Life quality of adult patients with cystic fibrosis

Abstract

Introduction: Cystic fibrosis is a genetic, multi-system chronic disease. The disease is caused by mutations in the CFTR gene. The incidence of the disease in Poland is estimated at 1 per 4400 births. In Poland in 2019, 1231 patients suffering from cystic fibrosis were registered.Objective of the study: Analysis of patients' quality of life suffering from cystic fibrosis. Material and methods: This cross-sectional study was conducted in a pulmonary ward between August and December 2022. The study group consisted of 52 adult patients suffering from cystic fibrosis. The mean age of the patients was 29.25 years (SD = 8.43). Twenty-three respondents received causal treatment. To collect the research material, a standardized Cystic Fibrosis Quality of Life (CFQoL) questionnaire was used, which covered number of domains: physical activity, social activity, treatment-related causes, respiratory system symptoms, emotional wellbeing, concerns for the future, interpersonal relations, body image, concerns about work/study, and personal data from a questionnaire filled in by the subject. Results: The study showed that, in total, patients obtained an average of 51.24 points in the CFQoL questionnaire (SD = 20.18). Low quality of life was demonstrated in 23 patients. In the assessment of the quality of life, patients rated physical functioning the highest, and fears about the future and fears about work/study the lowest. There was no significant relationship between gender (p = 0.449), age (p = 0.362), education (p = 0.905), marital status (p = 0.084), professional status (p = 0.096), and the area of residence of the respondents (p = 0.122) and their overall quality of life. A significant relationship was found between the overall quality of life and the use of oxygen and causative drugs in patients' treatment. Conclusions: Nearly half of patients with cystic fibrosis assess their quality of life as low. Patients who take causative drugs have a better quality of life. More frequent hospitalizations reduce the quality of life of patients.