title: Quality of Life in Children and Adolescents With Rare Bleeding Disorders in Southern Iran.
abstract: The aim of this study is to evaluate quality of life (QOL) in patients with rare bleeding disorders (RBDs). In this cross-sectional study, 52 consecutive children aged between 4 and 18 years old with RBDs registered at the Haemophila Center of Fars province in Southern Iran were investigated from January to April 2015. Quality of life was evaluated using Haemo-QOL questionnaire. Final score is defined between 0 and 100, and higher score of QOL shows worse condition. P value less than .05 was considered statistically significant. Mean age of the patients was 13.96 ± 4.50 and ranged from 4 to 18 years old including 28 males and 24 females. Family and friends were the 2 most impaired domains of Haemo-QOL in these patients. In univariate analysis, bleeding severity based on bleeding score, health status, and being bothered by the disease showed statistically significant correlations with QOL of the patients ( P < .05). In multiple linear regression models, only degree of being bothered by the disease was determined as an independent influencing factor on QOL. Taking together, Haemo-QOL of children with RBDs was better than what was reported in patients with hemophilia in our region, but it was worse than that reported in patients with hemophilia in other developing and developed countries. Due to chronic feature of bleeding disorders, more attention to different aspects of the disease, especially in 2 dimensions of family and friends through considering educational and psychological program for the patients and their family, are recommended to improve QOL of the patients with RBDs.
authors.affiliation: 1 Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Sezaneh
authors.initials: S
authors.lastname: Haghpanah
authors.affiliation: 2 Pediatric Department & Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Hassan
authors.initials: H
authors.lastname: Mohtadi
authors.affiliation: 3 Hematology Research Center, Dastgheib Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Mahboobeh
authors.initials: M
authors.lastname: Akbari
authors.affiliation: 1 Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Mehran
authors.initials: M
authors.lastname: Karimi
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005190:Family / Q000523:psychology; D005260:Female; D033062:Friends / Q000523:psychology; D006304:Health Status; D006470:Hemorrhage / Q000523:psychology*; D006801:Humans; D007492:Iran / Q000453:epidemiology; D008297:Male; D011788:Quality of Life / Q000523:psychology; D011795:Surveys and Questionnaires
keywords: Haemo-QOL; quality of life; rare bleeding disorders