Abstract
Introduction: Cystic fibrosis (CF) is associated with a high respiratory symptom and treatment burden. Elexacaftor/tezacaftor/ivacaftor (ETI) provides substantial improvements in physiological outcomes such as respiratory function and sweat chloride. This study sought to comprehensively examine an area of limited research in this field: the lived experiences of adults with CF after ETI initiation. METHODS: Adults with CF completed semi-structured interviews at least 6 weeks following the initiation of ETI. Participants shared their experiences regarding respiratory-related symptoms, airway clearance routines, and their capacity to participate in physical activity. Interviews were audio-recorded, transcribed verbatim and analysed using the Framework Method. RESULTS: Seventeen participants completed interviews (FEV(1) range before ETI initiation 31 to 87 % predicted). All participants reported improvements in respiratory-related symptoms including cough, sputum expectoration, dyspnoea, and chest tightness. As a result, there was a reduction in participants airway clearance treatment burden and improvements in their capacity to participate in physical activity, health-related quality of life and outlook for the future. The overall theme highlighted in this study was that ETI had "completely changed everything" and they can now do "everything I wasn't able to do before" due to the reductions in symptom and treatment burden. CONCLUSION: Participants consistently described their experiences with ETI in positive terms. The results of this study confirm that ETI is in fact perceived to be a "miracle drug" and has had a transformative effect on the everyday lives of adults with CF through improved HRQoL and overall wellbeing, which led to positive views regarding their future with CF and ongoing medical care.
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