The effects of exercise training on physical activity level, daily living activities, and participation in children with hemophilia

Introduction: Hemophilia is a rare inherited bleeding disorder characterized by dysfunction or deficiency of clotting proteins, which is difficult to diagnose and manage. Particilally due to intra-articular bleeding, it leads to joint pain, muscle weakness and loss of flexibility, physical inactivity and, as a result, a cycle of increased bleeding risk. Effective movement and individual physiotherapy interventions can improve physical activity levels, quality of life and participation of children with hemophilia. While there are few studies in the literature on the effect of exercise activity in children with hemophilia, there is no study that addresses body structure and functions, activity and participation together in children with hemophilia within the framework of the International Classification of Functioning (ICF). Objective: The aim of our study is to investigate the effect of individualized planned exercise program on joint health, pain, physical activity level, quality of life, and participation level in children with hemophilia. Methods: The first group (n=14) of children with hemophilia, , ranging from 8 to 18 years old, performed individually structured exercises under the supervision of a physiotherapist for 3 days a week, 1 hour per day. The second group (n=15) was recommended exercises programmed to suit their needs at home. Participants in both groups and their families were counseled about physical activity, exercise, preventive approaches, and the rest-activity cycle in case of bleeding. Both groups were followed up for a total of 8 weeks and evaluations were performed at baseline and after treatment. Demographic characteristics, pain, joint range of motion (ROM) and strength were measured using the Clinical evaluation form, Visual Analog Scale (VAS), goniometer and digital dynamometer, respectively. Joint health, functional capacity, participation, quality of life and physical activity were assessed using Hemophilia Joint Health Status (HESS), 6-Minute Walk Test (6MWT), Canadian Occupational Performance Measure (COPM), Pediatric Quality of Life (PedsQL) and International Physical Activity Questionnaire (IPAQ). Results: Post-study HESS, 6DYT, COPM, IPAQ, muscle strength, and range of motion (elbow, knee, and ankle) significantly improved in both groups (p<.05). IPAQ, 6MWT, muscle strength (elbow, knee, and plantar flexion), and ROM (knee and ankle flexion) significantly increased in both groups, with greater improvement observed in Group I, which was supervised by a physiotherapist (p<.05). There were no significant differences in pain and PedsQL scores in both groups. Conclusion: The results of our study demonstrated that individualized planned exercises applied to children with hemophilia, within the framework of the ICF, were effective in improving physical activity, participation, functional level and joint health. Physiotherapist-assisted exercises were found to be more successful than home exercises in improving several parameters. The necessity of recommending home exercises to hemophilic children who can not access to physiotherapy was emphasized. In addition to physical function tests, the inclusion of gait analysis, participation, and quality of life data showed potential benefits for musculoskeletal and psychosocial health in children with hemophilia.