Little Amélie McIntrye, of Morley, has faced a constant battle to stay healthy since she was diagnosed with the genetic condition when she was just three-and-a-half weeks old.
Cystic fibrosis (CF) is currently incurable and degenerative. Of those who died from the condition in 2019, the median age of death was just 31.
It causes a build-up of thick, sticky mucus in the lungs, digestive system and other organs and anything which leads to a serious infection, or for the lungs to stop working properly, can prove fatal.
Amélie’s daily treatment regime involves a cocktail of drugs to help ward off infections, exercise, physio to clear her airway - involving someone pounding her chest - and wearing a PEP mask, which uses pressure to help clear mucus in her lungs.
Despite this, her family say she “never complains” and her bravery has now inspired family friend Oliver Harrison to pledge to run 100 miles in 24 hours for the Cystic Fibrosis Trust next year.
The 39-year-old dad-of-two will take part in Endure24, a 24-hour running race at Bramham Park in July, where he will attempt to complete 20 laps of the five-mile route within the 24 hour period.
Although a keen runner, he has up to now only run half-marathon distances and admitted the challenge - just one mile short of the 101 miles in 24 hours recently achieved by ex-Rhinos captain Kevin Sinfield in aid of his former teammate Rob Burrow - will be “pretty difficult”.
But he said he will be spurred on by thinking of Amélie and all she has to go through.
He said: “You never hear her complain. She’s full of fun and full of life. She just gets on with it.
“If she can get on with what she has to go through and everything she does, it feels like an adult like myself should go the extra mile to do what we can do to support kids like Amélie.”
Amélie’s mum Natasha, 40, said she was “blown away” by Oliver’s challenge and his support for the cause.
“Everyone recognised what an incredible feat it was for Kevin Sinfield, a professional athlete with a team of physios and supporters accompanying him on different legs of his challenge, to complete for his friend.
"It's incredible that Oliver, a teacher who enjoys running, who is not a professional athlete, and who will be running on his own without a team of technical support during his challenge, wants to do that for us.”
Natasha and her family have themselves raised in the region of £15,000 for the CF Trust since Amélie’s diagnosis, which came following the routine heel-prick test all newborn babies are given.
Natasha recalled her shock at hearing the news.
“We were devastated. Amélie was an IVF baby. We hadn’t told anyone we were pregnant in case anything went wrong.
“When she finally arrived, everything seemed to be OK so we obviously told people we had a baby.
“And then a few weeks later we had to start telling people that she had cystic fibrosis.”
Natasha said at the time it felt like a “life sentence” for Amélie but, having been put under the immediate care of the CF clinic at Leeds Children’s Hospital, the family were told about new drugs available - and in the pipeline - which could improve her quality and length of life.
Amélie - who learned to swallow tablets at the age of three - currently has to take daily doses of antibiotics, CF-specific drug Orkambi every 12 hours, as well as medication to help her digest food every time she eats, on top of half an hour of exercise every day and an hour of intensive physiotherapy.
Natasha said: “Everyone knows that image of someone hitting a [CF] child’s back and chest to cough up the mucus.
“We had to start doing that when she was still tiny. She was 5lb 2oz.
“It was horrible. It was awful. I just used to cry through physio when she was a baby.
“It didn’t hurt her - it never has hurt her. But it’s just that thought you have to sit and hit and pound on your baby’s chest.
“And just the sound it makes. It was just horrific.”
But even with the treatment, the world can still be a dangerous place for Amélie, and others with CF.
Common coughs and colds carry potential infection risks and certain environments, such as mud, stagnant water and rotting leaves can harbour bacteria and fungi that can be extremely harmful.
Amélie has twice contracted pseudomonas - the biggest killer of people with cystic fibrosis - and beaten it, each time involving a year of extra treatment, with a twice-daily nebuliser, more antibiotics and a boosted exercise and physio regime.
Natasha said: “We’re really just trying to fight all the time to keep her healthy so she doesn’t get a chest infection because that’s when it’s a deteriorating situation.”
The family all shielded during the Covid-19 lockdown - and despite Natasha and husband Steven, 35, who work as teachers, both contracting the virus twice during the pandemic, Amélie has luckily managed to avoid it, along with brother Nathaniel, four, who doesn’t have CF.
“When we had to shield, she found that difficult. That was the first time she had started saying how unfair it was. But she dealt with it pretty well.”
She added: “She doesn't complain. If it affects her, she doesn't give that away. She just gets on with it and you can’t ask for any more than that. She doesn’t feel ‘woe is me’.”
The drug Amélie is currently taking, Orkambi, is said to extend life expectancy to around 50 but the family hope she will soon have access to a new drug, Kaftrio, which it is hoped leads to a “near normal” life expectancy.
Natasha said, for Amélie, this is all because her genetic fault is the most common form of cystic fibrosis.
But for others, whose CF is caused by different genetic faults, much of the research, drugs and medical breakthroughs are not applicable.
Natasha said this is one of the reasons she fundraises for the CF Trust - to help boost funding for those less fortunate.
“For Amélie, [the future] should be bright.
“But it’s just for those other people that don’t have the same gene as her. I was talking to parents - at a CF parents’ evening - of a 15-year-old girl who had 17 per cent lung function and she wasn’t eligible for any of those drugs because she had the wrong gene type.
“There are still so many other people that are struggling.”
To sponsor Oliver in his Endure 24 challenge in aid of the CF Trust, visit, https://justgiving.com/fundraising/oliver-harrison16Support the YEP and become a subscriber today. Enjoy unlimited access to local news and the latest on Leeds United. With a digital subscription, you'll see fewer ads, enjoy faster load times, and get access to exclusive newsletters and content. Click here to subscribe