A cystic fibrosis patient whose health has skyrocketed thanks to a miracle pill is now remembering her lost sister, who tragically died from the cruel disease before receiving the drug.
Devoted siblings Sophie and Danielle Jones, from Telford, Shropshire, were both born with CF, which in the days before the new NHS miracle drugs were introduced could dramatically shorten lives by clogging lungs with sticky mucus.
In 2017, then 16-year-old Sophie and seriously ill 19-year-old Danielle were given dream days by the Rays of Sunshine charity: Sophie had a pamper session, while Danielle had Geordie Shore reality TV stars Holly Hagan and Charlotte met Crosby.
Later that year, after being on the lung transplant list for two years, Danielle passed away – but luck protected her grieving sister when miracle drug Kaftrio became available on the NHS in 2020 following a campaign by the Daily Express.
Now Kaftrio has seen 23-year-old Sophie’s health improve to the point where she was able to run for Rays of Sunshine at the Birmingham Half Marathon in May, in memory of her sister – and the wonderful memories she made shared together.
Devoted sisters Danielle (left) and Sophie Jones, who were both born with cystic fibrosis (Image: Handout)
Sophie told the Daily Express: “I visit Danielle’s grave about once a month now and tell her all the news.”
“She was the first person I told about taking part in the half marathon; the first person I told about my new job in customer service, and I will go straight to her grave after the race to tell her how I did it.”
“Although we have other siblings that we love very much, Danielle and I literally couldn’t have been closer, we are best friends and sisters.”
“She was three years older than me, an age gap that usually exists between sisters – but because we had both inherited CF from our parents, we knew how the other felt, how exhausted and depressed we were.”
“Before Kaftrio I spent a week or two in hospital several times a year – I haven’t been there since November when I had chest pains and needed antibiotics.
“But I know that 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 hard – there are days when I struggle to breathe.”
“But going on Facebook to talk to other people with CF about Kaftrio is incredible, hearing their stories and supporting each other as we take on this new opportunity in life.”
“Whenever life feels hard, I remember what Danielle would say to me, that she gave everything to be where I am. Every step of the Birmingham Half she will be by my side, telling me to keep going.” to never give up.
Sophie at her older sister Danielle’s bedside in the hospital (Image: Handout)
Danielle and Sophie’s parents were shocked to learn she had CF weeks after their eldest daughter was born, as they did not know they were carriers of the faulty gene.
Three years later, Sophie was also born with the genetic condition and at that point there were no medications available to relieve the symptoms other than antibiotics and physical therapy.
But the CF bond the sisters shared bonded them incredibly together and meant they were able to support each other as their health deteriorated.
Sophie explained: “No one else can ever really know what it’s like to be told as a child that not only are you unlikely to live to 30, but your health will decline and you won’t have the same freedoms and experiences as you yourself.” Friends at school.
“Of course, we both put on brave faces as best we could and fought through it because we didn’t have much choice, but with lungs constantly clogged with mucus, every day meant a lot of medication, physical therapy and nebulizers around our airways to free up.
“And that was a good day – every few weeks we were in the hospital for at least a week, needing intravenous antibiotics and more to get back on our feet and functioning again.”
“And every year it got worse, a reminder that this was actually a degenerative disease for which there was no cure.
“My first memories of cystic fibrosis are from when I was about 4 years old, when I had to take these massive pills to digest every meal – the mucus that clogs the duct of my pancreas that normally provides the enzymes, that other, healthy people take for granted.”
“Soon I was no longer able to take part in simple activities such as playing sports or even running around the playground with friends, with regular fortnightly visits to the Princess Royal Hospital in Telford, hours of physiotherapy every day at home and nebulisers – medical devices” which provide the medication The substances you need to decongest your lungs turn into a mist so they can be absorbed into your body.
Now on kaftrio, healthy Sophie Jones, 23, is running a half marathon for her sister (Image: Handout)
Normally, those affected by cystic fibrosis are not allowed to be in the same room as other sufferers, as the mixing of the different pathogens in their lungs can be harmful to their health.
But because the sisters lived in the same house and had the same infections, they were always able to be close and support and hug each other.
Sophie told us: “We lifted each other up, we were always there for hugs. We were each other’s lifelines.”
“But when Danielle was about 16, she moved to the Royal Stoke Hospital, where adults with CF were treated, which meant we weren’t together as we were in hospital at the same time. And it was about that time that she really started to go downhill.
“It was also around this time that Rays of Sunshine approached us to make a wish – our original wish, of course, would have been a cure for CF, but that was beyond their control, so Danielle asked for a clothing shopping day followed by a meeting with Hollie.” and Charlotte from Georgie Shore.
“I had a pamper day, bought clothes and then did a professional photoshoot, which was amazing! We both visited each other’s day, which was lovely and really gave us a boost when we didn’t need it anymore.”
“What the organizers probably didn’t fully understand was how sick Danielle was at the time.”
Danielle spent most of the next year at Royal Stoke, where Sophie visited her and cuddled with her on her bed. Watching the life slip away from her was horrific, especially considering how much of a fighter she was.
She continued: “She was a mirror for my future, for what I wanted to do.”
“When she died in November 2017, my mother and father were by her side – my stepfather and I got the call and rushed to her, but we were too late.”
“The funeral took place on December 17th in a church near our house and was completely overcrowded, with hundreds of people pouring into the grounds because there was no room inside.
“It took a long time and seeing Danielle no longer in pain was a blessing – but we all knew we were leaving a hole in our hearts that would never heal.”
The frightened Sophie now began to fear that her health would be similar to that of her sister and that she too would deteriorate rapidly.
She said: “I was 17 at the time and although I wasn’t unwell enough to be on the transplant list like my sister, I was regularly bedridden, had a complete loss of appetite, couldn’t breathe and was constantly coughing.”
“It became almost impossible to socialize and I regularly had to take weeks off at my new job in a laundry. Without Danielle with me, I felt like I had no one to talk to and hug when I needed it so much.”
Danielle (left) has fun with her little sister Sophie (Image: Handout)
But after two years of campaigning with the community, the Daily Express revealed in 2020 that the NHS had struck a deal with US pharmaceutical company Vertex for their miracle pill Kaftrio – hailed as being “like a cure” for CF.
Now she can work full time at Kaftrio, socialize and even exercise in ways she never thought possible.
So bravely Sophie is now tackling this year’s Birmingham Half Marathon to raise money for the charity that made both of their dreams come true – and says she will be thinking of her older sister every step of the way.
She added: “Within a few weeks I felt much better, with clear lungs and lots of energy. I was able to take long walks without having to take breaks, and that summer I felt like a new person.”
“What was supposed to be euphoric was tinged with guilt for benefiting from this amazing new drug when Danielle never got the chance.
“With my physical fitness, I increased my confidence, socialized, and essentially lived a fulfilling life that everyone else takes for granted. According to reports, at Kaftrio there was no reason why you couldn’t live to be 60 or 70, twice what I had previously expected.” .
“One day I sat next to Danielle’s grave and told her the news – and knew she wouldn’t be mad, she would be so happy for me. She would tell me, ‘Just go out, run, live your life.’ “For me” it is what I have done – and what I will always do.”