Joint Health, Functional Ability and Physical Activity in Haemophilia

Abstract

In haemophilia outcome measurement has long been strongly focused on the level of body functions and structures while functional outcome and physical activity have been underrepresented. However, the past decade a shift has been made to a more total evaluation of the patients’ functional health status including the assessment of functional ability. Because adequate tools to measure functional ability were lacking, especially for children, the paediatric haemophilia activities list (PedHAL) was developed and tested in a Dutch and Romanian population. The PedHAL has shown to be feasible, valid and reliable. Future studies should be aimed at further refining this instrument. The historical cautiousness of health providers with regard to physical activity in patients with haemophilia may have led to a lack of knowledge in this area. With modern treatment, both participation and intensity of physical activity are increasing and may positively affect joint health and physical fitness; however it is not clear to what extent. A cross sectional study was performed to address this issue. It was found that children with haemophilia were as active as the general population and had normal levels of cardiorespiratory fitness. Children with severe haemophilia participated more in swimming and less in competitive soccer compared to children with non-severe haemophilia. We did not find a clear relation between activity levels, joint health and cardiorespiratory fitness, which might be explained by the relative good joint health status and the questionable reliability of self reported physical activity measure used in this study. Physical activity is increasingly advocated for haemophilic patients both in developed and developing countries. However, no systematic review and appraisal of current knowledge of exercise interventions in patients with haemophilia had been performed. In order to better understand the effects of exercise interventions on patients with haemophilia we conducted a systematic literature review. We included nine articles in a qualitative analysis. Overall the level of evidence and the quality of the included studies was low and therefore the evidence base for exercise interventions was rated as poor for all interventions and outcomes. Moreover, the description of training parameters was generally unsatisfactory and outcome measures used lacked uniformity. Suggestions for improving study methodology and training theory were proposed in order to help this field forward. In addition to late training effects, exercise may also have acute beneficial effects in haemophilia patients. We studied the effect of strenuous physical exercise on the level of clotting factor in patients with mild and moderate haemophilia A. Patients showed consistently increase in FVIII levels after exercise with a median increase of 2.5 times baseline activity. Even though these results are promising, the practical value of these findings requires further study.