Patient. 2026 Mar 21. doi: 10.1007/s40271-026-00806-5. Online ahead of print.
ABSTRACT
BACKGROUND: Cystic fibrosis (CF) is a progressive and multi-organ genetic condition most renowned for reduced lung function. The recent introduction of elexacaftor/tezacaftor/ivacaftor (ETI) has transformed physical health and life expectancy for many people with CF.
OBJECTIVES: This mixed methods study aimed to compare levels of physical health and self-concept before and after ETI commencement and explore lived experiences of ETI impact on self-concept: an individual’s overarching sense of identity.
METHODS: This study applied a mixed methods approach, using qualitative data to enrich and augment the insights gained by the quantitative data. Longitudinal quantitative data were collected from 20 adults to compare mean lung function, frequency of pulmonary exacerbations, body mass index (BMI), and self-concept scores before and after ETI commencement. Qualitative data were collected from 13 of these adults through in-depth, unstructured interviews, which were thematically analysed to identify key insights.
RESULTS: Descriptive and comparison statistical analysis demonstrated a significant increase in mean lung function and a significant decrease in mean frequency of pulmonary exacerbations following ETI commencement. No significant difference in BMI was identified. While mean self-concept scores before and after ETI commencement were not significantly different, thematic analysis of interview data highlighted that many participants had experienced self-concept recalibration across physical, social, and psychological domains of life related to ETI. The overarching theme of Self-concept recalibration and six subsequent themes emerged: Physical self: A ‘new’ CF and Weight gain – friend or foe; Social self: The gift of time and Goals and possibilities; and Psychological self: from Surviving to thriving and Into the unknown.
CONCLUSIONS: Findings highlight significant physical health improvement for many people with CF, and the potential for ETI to positively impact self-concept. However, challenges and nuanced self-concept experiences were also identified.
PRACTICE IMPLICATIONS: Integration of a self-concept approach to CF care is recommended.
PMID:41865142 | DOI:10.1007/s40271-026-00806-5
