What is Prader-Willi Syndrome?
Prader-Willi Syndrome (PWS) is a genetic spectrum disorder that results from an abnormality on the 15th chromosome. This syndrome:
- Is congenital and can affect anyone;
- Can be acquired due to brain trauma;
- Can affect appetite, growth, metabolism, cognitive function, and behavior;
- Is rare but one of the most common conditions seen in genetic clinics;
- Is life-threatening;
- Has no know cure.
What are the common characteristics of PWS?
Childhood & Beyond
Common characteristics observed in individuals with Prader-Willi Syndrome throughout childhood and beyond include:
- Developmental delays
- Speech and language delays
- High pain tolerance
- Challenging behaviors
- Short stature
- Childhood obesity due to insatiable hunger
- Skin picking
- Excessive daytime sleepiness
- Altered temperature regulation
What causes PWS?
The symptoms of Prader-Willi Syndrome are likely due to dysfunction of a portion of the brain called the hypothalamus, though it is not yet clear how the genetic abnormality causes hypothalamic dysfunction.
Who is affected by PWS?
It estimated that there are 350,000 to 400,000 individuals worldwide living with Prader-Willie Syndrome. PWS can be found in both sexes and across all ethnic groups. The prevalence of PWS varies across developed nations:
- In the US, the prevalence of PWS is 1 in 16,000-25,000.
- In Sweden, the prevalence of PWS is 1 in 8,000.
- In England, the prevalence of PWS is 1 in 52,000.
How is PWS diagnosed?
The criteria for diagnosing PWS was developed by 7 clinicians experienced with the syndrome in coordination with international experts. Diagnosis is based on clinical findings of symptoms such as hypotonia, obesity, mild intellectual disability, and behaviors relating to food and eating. Diagnostic criteria change with the age of the individual; there is one set of criteria for age 0-36 months, and another for age 3 years and above.
There are no known biological markers for PWS, but research is ongoing.
What is the impact of PWS on education?
- Difficulty with short term memory and sequencing;
- Cognitive difficulty with language combined with speech apraxia, which makes communication difficult;
- Challenging behaviors including tantrums, stubbornness, compulsivity, and difficulty with changes in routine.
What are the strengths of students with PWS?
Despite struggling with social frustration that often causes conflict, individuals with PWS have many positive social traits:
- Friendly and sociable with peers and adults
- Good sense of humor
- Kind, caring, and loving
- Like to be helpful and take messages
These individuals also have many positive character traits that can lead to success in school:
- Determined to succeed
- Good general knowledge and interest in the world around
Students with PWS tend to exhibit success in:
How does PWS impact families?
Children with PWS are typically happy and loving and make for great family members, but they do exhibit very challenging behaviors. Children with PWS often seem obstinate and can be aggressive particularly when it comes to food. As such:
- Parents of children with PWS have been surveyed as having particularly high levels of stress relative to parents of children with other disabilities.
- Non-affected siblings have very high incidence of PTSD.
PWS is unique and all encompassing, and solid understanding of its impacts is crucial to best treatment of children with the syndrome. It’s important for family and friends to understand that challenging behaviors and cognitive deficits are a result of the syndrome and not of the child’s personality or intentional spite.
Behavior and food management strategies are necessary in the home to limit:
- Stress caused by tantrum behaviors and stubbornness associated with perseveration and resistance to change.
- Negative physical effects of poor food management, such as extreme obesity and related medical disorders.
Parents should be educated on using positive reinforcement strategies, speaking in concrete terms, creating consistent routines, and re-directing repetitive behaviors.
What are some tips for teachers with students with PWS?
Behavior modification is likely the biggest challenge. To address:
- Stick to a consistent routine
- Re-direct perseverative or repetitive behaviors
- Be vigilant in anticipating and avoiding situations in which the student might lose control
- Teach appropriate responses to frustration or anger, then move student to de-escalation areas/activities and wait out any meltdowns
Effective, intentional communication is crucial in instruction. You should:
- Use “do” rather than “don’t” requests
- Be specific
- Outline limited choices to empower
Food prevention is important to the student’s physical health AND behavioral success. To help your student avoid the potentially negative effects of being faced with food:
- Never reward with food
- Keep food out of sight or out of room altogether
- NEVER try to take food away physically – negotiate a trade (i.e. sugar-free candy)
There are various other ways to increase your student's potential to be successful in your classroom. Making the student aware of a plan and getting buy-in can be a very effective way to ensure positive behavior. Providing appropriate ways to fidget with hands can help avoid skin-picking. Concentrating productivity in the morning reduces the effect of fatigue. Recognizing the student’s desire to connect socially but also understanding the potential for frustration in dealing with unpredictable peers can avoid conflict.
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