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.

HAEMOcare: The First International Epidemiological Study Measuring Burden of Hemophilia in Developing Countries

“…2 3 4 5 6 7 Few studies have been reported from developing countries. 8 9 10 Burden-of-disease studies are needed in developing nations to prioritize determinants of hemophilia care and to formulate adapted management strategies to improve outcomes.…”

Balance evaluation in haemophilic preadolescent patients using Nintendo Wii Balance Board®

“…The musculoskeletal system is the most affected in patients with haemophilia, with the presence of haematomas, haemarthrosis, synovitis and therefore haemophilic arthropathy. Articular haemorrhage or haemarthrosis is the leading cause of morbidity in severe haemophilia, triggering arthropathy in early ages and especially affecting the quality of life (QOL) . These disorders, especially in the lower limbs, involve immobilization and rest, limiting physical activity, favouring becoming overweight, decreasing muscle strength and affecting balance and gait, increasing the risk of falls, new muscle or joint bleeding, poorer physical condition and the creation of a vicious circle that favours the progression towards haemophilic arthropathy .…”

Patient‐reported outcomes in autosomal inherited bleeding disorders: A systematic literature review

“…All studies were published between 2000 and 2020 (Figure 2). Seven studies were conducted in the Netherlands, 22–28 five in the United Kingdom, 29–33 four in the United States, 34–37 three in Canada, 38–40 one in Sweden 41 and one in Iran 42 …”

“…Patient-reported outcomes in coagulation factor deficiencies Two studies 32,42 assessed PROs in coagulation factor deficiencies. Children with factor VII, X, XI, XIII and fibrinogen deficiencies reported an impaired HRQoL in the domains family and friends, indicating that the bleeding disorder had a negative impact on the relationships with their family and friends.…”