Monday, July 23, 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: 

Socialisation

"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

Sensory
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

Thursday, June 28, 2018

What teachers need to know: PWS in the classroom

by Linda Thornton

It is important that we recognise the learning ability of the child, and meet their individual, special needs as required. 

Students with PWS are very receptive to learning and are keen to please.  In general they have good reading skills, but poor numerical skills and handwriting can be slow to develop.  They show ability to learn computer skills and often have excellent fine motor skills, for instance, many are particularly clever with jig-saw puzzles, threading beads, and many show an aptitude for fine handiwork including needlework and knitting.  The IQ level generally falls in the just-below-normal category, but often shows “islands of
competence”, in other words, they might be equal with their peers in some areas, but need support to reach potential in others.

Throughout integrated primary and secondary education, it is important for the student to have teacher aide time.  Although this is not always available for many students with special needs, it should be applied for on all levels.  The need for teacher aides will not decrease as the student progresses.

Teachers should be as familiar as possible with the characteristics of this syndrome, even including the genetic sub-types, as this will impact on the learning ability of the student and the teaching strategies employed.

Genetic sub-types:  Within the deletion on Chromosome #15 which causes Prader-Willi syndrome come three sub-types:

  1. The most common is the actual Deletion (a deletion on their paternal side)
  2. Secondly comes the Maternal Disomy  (a double copy of the mother's chromosome)
  3. Thirdly is the the mosaic imprinting.
Certain characteristics are now being recognised in each group and a flurry of research has provided us with the information that those with UPD generally have better-developed expressive language (Roof et al., 2,000; Whittington et al 2004), but somewhat poorer visual memory and puzzle-solving skills (Dykens, 2002; Verdine, Troseth, Hodapp, & Dykens, in press). Individuals with UPD may be less apt to skin-pick (Dykens, Cassidy, & King, 1999; Symons et al., 1999

Comparing 12 participants with Deletion and UPD deletions, Butler et al. (2004) found that the Deletion group had lower reading, math, and adaptive behavior scores, and higher externalizing behaviors and severity of compulsions"

Those with the mosaic imprinting tend to have more behaviours similar to those with autism, so if your skills lie with teaching those with autism, many of your practices will be similarly employed with PWS. 

Although you may not be told the deletion type of your student, it will help you to understand why not all students with PWS are the same!

(Further reading scroll to "Differences in Genetic Subtypes")



Relative learning skills:

 Maths teaching needs to be conceptual, practical, and often repeated several times before there is understanding.  Teaching the use of a calculator immediately helps the level of understanding.  Once understanding has occurred, the concepts generally remain.  Like all students, they thrive on praise.
  • Good long-term memory skills
  • Receptive language
  • Good at puzzles
  • Visual processing (relative to auditory processing)
  • Basic maths skills (especially with calculator)
  • Reading skills
  • (Some) good computer skills
  • Social and friendly

Learning weaknesses:
  • Poor short-term memory skills (visual-motor and auditory-visual)
  • Expressive language
  • Poor fine and gross motor skills
  • Sequential processing deficit
  • Difficulty with abstract concepts
  • Short attention span
  • Communication difficulties (may end in temper outburst)

It is important that the behaviours or issues that challenge are identified.  Some will result in having to manage the actual environment with school lunches and all food being kept out of reach, money being held by teachers (for all students ) and some new management skills.

TEACHING STRATEGIES

Give positive instructions:
Students with PWS tend to have a fairly rigid or concrete way of thinking, and tend to work best to a set routine and positive timetable.  They can accept change if prepared for it beforehand, but a sudden, unexpected change may result in an uncooperative student.  It is sensible preparation to warn beforehand if something is to be postponed or cancelled.  If the student doesn’t clearly understand the instructions, you may find resistance to change.  Do make sure the student knows that this will not change lunchtime!  A common indicator of this might be persistent or repetitive questioning from the student in spite of having heard the answer.  Be patient, use visual as well as verbal, instructions.

Perseveration:
Constant persistent questioning is a common characteristic of the syndrome.  Answer questions up to two times, then write it down if necessary.  Check with the student to see they clearly understand your answer.  Ask them to tell you what they are going to do, or what the answer is; even ask them to write it down for you.  Clear communication is the key here.

Use visual schedules:
The student may have difficulty in finishing a task on time, or may display perseveration (repetitive questioning), a characteristic common to the syndrome.  Perseveration indicates an inability to process information coherently or sequentially.  Not finishing a task on time can incite some obessive compulsive behaviour.  In both cases, using both a verbal reminder, combined with a visual schedule, or prompt, will assist the student.  This may be anything from a simple egg-timer, to a daily pictorial class schedule to help with time-keeping. Children with PWS greatly enjoy achievement charts, no matter what the age.

Always have Plan A and Plan B:
It is sensible to have a fall-back plan in the likelihood that your Plan A does not eventuate.  This will prevent disappointment, frustration and anger, and keep the student in the loop.  Working out probabilities and possibilities beforehand, including extra staffing, is a precaution that is essential.  Occasions will arise when the student refuses to go on an outing, or to participate in a class activity.  Rather than have this spoil the day, Plan B can be brought into play.

Break tasks down into manageable parts:
  • Have no more than two-step directions
  • Have shortened assignments
  • Brief (or no) homework
  • Ask - don't demand
  • Control access to food and money
  • Identify areas of accessibility (to food and money)
All teachers have their own workable strategies - the above is designed to help understand the thinking of the student with PWS.

If you would like further information on strategies to use in the classroom, contact us.

Thursday, June 21, 2018

So, it's time for school...

This as a general guideline for parents of school-age children.  Of course, each country is different, and each culture is different.  You may, however, find some of this helpful.   (Linda Thornton)

Children with PWS are very receptive to learning, they generally have good reading skills, but poor numerical skills and their handwriting is slow to develop.  They show good ability to learn computer skills and often have good fine motor skills (jigsaw puzzles, threading beads etc). Their IQ level generally falls in the just-below-normal level, but often shows "islands of competence".  Maths instruction needs to be conceptual and practical and often repeated many times before there is understanding. Once understanding has occurred however, the concepts generally remain. Like all children they thrive on praise. Teaching the skills of using a calculator, for instance, is often more useful than trying to teach the times-tables.
Primary Schooling
On the whole, children with PWS can manage primary school years well. If the school is able to provide a teacher aide, then your child will cope better within the structure of the classroom.

Secondary School Options
  • Attending a mainstream school:  This is often difficult for any child with any disability and this is where a teacher aide is invaluable.  Check with the school to see whether a teacher aide is possible - and for how many hours a week.  If this works, then great!  If the school is genuinely interested in the welfare and learning of its special needs students, and is keen to learn how best to support your child, then this is a great opportunity.
  • Attending a school with a Special Unit attached:  Go and visit the special unit, spend some time there, see how it works and whether your son or daughter will be well-cared for.  Look at the work units that are being done; see if they are right for your child.  Sometimes the special units will integrate with mainstream students.  Take note of how they deal with this transition (is it a cooking programme with food involved, for example; ask how they will manage this for your son/daughter).
  • Special Residential Schools: Some countries have special residential schools for students with disabilities. If you have this option, go and visit the school; spend some time there, watch how the students interact, take note of how they manage meal times.  Take your son or daughter with you and make sure they feel comfortable with the idea of boarding away from home.

Talking to the school

Parents need to be prepared to talk to their son or daughter's teachers every time they change class.  Teachers need to know how to manage their student, and you need to make sure they understand what this really means.
Each time your child enters a new class, the pupils need to know why your child is different and how they can best support and befriend your son or daughter.  You may choose to take the opportunity of talking to the class yourself.  Personally, I always found that talking to the children meant telling them what they need to know, rather than the full-on description of PWS!  I always likened it to a child who has diabetes and, for their own safety, cannot eat sugary foods and must keep on a safe diet.  I would do this without my daughter being present.  I would also tell the students how important it was for my daughter NOT to be given any of their spare lunch as this would upset her diet.  Plus, I would always make sure the teacher will keep school lunches out of reach - somewhere safe like a locker, or, if that wasn't possible, then the teacher took full responsibility for handing out lunches, including my daughter's (this was to prevent her from eating all her lunch the minute she got to school, and from eating or sneaking food from other lunches).

Positive Instructions

Children with PWS tend to have a rigid way of thinking and tend to work best to a set routine and positive timetable. They can accept change if prepared for it beforehand, but a sudden unexpected change may result in non-cooperation - generally more so with an older child. It is sensible preparation to warn beforehand if something is to be postponed or cancelled and, if possible, what will take its place.

Sociability

Generally speaking, children with PWS are sociable and interactive with other children, but tend to mix with younger children rather than their peers whose natural physical ability will often leave the child with PWS behind. Some children prefer their own company or adult company and will seek frequently seek out a teacher's company.

With an ordinary classroom setting, children with PWS may have difficulty in settling and can become easily distracted. It is not "naughty" behaviour but part of the syndrome. They may work better with their 'own' desk and chair rather than continually moved around.

Simple behaviour modification techniques

Things like "ignore-redirect-praise" work well; removal from a situation which appears to be heating up and redirection to another task until the person has calmed down.  Basically with the younger person, the behaviours tend to be comparable with any child of his/her age.  These incidents may occur around food, possessions, or anxiety.

Eating behaviours at school

Because of the deletion in chromosome 15 (which governs normal ability to feel full), children with PWS are constantly on the lookout for food.  They may 'take' or steal, swap something for food, or just be opportunistic and take food when the opportunity arises.

Practical intervention from teaching staff will mean that:

  • lunchtime and playtime are supervised so that the child eats only what is prepared for these times (otherwise everything is likely to be eaten at once);
  • care must be taken to see that other children are not passing on unwanted food and that the youngster him/herself is not suggesting they might finish others' lunches for them.
  • Food discarded in rubbish-bins in the classroom will need to be removed so that it does not provide temptation.
  • Lunchboxes need to be placed in view of the teacher so that they also do not provide temptation. They may need to be handed out at each break.
  • Any programme which includes food, or cooking, will need to be supervised.
  • It is a good idea to have a notebook which goes home with the child, noting any change in dietary intake during the day. Accidents do happen!
Generally speaking...

  • Be aware of bullying
  • It doesn't pay to be sarcastic, or even use subtle humour. People with PWS do not respond well to such tactics.  
    It doesn't pay to argue. Make the statement, allow the person one more comment, warn that the discussion is over - and stick to it! You will never win an argument.
  • Don't ignore bad behaviour - try interventions to prevent it.
  • Don't use food as a reward or punishment. This can cause escalating behaviours.
  • Don't promise anything you cannot or will not do. They will not accept any reason for change.
  • Arguing often provokes further escalation in behaviours. Their concrete thinking doesn't lend itself to reasoning.
  • Showing a child what you expect of him/her gets better results than verbally explaining.
  • Keep your sense of humour!
  • If possible, ask for help and support from your local PWS Association or contact us for further help.


    (None of the children depicted has PWS)


Wednesday, May 30, 2018

Behave Yourself!!

All behaviour can be seen as communication.  In PWS, the questions "When do behaviours start?" and "How do I handle challenging behaviours?" are always asked.  Communication will often break down - between the parent and child, the child and teacher, or friends, or other family members, the options are endless!  So how do we try to avoid this breakdown?  The main issue arises from heightened anxiety levels, firstly from the person with PWS, and also from the person trying to understand.  We need to be able to look at anxiety and all its sub-forms, such as avoidance and non-compliance, denial, perseveration and muddledness, frustration, becoming argumentative or recognising a drop in communication levels, compulsive behaviours , all of these which can lead to a "blowout" or "meltdown" situation.  Let's explore some of the issues:



Behaviour as a Form of Communication


If we try to look at behaviour as a form of communication - that the person is trying to tell us something that they might not have the verbal ability or comprehension to do so, then we can separate the behaviour from the person and look at what they are trying to say.

All behaviour is a form of communication - it's how we perceive and interpret that behaviour that will lead to success or failure. It's a bit like learning another language.

We all perceive differently, therefore we communicate differently.



When do ‘behaviours' start?


So-called challenging behaviours are not usually around during the first few years of life. Teenage and young adult years are when we generally see the more challenging behaviours. As the person ages into their 30s and older, they are generally less challenging, but can have more medical challenges.



Why do we call behaviour "challenging"?


I think it's because we (as parents, or caregivers, siblings, grandparents - anyone, in fact, who is involved with the care of a person with PWS, no matter what the age) find these behaviours as out of the ordinary - they're not ‘normal', they can be embarrassing, we don't understand them, they can be scary and even dangerous and can quickly escalate to a total outburst and meltdown before you can turn around. 

Behaviours can quickly escalate from nothing out-of-the-ordinary to verbal outbursts and abuse, physical abuse, crying jags or hissy fits, violence, self-mutilation and trashing.

It can be very scary. 

There is a goal for success - it's simple: we need to gain the trust, respect, and bonding of the person in order to reach success. (in fact, that's true for any relationship!)


 "Challenging communication" has been described as ‘the emergence of disruptive or destructive behaviours is often the person's way of communicating with an incomprehensible and non-responsive world' (Donellon, Mirenda, Mesaros & Fassbender, p.18.1985)



So, it is learning to interpret the behaviour, as a communication, that is the key. The thing is, we interpret everyone's behaviour according to our own perceptions - whether we like, or dislike a person, what we judge them on, and how they measure up, etc. Well, guess what? People with PWS will also interpret everyone's behaviour, but according, of course, to their own perceptions. And we know that people with PWS have very concrete perceptions! We know that they make up their minds regardless of whether they are right or wrong, we know that they like hierarchy, and we know that once they've interpreted your communication, they will also have judged you, too.



We interpret behaviour by our own social codes; we know how to monitor our behaviours, how to hid our feelings, how to be polite, we understand how to reason, to make allowances, to compromise, to forgive. 

People with PWS will not have the same social code as you, and their behaviours are often described as manipulative. 

I would ask you to think about this word "manipulative". It means, getting what we want , usually by unfair or insidious ways and means. But we have learned several things about the syndrome and how it affects people: We know a person with PWS is very ego-centered, with little ability to "self monitor". We know that their world becomes ‘themselves', we know that most behaviours can be traced back to the how's and why's of food-sourcing. We also know that PWS is a ‘starvation syndrome' and survival for a person means eating. 

So - are the behaviours manipulative, or are they just a way of surviving in a world that is contrary and often scary? 

If we perceive PWS behaviours as manipulative, then the way we work with them, support them, and respond to them, will be skewed to our perception of being manipulated. 

Communication Breakdown


People with PWS like concrete outcomes, they like visual interpretations - something they can see will happen a certain way: a staff member (or teacher) will be there at a certain time, on a certain day, and they will be doing certain things. People with PWS are not good at guesswork, so, if you can, try to make your communications visual, and concrete, so their expectations are not put at variance.



Most "PWS behaviours" that result in a loss of self-control (be it verbal or physical) will have come from a communication breakdown and will have been exacerbated by anxiety. Anxiety, therefore, is the biggest precursor (or clue) to what might happen next. It is the best warning we will get, so learn to recognise what makes your person with PWS, anxious. It can be over little things: when is it going to happen, who will be there, what is expected of me, I can't do this, I don't understand... or it can be over bigger things: "will I get caught? why can't I have more? I hate you - I'm leaving."



Anxiety


We all know what it feels like to be anxious, frustrated, annoyed, or stressed. We know how easy it is for our anxiety levels to rise and for our behaviours to become abrupt, curt, even angry to the point of losing our tempers, saying the wrong thing and often making situations worse. Anxiety, if not controlled, will lead to an escalation in behaviour.



It's just the same, if not more, for people with PWS. Anxiety for them is the inabilitiy to control an already difficult world. We know that they are very ego-centric, that the world revolves around them, so think how easily things can get out of control for them.



Lots of behaviours will increase in intensity and many of these will be easily recognisable:



  • Avoidance or non-compliance
  • Denial
  • Perseveration, muddledness
  • Frustration
  • Argumentative
  • Drop in communication levels
  • Compulsive behaviours



If you see a rise in any of these behaviours, you will know that somewhere along the line there's been a communication breakdown. It's time to wind back the clock and find out what's gone wrong.



You can use a variety of strategies:


Avoidance/non-compliance

Give options, and offer to help with the task, or offer alternative solutions and be creative. Check that the task is understood, check the level of abilities around that task, break the task into manageable parts.



Denial

Denial is often used as self-defence (it wasn't me, I didn't do it!) and, quite frankly, there's no point in accusation unless you've caught the person red-handed; just note your suspicions and pass the information on ("I think there was a $10 note taken from my car - please keep a check on any new purchases..."). Don't use open or closed questions and expect an accurate answer - it won't happen. You may choose to ignore the situation rather than engage in argument.



Perseveration/muddledness

Take time to answer fully and check to make sure the person has understood you - get them to tell you what is required of them. Break the task into manageable components. Redirect to one task at a time. Use visual aids if necessary. Put a time limit on questioning. Make sure you have not given the person too many choices - that is a sure way to raise anxiety.



Frustration

Take time to backtrack. Ask simple closed questions (where a yes or no answer will help you quickly understand). Make sure your communication has been understood. Provide good, logical details and reasons and make sure of your facts. People with PWS appreciate being told the full facts rather than a "just because" answer.



Argumentative

You won't win an argument, so don't answer back, no matter how tempting it might be. Take a break, especially if the person is becoming vocal and abusive. Ask if they'd like some time to be on their own. Try to diffuse the situation by asking questions like "tell me what happened so we can try to sort it out". Try to reach a solution or compromise and if necessary, bring in a third person to listen.





Sometimes arguments come completely from left-field, especially when engaged in denial, and then, suddenly, the person with PWS has made themselves the victim and is accusing everyone of not understanding them, of being unfair, and misunderstood. Somehow, when it is perfectly obvious to everyone else that the person with PWS is in the wrong, they have neatly turned the tables on you, and you're the 'bad guy'. This just goes to prove that a concrete outcome has not yet been reached. It's better to leave things for a while, rather than engage in further argument.



Sometimes you can negotiate a catch phrase that the person feels comfortable with ("this is going nowhere, let's both back down a bit", or "can you find a way to resolve this for me?"). This is a good way of empowering the person to find a way to reach a compromise.



Drop in Communication Levels
This is not a common thing, most people preferring to raise the level of communication! However, when it does happen, you will notice the person seeming to withdraw into themselves, maybe start crying. Take a short break, give the person time to collect themselves. Then give them something important to do - a task they might enjoy. Don't insist if they don't want to, but leave the option open.



Compulsive Behaviours

This is sometimes linked with autistic-like behaviours and can present as possessiveness, absolute insistence, accumulation of possessions, repetitive behaviours, may seem
irrational. A task that might appear simple, becomes extremely time-consuming and can only be done their way. This might often happen just as you're leaving the house, about to do something, etc. Reasoning might not be possible and sometimes a person needs to learn from their mistakes and if they miss out on an outing, so be it. But make sure you have Plan B!



Blow-outs and Meltdowns:

These happen. No matter how well prepared you think you are, how well you think you have your strategies planned, meltdowns and blow-outs happen. You might have missed a precursor, or trigger, missed noticing a communication breakdown, and by the time you've realised it, it's way too late.



During a meltdown, make sure the person is safe, does not have access to sharp tools, breakable objects and so on. Make sure everyone else (including you) is safe. You will have to wait this one through - it will burn out, given time. During the recovery phase, you have only one duty - make sure you do not re-ignite the situation! Stay quiet, give the person space, play quiet music, carry on as though everything were normal. It can be hard to do, but it is the best way to bring a person out of a meltdown. And, please, don't forget - this is the syndrome working here.



When everything has calmed down - maybe the person with PW has had a sleep - you will probably find they will apologise for their behaviour. Nearly every person with PW I have ever met (and there have been many!) apologises and asks forgiveness. Accept the apology quietly. Now is the time to ask if they want to talk about it - if the answer is no, do not persist.



I once asked my daughter what it was like when she was in the middle of a melt-down. Her answer was that there was nothing she could do. It was like a volcano and she had to let it finish itself. She couldn't stop it. I thought that was an interesting insight.

These are just my own thoughts on behaviour and how to interpret it and maybe even understand it a little better ~ Linda Thornton