"An Amazing Transformation!" The Lived Experiences of Elexacaftor/Tezacaftor/Ivacaftor in Adults with Cystic Fibrosis.

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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 (FEV1 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. </p>.