Infection and inflammation, structural damage, and functional changes, is present early in people with Cystic Fibrosis. Periods of increased signs and symptoms (pulmonary exacerbations) can lead to poorer outcomes. Scheduled outpatient appointments allow for snapshots of health within a rigid system that can be cumbersome for families. Also, there is no biomarker which predicts a pulmonary exacerbation. Home monitoring and sample collection may provide an option to identify periods of pulmonary exacerbation earlier, however it is unclear if this is feasible or could have a negative impact on depression, anxiety, or quality of life.

Here I aimed to investigate the feasibility of home monitoring and sample collection for young people with CF and their parents and the impact on mood and quality of life while also exploring the accuracy of home spirometry and novel biomarkers.

With CF patient/ family input, I designed and tested a study specific mobile phone application with integrated Bluetooth equipment and led a multi-site, two country study. 144 participants were recruited and provided data for 6 months and had the option to collect a range of biological samples for biomarker exploration over the last 4 months.

Median requested data completion was 40.1%, significantly lower in adolescence. No negative impact on mood or quality of life was seen. The underread of unsupervised home spirometry compared to supervised clinic spirometry is an important clinical message to be considered by CF teams moving forward. Urinary MMP-9 did not demonstrate utility as a biomarker of pulmonary exacerbations. Signals from a large panel of urinary biomarkers were highly variable both between and within subjects...