On 29 May 2021, the European Haemophilia Consortium (EHC) Committee on Women and Bleeding Disorders held a webinar on three topics about women and bleeding disorders, including:
- Preparing for menarche,
- Fertility, and
- Ageing.
Here below is a summary of the discussions and take-away messages for each session.
This event was developed by the EHC Women and Bleeding Disorders Committee. The next virtual webinar will be held on Saturday 25 September 2021, from 10.00 to 12.00 hrs CET. To keep up to date with EHC Women and Bleeding Disorders activities, sign-up for our mailing list.
Session on preparing for menarche:
Session moderated by Tatjana Markovic
Speakers:
- Marion Bräuer,
- Geraldine Connolly, and
- Beatrice Nolan.
On bleeding disorders and heavy menstrual bleeding
During this session, speakers reminded participants of the reasons behind heavy menstrual bleeding in girls and women with bleeding disorders. From a gynaecological point of view, every month, girls and women experience bleeding from the endometrium, the womb’s lining. When the body’s clotting mechanism does not function as it should, girls and women may experience heavier menstrual bleeding. However, speakers reminded participants that other factors could influence the bleeding, irrespective of the bleeding disorder. In general, the more severe the bleeding disorder, the more likely it will be for a girl to experience heavy menstrual bleeding. Of all girls with heavy menstrual bleeding, only 40% have a bleeding disorder. In addition, it is important to remember that adolescents tend to have heavier menstrual bleeding in general. Furthermore, because this is a new experience and a very personal one, it is also difficult to assess abnormal periods. This may lead to a delay in diagnosis.
On preparing girls for heavy menstrual bleeding
During the workshop, participants heard about the Irish experience of preparing girls for heavy menstrual bleeding. This preparation usually starts before the first period, at the time of diagnosis. During this time, parents receive further information on the impact of the bleeding disorder on their daughter and potential symptoms, including heavy menstrual bleeding. Then at around ten or eleven years, parents are encouraged to have a conversation with their daughter. After that, the haematologist remains on standby for any further questions.
Speakers noted that teenage girls going through their menarche for the first time could be very vulnerable as they feel shame and do not wish to talk or being exposed to healthcare professionals. It is challenging for families with no history of bleeding disorders to identify and swiftly deal with the problem. This led to discussions on how to reach out to these girls. Participants mentioned apps such as TikTok, and speakers noted an Irish initiative called ‘Know your flow‘ developed by haematologist Dr Michelle Lavin. Speakers noted the importance of guidelines for general practitioners to identify abnormal heavy menstrual bleeding to refer girls to haematologists or specialised gynaecologists quickly. Speakers also noted that tracking periods is a helpful tool to identify abnormal heavy periods. Because periods in girls with bleeding disorders are heavy but regular, it is important to keep track of the days of bleeding and the number of menstrual protections used. This information will facilitate the diagnosis.
On treatment for heavy menstrual bleeding
Speakers also discussed treatment for heavy menstrual bleeding, including tranexamic acid, which slows down the breakdown of blood clots and hormonal treatment. The inconvenience of tranexamic acid is that it may cause side effects and is in the form of big pills several times a day. This treatment is recommended for the days of heavier flow. On the other hand, some parents may be reluctant to use hormonal treatment because they think it may lead their daughters to be sexually active. After sixteen years of age, girls can also consider having an intrauterine device releasing progesterone.
Final remarks
Participants also noted the importance of monitoring iron levels, and these girls may experience low iron. These should be monitored regularly to prevent anaemia.
Finally, participants highlighted the importance of peer support through the patient organisation, the importance of a comprehensive care approach, the availability of psychological support, and the need to talk and not be ashamed.
Session on fertility:
Session moderated by Diana Lighezan and Katja Peltoniemi
Speakers:
- Anna Tollwé,
- Debra Pollard,
- Roseline d’Oiron, and
- Nikoletta Lancsès (excused).
Lack of information and research
During the session, it was established that there is a widespread lack of information on the impact of rare bleeding disorders on female fertility.
Some bleeding disorders (such as FXIII deficiency, afibrinogenemia, and all the very severe bleeding disorders VWD, particularly type 3, and platelet disorders such as Glanzmann thrombasthenia) mechanistic physiopathology has been understood to link the disorder and infertility. For some of these conditions, there are effective treatment protocols. However, for milder disorders, we do not understand or know whether there is an impact on fertility and more research is needed.
There is also a lack of treatment protocols for women affected by bleeding disorders and seeking fertility treatments. During the session, it was agreed that the haematologist should accompany these women throughout their treatment and link with non-specialist with healthcare professionals.
Psychological burden
During the webinar, it was established that many women with bleeding disorders seeking to start a family experience anxiety and psychological burden. This burden comes from several factors:
- Women may be told from a young age that they will have difficulty for the conception, pregnancy, delivery and post-partum phase,
- Young girl will be given hormonal therapy from a young age to manage their bleeding, and the nomenclature used to designate the medication will be ‘contraceptive,’ which will strengthen the idea of not becoming pregnant,
- Women with bleeding disorders experience heavier and more prolonged bleeds, and they are left wondering whether this has an impact on their reproductive organs,
- Women need to stop their medication for their bleeding symptoms (often hormonal therapy) to get pregnant, and they will experience anxiety of not knowing how to manage their bleeding symptoms,
- There is a lack of standardised guidelines to manage women seeking fertility treatments,
- Women seeking fertility treatment will need to deal with non-specialist healthcare professionals, and this can be distressing for fear of mismanagement and lack of support.
Outcomes:
- Need to establish whether women with bleeding disorders face additional issues concerning fertility compared to the general population,
- Need for more research into the link between bleeding disorders and fertility,
- Need for guidelines to manage bleeding during fertility treatment,
- Need to raise awareness amongst haematologists on the need to support women during fertility treatments,
- Monitor girls and women’s reproductive organs’ health from an early age about heavy menstrual bleeding and other symptoms such as endometriosis,
- Educate women and clinicians on the potential for bleeds during fertility treatment
Session on ageing
Session moderated by Ana Pastor
Speakers:
- Yannick Collé,
- Gaëtan Duport,
- Ozlem Turan, and
- Alison Dougall.
This session addressed how to prepare for healthy ageing with bleeding disorders. It explored the main challenges, symptoms, impacts and risks related to menopause and ageing.
First of all, participants were reminded of the distinction between menopause, which occurs when a woman doesn’t have her periods for 12 consecutive months and peri-menopause, which can last several years before menopause and can start as early as in the 30s.
Gynaecological perspective
- 80% of women experience symptoms during menopause,
- 45% describe these symptoms as distressing,
- But only 10% seek medical assistance!
Menopause affects different body systems. The ovaries no longer produce female hormones, like estrogen, causing distressing symptoms such as night sweats, hot flushes, foggy brain (memory problems and concentration difficulties) and sexual functions. All of these symptoms affect daily personal and work life.
Recommendations:
It is key to individualize treatment to each woman’s profile. For example, to manage menopause symptoms, women can take hormone replacement therapy (HRT), which replaces the decreasing natural hormones. However, there is some hesitancy to take these medicines, and this is because they can, in some cases they can increase the risk of breast cancer and thromboembolic events. In general, it is safe to use these medicines with age-related medication (such as medicines to control blood pressure).
The advice is to start HRT before menopause and review the treatment regimen and doses every five years.
It is critical to educate healthcare professionals about the needs of women with rare bleeding disorders.
Physiotherapist perspective
Ageing affects muscles and causes the loss of muscle mass and the reduction in joints and bone density. These events can lead to osteoarthritis, which can cause pain and be debilitating. In addition, women can experience an increase in fat tissue, which can negatively impact body perception and self-esteem.
Recommendations:
It is critical to regularly exercise and have an active lifestyle to prevent bone and joint damage. This included cardiovascular exercise, walking and weight training.
Currently, there is no data on joint health in women with RBD, which needs further assessment and research. Some studies suggest that women with rare bleeding disorders can also experience micro-bleeds in their joints and lead to joint damage. Physiotherapy is increasingly used and recommended for women with bleeding disorders. There is also a rising awareness about the need for women with bleeding disorders to be included in multidisciplinary care.
There needs to be more advocacy on this point.
Patient perspective
Women with bleeding disorders are often diagnosed later than their male counterparts. During the workshop, we heard from a patient’s experience where the patient had to stop her hormonal treatment, and this led to a worsening of her bleeding symptoms. As a result, this led to performing a hysterectomy to stop the bleeds. The speaker considered whether there was an alternative to such drastic measure or if surgery was unavoidable.
Recommendations:
There needs to be more education and recognition of women with bleeding disorders and specific management protocols.
Dentist perspective
Ageing causes:
- decay (i.e., gum reduction),
- teeth can chip and break (due to acidic diet or teeth grinding),
- colour change in teeth, which can affect our self-confidence.
Recommendations
All of the issues mentioned above can be addressed with simple interventions such as fluoride toothpaste for gum decay, a healthy diet, the use of night mouth guards to prevent grinding, and tooth whitening treatments to remove colour changes.
Tooth bones and gums need to be taken care of early in life. Therefore education starting at a young age is key.
Some age-related drugs can affect teeth (such as blood pressure, osteoporosis and depression treatments) and can lead as a side-effect to a dry mouth. Therefore, hydration through chewing gum or fluoride toothpaste should be considered.
Finally, the risk of oral cancer increases with age, and therefore teeth need to be checked more regularly.
Final considerations:
- Participants considered why there is less or no use of factor concentrate in women with RBD.
- Participants called for more discussions around nutrition and alternative treatment, and these need to be discussed with healthcare professionals as they may interact with some medications.
- Speakers noted that the bleeding phenotype could evolve with age and worsen with menopause.
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.
