European principles of care for physiotherapy provision for persons with inherited bleeding disorders: Perspectives of physiotherapists and patients.

INTRODUCTION: In their Chronic Care Model, the World Health Organisation states that people with chronic disorders and their families should be informed about the expected course, potential complications, and effective strategies to prevent complications and manage symptoms. Physiotherapists are a key professional group involved in the triage, assessment and management of musculoskeletal conditions of persons with a bleeding disorder (PWBD). Nevertheless, recent reports describe access to physiotherapy for those with these conditions is only sometimes available. AIM: Access to high quality individualised physiotherapy should be ensured for all PWBD, including those with mild and moderate severities, male and female, people with von Willebrand Disease (vWD) and other rare bleeding disorders. Physiotherapy should be viewed as a basic requisite in their multidisciplinary care. METHODS/ RESULTS: Following a series of meetings with physiotherapists representing the European Association for Haemophilia and Allied Disorders (EAHAD) and PWBD representing the European Haemophilia Consortium (EHC) and a review of publications in the field, eight core principles of physiotherapy care for persons with a bleeding disorder have been co-produced by EAHAD and EHC. CONCLUSION: These eight principles outline optimum standards of practice in order to advocate personalised patient-centred care for physical health in which both prevention and interventions include shared decision making, and supported self-management.

Physiotherapist inter-rater reliability of the Haemophilia Early Arthropathy Detection with Ultrasound protocol.

INTRODUCTION: Prevention of arthropathy is the major goal of haemophilia treatment, and early detection of the first signs of joint damage is important so that prevention strategies can be initiated to limit physical disability and improve quality of life. AIM: The aim of this study was to determine the inter-rater repeatability of the HEAD-US protocol when performed by haemophilia physiotherapists. METHODS: Sixty-three joints (21 elbows, 21 knees and 21 ankles) were examined in 21 patients (mean age; 29.14 ± 10.09 years) according to the HEAD-US protocol by four of six physiotherapists blinded to clinical information. Inter-rater repeatability was evaluated by comparing the concordance of scores between the different clinicians using weighted Kappa (κw ) statistics and 95% confidence intervals. RESULTS: Substantial agreement was found when evaluating synovial hypertrophy (κw  = 0.69), articular cartilage (κw  = 0.60) and bone (κw  = 0.74). Near perfect repeatability (κw  > 0.80) was found when scoring synovitis profiles of the elbow joint and bone profiles of the knee joint. Repeatability was moderate for scoring synovitis and articular cartilage profiles of the ankle joint. A moderate correlation between HEAD-US and HJHS was found for the elbow and ankle joint and a strong correlation for the knee joint. Discordance between clinical and ultrasound examination was 19.30%. CONCLUSION: Overall, we found repeatability of the HEAD-US protocol was good when performed by physiotherapists, and our results support the emerging view that US imaging complements the physical examination when screening and monitoring joint health of people with haemophilia at the point of care.

Identifying biomechanical gait parameters in adolescent boys with haemophilia using principal component analysis.

INTRODUCTION: Improvements in the medical management for those with haemophilia have resulted in improved clinical outcomes. However, current treatment regimens do not alleviate all joint haemarthroses with the potential for long-term joint deterioration remaining. The evaluation of functional activities such as gait, using standardized tools to monitor children with haemophilia is emerging. AIM: This study explored differences in sagittal plane biomechanics of walking in adolescent boys aged 11-18 years with haemophilia and an age-matched group of typically developing boys. METHODS: A motion capture system and 2 force platforms were used to collect sagittal plane kinematic, kinetic and temporal spatial data during level walking. Principal component analysis (PCA) was applied to kinematic and kinetic waveform variables. Group differences in temporal spatial and principal component scores for each kinematic and kinetic variable were evaluated using independent t tests. RESULTS: Significant alterations (P < .05) in temporal spatial and kinetic parameters were found in adolescent boys with haemophilia. Compared with typically developing adolescent boys, boys with haemophilia walked with reduced stance phase duration and altered pattern of external ankle joint moments during push off and the beginning of swing. CONCLUSION: The use of PCA rather than predetermined discriminatory variables provided additional insight into biomechanical alterations in adolescent boys with haemophilia, with adaptations occurring during terminal double support and early swing, affecting the ankle joint. This finding might be a key biomechanical marker that could be used to evaluate the joint function and the progression of early haemophilic arthropathy.

Relationship between physical function and biomechanical gait patterns in boys with haemophilia.

INTRODUCTION: The World Federation of Haemophilia recommends joint and muscle health is evaluated using X-ray and magnetic resonance imaging, together with clinical examination scores. To date, inclusion of performance-based functional activities to monitor children with the condition has received little attention. AIM: To evaluate test-retest repeatability of physical function tests and quantify relationships between physical function, lower limb muscle strength and gait patterns in young boys with haemophilia. METHODS: Timed 6-minute walk, timed up and down stairs, timed single leg stance, muscle strength of the knee extensors, ankle dorsi and plantar flexors, together with joint biomechanics during level walking were collected from 21 boys aged 6-12 years with severe haemophilia. RESULTS: Measures of physical function and recording of muscle strength with a hand-held myometer were repeatable (ICC > 0.78). Distances walked in six minutes, time taken to go up and down a flight of stairs and lower limb muscle strength correlated closely with ankle range of motion, together with peak knee flexion and ankle dorsi and plantarflexion moments during walking (P < 0.05). CONCLUSION: Alterations in gait patterns of boys with haemophilia appear to be associated with changes in performance of physical function and performance seems to depend on their muscle strength. Timed 6-minute walk test, timed up and down steps test and muscle strength of the knee extensors showed the strongest correlation with biomechanical joint function, and hence might serve as a basis for the clinical monitoring of physical function outcomes in children with haemophilia.

Outcome measures monitoring physical function in children with haemophilia: a systematic review.

Our objective was to provide a synthesis of measurement properties for performance-based outcome measures used to evaluate physical function in children with haemophilia. A systematic review of articles published in English using Medline, PEDro, Cinahl and The Cochrane Library electronic databases was conducted. Studies were included if a performance-based method, clinical evaluation or measurement tool was used to record an aspect of physical function in patients with haemophilia aged ≤ 18 years. Recording of self-perceived or patient-reported physical performance, abstracts, unpublished reports, case series reports and studies where the outcome measure was not documented or cross-referenced was excluded. Description of outcome measures, patient characteristics, measurement properties for construct validity, internal consistency, repeatability, responsiveness and feasibility was extracted. Data synthesis of 41 studies evaluating 14 measures is reported. None of the outcome measures demonstrated the requirements for all the measurement properties. Data on validity and test-retest repeatability were most lacking together with studies of sufficient size. Measurement of walking and muscle strength demonstrated good repeatability and discriminative properties; however, correlation with other measures of musculoskeletal impairment requires investigation. The Haemophilia Joint Health Score demonstrated acceptable construct validity, internal consistency and repeatability, but the ability to discriminate changes in physical function is still to be determined. Rigorous evaluation of the measurement properties of performance-based outcome measures used to monitor physical function of children with haemophilia in larger collaborative studies is required.

Influence of ankle plantar flexor muscle architecture and strength on gait in boys with haemophilia in comparison to typically developing children.

Altered gait patterns, muscle weakness and atrophy have been reported in young boys with severe haemophilia when compared to unaffected peers. The aim of this study was to determine whether lateral gastrocnemius muscle size and architecture influenced biomechanical walking patterns of boys with haemophilia and if these relationships differed from age-matched typically developing boys. Biomechanical function of the knee and ankle during level walking, lateral gastrocnemius anatomical cross-sectional area, thickness, width, fascicle length and pennation angle and ankle plantar flexor muscle strength were recorded in 19 typically developing boys aged 7-12 years and 19 age-matched haemophilic boys with a history of ankle joint bleeding. Associations between gait, strength and architecture were compared using correlations of peak gait values. Haemophilic boys walked with significantly larger (P < 0.05) ankle dorsi flexion angles and knee flexion moments. The ankle plantar flexor muscles of haemophilic boys were significantly weaker and smaller when compared to typically developing peers. In the typically developing boys there was no apparent association between muscle architecture, strength and walking patterns. In haemophilic boys maximum muscle strength and ACSA normalized torque of the ankle plantar flexors together with the muscle width, thickness, fascicle length and angulation (P < 0.05) were associated with motion at the ankle and peak moments at the knee joint. Muscle strength deficits of the ankle plantar flexors and changes in muscle size and architecture may underpin the key biomechanical alterations in walking patterns of haemophilic boys with a history of ankle joint bleeding.

Orthopaedic co-morbidities in the elderly haemophilia population: a review.

Due to improvements in the treatment and medical care of haemophilia, the life expectancy of individuals with haemophilia has approached that of the general population. To review the main co-morbidities of the musculoskeletal system in elderly persons with haemophilia, we have performed a review of the literature on the musculoskeletal problems of elderly haemophiliacs. Chronic arthropathy is the main co-morbidity in the ageing person with haemophilia. Age-related orthopaedic co-morbidities include degenerative joint changes, osteoporosis, muscle atrophy or sarcopenia, muscle weakness and disturbance of gait and balance. Increased pain, muscle weakness and atrophy along with an increased risk of falling are key features of advanced haemophilic arthropathy and ageing. An ageing haemophilia population in which arthropathy continues to be the primary co-morbidity is a current challenge for those responsible for their care. Exercise programmes undertaken two to three times per week for at least 12 weeks seem most effective in reducing the impact of age-related changes on the musculoskeletal system. Establishing effective exercise programmes and strategies to identify individuals who would benefit from early surgical intervention together with presurgical physiotherapy prehabilitation is a priority for future research.

Consensus recommendations for the use of FEIBA(®) in haemophilia A patients with inhibitors undergoing elective orthopaedic and non-orthopaedic surgery.

A growing number of publications have described the efficacy and safety of FEIBA as a first-line haemostatic agent for surgical procedures in haemophilia A patients with high-responding FVIII inhibitors. The aim of this study was to provide practical guidance on patient management and selection and also to communicate a standardized approach to the dosing and monitoring of FEIBA during and after surgery. A consensus group was convened with the aims of (i) providing an overview of the efficacy and safety of FEIBA in surgery; (ii) sharing best practice; (iii) developing recommendations based on the outcome of (i) and (ii). To date there have been 17 publications reporting on the use of FEIBA in over 210 major and minor orthopaedic and non-orthopaedic surgical procedures. Haemostatic outcome was rated as 'excellent' or 'good' in 78-100% of major cases. The reporting of thromboembolic complications or anamnestic response to FEIBA was very rare. Key to the success of FEIBA as haemostatic cover in surgery is to utilize the preplanning phase to prepare the patient both for surgery and also for rehabilitation. Haemostatic control with FEIBA should be continued for an adequate period postoperatively to support wound healing and to cover what can in some patients be an extended period of physiotherapy. Published data have demonstrated that FEIBA can provide adequate, well tolerated, peri and postoperative haemostatic cover for a variety of major and minor surgical procedures in patients with haemophilia A. The consensus recommendations provide a standardized approach to the dosing and monitoring of FEIBA.

Comparison of muscle strength and in-vivo muscle morphology in young children with haemophilia and those of age-matched peers.

Recent advances in imaging have enhanced our understanding of the morphological adaptations of muscle in response to disease and altered use. Adaptation in muscle morphology has been linked to changes in muscle strength. To date, no studies have compared muscle morphology and strength in young children with haemophilia to that of typically developing children. This study explored differences in muscle strength and morphology between typically developing and age and size-matched boys aged 6-12 years with haemophilia and a history of recurrent haemorrhage in the ankle joint. Maximum muscle strength of the knee flexors (KF), extensors (KE), ankle dorsi (ADF) and plantar flexors (APF) was measured in 19 typically developing boys (Group 1) and 19 boys with haemophilia (Group 2). Ultrasound images of vastus lateralis (VL) and lateral gastrocnemius (LG) were recorded to determine muscle cross-sectional area (CSA), thickness, width, fascicle length and pennation angle. Muscle strength of the KE, ADF and APF were significantly (P < 0.05) lower in Group 2 when compared with Group 1. Muscle CSA and width of VL were significantly smaller and pennation angles significantly larger in Group 2 (P < 0.05). Muscle CSA and thickness of LG were significantly (P < 0.05) smaller in Group 2. Linear regression showed that LG muscle CSA and thickness were significant (P < 0.01) predictors of APF muscle strength. Following ankle joint bleeding in young boys with haemophilia, secondary adaptations in muscle strength and morphology were observed, suggesting that muscle function is more impaired than current clinical evaluations imply.

How does a lifetime of painful experiences influence sensations and beliefs about pain in adults with severe haemophilia? A qualitative study.

PURPOSE: To explore the life experiences of pain in people with severe haemophilia and understand how such experiences influence beliefs and sensation of pain in adulthood. METHODS: A qualitative inquiry approach using focus groups and semi-structured individual interviews was used. Participants included people with severe haemophilia living with chronic pain. Data were analysed using reflexive thematic analysis. RESULTS: Fourteen men with a median age of 47 (range 23-73) agreed to take part. Eleven participated in two focus groups and three were interviewed over telephone. Two themes were conceptualised from the data: (i) haemophilia and pain - an evolving life biography (the personal narrative, historical, social, and medical context, continuous adaptation of activity choices, surveillance of pain and its meaning); (ii) "My normal isn't normal" - identity and self-agency (pain as a feature of life and identify with severe haemophilia, loss of enjoyable activities balanced against staying active, barriers to participation). CONCLUSIONS: Pain is a constantly evolving, lifetime feature for many adults with haemophilia and it is viewed as part of their identity with their condition. Healthcare professionals working in haemophilia should try to better understand the influence of an individuals lived experience with their haemophilia on beliefs and behaviours of pain.Implications for rehabilitationSevere haemophilia is a rare bleeding disorder that results in musculoskeletal joint disease.Adults with severe haemophilia have experienced multiple episodes of bleeding related musculoskeletal pain since childhood.Pain beliefs and behaviours in adulthood appear to be influenced by a lifetime of painful experiences associated with haemophilia.In order to better support people with haemophilia and chronic pain, healthcare professionals in haemophilia need to better understand how an individuals lived experience of pain helps inform their beliefs about it.

The experiences and beliefs of people with severe haemophilia and healthcare professionals on pain management, and their views of using exercise as an aspect of intervention: a qualitative study.

PURPOSE: To explore the experiences, views and beliefs of people with severe haemophilia and healthcare professionals (HCPs) on approaches for pain management, as well as their views on exercise being used as an aspect of management. METHODS: Taking a qualitative inquiry approach using focus groups and semi-structured interviews, participants included people with severe haemophilia living with chronic pain and haemophilia HCPs. Data were analysed using reflexive thematic analysis. RESULTS: Fourteen men with haemophilia with a median age of 47 (range 23-73) and six haemophilia HCPs agreed to participate. Of the people with haemophilia, 11 attended two focus groups and three were interviewed over telephone. Healthcare professionals were interviewed face-to-face. Two themes were conceptualised from the data: (i) haemophilia management and pain management is discordant (imbalance between good haemophilia care but poor pain management, historical medico-social influences on pain management, the need for trust); (ii) uncertain about exercise but clear on what matters (conflicting views on exercise, the need for proof of safety, personalised care). CONCLUSIONS: Options for effective pain management remain limited and what is used is heavily influenced by beliefs and experience. Exercise as a treatment option in pain management is conceptually acceptable for people with haemophilia. Effective pain management requires understanding of individual beliefs and fears, and a personalised approach supported by knowledgeable, trusted clinicians.Implications for rehabilitationMusculoskeletal joint pain and its relationship with bleeding in people with haemophilia continues to be a management challenge.Current pain management strategies are of limited effectiveness with little evidence of an approach that reflects the multi-modal pain experience.Whilst exercise and rehabilitation approaches are conceptually possible for people with severe haemophilia, barriers remain regarding perception of overall safety and effectiveness.People with severe haemophilia may consider exercise as part of a pain management strategy if it is individualised, and they are supported to do it by clinicians who understand them and their haemophilia.

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.

Comparison of biomechanical gait parameters of young children with haemophilia and those of age-matched peers.

Quality of life for children with haemophilia has improved since the introduction of prophylaxis. The frequency of joint haemorrhages has reduced, but the consequences of reduced bleeding on the biomechanical parameters of walking are not well understood. This study explored the differences in sagittal plane biomechanics of walking between a control group (Group 1) of normal age-matched children and children with haemophilia (Group 2) with a target ankle joint. A motion capture system and two force platforms were used to collect sagittal plane kinematic, kinetic and temporal-spatial data during walking of 14 age-matched normal children and 14 children with haemophilia aged 7-13 years. Group differences in maximum and minimum flexion/extension angles and moments of the hip, knee and ankle joints, ground reaction forces and temporal-spatial gait cycle parameters were analysed using one-way anova. Significant changes (P < 0.05) in kinematic and kinetic parameters but not temporal-spatial parameters were found in children with haemophilia; greater flexion angles and external moments of force at the knee, greater ankle plantarflexion external moments and lower hip flexion external moments. These results suggest that early biomechanical changes are present in young haemophilic children with a history of a target ankle joint and imply that lower limb joint function is more impaired than current clinical evaluations indicate. Protocols and quantitative data on the biomechanical gait pattern of children with haemophilia reported in this study provide a baseline to evaluate lower limb joint function and clinical progression.

Changing patterns of bleeding in patients with severe haemophilia A.

Previous studies on the pattern of joint bleeding in patients with haemophilia have reported that the knee joint is most frequently affected. Home treatment data reporting bleeding frequency and location collected from 100 patients registered at six haemophilia centres in the UK have been analysed to determine current patterns of bleeding. Bleeding frequency has markedly decreased although bleeding into joints remains the main characteristic of haemophilia. However, the ankle joint has replaced the knee joint as the most common joint affected. Furthermore, it seems that the frequency of knee joint bleeding is also less than the elbow joint suggesting that the traditional pattern of joint bleeding in haemophilia has now changed significantly.

Rehabilitation of patients with haemophilia after orthopaedic surgery: a case study.

Preoperative and postoperative rehabilitation may be useful for improving the recovery of patients undergoing orthopaedic surgery, particularly in those with co-morbidity or special requirements. This case study, of a patient with haemophilia and inhibitors to factor VIII undergoing total knee replacement, demonstrates the benefits of 6 weeks' preoperative physiotherapy ('prehabilitation') combined with 6 weeks' postoperative rehabilitation. The supervised physiotherapy regimen was individually tailored to specifically increase range of motion and muscle strength, enabling rapid mobilization and recovery of function, whilst minimizing the risk of bleeding.