PB1150 - Improvement in the Musculoskeletal Health and Quality of Life-Outcomes among Patients with Severe Hemophilia A with Inhibitor after 2 years of Personalised Physiotherapy

Background: In people with hemophilia with inhibitors (PWHI) it is not easy to stop some bleeding episodes, therefore arthropathy occurs more often. Till now there are no recommendations for safe and effective physiotherapy in PWHI.

Aims: The aim of the study was to improve the musculoskeletal health and quality of life in PWHI due to the personalised rehabilitation.

Methods: Joint status was assessed using the Hemophilia Joint Health Score (HJHS) and Health-Related Quality of Life (HR-QoL) was assessed using the EQ-5D scale (EuroQoL). PWHI participated weekly in 60-90-minute rehabilitation sessions at home, and after 6, 12, 18 and 24 months of the programme they attended four 5-day rehabilitation camps of 5 hours per day held in Poznań. Rehabilitation was conducted only in patients taking regular aPCC prophylaxis, provided that the aPCC dose was always administered 1 hour before the start of the session.

Results: Twenty-four subjects (mean age 42.1 years, median 42 years) receiving a prophylactic regimen 3 times a week with aPCC 85 ± 15 IU/kg were included. 14 subjects took part in the entire project, as 10 had since switched to emicizumab prophylaxis. These were aged between 28 and 69 years (mean age 47.36 years, median 45.5 years). Thus, the mean age of PWHI increased by 5.26 years and the median by 3.5 years, as mainly younger people withdrew from the project. Figure 1 shows joints mobility improvement that was confirmed in the HJHS scores. Figure 2 presents the clear positive impact of the physiotherapy programme on HRQoL.

Conclusion(s): In adult patients with severe haemophilia A with an inhibitor, regular aPCC prophylaxis and an individualised, once-weekly rehabilitation programme were safe and led to significant improvements in both physical condition and health-related quality of life.