Abstract
BackgroundAssessment of quality of life (QOL) in patients with hemophilia is important for the disease outcome. In patients with hemophilia, repeated occurrences of hemarthrosis lead to limitations in range of motion (ROM) of major joints.ObjectiveThe aim of this work is to assess joint ROM and detect the presence of limitation of joint ROM in patients with hemophilia and their effect on the QOL in these patients.Patients and methodsThe study included 25 children with hemophilia recruited from Pediatric Hematology Unit, Fayoum University Hospital, during the period from June 2016 to December 2016. Their ages ranged between 4 and 16 years. Large joint examination and measurement of joint ROM were done at rheumatology clinic using goniometry by rheumatologist. QOL was assessed by using the hemophilia QOL questionnaire.ResultsThe most impaired dimensions of QOL were family, treatment, and physical domains. Total health-related quality of life (HRQOL) score, physical health, view of self, and sport and school scores were found worse with increasing age. Patients who had target joints had poor QOL score. Limitation of movement (LOM) of the knee and ankle joints mainly impaired the physical and social aspects and the overall HRQOL, and LOM of shoulder joint impaired the treatment domain.ConclusionAge of the patient was found a factor affecting the total HRQOL score, in addition to physical, view of self, and sport and school dimensions. LOM of the knee and ankle joints mainly impaired physical and social aspects and the overall HRQOL.
Highlights
Hemophilia is a bleeding disorder of X-linked hereditary
The findings of the present study revealed that the mean total quality of life (QOL) score was 45.1±14.7, ranging from 10.4 to 73.4
View of self, and sport and school scores were found worse with increasing age (P=0.005, 0.001, and 0.009, respectively), which indicates a poor QOL with increasing age
Summary
Hemophilia is a bleeding disorder of X-linked hereditary. Deficiency in coagulation factor VIII for hemophilia A and factor IX for hemophilia B is the cause of this disease [1]. Patients with severe hemophilia present with spontaneous bleeding since early childhood, in the musculoskeletal system (muscles and large joints) and, less commonly, mucosal or cerebral hemorrhages. 85% of all bleeding events occur in joints in patients with severe hemophilia, with the ankle, knee, elbow, hip, and shoulders being the most commonly affected joints [3]. Recurrent happenings of hemarthrosis and synovitis with joint destruction and pain lead to limitations in joint range of motion (ROM). Joint ROM limitations in patients with hemophilia are increased with more advanced age, high BMI, and non-white ethnicity [4]. Assessment of quality of life (QOL) in patients with hemophilia is important for the disease outcome. In patients with hemophilia, repeated occurrences of hemarthrosis lead to limitations in range of motion (ROM) of major joints
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