A cystic fibrosis sufferer with soaring health thanks to a wonder pill is now running in memory of her lost sister – who tragically died from the cruel condition before getting the drug.
Devoted siblings Sophie and Danielle Jones, of Telford, Shropshire, were both born with CF which could dramatically shorten lives by clogging up lungs with sticky mucus in the days before new NHS miracle medications.
In 2017 Sophie, then 16, and seriously-ill Danielle, 19, were gifted dream days out by charity Rays of Sunshine – Sophie having a pamper session while Danielle got to meet ‘Geordie Shore’ reality TV stars Holly Hagan and Charlotte Crosby.
Later that year, after two years on the lung transplant list, Danielle passed away – but luck shielded her grieving sister as in 2020 wonder drug Kaftrio became available on the NHS after a campaign by the Daily Express.
Now Kaftrio’s seen 23 year-old Sophie’s health soar enough for her to run the Birmingham Half Marathon in May for Rays of Sunshine in memory of her sister – and the wonderful memories they shared together.
Devoted sisters both born with cystic fibrosis, Danielle (left) and Sophie Jones (Image: handout)
Sophie told the Daily Express: “I go and visit Danielle’s grave about once a month now, and tell her all the news.
“She was the first person I told about doing the half marathon; the first person I told about my new job in customer services, and I’ll make a beeline to her grave after the race to tell her how I did.
“While we have other siblings who we love dearly, Danielle and I literally couldn’t have been closer, the best of friends as well as sisters.
“She was three years older than me, an age gap which normally separates sisters – but because we’d both inherited CF from our parents, we knew how the other felt, how exhausted and down we were.
“Before Kaftrio I’d spent a week or two in hospital multiple times a year – I haven’t been since November when I was chesty and needed antibiotics.
“But I know the more I run, the less my CF will affect me. If that’s not an incentive to start running, I don’t know what is! It’s still tough – there are days when I struggle to breathe.
“But going on Facebook to talk to other people with CF on Kaftrio, it’s incredible to hear their stories, to support each other as we grab this new chance at life.
“Whenever life feels tough, I remind myself what Danielle would say to me, how she’d have given anything to be where I am now. Every step of the Birmingham Half she’ll be by my side, telling me to keep going, never to give up.”
Sophie at her older sister Danielle’s bedside in hospital (Image: handout)
Danielle and Sophie’s parents were shocked when told weeks after the eldest daughter’s birth that she had CF, as they were unaware they were carriers of the faulty gene.
Three years later Sophie was also born with the genetic condition and at that time there were no existing drugs to alleviate the symptoms except for antibiotics and physiotherapy.
But the CF bond the sisters shared made them incredibly close and meant they were able to support each other as their health went up and down.
Sophie explained: “Nobody else can ever really know what it’s like being told as a child that not only will you be unlikely to make it to 30, your health will plummet so you won’t have the same freedoms, the same experiences as your friends at school.
“Of course, we would both put on brave faces as much as we could, we’d struggle through because we didn’t have much choice, but with lungs constantly congested with mucus, every day meant a load of meds, physio and nebulisers to clear our airways.
“And that was a good day – every few weeks we’d be in hospital for at least a week needing IV antibiotics and more to get us horizontal and functioning again.
“And every year it got worse, a reminder that this really was a degenerative disease without any cure.
“My first memories of CF are when I was around 4 when I had to take these massive pills to help me digest every meal – the mucus blocking the duct from my pancreas which normally provides the enzymes other, healthy people take for granted.
“Soon I couldn’t take part in simply activities like playing sports or even running around with friends in the playground, with regular two-week visits to the Princess Royal Hospital in Telford, home physio every day for hours, and nebulisers – medical devices which turn the medication you need to decongest your lungs into mist so it can be absorbed into your body.”
Now on kaftrio, healthy Sophie Jones, 23, is running half-marathon for her sister (Image: handout)
Normally CF sufferers are unable to be in a room with anyone else with the condition as mixing the different bugs in their lungs can prove hazardous for their health.
But as the sisters lived in the same house and shared the same infections it meant they could always be close and support and hug each other.
Sophie told us: “We’d pick each other up, we’d always be there for hugs. We were each other’s lifelines.
“But when Danielle was around 16, she moved to the Royal Stoke Hospital, where they treated adults with CF, which meant when we were hospitalised at the same time, we weren’t together. And it was around then that she really started to go downhill.
“Around then we also got approached by Rays of Sunshine to submit a wish – of course our real wish would have been a cure for CF, but that wasn’t within their powers, so Danielle asked for a clothes shopping day followed by meeting Hollie and Charlotte from Georgie Shore.
“I had a pamper day, clothes shopping, then a professional photoshoot, which was amazing! We both attended each other’s day, which was lovely, and really gave us a boost when we couldn’t have needed it more.
“What the organisers probably didn’t fully get was how sick Danielle was then.”
Danielle spent almost all the next year in Royal Stoke, where Sophie would go and visit her, cuddling up with her on her bed. Seeing the life drain from her was horrific, especially knowing how much of a fighter she was.
She went on: “She was a mirror for my future, what I had in store.
“When she passed in November 2017, she had my mum and dad there by her side – my stepdad and I got the call, and rushed to see her, but we were too late.
“The funeral was on 17th December in a church near our home and it was absolutely rammed, with hundreds of people spilling out into the grounds because there was no room left inside.
“It had been a long time coming and to see Danielle not in pain anymore was a blessing – but we all knew we’d be left with a hole in our hearts that would never heal.”
Scared Sophie now started to fear that her health would mirror her sister’s and she too would start to rapidly deteriorate.
She said: “I was 17 then, and while I wasn’t deemed poorly enough to be on the transplant list like my sister, I was regularly bedridden, with a complete loss of appetite, unable to breath, constantly coughing.
“Socialising in any way was becoming almost impossible, and I regularly had to take weeks off work at my new job in a laundry. Without Danielle with me, I felt I nobody to talk to, to hug when I needed one so badly.”
Danielle (left) having fun with little sister Sophie (Image: handout)
But after two years of campaigning with the community, in 2020 the Daily Express were able to reveal that the NHS had struck a deal with US drug firm Vertex for their wonder pill Kaftrio – hailed as “like a cure” for CF.
Now on Kaftrio she’s able to work fulltime, socialise and even exercise in a way she never dreamed possible.
So gutsy Sophie’s now taking on this year’s Birmingham Half Marathon to raise money for the charity which gave them both their dream wishes – and says every step of the way she’ll be thinking of her older sister.
She added: “Within weeks I felt so much better, with clear lungs, and loads of energy. I could take long walks without needing to stop for breaks, and by that summer I felt reborn.
“What should have been euphoric felt tinged with guilt, that I was benefitting from this amazing new drug when Danielle never got the chance.
“With my physical fitness came increased confidence, socialising, basically living a full life that everyone else takes for granted. Reports indicated that with Kaftrio, there was no reason you couldn’t live to 60 or 70, twice what was on my cards before.
“I sat next to Danielle’s grave one day and told her the news – and knew she wouldn’t be angry, she’d be so happy for me. She’d tell me: ‘Just get out there, run, live your life for me’, so I that’s what I did – and what I intend to always do.”
* To help Sophie fundraise for ‘Rays of Sunshine’ visit https://ajbellgreatbirminghamrun2024.enthuse.com/pf/sophie-jones