Abstract

Introduction: Cystic fibrosis (CF) is a serious genetic life-shortening disease. Quality of life (QoL) measurement related to CF children is a relatively new field of research, which includes the patient’s perspective in research and clinical practice. Objectives: This study aimed to evaluate the QoL in children with CF and its association with FEV1 (forced expiratory volume in 1 second). Patients and Methods: This cross-sectional study was carried out on 7-14 years old children with CF attending children’s medical center from March 2017 to March 2018. Throat swab cultures and spirometry evaluation was performed for all patients. FEV1 was determined and the 6-min walk test (6MWT) was conducted. The patient’s psychological status was assessed using the Persian version of pediatric QoL inventory. ANOVA, t test, and chisquare tests were used for data analysis. Results: Seventy-six subjects with the mean age of 10.49±3.18 years were studied since 59% of them (n=44) were boys. The mean total QoL was 65.34±17.73. Patients with lower pulmonary function had a lower QoL. There was a significant association between FEV1 and school and emotional functioning (P=0.005 and P=0.002, respectively). A significant association was found between SPO2 (peripheral capillary oxygen saturation) reduction after 6MWT and FEV1 decline (P=0.001). Additionally, a significant association was detected between FEV1 and the distance walked during 6MWT (P=0.030). Conclusion: Regarding the association between pulmonary function and QoL in CF patients and lower QoL score in our study, the importance of assessing pulmonary function in these patients should not be neglected.

Highlights

  • Cystic fibrosis (CF) is a serious genetic life-shortening disease

  • This study aimed to evaluate the Quality of life (QoL) in children with CF and to examine its association with FEV1

  • The study population included 76 subjects diagnosed with CF with the mean age of 10.49 ± 3.18 years, and mean body mass index (BMI) of 16.21 ± 2.83 kg/m2

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Summary

Introduction

Cystic fibrosis (CF) is a serious genetic life-shortening disease. Quality of life (QoL) measurement related to CF children is a relatively new field of research, which includes the patient’s perspective in research and clinical practice. Objectives: This study aimed to evaluate the QoL in children with CF and its association with FEV1 (forced expiratory volume in 1 second). Cystic fibrosis (CF) is a serious genetic life-shortening disease transmitted in the autosomal recessive mode with an incidence rate of 1 in 4500 births in Europe and the USA [1]. It is a chronic and progressive disease with diverse clinical manifestations occurring very early in childhood, sometimes from birth [2]. Several methods are used to assess pulmonary disease severity in CF patients including spirometry, chest imaging and quality of life (QoL) questionnaires. The best method for assessing CF lung disease is forced expiratory

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