An exploration of the experiences of cystic fibrosis in family systems

Introduction: Whilst Cystic Fibrosis (CF) treatment has recently been transformed with developments in modulator drugs (De Boeck, 2020), it remains a serious genetic condition (Bowen & Hull, 2015). Due to the new CF landscape, it is important to develop an up-to-date understanding of CF experiences. Since working together with whole families is key to improving care (Hisert et al., 2023), an understanding of experiences at a family-level was sought. As family-level research is less common, the methodology was also reviewed.
Methods: Four families with a child with CF were recruited and asked to provide creative items to depict their experiences of CF. Family-level interviews were conducted and the creative items provided by the family were used as stimuli in that family’s interview. Interviews were analysed with Interpretative Phenomenological Analysis (IPA).
Results: Results showed that the diagnosis and subsequent lifestyle changes required can feel overwhelming. Families need to negotiate how they live with CF, which can look different for each family. As the child develops or health needs change, renegotiations are needed. Children with CF stand out from their peers. Additionally, fear exists around the child becoming unwell and things becoming overwhelming again.
Discussion: This study supports models of family adaptation to childhood chronic illness but emphasises that the process is not linear. CF clinics should consider how they communicate the initial diagnosis and pacing the information they provide. Holistic support from CF clinics and peer support from older families with CF may help newly diagnosed families. In terms of the methodology, interviewing the family together created a supportive environment but affected what was said. The creative items gave the children power, but this was influenced by pre-existing family dynamics. Future family interviews should consider the location, length and timing of the interview as well as how the creative items are presented.