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.

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.

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.