Efficacy of rehabilitation interventions on functioning in patients with hemophilic arthropathy: A systematic review with meta-analysis.

BACKGROUND: Hemophilic arthropathy is a detrimental condition that crucially affects functional outcomes in hemophilic patients. In recent years, due to the advances in systemic therapies, growing attention has been raised in the rehabilitation field in order to improve functional outcomes of hemophilic patients. However, the optimal rehabilitation modalities in these patients are far from being fully characterized. OBJECTIVE: The present study aimed to assess the effects of different rehabilitation interventions on physical functioning and health-related quality of life of hemophilic arthropathic patients. METHODS: The review followed the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement. Five databases were systematically searched for randomized controlled trials (RCTs) published until June 22nd, 2023. The selection criteria included adult patients with hemophilia A and B receiving rehabilitation interventions. The outcomes were muscle strength, physical function, pain intensity, physical performance, and health-related quality of life. RESULTS: Out of 1,743 identified records, 17 studies were included in the qualitative synthesis. Rehabilitation interventions were categorized into exercise intervention, fascial therapy, and multimodal intervention. The findings suggested positive outcomes in terms of muscle modifications, range of motion improvements, joint health enhancements, pain intensity reduction, and quality of life improvements. More in detail, meta-analyses showed significant improvements in pain intensity [ES: -1.10 cm (-1.37, -0.82), p< 0.00001], joint health [ES: -1.10 (-1.38, -0.82), p< 0.00001], In accordance, exercise interventions showed significant benefits in terms of joint health [ES: -2.54 (-3.25, -1.83), p< 0.00001)] and quality of life [ES: 1.17 (0.48, 1.86), p< 0.0000)]. CONCLUSION: Rehabilitation interventions have a positive impact on functional outcomes and health-related quality of life of hemophilic arthropathic patients. Further studies are needed to better elucidate the role of a comprehensive intervention combining different rehabilitation approaches to treat hemophilic arthropathy.

Effectiveness of rehabilitation on pain and function in people affected by hemophilia.

INTRODUCTION: Literature provides unclear evidence of the effectiveness of many physiotherapy interventions on pain intensity, quality of life, and functional ability in hemophilic patients, and suggests that rehabilitation programs should be focused on functional goals and the disability of patients. AIM: The aim of the present study is to present the outcome of a case series of patients with hemophilia in which a rehabilitation program has been carried out on the basis of a specific individual patient's functional impairment. METHODS: Fifty-one patients were enrolled: 32 patients (Rehab Group) received a rehabilitative treatment, 19 patients for different reasons (living far from the hospital, family problems) did not receive rehabilitation (Control Group). The rehabilitation program was planned with respect to the emergent problems: musculoskeletal pain, joint range of motion limitation, muscle flexibility, walking difficulties, the appearance of hematomas/hemartro, and postural problems. All the patients were assessed at baseline (T0), after 1 month (T1), and after 3 months (T2) through visual analogic scale for musculoskeletal pain, the Hemophilia Joint Health Score for joint range of motion, the Functional Independence Score in Hemophilia for disability, and postural assessment by visual inspection. RESULTS: A significant reduction of pain and improvement of Hemophilia Joint Health Score and Functional Independence Score in Hemophilia score was found in the Rehab Group along with the follow-up. Pain in the Control Group was lower with respect to the other group at baseline and significantly decreased after 1 month. CONCLUSION: A rehabilitation program focused on individual impairment of patients with hemophylia determined satisfying results in terms of pain control, functional, and disability improvement in 3 months follow-up.

The role of the physiotherapist in the management of people with haemophilia: defining the new normal.

Physiotherapists aim to maximise quality of life and movement potential within the spheres of promotion, prevention, treatment/intervention and rehabilitation. Haemophilia care is witnessing a significant shift towards a new era of potentially life-changing treatments which offer a future of minimal or no bleeds for people with haemophilia. As such, physiotherapy intervention should be more proactive rather than reactive to treat and rehabilitate recurrent bleeding episodes. The role of the physiotherapist within the multidisciplinary team includes the differential diagnosis of musculoskeletal bleeding, supporting and encouraging higher levels of physical activity, rehabilitation to maximise physical potential and capabilities, assessment and treatment of non-bleed-related musculoskeletal issues, managing comorbidities and falls risk, and improving the longitudinal surveillance of musculoskeletal health. Encouraging and supporting people with haemophilia to become more active will improve wellbeing and improve health and health outcomes, and physical activity is becoming one of the most important outcomes for people with haemophilia. Recommendations on the best way to accurately capture these data are vital to ensure the full health benefits of new treatments for people with haemophilia are clear.

Safety and Effectiveness of Progressive Moderate-to-Vigorous Intensity Elastic Resistance Training on Physical Function and Pain in People With Hemophilia.

OBJECTIVE: Strength training is recommended for people with hemophilia; however, published data are anecdotal and have methodological limitations. The purpose of this study was to evaluate the safety and effectiveness of progressive moderate-to-vigorous intensity elastic resistance training on physical function and pain in this patient population. METHODS: A randomized controlled trial was conducted in a university laboratory setting where 20 patients (17 with severe, 1 with moderate, and 2 with mild hemophilia) aged 21 to 53 years received evaluations at baseline and 8-week follow-up. Participants were allocated to intervention (progressive strength training) or control (usual daily activities) groups. The intervention group trained 2 days per week during 8 weeks with elastic resistance. Intensity during the first 2 weeks was a 20-repetition maximum and increased progressively toward 15, 12, and finally 10 repetition maximum. The primary outcome was muscle strength. Secondary outcomes were the Timed "Up and Go" Test score, sit-to-stand, range of motion, Haemophilia Joint Health Score, kinesiophobia score, global impression of pain change, general self-rated health status, and desire to exercise. RESULTS: The intervention group showed greater strength improvements than the control group in almost all of the joints, with moderate to high effect sizes. The intervention group also showed better Timed "Up and Go" and sit-to-stand scores than the control group (moderate effect size), greater range of motion at the knee flexion with the right leg (trivial effect size), and better Haemophilia Joint Health Score at the left knee (small effect size). The intervention group showed greater overall pain reduction, self-rated overall status, and desire to exercise than the control group. CONCLUSIONS: Progressive strength training with elastic resistance performed twice a week during 8 weeks is safe and effective in people with hemophilia to improve muscle strength and functional capacity, reduce general pain, and improve self-rated health status and desire to exercise. IMPACT: This study provides evidence for the use of a specific strength training regimen for people with hemophilia. LAY SUMMARY: People with hemophilia of differing levels of severity, with adequate coverage with clotting factor, can safely engage in progressive strength training and can improve their functioning.

A Blended Physiotherapy Intervention for Persons With Hemophilic Arthropathy: Development Study.

BACKGROUND: 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. OBJECTIVE: The aim of this study was to develop a blended physiotherapy intervention for persons with HA. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Physiotherapy interventions for pain management in haemophilia: A systematic review.

PURPOSE: Approximately 35%-50% of people with haemophilia (PWH) report living with chronic musculoskeletal pain. Although exercise based rehabilitation is effective for pain in other arthritises, there are no published guidelines for management of chronic pain in PWH. This review aims to evaluate and appraise the current evidence of effectiveness of physiotherapy interventions on (a) pain intensity, (b) quality of life (QoL) and (c) function in PWH. METHODS: A systematic review of five databases AMED and CINAHL, EMBASE and MEDLINE and PEDro, as well as trial registries, grey literature and hand searching key journals was completed. Included studies were critically appraised and evaluated for risk of bias. The GRADE approach was used to rate the quality of the evidence. RESULTS: Nine trials consisting of 235 participants met the inclusion criteria. All studies had an overall risk of bias with low methodological quality. Meta-analysis was not possible due to heterogeneity across trials. Studies comparing a range of physiotherapy interventions against no intervention showed no clear beneficial effect on pain intensity or QoL. Only one study, investigating hydrotherapy or land-based exercise against control, showed positive effect for pain intensity, but rated very low on GRADE assessment. Studies comparing one physiotherapy intervention against another showed no clear benefit on pain intensity, QoL or function. LASER with exercise and hydrotherapy were shown to have some positive effects on pain intensity, but no clear benefit on function. CONCLUSIONS: At present, there is limited evidence for the use of physiotherapy interventions in addressing the issue of pain in PWH. Better designed trials with higher quality and explicit methodology along with user involvement are needed to assess the efficacy of any proposed intervention.

Guía de práctica clínica de hemofilia / Hemophilia Clinical Practice Guide

Generar recomendaciones basadas en la mejor evidencia disponible acerca del manejo de personas con hemofilia abordando principalmente tratamiento médico, quirúrgico y rehabilitación.

Telemedicine and telerehabilitation: current and forthcoming applications in haemophilia.

In persons with haemophilia (PWH), the importance of comprehensive disease management to prevent bleeding, joint damage and secondary diseases has been well established. However, because haemophilia is a chronic disease, intervention programmes carried out for prolonged periods of time may create problems of patient adherence. Driven by continuous technological innovation, telemedicine is being increasingly proposed as a way to provide PWH with a range of services designed to improve their health, saving the time and cost involved in going to the treatment centre, and increasing therapeutic adherence. The aim of this article is to identify and discuss the tools available for the management of PWH by means of telemedicine and information technology. Video conferences are helpful to obtain a rapid evaluation at a distance of the occurrence and severity of bleeding episodes by the personnel of the treatment centre. Cell phones and associated applications (apps) help to improve the regular implementation of replacement therapy and monitor any ensuing adverse effect. Portable sensors help to improve lifestyle and to monitor the degree of physical activity through the fulfilment of a given number of daily walking steps and other physical activities. In the context of telerehabilitation, exergames have the potential to improve the musculoskeletal function of PWH by exploiting the recreational features of videogames. Thus, telemedicine and its multiple applications may be useful in the management of haemophilia, especially for patients living far from specialised centres. However, since this is a recent and rapidly evolving field, published studies are few and have, so far, involved only a limited number of cases. Therefore, additional evidence needs to be obtained by means of accrual of cumulative data from multiple centres specialised in haemophilia.

Upper-Body Exercises With External Resistance Are Well Tolerated and Enhance Muscle Activity in People With Hemophilia.

BACKGROUND: Conventional nonresisted therapeutic exercises for people with hemophilia involve a careful, low-intensity approach to avoid injuries. Externally resisted exercise is highly efficient for increasing muscle strength in healthy adults but its feasibility for people with hemophilia remains unknown. OBJECTIVE: The purpose of this study was to evaluate muscle activity during upper-body rehabilitation exercises with 2 types of external resistance and without external resistance (conventional) and to examine tolerability, kinesiophobia, and possible adverse effects derived from the session. DESIGN: This was a cross-sectional study. METHODS: Twelve people with hemophilia A/B (11 with severe hemophilia undergoing prophylactic treatment, 1 with mild hemophilia) participated. During the experimental session, participants completed the Tampa Scale of Kinesiophobia and performed 2 exercises-elbow flexion and shoulder abduction-with 3 conditions for each exercise: elastic resistance (externally resisted), free weights (externally resisted), and conventional nonresisted. Surface electromyography signals were recorded for the biceps brachii, triceps brachii, upper trapezius, and middle deltoid muscles. After the session, exercise tolerability and kinesiophobia were assessed. Adverse effects were evaluated 24 and 48 hours after the session. RESULTS: Externally resisted exercises provided greater muscle activity than conventional nonresisted therapeutic exercises. The exercises were generally well tolerated and there was no change in kinesiophobia following the session. No adverse effects were observed in the following days. LIMITATIONS: Small sample size was the main limitation. CONCLUSIONS: In people with severe hemophilia undergoing prophylactic treatment, elbow flexion and shoulder abduction exercises with external resistance at moderate intensities are feasible and provide greater muscle activity than nonresisted conventional exercises.

Ranges and drivers of risk associated with sports and recreational activities in people with haemophilia: results of the Activity-Intensity-Risk Consensus Survey of US physical therapists.

INTRODUCTION: Limited evidence describes physical activity-associated bleeding risks for people with haemophilia, and risks are usually described only generically. AIM: To assess activity-specific ranges of risk for joint, soft tissue and head bleeds by identifying inherent and modifiable risk factors associated with each activity, based on opinions of expert physical therapists (PTs). METHODS: Physical therapists from US haemophilia treatment centres (HTCs) participated in a survey of 101 physical activities. For each activity, PTs provided minimum/maximum risk scores (low = 1; high = 5), and indicated specific bleeding risks in six joints and three injury types (bruising, muscle bleeding, head injury). Risk drivers were identified from free-text comments and explored at a consensus meeting, where they were categorized as inherent or modifiable and activity-driven or patient-driven. RESULTS: Of 32 invited PTs, 17 participated; median experience was 24 years as a PT and 16 years at an HTC. Only a few activities had a wide range of risk assessments encompassing both lower and upper ends of the response range. Joint injury risks were consistent with position and physical requirements, and head and muscle bleed risks were associated with physical contact. Eight PTs participated in the consensus meeting; key risk drivers identified included progression from seasonal to year-round participation, overtraining and improper body mechanics. Inherent risks included impact with surface/ball/equipment and field/surface condition; modifiable risks included safety equipment and tricks/stunts. CONCLUSIONS: These data provide a framework for discussion with patients/families, recognizing how certain activities may be modified to decrease risk, and identifying activities with nonmodifiable inherent risks.

Effect of whole body vibration training on quadriceps strength, bone mineral density, and functional capacity in children with hemophilia: a randomized clinical trial.

OBJECTIVES: The objective was to evaluate the effects of whole body vibration training on quadriceps strength, bone mineral density, and functional capacity in children with hemophilia. METHODS: Thirty children with hemophilia with age ranging from 9 to 13 years were selected and randomly assigned to either the study group that received whole body vibration training (30-40 Hz, 2-4 mm of peak-to-peak vertical plate displacement for 15 minutes/day, 3 days/week/ 12 weeks) plus the conventional physical therapy program or the control group that performed a conventional physical therapy program only. Outcomes included quadriceps strength, bone mineral density, and the functional capacity. RESULTS: Children in the study group showed a significant improvement in all outcomes compared with the control group. The quadriceps peak torque after treatment was 70.26 and 56.46 Nm for the study and control group, respectively (p⟨0.001). The lumbar spine bone mineral density after treatment was 0.85 and 0.72 g/cm2 for the study and control group, respectively (p⟨0.001). The functional capacity after treatment was 325 and 290 m for the study and control group, respectively (p=0.006). CONCLUSIONS: Whole body vibration training is an effective modality in increasing quadriceps strength, bone mineral density, and functional capacity in children with hemophilia.

Health promotion for young patients with haemophilia.

The haemophilia treatment centre of the Clinic for Children and Youth Medicine in Jena extends medical care by health-promotion measures, namely: health counselling, adjuvant exercise therapy and school sports. In addition to the regular medical checks at the treatment centre patients are examined regarding physical fitness, joint situation, quality of life in general and disease-specific manner, as well as psycho-social and nutritional behaviour. Findings and medical results of the examinations are integrated into an individual advice on therapy, school sports, and health recommendations. This aimed at strengthening health-related resources and minimizing potential injuries. First long-term evaluation shows an increase of activity behaviour and physical fitness without increasing bleeding rate and maintained joint function. CONCLUSION: Combining functional prevention diagnostics and individual health counselling shows signs of improved patient's health knowledge, self-competence and physical fitness.

Follow-up of a 6-month Programmed Sports Therapy (PST) in patients with haemophilia.

Studies describe benefits of exercise in Patients with haemophilia (PwH), but research on training sustainability is rare. Aim of this study was to observe the follow-up of a guided 6-month training intervention in PwH. This study included 28 PwH after a training intervention (RCT) over 6 months. N=17 continued training (TC), N=11 stopped training (TS) after the study time. Strength measurements and subjective physical performance were tested.The RCT revealed that all muscles tested increased significantly after training. In the follow-up phase, the muscles of TC increased further (in TS decreased), but not all of them significantly (M. triceps brachii: p=0.017; M. biceps brachii: n.s.; M. latissimus dorsi: n.s.; M. rectus abdominis: p=0.002; M. biceps femoris right: n.s.; left: p=0.028; M. quadriceps femoris both n.s. = not sig.). Subjective performance showed no clear changes in the follow-up phase.This is the first study evaluating a follow-up phase of programmed sports therapy (PST) in PwH. PST (6-month) resulted in some PwH continuing the successful training. Benefits were training routine, safety and partially further positive training effects, e.g. in strength performance.

Exercise for haemophilia.

BACKGROUND: Haemophilia is a bleeding disorder associated with haemorrhaging into joints and muscles. Exercise is often used to aid recovery after bleeds, and to improve joint function in the presence of arthropathy. OBJECTIVES: Our objective was to systematically review the available evidence on the safety and effectiveness of exercise for people with haemophilia. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register and electronic databases PubMed, OVID-Embase, and CINAHL. We hand searched abstracts from congresses of the World Federation of Hemophilia and the European Hematology Association, trial registries and the reference lists of relevant articles.Date of the last search of the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register: 14 December 2016. SELECTION CRITERIA: Randomized or quasi-randomized controlled studies comparing any exercise intervention considered relevant in haemophilia management including supervised, unsupervised, aquatic, strengthening, aerobic or cardiovascular, stretching, proprioceptive and balance training exercise programs in males of any age with haemophilia A or B of any severity (those with co-morbidities were not excluded). DATA COLLECTION AND ANALYSIS: Two authors reviewed the identified abstracts to determine their eligibility. For studies meeting the inclusion criteria, full articles were obtained. The two authors extracted data and assessed the risk of bias. Any disagreements were resolved by discussion. The authors contacted study investigators to obtain any missing data. MAIN RESULTS: Eight studies were included, which represented 233 males with all severities of haemophilia A and B, ranging in age from eight years to 49 years. Study duration ranged from four to 12 weeks. Exercise interventions varied greatly and included resistance exercises, isometric exercises, bicycle ergometry, treadmill walking and hydrotherapy; therefore, comparison between studies was difficult.None of the studies measured or reported adverse effects from the interventions. None of the studies reported outcomes regarding bleed frequency, quality of life or aerobic activity. Overall risk of bias across all studies was assessed as unclear.Very few studies provided sufficient information for comparison. None of the studies reported data that favoured the control group. One study reported that six weeks of resistance training improved joint health status (Colorado score) compared to controls. The addition of pulsed electromagnetic fields also improved ankle scores compared to exercises alone, but this was not seen in the elbows or knees.Two studies reported statistically significant improvements in pain intensity after exercise interventions compared to controls. Hydrotherapy exercises produced significant decreases in pain compared to controls and land-based exercise groups.Two studies found improvement in joint motion in the exercise group compared to controls. One study compared land- and water-based exercises; there was no difference in the range of motion between the two groups; however, the water-based exercise group did show improvement over the control group.One study, comparing joint traction and proprioceptive neuromuscular facilitation for the elbow to a control group, showed no differences in biceps girth or strength after 12 weeks of intervention.Some studies reported comparisons between interventions. In one study, treadmill training significantly improved balance in children compared to bicycle ergometry. Another study added partial weight bearing exercises to quadriceps exercises and showed improved walking tolerance.Four studies evaluated quadriceps or hamstring strength (or both). The addition of bicycle ergometry and exercises with weights was more effective than static exercises and treadmill walking for strengthening knee flexors and extensors. Partial weight-bearing exercises through range were more effective than static and short arc exercises for improving knee extensor strength. The addition of treadmill walking to ultrasound, stretching and strengthening exercises showed increased peak torque of knee flexors and extensors and decrease in knee effusion.The results should be interpreted with caution due to the quality of evidence (GRADE) as outlined in the summary of findings tables, which demonstrates that all but one of the outcomes assessed were rated as low or very low due to the small sample sizes and potential bias. AUTHORS' CONCLUSIONS: These results must be considered with caution. There is a lack of confidence in the results due to the small number of included studies and the inability to pool the results due to the heterogeneity of outcome measures. Most exercise interventions produced improvement in one or more of the measured outcomes including pain, range of motion, strength and walking tolerance. Hydrotherapy may be more effective than land exercises for pain relief in adults. Functional exercises such as treadmill walking and partial weight bearing exercises seem to be more effective than static or short arc exercises for improving muscle strength. These findings are consistent with the many non-controlled intervention reports in the haemophilia literature. No adverse effects were reported as a result of any of the interventions. However, some groups used prophylactic factor prior to exercise and other groups studied only subjects with moderate haemophilia. Therefore, the safety of these techniques for persons with severe haemophilia remains unclear.

Evidence for and cost-effectiveness of physiotherapy in haemophilia: a Dutch perspective.

INTRODUCTION: Musculoskeletal impact of haemophilia justifies physiotherapy throughout life. Recently the Dutch Health Care Institute constrained their 'list of chronic conditions', and withdrew financial coverage of physiotherapy for elderly persons with haemophilia (PWH). This decision was based on lack of scientific evidence and not being in accordance with 'state of science and practice'. METHODS: In general, evidence regarding physiotherapy is limited, and especially in rare diseases like haemophilia. 'Evidence based medicine' classifies and recommends evidence based on meta-analyses, systematic reviews and randomized controlled trials, but also means integrating evidence with individual clinical expertise. For the evaluation of physiotherapy - usually individualized treatment - case studies, observational studies and Case Based Reasoning may be more beneficial. RESULTS: Overall annual treatment costs for haemophilia care in the Netherlands are estimated over 100 million Euros, of which 95% is covered by clotting factor concentrates. The cost for physiotherapy assessments in all seven Dutch HTCs (seven centres for adult PWH and seven centres for children) is limited at approximately 500 000 Euros annually. Costs of the actual physiotherapy sessions, carried out in our Dutch first-line care system, will also not exceed 500 000 Euros. Thus, implementation of physiotherapy in haemophilia care the Netherlands in a most optimal way would cost less than 1% of the total budget. AIM: The present paper describes the role of physiotherapy in haemophilia care including available evidence and providing suggestions regarding generation of evidence. Establishing the effectiveness and cost-effectiveness of physiotherapy in haemophilia care is a major topic for the next decennium.