Prader-Willi syndrome 

Prader-Willi syndrome is a rare genetic disorder that causes characteristics such as obesity due to an excessive appetite. A psychiatrist explains the different symptoms. Also find out how Sharon copes with her daughter Daisy's condition, and how she prevents Daisy from overeating.

Managing Prader-Willi syndrome: a guide for parents

Transcript of Prader-Willi syndrome

Prader-Willi syndrome is a genetically determined disorder,

so it's often now suspected at birth

because of this combination of floppiness, failure to thrive,

difficulties feeding.

But what's striking about children with Prader-Willi syndrome

is that as they grow, and as they reach usually two, three, four years of age,

the child seems to develop a much greater interest in foods.

The child will take as much food as the parents will offer them.

And then you begin to see the child becoming obese.

Maybe it's better thinking of it as a syndrome of starvation,

in the sense that people with Prader-Willi syndrome

are fooled into thinking that they're still hungry,

even though they've eaten a large amount of food.

We felt that it was really important

to put some rules and structure in place to support Daisy.

She eats at regular times.

Do you want some salad with yours?

(Daisy) Yeah. Can you get some chutney out, please?

Yes, darling.

Our cupboards aren't locked and our kitchen isn't locked,

but Daisy can manage that. It's worked for her.

(Tony Holland) As the child gets older,

not only do you see a persistence of this tendency to overeat,

but their development tends to be mildly delayed.

It may become apparent that they have some, usually mild, learning disability.

So we recognise now that the slow growth in childhood,

the failure of a growth spurt prior to puberty

and the short stature characteristic of people with Prader-Willi syndrome

is because of a relative growth hormone deficiency.

So nowadays the growth hormone would be replaced starting in early childhood.

And so children with Prader-Willi syndrome may now be of normal stature.

(mother) It's a complex syndrome

with lots of seemingly unrelated conditions with it.

In addition to the whole eating disorder,

there are lots of other aspects to the syndrome.

Poor body temperature control, which Daisy suffers from a lot.

She has poor muscle tone, so she tires easily.

She can't exert herself physically very well.

And so the whole weight loss and eating cycle

is a bit of a vicious circle.

There's often a tendency to repetitive and ritualistic behaviours,

sometimes referred to as obsessive behaviours.

As they get older, they seem prone to developing depression or mood problems.

(Daisy) It can make me very emotional at times.

I just feel really frustrated.

And it's not really like I'm actually upset.

It's just like PWS is pulling me out

and I'm trying to stay right back here.

And with myself stretching,

I'm getting closer and closer towards a more stressful situation.

She gets very emotional and very upset very easily.

And sometimes that can be because although Daisy's an articulate child

with good understanding, she might get confused by a social situation

and she needs something explaining to her fully

until she's clear in her head before she can move on.

And sometimes that can take quite a long time.

The issue of what school children with Prader-Willi syndrome go to

is a difficult one.

What you find is that in terms of intellectual ability,

they would be best suited having special support in a mainstream school.

But many of them do end up in special schools

largely because of their behavioural difficulties.

(mother) She does quite well at school.

She's quite successful, especially at things like reading and writing.

And so she's managed to achieve a lot more

than I think that we thought she would.

And she's managed to go through mainstream primary and secondary,

which is quite unusual for people with Prader-Willi,

but at the moment she's succeeding with that with support in place.

I do like school.

I think I get brilliant support. I have three TAs.

I just come into lessons and yeah, they make sure I'm OK

and that's really, really lovely.

I always saw myself being a teacher.

But I think instead of that now, um...

I think I'm going to work with animals.

We've now seen generations of children

grow up and become adults with the syndrome.

The question is how best to support them once they become adults,

because with adult life, the big difference is independence.

And people with Prader-Willi syndrome,

although they may have some minor degree of a learning disability,

are able to lead independent lives.

The Prader-Willi Association were very supportive and helpful

and put us in touch with other people we could talk to,

and that was the most helpful thing,

talking to other parents and hearing how their children actually were

and all the things they could actually do.

I think one can give hope to parents and say "There is much that you can do

that will contribute to the wellbeing of your child."

We understand the syndrome much more and one can put oneself in their shoes

in a way that wouldn't have been possible before.

And my mum, yeah, we...

we sometimes argue and things,

but she's really helped and supported me through this,

and although I sometimes say I feel like she doesn't really understand,

she always does.

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