Latest / EHA Unplugged / Optimizing Global Care for Women with von Willebrand Disease (VWD) (EHA2024)

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Optimizing Global Care for Women with von Willebrand Disease (VWD) (EHA2024) · EHA Unplugged

• 0:06
  In this episode of EHA Unplugged, we will revisit a
• 0:10
  spotlight talk from EHA 2024. Doctor Michelle Lavin from Saint
• 0:15
  James's Hospital in Dublin discusses optimizing global care
• 0:18
  for women with von Willebrand disease.
• 0:20
  Good. Morning everybody, and I'm
• 0:24
  honored to be given the. Opportunity to speak today, but
• 0:28
  also for E had to actually prioritize this area.
• 0:31
  Of women with bleeding. Disorders, which has.
• 0:33
  Long been neglected, I think, by the community, not only in
• 0:36
  hemostasis and thrombosis. But.
• 0:38
  Of all of hematology, my name is Doctor Michelle Lavin.
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  I'm a consultant hematologist treating adults.
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  With bleeding disorders. In the National Coagulation
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  Center in Saint James's in Dublin, and I'm also.
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  A researcher in the Irish Center for Vascular Biology in the
• 0:53
  RCSI. And as you.
• 0:55
  Can hear very passionate about. The care for women and girls.
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  With eating disorders, I'm. Going to start with a question,
• 1:00
  and that question is why is it? OK to trivialize.
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  Bleeding in women because this happens on a.
• 1:05
  Daily basis. And it's, you know, overt and
• 1:09
  pernicious within our medical societies and this case.
• 1:13
  Is a a lady who. Was in with me recently.
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  And it really exemplifies what we see on a very regular basis.
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  In my clinic. So I was referred to an 18 year
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  old. Woman who had you know the.
• 1:23
  Point of such heavy periods that.
• 1:26
  Finally, somebody thought could. This be a bleeding disorder, but
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  she had suffered from long. Term iron.
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  Deficiency throughout her. Teenage years.
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  She'd had nosebleeds. As a child and was advised.
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  That they she would grow. Out them despite needing
• 1:38
  cauterization in childhood. And she had easy bruising.
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  But that was, you know, normal for her.
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  And I think we forget to. Ask what?
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  How has this impacted you the most?
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  Or where has your bleeding caused?
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  You the most trouble. And for her, it was that she
• 1:53
  loved to dance. And she used to.
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  Do ballet and hip hop. But the classes were an hour
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  long and she had soaked through on three occasions and after the
• 2:01
  third time of embarrassing. What she felt herself by,
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  soaking through her clothes, she quit.
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  Dancing so she had given up something that she loved because
• 2:09
  of the impact of bleeding and when we.
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  Tested her she. Had type 1 Von Willebrand
• 2:13
  disease and was not only iron deficient but also anemic.
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  And as I was asking her the questions, this often happens
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  that the mum is there nodding to.
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  All the responses. As well, so I took the mum aside
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  and and did a consultation with her and she had heavy menstrual
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  bleeding. She had never really gone to the
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  GP. About it or when she.
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  Had it had been, she was told this was normal.
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  She'd had. Bruising all her.
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  Life but had. Three children and had two
• 2:38
  postpartum hemorrhages. One was described to her.
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  As mild, whatever that means when it comes to bleeding and.
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  Excessively after childbirth. And one was actually quite
• 2:47
  significant and she needed a transfusion and she had been
• 2:50
  going to the GP recently. But more about.
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  Perimenopausal bleeding, which is often when we'll see women
• 2:55
  encountering problems. And when we tested her, she had.
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  Low von Wellebrand factor. She was also iron deficient and
• 3:02
  anemic. But she had had.
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  GI endoscopies twice and had an ultrasound pelvis once but no
• 3:09
  one had. Thought about could this be a
• 3:11
  bleeding disorder? This is a very typical.
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  Passion that people have to jump through so many hoops before.
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  Anyone even considers? Could this merit a hemostatic?
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  Referral, so we wrote. This article recently about the
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  challenges facing women and. Girls with bleeding disorders.
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  And they're really. Myriad they.
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  Face it in terms of diagnostic inequalities, but also
• 3:31
  suboptimal treatments. And to focus first of.
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  All on the diagnostic inequalities that face women and
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  girls. Of bleeding disorders, you know
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  they're both self. Imposed as well as system
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  imposed. Because for women.
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  Themselves, they may not recognize what heavy
• 3:46
  menstruation is. We don't openly.
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  Discuss heavy menstrual bleeding.
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  People don't understand. When their periods are heavy.
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  And there's also a normalization, and this is heard
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  time and time again within families.
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  Where a woman doesn't appreciate her periods are heavy because
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  they're similar to her sister and her mother, who may also
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  experience. Heavy menstrual bleeding.
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  Significant stigma exists regarding menstruation
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  internationally, and it's easy to dismiss this as, oh, this is
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  in some other country. Or some other place or we're
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  we're open about periods. We provide care and support to
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  women. But even in this Conference
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  Center today, there isn't one female toilet that has menstrual
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  products. Available or for sale?
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  You know, it's as if periods don't exist.
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  And there's poor awareness when people do go to general
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  practitioners or primary. Care there's poor.
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  Awareness about what constitutes abnormal bleeding they may not
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  recognize heavy nosebleeds as being particularly heavy or
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  problematic and when somebody attends a.
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  An ear, nose and throat. Consultant and has cauterization
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  everyone is looking at the individual bleeding symptom that
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  relates to. Their speciality.
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  Be it gynecological bleeding, no nasal bleeding, you know are.
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  A. Leading complaints to the GP and
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  they're not looking at the. Overall Global.
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  Phenotype of that individual and putting it together saying this.
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  Could be suggestive of a bleeding disorder.
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  Cumulatively this. Leads to delays in referral and
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  diagnosis, and there's good. Data to support that this.
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  Particularly effects women in the Dutch.
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  Study They looked at all patients diagnosed with a
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  bleeding disorder. And found that there was a.
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  Five year additional. Delay in referral and diagnosis
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  in women versus men. And this was not just related to
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  gynecological bleeding. Because even in childhood before
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  the onset. Of monarch.
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  Girls experienced longer. Times and longer.
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  Persistent bleeding before consideration of referral.
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  And that's probably. Related to the fact that people
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  are aware. Of hemophilia and have bleeding
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  disorders and men very. Tightly associated in their.
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  Learning and don't. Appreciate there are other
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  bleeding disorders and that. Women are more.
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  Predominantly affected because. Of gynecological bleeding.
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  Even if you are referred, or if you do have tests before them,
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  there's. Inequalities in specialist
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  testing. Because.
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  There's von Willebrand factor antigens and.
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  Activities are expensive tests to deliver.
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  They're specialized. They require expert.
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  Interpretation. And that's not universally.
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  Available. Even within higher income
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  settings and is very. Has a very.
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  Low availability in lower and. Lower middle.
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  Income countries So what are the impacts of these for von
• 6:27
  Willebrand disease diagnosis and registration you.
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  Know is there. Actually an influence on these
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  factors and I would. Argue there is because we have
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  data from the world. Federation Annual Global.
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  Survey and we looked at the registered individuals.
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  With von Willebrand. Disease worldwide.
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  Now to remind. You the expected.
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  Prevalence of hemophilia is one in 10,000 and based on studies.
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  By Rodrigo. ET al.
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  And Bowman ET al. The expected prevalence.
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  Of reduced von Willebrand factor levels and bleeding should be
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  one in 1000 when we looked at the annual global.
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  Survey Data. And looked at the number of
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  patients. Registered per.
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  Population For each individual country, we had to reduce the
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  denominator from 1000 to 1,000,000 to actually get a
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  sensible number that could. Be, you know, displayed.
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  For some countries. So we should expect to see one.
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  1000 patients with von Willebrand disease per million
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  population. What we found was a median.
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  VWD registrations. In high income settings.
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  Was 45. There was a significant range
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  between 1 and 332, which I'm proud to say is Ireland and then
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  that range between 45 per million in high income countries
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  right down to point. 5. Per million in low income
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  countries. So even the most severe.
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  Phenotypes aren't being diagnosed in low income settings
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  and what was interesting was the gender distribution and how this
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  changed and was VE and varied based on income status in high
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  income countries. You see 2/3 of the.
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  Population are female that are registered with Fomwella brand
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  disease. Now it's an autosomal.
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  Disorder, so you would expect an.
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  Equal male and female prevalence but because of gynecological
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  bleeding and that burden, women are.
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  Disproportionately represented in.
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  VWD registration numbers, but in low income settings you can see
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  this completely changes to where males exceed females.
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  So income status. Is influencing not only the
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  number of registrations, but the ability of a woman to get a
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  diagnosis and registered with von Willebrand disease.
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  So what about if you get through these myriad of.
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  Challenges you finally make it to the hemophilia treatment
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  center. What does that mean in terms of
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  are you? Getting the best care is the
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  care. Being delivered to you the.
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  Same. As for men with your condition
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  and I would say no, we're actually failing women even in
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  our own centers and I work in AHTC.
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  So I. I'm criticizing myself as well.
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  Because our centers. Even the name hemophilia is
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  very. Gendered.
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  It's very male. Specific to a.
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  Lot of people and our centers have grown up.
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  Around the needs of men within severe hemophilia and we have
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  transposed the outcomes that we use for men like annual bleed
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  rates. Joint.
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  Bleeding rates onto von Willebrand disease.
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  Where they're. Suboptimal and.
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  They don't consider the degree of.
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  Bleeding that people can have. If you have 12 periods a year.
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  And use an annual bleed rate. You will have a bleed rate of
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  12. That doesn't tell you if they're
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  20 days long or two days long. It doesn't tell you anything.
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  About the severity of it. So we can't use these outcome
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  measures. That we have traditionally.
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  Built up around men and applied them to women and expect.
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  To get good results. We lack female care pathways.
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  If somebody comes in with. A.
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  A hemarthrosis we would never. Dream of ringing the orthopedic
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  team and asking them what to do. We manage hemarthrosis.
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  Ourselves if somebody. Comes in with acute heavy
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  menstrual bleeding. Is that the same?
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  Are you ringing your gynecology? Colleagues saying how do I
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  manage this, this strange hormonal bleeding?
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  I don't know what to do, you know?
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  We don't see it as actually part of our care to deliver.
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  Management of gynecological bleeding and that's adding to
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  the problems that women face. We have knowledge gaps on the
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  optimal treatments because women do not tend to be represented in
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  clinical trials. Of bleeding disorder products.
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  And despite knowing that the significant differences between
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  the pharmacokinetics and pharmacodynamics for drugs.
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  We. Take results in a male
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  population and apply that in a female population, and I think
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  we have a. Real lack of culture of
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  prophylaxis, not only. For women, but for von
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  Willebrand disease in general. From the World Bleeding Disorder
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  Registry, they took data. From around the.
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  World this year and for the. First time von Willebrand
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  disease was included in the. Worldfed's registry.
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  And when they looked at the data internationally from low middle
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  income and high income countries, 28% of adults with
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  hemophilia were on prophylaxis and 2% of people with von
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  Willebrand disease were on prophylaxis.
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  So everybody with von Willebrand disease is disadvantaged.
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  But I think in. Particular women are because
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  when we. Think about prophylaxis.
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  We think about that hemophilia model of long term.
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  Regular, you know. Continuous prophylaxis.
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  Whereas women. Might only need.
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  A short term prophylaxis as a bridge to a more definitive
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  treatment. Like an IUD insertion for heavy
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  menstrual bleeding, or whilst they're trying to get pregnant
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  and have to come off hormonal contraception and there's no way
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  to guide dosing. There's no dosing.
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  Regimens for periodic. Prophylaxis for.
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  Heavy menstrual bleeding because we don't.
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  Even consider it. As an indication.
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  So cumulatively you can see. The challenges that are imposed
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  by society itself, by our healthcare.
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  Systems, but. Also by our hemostasis.
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  Community on women and girls accessing the care that they
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  need for von Willebrand disease. Now it can't all be bad news.
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  There are good things happening and I think it's fantastic even
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  when to have this session at EHA, you know, to be talking
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  about women with bleeding disorders and congresses.
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  All have tracks. For women.
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  And girls with bleeding disorders.
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  Which is fantastic now and. There's been a.
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  Number of changes that. Will hopefully help.
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  Women and girls internationally, not only did we have the Ashe
• 12:01
  2021 guidelines and that brought for the first time, a consensus
• 12:05
  definition of postpartum hemorrhage and heavy menstrual
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  bleeding. Can you believe it?
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  The hematology literature we didn't have a.
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  Consensus amongst us? As to what?
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  Heavy menstrual bleeding. Was or postpartum?
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  Hemorrhage was before that point, but also in 2021.
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  There was the first European principle.
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  To care for women and girls. Of bleeding disorders, so these
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  were the first. Principles to outline.
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  How we should actually look after?
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  Women and girls to attend our centres.
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  And it wasn't just for Europe. These are the.
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  First ones internationally. So the first time in the world
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  we said these are the standards. We set for our women.
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  This isn't hemostasis specific. But I.
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  Think it's adjacent to the issues that women face and.
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  The World Health. Organization last year released
• 12:46
  accelerating anemia reduction report and this looked at.
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  Actually. Highlighting the importance and
• 12:53
  the prevalence of anemia amongst women and they focused.
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  On a number of areas. Including iron malaria
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  treatment, folic acid supplementation, prevention of.
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  Postpartum hemorrhage, but a very.
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  Useful. Point in this report to really.
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  Bring it home to governments that don't think it's worth
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  investing in. Women's Health is.
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  That for every $1.00 invested. In interventions aimed at
• 13:14
  reducing anemia in women. Those 12.
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  Dollars in economic. Returns could.
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  Be realized and this. Is how we leverage care and.
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  Improvements. So we need to.
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  Actually identify anemia, identify our own deficiency and
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  treat it and allied. With this I was very happy to
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  work with. The International.
• 13:32
  Federation of Obstetrics and Gynecology, OR.
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  Figo and. Last year, they released their
• 13:36
  first. Position statement on iron
• 13:38
  deficiency and anemia in women and girls and they are relating,
• 13:42
  as simple as that concept should be.
• 13:44
  Iron deficiency and. Heavy menstrual bleeding.
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  Surely all of us should think about that, but actually, if you
• 13:49
  look at The Who iron deficiency sits within.
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  Nutrition, not health. It's.
• 13:55
  Not considered a medical problem, it's considered a
• 13:57
  dietary issue. Now I have yet to come across a
• 14:00
  woman with a bleeding. Disorder.
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  Who could eat her way? Out of iron deficiency.
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  Because if you're bleeding. You will lose some.
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  You know, if you have heavy bleeding, you cannot maintain
• 14:08
  your dietary intake. You will have.
• 14:10
  In there. Invariably iron deficiency and
• 14:12
  it's. Only the rate at which that
• 14:14
  develops that can vary, so the Figo.
• 14:17
  Statement has said. That iron deficiency is highly.
• 14:19
  Prevalent and the symptom of heavy menstrual bleeding is the
• 14:22
  most common cause, and it puts the onus on obst obstetricians
• 14:26
  and gynecologists internationally to.
• 14:28
  Test for to. Check and to treat iron
• 14:31
  deficiency as well as identification and treatment of
• 14:33
  the underlying 'cause this. Is a really big step.
• 14:35
  Forward, as simple as it sounds, and finally, within von
• 14:39
  Willebrand disease, we now. Have the core VWD data set, so
• 14:44
  the. Problem with von Willebrand
• 14:45
  disease studies. Is not only is there very few of
• 14:47
  them, but that everybody? Uses completely different
• 14:51
  outcome measures. So it's very difficult to
• 14:53
  compare. Products between trials and the
• 14:55
  core. VWD project seeks.
• 14:58
  To harmonize outcomes for all studies involving patients with
• 15:01
  von Willebrand disease and female.
• 15:04
  Health was felt to. Be such an.
• 15:05
  Important area within this that they have their own subset of
• 15:09
  core outcomes. There's a broader core outcome
• 15:11
  set, but here I have the women. Girls and persons with the
• 15:14
  potential to menstruate. Outcome sets that are
• 15:16
  recommended if women are included in studies.
• 15:19
  So hopefully this will. Actually get.
• 15:20
  Into some more. Granular detail on the impact of
• 15:23
  heavy menstrual bleeding of new products.
• 15:27
  So in conclusion today. You know, I think it's a.
• 15:30
  Time of exciting change. There's momentum behind Women's
• 15:33
  Health, and I would. Advocate for you all to go.
• 15:36
  Back to your centres and to look what you're doing critically and
• 15:39
  appraise. How we can improve our
• 15:41
  detection, our awareness? Within our local communities and
• 15:44
  our. Treatment for.
• 15:44
  Our women who attend us, you know, we can't just keep doing
• 15:47
  things. My least favorite thing in
• 15:49
  medicine. Is why do you do it?
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  Because we always did. You know it's time to change
• 15:53
  things up. So we need to.
• 15:56
  Recognize we need to treat and we need to investigate heavy
• 15:59
  menstrual bleeding. Clearly, women with bleeding
• 16:01
  disorders have more. Than heavy menstrual bleeding.
• 16:04
  But this is a. This is a.
• 16:05
  Cardinal sign of a bleeding disorder.
• 16:08
  You know we. Can use this to identify women
• 16:11
  and not only reach that woman but her wider family who may
• 16:14
  also be affected. With a bleeding disorder, we
• 16:17
  need to improve. Awareness of von Willebrand
• 16:19
  disease internationally people don't know what it is, they
• 16:22
  don't know they. Should care about it.
• 16:23
  And the most frequent response I get from GPS is.
• 16:26
  I don't have anyone with that condition and I think.
• 16:29
  You probably do, you just don't realize it yet.
• 16:32
  So we need. People to care?
• 16:34
  About von Willebrand disease more and we should be informing
• 16:37
  that this is a mucotaneous bleeding disorder.
• 16:39
  Because that's. Telling you what's wrong and
• 16:41
  what symptoms people face. We need to develop cheaper
• 16:45
  easier. Hemostatic testing.
• 16:47
  It's very complex and expensive. To deliver VWD.
• 16:50
  Testing, and that's imposing a barrier on people who may have
• 16:53
  this condition. In order to access the care.
• 16:56
  And finally, for the women and girls who are diagnosed.
• 16:59
  We need to develop evidence based care to address.
• 17:02
  Their specific needs, not what we feel they need, but what?
• 17:05
  They actually desire for us to treat, to improve.
• 17:08
  Their outcomes? So they can be like that girl at
• 17:10
  the start. Continuing in their dance
• 17:12
  career. Continuing with their daily
• 17:14
  activities, participating. In working schools to the
• 17:17
  fullest and not being left behind.
• 17:19
  And with that, I'd like to thank you all for attending and
• 17:22
  showing enthusiasm for women and girls.
• 17:27
  Just because the community at EHA might not know, but could
• 17:31
  you give a bit more information on if there are new treatments
• 17:35
  coming for from Wilibrand? Because I think traditionally
• 17:38
  people know about the IV formulations, but do you have
• 17:42
  any insights in what is out there?
• 17:45
  Well, in terms of von Willebrand disease, I mean, I still think
• 17:48
  we're so far behind hemophilia and you have traditionally we've
• 17:52
  relied on plasma drive, von Wellebrand factor concentrates
• 17:55
  and in the setting of things like the.
• 17:57
  Infected blood inquiry. In the UK.
• 17:58
  That's obviously very. Challenging to recommend
• 18:00
  prophylaxis with plasma drive products of any variety, even
• 18:03
  when they're doubly virally inactivated.
• 18:05
  And considered safe. We have obviously a new
• 18:07
  recombinant von Wellebrand factor that's recently been
• 18:09
  licensed for. Prophylaxis in Europe.
• 18:11
  It's been more widely available in the US for longer.
• 18:14
  These are very expensive products.
• 18:15
  And then there's. Pipeline products that are
• 18:17
  coming through like BT200 that are still undergoing clinical
• 18:20
  trials and there's. Also VGA. 036 is on the line,
• 18:24
  but things are. Slower to progress.
• 18:26
  On the R for wrong for hemophilia.
• 18:29
  What I'm worried about is that. Expensive products coming out
• 18:32
  doesn't improve access to care and we need to be looking and
• 18:35
  taking a step back at what we already have in our
• 18:37
  armamentarium and offering that to people.
• 18:40
  So DDAV. P is a fantastic treatment
• 18:42
  desmopressin and that's broadly available.
• 18:45
  It's on The Who Essential Medicines list.
• 18:47
  So it should be in every. Country tranexamic acids that's
• 18:50
  also an essential part of most women I have and most patients I
• 18:54
  have von Willebrand disease will have tranexamic acid at home.
• 18:57
  That's cheap unless you live in America, but that's a cheap and
• 19:01
  cost effective. Option.
• 19:03
  Unfortunately, that's only. In an IV formulation on The Who
• 19:06
  Essentials Medicines list based on the woman's study for
• 19:08
  postpartum hemorrhage, oral tranexamic acid isn't an
• 19:11
  essential medicine. So that's something that we're
• 19:13
  campaigning for as a community to make, you know, if we can't
• 19:16
  have, if you're in a country and you can't get access to maybe
• 19:19
  plasma, Dr. VWF concentrator recombinant, the fact that you
• 19:22
  can't get even oral tranexamic acid is a massive barrier.
• 19:25
  So I think what we need to do is, and I worry in hemophilia
• 19:29
  treatment that we have such fantastic treatments, but we're
• 19:32
  increasing the disparities globally.
• 19:34
  I want everyone to come along together and bring people up
• 19:36
  together rather than I don't. I hope that drug companies won't
• 19:39
  just focus on very expensive treatments.
• 19:41
  They'll be available to few, but improve treatment for all.
• 19:43
  My question is on how do you think we can improve the access
• 19:48
  to the diagnosis and also, you know, one thing that I've seen
• 19:54
  in the testing of the von Willebrand disease is high false
• 19:59
  negativities. How can that be improved?
• 20:02
  Yeah, so. Two questions there.
• 20:05
  The first. One about false negatives.
• 20:07
  So there's so many factors that affect VWF levels.
• 20:11
  And most 16 year olds that come to my clinic for the first time
• 20:14
  will have. High levels of.
• 20:15
  Phobotomy. Related stress so.
• 20:18
  I will tell them I don't mind if your levels are normal now I
• 20:20
  know they're. Probably much lower, you know,
• 20:22
  we'll see how you are after. A couple of visits and you
• 20:24
  realize we're not terrifying. Umm, but.
• 20:26
  Certainly. Stress will temporarily.
• 20:28
  Put up VWF levels what we have looked.
• 20:30
  At in our center is we reviewed about two and a half thousand.
• 20:34
  VWF levels over A2 year. Period.
• 20:36
  And we found that if your initial.
• 20:37
  Level was above. 70. We had only one case where the
• 20:41
  plasm. VWF level on repeat at any point
• 20:44
  was less than. 50 so. Once you're over. 70 or 80?
• 20:47
  I think your levels are probably above 50 at.
• 20:49
  Baseline, even if even if you have things like for botting.
• 20:51
  Related Stress. So that would be.
• 20:53
  About, you know, having thresholds or understanding what
• 20:56
  how von Willebrand factor can be affected.
• 20:58
  People often. Worry about doing VWF testing on
• 21:00
  the pill. That's something I hear a lot.
• 21:02
  You would need to be on a a greater than 50 microgram or 50
• 21:06
  microgram, ethanol, oestrol pill and most.
• 21:08
  Pills are 30. Or 25, so that isn't as much of
• 21:12
  an issue now. Because there's lower.
• 21:13
  Eastern Formulations. The first part of your.
• 21:16
  Question was about how do you improve testing worldwide and
• 21:18
  that's a much trickier issue. It's very it's the world feds
• 21:23
  to. Be fair are trying to work on
• 21:25
  point care testing for like you know, blood drop from Wellebrand
• 21:28
  factor antigens. So at least you could get an
• 21:30
  antigen in a low resource setting.
• 21:32
  You know, on a on a. Card based test.
• 21:34
  It's very challenging to deliver that.
• 21:37
  And there's also, I think potential for paired schemes
• 21:40
  that you could have centers internationally that samples
• 21:44
  could be negotiated and funded to send to those centers to test
• 21:48
  and relay. The results back we live in a
• 21:50
  much more interconnected. World and there's opportunities
• 21:53
  to actually provide real time results through, you know,
• 21:56
  emailing information. Forward and back if we set up.
• 21:59
  Those networks properly because I think investing in labs in
• 22:02
  every country that can. Deliver VWD.
• 22:04
  Is probably unrealistic, but investing in partnerships.
• 22:07
  To help and support will help. People get diagnosis and build.
• 22:10
  Up capacity.