| Summary: | The combination of physical and psychosocial factors observed in cystic fibrosis (CF), has the potential to severely impact on the health related quality of life (HRQoL) experienced by adults with CF. To date, FIRQoL has not been systematically assessed in adults with the disease. This is primarily for two reasons; (a) complete testing and validation of an appropriate generic measure for this population has not been conducted and (b) as yet, no disease specific HRQoL for adults with CF has been developed, tested, validated or published. The present work has addressed both of these deficits by testing and validating a generic measure of HRQoL, the short form 36 (SF-36) item questionnaire, and secondly, by addressing the primary aim of developing, testing and validating a disease specific measure of HRQoL for adults with CF. The research achieved a partial validation of the SF-36 although frill validation proved to be equivocal. Structural validation of the measure using principal components analysis, Cronbach alpha coefficients and correlational techniques indicated that the measure was robust. However, discriminatory sensitivity proved problematic, indicating that across some domains, the SF-36 may not be sensitive to differences based on disease severity. The most original and significant achievement of the work has been the development and validation of a disease specific measure for adults with CF, the cystic fibrosis quality of life (CFQoL) questionnaire. Testing has shown the CFQoL to be structurally robust, reliable, discriminatory and also sensitive to transient changes in health status. Furthermore, using correlational techniques, the work has also highlighted the weak relationships between aspects of HRQoL and clinical indicators. In addition, the information gleaned from the CFQoL during the validation process, identified specific problem areas amongst adults with CF that may be amenable to psychosocial interventions, and which have not previously been targeted in the population. This information would fUrther enhance the multidisciplinary approach to treatment of adults with CF, making interventions truly holistic.
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