ArticleOrphanet journal of rare diseases2026
Determinants of health-related quality of life in adults with cystic fibrosis: the role of selected sociodemographic and treatment-related factors.
Article in Orphanet journal of rare diseases, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundIn recent years, advances in modern, effective therapies have significantly increased the life expectancy of patients with cystic fibrosis (CF). However, this increase presents new challenges. It is becoming increasingly important to measure the health-related quality of life (HRQoL) and the factors that influence it in this patient population. This study aims to assess the HRQoL of adult patients with CF in Poland and to analyse the sociodemographic and health factors that affect it.
methodsA cross-sectional study was conducted online using the Polish version of Cystic Fibrosis Questionnaire-Revised 14+ (CFQ-R 14+) between January and May 2024. The questionnaire included also sociodemographic questions such as gender, age, marital status, education level, place of residence, and educational or professional activity. Additionally, it included questions on health and treatment, such as pain level, comorbidities, and the use of cystic fibrosis transmembrane conductance regulator (CFTR) modulators. Various statistical methods were employed, including Student’s t-test for independent samples, the Kruskal-Wallis test, and correlation analyses, to assess the impact of individual factors on HRQoL.
resultsThe analysis included responses from 220 individuals across several centers in Poland. The average age of the respondents was 30.56 years (min = 18, max = 61). The highest-rated aspects of HRQoL were related to food-related (eating, digestive symptoms), while the lowest ratings were given for vitality, emotional functioning, and health perceptions. Generally, individuals who were educationally or professionally active, those taking CFTR modulators, and those experiencing less pain reported a better HRQoL across almost all domains. Factors such as gender, marital status, education, and comorbidities influenced some aspects of HRQoL. In contrast, other factors, such as age and place of residence, did not affect patients’ HRQoL.
conclusionsThe HRQoL of adult patients with CF in Poland varies across assessed domains and is significantly influenced by both health and social factors. Integrating psychological and social support into treatment may help further improve patients’ HRQoL.
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