Experiences of individuals receiving an adult diagnosis of Cystic Fibrosis: an interpretative phenomenological analysis

Cystic fibrosis (CF) is a life-limiting, inherited disorder caused by a faulty gene, which affects many parts of the body, most predominantly the lungs and gut. Currently no cure exists for CF and treatment regimens are intensive and time consuming. CF results in substantial psychosocial sequelae.

The salience of disclosure of an individual’s CF diagnosis has increased as the average life expectancy has extended. The integrative review aimed to synthesis evidence exploring the experiences of individuals who disclose CF to others. Five electronic databases were searched and eight studies met the inclusion criteria. Findings indicated that disclosure of CF is a complex process, with the potential to elicit positive support and development, whilst, conversely, being associated with high anxiety and possible negative effects. As such, individuals with CF undergo a multifaceted decision-making process before disclosing. Findings were discussed in relation to relevant literature and theories, clinical implications and future research.

CF is predominantly diagnosed during infancy. However, a small proportion of individuals, who present clinically and socially differently, are diagnosed in adulthood. Therefore, the empirical study aimed to explore the experiences of individuals who received a diagnosis of CF as adults, including psychological impact and support around diagnosis. Semi-structured interviews were conducted with six participants and analysed using Interpretative Phenomenological Analysis. Four super-ordinate themes, comprised of 12 sub-themes, emerged from the data. The super-ordinate themes were: ‘Inexplicable and failing’; ‘The blow of diagnosis’; ‘Processing journey’ and ‘Managing others’. Themes were discussed in relation to relevant literature and theory, with recommendations made for clinical practice and future research.