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.

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. Disclosures Karimi: Octapharma: Other: Investigator.