The European Haemophilia Consortium (EHC) is pleased to announce the publication of Barriers and challenges faced by women with congenital bleeding disorders in Europe: Results of a patient survey conducted by the European Haemophilia Consortium by EHC’s PARTNERS Consultant Declan Noone, EHC Steering Committee Member and Women’s Committee Head Naja Skow-Rasmussen, University Medical Center Utrecht’s (UMC Utrecht) Dr. Karin P.M. van Galen, Michelle Lavin, Clinical Lead for coagulation haematology research at the Irish Centre for Vascular Biology at the Royal College of Surgeons in Ireland and Dr. Rezan A Kadir, Consultant Obstetrician and Gynaecologist with a subspecialty in Fetal Medicine at the Royal Free Hospital in London, UK, in Haemophilia Journal, the Official Journal of the World Federation of Hemophilia, the European Association for Haemophilia and Allied Disorders (EAHAD) and the Hemostasis & Thrombosis Research Society.
This timely and ground-breaking survey highlights the critical need for better education, improved diagnosis, access to treatment and psychosocial support for women with bleeding disorders. The publication is part of EHC’s continuing effort to reach and assist often neglected members of the bleeding disorders community. The publication also focuses attention on EHC’s upcoming May 24-26 First European Conference on Women and Bleeding Disorders in Frankfurt, Germany.
by Declan Noone, Naja Skouw‐Rasmussen, Michelle Lavin, Karin P. M. van Galen, Rezan A. Kadir
First published: 29 April 2019 https://doi.org/10.1111/hae.13722
Abstract
Introduction
Historically, issues faced by women with bleeding disorders (WBD) have been underestimated. While advances in genetic testing have resulted in improvements, significant challenges remain in the initial recognition of abnormal bleeding and referral of WBD.
Methods
The European Haemophilia Consortium (EHC) developed a questionnaire for WBD to provide insights into the barriers and challenges faced by WBD in Europe.
Results
In total, 709 WBD responded to the survey from 32 countries, predominantly from western European countries (94%). A delay in ascertaining the diagnosis of a congenital bleeding disorders (CBD) remains, with a median age at diagnosis of 16 years. The presence of family history is strongly associated with a lower median age at diagnosis of 6 years. WBD reported significant disease impact on their day‐to‐day life, most evident for the rarer CBD. The bleeding symptom of biggest impact on daily life is heavy menstrual bleeding (HMB), reported by 55% of women. Importantly, 25% of WBD reports that their condition severely impacted their decision to have or has prevented them from having children. Respondents registered with Haemophilia Treatment Centres (HTC) are 2.2 times more likely to receive treatment compared to WBD in other hospital services.
Conclusion
Improved education for both patients and healthcare providers is essential to improve time to diagnosis, access to treatment and psychosocial supports for WBD in Europe.
Jim is aged 66 and has Haemophilia A with a high titer inhibitor which he developed at age 14.
Maria Elisa Mancuso (MD, PhD) is a Haematologist and works as a Senior Haematology Consultant at the Center for Thrombosis and Haemorrhagic Diseases of IRCCS Humanitas Research Hospital in Rozzano, Milan, Italy. She is Adjunct Clinical Professor at Humanitas University. She obtained a post-degree in Clinical and Experimental Haematology and a PhD in Clinical Methodology. She is involved in clinical research and has published several original articles in peer-reviewed journals a The Lancet, Blood, Journal of Thrombosis and Haemostasis, Haematologica, Thrombosis and Haemostasis, British Journal of Haematology and Haemophilia. She is reviewer for several peer-reviewed journals and member of the Editorial Board of JTH. She is a member of several scientific societies (ISTH, WFH, ASH, EAHAD, SISET, AICE) and was a medical member of the Inhibitor Working Group of the European Hemophilia Consortium. She is co-chair of the ADVANCE Study Group. She has acted also as co-chair of the Scientific and Standardization Subcommittee of ISTH on FVIII, FIX and rare bleeding disorders. She has been involved as principal and co-investigator in several clinical trials, and she takes care of both children and adults with hemophilia and other congenital bleeding disorders with a specific scientific interest in novel therapies, prophylaxis, inhibitors, and chronic hepatitis C.
As a patient with factor II deficiency, the diagnostic and treatment of rare bleeding disorders is a matter dear to my heart. My motivation to participate in the work of the ERIN committee is to improve both diagnostic and treatment for patients with rare bleeding disorders across Europe.
Economist and financial expert by profession, executive coach and trainer by passion and haemophilia advocate by every drop of my blood through my son (who has severe haemophilia A with inhibitors). Bringing a good decade of practical experience from the corporate insurance world, laser focus, growth mindset and resilience from my own experience, offering you anything I can just do, in hope that together we can make life more fulfilled for those impacted by bleeding disorders.
Amy Owen-Wyard is a Registered Mental Health Nurse. With experience working with children, young people, their families and adults with severe and enduring mental health conditions. Amy was also involved in a service improvement to provide a holistic care approach for those in general hospitals to support both their mental and physical health, whilst sharing her expertise and knowledge in mental health with the wider multidisciplinary team.
