What is Prader Willi Syndrome?

May is Prader Willi Syndrome Awareness Month

What Exactly is Prader Willi Syndrome?

Prader Will Syndrome is a genetic disorder resulting from an abnormality of chromosome 15 such as a loss of active genes. In most cases (70%) the paternal copy is missing and in some cases (25%), will exhibit two maternal copies of Chromosome 15. The genetic disorder was initially described by John Langdon Down and was named after Drs. Andrea Prader, Heimrich Willi and Alexis Labhart in 1956 and is found in 1 in 20,000 births affecting both sexes. It is also the most common recognized genetic form of obesity.

During childhood, individuals diagnosed with Prader-Willi Syndrome tend to eat constantly leading to obesity and for some, type 2 diabetes will develop. This complex disorder affects appetite,growth, metabolism, cognitive functioning and behavior.

Signs and Symptoms

People with Prader-Willi Syndrome (PWS) tend to never feel full (hyperphagia) which leads to constant eating. Signs in infants include, problems with strength, coordination and balance. Often there are feeding problems at birth, delayed speech and gross motor development. Children may be born with almond-shaped eyes and undeveloped sexual organs. Cognitive disabilities and developmental delays may also be present.

As children began to grow, constant craving for food often leads to behavior challenges including hoarding food, eating frozen food and food left in the garbage causing controlling or manipulative behavior.

Medical Issues

Medical concerns may include the following:

  • Sleep Apnea
  • Respiratory/Breathing
  • High pain tolerance
  • Severe stomach illness
  • Difficulty with vomiting reflex
  • Excessive appetite
  • Binge eating
  • Eye problems
  • Choking
  • Hypothermia
  • Leg Swelling
  • Consuming unsafe items
  • Negative reactions to medications
Teaching Strategies

Most people diagnosed with Prader Willi Syndrome fall between the moderate and mild levels of an intellectual disability meaning there may be challenges in the area of reasoning, problem-solving, planning, judgment, abstract thinking and learning.  A child or student functioning at the moderate level may lag behind their peers in the area of language and pre-academic skills. Adults may function at an elementary school level and will require support in both work and daily living skills. For children and students functioning at the mild level, there may be difficulties in the area of reading, writing, math and money management. as children grow into adults, there may be a need for support in abstract thinking, executive functioning (planning, prioritizing and flexibility) as well as short-term memory and money management.  Teaching strategies should focus on the following:

  • Aggression management
  • Anger management skills
  • Anxiety management
  • Emotional regulation
  • Personal safety
  • Social skills

Keep in mind that many children and adults diagnosed with Prader-Willi Syndrome may have additional challenges in learning due to medication. Some people take medication such as a growth hormone therapy which can cause fatigue.  The following teaching strategies may also be useful when teaching a student diagnosed with Prader Willi Syndrome:

  • Use a multi-sensory approach. This involves a teaching style that includes auditory, visual, tactile, spatial, and kinesthetic (hands on activities)
  • Break learning into small steps. Check for understanding by asking the student to repeat back to you.
  • Teach a skill at least 2-3 times a day. This will help the student retain information.
  • Managing perseveration. Set up a rule where the student can a question no more than 3 times. After the third answer. Ask the student to repeat the response back to you.
Adult Day Program/Residential Setting

Most people with Prader Willi Syndrome due to their cognitive level, will be provided services in either a day habilitation program or live in a community providing residential services. Once a person becomes an adult, it becomes a little bit more tricky on maintaining issues especially behavioral. For instance, while living at home, a parent has the right to lock the refrigerator which is often suggested by experts. However, this becomes a violations of a person’s rights once they reach adulthood. Typically, committees meet to help make the right decisions along with family members and the adult diagnosed with Prader Willi Syndrome. Here are some suggestions.

  • Allow the person to have control of what is important to them. Have discussions on nutrition and staying healthy. Check to see if this may be an appropriate topic the person may want to improve by adding to their person-centered plan. Hold discussion groups in both day programs as well as in residential to discuss various topics on health and nutrition including holding classes on mindfulness and meditation.
  • Trips to shopping malls can be very tricky. Try to avoid mall’s eatery and plan if it is a group trip to have people bring their own lunches.
  • When teaching, allow time before giving additional prompts
  • Give praise as much as you can when it is appropriate.
  • Use visuals as much as you can including graphics and pictures.
Staff Training

Staff training on Prader-Willi Syndrome should include the following topics:

  • Overview of Prader-Willi Syndrome including, causes, symptoms, characteristics, nutrition, and self-regulation.
  • Impact on the family including the stresses families experience.
  • Teaching techniques including problem-solving, forward shaping and role-modeling.
  • Individual rights
  • Managing behavior and crisis intervention
  • Community inclusion trips and activities
Resources

Foundation for Prader-Willi Research

Prader-Willi Syndrome Association (USA)

Prader-Willi Syndrome (Mayo Clinic)

Reference

Prader-Willi Syndrome Association

 

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The Importance of Hearing Young People With Autism

Published by: U.K. Youth
Written by: Maya Hattenstone

There are around 700,000  people on the autism spectrum in the UK, many of these are young people and I am one of them. And you don’t hear from us often enough!

Being a young person with autism you can get lost in anxiety; worrying that people are judging you, that you are not accepted, that you sound strange when you talk. Too often we end up silencing ourselves with our self-consciousness.

I was diagnosed with Pathological Demand Avoidance Syndrome (a condition on the autism spectrum) when I was seven years old.  People with PDA have communication and social interaction difficulties. I found school life and academic work hard. In fact I found a lot of life hard.

For a long time I didn’t know how to be a voice and didn’t want to be a voice. So, like many autistic people my instinct was to withdraw – into silence in social situations, or simply avoid those awkward situations in the first place. Click here to read the rest of the story

Global Developmental Delays

Global developmental delays describes when children do not meet their developmental milestones. Generally from the age of 2 months to 5 years old. Although each child is different in their development, milestones are established in order to determine functional skills on age specific tasks.

Delays can occur in the following area:

Gross motor- Involves the use of larges muscle groups such as walking, crawling and standing. May impact children diagnosed with cerebral palsy.

Fine Motor- Small movement in the fingers used for drawing, painting, buttoning, coloring, and shoe tying.

Speech and language delay- A delay in language may be due to motor-oral problems.

Cognitive- Delays can be caused by, infections, ,metabolic, toxic, trauma, and chromosomal abnormalities (Down syndrome, Turner syndrome, etc.)

Social/Emotional Skills- Shows signs of delay in responding and interacting with other people. Common cause may be autism spectrum disorder

The following articles provide information on understanding global developmental delays:

6 things I’d tell the parent who just heard the word ‘Global Developmental Delay’

Causes and symptoms of developmental delays

Developmental delays and disabilities

Global Developmental Delay

How a child develops

Recognizing developmental delays in your child

Types of developmental delay in children

Understanding developmental delays

What causes developmental delay?

What you need to know about developmental delays

Anxiety On The Spectrum

Anxiety is one of the co-occurring  disorders that affect autistic children. A study published by the Journal of Child and Family Studies found that autistic children had higher anxiety levels compared to neurotypical children. It is estimated that 40% of autistic teens display signs and symptoms of anxiety.

Why Autism and Anxiety?

There are many reasons anxiety affects autistic children in large numbers. Bill Nason, moderator of the Facebook page, Autism Discussion Page and psychologist, explains that daily experiences that impact their nervous system including sensory, cognitive, social, and emotional vulnerabilities leave autistic children and teens with daily high levels of stress. He explains what comes naturally for neurotypicals, is hard work for them placing their nervous system on high alert even during its resting state. High levels of anxiety make take the form of mood swings, rigid and inflexible thinking and obsessive compulsive behavior.

What are the signs of anxiety?
Physical Signs

Complains about feeling sick

Complains about headaches

Difficulty sleeping

Fidgets and spins

Worrying

Worries about making a mistake

Difficulty in performing in exams

Is afraid of being placed in a new situation

Social

Apprehensive of meeting new people

Displays difficulty in joining new groups

Avoids interacting with peers

Worried about being laughed at.

Types of Anxieties

Anxiety of uncertainty- fear of anything new and unfamiliar
Social anxiety- difficulty interacting with others during social events
Sensory overload- Becomes anxious in settings that present strong sensory stimulation
Generalized  anxiety- non-specific ongoing pervasive anxiety

Strategies for Reducing Anxiety
  1. Rest
  2. Exercise or physical activity
  3. Allow time to participate in a favorite activity
  4. Self-stimulation can be used for calming purpose
  5. Relaxation techniques such as mindfulness and meditation
  6. Build structure into daily routines
  7. Review the day including what is expected of them
Additional Resources

Anxiety in autistic adults

Classroom ideas to reduce anxiety

Managing anxiety in children with autism

 

 

The Exhaustion Of My Life As An Autistic Person

Website: The Mighty

Written by: Violet Haze

One of the hardest things for me to deal with as an autistic person is people not understanding what life is like on a daily basis. Nobody has any idea how much energy goes into ensuring I don’t mess up too badly or that I “get things done” when they need doing. Well, they might, but many people in my life didn’t until I received my diagnosis, and even then, it’s hard for them to understand sometimes.

Ever been so tired after a busy day that you sit down and before you know it, you’re waking up out of nowhere and it’s the next day already… when you weren’t even finished with the day before? This has been my reality since I was young. A few hours of an activity that didn’t involve being at home, and for the next day or even two, I’m so tired I can’t do anything except lay around and sleep. The exhaustion of autism is real and tangible in my everyday life. Click here to read the rest of the story.

Motor Development: How Autism Affects Motor Skills

Motor development
Website:Profectum
Written by: Serena Wieder

Moving our bodies throughout our day to day lives is something most of us do without giving it much thought — but it actually takes a considerable amount of skill.

The central nervous system controls both fine and gross motor skills. Fine motor skills include small movements, such as writing and drawing. Gross motor skills include larger movements such as walking and throwing a ball.

Motor development in autistic children has been the subject of study for years. The reason why is because autism is a neurological condition without any defining physical characteristics. Differences in brain functioning in autistic children are not easy to detect, so professionals will often observe behavioral patterns such as those exhibited by the development of motor skills.

Professionals also find it beneficial to observe motor development in autistic children because it can be measured over time, and results of testing can be easily reproduced. Observing motor skills can help professionals discover brain functioning differences, even in cases of high functioning autism. Click here to read the rest of the story

Classroom Accommodations for Austistic Students


A few weeks ago, I had the opportunity to speak to a parent who voiced her frustration with her daughter’s school. Although her daughter is diagnosed with autism, she falls on the mild range of the spectrum meaning her deficits are ignored. This becomes challenging for a teacher who may not recognize the signs and symptoms of an autistic child.

Girls, in particular, often develop the ability to disappear in a large group. Imagine the amount of energy it takes to pretend you hold the same characteristics of others.  This leads to both depression and anxiety in children with autism. There are also sensory challenges a student with autism may face including auditory, visual and tactile.

Reading non-verbal cues forces a child and even some autistic adults to work harder everyday which causes exhaustion and can possibly lead to anxiety.

There are a number of ways to accommodate  a student with autism. If you are a teacher, read as much information as you can on autism. each child is different so it will help to get feedback from parents who can help provide the right accommodations.

The following articles provide great information on both modifications and accommodations  which can be put into the child’s IEP:

10 tips for making middle-school work for kids with autism

14 possible IEP accommodations for children with autism/ADHD

20 classroom modifications for students with autism

23 classroom accommodation suggestions for kids with autism and Asperger’s syndrome

Accommodations and supports for school-age students with autism

Asperger syndrome/HFA and the classroom

Common modifications and accommodations

IEP considerations for students with autism spectrum disorder

Recommendations for students with high-functioning autism

Supporting learning in the student with autism

The Power of Paws: The Therapeutic Benefits of Dogs for Autistic Students

Guest Blogger, Jeremy Divinity

The classroom is a social environment where student success is dependent on the ability to interact well with others. Whereas, 72% of students on the autism spectrum have additional mental health needs that cause challenges in the classroom.

Although the learning disabilities that are associated with ASD (Autism Spectrum Disorder) are unique to each child many autistic students share the same development problems: social interaction, language, and behavior.Autism can hinder a student’s ability to communicate and share experiences with others. Compared to their peers, autistic students are four times more likely to need extra learning and social support. This lack of social-emotional competence leads to a decrease in their connection to the learning environment and academic performance.

ABA (Applied Behavior Analysis) treatments for autistic children have proven that the behavior of autistic students can be changed. Studies have demonstrated that ABA techniques produce improvements in communication, social relationships, and school performance.With the right accommodations, including proper modifications to the educational environment, along with the addition of positive reinforcement, autistic students can overcome the many barriers to learning.To put in place effective ABA techniques, educators need a better understanding of autism and how it may affect learning. Teachers are being called upon to be innovative and creative due to the unique challenges that students with ASD provide, this includes modifying their education programs.

One ABA treatment that is growing in popularity is the use of therapy dogs. If you are unfamiliar with therapy dogs and the benefits of therapy dog treatment, here is a brief history lesson: Smoky, a Yorkshire terrier, and World War 2 veteran was the first official therapy dog whose service on and off the battlefield would pave the way for future therapy dogs. Injured soldiers relied on Smoky, their canine companion, for entertainment to boost their morale. Today, therapy dogs act as a safety net, guardian, and friend who are trained to respond to a child’s most repetitive behaviors. Due to their calming influence, therapy dogs are becoming popular in the autism community. The special relationship between the therapy dog and child stimulates positive changed behavior. For children with ASD, their furry companions are not only their best friend but also offer therapeutic benefits.

Teachers and therapists have found that therapy dogs not only act as “social catalysts” that promote social interaction but also increase the activity levels of autistic students. In a study of 22 children, kids who engaged in therapy dog sessions were more talkative and socially engaged, while also less aggressive.The calming demeanor and influence of therapy dogs aid autistic students in managing the sensory challenges of the school environment. Therapy dogs can mitigate the impact of autism in the classroom by providing stability in what may seem like an unfamiliar environment.The relationship a therapy dog has with a child extends deeper than just companionship, therapy dogs can provide both practical and emotional support. Here are some of the most common therapeutic benefits that therapy dogs provide for autistic students:

Companionship

Therapy dogs show unconditional love, and often times, a loving friendship develops. Both therapy dog and patient enjoy each other’s company in nonverbal ways which assists with everyday life. For example, therapy dogs de-escalate emotional meltdowns by gently interrupting any self-harming behaviors.

Social Interaction

The biggest challenge faced by students with autism is social interaction with peers. When introduced to the classroom, therapy dogs can increase a child’s participation and functional level. After interacting with their canine companions, students with ASD transfer over their new-found social relationships with other students.

Behavior Management

Another benefit of therapy dogs is that they can assist with behavior management by their comforting and calming demeanor. Many therapy dogs are specifically trained to decrease inappropriate behavior by acting as a source of comfort, such as leaning against a child or gently across their lap.

Academic Performance

The most important benefit that therapy dogs can provide for students is an improvement in academic performance. After introducing therapy dogs, you will find that your students are more attentive. While also being better behaved with a new-found self-confidence – which is key to academic success. Autistic students face many challenges in the classroom. To help autistic students overcome barriers to learning school administrators, teachers, and parents must be equipped with the right accommodations. Therapy dogs mitigate the impact of autism and assist in managing the sensory overload of the school environment, and provide students with autism with the stability needed to be successful in the classroom.

Resource Articles

Autism and Pets: More Evidence of Social Benefits

Dogs de-stress families with autistic children according to research

How dogs help children with autism

Pets may help improve social skills of children with autism

 

 is an education blogger for Teach.com and freelance writer from Los Angeles. Read more at http://www.JeremyDivinity.com.

 

Autism Family Resources


Here are some family autism resources for both families and educators.

15 Behavior Strategies for Children on the Autism Spectrum

 

How to Create a Backyard Sanctuary for Kids with Disabilities

 

For Educators: Strategies for Working With Children With Autism Spectrum Disorder

 

Helping Asperger’s Teens To Survive and Thrive: 15 Key Steps

 

Creating a Home Atmosphere of Solitude to Help Cope with Adult Autism

Autism Timeline: A History of Autism

In the 110 years since Swiss psychiatrist Eugen Bleuer coined the term autism, much has changed over the years. The journey of understanding autism continues to grow and while the autism has changed over the years, there are still many more things to discover. Hopefully we are moving from awareness to getting to a place of simply accepting people who bring special gifts to the world.

1908- Swiss psychiatrist, Eugene Bleuer is the first to use the autism to describe individuals with schizophrenia who lost contact with reality.

1912- Dr. Bleuler publishes “Das Autistische Denken” in a journal of psychiatry and presents his thoughts on how a person with autism experiences the world.

1938- Dr. Hans Asperger presents a lecturer on child psychology. He adapts Bleuler’s term “autism” and uses the term “autistic psychopathy” to describe children showing social withdrawal and overly intense preoccupations.

1938- Beamon Triplett writes a thirty-three page account of his 4 year- old Donald’s unusual behavior and sends it to Leo Kanner.

1943- Dr. Leo Kanner describes a childhood disorder involving social and language impairments and the presence of restricted or repetitive behaviors. The account of 11 children leading to a distinct syndrome.

1944- Dr. Hans Asperger reports on 4 children with a pattern of behavior he terms autistic psychopathy- behaviors include reduce empathy, difficulties with forming friendships, impairments in the ability to maintain reciprocal conversations.

1952- The first edition of DSM (Diagnostic and Statistical Manual) is published.

1959- LSD is used as treatment for autistic schizophrenic children.

1962- The National Autistic Society was created- The first autism organization.

1965- National Society for Autistic Children was founded.

1966- South African psychologist, Victor Lotter publishes the first prevalence study on autism in England.

1966- 4.5 in 10,000 are diagnosed with autism in the United States.

1966- Childhood autism rating scale introduced.

1967- Bruno Bettlheim publishes infantile autism and the Birth of Self becomes bestseller; blames mothers for autism.

1969- Dr. Kanner exonerates parents of responsibility for their children.

1970- Lorna Wing uses the term autistic spectrum to describe a concept of complexity rather than a straight line from severe to mild.

1972- Dr. Eric Schopler founds Division TEACCH  at the University of North Carolina.

1977- National Society for Autistic Children added sensory processing as one of the definitions.

1979- Autism spectrum first used by Lorna Wing and Judith Gould

1980- The prevalence is estimated 4 in 10,000

1980- Autism added to DSM-III

1980- Autism is listed as a mental disorder for the first time in the DSM.

1986- Temple Gradin publishes Emergence: Labeled Autism

1988- The movie Rainman popularized and awareness of the disorder increases among the general public.

1991- Sally Ozonoff suggested executive functioning impairs individuals with autism.

1994- The American Psychiatric Association adds Asperger’s disorder to DSM.

1996- Australian sociologist, Judy Singer coins the term Neurodiversity

1998- Andrew Wakefield reports an association between autism and MMR and bowel disease.

2000- 1 in 50 children according to the CDC are diagnosed with autism

2006- Autistic Self-Advocacy Network founded. A non profit organization run by and for autistic people.

2009- 1 in 110, children according to the CDC are diagnosed with autism

2012- 1 in 88 children are diagnosed with autism.

2013- Asperger’s disorder is dropped from the DSM-5

2014-1 in 68 children in the U.S. have autism.