Behavior Characteristics
of
Prader-Willi
Syndrome
Cindy Stolp
Prader-Willi syndrome (PWS)
PWS is a chromosomal
disorder from a
deletion or disruption
of genes in the
proximal arm of
chromosome 15
Deletion 70%
Chromosome 15
Maternal Disomy – 25%
Imprinting defect – 5%
(pwsausa.org)
Prader-Willi Syndrome
• 1 in 12-15,ooo
• Rare disorder
• But
• PWS is the most
common genetic cause
of obesity
• All races
• Both sexes
(pwsausa.org)
Diagnosis
• Diagnosis often
delayed until early
childhood.
• Genetic testing detects
nearly 100% of
Prader-Willi
Syndrome
Newborns
•
•
•
•
•
Difficulty feeding
Poor sucking reflex
Failure to thrive
Hypotonia
Early developmental
milestones are delayed
After the
st
1
year
Victor 9 months
• Difficulty with feeding
transitions into
• hyperphagia
Victor
24 months
Treatment
What doesn’t work
• Appetite suppressants
What has worked
• Extremely low-calorie
diet (their entire life)
• Limited access to food
Prader-Willi Food Pyramid
• Typical person – 1600 – 2800 calories per day
• Person with PWS – 800 – 1200 calories per day
• Vegetables make up the bulk of the
recommended diet
Common Facial Characteristics
• Almond Shaped Eyes
• Eye Abnormalities
(Esotropia & myopia)
• Thin upper lip
• Narrowing of the
temples
Common Characteristics
•
•
•
•
Short stature
Relatively small hands
Relatively small feet
Between 1-6 progress
into development of
morbid obesity
Recommended Team Members
Primary care physician
Ophthalmologist
Pediatric endocrinologist
OT
PT
Speech Therapist
ECSE & Special Education
Behavior Problems
•
•
•
•
Temper tantrums
Stubbornness
High pain threshold
Sleep disturbances
• Obsessive compulsive
behaviors
• Repetitive thoughts
• Verbalizations
• Collecting & hoarding
possessions
• Picking at skin
Need to Seek Food
• Garbage
• Leftovers from
cafeteria trays
• Scraps off the floor
• Frozen food
• Stealing to obtain food
• Dangerous
• High threshold for
pain
• Decreased ability to
vomit
Need for Routine & Consistency
Strategies
•
•
•
•
Visual schedules or lists
Discuss changes in schedules or staff
Provide praise for flexibility
Breakdown procedures into concise steps
Obsessive Thinking
Strategies
• Less is best – give smaller amount of work
at one time
• Don’t give more info than necessary
• Use”strategic timing” – schedule an activity
they have difficulty ending right before
snack or lunch
Emotional Control
• Strategies
• Over stimulation in crowded hallways – enter & exit
school in a less populated area
• At the beginning of the day, review the daily schedule to
help decrease anxiety
• Encourage communication
• Listen carefully
• Teach, reteach & practice frustration coping strategies
• Don’t try to reason with them when out of control
Food Cravings
• Educate all people working with the student
• They will lie to get food, “I didn’t have breakfast
this morning” – check with parents
• Avoid letting them have money –
– money buys food
•
•
•
•
Don’t delay snacks
Limit the visibility of food
Be aware of smells – popcorn
Avoid using food as a reward or incentive
Poor Stamina
• Strategies
•
•
•
•
Send them on an errand – take a walk
Schedule high energy activity after lunch
Deep breathing activities – balloons
Schedule rest times or quiet activities
Scratching &
Picking Skin
• Strategies
• Keep hands busy – playdoh, hand held games
• Keep nails short
• Apply lotion liberally – keeps the skin moist
and slippery
Security
• Baby monitors
• Security system with
alarms
• Motion detectors
• 160 degree mirrors
• (see what’s going on behind you)
Recent research
• The brain’s pleasure center
• 3x as much neurotransmitter
gammaaminobutyric acid in their blood
• Neurotransmitters inhibit dopamine
I’m still hungry
1999 U.S. Supreme Court
• “Developmentally disabled people must be
given the choice to live in the community if
they can be supported there.”
• www.pwsaa.org
Global Delays
• Learning disabilities
are always present
• Ranging from
• Low normal
intelligence
• To mental retardation
• Mainstreamed into
classroom
• Need additional
physical activity
periods
Perspective on Behaviors
(Teresa Kellerman)
• Appear to be
Lazy & lack motivation
Manipulative, Lying & stealing
Lack of empathy & Lack of conscience
Stubborn
Temperamental
Controlling