In this section, we will give a brief overview of:
It is a common misunderstanding that bleeding disorders affect men exclusively; however, women also suffer from bleeding disorders. Some women have similar symptoms to men and bleeds that can be amplified during menstruation and childbirth. In contrast, others carry the gene and do not experience any symptoms, although, as men, they can pass on the gene to their children.
Moreover, some women with a defective gene for haemophilia can have similar symptoms to severe haemophilia even though their clotting factor levels are above 40 per cent. In this case, this individual will be called asymptomatic carrier.
Besides the ‘regular’ bleeds that are experienced by men with a bleeding disorder, women experience other symptoms. Women may experience heavier and longer menstrual flows, which besides causing the iron deficiency, weakness and fatigue can also be debilitating. Women with bleeding disorders are also more likely to experience more significant pain during their menstrual bleeding, and they may also experience a small amount of internal bleeding during ovulation, which can cause abdominal and pelvic pain. Moreover, during ovulation, women with bleeding disorders are also more at risk of developing haemorrhagic ovarian cysts. The development of ovarian cysts in healthy women is a common and benign phenomenon. However, in women with bleeding disorders, they put the individual at greater risk of internal bleeding. This bleeding can be severe or even life-threatening, especially in carriers with very low clotting factor levels and may require urgent medical attention.
Women with bleeding disorders also have endometriosis, a painful condition in which endometrial tissue, the tissue that lines the uterus, forms in the abdomen or other parts of the body. Although we do not yet understand the cause of endometriosis, women who experience heavy menstrual bleeding are more at risk of developing this condition.
Finally, during perimenopause, the three- to the ten-year period before menopause when hormones are ‘in transition,’ heavy and irregular menstrual bleeding occurs more commonly. This may lead to gynaecological conditions (such as fibroids, polyps, etc.) and women with bleeding disorders and carriers of haemophilia are at risk of more severe bleeding symptoms during this phase of their life and may require treatment.
Because of the common misconception that bleeding disorders only affect men, many women who suffer from bleeding disorders remain undiagnosed. Both patients and physicians in many European countries have been developing programmes to raise awareness so that these women can be properly diagnosed and cared for. You can find some examples of these programmes here:
Family planning
Women with clotting factor deficiencies and families with a history of bleeding disorders should receive genetic counselling about the risks of having an affected child well in advance of any planned pregnancies and should see an obstetrician as soon as they suspect that they are pregnant. The obstetrician should work closely with the staff of the haemophilia/bleeding disorder treatment centre to provide the best care during pregnancy and childbirth and to minimize the potential complications for both the mother and the newborn.
Haemophilia and other bleeding disorders can be diagnosed early on in the pregnancy. Several testing techniques are available to families, which include:
- Ultrasound
- Chorionic Villus Sampling
- Amniocentesis
- Foetal blood sampling
- Maternal blood test
Prenatal testing can help families to make informed decisions and preparations for pregnancy and delivery. Today families at risk of giving birth to children with genetic defects can also make use of pre-implantation genetic diagnosis (PGD), a technique that involves testing cell(s) from embryos created outside the body by in vitro fertilisation (IVF) for a genetic disorder. Tests are carried out for the specific disorder that the embryos are known to be at significant risk of inheriting. Unaffected embryos are selected for transfer to the uterus in the hope that a normal birth will ensue.
Women with certain factor deficiencies (such as factor XIII deficiency and afibrinogenemia) may be at greater risk of miscarriage and placental abruption (premature separation of the placenta from the uterus that disrupts the flow of blood and oxygen to the foetus). Therefore, these women require treatment throughout the pregnancy to prevent these complications.
The main risk related to pregnancy is postpartum haemorrhage. All bleeding disorders are associated with a greater risk of increased bleeding after delivery. The risk and the severity of the bleeding can be reduced with appropriate treatment. This treatment is different for each woman and depends on her personal and family history of bleeding symptoms, the severity of the factor deficiency, and the mode of delivery (vaginal birth vs caesarean section). Factor replacement may be necessary in some cases.
Resources
For further information about women and bleeding disorders, we recommend that you consult the following websites:
Resources to assess heavy menstrual bleeding
Heavy menstrual bleeding is one of the primary symptoms of women affected by rare bleeding disorders and it can have a severe impact on daily quality of life. However, many women are still unaware of how heavy their periods are and whether they should seek medical help. Here below we give you some useful resources to assess your menstrual bleeding:
EHC 7-2-1 rule campaign
https://www.ehc.eu/ehc-womens-committee-marks-2024-international-womens-day/
This meticulously crafted graphic illustrates the 7-2-1 rule, a vital guideline for recognising signs of heavy periods. Download it in 10 languages or download the customisable blank template from our website via the above link.
Know your flow campaign
This is an initiative led and developed by the Irish Centre for Vascular Biology and focused on improving care for people with bleeding disorders. Researches identified a common pattern; women who were later diagnosed with bleeding disorders often did not recognise their periods as being heavy. The aim of the campaign is to improve recognition of heavy bleeding and diagnosis of bleeding disorders.
Blood Sisterhood App
The Haemophilia Foundation of America has developed an app to track monthly menstrual cycles, log symptoms and record treatment used. This app has been developed specifically with women with rare bleeding disorders in mind. It acts as a sort of electronic diary to keep track of the frequency and intensity of your periods and will help you talk about your cycle and identify issues with your consulting clinician. It can be downloaded for free either on the App Store or on Google Play.
Self-BAT
The Self-BAT (self-administered bleeding assessment tool) is a scientifically validated scoring tool developed by Dr Paul James and targeted at individuals who are concerned about bleeding. Taking the test will help you better understand whether current or previous, bleeding episodes are normal or abnormal. The test is available in English and French.
Swedish Haemophilia Society Self-Assessment Chart
The Swedish Haemophilia Society has developed a self-assessment chart to help you to keep track of the frequency and intensity of your menstrual flow. This will help you in preparing your doctor visit and assessing whether you suffer from heavy menstrual bleeding. Consult the chart here.
Webinar on the management of heavy menstrual bleeding in women with bleeding disorders
Treatment options for women with bleeding disorders
The treatment type you will be offered will depend on your bleeding disorder, its severity and your bleeding phenotype (that is your bleeding pattern). It will also depend on whether the treatment is given to prevent recurring bleeds or to prevent or treat bleeds in a particular situation like a surgery, childbirth or a severe bleed (breakthrough bleed).
This article and the treatment options listed below are meant to be informative, and we recommend that readers check-in with a specialised healthcare provider to discuss treatment options adapted to them.
In general, three categories of treatments are used for women with bleeding disorders: hormonal therapy, haemostatic treatment and surgical procedures. In addition to these, your doctor may also prescribe iron supplements, either as a pill or as an injection, for more severe cases. Finally, some women affected by rare bleeding disorders may also require blood transfusions, although this is rarer.
Hormone therapy
When we talk about hormone therapy, we most often refer to oral contraceptives commonly known as the ‘the pill.’
The pill raises the levels of factors II, VII, VIII, X and von Willebrand factor in the blood. For many women with bleeding disorders and who suffer from menorrhagia, this hormone therapy alone is effective in reducing bleeding to normal.
This hormone therapy will not improve factor levels for women with:
- deficiencies in factors I, V, IX, XI and XIII, and
- Type 2 VWD (which is not a problem of quantity of VWF but rather the way it works).
However, oral contraceptives can be helpful even for these women. They regulate the menstrual periods and reduce the flow of blood[1].
Some women may experience bleeding during their ovulation (approximately mid-cycle) and it is important to talk about this to your healthcare provider because there are types of hormonal treatment that will prevent you from ovulating altogether during your cycle and avoiding this type of bleed.
As many women will already know there are a variety of hormonal contraceptives in the forms of pills, vaginal rings, transdermal patches and intra-uterine devices. The choice of this hormonal treatment should be in line with your medical needs and lifestyle.
This hormonal therapy may be prescribed to young girls even before their first period to avoid heavy bleeding. During our webinar we discussed the apprehension that many parents may have with their pre-teen daughters being prescribed what is seen as primarily a contraceptive, implying that this will speed up their daughters’ sexual maturity. It is important that parents and girls realise these hormonal treatments are medical treatments to manage bleeding disorder and prevent heavy menstrual bleeds. Communication between the patient, the parents and the healthcare professional will be key.
Other hormone therapies may be prescribed when oral contraceptives do not work well. These include progesterone. They work by thickening the lining of the uterus. This makes it less prone to bleed. However, these products cannot be taken for long periods of time.
[1] Source: Canadian Haemophilia Society
Haemostatic treatments
These are a group of treatments that will correct defects in a person’s coagulation system. For women with bleeding disorders the most commonly used are:
- Desmopressin,
- Antifibrinolytic agents (like tranexamic acid),
- Clotting factor concentrates.
Desmopressin is a synthetic drug, which is a copy of a natural hormone. It acts by releasing von Willebrand Factor (VWF) stored in the lining of the blood vessels. The increased VWF, in turn, transports extra factor VIII. Desmopressin is not made from blood.
Desmopressin is the treatment of choice for Type 1 VWD, haemophilia A carriers and most platelet function disorders. It is sometimes effective in Type 2A VWD.
Desmopressin can be taken in three different ways:
- It can be injected into a vein.
- It can be injected under the skin.
- It can be taken by nasal spray.
Desmopressin is effective for almost all people with Type 1 VWD. However, different people respond to desmopressin in different ways. Therefore, a doctor needs to do tests to find out each individual’s response to the drug. Ideally, these tests are done before any urgent need for the drug, such as surgery.
Since desmopressin acts by releasing VWF stored in the body, you cannot ‘go to the well’ too often. A sufficient amount of time, usually 24 hours, must elapse between doses of desmopressin to allow the body to rebuild its stores.
In major surgery, desmopressin alone may not be enough to control bleeding. In such a case, a person should also receive factor concentrates. (See Clotting factor concentrates)
Desmopressin is of no help to women with:
- Type 2 VWD (except, in some cases, Type 2A)
- Type 3 VWD
- Hemophilia B (factor IX deficiency)
- Glanzmann Thrombasthenia (a type of platelet function disorder).
Source: Canadian Hemophilia Society
Antifibrinolytic agents, like tranexamic acid, are medicines that help to hold a clot in place once it has formed. They act by stopping the activity of an enzyme, called plasmin, which dissolves blood clots.
They do not help to actually form a clot. This means they cannot be used instead of desmopressin, VWF concentrate or factor concentrates.
They can be used to hold a clot in place in mucous membranes such as:
- the inside of the mouth,
- the inside of the nose,
- inside the intestines,
- inside the uterus (the womb).
These medicines have proven very useful for women with bleeding disorders. They are used:
- before dental work
- when a person has a mouth, nose and minor intestinal bleeding
- for women with heavy and /or prolonged menstrual bleeding.
These medicines can even be combined with the use of oral contraceptives for women who do not respond to desmopressin.
Source: Canadian Haemophilia Society
Factor concentrates exist for many of the bleeding disorders that affect women. They replace the missing factor. Factor concentrates can be used:
- when desmopressin, hormone therapy and antifibrinolytic drugs are not effective
- for surgery, or
- after a serious accidental injury.
Factor concentrates are injected into a vein. They can be administered at a clinic, doctor’s office or emergency department. Many people learn to inject them at home.
Source: Canadian Haemophilia Society
Surgical options
For some women, the medical treatments described above will not work. Heavy, prolonged bleeding during the menstrual cycle will continue. For these women, gynaecological procedures, including surgery, may be considered. These include both options that will spare fertility, such as intrauterine balloon tamponade and suction evacuation or curettage, but also invasive procedures that can cause infertility like endometrial ablation, uterine artery embolization, hysterectomy, laparoscopy or oophorectomy.
As most of these surgeries may impact your ability to have a family, it is crucial to communicate to your healthcare provider and discuss with them your desire for a family, if this is important to you.
The purpose of this surgery is to destroy the lining of the uterus. This is the endometrial tissue, which bleeds so much during menstruation. The operation is done through the vagina and no surgical cutting is needed.
Source: Canadian Hemophilia Society
The purpose of this surgery is to remove the uterus so that menstrual bleeding stops once and for all. This is major surgery and will result in infertility.
Source: Canadian Hemophilia Society
This is a procedure that is done to reduce post-partum bleeding. It consists of a medical device in the shape of a balloon that is inserted (deflated) in the uterus and then filled with saline. This device puts pressure on the uterine wall to stop bleeding. This can be used in conjunction with other antifibrinolytic agents and coagulation factor concentrate.
The purpose of this surgery is to remove endometrial tissue that has formed outside the uterus. This tissue bleeds during menstruation and can cause pain in the pelvis and abdomen. This operation can reduce pain and bleeding in women who do not respond to hormone therapy and other medical treatments.
Source: Canadian Hemophilia Society
This is the removal of the ovaries, and its purpose is to stop bleeding from the ovaries. This is a major surgery that will cause infertility and women will need to take hormones until the age of menopause.
Source: Canadian Hemophilia Society
NOT RECOMMENDED IN WOMEN WITH BLEEDING DISORDERS
The purpose of this surgery is to scrape and clean the lining of the uterus. This is done to diagnose another problem. However, this is not effective in reducing bleeding. It may cause additional bleeding. Also, it may remove any existing platelet plugs and fibrin clots and make the bleeding worse.
Source: Canadian Hemophilia Society
This is a minimally invasive procedure for uterine fibroids, which may cause increased bleeding. The goal is to block the fibroid blood vessels, to stop the blood supply to the fibroid and cause them to shrink and die.
Further reading on treatment options for women with bleeding disorders:
There are many haemophilia societies that provide detailed information on women with bleeding disorders such as:
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.
