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BackgroundThe aim of our study was to evaluate factors affecting cystic fibrosis (CF) patients’ health-related quality of life (HRQoL) and to assess the level of agreement on HRQol between children and their parents.MethodsFifty-nine patients (mean age: 14.03 ± 4.81 years) from 5 Hungarian CF centres completed the survey. HRQoL was measured using The Cystic Fibrosis Questionnaire-Revised (CFQ-R). Parents were asked to fill out a questionnaire about their smoking habits, educational level and history of chronic illness. Disease severity was assessed using the physician-reported Shwachman-Kulczycki (SK) score system. Spirometry, Body Mass Index (BMI) percentile (pc), hospitalisation and Pseudomonas aeruginosa (PA) infection were examined as physiologic parameters of CF, and the impact of these factors on HRQoL was assessed. A multivariate regression analysis was performed to identify the most important factors affecting HRQoL. The level of significance was set to 0.05.ResultsPassive smoking and parental educational level and chronic diseases status did not have a significant impact on the patients’ HRQoL (p > 0.05). Significantly lower SK scores and spirometry values were found in low BMI pc patients (p < 0.001), in hospitalised (p < 0.01) and in PA-infected patients (p < 0.01), than in the adequate-weight, non-hospitalised and PA culture-negative subgroup. Lower CFQ-R scores were detected in hospitalised patients than in non-hospitalised patients in their Physical functioning domain. PA-infected patients had HRQoL scores that were significantly worse in the Body image (p < 0.01) and Respiratory symptoms (p < 0.05) domains than the PA culture-negative patients. Patients with a low BMI pc (<25th BMI pc) had significantly lower scores in the Eating, Body image and Treatment burden domains, than the adequate-weight patients (>25th BMI pc) (p < 0.01). A strong child–parent agreement was found in the Physical functioning domain (r = 0.77, p < 0.01).ConclusionsPassive smoking, parental educational level and chronic diseases of parents do not affect the HRQoL of CF patients. In contrast, hospitalisation, PA infection and malnutrition have a significant and negative impact on patients’ HRQoL and the clinical severity of the disease. Parents and children were consistent in their scoring of symptoms and behaviours that were observable.

Inhaled corticosteroids was the most frequent treatment prescribed for asthma patients in the study. However, patients reported substantial asthma-related morbidity. Children used more resources than adults, despite being classified as having better control. Patients with poor control of asthma symptoms incurred the highest societal cost, improving patient control may reduce cost to society by 40% or more.

EudraCT No.: 2004-001233-41.

Impedance of the total respiratory system was measured in 121 healthy children aged 4-16 years during spontaneous breathing by pseudo-random forced oscillations between 3 and 10 Hz. Total respiratory resistance (Rrs), inertance (Irs) and compliance (Crs) were determined by least-mean-squares fitting. Estimates for inertance were reliable only for the larger children, where the values of Irs (0.0127 +/- 0.0034 SD) were similar to those reported for normal adults. Rrs correlated significantly (P less than 0.001) with height (r = -0.868), age (r = -0.865), and, in a subpopulation of the 6- to 16-year-old children, with forced vital capacity (r = -0.803). The corresponding correlation coefficients for Crs were 0.873, 0.844, and 0.853, respectively. Crs amounted to about a third of the static total compliance values of Sharp et al. (J Appl Physiol 1970; 29: 775-779) over the same interval of heights. In these relationships no significant difference was found between boys and girls.