Prader Willi


Story Details

Henry Szaraz is 16 and weighs 150 kilos he is literally eating himself to death.
Food is Henry's addiction but it is comparable with a heroin addiction or something the need to get that next bit of food is so strong money has to be watched, cupboards have to be locked fridges have to be locked. Henry's mother Ginny says his insatiable urge to eat means she has to be on guard 24 hours a day.

If I leave the room or I am in an another part of the house on the computer or something there is enough time for Henry to steal food. I have to lock the door every time I leave the room.
“So if you are gone for a minute he is in here?” asked Madeleine

When he was only six weeks old henry was diagnosed with Prader Willi Syndrome.a part of the brain which controls appetite is damaged or missing resulting in constant hunger.
A person with Prader Willi can be in a room where there is a morning tea on and there will be a plate of biscuits on the table he might have one with everyone else nobody will notice but he will leave the room with the rest of them.
With permission we set up surveillance cameras in their home to show just how bad things get.

Within minutes of being left alone Henry quietly opens the fridge he then spots a tomato in the corner of the kitchen, subtly eating it before anybody catches on.

Half an hour later and Henry is still hungry, he finds a key to open to the locked cupboard to steal some biscuits.
He then launches a second raid taking the stash into his bedroom where he knows he won't be caught.

So say in three months he can put on ten kilos and it depends on school holidays are difficult because there is no routine he thinks it is food time all the time. He is always asking when is lunch when is lunch.
“If the carers don't have it tight enough that is tight, but if it is not tight enough I can open it up and get my fingers.” Said Henry
“So that is how hungry you get?” Asked Madeleine
“Yeah” Said Henry

For the one in 15 thousand children born with Prader Willi Syndrome most have the odds against them. They are under developed, short with small hands and feet, they have low muscle tone, a high risk of infertility and a much shorter life expectancy. For 35 year old Kate Towers living independently is next to impossible.

When the carers come each day they put my breakfast my lunch and dinner in here and my snacks and then I can feed myself.
“So you have to resist temptation, do you?” Asked Madeleine
‘Just!” Said Kate

“How hard is it doing everyday things for you?” Asked Madeleine
“It is pretty hard, walking is a big effort. I have to use my walking frame I used to walk with two walking sticks but I am a lot slower now.” Said Kate

From the outside this house looks like every other house in this suburban street but it is not it has changed these guys lives. Absolutely and we need more of it, we have a whole up and coming generation of kids that need to be catered towards.

Cate Fogarty from the Prader-Willi association is lobbying for more funding to build Prader Willi homes equipped with 24 hour care.

In a Prader Willi specific home, the residents can exercise for 45 mins a day. They each have their own bedroom, bathroom and living quarters and inside the house the kitchen is securely locked up to ensure they don't over eat yet this is one of three homes in the country and there is a desperate need for more.

My dream is to build 8 new Prader Willi homes throughout NSW I would like to get more government support so our children have the funds and are able to live these independent lifestyles that they deserve.
Drug companies do not put money in to it because there are not enough people with it and they won't make enough profit out of developing a drug.

Professor Lesley Campbell and her team at the Garvin Institute have spent 6 years trying to find a cure for Prader Willi Syndrome.

The most important thing in the end was what can we do to help quickly with that appetite and there was a diabetes drug out which we did a very short test on with a meal and we published that result saying that drug might be useful.

The break through is a drug called Byetta which was found to suppress appetite and help weight issues in Prader Willi sufferers.
It is a synthetic hormone but it is one of the first one that were made for the diabetes and it is indictable twice a day already. There are other varieties of that that are coming once a week once a month and the hope is one of those will be even more suitable and more and more trials should be done.

A breakthrough would be wonderful news for parents like Linda Skie who's 6 year old daughter Mia has Prader Willi.
Since we got the diagnosis so early, we were able to be vigilant with her food intake, and luckily she hasn't got such a hunger yet, we have to be very careful with her food intake that she doesn't have too many calories, but because we have been regimented she does follow the rules that we've put in place.

“It is that hunger that will inevitably kick in’ Said Madeleine.

“I think my biggest fear is how she'll feel about being different and not fitting in. Just finding her place in the world being a bit different, I think that's a pretty hard thing to do. Until you love someone with a disability you don't really think about it but once you love someone who has a disability you realise how difficult it is and how life changing it is.” Said Linda