What is VWD?
VWD (von Willebrand disease or von Willebrand disorder) is the most common inherited bleeding disorder worldwide. It affects both females and males equally.
It is a genetic condition and people are born with it. People with VWD have a mutation in the gene that makes a protein in the blood called von Willebrand factor (VWF). As a result, they do not have enough VWF or it doesn't work the way it should. This means they can have problems with controlling bleeding: it can take longer for blood to clot and for bleeding to stop.
How do you get VWD?
VWD is a genetic condition and it is usually passed from parent to child, in the same way as other genetic information such as the colour of their hair or their eyes.
Click here for more information about VWD and inheritance
Date last reviewed: 22 September 2020
What are the symptoms of VWD in females?
The symptoms of VWD vary greatly from person to person. Bleeding often involves the mucous membranes (the delicate tissues that line body passages), such as the nose, mouth, uterus, vagina, stomach and the intestines.
The more common symptoms of VWD (von Willebrand disease or von Willebrand disorder) in females are:
- Frequent nosebleeds, or nosebleeds that are hard to stop
- Bruising easily
- Very heavy periods, or periods that last more than 8 days
- Bleeding for a long time from small cuts
- Bleeding from the gums, usually after trauma/injury
- Heavy bleeding that lasts longer than expected after childbirth (particularly when bleeding increases 3 days or more after giving birth)
- Bleeding that continues for a long time after injury, medical procedures, surgery or dental work.
Less common symptoms that older women may experience are:
- Blood in faeces (poo) due to bleeding in the intestines or stomach
- Blood in urine (wee) due to bleeding in the kidneys or bladder.
Females with severe forms of VWD, particularly type 3 VWD, may also have other bleeding problems similar to severe haemophilia, such as:
- Bleeding episodes that happen for no obvious reason
- Bleeding into joints and muscles which cause swelling and pain.
Heavy menstrual bleeding (also called abnormal uterine bleeding or menorrhagia) in women and girls with VWD may involve:
- Heavy menstrual periods (eg, soaking through a tampon and pad around two hourly, or needing to change during the night)
- Menstrual bleeding for longer than normal (eg, longer than 8 days)
- Bleeding with clots bigger than a 50 cent piece in size.
Heavy menstrual bleeding can lead to anaemia (low red blood cell count/low blood iron levels), with symptoms of fatigue, paleness, lack of energy and shortness of breath.
Some women and girls with VWD also experience:
- Pain during their menstrual periods (dysmenorrhoea)
- Abdominal pain and sometimes bleeding during ovulation (when an egg is released from the ovaries, around the middle of the menstrual cycle).
Although these can be symptoms of VWD, they can also be symptoms of a gynaecological disorder, so it is important to consult a gynaecologist.
If you are a woman or girl with VWD, a holistic or comprehensive care approach to your health care can help you to achieve better health and quality of life. Specialist gynaecological care over your lifetime is important to manage any gynaecological issues that occur. These may not be related to VWD, but in some cases VWD may make the bleeding problems worse.
Ideally your medical care team should work together on your health care and should include:
Date last reviewed: September 2020
- A gynaecologist
- A haematologist specialising in bleeding disorders
- A GP or paediatrician or obstetrician, if relevant at the time
How is VWD diagnosed in females?
VWD can be difficult to diagnose. Repeated testing may be needed to confirm the diagnosis. Common experiences such as stress, exercise, pregnancy and hormonal levels can affect the results. Understanding the laboratory test results is complex and needs to be done by specialists with experience in VWD.
Many women and girls' symptoms are mild and they may not be diagnosed until they have a major bleeding episode, for example, after childbirth, surgery or an injury. However, if they have a severe form of VWD, they will usually have major bleeding problems as a baby or small child and will often be diagnosed within their first year of life.
Diagnosing VWD involves:
Click here for more information on diagnosing VWD
- A personal history of abnormal bleeding or bruising from mucous membranes (such as the nose, mouth, uterus, vagina, stomach and intestines) or skin after injury, trauma or surgery and
- A family history of bleeding problems and
- Specialised laboratory test results for VWD.
Different types of VWD
There are three main types of von Willebrand disease. Bleeding symptoms can vary from person to person within each type.
Click here for more information on VWD types
Date last reviewed: 22 September 2020
How is VWD treated?
With diagnosis and appropriate treatment, bleeding problems with VWD can be dramatically reduced and sometimes even eliminated.
Women who have menorrhagia or abnormal vaginal bleeding need a full gynaecologic consultation before treatment to understand any gynaecological issues.
Bleeding disorder treatments for women and girls with VWD include:
- Tranexamic acid and aminocaproic acid, antifibrinolytic drugs which can reduce bleeding by slowing the breakdown of blood clots
- Oral contraceptives (“the Pill”) combining the hormones oestrogen and progesterone. The hormones increase VWF and factor VIII in the blood and reduce menstrual blood loss. Although it also has the effect of preventing pregnancy, in this case the treatment’s aim is to manage VWD symptoms and so it may also be suitable for teenage girls who are not sexually active and women who are not specifically seeking birth control
- An intrauterine device (IUD), releasing the hormone progesteronewhich reduces bleeding
- Desmopressin (DDAVP), a synthetic hormone which stimulates the body to release VWF and factor VIII
- Clotting factor concentrate made with von Willebrand factor (VWF) and factor VIII (FVIII), which replaces the missing VWF and FVIII in the blood and helps blood to clot. This clotting factor concentrate is made from the plasma (pale yellow fluid part) in human blood and is produced from blood donations. This clotting factor concentrate is used in uncommon circumstances, such as when other treatments have not been effective, and when the woman is trying to become pregnant, or when it is likely the person will need treatment for more than 2-3 days, and in discussion with the specialist haematologist at the Haemophilia Centre. The treatment is infused (injected) into a vein in the arm
- Iron supplements for anaemia.
Women with bleeding disorders should avoid taking non-steroidal anti-inflammatory drugs for period pain, unless prescribed by a doctor with expertise in VWD (ie, Naprosyn/naproxen, ibuprofen, etc – these have many brand names; ask your local pharmacist to check for you). These medicines can interfere with the way platelets promote clotting and cause bleeding to go on for longer.
Generally, treatment options with medication will be exhausted before considering surgery such as hysterectomy (surgical removal of the uterus) or procedures such as endometrial ablation, where the lining of the uterus is destroyed to reduce menstrual blood loss. Surgery and some procedures have their own risk of bleeding complications for women with VWD.
However, some women with VWD may need to have gynaecological surgery or procedures for other reasons. If this happens, it is important that this is managed in a team, with discussion between the woman, the Haemophilia Centre and the gynaecologist and/or surgeon.
Date last reviewed: September 2019
How might things change over a lifetime?
Puberty: menstrual bleeding can be especially heavy when a girl first starts having periods. When there is a family history of VWD or it is known that she has VWD, a girl should be followed closely by her medical team during puberty and may need treatment if she has heavy bleeding.
Sexual intercourse: some women with VWD may experience bleeding if there are small tears in their vagina after sexual intercourse. This can happen during their first sexual experience when the hymen is broken. It can also occur after childbirth and menopause when the vaginal wall may be thinner and dryer due to a drop in oestrogen levels - oestrogen creams for the vaginal wall and/or lubricants can help with this.
Pregnancy and childbirth: most women with VWD do not have a problem with delivering a healthy baby. Pregnancy can cause blood levels of VWF to increase, decreasing the likelihood of bleeding complications during pregnancy and delivery. However, this needs to be monitored as women with VWD can have heavy bleeding for an extended period after delivery when their factor levels return to their usual levels.
To minimise the chances of complications:
- Discuss VWD with a genetic counsellor, your haematologist and an obstetrician before you become pregnant
- Before you have any invasive procedure, such as amniocentesis, ask your haematologist if you are at risk of bleeding and whether anything needs to be done to prevent it
- During your third trimester, you should have blood tests to measure VWF to help plan for delivery and for any treatments to prevent potential post-delivery bleeding
- Discuss your choices for anaesthesia, especially an epidural, with your haematologist, obstetrician, and if possible, your anaesthetist
- Unless prenatal testing has shown the opposite, it should be assumed that the baby may have VWD and delivery methods should be as gentle as possible. A caesarean section is not usually required.
For information on how VWD is passed on from parent to child, see the inheritance section.
Menopause: When a woman begins menopause, her body’s erratic hormone regulation can increase her risk of unpredictable and heavy menstrual bleeding. However, for some women with VWD, levels of VWF rise as they age and normalise so that bleeding problems reduce. Keeping a close relationship with her gynaecologist in the years before menopause will help a woman with VWD be prepared to manage any problems that might occur.
Date last reviewed: September 2019
Australian Bleeding Disorders Registry. Annual report, 2018-19. Canberra: National Blood Authority, 2020 [PDF 2.2MB]
Australian Bleeding Disorders Registry. Addendum to Annual report 2015-16. Canberra: National Blood Authority, 2017.
Haemophilia Foundation Australia. A guide for people living with von Willebrand disorder. Melbourne: HFA, 2010.
Lillicrap D, James P. Von Willebrand Disease: an introduction for the primary care physician. Treatment of Hemophilia, No 47. Montreal: World Federation of Hemophilia, 2009.
National Blood Authority; Australian Haemophilia Centre Directors’ Organisation. Evidence-based clinical practice guidelines for the use of recombinant and plasma-derived FVIII and FIX products. Canberra: Australian Health Ministers’ Advisory Council, June 2006 National Heart, Lung and Blood Institute. The diagnosis, evaluation, and management of von Willebrand Disease. Washington, DC: National Institutes of Health, US Department of Health and Human Services, December 2007
Your guide to von Willebrand Disease. Washington, DC: National Institutes of Health, US Department of Health and Human Services, January 2008
Some information and images adapted from:
Page D. All about von Willebrand Disease… for people with von Willebrand Disease and their families. 2nd ed. Montreal: Canadian Hemophilia Society, 2007.
Many thanks to the HFA VWD Consumer and Health Professional Review Panels for their suggestions on topics and content to include.
This information was reviewed by Penny McCarthy, Megan Walsh and Salena Griffin from Australian Haemophilia Nurses’ Group; Leonie Mudge, Peter VanderLinden and Sharon Hawkins from Australia/New Zealand Haemophilia Social Workers’ and Counsellors’ Group; Wendy Poulsen and Matthew Stewart from Australian and New Zealand Physiotherapy Haemophilia Group; Dr James Daly, Dr John Rowell and Dr Simon McCrae from Australian Haemophilia Centre Directors’ Organisation; and VWD community representatives from the HFA VWD Consumer Review Panel.
Date last reviewed: 24 December 2020