Health News - Hollywood movie Extraordinary Measures highlights the real world of families facing up to Pompe disease

Published: 11 March 2010
by TOM FOOT

FROM the futuristic world of Avatar to the bomb-ravaged Iraqi landscape depicted in The Hurt Locker, this year’s Oscars went to Hollywood blockbusters set in faraway lands.
But Harrison Ford has already been tipped to pick up a gong next year for his role in a film that will feel a little closer to home, for staff at the Royal Free Hospital at least.
Extraordinary Measures, re­leased later this month and co-starring Brendan Fraser, is the true ­story of a father’s quest to find a cure for his children after they are diagnosed with Pompe disease.
Pompe patients travel from right across the country to attend a specialist clinic at the Royal Free – one of only six treatment centres in the UK.

Paul Hanlon, 49, who has been receiving treatment for Pompe disease at the Pond Street hospital since he was diagnosed last year, said: “It is fantastic that the film highlights Pompe disease and I am looking forward to seeing it. From what I’ve read it sounds like it might have a typical Hollywood happy ending, but as long as it ­raises awareness it can only be a good thing. It hopefully means that when I tell people I have Pompe disease they might actually know what it is.”
Pompe patients lack an enzyme in their blood that breaks down glycogen, which is converted into energy needed for muscles to function. It causes progressive weakening of muscles and can affect breathing and mobility.
Mr Hanlon, who has had to give up his job as a senior bank ­man­ager near his home in Surrey, could not understand why he was so tired all the time. He sought help after he became confined to a wheelchair.
“It may sound strange but when I was diagnosed I felt very positive. I had been through the upsetting stuff years ago and it felt good to have a name to put to it,” said Mr Hanlon.

In Extraordinary Measures, ­scientist Ford discovers a cure for two Pompe patients. But in reality, there is no Hollywood ending, although experts at the Royal Free have made a significant breakthrough in treatment of the condition.
Four years ago, they gave Pompe patients enzyme replacement therapy (ERT) using the drug Myozyme. Before this, sufferers were only offered supportive treatment such as physiotherapy or pain killers. It means Mr Hanlon can be treated from the comfort of his own home.
He added: “I was recently assessed to see how I was doing on the treatment and the physio­therapist could see a definite improvement. I feel better.”
Dr Derralynn Hughes, senior lecturer and consultant in haematology at the Royal Free, said: “The Royal Free has pioneered home treatment which means that, after the first six months, patients can stop coming into hospital and instead have a nurse visit them at home and administer the treatment.”

No10 nurses Visit for award winners

NURSES at the Royal Free Hospital in Hampstead made the journey from Pond Street to Downing Street to receive personal thanks from the Prime Minister for all their hard work, writes Josie Hinton.

The visit followed their success in the Nursing Times Awards in November, when they were praised for best practice in infection ­control. 

The team were celebrated for their ability to work together and their treatment of both patients and staff.

Matron Debbie Barry said: “It was great to be personally thanked for the work we do by the Prime Minister. To have the opportunity to have a look around inside number 10 was amazing – an extra bonus to winning the Nursing Times Award.”