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PWS MSU Denver

PWS
by

Corbett Kesler

on 10 December 2014

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Transcript of PWS MSU Denver

Prader-Willi Syndorme
What is Prader-Willi Syndrome
How does it affect Infants?
How does it affect children in their early childhood?
It causes children to have low muscle tone, short stature if not treated with growth hormone, incomplete sexual development, and a chronic feeling of hunger. This chronic feeling of hunger can be life threatening and cause obesity in children.
Prader-Willi Syndrome (PWS) is a disability caused by a chromosomal error: autosomal type. This disability causes obesity in children because of an abnormality in the 15th chromosome. It is a disorder present at birth that results in a number of physical, mental and behavioral problems.
Specific symptoms are:
-Small hands and feet
-Poor to little muscle tone
-Distinct facial features including a narrow face, almond shaped eyes, narrowing of the head at the temples, a turned-down mouth and a thin upper lip
-Failure to thrive, feeding may be difficult because they may have poor sucking reflex due to a decrease in muscle tone
-Lack of eye coordination
-Generally poor responsiveness
•Nearsightedness (myopia)
•Light skin compared with other family members
•High pain tolerance
Children can have:
-Food craving and weight gain
-Undeveloped sex organs
-Poor growth and physical development
-Learning disabilities
-Delayed motor development
-Speech Problems
-Behavior problems
-Sleep disorders
-Scoliosis
What happens in Prader-Willi syndrome
PWS is a genetic disorder that occurs in approximately one out of every 15,000 births.
PWS affects males and females with equal frequency and affects all races and ethnicities.
PWS is recognized as the most common genetic cause of life-threatening childhood obesity.

Obesity-related complications
•Type 2 diabetes. Type 2 diabetes is high blood sugar level due to your body's inability to use insulin efficiently. Insulin plays a vital role in making blood sugar (glucose) — your body's fuel — available to your cells.

•Heart disease and stroke. They are more likely to have high blood pressure, hardened arteries, high cholesterol, and other factors that can lead to heart disease and stroke.

•Sleep apnea. This sleep disorder is characterized by pauses in breathing during sleep. The disorder can cause daytime fatigue, high blood pressure and, rarely, sudden death. People with Prader-Willi syndrome may have sleep apnea even if they aren't obese, but obesity can worsen sleep problems.
Complications of hypogonadism
This is a condition in which your sex organs don't secrete sufficient amounts of the sex hormones testosterone (males) and estrogen and progesterone (females). These may include:
•Sterility :most people with this disorder are unable to have children (sterile).
•Osteoporosis. Osteoporosis is a condition characterized by weak and brittle bones, which can break easily. People with Prader-Willi syndrome are at an increased risk of developing osteoporosis because they have low levels of sex hormones, which help maintain strong bones.
How to deal with Prader-Willi
Parents:
•Pick up your child carefully. They have poor muscle tone, which increases their risk of slipping between your hands when you lift them under their armpits.
•Take steps to help prevent binge eating. A low-calorie diet is essential to keep your child from becoming overweight. Shop smart and try to keep store food out of your child's reach. Lock pantries, refrigerators and cupboards. Use small dishes for serving meals.
•Supplement your child's diet. Ask your doctor if supplemental vitamins or minerals are necessary to ensure balanced nutrition.
•Schedule appropriate screening tests. Talk to your doctor about how often you need to have your child screened for complications of Prader-Willi syndrome, including diabetes, osteoporosis and an abnormally curved spine (scoliosis).
Educators:
Having any food anywhere in the classroom
will impede their ability to concentrate and learn.
Snack items must be kept in another area,
such as another classroom or the office.

Treatments
-Good infant nutrition
-Growth hormone treatment
-Sex hormone treatment
-Healthy diet
-Overall development
-Mental health care
Cognitive Factors:

Individuals with PWS are more prone to suffer from psychotic symptoms and psychiatric disorders than the general population.

Weight appears to be a factor in the degree in which said symptoms manifest themselves. Thinner children are sometimes noted to manifest more acute maladaptive behaviors and symptoms of psychosis than heavier children. It is unclear if the weight factor is responsible for this effect or if the extra external controls to keep the weight down are exacerbating the manifestation of maladaptive and psychotic symptoms.


Education
Classroom setting
Clear Expectations
Visual cues
Consistency
Positive Praise
Concrete
Keep instruction simple
Limit repetitions
Foreshadow
Positive Support Model
Ask for the behavior you want to see
Be Specific
Give Choices
Replacement behaviors or calming rituals
Practice replacement behavior
do NOT attempt to physically take food away
Visual schedules
Timer
One direction at a time
Common Behaviors at school
Anxiety
Rigidity
Repetitive
Stubbornness
Oppositional
Aggressive
Laurie Anderson, Courtney Berry, Dawn Besocke,
Corbett Kesler, Gloria O

References
http://www.uptodate.com/contents/epidemiology-and-genetics-of-prader-willi-syndrome
PWS is a complex genetic condition that affects many parts of the body.
In infancy, weak muscle tone (hypotonia), feeding difficulties, poor growth, and delayed development.
Beginning in childhood, develop an insatiable appetite, which leads to chronic overeating (hyperphagia) and obesity.
May develop type 2 diabetes mellitus (the most common form of diabetes).
http://www.nhs.uk/conditions/prader-willi-syndrome/Pages/Introduction.aspx
Early Intervention
Strategies for learning and teaching. Special education support service. Retrieved from
http://www.sess.ie/categories/assessed-syndromes/prader-willi.com.
2014.Positive behavior strategies. Prader-Willi syndrome association. Retrieved from
http://pwsausa.org.
Cognitive and psychiatric issues in Prader-Willi syndrome. Retrieved from
http://www.medicalhomeportal.org/issue/cognitive-and-psychiatric-issues-in-prader-willi-syndrome

http://ghr.nlm.nih.gov/condition/prader-willi-syndrome
http://www.fpwr.org/about-prader-willi-syndrome/
http://www.ipwso.org/#!what-is-prader-willi-syndrome/clxd
Transition Services
Transition services are aimed at providing students and their families with the practical and experiential skills and knowledge that will assist in a successful transition to adult life.

employment
post school activities
community experiences
activities of daily living

Behavioral
Numeracy Curriculum
Students with PWS find it difficult to grasp abstract concepts.
Breaking down concepts into small steps.
Use real life applications will more successful with a student with PWS.
Literacy Curriculum
Generally reported to be a strength.
The reciprocity of reading and writing should be emphasized.
What one reads, one can also write.
Writing might be affected by poor muscle development.
PWS students have been found to learn words by sight rather than by phonic analysis.
Rigid Thinkers
Accommodations
Break tasks down tasks into manageable parts
Always have Plan A and Plan B
Use visual schedules
Give positive instructions
Heavy curriculum lifting in the morning.
Students with PWS need to receive and store information in an orderly manner.
Have difficulty transitioning between activities and become fixated on an idea to the point of overshadowing their learning.
Assistive Technology
Visual Schedules
Social Stories
Simple Speech Generating Device- Voice output device that range from speak a single message to device with multiple cells.
Chatbox
GoTalk
Speech to text
Dragon
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