Women and bleeding Dr. Nita Radhakrishnan Sir Ganga Ram Hospital, New Delhi, India Annual Meet WG HFI 6 Dec 2014

Woman Challenges in the life of a woman Many Hemostatic challenges in the life of a woman Menstruation (Menorrhagia = Heavy menstrual bleeding) Pregnancy Childbirth

Bleeding disorders in women Hemophilia A, B carrier state Von Willebrand disease Rare bleeding disorders Inherited platelet disorders

What has history taught us?

Royal disease Queen Victoria Had no ancestors with hemophilia 9 children Disease appeared in her 8th child Leopold who was “very delicate” “We must have some strong blood” Spread to the royal family of England Spread to German, Russian and Spanish royal families

Von Willebrand disease Most common bleeding disorder 1% of the population Eric von Willebrand Index case: 5 year old died eight years later following menstrual bleeding 23 affected family members Her grandmother died following delivery

Why is bleeding more in women in VWD? More hemostatic challenges in a woman

Menstrual Cycle is a Vital Sign American College of Obstetricians and Gynecologists American Academy of Pediatrics Normal menstruation Begins at 11 to 14 years of age Normal interval is is 21 to 45 days Normal length of menstrual flow is 7 days or less Not more than 3-6 pads/ tampons per day What is not normal? Heavy menstrual bleeding >7 days Loss of more than 80 ml per menstrual cycle Ovarian cysts with bleeding Endometriosis Bleeding following delivery lasting > 24hours

Ovarian cysts with bleeding Ovulation with bleeding is never normal 7% of VWD reported to have bleeding ovarian cysts Can bleed into the abdominal cavity Increases risk of endometriosis

Problems during menstruation

Pregnancy and bleeding 4067 deliveries in women with vWd 2000-2003 (USA) Risk of bleeding before delivery 10.2 Risk of bleeding following delivery 1.5 Risk of receiving blood transfusion 4.7 Risk of death in mother low (but still high than normal women) James A 2007

Problems during childbirth

Childbirth in women with inherited bleeding disorders Primary PPH Secondary PPH Normal 5 0.7% Hemophilia carriers 22% 11% VWD 19% 29%

It is also not normal Prolonged bleeding from cuts Following surgery Bruising without any trauma Nose bleeds Following tooth extraction Blood in stools Anemia requiring blood transfusion

What is the prevalence of inherited bleeding disorders in women with menorrhagia?

Systematic review 998 women with menorrhagia: 13% had vWD Shankar M BJOH 2004

7/75 women with menorrhagia had underlying bleeding disorder N Sherif 2014 53% had underlying bleeding disorder Rosa Diaz 2014

47.8% had some underlying hemostatic disorder 17.7% platelet dysfunction 13.3% Von Willebrand disease 12.4% factor deficiency V Serevalli 2013 14.5% had bleeding disorders 6.5% von Willebrand disease in five 5.2% platelet function disorder Turkiz Gursel 2014

120 women with menorrhagia 19.16% had bleeding disorder 11.6% VWD 3.3% Glanzmann thrombasthenia Sucheta Trasi 2005

Testing for bleeding disorders is a must for Adolescent girls who present with menorrhagia Adult women with chronic menorrhagia who have no other cause Women who are about to undergo hysterectomy for menorrhagia ACOG 2002

If VWD presents more in women, why are less women diagnosed in India? Gender issues If VWD presents more in women, why are less women diagnosed in India?

HTCs were originally created to treat men with hemophilia Since 1990s almost 9000 women have sought medical care in HTCs across USA Indian data?

Number of women in HTCs in USA

What proportion of women with inherited bleeding disorders have menorrhagia?

70-100% of those with vWD 10-70% of those with other inherited disorders 5-98% with inherited platelet disorders Significantly longer, heavier menstruation Significantly more hysterectomies for menorrhagia

What are the chances of needing medical care once diagnosed? S Sanders 2012

How does bleeding affect their lives?

Health related QOL in VWD Women with VWD significantly worse than those of men with VWD Almost as low as that of severe hemophilia More school absenteeism Less women likely to undergo secondary education than men (5.5% vs 35%)

QOL differences between men and women with VWD

QOL differences between those who had menorrhagia and who did not

Kadir K Khair 2013

K Khair 2013

K Khair 2013

K Khair 2013

Whom do you speak to often?

What restrictions do you face? K Khair 2013

Health related QOL in VWD 39% cut down their activities due to menorrhagia 39% had to cut down the work that they do 47% felt they had accomplished less 19.3% felt that dysmenorrhea affected their lives 22.6% did not go to school/work during periods 48.4% were hospitalized for a heavy menstrual bleeding Fadhlaoui A 2012

Survey of health related QOL study SF 36 v2 survey 30 subjects 3 groups Girls (Indian) with bleeding disorders Girls with leukemia undergoing chemotherapy Normal age matched controls Radhakrishnan N (unpublished)

How healthy are you? 1 very good – 5 very poor

Does health limit you in your activities Does health limit you in your activities? 1: A lot 2: Little 3: Not at all

Has you health interfered with social activities? 1:No 5:Very much

Factors that determined QOL Underlying bleeding disorder Being female Bleeding disorder (VWD) and being female

Is there a severity scale for QOL?

Awareness is less in at all stages

Surveying gynecologists 983 ACOG members surveyed in 1996 8% of their patients complained about menorrhagia 74% patients presumed to have anovulatory cycles 4% doctors considered VWD as a cause of menorrhagia 3% considered referring them to specialists <1% of women should have a bleeding disorder VR Byams 2007

Surveying gynecologists Repeated the survey in 2009 1200 gynecologist interviewed by mail 42.4% (503) responded to the survey >70% enquired about other sites of bleeding >60% enquired about a family history of bleeding 77% said they would consider VWD as a cause What caused this change? Efforts by hematology providers, community based organizations, government to raise awareness VR Byams 2012

Surveying gynecologists UK > 80% of the respondents considered the prevalence of VWD in women with menorrhagia to be <1% 12% and 2% of the respondents would consider testing for VWD in an 18 and a 35 year old Chi C 2006

How can we improve? Raise awareness (Advocacy) General public Schools Young girls Doctors

Day Pad 1 2 3 4 5 6 7 8 20 Daily score Day Tampone 1 2 3 4 5 6 7 8 5 20 Daily score Total Score >100 is equivalent to >80ml blood loss per cycle Total score: PICTORIAL BLEEDING ASSESSMENT CALENDAR Higham JM, O’Brien PM, Shaw RW Assessment of menstrual blood loss using a pictorial chart Br J Obstet Gynecol 1990; 97:734-9

International efforts to raise awareness Women bleed too (UK) Project Red Flag (USA) MyGirlsBlood WG HFI

To conclude Lets protect girls with any bleeding disorder Collaborative efforts Look after menstrual hygiene Heavy menstrual bleeding Identify potential bleeders from the population Prevent complications

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