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The literature review found that pulmonary exacerbations (PEXs) impact on the quality of life (QOL) of people with cystic fibrosis (CF). There is currently no standardised definition of a PEX in CF to inform patient care and QOL. Before a definition can be achieved agreement must be attained on the indicators of a PEX. This thesis aims to identify important indicators of a PEX in adults with CF (ACFs) and establish consensus among ACFs and CF health professionals (CFHPs). The Delphi technique was used to conduct parallel web surveys over a series of rounds across 13 CF centres in the UK and Ireland. A range of sources were utilised to develop 48 statements that informed the Delphi surveys in Round 1 (R1). A 75% consensus level was applied for R 1 and Round 2 (R2). R 1: Participants rated their level of agreement with each statement. ACFs (n=31): 21 consensus statements. CFHPs (n=38): 23 consensus statements. Five additional statements were generated through open questions and added to R2. R2: Statements not reaching consensus in R1 and the 5 additional statements were presented to participants to re-rate. ACFs (n=28): 1 0 consensus statements. CFHPs (n=38): 7 consensus statements. R3: Analysis of R 1 and R2 found 26 consensus statements common in both groups. Additional consensus statements were identified from each group (ACF: 5; CFHP: 4). 35 statements were presented to both participant groups in R3 to rate from 1-10. Results were averaged and ranked in importance. Agreement was established within each participant group on 35 important indicators of a PEX. Each group identified a different hierarchy of indicators. This consensus is an important step in the development of a tool to be used by CFHPs to identify when a PEX is occurring and provides a strong platform for further research into PEXs in CF.

This research explores the educational and employment experiences of young people with Cystic Fibrosis (CF) and was carried out as two separate studies. Study one consists of a small-scale survey examining the educational and employment history of 162 patients attending the Leeds regional Adult CF centre. Analysis of the data was conducted using non-parametric (Spearman‟s) correlations, t-tests and logistic regressions. Study two consists of qualitative analysis of interviews with six young people with CF discussing educational and employment experiences and their understanding of the factors that influenced the development and attainment of aspirations. Using Grounded Theory (GT) methodology, an overall theoretical model of participants‟ experiences has been developed, providing some speculations as to why many do not fulfil their potential. Study one found the educational attainment and employment of people with CF to be lower than those in the general population (OECD, 2011; 2008). Socioeconomic status was the factor most predictive of school leaving age, educational attainment at degree level and perceived impact of CF on education and career and disease severity and educational level the factors most predictive of employment. Findings from study two revealed several themes: physical and practical consequences of CF and in some cases the actions of others, illustrated many practical and prejudicial barriers that prevented many from attaining their educational and occupational aspirations. The reality of struggles to achieve and maintain educational and occupational performance because of CF, and worries about their health and life expectancy had a significant impact on self-belief and belief in educational and workplace providers. This lowered expectations of achieving educational and occupational success, resulting in a withdrawal from aspirations and subsequent educational and occupational attainment. It is hoped that the findings of this study will contribute to the wider CF literature and that of other chronic illnesses and disability.

This thesis commences with a review of the literature on eating disorders and disturbed eating attitudes and behaviours (DEABs) in individuals with cystic fibrosis (CF). Certain physiological and psychological factors related to CF may contribute to the development of issues with food and eating. The major conclusions of the review support the notion of the presence of some DEABs in people with CF. However, it seems that the presence of diagnostic eating disorders is no higher than that found in the general population. Limitations of the research are discussed and areas for future research are identified. Following from this, the empirical paper investigated the relationships between eating behaviours and attitudes, coping styles and body image in individuals with CF. The findings suggest that females with CF present with higher rates of DEABs and males present with poorer body image. DEABs were found to be associated with poorer body image and unhelpful coping strategies. Clinicians should screen for DEABs at clinic appointments to ensure that any difficulties do not impact on the health of the individuals. The results are considered in relation to prior research, and methodological limitations as well as clinical implications are discussed.

Background and aims: Cystic Fibrosis (CF) is the most common genetic, life threatening disorder in the UK (Cystic Fibrosis Trust, 2010). Given the trajectory of the illness, adolescence may be a particularly challenging period, during which young people become more aware of differences from peers, and are faced with the task of balancing increasing illness demands with the drive to aspire to developmental goals. Nevertheless, little research specifically explores how young people with CF reconcile their illness experiences with the emerging sense of self. In an attempt to address this gap in the literature, this study sought to hear the narratives of young people with CF with reference to the local and broader contextual factors influencing their construction, with the aim to further understanding, inform clinical practice and improve support for young people with CF. Methodology: A qualitative approach was employed. A purposive sample of six participants diagnosed with CF and aged between 12 and 16 years was recruited. Participants were asked to take photographs of their experiences of life as a young person with CF which were used alongside a semi-structured topic guide in individual interviews to explore the young person’s narratives. The interviews were audio-recorded, transcribed, and analysed using a narrative approach to explore both what was said and how it was told.Analysis and Findings: The researcher’s global impressions of each person’s narratives, along with details of the local context of the interview were presented. Following this, similarities and differences across the narratives were considered with particular attention to how the main storylines were interwoven with participants’ emotional experiences, the identity work taking place through the narrative, and the broader narratives available to them. It emerged that (i) CF was perceived as part of participants’ normality which they had grown accustomed to over time, (ii) participants drew on cultural narratives to position themselves as normal teenagers, to maintain a positive sense of self, though also leading them to minimise difficulties and distress, and (iii) participants continued to position themselves within the norm as they talked of their futures, describing similar hopes to their peers, and again played down concerns about how CF might impact on their futures. These findings are discussed with reference to the clinical implications, strengths, and limitations of the methodology, and directions for future research.