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ObjectiveT2-relaxometry could differentiate between physiological and haemorrhagic joint effusion (≥ 5% blood) in vitro. Are quantitative T2-relaxation time measurements of synovial fluid feasible and reproducible in vivo in clinically bleed-free joints of men with haemophilia?Materials and methodsIn this cross-sectional study, we measured T2-relaxation times of synovial fluid in clinically bleed-free ankles, knees or elbows of men with severe haemophilia A using a T2-mapping sequence (duration ≤ 7 min) at 3 Tesla MRI. Manual and circular regions of interest (ROI) were drawn in the synovial fluid of each joint by two independent observers to measure T2-relaxation times. Measurement feasibility was expressed as the success rate of the measurements by both observers. The interobserver and intraobserver reproducibility of the measurements were evaluated by the intraclass correlation coefficient of absolute agreement (ICC) and the limits of agreement (LoA) from Bland Altman analysis.ResultsWe evaluated 39 clinically bleed-free joints (11 ankles, 12 knees, 16 elbows) of 39 men (median age, 24 years; range 17–33) with severe haemophilia A. The success rate of the T2-measurements was ≥ 90%. Interobserver reliability was good to excellent (manual ROI: ICC = 0.92, 95% CI 0.76–0.97; circular ROI: ICC = 0.82, 95% CI 0.66–0.91) and interobserver agreement was adequate (manual ROI: LoA = 71 ms; circular ROI: LoA = 146 ms). Intraobserver reliability was good to excellent (manual ROI: ICC = 0.78, 95% CI − 0.06–0.94; circular RO: ICC = 0.99, 95% CI 0.98–0.99) and intraobserver agreement was good (manual ROI: LoA = 63 ms; circular ROI: LoA = 41 ms).ConclusionT2-relaxometry of synovial fluid in haemophilia patients is feasible with good interobserver and intraobserver reproducibility.

Joint bleeds are the hallmark of hemophilia, leading to a painful arthritic condition called as hemophilic arthropathy (HA). Exercise programs are frequently used to improve the physical functioning in persons with HA. As hemophilia is a rare disease, there are not many physiotherapists who are experienced in the field of hemophilia, and regular physiotherapy sessions with an experienced physiotherapist in the field of hemophilia are not feasible for persons with HA. Blended care is an innovative intervention that can support persons with HA at home to perform the advised physical activities and exercises and provide self-management information. The aim of this study was to develop a blended physiotherapy intervention for persons with HA. The blended physiotherapy intervention, namely, e-Exercise HA was developed by cocreation with physiotherapists, persons with HA, software developers, and researchers. The content of e-Exercise HA was compiled using the first 3 steps of the Center for eHealth Research roadmap model (ie, contextual inquiry, value specification, and design), including people with experience in the development of previous blended physiotherapy interventions, a literature search, and focus groups. A 12-week blended intervention was developed, integrating face-to-face physiotherapy sessions with a web-based app. The intervention consists of information modules for persons with HA and information modules for physiotherapists, a graded activity program using a self-chosen activity, and personalized video-supported exercises. The information modules consist of text blocks, videos, and reflective questions. The patients can receive pop-ups as reminders and give feedback on the performance of the prescribed activities. In this study, we developed a blended physiotherapy intervention for persons with HA, which consists of information modules, a graded activity program, and personalized video-supported exercises.

Legacy hemophilia‐specific questionnaires are considered too long, show floor‐/ceiling effects, and/or include irrelevant questions. Patient Reported Outcomes Measurement Information System (PROMIS) item banks, including Computer Adaptive Tests (CATs) and short forms, were designed for more efficient outcome assessment. Evaluate the feasibility, measurement properties, and relevance of seven PROMIS CATs and two short forms in patients with hemophilia. In this cross‐sectional study, Dutch adults with hemophilia completed nine PROMIS item banks electronically. Feasibility was assessed by number of items and floor/ceiling effects. Reliability was determined as the proportion of reliable scores (standard error ≤3.2). Construct validity was assessed by comparison with legacy instruments and expected differences between subgroups. Relevance of item banks was determined by proportions of limited scores. Overall, 142 of 373 invited patients (mean age, 47 [range, 18‐79], 49% severe hemophilia, 46% receiving prophylaxis) responded. Per CAT item bank, mean number of items answered varied from 5 (range, 3‐12) to 9 (range, 5‐12), with floor effects in pain interference (26% lowest scores) and depression (18% lowest scores). Construct validity and reliability were good for physical function, pain interference, satisfaction with social roles and activities, and fatigue. The CAT physical function showed the most limited scores (38%). The self‐efficacy short forms showed ceiling effects (22%‐28%) and no relation with the legacy instruments. The PROMIS CATs physical function, pain interference, satisfaction with social roles and activities, and fatigue are feasible, reliable, and valid alternatives to legacy instruments for patients with hemophilia, with few items and low floor‐/ceiling effects.

Background: Joint bleeds are the hallmark of haemophilia, and can lead to disabling haemophilic arthropathy. Consequently, the movement behaviour of adults with haemophilia differs from that of healthy adults. It seems unlikely that a single outcome is able to reflect all relevant information regarding movement behaviour. The aim of the current study was to identify patterns of movement behaviour within persons with haemophilia (PWH) and compare clinical characteristics between patterns of movement behaviour. Methods: A total of 105 PWH [70% severe haemophilia; median age 43 years (30.0–54.0)] were included in the study. Hierarchical cluster analysis was used to identify patterns of movement behaviour. Clustering variables included seven parameters of movement behaviour: sitting, standing, walking, biking, running, frequency of active bouts and length of active bouts. Clinical characteristics included age, severity of haemophilia, joint health, physical functioning and pain. Clinical characteristics were compared between identified clusters by Kruskall–Wallis test. Movement behaviour was assessed with the Activ8 accelerometer, joint health was assessed on the Haemophilia Joint Health Score, physical functioning on the Haemophilia Activity List and the 40 m self-paced walk test and pain on the Numerical Pain Rating Score. Results: Cluster analysis identified three clusters, which were defined as: ‘sedentary’ (57%), ‘bikers and runners’ (22%) and ‘walkers’ (20%). The ‘bikers and runners’ showed better joint health and experienced fewer limitations in activities than the ‘walkers’ and the ‘sedentary’. The ‘walkers’ perceived fewer limitations in activities than the ‘sedentary’, with comparable joint health. We did not identify differences in pain, walking speed and age between the clusters. Conclusions: We identified three patterns of movement behaviour. The majority of PWH was identified as sedentary, whereas less sitting and regular walking during the day seemed to be more beneficial.

Adding a personal trainer enforced persons with hemophilia in conducting the training and demonstrated that this might help participants to maintain physical activity even after the study terminated. Improvements in the treatment of persons with hemophilia in the past 40 years have radically changed the impact of hemophilia in developed countries. 2 From a disabling condition that strongly affected life expectancy of persons with hemophilia, it now is a more easily treatable chronic health condition in which life expectancy is approaching that of people in the general population. 2 As a result of this improved treatment, the impact of non–hemophilia-related comorbidities such as cardiovascular disease and malignancy increases over time. 3,4 Regular physical activity is important to maintain muscular fitness, maintain a healthy weight, and reduce the risk of cardiovascular disease, type 2 diabetes, and malignancy. 5 Although the World Health Organization released a report on the importance of regular physical activity in 2010, 6 there is a rising number of people in the general population who have insufficient physical activity, especially in high-income Western countries. 7 Guidelines for hemophilia care recommend supporting physical activity to enhance muscle strength and coordination, maintain a healthy weight, and support bone health in persons with hemophilia. 8 Studies in the Netherlands and Australia showed sports participation in children with hemophilia is almost similar to that of the age-matched healthy peers. 9-11 Although sports participation in young persons with hemophilia is improving, a recent study showed that physical activity levels in adults with hemophilia are lower compared to age-matched healthy controls. 12 This might be an echo from the past as traditionally people with hemophilia were discouraged from participating in sports because of the high risk of bleeds when prophylactic treatment was not yet available. [...]bleeding in the past leading to hemophilic arthropathy may have impaired the ability to perform activities such as running and biking. 13 Moreover, the concerns regarding the risk associated with exercise or physical activity are still present in part of the adult population outweighing the benefits for them. 14 This indicates the importance of studies that explore ways to overcome barriers and fears that might hinder persons with hemophilia from participating in sports or other forms of physical activity. Help individuals to maintain positive behavior Facilitate behavior self-regulation Reshape environment and use cues Provide resources Provide support Be aware of the social support (or lack of support) a person receives on an individual, social, and community level.

The physiotherapist plays an essential role for people with haemophilia, an inherited bleeding disease responsible for musculoskeletal complications. Yet, with the advent of new and advanced therapies, the medical landscape is changing, and physiotherapy must adapt alongside. This paper considers whether there will still be a need for physiotherapy in the era of advanced therapies, and discusses ways in which services should evolve to complement emerging treatment paradigms for haemostasis in people with haemophilia. Ultimately, physiotherapy will remain an important element of care, even for people with little joint damage and low risks in the era of the new mild phenotype. However, competencies will need to evolve, and physiotherapists in both primary care and specialist treatment centres should work with haematology colleagues to develop more sensitive tools for detecting early joint changes. Physiotherapists will also play a crucial role in counselling and physically coaching, monitoring the musculoskeletal status of people with haemophilia who have transitioned to new treatments.

For persons with hemophilia, optimization of joint outcomes is an important unmet need. The aim of this initiative was to determine use of ultrasound in evaluating arthropathy in persons with hemophilia, and to move toward consensus among hemophilia care providers regarding the preferred ultrasound protocols for global adaptation. A global survey of hemophilia treatment centers was conducted that focused on understanding how and why ultrasound was being used and endeavored to move toward consensus definitions of both point‐of‐care musculoskeletal ultrasound (POC‐MSKUS) and full diagnostic ultrasound, terminology to describe structures being assessed by ultrasound, and how these assessments should be interpreted. Next, an in‐person meeting of an international group of hemophilia health care professionals and patient representatives was held, with the objective of achieving consensus regarding the acquisition and interpretation of POC‐MSKUS and full diagnostic ultrasound for use in the assessment of musculoskeletal (MSK) pathologies in persons with hemophilia. The recommendations were that clear definitions of the types of ultrasound examinations should be adopted and that a standardized ultrasound scoring/measurement system should be developed, tested, and implemented. The scoring/measurement system should be tiered to allow for a range of complexity yet maintain the ability for comparison across levels. Ultrasound is an evolving technology increasingly used for the assessment of MSK outcomes in persons with hemophilia. As adoption increases globally for clinical care and research, it will become increasingly important to establish clear guidelines for image acquisition, interpretation, and reporting to ensure accuracy, consistency, and comparability across groups.