Performance of instrumental activities of daily living in patients with haemophilic arthropathy. A cross-sectional cohort study.

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 (R2 adj = 0.51). Loss of predictive capacity is acceptable with good mean internal (R2 mean = 0.40) and external (R2-r2 = 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.

Occupational therapy in the management of haemophilia patients: A scoping review.

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.

180-degree immersive VR motion visualization in the treatment of haemophilic ankle arthropathy.

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; η2 p = .63), pressure pain threshold of the lateral malleolus (F = 12.34; η2 p = .29) and range of motion (F = 11.7; η2 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.

The relationship between chronic pain and psychosocial aspects in patients with haemophilic arthropathy. A cross-sectional study.

BACKGROUND: Pain is a major characteristic in haemophilic arthropathy. Identifying the psychosocial variables affected by pain can help when addressing these patients. AIM: To assess the relationship between perceived intensity of chronic pain and joint damage, kinesiophobia, catastrophism, anxiety and perceived quality of life in adult patients with haemophilic arthropathy. METHODS: Multicentre cross-sectional descriptive study. Seventy-seven adult patients with haemophilic arthropathy were recruited. The usual and maximum pain intensity (Visual Analog Scale), joint status (Haemophilia Joint Health Score), Kinesiophobia (Tampa Scale of Kinesiophobia), catastrophism (Pain catastrophizing scale), anxiety (State-Trait Anxiety inventory) and perceived quality of life (36-Item Short Form Health Survey) were evaluated. The correlation between usual and maximum pain intensity with quantitative variables was obtained with Spearman`s correlation test. Kruskal-Wallis one-way ANOVA analysed differences in perceived pain according to the severity and type of treatment, and development of inhibitors. RESULTS: The usual intensity of perceived pain correlated positively with catastrophism, kinesiophobia, and state and trait anxiety. The same results were obtained when analysing the maximum perception of pain. We found an inverse correlation between the physical component of perceived quality of life and usual and maximum pain intensity perceived by patients. CONCLUSION: Psychosocial factors affect the painful experience of patients with haemophilic arthropathy. Pain intensity affects the quality of life of these patients.

LongHest project: A prospective, observational study of extended half-life treatment in the musculoskeletal health of patients with severe haemophilia A.

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.

Safety and efficacy of a self-induced myofascial release protocol using a foam roller in patients with haemophilic knee arthropathy.

BACKGROUND: Haemophilic knee arthropathy presents functional and structural alterations and chronic pain. Self-induced myofascial release aims to treat fascial restrictions and improve functionality. AIM: This study investigated the safety and effectiveness of a self-induced myofascial release protocol in patients with haemophilic knee arthropathy. METHODS: Twenty-five patients with bilateral haemophilic knee arthropathy were recruited (n = 50 knees). The patients followed an intervention protocol, with daily exercises for 8 weeks. The dependent variables were: safety of the technique (periodic telephone monitoring), joint state (Haemophilia Joint Health Score), pain intensity (visual analogue scale), pressure pain threshold (pressure dynamometer), range of motion (universal goniometer) and hamstring flexibility (Fingertip-To-Floor test). The resulting values were measured at baseline (T0) and after the intervention (T1). Paired t-test compared the means between the assessments. Effect size was obtained using Cohen's d mean difference formula. The minimum detectable change of each variable was calculated. RESULTS: There were no cases of joint bleeding either during or after the procedure. The results showed improvements after the experimental period in joint state (Mean difference [MD]: 1.38; 95% confidence interval [95%CI]: .94;1.81), pain intensity (MD: 1.19; 95%CI: .70;1.67), pressure pain threshold (MD: -23.25; 95%CI: -26.25;-19.84), flexion (MD: -4.36; 95%CI: -5.70;-3.01), loss of extension (MD: 4.10; 95%CI: 3.01;5.18) and hamstring flexibility (MD: 3.54; 95%CI: 2.61;4.46). CONCLUSIONS: Myofascial self-release using a foam roller is safe in patients with haemophilic knee arthropathy. A myofascial self-release protocol can improve perceived pain, range of motion and knee joint status, as well as hamstring flexibility in patients with haemophilic knee arthropathy.

Effect of a Fascial Therapy Treatment on Quality of Life in Patients With Hemophilic Elbow Arthropathy: A Randomized Controlled Trial.

OBJECTIVE: To evaluate the effect of fascial therapy on the perceived quality of life, pain intensity, and joint health in people with hemophilia. DESIGN: Single-blind randomized controlled trial. SETTING: Hemophilia Patient Associations. PARTICIPANTS: Patients with hemophilia (N=69) were randomly allocated to an experimental group (fascial therapy protocol) or to a control group (no intervention whatsoever). INTERVENTIONS: The fascial therapy intervention lasted 3 consecutive weeks with one 45-minute weekly session. MAIN OUTCOME MEASURES: The variables evaluated were perceived quality of life (Short Form Health Survey-36), pain intensity (visual analog scale) and joint health (Hemophilia Joint Health Score) at baseline and at 3 and 12 weeks. RESULTS: The mean values of the physical (38.64±9.41 and 41.92±12.16) and mental (45.77±6.25 and 55.02±9.73) components of the Short Form Health Survey-36 improved after the intervention in the experimental group, as well as those for pain intensity and joint health (P<.001). There were differences (P<.001) in the intergroup effect in the physical role variables (F=49.22), emotional role (F=229.71), mental component (F=9.86), intensity of pain (F=24.74), and joint health (F=55.31). CONCLUSIONS: A fascial therapy protocol for patients with hemophilic elbow arthropathy can improve their perceived quality of life. Elbow pain intensity improved in patients treated with fascial therapy. This technique can improve elbow joint health.

The effectiveness of manual therapy in addition to passive stretching exercises in the treatment of patients with haemophilic knee arthropathy: A randomized, single-blind clinical trial.

BACKGROUND: Haemophilic arthropathy is characterized by joint restrictions. One of the most affected joints in haemophilia patients is the knee. AIM: This study evaluates the effectiveness of manual therapy and passive muscle stretching exercises for reducing the frequency of hemarthrosis and pain and improving joint health and range of motion in patients with haemophilic knee arthropathy. METHODS: Twenty eight patients with haemophilic knee arthropathy were randomized to an experimental group or to a control group (without intervention). Manual therapy sessions included joint traction and gliding manoeuvers, in addition to passive muscle stretching. The intervention included one 60-minute with two weekly sessions over a 12-week period. We evaluated the frequency of knee hemarthrosis (self-reporting), joint health (Hemophilia Joint Health Score), range of motion (goniometry) and perceived knee pain (visual analogue scale). A baseline evaluation was performed at the end of the intervention and after a 12-week follow-up period. RESULTS: The frequency of hemarthrosis dropped significantly in the experimental group compared to the control group (F = 11.43; P < .001). Compared to the control group, the experimental group had consistently better results in the variables for joint health (F = 13.80; P < .001), range of motion in knee flexion (F = 24.29; P < .001) and loss of extension (F = 8.90; P < .001), and perceived pain (F = 49.73; P < .001). CONCLUSIONS: Manual therapy using joint traction and gliding manoeuvers, in addition to passive muscle stretching, reduces the frequency of hemarthrosis in patients with haemophilia. Manual therapy with passive muscle stretching exercises improves joint health, range of motion and perceived joint pain.

Quality of life and its predictors among adult patients with haemophilic arthropathy. An observational study.

BACKGROUND: Recurrent hemarthrosis that begin in childhood lead to progressive joint deterioration. Patients with haemophilia have chronic pain, functional disability and a reduced perception of health-related quality of life. PURPOSE: To analyse the perceived quality of life of adult patients with haemophilic arthropathy and its relationship with pain, joint condition, kinesiophobia and catastrophism. METHODS: Eighty-three adult patients with haemophilia were included in this multicentre, cross-sectional, descriptive study. Perceived quality of life (36-Item Short Form Health Survey), perceived usual and maximum pain (visual analogue scale), joint condition (Haemophilia Joint Health Score), kinesiophobia (Tampa Scale of Kinesiophobia) and catastrophism (Pain Catastrophizing Scale) were assessed. Sociodemographic, clinical and therapeutic variables and drug consumption for pain control were collected. Descriptive statistics used means and standard deviations. The correlation of quality of life with the dependent variables was calculated with the Pearson correlation test. The differences in quality of life as a function of the binomial variables were calculated with Student's t-test for independent samples. RESULTS: Physical component of quality of life perceived by patients with hemophilia is lower than Spanish population (30.51 VS 48.85). Regarding the mental component, patients with hemophilia showed higher values (56.07 VS 49.97). Catastrophism correlated (p < .05) with all items of quality of life questionnaire. Kinesiophobia correlated (p < .05) with all items of quality of life except to role-emotional (r = -.18; p > .05). Habitual and maximal joint pain correlated with all items except to role-emotional (r = - .19 and r = - .09, respectively) and mental component score (r = - .16 and r = - .07, respectively). Catastrophism and weekly drug intake were inversely correlated with quality of life. Age was positively correlated with perceived quality of life. There were differences in quality of life as a function of the severity of haemophilia and the intake of drugs for pain control. CONCLUSIONS: The perceived quality of life of adult patients with haemophilia is worse than that of the Spanish population. Pain, kinesiophobia, catastrophism, haemophilia severity and the intake of pain-control medication influence the quality of life of these patients.

Evaluating international Haemophilia Joint Health Score (HJHS) results combined with expert opinion: Options for a shorter HJHS.

INTRODUCTION: The Hemophilia Joint Health Score (HJHS) was developed to detect early changes in joint health in children and adolescents with haemophilia. The HJHS is considered by some to be too time consuming for clinical use and this may limit broad adoption. AIM: This study was a first step to develop a shorter and/or more convenient version of the HJHS for the measurement of joint function in children and young adults with haemophilia, by combining real-life data and expert opinion. METHODS: A cross-sectional multicenter secondary analysis on pooled data of published studies using the HJHS (0-124, optimum score 0) in persons with haemophilia A/B aged 4-30 was performed. Least informative items, scoring options and/or joints were identified. An expert group of 19 international multidisciplinary experts evaluated the results and voted on suggestions for adaptations in a structured meeting (consensus set at ≥ 80%). RESULTS: Original data on 499 persons with haemophilia from 7 studies were evaluated. Median age was 15.0 years [range 4.0-29.9], 83.2% had severe haemophilia and 61.5% received prophylaxis. Median (IQR) HJHS total was 6.0 (1.0-17.0). The items 'duration swelling' and 'crepitus' were identified as clinically less informative and appointed as candidates for reduction. CONCLUSION: Analysis of 499 children and young adults with haemophilia showed that the HJHS is able to discriminate between children and adults and different treatment regimens. Reduction of the items 'duration swelling' and 'crepitus' resulted in the HJHSshort , which had the same discriminative ability. Additional steps are needed to achieve a substantially shorter HJHS assessment.

Effect of manual therapy in patients with hemophilia and ankle arthropathy: a randomized clinical trial.

OBJECTIVE: The aim of this study was to evaluate the effects of a manual therapy using fascial therapy on joint bleeding, joint pain and joint function in patients with hemophilic ankle arthropathy. SETTING: Hemophilia patient associations. DESIGN: Randomized, controlled trial, multicenter and intention-to-treat analysis. PARTICIPANTS: A total of 65 patients with hemophilic ankle arthropathy. INTERVENTION: The experimental group (n = 33) received one fascial therapy session per week for three weeks. The control group (n = 32) received no treatment. OUTCOME MEASURE: The primary outcome was frequency of joint bleeding measured using self-reporting. Secondary outcomes were joint pain (under load-bearing and non-load-bearing conditions) measured using the visual analog scale; joint condition was measured using the Hemophilia Joint Health Score. Outcomes were measured at baseline, posttreatment and after five months of follow-up. RESULTS: Improvements in the frequency joint bleeding at T0, T1 and T2 were significantly higher in the experimental group (T0: mean (SD) = 1.56 (1.30); T1: mean (SD) = 0.00 (0.00); T2: mean (SD) = 0.27 (0.57)) compared to the control group (T0: mean (SD) = 1.70 (1.78); T1: mean (SD) = 0.05 (0.21); T2: mean (SD) = 0.58 (0.85)). Mean improvement of joint state after the study period was 1.74 points (±1.66) for patients in the experimental group, while the control group exhibited a joint deterioration with 0.43 points (±0.85). Ankle joint pain under load-bearing and non-load-bearing conditions improved in the experimental group with -1.72 (±1.86) and -0.50 (±1.39) points, respectively. CONCLUSION: The study showed that fascial therapy is favorable for patients with hemophilic ankle arthropathy.

Coping strategies in young and adult haemophilia patients: A tool for the adaptation to the disease.

INTRODUCTION: Chronic diseases, after diagnosis, involve changes that have to favour coping with the new situation. The resources used will help control, manage and adapt to the disease. The psychological aspects may be influencing how the individual faces the situation. AIM: To assess whether perceptions or beliefs and illness behaviour influence the choice of coping strategies for young and adult patients with haemophilia. METHODS: Multicenter cross-sectional descriptive study. We recruited 63 patients with haemophilia A and B, adolescents, young and adults, and both types of treatment. A clinical and sociodemographic data sheet, the Coping Strategies Inventory (CSI), the Illness Perception Questionnaire-revised (IPQ-R) and the Illness Behaviour Questionnaire (IBQ) were used. RESULTS: Patients with haemophilia use appropriate coping strategies, both cognitive and behavioural. Most of them are on-demand treatment, and despite arthropathy, they perceive good control of haemophilia. However, patients in prophylactic treatment are those employed more maladaptive coping strategies, less perception of control and hypochondriacal behaviour to the disease. The age variable may be relevant but we did not find significant differences. CONCLUSIONS: Coping strategies used by patients with haemophilia are adequate. Although it is noted that the perception of the disease, its controllability or not, affects illness behaviour and consequently how coping with haemophilia. These are based on personal characteristics, cognitive and attitudinal dispositions that the individual consciously use to solve or face adverse situations. The analysis of coping styles of patients could be a tool for professionals to manage properly the disease.

Manual therapy in the treatment of patients with hemophilia B and inhibitor.

BACKGROUND: The main clinical manifestations of hemophilia are muscle and joint bleeding. Recurrent bleeding leads to a degenerative process known as hemophilic arthropathy. The development of inhibitors (antibodies against FVIII/FIX concentrates) is the main complication in the treatment of hemophilia. The objective was to assess the safety and efficacy of manual therapy treatment in a patient with hemophilia and inhibitor. CASE PRESENTATION: A 26-year-old patient with hemophilia B and inhibitor received physiotherapy treatment based on manual therapy for 3 months, with a frequency of 2 sessions per week. The joint status was evaluated using the Hemophilia Joint Health Score; pain was assessed with the Visual Analog Scale; and the range of movement was evaluated using a universal goniometer. The patient developed no joint bleeding in the knees or ankles as a result of the physiotherapy treatment. Following treatment, improvements were noted in the range of movement of knees and ankles, the perception of pain in both knees, and ankle functionality. CONCLUSIONS: Until now, manual therapy using joint traction was contraindicated in patients with hemophilia and inhibitor, as it was feared to cause possible joint bleeding. This is the first case study to address the safety and efficacy of manual therapy in a patient with hemophilia and an inhibitor. The results of this study may help to establish which manual therapy treatments are indicated in patients with hemophilic arthropathy and inhibitors. Thus, a physiotherapy program based on manual therapy may be safe in patients with hemophilia and inhibitor and such therapy may improve joint condition, pain, and joint range of motion in patients with hemophilia and inhibitor. Randomized clinical trials are needed to confirm the results of this case study.

Effectiveness of an Educational Physiotherapy and Therapeutic Exercise Program in Adult Patients With Hemophilia: A Randomized Controlled Trial.

OBJECTIVE: To assess the efficacy of an educational physiotherapy home exercise intervention for physical improvement, pain perception, quality of life, and illness behavior in patients with hemophilic arthropathy. DESIGN: Single-blind randomized controlled trial. SETTING: Home. PARTICIPANTS: Patients with hemophilia (N=20) were randomly allocated to an educational intervention group or to a control group. INTERVENTIONS: The educational intervention was performed every 2 weeks over a 15-week period, and home exercises were carried out once a day, 6 days a week, over the same period. MAIN OUTCOME MEASURES: Joint status was evaluated using the Gilbert scale; pain was assessed using the visual analog scale; illness behavior was evaluated using the Illness Behavior Questionnaire; and the perception of the quality of life was evaluated using the A36 Hemophilia-QoL questionnaire. RESULTS: We observed significant differences in the intervention group and the control group for both quality of life and illness behavior. There was no significant improvement in joint status; however, an improvement was noted in terms of perception of pain in the ankle. CONCLUSIONS: A physiotherapy program based on educational sessions and home exercises can improve the perception of pain in patients with hemophilic arthropathy of the ankle. It improves some variables in the perception of the quality of life and illness behavior.

Sporting Activities and Quality of Life in Children With Hemophilia: An Observational Study.

INTRODUCTION: Sports activities are part of multidisciplinary treatments in people with hemophilia. AIM: The objective of this study was to assess the incidence of sports activities in the quality of life as perceived by children with hemophilia. METHODS: A total of 53 children with hemophilia aged 7 to 13 years and 51 children without hemophilia were evaluated. The perception of quality of life, clinical variables, and the frequency of sports activities were registered. The joint condition of patients with hemophilia was measured with the Spanish version of the Haemophilia Joint Health Score. RESULTS: There were no significant differences in the perception of quality of life between children with hemophilia and children without hemophilia. Sports activities in people with hemophilia promoted a greater health satisfaction. CONCLUSIONS: Sports activity in children with hemophilia is associated with an improved quality of life and joint health. It is also associated with improved psychosocial wellness.

Immersive VR movement visualization in patients with hemophilic knee arthropathy: randomized, multicenter, single-blind clinical trial.

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).

Changes in Quality of Life, Adherence, and Kinesiophobia in Patients with Hemophilia Treated with Extended Half-Life Treatment: Final Results of the LongHest Project.

(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.

Immersive visualization of movement in patients with hemophilic ankle arthropathy. Multicenter, single-blind, randomized clinical trial.

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.

Stability, Balance, and Physical Variables in Patients with Bilateral Hemophilic Arthropathy of the Ankle versus Their Healthy Peers: A Case-Control Study.

(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.

Safety and efficacy of myofascial release therapy in the treatment of patients with hemophilic ankle arthropathy. Single-blind randomized clinical trial.

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.