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Rubén Cuesta-Barriuso,1,2 Elena Donoso-Úbeda,3 Javier Meroño-Gallut,4 Roberto Ucero-Lozano,5 Raúl Pérez-Llanes3 1Department of Surgery and Medical-Surgical Specialties, University of Oviedo, Oviedo, Spain; 2Royal Victoria Eugenia Foundation, Madrid, Spain; 3Department of Physiotherapy, Catholic University San Antonio-UCAM, Murcia, Spain; 4Physiotherapy Service, Tu Bienestar 360°, Murcia, Spain; 5Department of Physiotherapy, European University of Madrid, Madrid, SpainCorrespondence: Rubén Cuesta-Barriuso, Department of Surgery and Medical-Surgical Specialties, University of Oviedo, Campus de El Cristo s/n, Faculty of Medicine, Oviedo, 33006, Spain, Tel +34 985 103 386, Email cuestaruben@uniovi.esAbstract: Hemophilia is a congenital coagulopathy characterized by a deficiency of one of the clotting factors. It is characterized by the development of hematomas and hemarthrosis, either spontaneously or after minor trauma. The recurrence of hemarthroses leads to progressive and degenerative joint damage from childhood (hemophilic arthropathy). This arthropathy is characterized by disabling physical effects that limit the functionality and quality of life of these patients. Medical progress achieved over the last decade in the drug treatment of hemophilia has improved the medium and long-term prospects of patients with more effective and long-lasting drugs. The universal use of safer, more effective and prolonged prophylactic treatments may promote the prevention of bleeding, and also therefore, of the development of hemarthrosis and joint damage. A number of imaging instruments have been developed for the assessment of hemarthrosis and hemophilic arthropathy, using ultrasound, magnetic resonance imaging and simple radiology. Different physical examination scores and questionnaires allow the assessment of joint health, self-perceived activity and functionality of patients with hemophilia. The approach to these patients should be interdisciplinary. Assessment of the processes that affect pain in these patients and the development of pain education models should be implemented. Expert advice and information to patients with hemophilia should be based on individual functional prevention diagnoses, advice on available therapies and sports practice, as well as health recommendations.Keywords: hemophilia, joint disease, pain, diagnosis, management

Rubén Cuesta-Barriuso,1,2 Ana Torres-Ortuño,3 Joaquín Nieto-Munuera,3 José Antonio López-Pina4 1Department of Physiotherapy, University of Murcia, Murcia, Spain; 2Royal Victoria Eugenia Foundation, Madrid, Spain; 3Department of Psychiatry and Social Psychology, University of Murcia, Murcia, Spain; 4Department of Basic Psychology and Methodology, University of Murcia, Murcia, SpainCorrespondence: Rubén Cuesta-BarriusoDepartment of Physiotherapy, University of Murcia, Campus Espinardo, Murcia, 30200, SpainTel +34 868 887 286Email ruben.cuestab@gmail.comBackground: Hemophilia is characterized by the development of joint bleeds that cause long-term joint damage (hemophilic arthropathy). Joint damage leads to disability and affects psychosocial aspects in patients with hemophilia.Objective: To compare the clinical situation, perception of disease and quality of life, and coping strategies in adult patients with hemophilia in El Salvador and Spain.Methods: In this comparative clinical study, 43 patients with hemophilia aged between 18 and 50 years old from Spain and El Salvador participated. After obtaining the patients’ consent, they completed the Illness Perception Questionnaire-Revised (IPQ-R), Hemophilia-QoL and Inventory of Coping strategies questionnaires. Joint status was assessed using the Hemophilia Joint Health Score and based on a record of clinical and treatment data.Results: Hemophilia patients from Spain showed an improved perception of quality of life (p < 0.05), although there were only differences in the self-criticism variable (p = 0.04) for coping strategies. Joint damage and age correlated (p < 0.05) negatively with perception of disease, perceived quality of life and coping strategies in both populations. There were differences (p < 0.05) between the two populations based on HIV and HCV coinfections in perception of disease and perceived quality of life.Conclusion: Patients with hemophilia in El Salvador exhibit a poorer perception of disease and quality of life. Despite differences in access to treatment from one country to the other, there is no difference in coping with the disease. Older patients are better able to adapt to the disease.Keywords: hemophilia, joint disease, quality of life, illness perception, coping strategies

Ana Torres-Ortuño,1 Rubén Cuesta-Barriuso,2,3 Joaquín Nieto-Munuera,1 Álvaro Castiello-Munuera,1 José Antonio López-Pina4 1Department of Psychiatry and Social Psychology, University of Murcia, Murcia, Spain; 2Department of Physiotherapy, University of Murcia, Murcia, Spain; 3Royal Foundation Victoria Eugenia, Madrid, Spain; 4Department of Basic Psychology and Methodology, University of Murcia, Murcia, SpainCorrespondence: Rubén Cuesta-BarriusoDepartment of Physiotherapy, University of Murcia, Murcia, SpainTel +34 868887286Email ruben.cuestab@gmail.comIntroduction: The episodic or on-demand administration of clotting factor concentrates in hemophilia patients in the event of hemorrhage is employed to restore hemostasis. Adherence to on-demand treatments needs to be assessed in order to improve patient management, avoiding adverse effects and serious clinical complications.Aim: To validate the Spanish version of the treatment adherence scale in patients with hemophilia, namely, Validated Hemophilia Regimen Treatment Adherence Scale-PRN (VERITAS-PRN).Methods: Eighty-five patients were recruited in three hemophilia patient associations in Spain. The VERITAS-PRN scale was adapted through a back-translation process from English to Spanish. A native Spanish bilingual translator translated the scale from English to Spanish, and subsequently another native English bilingual translator translated the scale from Spanish to English. The scale was applied twice (two months apart) to assess test–retest reliability.Results: Internal consistency reliability was slightly lower in the VERITAS-PRN in Spanish (0.80) versus the English version (0.85). There were no differences (p > 0.05) between the means of the dimensions or in the total scores between the sample of patients in Spain and the USA. The test–retest reliability coefficient of the scores on the total scale was 0.80 [CI, 0.74– 0.86]. The test–retest reliability coefficient was greater than 0.90 in all subscales.Conclusion: The Spanish version of VERITAS-PRN has high consistency and empirical validity. This scale is useful for assessing the degree of adherence to treatment in adult patients with hemophilia following episodic infusion treatment.Keywords: hemophilia, adherence, validity, reliability, on-demand treatment, VERITAS-PRN

Ana Torres-Ortuño,1,2 Rubén Cuesta-Barriuso,3,4 Joaquín Nieto-Munuera,1 Álvaro Castiello-Munuera,1 Manuel Moreno-Moreno,2,4,5 José Antonio López-Pina6 1Department of Psychiatry and Social Psychology, Faculty of Medicine, University of Murcia, Murcia, Spain; 2Association of Hemophilia, Murcia, Spain; 3Department of Physiotherapy, School of Biomedical and Health Sciences, European University of Madrid, Madrid, Spain; 4Royal Foundation Victoria Eugenia, Madrid, Spain; 5Hematology and Hemotherapy Service, University Clinical Hospital Virgen de la Arrixaca, Murcia, Spain; 6Department of Basic Psychology and Methodology, Faculty of Psychology, University of Murcia, Murcia, SpainCorrespondence: Ana Torres-OrtuñoDepartment of Psychiatry and Social Psychology, Faculty of Medicine, University of Murcia, Campus de Espinardo s/n, 30100, SpainTel +34 868887170Fax +34 913508Email atorres.ortuno@gmail.comPurpose: To build a multidimensional questionnaire of adherence for a patient with hemophilia that includes not only clinical but also psychosocial aspects.Patients and Methods: One hundred and forty-six patients with haemophilia (A and B) were recruited from several Hemophilia Treatment Centers for this study. The recruitment was performed through a personal interview (20– 25 mins for each patient). A literature review was carried out (different databases). In the first version (136 items), all items were subjected to an external judgment (experts in hemophilia) and also presented and discussed with a group of patients. It was made a study of content validity and homogeneity index and reliability coefficients of score were calculated with an alpha coefficient. Empirical validation was made with Pearson correlation.Results: The Haemo-Adhaesione scale was composed of 5 dimensions and 10 questions about basic haemophilia concepts. It was shown to be valid and reliable to assess adherence in the Spanish population. Its internal consistency was good in all dimensions. The Rasch model was used to confirm the multidimensional structure of the scale. Significant and negative correlations were observed with the VERITAS-Pro and VERITAS-PRN scale in all dimensions, and in the total score.Conclusions: The Haemo-Adhaesione scale is a good measure of adherence for PWH. Its multidimensional structure favors the inclusion of the objective and subjective aspects implicit in its definition, as well as its dynamic nature. Patients with greater adherence are those who have more awareness of their disease, and as a result, they are more compliant with prescription and their self-care. It also discriminates between patients who follow one treatment or another.Keywords: hemophilia, adherence, young, adults, scale

Rubén Cuesta-Barriuso,1–3 Ana Torres-Ortuño,4 Pilar Galindo-Piñana,4 Joaquín Nieto-Munuera,4 Natalie Duncan,5 José Antonio López-Pina6 1Department of Physiotherapy, School of Biomedical and Health Sciences, European University of Madrid, 2Fishemo, Centro Especial de Empleo, Spanish Federation of Hemophilia, 3Royal Foundation Victoria Eugenia, Madrid, 4Department of Psychiatry and Social Psychology, Faculty of Medicine, University of Murcia, Murcia, Spain; 5Indiana Hemophilia & Thrombosis Center, Indianapolis, IN, USA; 6Department of Basic Psychology and Methodology, Faculty of Psychology, University of Murcia, Murcia, Spain Purpose: We aimed to conduct a validation in Spanish of the Validated Hemophilia Regimen Treatment Adherence Scale – Prophylaxis (VERITAS-Pro) questionnaire for use in patients with hemophilia under prophylactic treatment.Patients and methods: The VERITAS-Pro scale was adapted through a process of back translation from English to Spanish. A bilingual native Spanish translator translated the scale from English to Spanish. Subsequently, a bilingual native English translator translated the scale from Spanish to English. The disagreements were resolved by agreement between the research team and translators. Seventy-three patients with hemophilia, aged 13–62 years, were enrolled in the study. The scale was applied twice (2 months apart) to evaluate the test–retest reliability.Results: Internal consistency reliability was lower on the Spanish VERITAS-Pro than on the English version. Test–retest reliability was high, ranging from 0.83 to 0.92. No significant differences (P>0.05) were found between test and retest scores in subscales of VERITAS-Pro. In general, Spanish patients showed higher rates of nonadherence than American patients in all subscales.Conclusion: The Spanish version of the VERITAS-Pro has high levels of consistency and empirical validity. This scale can be administered to assess the degree of adherence of prophylactic treatment in patients with hemophilia. Keywords: hemophilia, adherence treatment, validity, reliability

Introduction Alterations in the musculoskeletal system, especially in the lower limbs, limit physical activity and affect balance and walking. Postural impairments in haemophilic preteens could increase the risk of bleeding events and deteriorate the physical condition, promoting the progression of haemophilic arthropathy. Aim This study aims to evaluate static postural balance in haemophilic children, assessed by means of the Wii Balance Board® ( WBB). Methods Nineteen children with haemophilia and 19 without haemophilia aged 9-10 years, have participated in this study. Postural balance was assessed by performing four tests, each one lasting 15 s: bipodal eyes open ( BEO), bipodal eyes closed ( BEC), monopodal dominant leg ( MD) and monopodal non-dominant leg ( MND). Two balance indices, standard deviation of amplitude ( SDA) and standard deviation of velocity ( SDV) were calculated in the anterior-posterior ( AP) and medial-lateral ( ML) directions. Results Index values were higher in haemophilic group and the differences were statistically significant ( P < 0.05) in only six ( SDAAP in BEO, BEC and MD conditions, SDAML in BEO, SDVAP in BEO and SDVML in MND condition) of 16 parameters analysed. Conclusion Tests performed indicate a poorer static postural balance in the haemophilic cohort compared to the control group. Accordingly, physiotherapy programmes, physical activity and sports should be designed to improve the postural balance with the aim of preventing joint deterioration and improving quality of life. [ABSTRACT FROM AUTHOR]

Introduction Alterations in the musculoskeletal system, especially in the lower limbs, limit physical activity and affect balance and walking. Postural impairments in haemophilic preteens could increase the risk of bleeding events and deteriorate the physical condition, promoting the progression of haemophilic arthropathy. Aim This study aims to evaluate static postural balance in haemophilic children, assessed by means of the Wii Balance Board® ( WBB). Methods Nineteen children with haemophilia and 19 without haemophilia aged 9-10 years, have participated in this study. Postural balance was assessed by performing four tests, each one lasting 15 s: bipodal eyes open ( BEO), bipodal eyes closed ( BEC), monopodal dominant leg ( MD) and monopodal non-dominant leg ( MND). Two balance indices, standard deviation of amplitude ( SDA) and standard deviation of velocity ( SDV) were calculated in the anterior-posterior ( AP) and medial-lateral ( ML) directions. Results Index values were higher in haemophilic group and the differences were statistically significant ( P < 0.05) in only six ( SDAAP in BEO, BEC and MD conditions, SDAML in BEO, SDVAP in BEO and SDVML in MND condition) of 16 parameters analysed. Conclusion Tests performed indicate a poorer static postural balance in the haemophilic cohort compared to the control group. Accordingly, physiotherapy programmes, physical activity and sports should be designed to improve the postural balance with the aim of preventing joint deterioration and improving quality of life. [ABSTRACT FROM AUTHOR]

(1) Background: Soccer accounts for 30% of all sports injuries. Muscle injuries in soccer, not caused by trauma or contact, are the most common. The objective was to assess the risk of injury based on the footwear used by soccer players and the playing field and to identify the best predictive model of muscle injuries; (2) Methods: An ambispective cohort study. The primary variable was the number of muscle injuries in the last three seasons. The secondary variables were age, body mass index, type of soccer shoes and turf, training load and position on the field. The possible confounding variables were motivation for the choice of footwear, date of injuries, time playing and regular first-team player status; (3) Results: 156 players were recruited. The risk of injury is 1.03 (95% CI: 0.83; 1.27) times more frequent in players competing on artificial turf. The risk of injury is slightly higher in first-team players than in substitutes (RR = 1.02; 95% CI: 0.79; 1.32). There was no statistically significant relationship between the position on the field ( p = 0.91), the type of shoe ( p = 0.69) and the motivation to buy the shoes ( p = 0.82), regarding the risk of muscle injury in these athletes. The best model to estimate risk of injury includes age, training load and soccer shoe type as confounding variables (AIC = 190.5; p = 0.10; χ 2 (3) = 10.14; p = 0.02); (4) Conclusions: The risk of muscle injury is higher in non-professional soccer players competing on artificial pitches and in those who are regular starters on their teams. Field position, soccer shoe and motivation to purchase the soccer shoe are variables that do not increase the risk of muscle injury in these athletes. The best predictive model of injury includes age, training load and shoe type.

Background: The development of haemophilic arthropathy causes joint damage that leads to functional impairment that limits the performance of activities in patients with haemophilia. The aim was to identify the best predictive model for performing instrumental activities of daily living in adult patients with haemophilia arthropathy., Methods: Cross-sectional cohort study. 102 patients were recruited. The dependent variable was the performance of instrumental activities of daily living (Lawton and Brody scale). The dependence on the performance of activities of daily living was the dependent endpoint (Barthel scale). The secondary variables were joint damage (Hemophilia Joint Health Score), pain intensity, and clinical, anthropometric, and sociodemographic variables., Results: The degree of dependence, joint damage, pain intensity, and marital status (Cp = 5.60) were the variables that best explain the variability in the performance of instrumental activities of daily living (R 2 = 0.51). Loss of predictive capacity is acceptable with good mean internal (R adj  mean = 0.40) and external (R 2 -r 2 -r 2  = 0.09) validation. According to the predictive pattern obtained, patients with haemophilia, who were married, without joint pain or damage, and independent in their day-to-day lives, had a score of 7.91 points (95% CI: 7.42; 8.39) in the performance of instrumental activities of daily living., Conclusions: The predictive model for the functional capacity of instrumental activities of daily living in haemophilia patients encompasses factors such as level of autonomy, joint impairment, pain severity, and marital status. Notably, despite the presence of joint damage, individuals with haemophilia exhibit a significant level of independence in carrying out both basic daily tasks and instrumental activities of daily living., International Registration Number: Id NCT04715100., (© 2024 The Author(s). Haemophilia published by John Wiley & Sons Ltd.)

Objective: To evaluate the efficacy of immersive movement observation in adult patients with haemophilic ankle arthropathy., Design: Multicentre, single-blind, randomized clinical trial., Subjects: 48 patients with haemophilia., Methods: Patients were randomly allocated to 2 groups (180º immersive video-based visualization of movement and a control group with no intervention). Twenty-eight consecutive 15-min home sessions, 1 per day, of immersive visualization of ankle flexion-extension movement were carried out. Three evaluations were performed: pretreatment (T0), post-intervention (T1), and at 16 weeks' follow-up (T2). The primary variable was joint-pain intensity (visual analogue scale). The secondary variables were conditioned pain modulation (Conditioned Pain Modulation Index), pressure pain threshold (pressure algometer), range of motion (goniometry) and kinesiophobia (Tampa Scale of Kinesiophophia)., Results: There were intergroup differences in pain intensity (F = 37.14; p < 0.001), conditioned pain modulation (F = 5.40; p = 0.006), and dorsal (F = 19.17; p < 0.001) and plantar (F = 9.27; p<0.001) ankle flexion. More than 50% of experimental group patients exhibited changes exceeding the minimum detectable change in pain intensity (MDC = 0.43), and the pressure pain threshold in the extensor carpi radialis longus muscle (MDC = 1.34) and malleolus (MDC = 4.93)., Conclusions: 180º immersive video-based visualization of movement can improve the intensity of pain, conditioned pain modulation, and ankle range of motion in patients with haemophilic ankle arthropathy.

Purpose: To evaluate the efficacy of an immersive movement visualization intervention in patients with hemophilia and hemophilic knee arthropathy., Materials and Methods: Randomized, single-blind clinical study. Twenty-eight patients with hemophilia were recruited. Patients were randomized to an experimental group (four weeks of immersive movement visualization) and a control group (no intervention). The intensity of pain, pressure pain threshold in the knee, tibialis anterior muscle, lower back level, conditioned pain modulation, range of knee motion, and lower limb functionality were evaluated., Results: There were statistically significant differences in the intergroup effect on knee pain intensity ( F  = 23.71; p  < 0.001) and lower limb functionality ( F  = 7.11; p  = 0.003). 42.86% of the patients in the experimental group exhibited changes greater than the minimum detectable change (MDC) in functionality. 39.29% of the patients subject to the intervention experienced changes greater than the MDC in the knee pressure pain threshold., Conclusions: Immersive motion visualization can improve the intensity of joint pain and functionality in patients with hemophilic knee arthropathy. Functionality, pressure pain threshold, and pain intensity improved in those patients who conducted immersive movement visualization.Implications for rehabilitationImmersive visualization of movement significantly improves intensity of joint pain, functionality, pressure pain threshold, joint health, and conditioned pain modulation in patients with hemophilic knee arthropathy.The fact that it is a therapy without potential aversive stimuli makes it a possible access pathway for patients with high levels of kinesiophobia and/or catastrophism.This low-cost, home-based technology allows its use in patients far from hemophilia reference centers or with difficult access to physiotherapy treatments.The immersive visualization of movement influences the democratization of treatment, in accordance with the WHO's Sustainable Development Goal 3 (health and well-being for all).

(1) Background: The recurrence of hemarthrosis in patients with hemophilia triggers a pathophysiological process of degenerative, progressive, and irreversible joint destruction. This hemophilic arthropathy is characterized by chronic pain, muscle atrophy, loss of mobility, and proprioceptive alterations. As the same joint undergoes repeated hemarthrosis, the function of the mechanical receptors deteriorates, causing a pathophysiological modulation and deterioration of the musculoskeletal system. The objective was to analyze the differences in stability and balance, as well as in ankle dorsal flexion, functionality, and muscle strength, between patients with bilateral hemophilic arthropathy and their healthy peers. (2) Methods: A cross-sectional descriptive case-control study was performed. Twenty-two participants were recruited: 10 adult patients with bilateral hemophilic arthropathy of the knee and ankle and 12 healthy subjects. The variables were balance (Rs Scan pressure platform), ankle dorsiflexion range of motion (Leg Motion), functionality (2-Minute Walk Test), and ankle dorsal strength (dynamometry). (3) Results: Statistically significant differences ( p < 0.05) were found in the balance without visual support in the Max-Y variable (MD = 2.83; CI95%: 0.33;5.33; Effect size (d) = 0.67), ankle dorsiflexion (MD = 16.00; CI95%: 14.30; 20.0; d = 7.46), and strength of the ankle flexor muscles (MD = 128.50; CI95%: 92.50; 153.60; d = 2.76). (4) Conclusions: Ankle range of motion in dorsal flexion, functionality, and muscle strength in dorsal flexion is poorer in patients with bilateral lower limb hemophilic arthropathy than in their healthy peers. Patients with bilateral hemophilic ankle arthropathy have statistically poorer stability and balance without visual support than their healthy peers.

(1) Background: Hemophilia is a bleeding disorder characterized by hemarthrosis. Prophylaxis is the gold standard for bleeding prevention. Extended half-life (EHL) recombinant FVIII replacement products have shown to be associated with low bleeding rates. The aim was to evaluate the efficacy of EHL prophylaxis in improving perceived quality of life, adherence to treatment, and kinesiophobia in patients with hemophilia. (2) Methods: This was a prospective cohort study. Forty-six patients from different regions, who had started EHL FVIII concentrate prophylactic treatment, were evaluated at baseline and at 12-month follow-up. The study variables were as follows: perceived quality of life (36-Item Short Form Health Survey), adherence to treatment (Validated Hemophilia Regimen Treatment Adherence Scale-Prophylaxis), and kinesiophobia (Tampa Scale of Kinesiophobia). (3) Results: There were statistically significant differences in the domains Role-Physical ( p < 0.001), Bodily Pain ( p < 0.001), Role-Emotional ( p < 0.001), Vitality ( p = 0.04), and Social Functioning ( p = 0.01) and the total scores, Physical Health ( p < 0.001) and Mental Health ( p < 0.001) on perceived quality of life. There were significant differences in the domains Skipping ( p < 0.01), Communicating ( p < 0.001), and the total score ( p = 0.01) in terms of adherence. There were also significant differences in kinesiophobia ( p = 0.02) after the study period. (4) Conclusions: EHL prophylaxis can improve the perceived quality of life of people with hemophilia. This prophylactic regimen, which requires fewer infusions, may improve adherence to treatment in adult patients with hemophilia over a 12-month period. The administration of extended half-life factor VIII concentrates can reduce kinesiophobia in adult patients with hemophilic arthropathy.

Background: Hemophilia is characterized by degenerative joint damage. Patients with hemophilic arthropathy present joint damage, reduced range of motion, and decreased strength and functional capacity. Myofascial release therapy aims to decrease pain and improve tissue mobility and functionality., Objectives: To evaluate the safety and efficacy of myofascial release therapy in patients with hemophilic ankle arthropathy., Method: Single-blind randomized controlled trial. Fifty-eight adult patients with hemophilia were randomly allocated to the experimental group (myofascial release therapy with foam roller) or the control group (no intervention whatsoever). The daily home protocol of myofascial release therapy for the lower limbs using a foam roller lasted eight consecutive weeks. The primary variable was the safety of myofascial release therapy (weekly telephone follow-up). The secondary variables were pain intensity (visual analog scale), range of motion (goniometer), functional capacity ( 2-Minute Walk Test ) and muscle strength (dynamometer), at baseline and at 8 and 10 weeks., Results: During the experimental phase, none of the patients in the experimental group developed ankle hemarthrosis. There were statistically significant changes in time*group interaction in ankle dorsal flexion (F [1.75]  = 10.72; p  < .001), functional capacity (F [1.16]  = 5.24; p  = .009) and gastrocnemius strength (F [2]  = 26.01; p  < .001). The effect size of the changes after the intervention was medium-large in pain intensity (d = -1.77), functional capacity (d = 1.34) and gastrocnemius strength (d = 0.76)., Conclusion: Myofascial release therapy is a safe form of physical therapy for patients with hemophilia. Myofascial release therapy can effectively complement prophylactic pharmacological treatment in patients with hemophilic arthropathy, improving range of motion in dorsal flexion, functional capacity and gastrocnemius strength.

Background: Haemophilia is a haematological disease, although most haemorrhages occur in the locomotor system. Patients are physically disabled from an early age and have a poorer perception of quality of life. In the day-to-day lives of patients and their families, psychosocial well-being, the disease's physical, personal, and social impact, as well as work-related problems are the most complicated aspects of the disease that need to be addressed., Objective: To identify the role of occupational therapy in managing patients with haemophilia and to analyse the therapeutic potential of occupational therapy in treating these patients., Methods: A scoping review was conducted to identify the role of occupational therapy in managing patients with haemophilia and to analyse the therapeutic potential of occupational therapy in treating these patients. The review was registered in the international registry PROSPERO (Id: CRD42022319637). The databases consulted were SCOPUS, PubMed, PsycINFO, Web of Science and Science Direct, including all studies published until 14 August 2023., Results: No single study was found that specifically developed an occupational therapy intervention for patients with haemophilia. Measurement instruments have been identified, specific for patients with haemophilia and generic, that can be useful for the functional evaluation of these patients in the occupational therapy approach. Different studies showed the importance of multidisciplinary treatment, including occupational therapy., Conclusions: The use of occupational therapy could be effective in improving autonomy and quality of life in haemophilia patients. Therefore, it is of paramount importance to conduct research studies within the field of occupational therapy., (© 2023 The Authors. Haemophilia published by John Wiley & Sons Ltd.)

Background: Hemophilia is a congenital coagulopathy characterized by degenerative joint damage. Self-induced myofascial therapy aims to decrease pain and improve tissue mobility, functionality and proprioception., Aim: The aim of this study was to evaluate the safety and efficacy of self-induced myofascial release in patients with hemophilic knee arthropathy., Design: This is a randomized clinical study., Setting: This study was carried out in different patient associations., Population: Fifty-two patients with hemophilia were included in the study., Methods: Patients were randomized to the experimental group (daily home protocol of foam roller-based self-induced myofascial therapy for 8 weeks) or the control group (no intervention). The variables were the frequency of hemarthrosis (self-reporting), pain intensity (visual analog scale), range of motion (goniometry) and muscle strength (dynamometry). All variables were evaluated at baseline, post-treatment and after a 10-week follow-up., Results: The patients included in the experimental group showed significant improvements in terms of a decrease in frequency of hemarthrosis (mean difference [MD]=-0.61; 95% confidence interval [CI]: -0.81; -0.41) and pain intensity (MD=-0.33; 95% CI: -0.48, -0.18), increased range of motion (MD=0.88; 95% CI: 0.39; 1.37), strength in quadriceps (MD=0.88; 95% CI: 0.39; 1.37). (MD=12.39; 95% CI: 3.44; 21.34) and hamstrings (MD=7.85; 95% CI: 0.60; 15.11). There were intergroup differences in the frequency of hemarthrosis (F=14.51; P<0.001), pain intensity (F=9.14; P<0.001) and range of motion (F=13.58; P<0.001)., Conclusions: Self-induced myofascial therapy can be an effective complementary technique in the treatment of patients with hemophilic arthropathy. Self-induced myofascial therapy can reduce the frequency of knee hemarthrosis in patients with hemophilia. This technique can improve pain intensity and range of motion in patients with hemophilic knee arthropathy., Clinical Rehabilitation Impact: Hemophilic knee arthropathy is characterized by chronic pain, decreased range of motion, and periarticular muscle atrophy. Foam roller-based self-induced myofascial therapy can reduce the frequency of hemarthrosis and pain intensity and improve range of motion in patients with hemophilic arthropathy. Foam roller-based self-induced myofascial therapy is safe and effective in the treatment of patients with hemophilia. The inclusion of self-induced myofascial therapy exercises in the approach to degenerative joint pathologies may be an effective and safe treatment option.

(1) Background: Hemophilia is characterized by recurrent hemarthrosis leading to degenerative arthropathy. The aim was to evaluate the differences in muscle strength and activity and the pressure pain threshold between patients with knee arthropathy and their healthy peers; (2) Methods: A case-control study in which 23 adult patients with knee arthropathy and 24 healthy peers matched in terms of characteristics were recruited. The study variables were quadriceps muscle strength, muscle activation and the pressure pain threshold; (3) Results: There were significant differences between the two groups in quadriceps strength on the dominant (CI95%: 64.69, 129.2) and non-dominant (CI95%: 29.95, 93.55) sides and in the pressure pain threshold on the dominant (CI95%: 3.30, 43.54) and non-dominant (CI95%: 3.09, 45.25) sides. There were differences in neuromuscular fatigue on the non-dominant side in the vastus medialis (CI95%: 8.72, 21.51), vastus lateralis (CI95%: 4.84, 21.66) and rectus femoris (CI95%: 6.48, 24.95) muscles; (4) Conclusions: Muscle strength and the pressure pain threshold are lower in patients with hemophilia. Quadriceps muscle activation in patients with hemophilic knee arthropathy does not in any way differ from activation in healthy subjects. However, muscle fatigue is greater in patients with knee arthropathy. Strength training in patients with hemophilia should focus on the activation of the vastus medialis and lateralis muscles.

Background: Patients with haemophilic arthropathy suffer chronic pain that affects and restricts their quality of life. Visualization of movement through immersive virtual reality is used for pain management., Aim: To evaluate the efficacy of 180-degree immersive VR motion visualization therapy in patients with haemophilic ankle arthropathy., Methods: Prospective, multicentre pilot study. Fifteen adult patients with bilateral haemophilic ankle arthropathy were recruited (mean age: 42.73 ± 12.36 years). The intervention lasted 4 weeks, with daily home sessions of 180-degree immersive motion visualization. The patients were given virtual reality glasses to use with their smartphones. From the YouTube mobile app® they accessed the recorded video with access from the He-Mirror App®. The study variables were joint state (Haemophilia Joint Health Score), pressure pain threshold (pressure algometer), muscle strength (dynamometry) and range of motion (goniometry). Three evaluations were performed: at baseline (T0), after the intervention (T1) and at the end of a 16-week follow-up period (T2)., Results: No patient developed ankle hemarthrosis during the experimental phase. In the repeated measures analysis we found statistically significant differences in joint state (F = 51.38; η 2 p = .63), pressure pain threshold of the lateral malleolus (F = 12.34; η 2 p = .29) and range of motion (F = 11.7; η 2 p = .28)., Conclusions: Therapy using immersive motion visualization does not cause hemarthrosis. This intervention can improve joint condition, pressure pain threshold and range of motion in patients with ankle arthropathy. Changes greater than the MDC were reported in more than 40% of patients for the variables pressure pain threshold, anterior tibialis strength and range of motion, which were considered clinically relevant., (© 2022 The Authors. Haemophilia published by John Wiley & Sons Ltd.)

Background: Hemophilic ankle arthropathy is manifested by degenerative functional alterations (reduced muscle strength, mobility, and proprioception) and chronic pain. Myofascial release techniques are used to treat soft tissue adhesions, relieve pain, and reduce tissue sensitivity. The aim was to evaluate the safety of self-induced myofascial release in patients with hemophilic ankle arthropathy and to assess possible changes in musculoskeletal variables. Methods: We recruited 20 patients with ankle hemophilic arthropathy. Patients carried out a daily self-induced myofascial release exercise program using a foam roller over a period of 8 weeks. The primary variable was the frequency of hemarthrosis (regular telephone follow-up). Secondary variables were pain intensity (visual analog scale), range of motion (goniometry), and functional capacity of the lower limbs (six-minute walk test). Three evaluations were performed: pre-treatment (T0), post-treatment (T1), and at 8 weeks follow-up (T2). Results: There was a lower, non-significant, association in the frequency of hemarthrosis between the experimental and follow-up periods, compared to the pre-study period (SE = 0.50; 95%CI: −1.67; 0.28). There were significant within-subject changes in intensity of pain (T0: 4.91; T1: 2.79; T2: 2.46; p < 0.001), plantar flexion (T0: 125.55; T1: 131.5; T2: 130.30; p = 0.01), and functionality of the lower limbs (T0: 173.06; T1: 184.85; T2: 178.39; p = 0.009). Conclusions: Self-induced myofascial release is safe in patients with hemophilic ankle arthropathy. A protocol based on self-induced myofascial release can lead to changes in pain intensity, range of ankle motion in plantar flexion, and functionality in hemophilic patients.

(1) Background: Hemarthrosis is a typical clinical manifestation in patients with hemophilia. Its recurrence causes hemophilic arthropathy, characterized by chronic joint pain. Watching movement recorded from a first-person perspective and immersively can be effective in the management of chronic pain. The objective of this study was to evaluate the effectiveness of an immersive virtual reality intervention in improving the pain intensity, joint condition, muscle strength and range of motion in patients with hemophilic knee arthropathy. (2) Methods: Thirteen patients with hemophilic knee arthropathy were recruited. The patients wore virtual reality glasses and watched a flexion-extension movement of the knee on an immersive 180° video, recorded from a first-person perspective over a 28-day period. The primary variable was the pain intensity (visual analog scale). The secondary variables were the joint status (Hemophilia Joint Health Score), quadriceps and hamstring strength (dynamometry), and range of motion (goniometry). (3) Results: After the intervention period, statistically significant differences were observed in the intensity of the joint pain (Standard error [SE] = 19.31; 95% interval confidence [95%CI] = -1.05; -0.26), joint condition (SE = 18.68; 95%CI = -1.16; -0.52) and quadriceps strength (SE = 35.00; 95%CI = 2.53; 17.47). We found that 38.46% and 23.07% of the patients exhibited an improvement in their quadriceps muscle strength and joint condition above the minimum detectable change for both variables (8.21% and 1.79%, respectively). (4) Conclusions: One hundred and eighty degree immersive VR motion visualization can improve the intensity of joint pain in patients with hemophilic knee arthropathy. An intervention using immersive virtual reality can be an effective complementary approach to improve the joint condition and quadriceps strength in these patients.

Background: Prophylactic treatment is the gold standard in the treatment of patients with haemophilia. Prophylaxis with extended half-life (EHL) treatment has shown long-term safety and efficacy in patients with haemophilia., Aim: To evaluate the efficacy of prophylaxis with EHL treatment in the frequency of haemarthrosis and musculoskeletal health in adult patients with severe haemophilia A., Methods: Prospective cohort study. Forty-six patients with severe haemophilia A were recruited. The frequency of haemarthrosis (self-reports), joint condition (Haemophilia Joint Health Score), pain intensity (visual analogue scale), range of motion (goniometry), and strength (dynamometry) and muscle activation (surface electromyography) were evaluated. Three assessments were carried out: at baseline (T0), at 6 months (T1) and at 12 months following treatment (T2)., Results: There were significant changes in the within-subject effect in the frequency of haemarthrosis in elbow (F(1.05;96.20) = 3.95; P < .001) and knee (F(1.73;157.99) = 9.96; P < .001). Significant within-subject effect in elbow pain intensity (F(2;182) = 63.51; P < .001) was found. The mean values of the frequency haemarthrosis in elbow (from .66±1.01 to .04±.20) and knees (from .55±.68 to .33±.53) decrease after the period study. The intensity of elbow pain and (from 3.08±1.69 to 2.67±1.73), decrease after the 12-month follow-up period., Conclusions: Prophylaxis with extended half-life treatment reduces the frequency of haemarthrosis in elbow and knee in adult patients with haemophilia. EHL treatment reduces the intensity of elbow pain in patients with haemophilic arthropathy., (© 2022 The Authors. Haemophilia published by John Wiley & Sons Ltd.)