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    Poster Networking

    Hemophilia and Rare Bleeding Disorders

    Poster Networking Session

    PB0670 - Nationwide survey on the care reality of people with mild hemophilia A and B in Germany - The Burden of mild Hemophilia

    Monday, June 26, 2023
    18:30 – 19:30 ET
    Room: Exhibition

      Presenting Author(s)

    • Sonja Rosa Alesci, MD, PhD (she/her/hers)

      Head of
      IMD blood coagulation Hochtaunus
      Bad Homburg, Hessen, Germany

      • Co-Author(s)

    • Susan Halimeh

      Direktor
      Gerinnungszentrum Rhein-Ruhr
      Duisburg, Nordrhein-Westfalen, Germany

    • Martin Olivieri (he/him/his)

      Head of the Paediatric Thrombosis and Haemostasis Unit
      Paediatric Thrombosis and Haemostasis Unit, Paediatric Haemophilia Center, Dr. von Hauner Children's Hospital, LMU Clinic, Munich, Germany
      Munich, Bayern, Germany

    • Wolfgang Miesbach, MD

      Professor and Head of Coagulation Disorders and Comprehensive Care Centre
      University Hospital Frankfurt, Frankfurt, Germany
      Frankfurt, Hessen, Germany

    • Katharina Holstein, MD (she/her/hers)

      physician
      University Hospital Hamburg Eppendorf
      Hamburg, Hamburg, Germany

    • Christoph Koenigs, Dr. Dr.

      Professor
      University Hospital Frankfurt, Department of Paediatrics and Adolescent Medicine, Frankfurt, Germany
      Frankfurt, Hessen, Germany

    Background: Patients with mild hemophilia (residual factor activity > 5 to < 40% represent a very heterogeneous group. Therapeutic approaches are as individual as the bleeding tendency. It is likely that the extent of disease and bleeding risk of patients with mild hemophilia are underestimated, resulting in an undersupply of factor concentrate, e. g. during surgical procedures or trauma. So far, not much data exists on both joint problems of patients, nor on the management of surgical procedures.

    Aims: To gather insights into the treatment situation of patients (12 years and older) with mild hemophilia A or B and their quality of life.

    Methods: Using an anonymous cross-sectional patient survey, we documented online patients 'experience with factor concentrates and other treatments. Patients ≥12 years with diagnosed mild hemophilia A or B participated. We evaluated patients ‘satisfaction regarding the therapy situation, support by physicians, and assessed the impact on Quality of Life (EQ-5D-5L).

    Results: We included 44 patients (35 hemophilia A, 5 hemophilia B, 3 unknown) with a median age at survey of 33 years. The median age at diagnosis was 6.0 years. Median factor activity was 14.0%. 84.2% (n=32) were treated with factor concentrates in the past. The most frequent reasons for treatment were surgery or joint bleedings (each 65.6%, n=21). Approximately half of the patients (n=20) had complications caused by untreated bleeding due to accidents (4), surgical bleeding (11), dental treatment (10), joint bleeding (10) and spontaneous bleeding (2). Only 4 patients (10.3%) were prophylactically treated with factor concentrates. Health state indicates slight problems with mobility, with washing/ dressing, moderate pain and slight anxiety or depression

    Conclusion(s): Bleeding complications, especially joint bleeding, seem to be highly underestimated in patients with mild hemophilia A and B. To start an early prophylaxis to avoid joint damage should be discussed even in patients with mild hemophilia.
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