A systematic review of physical activity in people with haemophilia and its relationship with bleeding phenotype and treatment regimen

Abstract

Introduction Although the measurement of physical activity (PA) amongst people with haemophilia (PWH) has become increasingly widespread in recent years, the relationship between PA and bleeding phenotype remains poorly understood. In addition, the influence of various treatment regimens on this relationship has not been defined. Aim This review aimed to systematically assess the data that are available regarding PA levels amongst PWH, as well as the relationship between PA and bleeding. Methods A systematic search of the online databases EMBASE, Cochrane, MEDLINE Ovid, CINAHL and Web of Science was conducted by two independent reviewers. Quality assessment was undertaken using the AXIS Critical Appraisal Tool for Cross‐sectional Studies and the STROBE checklist. Results Of 1902 sources identified overall, 36 articles were included. Low‐to‐moderate transparency of reporting and various sources of bias were identified. PA levels varied amongst heterogeneous samples of PWH. The relationship between PA and bleeds was inconclusive, although there was evidence that improvements in treatment over recent decades have appeared to enable PWH to become more physically active. Conclusion Based upon the limited available evidence, the relationship between PA and bleeding phenotype in PWH remains unclear. However, with the development of improved prophylaxis treatment regimens in recent years, there is evidence that PA levels have increased, especially amongst people with severe haemophilia. The use of validated outcome measures of PA and more robust reporting of bleeds and treatment regimen are warranted in future research, especially in a rapidly evolving era of new treatments for PWH.

Thromboelastography and thrombin generation assessments for pediatric severe hemophilia A patients are highly variable and not predictive of clinical phenotypes

“…These findings were not unexpected, given that other efforts to use global hemostatic assays to predict the therapeutic requirements of SHA patients have been unsuccessful 50–52 . This may be attributed to an assortment of non‐exclusive factors, which include: (1) at low TF levels, even those SHA samples with the strongest relative hemostatic activity in CAT and TEG assays were still weak relative to normal donor samples, and thus indicated ineffective FVIII‐mediated hemostasis in vivo ; (2) all the patients in our cohort received rFVIII replacement therapy, which ensured they spent little or no time at the baseline levels of hemostatic function we assessed via CAT and TEG assays; (3) like many studies, ours was insufficiently powered to detect subtle relationships between hemostasis assay results and clinical parameters; and (4) key clinical outcomes such as joint bleeds are affected by many factors that show significant variation among patients and can act in combination, including patient physical activity, 53 body mass index, 54 and factor clearance rates 55 …”

“…) key clinical outcomes such as joint bleeds are affected by many factors that show significant variation among patients and can act in combination, including patient physical activity,53 body mass index,…”

Exploration of the minimum necessary FVIII level at different physical activity levels in pediatric patients with hemophilia A

“…According to Buxbaum et al, in the current social environment and with medical support, patients with hemophilia can be as physically active as their normal peers ( 16 ). A recent systematic review showed that physical activity levels varied greatly among heterogeneous samples of patients with hemophilia ( 4 ). In this study, patients participated in at least one physical activity besides routine walking.…”

“…With the widespread prophylaxis, physical activity levels of patients with hemophilia have increased ( 4 ). With appropriate FVIII level, the benefits of physical activity outweigh the risk of bleeding ( 5 ).…”

Hemophilia Patient Experience in a Physical Therapy-Guided Health Education Intervention: A Mixed-Method Design

“…This idea coincides with the review by Lobet et al, which states that physical therapy has an essential role in combination with other new treatments in people with hemophilia, especially in their musculoskeletal complications [ 34 ]. In hemophilia management, the physiotherapist plays an important role in patient education and physical training, given that exercise can be helpful for them [ 35 ]. Moreover, physical therapy educational programs can reach higher patient satisfaction compared to physical therapy alone in patients at both a chronic and an acute phase [ 36 ].…”