„conneCT CF - Coaching and Telemedicine in Cystic Fibrosis Patients“

Lung disease severity is a key determinant of morbidity in pwCF, despite CFTR-modulator therapies (CFTRmt). We hypothesized that digital technologies to support adherence to inhalation therapy could improve lung health and QoL in pwCF. Multicenter, randomized controlled trial in pwCF aged ≥12y over 18mon. Participants were assigned to either a control group (CG) receiving standard care or an intervention group (IG) using a data-tracking nebulizer, home spirometry, and a self-management app (PARI Connect). The app visualizes adherence and home spirometry results for pwCF and provides a web portal for physicians. The intervention also included telephone coaching and video-conferencing. A total of 296 pwCF (148 IG, 148 CG) were included. Baseline characteristics were similar between IG and CG, with a mean age of 31.3y (SD 10.8) and a mean ppFEV1 of 70.2% (SD 24.7), with a ppFEV1 <40% in 11.1%. Infection with P. aeruginosa was present in 156 participants (52.7%), and 252 (85.1%) received a CFTRmt (>90% elexacaftor/tezacaftor/ivacaftor). At the start of the intervention, when IG participants were already using the app (without full intervention components), adherence to nebulizer therapy was higher in IG (58%) compared to CG (45%). Adherence remained higher in IG with 52% in IG and 38% in CG at study’s end. PwCF with a ppFEV1 <40% had a 2.4-fold higher risk of a pulmonary exacerbation (PEX) vs. those with higher ppFEV1. An increase of 10pp in adherence was associated with a significantly reduced risk of a PEX (-9%). The innovative care significantly improved adherence to inhalation therapy. The link between higher adherence and reduced PEX risk highlights the importance of inhalation therapy for pwCF with chronic lung disease, even in the CFTRmt era.