Recommendations on multidisciplinary management of elective surgery in people with haemophilia.

Planning and undertaking elective surgery in people with haemophilia (PWH) is most effective with the involvement of a specialist and experienced multidisciplinary team (MDT) at a haemophilia treatment centre. However, despite extensive best practice guidelines for surgery in PWH, there may exist a gap between guidelines and practical application. For this consensus review, an expert multidisciplinary panel comprising surgeons, haematologists, nurses, physiotherapists and a dental expert was assembled to develop practical approaches to implement the principles of multidisciplinary management of elective surgery for PWH. Careful preoperative planning is paramount for successful elective surgery, including dental examinations, physical assessment and prehabilitation, laboratory testing and the development of haemostasis and pain management plans. A coordinator may be appointed from the MDT to ensure that critical tasks are performed and milestones met to enable surgery to proceed. At all stages, the patient and their parent/caregiver, where appropriate, should be consulted to ensure that their expectations and functional goals are realistic and can be achieved. The planning phase should ensure that surgery proceeds without incident, but the surgical team should be ready to handle unanticipated events. Similarly, the broader MDT must be made aware of events in surgery that may require postoperative plans to be changed. Postoperative rehabilitation should begin soon after surgery, with attention paid to management of haemostasis and pain. Surgery in patients with inhibitors requires even more careful preparation and should only be undertaken by an MDT experienced in this area, at a specialized haemophilia treatment centre with a comprehensive care model.

Postural control during a transition task in haemophilic children, adolescents and young adults with haemophilic ankle arthropathy.

BACKGROUND: The aim of this study was to determine whether young haemophilic boys with and without MRI-based signs of ankle arthropathy demonstrate reduced balance ability during a transition task with eyes open and eyes closed. METHODS: Thirty-four haemophilic bodies and 28 typically developing boys aged 6-20 years participated to this study. Structural integrity of the tarsal foot joints of all haemophilic boys was assessed with MRI. All participants performed a standard transition task from double-leg stance to single-leg stance with eyes open and eyes closed. Comparison of balance features derived from the centre of pressure displacement captured by a single force platform was performed between the different haemophilia subgroups and sex-age-height matched peers. FINDINGS: The haemophilic boys without signs of arthropathy presented only a higher intermediate phase velocity during the eyes closed condition (P = .05). The haemophilic boys with signs of arthropathy had significantly higher displacement after the time to new stability point, and 95% Ellipse Sway Area and Balance Area compared to their matched peers during eyes open test (P < .05). Similar findings were observed during the eyes closed test for the displacement after the time to new stability point and 95% Ellipse Sway Area (P < .05). No significant differences were observed between affected and non-affected side of the unilateral affected patients. INTERPRETATION: We suggest that the pathophysiological cascade associated with chronic bleeding episodes should not be considered as a "simple" musculoskeletal injury, hence more as a complex neurophysiological dysfunction which may originate both from unilateral and bilateral deterioration of the musculoskeletal system.

Assessment of passive musculoarticular ankle stiffness in children, adolescents and young adults with haemophilic ankle arthropathy.

OBJECTIVES: To measure passive musculoarticular ankle stiffness (PMAAS) and its intra- and interday reliability in adult control subjects without ankle disorders. We also sought to quantify PMAAS in children, adolescents and young adults with haemophilia (CAAwH) taking into account the accurate tibiotalar and subtalar joints structural status obtained by magnetic resonance imaging (MRI). METHODS: We included 23 CAAwH and 23 typically developing boys (TDB) matched by age, weight and height, along with 25 healthy volunteers for reliability assessment. All CAAwH underwent bilateral ankle MRI, with anatomical status assessed using the International Prophylaxis Study Group MRI scale. All CAAwH underwent PMAAS testing for both sides randomly vs the dominant side (DS) in TDBs. For assessing viscous stiffness (VS) and elastic stiffness (ES), eight different oscillation frequencies were randomly repeated three times for each subject. RESULTS: Good-to-excellent intra- and interday reliability was observed for ES and VS variables. No relevant differences were observed between the ankle viscoelastic properties in CAAwH without joint damage and matched TDBs, whereas the study revealed significantly increased ES in the affected ankles of CAAwH with severe unilateral joint involvement compared to the non-affected joint. CONCLUSION: This study confirmed increased ES in the severely affected ankles of CAAwH compared to non-affected sides. No differences in the ankle viscoelastic properties of CAAwH with or without joint damage were observed, however, compared to matched TDB.

Reliability and clinical features associated with the IPSG MRI tibiotalar and subtalar joint scores in children, adolescents and young adults with haemophilia.

OBJECTIVES: To assess the reliability of the IPSG MRI scale for tibiotalar (TTJ) and subtalar joint (STJ) changes in young haemophilic patients, correlating MRI findings with functional scores and 3D-rearfoot kinematics. METHODS: A total of 37 haemophilic patients underwent bilateral MRI of the footankle, clinical evaluation and quantitative assessment of their 3D-rearfoot kinematics during walking. TTJ and STJ soft tissues were assessed twice along with osteochondral changes by two radiologists using the IPSG MRI scale. Inter- and intra-observer reproducibility of MRI scoring were tested by means of kappa statistics. Correlational analyses were performed between MRI findings and the Haemophilia Joint Health Score 2.1 (HJHS) and 3D-rearfoot kinematic data. RESULTS: The intra-reader reliability of MRI scoring was good to excellent (Kappa: 0.62-1), whereas the inter-reader reliability was moderate to good (Kappa: 0.54-0.79). Weak yet significant correlations were found between the frontal plane rearfoot range of motion (ROM) during loading response of gait and STJ score, as well as between frontal plane rearfoot ROM during the terminal stance phase and the rearfoot osteochondral lesions. CONCLUSION: The IPSG score appears applicable to not only the TTJ but also the STJ. Contrary to TTJ lesions, those of the STJ do not correlate with the HJHS but do with 3D-rearfoot kinematic data.

Deficits of ankle muscle strength not found in children, adolescents and young adults with haemophilic ankle arthropathy.

OBJECTIVES: Adequate management of haemophilia patients requires early detection of joint impairment in relatively asymptomatic patients. This study sought to quantify the impact of the ankle's structural impairment on muscle strength in children, adolescent and young adults with haemophilia (CAAwH). METHODS: Twenty-three CAAwH underwent bilateral magnetic resonance imaging (MRI) assessing the anatomical status of tibiotalar joint (TTJ) and subtalar joint (STJ) using the International Prophylaxis Study Group MRI scale. An isokinetic dynamometer enabled a detailed evaluation of muscle strength at slow and fast speed. In parallel, 10 typically developing healthy boys (TDB) participated in a 1-week interval test-retest assessment to assess the test's reliability. RESULTS: Forty-six MRI ankle scores were obtained, with 11 patients unilaterally affected and one bilaterally. Of the 13 affected feet, nine showed abnormalities at TTJ, three at the posterior STJ and the remaining one at both joints. Muscle strength was not reduced in CAAwH exhibiting TTJ and/or STJ arthropathy, as compared to healthy TDB, nor was there any difference between the CAAwH's affected or unaffected sides. CONCLUSION: Contrarily to adult patients, CAAwH with repeated ankle bleeding may be less impaired than current structural evaluations imply, with possibly a latency between the occurrence of structural and functional damage.

Biomechanical markers and theoretical concepts related to haemophilic ankle and subtalar joint arthropathy: introducing the term 'haemophilic tarsal pan-arthropathy'.

BACKGROUND: Although regular factor replacement can reduce the incidence of joint bleeds and slow down the development of haemophilic arthropathy, the ankle joint remains particularly vulnerable even in children with haemophilia on primary or secondary prophylaxis and is now the primary joint affected. The heterogeneity in the pathoaetiology of haemophilic ankle arthropathy means that the functional consequences of early stage of ankle arthropathy are difficult to define as early morphological and structural changes can be observed in clinically asymptomatic ankles. In this context, understanding biomechanics of the normal and arthritic foot is complex and difficult to quantify unless considering the foot as multiple functional segments using more sophisticated assessment tools such as multisegment foot models. However, this understanding can undoubtedly aid in the analysis of an underlying clinical problem and provide a strategic basis for a more optimal management. AIMS: The purpose of this narrative review was firstly to revise information on the anatomy and biomechanics of the foot and ankle. Finally, related biomechanical markers of human motor performance, which are potentially implicated in the development of haemophilic ankle arthropathy, will be discussed based on published literature and expert opinion. MATERIALS AND METHODS: Searches in published literature were limited to the year 2000 onwards. RESULTS: Although the ankle (tibiotalar joint) is the most commonly affected joint, associated subtalar joint (SJT) involvement is often seen. This would therefore imply that an alternative phraseology might be better. DISCUSSION AND CONCLUSION: In this context, the authors propose the use of 'haemophilic tarsal pan-arthropathy' (HTPA) which encompasses both tibiotalar and subtalar joints.

Managing haemophilia for life: 4th Haemophilia Global Summit.

The 4th Haemophilia Global Summit was held in Potsdam, Germany, in September 2013 and brought together an international faculty of haemophilia experts and delegates from multidisciplinary backgrounds. The programme was designed by an independent Scientific Steering Committee of haemophilia experts and explored global perspectives in haemophilia care, discussing practical approaches to the optimal management of haemophilia now and in the future. The topics outlined in this supplement were selected by the Scientific Steering Committee for their relevance and potential to influence haemophilia care globally. In this supplement from the meeting, Jan Astermark reviews current understanding of risk factors for the development of inhibitory antibodies and discusses whether this risk can be modulated and minimized. Factors key to the improvement of joint health in people with haemophilia are explored, with Carlo Martinoli and Víctor Jiménez-Yuste discussing the utility of ultrasound for the early detection of haemophilic arthropathy. Other aspects of care necessary for the prevention and management of joint disease in people with haemophilia are outlined by Thomas Hilberg and Sébastian Lobet, who highlight the therapeutic benefits of physiotherapy and sports therapy. Riitta Lassila and Carlo-Federico Perno describe current knowledge surrounding the risk of transmission of infectious agents via clotting factor concentrates. Finally, different types of extended half-life technology are evaluated by Mike Laffan, with a focus on the practicalities and challenges associated with these products.

The benefits of exercise for patients with haemophilia and recommendations for safe and effective physical activity.

Most health care professionals involved in the management of people with haemophilia (PWH) believe that exercise is beneficial and its practice is widely encouraged. This article aims to demonstrate that appropriate exercise (adapted to the special needs of the individual PWH) may be beneficial for all PWH through improved physical, psychosocial and medical status. Based on evidence gathered from the literature, many PWH, particularly those using long-term prophylaxis or exhibiting a mild/moderate bleeding phenotype, are as active as their healthy peers. PWH experience the same benefits of exercise as the general population, being physically healthier than if sedentary and enjoying a higher quality of life (QoL) through social inclusion and higher self-esteem. PWH can also gain physically from increased muscle strength, joint health, balance and flexibility achieved through physiotherapy, physical activity, exercise and sport. Conversely, very little data exist on activity levels of PWH in countries with limited resources. However, regarding specific exercise recommendations in PWH, there is a lack of randomized clinical trials, and consequently formal, evidence-based guidelines have not been produced. Based on published evidence from this review of the literature, together with the clinical experience of the authors, a series of recommendations for the safe participation of PWH in regular physical activities, exercises and sport are now proposed. In summary, we believe that appropriately modified programmes can potentially allow all PWH to experience the physical and psychosocial benefits of being physically active which may ultimately lead to an improved QoL.

Impact of multiple joint impairments on the energetics and mechanics of walking in patients with haemophilia.

Few studies have assessed the changes produced by multiple joint impairments (MJI) of the lower limbs on gait in patients with haemophilia (PWH). In patients with MJI, quantifiable outcome measures are necessary if treatment benefits are to be compared. This study was aimed at observing the metabolic cost, mechanical work and efficiency of walking among PWH with MJI and to investigate the relationship between joint damage and any changes in mechanical and energetic variables. This study used three-dimensional gait analysis to investigate the kinematics, cost, mechanical work and efficiency of walking in 31 PWH with MJI, with the results being compared with speed-matched values from a database of healthy subjects. Regarding energetics, the mass-specific net cost of transport (C(net)) was significantly higher for PWH with MJI compared with control and directly related to a loss in dynamic joint range of motion. Surprisingly, however, there was no substantial increase in mechanical work, with PWH being able to adopt a walking strategy to improve energy recovery via the pendulum mechanism. This probable compensatory mechanism to economize energy likely counterbalances the supplementary work associated with an increased vertical excursion of centre of mass (CoM) and lower muscle efficiency of locomotion. Metabolic variables were probably the most representative variables of gait disability for these subjects with complex orthopaedic degenerative disorders.

Functional impact of custom-made foot orthoses in patients with haemophilic ankle arthropathy.

Although foot orthoses are often prescribed to patients with haemophilia (PWH) and ankle arthropathy, the efficacy and biomechanical effects of such devices are not fully understood. We experimentally investigated the effects of orthopedic insoles (OI) and shoes (OS) in PWH presenting ankle arthropathy, with specific attention being paid to pain, spatiotemporal parameters, kinematics and kinetics of lower limb joints, as well mechanical and energetic variables. Using three-dimensional gait analysis (3DGA), synchronous kinematics, kinetics, spatiotemporal, mechanics, and metabolic gait parameters were measured in 16 PWH with ankle arthropathy. The revised Foot Function Index (FFI-R) and 3DGA were determined in patients wearing neutral running shoes at two time points (T0 and T1), with OI (n = 11) or OS (n=5) being subsequently prescribed. Patients, while wearing their orthoses, were re-evaluated using 3DGA, FFI-R, and satisfaction questionnaires (T2). OI and OS provided significant pain relief and comfort improvement in more than half of the patients, with minimal side effects. OI had limited impact on gait pattern, whereas OS significantly improved the propulsive function of the ankle. Biomechanical changes induced by OI and OS were independent of their ability to improve comfort, while being insufficient to influence knee and hip kinematics and kinetics, or mechanical and energetic variables. These findings suggest that OI and OS may have beneficial effects on ankle joints in PWH. Self-reported clinical tools such as FFI-R and satisfaction questionnaires are sufficiently sensitive for assessing the efficacy of foot orthoses in PWH.

Body structure versus body function in haemophilia: the case of haemophilic ankle arthropathy.

Imaging and clinical scores are the main tools used to evaluate the progression of haemophilic arthropathy (HA). Based on haemophilic ankle arthropathy, this study aimed to explore the concordances between structural and clinical alterations, determined by standard radiological and clinical scores, and functional alterations assessed by three-dimensional gait analysis (3DGA). In total, 21 adult haemophilia patients underwent extensive ankle evaluation using the physical examination part of the World Federation of Haemophilia joint score, the Arnold-Hilgartner and the Pettersson radiological scores, and self-reported ankle function assessment using the revised Foot Function Index. Significant associations were found between self-reported ankle function assessment and the three 3DGA variables representative of joint function (range of motion, peak plantar flexion moment, and peak power generated at the push-off phase). Radiological and clinical scores were compared with ankle muscle peak power measurement, the most reliable 3DGA gait variable for ankle function. No significant associations were found between both clinical and functional scores and the 3DGA functional assessment. This discordance may be explained by the lack of a direct relationship between functional alterations detected by 3DGA and the structural changes assessed using X-ray or clinical scoring. Another explanation may be the limitation of clinical and radiological scoring systems in properly determining the severity of HA. Functional assessments such as 3DGA should be used more frequently when monitoring the progression of ankle arthropathy or the effects of therapeutic interventions in adult haemophilia patients.

Natural progression of blood-induced joint damage in patients with haemophilia: clinical relevance and reproducibility of three-dimensional gait analysis.

SUMMARY: A major complication in haemophilia is the destruction of joint cartilage because of recurrent intraarticular and intramuscular bleeds. Therefore, joint assessment is critical to quantify the extent of joint damage, which has traditionally been evaluated using both radiological and clinical joint scores. Our study aimed to evaluate the natural progression of haemophilic arthopathy using three-dimensional gait analysis (3DGA) and to assess the reproducibility of this technique. We hypothesized that the musculoskeletal function was relatively stable in patients with haemophilia. Eighteen adults with established haemophilic arthropathies were evaluated twice by 3DGA (mean follow-up: 18 +/- 5 weeks). Unexpectedly, our findings revealed infraclinical deterioration of gait pattern, characterized by a 3.2% decrease in the recovery index, which is indicative of the subject's ability to save energy while walking. A tendency towards modification of segmental joint function was also observed. Gait analysis was sufficiently reproducible with regards to spatiotemporal parameters as well as kinetic, mechanical and energetic gait variables. The kinematic variables were reproducible in both the sagittal and frontal planes. In conclusion, 3DGA is a reproducible tool to assess abnormal gait patterns and monitor natural disease progression in haemophilic patients.

The role of physiotherapy after total knee arthroplasty in patients with haemophilia.

With the availability of clotting factor concentrates, advances in surgical techniques, better implant design, and improvements in postoperative management, total knee arthroplasty has become the treatment of choice for haemophilia patients suffering from end-stage haemophilic knee arthropathy. The success of this surgery is also dependent on close collaborations among the orthopaedic surgeon, the haematologist and the physiotherapist. Although haemophilic patients undergoing this surgery would likely benefit from a targeted rehabilitation programme, its specificities, modalities and limitations have thus far not been extensively studied. Employing the published data of rehabilitation after knee prosthesis in patients with osteoarthritis and haemophilic arthropathy along with clinical experience, the authors present a comprehensive and original review of the role of physiotherapy for patients with haemophilia undergoing knee arthroplasty.

Physiotherapy following elective orthopaedic procedures.

As haemophilic arthropathy and chronic synovitis are still the most important clinical features in people with haemophilia, different kinds of invasive and orthopaedic procedures have become more common during the last decades. The availability of clotting factor has made arthroplasty of one, or even multiple joints possible. This article highlights the role of physiotherapy before and after such procedures. Synovectomies are sometimes advocated in people with haemophilia to stop repetitive cycles of intra-articular bleeds and/or chronic synovitis. The synovectomy itself, however, does not solve the muscle atrophy, loss of range of motion (ROM), instability and poor propriocepsis, often developed during many years. The key is in taking advantage of the subsequent, relatively safe, bleed-free period to address these important issues. Although the preoperative ROM is the most important variable influencing the postoperative ROM after total knee arthroplasty, there are a few key points that should be considered to improve the outcome. Early mobilization, either manual or by means of a continuous passive mobilization machine, can be an optimal solution during the very first postoperative days. Muscle isometric contractions and light open kinetic chain exercises should also be started in order to restore the quadriceps control. Partial weight bearing can be started shortly after, because of quadriceps inhibition and to avoid excessive swelling. The use of continuous clotting factor replacement permits earlier and intensive rehabilitation during the postoperative period. During the rehabilitation of shoulder arthroplasty restoring the function of the rotator cuff is of utmost importance. Often the rotator cuff muscles are inhibited in the presence of pain and loss of ROM. Physiotherapy also assists in improving pain and maintaining ROM and strength. Functional weight-bearing tasks, such as using the upper limbs to sit and stand, are often discouraged during the first 6 weeks postoperatively. This may be influenced by the condition of the joints of the lower limbs. Attention should be given to the total chain of motion, of which the shoulder itself is only a part. We conclude that physiotherapy management is of major importance in any invasive or orthopaedic procedure, regardless of which joints are involved. Both pre- and postoperative physiotherapy, as part of comprehensive care is needed to achieve optimal functional outcome and therefore optimal quality of life for people with haemophilia.