Pharmac 'short-changes' two-year-old Cameron

Karen O'Reilly knew there was something wrong with Cameron the moment he was born.

"He was like a rag doll, just extremely floppy."

Cameron, now 2, also had breathing difficulties, and during his first five months could not cry because he was not strong enough to fully inflate his lungs.

The diagnosis was genetic disorder Prader-Willi Syndrome, a condition largely characterised by an obsessive compulsion to eat, and poor muscle tone and development.

Cameron's father, Rody, said constant hunger kicks in when a Prader-Willi child is between 2 years and 6 years, but so far Cameron has not shown signs of an abnormal appetite.

Government drug agency Pharmac on Tuesday announced that a growth hormone which helps alleviate the syndrome's main symptoms would be fully funded, but only about a third of the estimated 45 New Zealand Prader-Willi children would benefit. The rest would be excluded largely because of a height criteria which stipulates that the child must be shorter than the average height of his or her peers.

Pharmac have told the O'Reillys that Cameron would be excluded from the subsidy because he is at a normal height for his age, and it was not possible to project his growth without the treatment, which the couple had been paying for privately since last April.

The hormone treatment, which is administered through nightly injections, started when Cameron was a year old - when it appeared that "there was no prospect of Pharmac funding it", said Mrs O'Reilly.

"It's a dangerous game to wait when it's your child's future."

But it would come at a hefty price tag. With two other children under five to support on a part-time wage and a primary school teacher's salary, the O'Reillys could only afford half the recommended dose, which still costs them $10,000.

They want Pharmac to extend treatment to all Prader-Willi children, as the benefits would be immense.

"We want them to be able to run, swim and cycle, and not die at 35 from heart disease from obesity," said Mr O'Reilly.

The national director of the Prader-Willi Syndrome Association, Linda Thornton, said Pharmac's decision was disappointing. The association had been lobbying for the past 11 years for the treatment to be funded.

"I just don't see that this is fair. They are discriminating against children because they are a fraction too tall, for God's sake. It's heightist.

"My daughter, who's 22 now, will never benefit from the growth hormones. It's not the end of the world, but it is for the parents who know that it's out there and see it as part of the treatment that their kids deserve."

But Pharmac medical director Dr Peter Moodie said the decision was made by a panel of endocrinologists including one nominated by the Prader-Willi Association.

The endocrinologist, Esko Wiltshire, was in agreement with the panel, but did not respond to emails when Pharmac sought to reconfirm the decision taken following lobbying by the association, said Dr Moodie.

A third of the 45 children estimated to have the condition would benefit, costing the agency $400,000 a year. That number is disputed by the association.

Dr Moodie said clinical data had shown that children of average height were producing enough growth hormone in their own right, and would not benefit as much from additional hormone treatment.

"This was a clinical decision, it wasn't a financial decision," he said.

Parents who could show that their children fitted the criteria before they were started on the growth hormone would be funded, he said.


Prader-Willi Syndrome

* Genetic disorder first described in 1956 by Swiss endocrinologists.

* Signs include excessive hunger and preoccupation with food leading to excessive or rapid weight gain, small stature, and poor muscle tone and development.

* Growth hormone injections can help with muscle development and growth, and may reduce food preoccupation and weight gain.