(283) Expectations and experiences of adolescents with cystic fibrosis: influence of Elexacaftor/Tezacaftor/Ivacaftor on treatment adherence and identity

Background: Clinical trials and real world studies have demonstrated the positive impact of CFTR modulator therapies on respiratory function, nutritional status and quality of life for those living with CF. To date, there is limited knowledge on the lived experience of adolescents on these new modulator therapies. The aim of this study was to explore the lived experience of young people on Elexacaftor/Tezacaftor/Ivacaftor, to understand how they feel about having CF, and how it has impacted their adherence to both new and existing treatments.

Methods: Participants were recruited from RECOVER, a multi-centre real world post-marketing study in Ireland and the UK, approximately one year following commencement of Elexacaftor/Tezacaftor/Ivacaftor. This qualitative study explored the lived experiences of seventeen adolescents in Ireland (52.9% male; mean age= 13.8) with a semi-structured interview. Interview data were analysed using reflexive thematic analysis. Demographic and health variables, for example past modulator use, comorbidities, FEV1 markers of lung function, BMI, and objective adherence data were collected from patient records. The Patient Health Questionnaire (PHQ-9), General Anxiety Disorder (GAD-7) scales and the psychosocial domains of the Cystic Fibrosis Questionnaire-Revised (CFQ-R) were used to assess psychosocial adjustment, as well as three 10-point scale items measuring: importance of new CFTR treatment, importance of maintaining existing treatments, and the confidence to carry out treatments.

Results: Initial analysis of the quantitative measures suggest this cohort have improved psychosocial adjustment compared with other recent studies in CF populations, however the levels of anxiety and depression remain higher than a typical adolescent population. Qualitative findings are being established using reflexive theme analysis, with initial themes pointing toward defence mechanisms, changing identity and changing family roles, and how these have an impact on the adherence behaviours and psychosocial adjustment of young people. A full dataset with further analysis will be presented at the conference.

Conclusions: Preliminary results indicates young people with CF have a changing sense of identity: self-identity and identity as a young person living with CF, following commencement of Elexacaftor/Tezacaftor/Ivacaftor. The results may help clinicians understand young people’s experience of improved health, as well as their motivations to alter or reduce treatment adherence.

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