Prader-Willi Syndrome at

What is Prader-Willi Prader-Willi

Infancy
Even before infancy, PWS begins its first phase,
phase 0. This phase occurs in utero and is
Syndrome?
Syndrome
characterized by decreased fetal movements, birth • PWS is a complex genetic disorder that
weight and length. affects appetite, metabolism, cognitive What We Need to Know as Teachers
function and behavior
The phase connected with infancy is known as the and Parents
first phase. In this phase, the infant is hypotonic • Individuals with PWS may often have
and not obese. First phase consists of two sub- low muscle tone, short stature,
phases: incomplete sexual development, cognitive
disabilities, behavioral problems, feelings
1. Sub-phase 1a (median age range = 0-0.75
of insatiable hunger and a slowed
years) is characterized by poor appetite, feeding
metabolism that can lead to excessive
difficulties, and failure to thrive.
eating and/or life-threatening obesity
2. Sub-phase 1b (median age range = 0.75-2.08
years) occurs when the infant is growing
steadily along the normal growth curve which
is associated with improved appetite. PWS Basic Facts
The last phase associated with infancy is the second
main phase occurring between 18-36 months of • PWS can occur in any family and is
age. It is in this phase that weight starts to increase not preventable
and reaches normal percentiles according to the
• PWS is due to a lack (or deletion) of
normal weight curve. It is also made up of two sub-
several genes on one of an individual’s
phases:
two chromosome 15’s – the one that is
1. Sub-phase 2a (median age range = 2.08-4.50 normally contributed by the father
years) is when the child’s weight increases to a
normal range without significant caloric • Approximately 12,000 to 15,000 people
increase. During this phase, the children do not have PWS Patricia Breault
have an increased appetite or increased interest
• PWS is the most common known EDS 521
in food.
genetic cause of life-threatening Dr. Simms
2. Sub-phase 2b (median age range = 4.5-8.0 childhood obesity
years) occurs when the child has abnormally
increased, but not necessarily insatiable, • PWS is a multiphase syndrome
appetite which leads to overweight/obesity if
the diet is not appropriately regulated.
 PWS in Early
Childhood and
Adulthood
PWSA (USA) provides the Wyatt Special Education
Advocacy Training for teachers who may encounter
Once children reach early childhood,
they enter the next phase known as the
third phase (median age of onset is 8.0
years and lasts into adulthood).
This phase is characterized by an
insatiable appetite/drive to eat,
accompanied by intense or relentless
food-seeking.
The third phase is often known as the
classic phase because it is the phase most
people typically associate with PWS.
Research indicates that this phase may
appear as early as 3 years of age or as late
as 15 years of age.
The last phase associated with PWS is
known as the fourth phase which may
occur later in adulthood.
This phase occurs when an adult
individual who was previously in phase
three no longer has an insatiable appetite
and can feel full.
Family members or caretakers note a
significant improvement in appetite and
weight control in the last phase.
However, phase four only occurs in a
minority and most adult individuals with
PWS will remain in phase three.
What Teachers
Need to Know
students with PWS.
Module 1 – The Power of Inclusion -
Includes highlights such as accommodations
and intentionally organizing and structuring
inclusion in a classroom
Module 2 – Responding to Behavioral
Challenges – Includes a discussion of
Functional Behavioral Assessments and
Behavior Intervention Plans
Module 3 – Effective Advocacy
Module 4 – PWS Specific School Challenges
and Solutions – Includes a discussion of food
security, supervision, and school staffing
Additional Teacher Strategies for Students
with PWS who have:
• Anxiety (teachers should reframe, redirect,
and use calming rituals and visual schedules)
• Difficulty transitioning (teachers should use
timers, count downs, and natural extensions)
• Great expectations (teachers should set up
realistic expectations, get their facts straight,
and should not tell the student too much-too
soon)
• Issues with perseveration (teachers should use
concrete reminders, tally sheets, and
visual/verbal reminders)
• Oppositional behaviors (teachers should get
cooperation/help and compromise-not
negotiate)
• Aggressive behavior (teachers should read
body cues, validate feelings-do not excuse
behavior, and avoid eye contact but stay calm
and confident)
What Parents
Need to Know
• Appetite suppressants/diet
medications do not work consistently
for individuals with PWS.
• Some promising drug studies require
individuals with PWS to have an
extremely low-calorie diet all their
lives and must have their environment
designed so that there is very limited
access to food.
• Parents should appropriately manage
their child’s access to food. This may
mean locking the door to the kitchen,
locking cabinets containing food,
and/or locking the fridge.
• As adults, individuals with PWS can
control their weight best in a
supportive living home designed
specifically for people with PWS,
where food access can be restricted
without interfering with the rights of
those who don’t need such restriction.
As a parent, where can I get more
information on PWS?
Parents can contact the Prader-Willi
Syndrome Association (USA) at 1-800-
926-4797 or email info@pwsausa.org
For new parents, the PWSA (USA)
provides The Package of Hope, which
contains a variety of publications to help
answer questions about nutrition, medical
concerns, and the use of growth hormone
as a treatment for PWS.
 References

Prader-Willi Syndrome Association (USA). (2016). About Prader-Willi Syndrome. Retrieved from
https://www.pwsausa.org/about-PWS/#1443576713730-8c3e4e0b-b14f
Prader-Willi Syndrome Association (USA). (2016). Basic Facts. Retrieved from https://www.pwsausa.org/basic-
facts/
Prader-Willi Syndrome Association (USA). (2016). FAQ’S. Retrieved from https://www.pwsausa.org/faqs/
Prader-Willi Syndrome Association (USA). (2016). Parader-Willi Syndrome Diagnosis. Retrieved from
https://www.pwsausa.org/new-diagnosis/
Prader-Willi Syndrome Association (USA). (2016) Prader-Willi Syndrome Teacher Tips. Retrieved from
https://www.pwsausa.org/school-issues/
Prader-Willi Syndrome Association (USA). (2016). Wyatt Special Education Advocacy Training (2017-2018).
Retrieved from https://www.pwsausa.org/wseat-webinar-series/

Patricia Breault
EDS 521
Dr. Simms
PWS Brochure