Clinical characteristics and outcomes of cystic fibrosisin Palestine: Cross sectional study.

Abstract

To assess the clinical characteristics and outcomes of cystic fibrosis in Palestine by studying the quality of life (QoL) of participants. This cross-sectional study involved the application of Cystic Fibrosis Questionnaire-Revised (CFQ-R) to participants attending the pediatric pulmonology clinic at Caritas Baby Hospital between January and May 2017. Health status was assessed by measuring pulmonary function test (FEV1 ), body mass index (BMI), age of CF diagnosis, and presence of other affected siblings or deaths in the family. There were 77 participants from 58 families: 46.8% (36/77) were males, and 53.3% (41/77) were females. The mean age was 10.7 years (range: 0.5-36 years). The participants were divided into three groups by age in years: group I ( < 6), II (6-13), and III (≥ 14). The highest and lowest CFQ scores were for the eating domain in group III (55.6 ± 22.5) and the body domain in group II (14.5 ± 17.7), respectively. Mean illness severity was 69.6% (range: 33%-111%). The mean BMI was 15.9 (range: 9.6-23.1). The mean age at the time of diagnosis was 4.2 years (± 6.3). The study showed that 1.7%of the families (1/58) had four affected siblings, and 21% (12/58) had death cases related to CF, of which 58.3% (7/12) were from the Hebron district. Finally, all parameters for CF participants in West Bank, Palestine were noticeably lower than those reported in other countries. This study illustrates the need for new therapies for CF participants in Palestine to improve QoL, health status, and longevity.