Quality of Life in Children with Rare Bleeding Disorders

Background: Patients with rare bleeding disorders (RBDs) face with several problems due to the chronic feature of the disease. So their physical and mental health is affected. Objective: The aim of this study is to evaluate quality of life (QOL) in patients with RBDs using haemo-QOL questionnaire.

Methods: In this cross-sectional study, 52 consecutive children aged between4-18 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 by expert nurses using haemo-QOL questionnaire in different age groups including 4-7, 8-12, and 13-16/18 years old. Final score range is defined between 0-100 and higher score of QOL shows worse condition. Disease severity was determined based on coagulation factor levels: mild >5%, moderate 1-5% and severe <1%. P value less than 0.05 was considered statistically significant.

Results: Mean age of the patients was 13.9 ± 4.5 ranged from 4 to 18 years old including 28 males and 24 females. The distribution of frequency of RBDs was as follow: Factor I deficiency 9; Factor V deficiency 2; Factor VII deficiency 15; Factor X deficiency7; Factor XI deficiency 8; Factor XIII deficiency 8; and combined Factor V&VIII deficiency 3 patients. Overall QOL of the patients was 41.15 ± 14.72. In Univariate analysis, from all evaluated variables only bleeding severity based on bleeding score showed a statistically significant correlation with QOL of the patients (r =0.298, P=0.032). In multiple linear regression models only degree of being bothered by the disease was determined as an independent influencing factor on QOL.

Conclusion: Based on our results, QOL of children with RBDs was correlated significantly with bleeding severity but not correlated with disease severity based on factor level. Due to chronicity and feature of bleeding disorders, more attention to different aspects of the disease is necessary to improve QOL of the patients with RBDs.