Diagnosis and management of haemophilia

BMJ 2012; 344 doi: http://dx.doi.org/10.1136/bmj.e2707 (Published 2 May 2012)
Cite this as: BMJ 2012;344:e2707

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The “Parents Perspective” illustrated in Fijnvandraat et al(1) highlights the responsibility of the clinical team to “coach and guide” an individual and family newly diagnosed with a rare, genetic condition. There is an ever increasing requirement for specialist teams to provide and maintain a portfolio of contemporary, public-information resources, made increasingly challenging given the public’s expectation of information immediacy offered by the internet.

We simulated 30 opportunistic patient internet searches for haemophilia information in each of the 3 most common search engines (2) using term permutations: haemophilia; hemophilia; +/- A or B; carrier; information; child; treatment. First position of every search in every search engine was Wikipedia. However, identification of other websites was more dependent on search term combinations and search engine. For example, the UK national haemophilia society website was found in 26/30 and 21/30 searches for Bing and Yahoo respectively but only 3/30 in Google. In addition, despite the known existence of multiple bespoke haemophilia websites these rarely appeared in the top 10 search results.

Daily analysis of editorial authorship of the Wikipedia Haemophilia site for 4 weeks revealed 39 edits. Nine different editors had a profile, none with explicit haemophilia expertise. Remaining edits were made by 16 anonymous IP addresses. Could the public’s thirst for detailed knowledge be better served if medical professionals engaged with Wikipedia to correct the inaccurate, disseminate the accurate, highlight advances and champion best practice? Given the ongoing debate about open access, should publishing groups/authors be mandated to place reference to reviews such as this BMJ review(1) or to key studies such as Nathwani et al(3) in a public domain like Wikipedia? Currently, Wikipedia only references The New York Times reporting of the Nathwani trial’s publication(4).

Wikipedia provides a truly accessible, global vehicle to disseminate information for the good of patients anywhere, new and old, their families, students, their teachers or anyone of the wider world web who will always be impossible to pigeon hole for more bespoke portals. However, as a professional group, we haven’t decided whether we are, or should be phobic of engaging with Wikipedia more constructively.

1. Fijnvandraat K, Cnossen MH, Leebeek FW, Peters M. Diagnosis and management of haemophilia. BMJ 2012;344:e2707
2. http://www.hitwise.com/uk/datacentre/main/dashboard-7323.html
3. Nathwani AC, Tuddenham EG, Rangarajan S, Rosales C, McIntosh J, Linch DC, Chowdary P, Riddell A, Pie AJ, Harrington C, O'Beirne J, Smith K, Pasi J, Glader B, Rustagi P, Ng CY, Kay MA, Zhou J, Spence Y, Morton CL, Allay J, Coleman J, Sleep S, Cunningham JM, Srivastava D, Basner-Tschakarjan E, Mingozzi F, High KA, Gray JT, Reiss UM, Nienhuis AW, Davidoff AM. Adenovirus-associated virus vector-mediated gene transfer in hemophilia B. N Engl J Med. 2011 Dec 22;365(25):2357-65.
4. Nicholas Wade (December 10, 2011). "Treatment for Blood Disease Is Gene Therapy Landmark". The New York Times.

Competing interests: None declared

Michelle Kint, Visiting Medical Student

Daniel P.Hart

The Royal London Hospital, Haemophilia Centre, Haematology Day Unit, Ground Floor, South Tower, The Royal London Hospital, Whitechapel, London, E1 1BB, UK.

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The prevalence of haemophilia (Usually von Willebrand disease) amongst women undergoing hysterectomy to treat heavy menstrual bleeding is estimated as being between 10 – 25% ¹ In patients with haemophilias undergoing surgical procedures, it is important that there is a perioperative management plan in order to minimise bleeding and the complications this can cause.

Although we know there is a high prevalence of haemophilia in women with heavy menstrual bleeding, it is very rarely, if ever, considered in clinical practice. Despite the high prevalence of von Willebrand disease amongst women undergoing surgery for heavy menstrual bleeding, there have only been, for example, two articles on von Willebrand disease in the British Journal of Obstetrics and Gynaecology since 2000.

Thus in the 21st centaury we have this astonishing situation where gynaecologists are regularly undertaking invasive and major surgery on women, oblivious to the fact that they are at high risk of having a haemophilia. And fail to have a perioperative management plan to minimise bleeding, and the complications that may develop as a consequence. In many cases the need for surgery with all its attendant risks could be obviated by the use of desmopressin.

References

1 Shankar M, Lee C, Sabin C,Economides D, Kadir R. Von Willebrand disease in women with menorrhagia: a systematic review. British Journal of Obstetrics and Gynaecology 2004; 111:734–740

Competing interests: None declared

Malcolm John Dickson, Consultant Obstetrician & Gynaecologist

Nicola RK Anders

The Royal Oldham Hospital, Rochdale Road, Oldham, Lancashire, OL1 2JH

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