‘Our world was torn apart’ - eight-year-old’s brave fight against illness which will cut short her life
- Credit: Archant
The family of a “wonderful daughter” whose life could be tragically cut short by cystic fibrosis say a new life-extending drug which will be available on the NHS is “the best news we’ve had for years”.
The family of a "wonderful daughter" whose life could be tragically cut short by cystic fibrosis say a new life-extending drug which will be available on the NHS is "the best news we've had for years".
Oli Burnside said the news he and his wife Kate's daughter Freya had the genetic disorder - which affects one in 9,000 people and causes sticky mucus to build up in the lungs, shortening life expectancy - meant "our world was torn apart".
Even though they say the eight-year-old is a "proper fighter", they have to live a daily battle to protect her from illness - as children with CF are more likely to develop lung infections.
Mr Burnside, who co-manages The Plough and Sail and The Golden Key pubs in Snape with his brother Alex, also said the predicted life expectancy of CF sufferers of between 35 and 40 was "the hardest part of all".
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But now NHS England has confirmed that it has reached a deal with Vertex Pharmaceuticals to make the drug Orkambi available to CF sufferers free of charge, with the medication set to be available in less than 30 days.
"It is the best news we've had for years," said Mr Burnside, who is hosting a Meet Up Mondays event at The Golden Keys on Monday, October 27 to help raise money for CF, alongside supporting older people.
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"What it will do for Freya is create a better lung function for her. Everything looks so much more positive than it did a few days ago.
"People who have been campaigning should be proud that what they've done has really made a difference."
Mr Burnside said "our lives changed forever" when Freya was diagnosed with CF at just three months old.
She undergoes daily physiotherapy to help clear mucus from the lungs and keep her healthy - but the family also have to be careful about making sure Freya is not exposed to potential germs and illnesses.
"My wife and I have tried to let her be as much of a child as she can, but we have to keep a close eye on her," he said.
"You can go out but you've got to be careful. You've got to be as careful as you can with everything.
"We try and avoid confined spaces - you can never be quite sure what bugs that she can catch and develop something more serious. They can attack the immune system and get into her lungs.
"Daily life is just the way it is when you're a CF parent. You learn every day a little bit more about it."
There is no cure for CF, so people with the condition may need to take different medicines every day to help digest food and treat and prevent lung problems, as well as undergo daily physiotherapy, antibiotics and take enzyme tablets with food.
Taking medication can be difficult for any child but Mr Burnside said Freya is "very good with taking her medication because she knows it keeps her well".
He added: "As a child away from CF she is a proper fighter and a wonderful daughter. She's now getting to a stage of understanding how she is different from other people.
"The hardest part is not knowing the future. Cancer is a horrible disease, but a lot of people survive and go on to live a long and healthy life.
"But CF is not like that. Your child's life is only going to get worse. It's a constant struggle every day."
Mr Burnside also said it was a difficult choice for he and his wife to have a second child, Spencer.
"It was a very hard time to decide to have a sibling for her in the first place," he said.
Around 5,000 NHS patients will have full access to Orkambi following an agreement between Vertex Pharmaceuticals and NHS England.
Health secretary and West Suffolk MP Matt Hancock said giving the green light to Orkambi was the "proudest moment of my career as health secretary and a joyous day for thousands of patients with cystic fibrosis".
Shortly after the announcement, he Tweeted: "I know personally how important this is - and I've met many sufferers. Things like this are the reason I do this job.
"For those who say the system doesn't work, this proves them wrong. We can get lifesaving drugs at a fair price, thanks to Nice and the NHS. We should all reject calls to undermine that."