Sophie Pierce is a courageous woman on a mission. In January 2025, the 30 year old from West Wales will be setting off in a rowing boat — with her two teammates, Janine and Polly — to cross the Atlantic Ocean from Lanzarote to Antigua. It’s an ambitious challenge for anyone, but all the more inspiring because Sophie has the genetic disorder, cystic fibrosis.
If Sophie completes the row, covering 3,200 miles in around 50 days, she’ll set a new world record for being the first person with cystic fibrosis to row any ocean.
“I have to keep my lungs as healthy as possible which naturally means doing exercise,” she told the Pembrokeshire Herald. “But salt water is particularly good as it helps to clear the lungs of mucus. And research has proven that surfers who have cystic fibrosis have excellent lungs because they are exposed to salt water on a regular basis.”
For someone with a chronic lung condition, preparing for a challenge like this is a massive undertaking, requiring months of planning and a rigorous, multifaceted training regime.
Sophie has been steadily building up her fitness, supported by physiological testing similar to what elite athletes do, with the help of a team of health professionals. These include specialist physios, dieticians, and her Consultant in Respiratory Medicine and Cystic Fibrosis, Dr Charlotte Addy, at NHS Wales.
“What’s interesting is Sophie’s always presumed it’s her lungs that limit her when she’s training,” explains Dr Charlotte Addy, “but because of this more involved physiological testing, we’ve been able to look at the effect her muscle limitations have had [on her body], and her cardiac limitations, and then change her training to build her up.”
Sophie is working with dieticians to ensure she eats well and gets essential nutrients, a key focus for people with cystic fibrosis due to the effect of the condition on digestion and the functioning of the pancreas.
Cystic fibrosis, which affects 10,900 people in the UK, is a genetic condition that causes a build-up of thick sticky mucus in the lungs, digestive system and other organs. This can have a wide range of effects on the body.
“She’s learning about her condition as she trains, and moving into zones she’s never trained her lungs into before,” says Dr Addy. “We’ve spent a long time talking about the impact of cystic fibrosis on her lungs. Because if you’re out in the middle of the Atlantic Ocean and there’s a problem, you’ve really got to understand your condition.”
Sophie would never have imagined taking on such an epic challenge before the introduction of new, game-changing disease modifying drugs, which became available in 2020. They have transformed the lives of many respiratory patients, helping them live more freely and independently.
“Sophie’s challenge is a great example of how we can help people do things they didn’t think they could do,” says Dr Addy. “Innovation is not just about fantastic new medications. It’s also the multi-professional care and self-management. She couldn’t possibly achieve this if she didn’t have the skills and the ability to look after herself,” she explains. “She set out to raise money but has ended up learning so much about her condition, and what her body can achieve.”
Dr Addy believes this is key for patients with all kinds of chronic lung disorders to live a richer, fuller life — having the confidence to push themselves in their physical fitness comes with understanding their body, through self-monitoring, and being supported by a multi-disciplinary team.
“If you understand your condition, it doesn’t limit you,” she says. “Just being able to monitor and manage your condition more effectively and feeling confident in doing that, increases what you can achieve.”
It’s an exciting time in respiratory care, with innovations in models of care and access, and support for self-management through apps and remote monitoring.
“We have a study called ‘Project Breathe’ where patients track their activity with a Fitbit; they can measure things like their weight and height at home, and to a degree keep an eye on themselves. They then contact us when they feel they need to be seen, rather than us relying on set time-points.”
Ultimately it’s moving away from traditional, routine appointments, to more patient-initiated care, supported by digital platforms.
“For younger patients, who are trying to juggle lives and children and working patterns, what they really want is accessible care when they need it — reactive care that can be stepped up or down depending on how their condition is going. We want to promote that self-management so people can manage their own systems.”
The role of digital tech in respiratory care
Digital tech is clearly central to the future of respiratory care, but what’s crucial is that the tech is co-designed with patients, to ensure their needs are the heart of it, says Dr Addy. After all, “all these things that are possible will only be possible if people want to engage with them.”
NHS Wales has a respiratory app that allows patients to track their own symptoms, and gives them advice on when to phone for help, and what to do to step up or down their treatment.
Of course, AI is now coming into play, too. The ‘Project Breathe’ research, for example, will use AI to drill through data to then help practitioners pick out the markers of a chest infection early, so action can be taken sooner.
“I’ve found — actually through my own PhD thesis — that people can respond quicker than we think to treatment” says Dr Addy. “And that those markers of response might not be the traditional measures we’ve always used, like lung function. Actually, it’s things like step count, heart rate, activity levels, sleep… these are a really good measure of people becoming unwell, and are very easy for us to monitor now. This for me is one of the big innovations, because of how widely applicable it’s going to be for all sorts of different patients.”
Virtual leisure centres
Older patients are on board with the tech too, says Dr Addy. “I think sometimes we underestimate them, in terms of their engagement with these platforms. We’ve seen lots of people embrace virtual consultations, for example. Lots of our older patients have iPhones and iPads and Fitbits now.”
“We also have a virtual leisure centre that we’re using, as part of Beam, (a specialist platform offering access to programs, classes, and community support). Here, people can do virtual spinning classes, one-on-one exercise sessions, tailored videos specific to their condition. Our patients are finding all of these things really helpful.”
“This focus on allowing people to manage their own condition, to have access to what they need to support their self-management, when they need to access it — is so important. We’re breaking away from rigid structures and traditional methods of care, and that’s the most innovative thing we can do.”
Understanding chronic respiratory diseases
Chronic respiratory diseases are the third largest contributor to mortality in the UK. ITN Business’ upcoming programme, ‘Breathing Better Together’, produced in partnership with Respiratory Futures, looks at how exposure to the drivers of health inequalities impact respiratory disease and lung conditions in the UK, and what initiatives can be implemented to help reduce this burden on what is often the most vulnerable parts of our community.
The past few years have highlighted some of the challenges in respiratory care, including pressure on the healthcare workforce, wait times to access services, and the appropriateness of the current structure of services.
Explore our newly launched programme, Breathing Better Together, where you can find interviews, insights and more inspiring stories within the realm of respiratory care.