Tools used to measure quality of life in adults with cystic fibrosis- a systematic review.

Background: Measuring the quality of life in patients with cystic fibrosis is important, both in terms of assessing the implementation of new therapies and monitoring their effects, as well as the ongoing evaluation of patients' condition. The objective of this study is to present tools for measuring the quality of life in adult patients with cystic fibrosis, along with their characteristics and measurement properties.

Methods: The systematic review was performed according to the PRISMA guidelines based on a previously prepared research protocol (PROSPERO: CRD42023491030). Searches were performed in Medline (via PubMed), Embase (via OVID), and Cochrane Library databases. In addition, manual searches of bibliographies from the studies included in the analysis and grey literature were performed. Quality assessment of the included studies was performed according to the guidelines of COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN).

Results: The systematic search identified 3,359 studies, of which 26 met the inclusion criteria for the analysis. Two publications were additionally included as a result of the manual search. A total of 16 tools for measuring the quality of life in adults with cystic fibrosis were identified, the measurement properties of which were presented in the included studies. Among these tools, the Cystic Fibrosis Questionnaire-Revised (CFQ-R) and the Cystic Fibrosis Quality of Life Questionnaire (CFQoL) were most frequently analyzed. There were also other new, promising tools.

Conclusion: Most studies reported acceptable measurement properties of tools used to measure quality of life in adult patients with cystic fibrosis. In many cases, however, significant limitations were observed related to the lack of comprehensive analysis of the factor structure and other aspects related to validation and responsiveness. There have also been problems with the reliability of some tool scales (including the CFQ-R 14+). The small number of studies makes it difficult to present clear conclusions regarding the usefulness of existing tools. In turn, new tools that may be used in economic analyses (CFQ-R-8 dimensions) or in individualized assessment of quality of life using a mobile application (Q-Life) seem promising. However, further research on large patient populations is necessary to analyze the measurement properties of all tools.