Abstract
OBJECTIVES:The current study sought to evaluate the quality of life of young patients with cystic fibrosis and correlate these results with the clinical parameters indicative of disease severity.METHODS:This cross-sectional study applied the validated Portuguese version of a cystic fibrosis specific quality of life questionnaire to clinically stable patients aged 14 to 21 years old. The correlations between the questionnaire domain scores and forced expiratory volume in one second (FEV1) values, the Shwachman-Kulczycki score, and body mass index were assessed, and correlations were considered as significant when p<0.05.RESULTS:A total of 31 patients (11 females; 16.4±2.3 years old) were evaluated, and the median scores on the questionnaire domains ranged from 66.7 to 100. A significant correlation was found between body mass index and the weight (r=0.43, p=0.016) and the eating questionnaire domains (r=0.44, p=0.013); between FEV1 and the physical (r=0.53, p=0.002) and treatment burden (r=0.41, p=0.023) domains; and between the Shwachman-Kulczycki score and the physical (r=0.39, p=0.03), health (r=0.41, p=0.023), and role (r=0.37, p=0.041) domains. A significant difference was found amongst patients with FEV1 values above or below 60% of the predicted value with regard to the role and health domains. No differences in the scores were found according to gender.CONCLUSIONS:The current cystic fibrosis specific quality of life questionnaire scores exhibited wide variability across all domains; however, they indicated a relatively satisfactory quality of life amongst the patients studied. Certain domains exhibited significant correlations with clinical parameters; thus, this instrument has consistent associations with clinical outcomes.
Highlights
The survival of patients with cystic fibrosis (CF) has increased significantly over recent decades because of earlier diagnoses, better approaches to the treatment of lung and pancreatic diseases, and improvements in nutrition
The inclusion criteria were a confirmed diagnosis of CF according to the Cystic Fibrosis Foundation guidelines [18], an age between 14 to 21 years, and clinical stability over the previous 15 days
A significant correlation was found between Body mass index (BMI) and the weight and eating domains as well as between FEV1 and the physical and treatment domains (Figure 1)
Summary
The survival of patients with cystic fibrosis (CF) has increased significantly over recent decades because of earlier diagnoses, better approaches to the treatment of lung and pancreatic diseases, and improvements in nutrition. Half of the current CF population in developed countries consists of adults [1,2]. The symptoms of CF and their complex treatment regimen impose a high burden on both patients and parents, eventually increasing their levels of stress and affecting their quality of life [3,4]. Health-related quality of life (HRQoL) measures and assesses symptoms and daily functioning from the patient’s perspective. Quality of life (QoL) measurements have been.
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