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

Italian Journal of Pediatrics

Agnes Meszaros⁶  Eniko Solyom¹  Kalman Gyurkovits²  Gyongyi Szekely²  Katalin Bolbas²  Lajos Kovacs⁴  Rita Ujhelyi⁵  Klara Holics⁵  Laszlo Kadar³  Reka Bodnar⁵ 

[1] Department of Paediatrics, Velkey Laszlo Paediatric Health Centre Borsod-Abauj-Zemplen County and University Teaching Hospital, Szentpeteri kapu 72 -76, 3526 Miskolc, Hungary;Department of Paediatrics, Kaposi Mor Teaching Hospital, Petofi u. 4, 7257 Mosdos, Hungary;Department of Paediatrics, Pulmonological Institute, Munkacsy M u. 70, 2045 Torokbalint, Hungary;First Department of Paediatrics, Semmelweis University, Bokay J. u. 53-54, 1083 Budapest, Hungary;CF Unit, Heim Pal Children’s Hospital, Ulloi ut 86, 1089 Budapest, Hungary;Department of Pharmacy Administration, Semmelweis University, Hogyes Endre u. 7-9, 1092 Budapest, Hungary

关键词: Hospitalisation;    Pseudomonas aeruginosa;    Malnutrition;    Parent–child agreement;    Health-related quality of life;    Cystic fibrosis;   

Others  :  802279
DOI  :  10.1186/1824-7288-40-50

 received in 2013-11-30, accepted in 2014-05-28,  发布年份 2014

【 摘 要 】

Background

The 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.

Methods

Fifty-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.

Results

Passive 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 (<25^th BMI pc) had significantly lower scores in the Eating, Body image and Treatment burden domains, than the adequate-weight patients (>25^th BMI pc) (p < 0.01). A strong child–parent agreement was found in the Physical functioning domain (r = 0.77, p < 0.01).

Conclusions

Passive 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.

【 授权许可】

   
2014 Bodnar et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

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