Citation
J Haem Pract 2014; 1(2):J Haem Pract 2014; 1(2): 2-7. doi: 10.17225/jhp00016

Authors: Robin Sager

Robin Sager
Haemophilia Clinical Nurse Specialist
The Royal London Hospital, Whitechapel, London E1 1BB, UK.

Abstract

It is said that men have haemophilia and women are carriers, affected or unaffected. The terminology used is significant. Why it is that men are described as ‘having’ haemophilia and women with haemophilia are described as affected carriers? After all the men ‘carry’ the gene just as the women do and pass it on to their daughters. Likewise many women have Factor IX or Factor VIII levels that, if they were a man, would be categorised as mild or even in some cases moderate or severe haemophilia. Yet haemophilia is widely viewed as a condition affecting men with women as the passive vehicle for its transmission from generation to generation.
There are many issues for women that are affected by this choice of terminology. Among them are their ability to access healthcare and their acknowledgement within healthcare systems throughout the world. Are women with the same factor level as men viewed in the same manner by healthcare professionals and do they acknowledge their own bleeding disorder as being the same as their male counterparts? How many women are unaware of their own bleeding disorder because they have only been viewed as ‘carriers’? Research into these issues is limited. This paper explores the aspects that affect women with haemophilia, from the definition in its historical context, obtaining a diagnosis, psychosocial elements, access to treatment, changing attitudes and acknowledgements within both the medical community and the wider community.

Acknowledgements

RS wrote this article as an assignment on the Essentials Of Haemophilia course, run by the Haemophilia Nurses’ Association and Canterbury Christ Church University.

References

  1. Blackwell’s Dictionary of Nursing. Oxford: Blackwell Science Ltd, 1994.
  2. Merriam Webster Dictionary (2013). Available at: http://www.merriamwebster.com/medical/haemophilia?show=0&t=1375010894 (accessed: 28 July 2013).
  3. Pemberton S. The Bleeding Disease: Hemophilia and the Unintended Consequences of Medical Progress. Baltimore: The Johns Hopkins University Press, 2011.
  4. Resnik S. Blood Saga: Hemophilia, AIDS, and the Survival of a Community. Berkley: University of California Press, 1999.
  5. White GC, Rosendaal F, Aledort LM, et al. Definitions in haemophilia. International Society on Thrombosis and Haemostasis, 2000. Available at: http://c.ymcdn.com/sites/www.isth.org/resource/group/d4a6f49a-f4ec-450f-9e0f-7be9f0c2ab2e/official_communications/defhem.pdf (accessed 26 August 2013
  6. Mauser-Bunschoten EP. Symptomatic Carriers of Hemophilia. Montreal: World Federation of Hemophilia, 2008. Available at: http://www1.wfh.org/publications/files/pdf-1202.pdf (accessed 11/8/13).
  7. Srivastava A, Brewer AK, Mauser-Bunschoten EP, et al. Guidelines for the management of hemophilia. Haemophilia 2013; 19(1): e1-47.
  8. Eckhardt CL, van Velzen AS, Peters M, et al. Factor VIII gene (F8) mutation and risk of inhibitor development in nonsevere hemophilia A. Blood 2013; 122: 1954-62.
  9. NHD User Manual. National Haemophilia Database, 2013. Available at: https://nww.mdsas.nhs.uk/NHD/pages/NHDUserManual.pdf (accessed 16 August 2013).
  10. Soucie JM, Evatt B, Jackson D. Occurrence of Hemophilia in the United States. American Journal of Hematology 1998; 59: 288–294.
  11. Byams VR, Kouides PA, Kulkarni R, et al. Surveillance of female patients with inherited bleeding disorders in United States Haemophilia Treatment Centres. Haemophilia 2011; 17 (Suppl.1): 6-13.
  12. Schulman S. Mild Haemophilia. Montreal: World Federation of Hemophilia, 2006, revised 2012. Available at: http://www1.wfh.org/publications/files/pdf-1192.pdf (accessed: 6 July 2013).
  13. World Federation of Hemophilia. Carriers and Women with Hemophilia, Montreal: World Federation of Hemophilia, 2012.
  14. Stonebraker JS, Bolton-Maggs PHB, Soucie JM, et al. A study of variations in the reported hemophilia B prevalence around the world. Haemophilia 2012; 18(3): e91-4.
  15. Stonebraker JS, Bolton-Maggs PHB, Brooker M, et al. A study of reported factor IX use around the world. Haemophilia 2011; 17(3): 446-55.
  16. Kasper CK, Lin JC. How many carriers are there? Haemophilia 2010; 16(5): 842.
  17. Winikoff R, Amesse C, James C, et al. (2004) The role of haemophilia treatment centres in providing services to women with bleeding disorders. Haemophilia 2004; 10: 196-204.
  18. Bolton-Maggs PHB. Optimal haemophilia care versus the reality. British Journal of Haematology 2006; 132 (6): 671-682.
  19. Skinner MW. WFH: Closing the global gap – achieving optimal care. Haemophilia 2012; 18:1–12.
  20. Plug I, Mauser-Bunschoten EP, Brocker-Vriends AHHT, et al. R. Bleeding in carriers of hemophilia. Blood 2006; 106(1): 52-56.
  21. Renault NK, Howell RE, Robinson KS, et al. Qualitative assessment of the emotional and behavioural responses of haemophilia A carriers to negative experiences in their medical care. Haemophilia 2011; 17(2): 237–245.
  22. Khair K, Holland M, Pollard D. The experience of girls and young women with inherited bleeding disorders. Haemophilia 2013; 19(5): e276-81.
  23. James AH. More than menorrhagia: a review of the obstetric and gynaecological manifestation of bleeding disorders. Haemophilia 2005; 11: 295-307.
  24. Chi C, Lee CA, Shiltagh N, et al. Pregnancy in carriers of haemophilia. Haemophilia 2007; 14: 56-64.
  25. Ebrahim SH, Kulkarni R, Parker C, Atrash HK. Blood disorders among women: Implications for preconception care. American Journal of Preventive Medicine 2010; 38: S459-S467.
  26. Franchini M, Favaloro EJ, Lippi G. Mild hemophilia A. Journal of Thrombosis and Haemostasis 2010; 8: 421–432.
  27. Peerlinck K, Jacquemin M. Mild haemophilia: a disease with many faces and many unexpected pitfalls. Haemophilia 2010; 16 (Suppl. 5): 100–106.
  28. Cassis FRMY, Querol F, Forsyth A, Iorio A. Psychosocial aspects of haemophilia: a systematic review of methodologies and findings. Haemophilia 2012; 18: e101-e114.
  29. Jutel AG. Putting a Name to It: Diagnosis in Contemporary Society. Baltimore; Johns Hopkins University Press, 2011.
  30. Haemophilia Society. Women Bleed too UK Haemophilia Society (No Date) For carriers of haemophilia with bleeding symptoms.
  31. NHF. Victory for Women with Blood Disorders. National Hemophilia Foundation, 2006. Available at: http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=339&contentid=1564&rptname=women (accessed 18/8/2013).
  32. Association Francaise des Hemophiles, 2012. Les femmes et l’hemophilie. Available at: http://www.afh.asso.fr/spip.php?article222 (accessed 18/8/2013).
  33. WFH. Definitions and terminology. Montreal; World Federation of Hemophilia, 2012. Available at: http://www.wfh.org/en/abd/carriers/carriers-definitions-andterminology-en (accessed 11/8/13).
  34. Koerper MA. Time to End a Sex-Based ‘Truism’. Hemaware (Summer), 2012. Available at: http://www.hemaware.org/story/time-end-sex-based-truism (accessed: 3 August 2014).
  35. Aldridge S. The Carrier Barrier. Hemaware (Summer), 2012. Available at: http://www.hemaware.org/story/carrier-barrier (accessed: 3 August 2013).
  36. Evatt BL. The tragic history of AIDS in the hemophilia population, 1982-1984. Journal of Thrombosis and Haemostasis 2006; 4(11): 2295-2301.
  37. Mannucci PM. Desmopressin (DDAVP) in the treatment of bleeding disorders: the first 20 years. Blood 1997; 90(7): 2515-2521.

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