Maintenance of musculoskeletal function in people with haemophilia

Abstract

Bleeding affecting muscles and joints is the most common manifestation of haemophilia. In the long term, prevention of musculoskeletal abnormality in haemophilic patients is cost effective. Early diagnosis and proper treatment is fundamental to prevent disability. Prevention can be divided into secondary and tertiary levels. Secondary prevention concentrates on how to handle a haemophilic child, and on the education of the family so that they develop a safe and healthy lifestyle and awareness of the importance of early diagnosis and treatment. Tertiary prevention deals with minimizing the musculoskeletal consequences of muscle and joint bleeding. Coagulation factor replacement, appropriate care of the bleeding site and early physical therapy helps to prevent complications and yields the best results. Recurrent episodes of joint bleeding can lead to chronic synovitis. Initially synovitis demands conservative treatment. However, conservative methods are often not successful. Chronic synovitis is the most important cause of joint degeneration and the aim of rehabilitation is to achieve functional status using special casting techniques, orthoses or preventative surgery. Surgical, radioisotope or chemical synovectomy may be used. Technical requirements and availability of haemostatic cover limit the use of surgical and radioisotope synovectomy. The author has found chemical synovectomy with rifampicin to be an easier and more effective procedure than surgery. Problems related to joint deformities, muscle atrophy and chronicle pain demand comprehensive care. They are the main factors affecting the maintenance of musculoskeletal function and the social integration of people with haemophilia.