WHAT
IS PRADER-WILLI SYNDROME?
Prader-Willi
Syndrome is a neurobehavioral genetic disorder
affecting approximately 1 in 16000 newborns.
It is not inherited and is not related to medical, environmental or
psychosocial factors. PWS
occurs in people of both sexes.
THE
MAIN CHARACTERISTICS OF PWS
Individuals with
PWS generally exhibit short
stature, low muscle tone (hypotonia), incomplete sexual development, behavioural
problems, speech deficits, and some degree of mental retardation or learning
disability. The biggest problem for
the persons with PWS is their insatiable appetite
owing to a malfunction of the hypothalamus, that part of the brain that controls
hunger and satiety. They also generally require fewer calories to gain
weight,
thus exacerbating their tendency toward obesity. It is interesting to note that
most newborns with PWS, because of the hypotonia,
are unable to suck well enough to thrive. Often
they must be tube-fed until their muscle control improves.
The increased and obsessive interest in food begins around the school
–age years and can result in life-threatening obesity if not controlled
through diet, increased activity and restriction on access to food.
The main
characteristic of PWS
is, however, the
inability of the sufferer to control his/her appetite for food. As a consequence
their diet must be rigidly managed to avoid all of the health problems
associated with obesity.
TYPICAL
BEHAVIOURS IN PWS
The
behaviours of children with PWS is one of the main
concerns for the families. Many people with PWS exhibit
obsessive/compulsive behaviours and do not respond well to changes in routine.
Usually they are not being deliberately difficult, they are having
genuine difficulties coping with a situation. The fact they have specific
learning difficulties, which make it difficult for them to understand abstract
concepts, as well as their rigid way of thinking is linked to their emotional
immaturity. They can be prone to
mood swings, going from happiness to anger or sadness in a very short time
period, usually triggered by a relatively trivial event.
Research
has found that people with PWS are more vulnerable
to having a temper tantrum, again probably linked to their immature emotional
development. They display temper tantrums similar to those normally associated
with 2-5 year olds and the years between 12 and 25 can be particularly difficult (as it can be with young
people that DO NOT have PWS!). They nearly always
show genuine remorse after an outburst of behaviour and are really sorry for
what happened, but this does not seem to prevent them from doing it again.
Skin
picking is very common in PWS. It
may be a small graze which is picked continually and is thus never allowed to
heal or they may make a wound where there was no wound previously.
It is thought that skin picking can actually be a pleasurable activity for the
person with PWS. Their high pain threshold does not warn them that the
activity is becoming dangerous. Skin picking releases endorphins in the brain which makes the person feel
good. This then enforces the addictive nature of
the habit.
As far
as we know people with PWS feel hungry for a good deal of the
time. The mechanism which tells them they are full does not function very well.
They can
feel desperate to eat something; sometimes anything and it can vary from person
to person with some who have only a relatively mild preoccupation with food to
others who will go to extraordinary lengths to obtain it.
THE
POSITIVE SIDE OF PWS
Most
people with PWS have a wonderful sense of humour.
They have a very loving and giving nature and oodles of charm.
Praise for good behaviour is very important and helps to build self
esteem.
Negotiation about behaviour with the person
with PWS can have very positive outcomes and things that others may consider
insignificant maybe very important to the person with PWS.
Start
each day afresh. The person with
PWS will have mostly forgotten what happened yesterday.
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