thin upper lip, almond shaped eyes.

Stages Of PWS Failure to Thrive Birth to 2-5 Thriving Stage 2-5 through life Low birth rate Weak Muscle Tone Feeding difficulties Small hands and feet Development delays Excessive Sleepiness Underdeveloped genitals Increased appetite and weight control problems Behavior problems Learning problems, 60% having cognitive dis. Fine and gross motor delays Social skills deficit

Symptoms of Prader Willi Food cravings Underdeveloped sex organs [produce low hormones] Poor physical development Increased risk of learning disabilities Speech problems Behavioral problems [very stubborn when denied food] Sleep disorders [sleep apnea] Scoliosis [develop an abnormal curvature of the spine]

Medical Conditions Related to Prader Willi Type 2 Diabetes Heart Disease Strokes Sleep apnea Sterility Osteoporosis

Treatments Good nutrition Growth hormone treatment Overall development Physical activity Mental health care

Physical Activity: Treadmills Promotes Cardiovascular Endurance Can help lose or maintain weight Stamina—people with PW tend to get tired easily Incorporate goal setting for the student

Physical Activity: Pedometer Could be used in daily PE class to monitor steps, set goals and record work effort They could benefit from this because of the “number” it gives them Instructors can provide a number for them to reach in the amount of time they they have them in class

Physical Activity: Nutritional Puzzle Relay Educating children as well as keeping them active Promotes cardiovascular activity Promotes student collaboration Promotes strategic actions

Importance of Leisure Activities Most with PW have difficulty participating in competitive sports Leisure activities gives the student a chance to enjoy themselves with little or no competition Promote them to continue to be active for a lifetime

Instructional Strategies Because of the hypothalamus is effected, they have a poor short term memory Their long term memory, however, is extremely strong Keeping instructions clear, concise and to the point May have to do lots of reminding Encouragement

Lessons for teachers Never have food in the classroom (distraction) Monitoring food is a medical necessity (have a snack plan) http://www.pwsausa.org/Educator/sampleletter.htm No homework- children may understand the information in the environment it is taught but struggle outside that environment Lack of structure and consistency can lead to anxiety Helpful or empathetic approach to anger is better than a threatening approach. Students require instruction on appropriate ways to handle anger

Physical Activity Modifications: Soccer As far as skill level, keep them with others at the same level as theirs. Smaller playing field for those with a low level of ability Once they become unsuccessful, it’s going to be difficult to rekindle the fire again

Physical Activity Modifications: Track & Field Let them choose what 3 activities they would like to practice for the day, but they must have at least 2 running events. Do not time them unless they want to be timed.

Community Recreation & Competitive Sports YMCA Lion’s Club Summer School Opportunities Swimming at local pool

RESOURCES http://www.aafp.org/afp/2005/0901/p827.html http://www.pwsausa.org/Educator/General%20Info%20%20Overview.pdf http://www.mayoclinic.com/health/prader-willi-syndrome/DS00922 http://www.pwsausa.org/faq.htm http://www.pwsausa.org/syndrome/index.htm http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002572/