Monday, 12 November 2018

From Ireland to Munich

Gina Mullis is a Kiwi, living and working in Ireland.   This was her first experience of a PWS conference and she writes...

I was very fortunate to be given the opportunity to attend the IPWSO 5th International Caregiver’s Conference in Munich this year.  It was my first time attending and I was unsure of what to expect.  Having had little direct experience of working with individuals with Prader-Willi Syndrome (PWS) and their families I hoped to get a broad understanding of the syndrome from a clinical perspective and insights from others already working in the field with respect to evidence-based best practice approaches to providing therapeutic supports and interventions. 

The two group workshops I attended on Early Intervention and Aging in People with PWS did give me the broad understanding that I was hoping for at the outset by providing valuable information on the ways in which clinical staff can best support the social, emotional, and cognitive development of individuals with PWS throughout the lifespan.  However, the greater insights came out of discussions with parents and care staff working with and caring for individual’s with PWS, all of whom were incredibly generous in their willingness to share their own personal experiences.    

Resilience Care are in the process of setting up a residential home here in Ireland specifically for individual’s with PWS and I feel confident and enthused about my role in this enterprise as a result of the knowledge gained and the relationships established at the IPWSO Caregiver’s Conference.  I look forward to developing the professional and personal relationships we established at the conference through ongoing dialogue (thank you Norbert and Hubert for the word) and sharing of information and experiences, and can’t wait to see you all again next year in Cuba.

Kiwis may be flightless birds, but these two found their way to Munich from opposite sides of the world!
          Linda Thornton & Gina Mullis

Tuesday, 9 October 2018

Our friends in Slovakia

Maria Benekova, our IPWSO representative from Slovakia and recipient of an educational grant to attend the Caregivers' Conference in Munich (August/September this year) has written a report telling us a little about life in Slovakia and how, by attending the conference, she is managing to cope just a little better!

Slovakia is a landlocked country with a population of approximately 4.2 million.   "It is known for its dramatic natural landscape and many castles. Near the Austrian border, capital city Bratislava features a pedestrian-only Old Town with a lively cafe scene. Rising on a hill above the Danube River, Bratislava Castle houses a branch of the Slovak National Museum, with displays ranging from Roman artifacts to 20th-century arts and crafts." (Wikipedia)

Dear friends,
My name is Maria Benedekova. I would like to share with you my impressions from the 5th International Caregivers´ Conference that was held in Munich, Germany, in August this year. 

I am the mother of Tomas Benedek (16y) who has PWS. We live in Slovakia and are member of the Slovak PWS association ( Our association has been an IPWSO member for several years. I have represented our association in IPWSO for about 10 years. I also attended several conferences organized by IPWSO, the last was the International Caregivers´ Conference held in Munich this August.

For me this has been the best conference so far, because I as a mother am at the same time also the caregiver of my son. In Slovakia we have no homes, not even day centers for PWS, our children go to school for children with special needs, usually they are in class with children with Autistic Spectrum Disorder. After they complete their education, they either go to a day center where they spend a few hours with other adults with disabilities or they stay at home with their parents, have no job, get frustrated, put on weight, have behavioral tantrums, etc.  That’s why, we as parents are also the caregivers at the same time. 

The topics that were discussed during the conference were very interesting, I took part in two workshops focused on self-determination and relations, friendships and sexual education. The fact that the attendees worked in workshops gave us the opportunity to speak about our problems, think of behavior management methods and ask questions related to life with a young or adult person with PWS.  I asked many questions, received many valuable answers that I am applying into my every day life routine with my son Tomas. 

I want to thank the conference organizers for giving me the grant to participate in the conference. I met lot of people I knew already from other IPWSO conferences and I made new contacts with caregivers from countries that are not so far away from Slovakia (Austria, Switzerland) who work in day centers where they look after persons with PWS. I hope to visit some of these centers in the future and maybe get an inspiration and know – how that our Slovak PWS association can use in the future when establishing a home or center for people with PWS.

Maria Benedekova
Slovak PWS association

Monday, 23 July 2018

Thinking about autism and PWS

by Linda Thornton

I think today it is recognised that many PWS behaviours fit into the autistic spectrum.  As far back as 2007,  a study “Autistic Symptomatology, PWS and its Derivations for Treatment” looked at the PWS diagnostic approaches that are also found in autism in order to make a therapeutic connection.  The results reported problems in PWS which represent three major clusters also found in autism:
  1. Socialisation (alteration of social development, especially interpersonal development and ranged from loneliness to excessive sociability with strangers.
  2. Verbal and non-verbal communication
  3. Restrictive and repetitive parameters of conduct (rigid aspects and limited interest)
Other studies have looked at the genetic subtypes of PWS to see whether one might be more prone to having autistic-like behaviours than another with a prediction that those with the uniparental disomy (UPD) diagnosis would have more autistic-like behaviours. From my own personal observations throughout New Zealand, I have seen children with the imprinting diagnosis to show more autistic-like behaviours than the other two subtypes. 

Prader-Willi Syndrome is listed as one of a large group of disorders associated with autism and looking at the three areas of comparison above, we can start to see where they connect: 


"People with autism are kind, sensitive people, but they may not read signals or know how to respond."  This is also the same as people with PWS

"Often a person with autism becomes anxious and depressed, has low self-esteem and becomes angry because of this." This can be noticed in PWS

People with autism are sensitive to sensory things like sounds, smells, touch.  Quite often they cannot stand to wear certain clothing against their skin. Often noted in some people with PWS

A lot of people with autism can not tolerate loud noises.  They actually hurt the person.  High pitched continuous noises such as hair dryers, or small motors are annoying.  Fire alarms, sirens, loud shrill voices, there are many noises that can not be tolerated.  Could this be a reason for sleeplessness at night?  Could there be a noise-intolerance level that we are not picking up in PWS?

Because of their sensitivity to touch, people with autism do not like to be cuddled, petted, tickled.  However, many of them do like to be held closely (not necessarily be another person).  Dr Temple Grandin (one of the world‟s most celebrated people with autism) recognised the sudden docility of cattle when held in a crush.  As a child, she wanted the same feeling, so built her own “squeeze machine” which held her tight, safe, and secure.  She could control the amount of pressure and release when necessary.  This is now recognised as a form of treatment in autism – “sensory integration therapy” – the deep pressure (rather than a cuddle or pat) allows comforting relaxation.  Could swaddling a baby when going to sleep be part of the same feeling of relaxed safety and comfort?  Could it help our children with PW whose sleeping patterns are disrupted?

Sensitivity to taste and texture of food – many people with autism do not like their foods muddled on the plate, can not bear to have more than one taste in their mouths at a time, find the mixture of flavours so obnoxious that they even vomit.  I know that this isn't a common characteristic in PWS, but I have witnessed a person carefully take off all the parsley flakes from a portion of rice on her plate.  And I know some with PWS who have an absolute objection to the taste of some foods.

Sensitivity to smell will often cause a person with autism to go right off another person because they may have, for example, bad breath, or be wearing a perfume which is just not tolerable to the senses of the autistic person.  I know that many people with PWS are sensitive to smells and can sniff out a fish-and-chip shop at 100 paces, or hear the sound of rustling chocolate paper through deep sleep!

Routine and consistency
People with autism do not like change.  They need consistency and routine.  If you tell them you are going to the library – that is exactly what it means.  It does not mean stopping off at grandma's on the way there or back.  It's not what you said.  Do not automatically expect a person with autism, or with PWS to be able to cope with change or surprise.  Surprises can lead to panic attacks.

Using pictures to tell the sequence of an activity is the best way you can help a child with autism.  It's also a way that works in PWS.  This is a way of reducing anxiety, introduces a routine that can be relied upon and teaches sequencing.
Children with autism find it difficult to share their toys.  By sharing, they lose control.  As it is important for the child to retain control, the concept of sharing does not make sense. A sense of control is important to any child with a disability.  Making sense of their own world (control) is the only way they can start to make sense of the real world

People with autism can be seen as being self-centered.  This is essentially recognised in PWS as well

Verbal and non-verbal communication
 Behaviour is a form of communication.  This includes all behaviours whether we regard them as socially acceptable or not.  Body language is an important signal for communication and often relates to the need for calming down.   In autism this may take the shape of “fingering”, or “stimming” (stimulatory behaviour, for example, spinning a lid, flicking a twig, flicking pages).  Many children with PWS will "stim‟, or appear hypnotized by repetitive movement.

It may mean that stimming behaviour portrays anxiety, or may be anticipating an unwelcome surprise or change in routine, it may be that the person is not coping with the noise or things surrounding him/her and so turns to “stimming” in order to put a form of control back into their surroundings.

 A person with autism interprets language in the form of pictures – almost like a video playing over in their mind.  It is difficult to grasp the concept of words (squiggles on paper) but easy to grasp a visual image.  This is a particularly good way of teaching a child with PWS as well.  

 Repetitive speech in autism is called “echolalia”.  However, to a person with autism, this can be a normal communication.  However annoying it might be to another person to hear someone repeat the same phrase over and over, the repetition to an autistic child is a reinforcement of the whole concept of the word.  For example “Mummy" comes to mean "Mummy" because of the whole of the experience, that is, the smell of Mummy, the house where Mummy lives, the shape of Mummy, the sound of Mummy, etc. Most children begin their language acquisition by using this gestalt form (ie a word that is more than the sum of its parts) but quickly change over to an analytic form when the recognise that "Mummy" is a word that can represent other things besides the whole of their experience with their specific "Mummy."

Repetitive questioning as PWS communication is very common.  Usually we treat it as a difficulty to comprehend the message.  We generally answer the question and tell the child to repeat our answer in the hope this will stop the questioning.  More often than not, it doesn't work and the repetitive questioning goes on and on.   

Repetitive Behaviour
Being aware of a repetitive behaviour that might lead into further agitation will give the parent or caregiver a "heads up‟ or signal. 

People with autism like order, predictability and perspective.  Many of them love to watch model trains, and when they're older, become train-spotters.  Getting down to ground level to watch a model train go round and round on tracks allows the child to become part of the ordered predictability.  They are almost mesmerized by this and seem to be in another world. Although a person with PWS may not become fixated by this repetitive movement, they do like order, predictability and perspective.  Some of them are completely happy in an environment of rules and regularity.  Some are very tidy – and some aren't!

At an International PWS Conference I heard a paper given on behaviour where it was suggested that a child with PW might become so fully absorbed in the task at hand (in this case it was a jig-saw puzzle) that they appear to be “in the flow” or “at one” with the object.  This was to be encouraged as a time of well-being and wholeness, and could be used as a reward. Being “in the flow” might also account for the serenity of the child with autism when stimming or absorbed in their "special subject‟.  A person with PWS can also become absorbed in their "special subject‟, appear more serene and "in the flow‟.

Taking away the stimming object (which may be seen as inappropriate) will always be replaced by something else of their choice.  Be careful in taking away something considered inappropriate as it might be replaced by something even more inappropriate.  

When a person becomes terrified of a world full of scary sensory experiences, they become almost shell-shocked and need a coping or escape mechanism.  They can become mesmerized by a repetitive action or sensation and will do this repetitive action over and over in order to allay their fear and anxiety around what will be their next bad experience.  Often the person will spin, or flap hands. 
This is a clear signal for sensory overload. This is similar to the experience of a person with PWS whose world becomes out of kilter, a sense of loss of control may erupt with challenging physical behaviours.

People with autism like neatness and order.  Some people (not all) with PWS show similar need for order and can tell immediately if something has been disturbed, replaced, or is no longer there.  For a person with autism this can mean more than just “something"s gone”, it can mean if a detail is changed, the whole picture becomes different.  It is no longer the same.  For a "normal‟ person this can be difficult to understand, and words of platitude will not help. 

I have witnessed this many times in PWS.  If a stitch is out of place, the garment is ruined, useless; if a toy has a blemish, it is wasted, imperfect,

Obsessive compulsive disorder:  this seems to be the main area where people with PWS gain the label of having autistic-like behaviours.  What does it mean?  In autism, obsessive compulsive behaviours are a means of reducing anxiety; the behaviours themselves become almost irresistible in their repetitiveness and therefore an obsession.  Often these obsessive repetitive behaviours are done to reduce a negative experience, to allay anxiety or what could go wrong (eg handwashing), or a fear of contamination.  In their extreme, behaviours take over from language and the ability to speak reduces.  

Having identified behaviours where PWS and autism cross over, the next question is how to manage the behaviour in a way that is win-win.  Some of the strategies offered at the autism seminar were:

Strategies for coping

Strategies for reducing stress levels: identify the stress, teach deep breathing and relaxation.  Anxieties are often at their most profound around puberty.

Strategies to reduce repetitive questioning or echolalia:  don't ask too many questions.  Make statements, comments, and praise.  Questions confuse a person and raise more questions from them.  Repetitive questioning can also give a person a sense of satisfaction or reassurance that you have not changed your mind.

 Disappearing into one's own realm by rocking, spinning, or becoming absorbed in their
“special interest” helps to give the person comfort.  It alters their state of consciousness (in other words, shuts out all the unwanted things).  It is possible to confine some behaviours to a time or place that is more acceptable.

 Self-injury is also common in autism as it can be in PWS.  However, in autism this can also lead to epilepsy and with severe autism this is as common as 1 in 3 people especially during puberty.  Self-injury can be caused from depression (“what's wrong with me/I hate myself”) or extreme rage when the intensity of emotion is overpowering. Skin-picking, pulling toenails or fingernails, even head-shaving and piercing with sharp object can be seen as self-injury.  

Children and adults who are unable to meet a steady gaze because they feel threatened by it, are often attracted to animals who do not hold a gaze either, and are non-judgmental.  Sitting alongside a person means they do not have to meet your gaze and they feel more comfortable.

Music therapy is proving very successful in children with autism especially when learning to speak.  Singing, rather than saying, is a much easier way to learn to vocalize.  It's a good way to "pattern" behaviour and to learn how to sequence.  For example, you can have your very own “getting dressed” song which teaches the child the correct sequencing.  Songs for other special activities; just starting to sing a line of the familiar song will capture the child's attention.  This is also a technique successfully used by teachers. Choice of music for personal down-time is interesting – many choose heavy rock or heavy metal music as a release for anger.

 Anger:  the similarities with PWS here are obvious: 
  • Two-thirds of adolescents with Asperger's syndrome have a secondary mood disorder, anxiety, depression, anger.  Many suffer bullying and teasing; many can not understand emotions; many show social anxiety about making mistakes and often become controlling as a defence mechanism; many get exhausted at school from socialising as well as learning and, once at home, a different personality arises.
  • Anger is often a "quick fix" or breaking something to release energy.  Often this results in expulsion from school.  I have heard of a young man with PWS who will break glass and once into breaking glass, the repetitive action for him is a release for his anger.  
  • Often the anger is due to a feeling of not being valued (although this in itself is not often able to be vocalized or explained).   Anger can be symptomatic of depression.  
  • Lunar cycle:  the full moon can be a time for problems – I have heard this mentioned often! Keeping a diary over the months will indicate whether the lunar cycle is indicative of behavioural disturbances
  • Keeping a visual thermometer of anger and sitting alongside the person, you can ask the child whereabouts on the thermometer they see themselves.  They can then start to identify emotions of anger from low-level to high.
  • A survey of 238 children and adolescents showed 64% had more problems with anger; management than typical children, 58% had rapid mood changes more than typical children.           
In conclusion:
By looking at others' behaviours we can see how we can share strategies and understand other similar behaviours, we can start to see the world from their different perspectives.  This may help with better management and our understanding of the ways in which people with PWS see their world.

Understanding behaviour as a form of communication means we have to better understand the behaviours and what they mean.  

More information in Autism