Factors influencing quality of life and disease severity in Hungarian children and young adults with cystic fibrosis

Abstract

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.

Health related quality of life in Indian children with cystic fibrosis

“…The positive association between scores in Respiration domain and spirometry [16] have also been previously reported [16,26], thereby validating CFQ-14+ showing correlation between pulmonary function and Respiration domain. Similar to our study, an Italian study also found lower Respiration scores for Pseudomonas positive, compared to negative patients [19].…”

“…The CFQ-R is the only patient-reported outcome instrument with versions for both children and the caregivers. It has been translated into 34 languages all over the world [17][18][19][20][21]. Our objective was to use CFQ-R to assess Quality of Life (QoL) in Indian children with CF.…”

Comparison of health-related quality of life among children with cystic fibrosis and their parents in two Eastern European countries

“…HRQOL was measured using the Cystic Fibrosis Questionnaire-Revised (CFQ-R). The CFQ-R is a widely used, validated, disease-specific instrument for HRQOL measurement in patients with cystic fibrosis [12][13][14][15][16]. The Hungarian and Polish [17] versions of the CFQ-R were used to assess HRQOL from patients' (patient-report) and their parents' perspectives (parent-proxy report).…”

Impact of <b><i>Pseudomonas aeruginosa</i></b> Infection on Respiratory Muscle Function in Adult Cystic Fibrosis Patients

“…However, Pseudomonas aeruginosa infection in CF patients remains a major problem, with a negative impact both on overall survival and Quality of Life (QoL) [2,3,4]. …”