Cendl Xanthe, Lead Research Nurse at the All-Wales Adult Cystic Fibrosis Centre, talks about her remote health monitoring study for people with Cystic Fibrosis (CF), and the importance of people with rare diseases taking part in clinical trials.

Project Breathe is a remote monitoring study that started at the All-Wales Adult Cystic Fibrosis (CF) Centre, University Hospital Llandough, in the summer of 2020. It aims to investigate whether equipment and monitoring software can be used to change the way that care for CF patients is delivered. There’s currently no cure for cystic fibrosis, although a number of treatments are available to help control the symptoms, prevent complications and make the condition easier to live with.

The study supplied a lung function monitor, activity monitor, oxygen saturation reader, scales and thermometer which connect via Bluetooth to a dedicated, free to use app on both android and apple devices. Subjective scores of cough and wellness were also added, and the app is able to track health events and medication use to add to the overall, holistic overview of the patient.

The study had an inclusive eligibility criterion, meaning we were able to approach almost all people in our care to offer them this exciting opportunity, with the target recruitment being 100 people (1/3 of our adult patient population). People with CF were able to take part regardless of their genetic mutation, age, disease severity or socio-economic background as all the required equipment was supplied. Set up was supported by research nurses and ongoing support was supplied via email, phone or in person, to encourage engagement and overcome barriers to inclusion such as technological ability and living distance from the centre.

Remote support proved popular with those involved and the team were able to run support groups and gain valuable feedback using online platforms. Using Project Breathe enabled remote care to be possible throughout an uncertain time and beyond, helping to accelerate care nearer to home to become more of a possibility for those we care for. The dashboard developed by the project team connects care teams to patient data, allowing for timely review of health trends both face to face in clinic and virtually. People with CF who regularly entered data are also able to review their own lung function, weight, and wellness trends amongst others, promoting a sense of ownership over their own data and health outcomes.

Pulmonary exacerbations in CF patients, which generally entail an increase in respiratory symptoms accompanied by a decrease in lung function, can be spotted earlier and reported to the clinical team to allow for early intervention, reduced hospital inpatient stays and prompt diagnosis and treatment.

Overall, project breathe has been a positive experience for both those participating and healthcare staff caring for people across Wales with CF. The project goes from strength to strength and is ever evolving by listening to those using the app to improve and add features, as well as developing machine learning algorithms to predict exacerbations before clinical symptoms present.

It's very important that people with rare diseases are able to take part in research to improve their care. People sharing their experiences in a trial can help other patients and the NHS to learn what works best in managing a condition. I would encourage anyone with a rare condition to take the opportunity to participate in clinical research like Project Breathe if they can.