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:
- Socialisation (alteration of social development, especially interpersonal development and ranged from loneliness to excessive sociability with strangers.
- Verbal and non-verbal communication
- 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.
