Common questions about von Willebrand disease (VWD)
What is von Willebrand disease (VWD)?
Von Willebrand disease (VWD – also known as von Willebrand disorder) is a bleeding disorder in which people do not have enough of a protein called von Willebrand factor in their blood or the von Willebrand factor does not work properly. Because of these problems, it takes longer for bleeding episodes to stop.
Von Willebrand disease occurs in both males and females. It occurs when there is a change in a person’s von Willebrand factor (VWF) gene affecting their blood clotting and causing VWD. VWD is usually inherited and a person is born with it.
Bleeding from VWD often involves the mucous membranes, the delicate tissues that line body passages, such as the nose, mouth, uterus, vagina, stomach and intestines.
VWD symptoms vary, even in the same family, and may include frequent nose and gum bleeds, easy bruising, heavy menstrual periods, prolonged bleeding after injury and trauma, childbirth, dental extractions and surgery. When people have the severe form of VWD, they may also have bleeding into joints and muscles.
Many people have a mild form with few symptoms. However, with all forms of VWD there can be bleeding problems. Some people with a mild form of VWD bleed quite often, eg with nosebleeds, bruising and heavy periods. A smaller number of people have the severe form of VWD and as mentioned above, may also experience joint and muscle bleeds, similar to haemophilia.
Current information about von Willebrand disease, statistics and some brief information about treatments is available in the Australian Bleeding Disorders Registry (ABDR) Annual Report.
Read the HFA booklet A guide for people living with von Willebrand disorder for more information on VWD, inheritance, treatment, living well and special issues for women and girls
How common is VWD?
VWD is the most common type of bleeding disorder worldwide.
Most people with VWD have a mild form with few symptoms. In comparison to the number of people with the mild form of VWD, the form of VWD causing moderate bleeding is uncommon, and the severe form is rare.
If VWD is so common, why haven’t I heard of it?
More than 2,500 people have been diagnosed with VWD in Australia but many others are thought to be undiagnosed.
Because most people with VWD have the mild form, they may not be aware they have VWD until they have a serious injury, surgery or dental extractions, or serious bleeding after childbirth. Heavy menstrual bleeding is a common symptom in females but may not be recognised as VWD until other bleeding symptoms are investigated.
VWD can be difficult to diagnose and repeated testing may be needed to confirm the diagnosis. Understanding the laboratory tests is complex and needs to be done by specialists with experience in VWD.
Is von Willebrand disease only an inherited condition?
There is not always a family history. Some people have VWD because there was a mutation or change in the von Willebrand factor gene during their foetal stage.
When this happens, the gene change can be passed on from the person to their children like someone who has inherited the condition. VWD can also be acquired rather than inherited, but this is very rare.
Can women get VWD?
Yes. Because the VWD gene is linked to an autosome (a chromosome that is not a sex chromosome), not an X or Y chromosome, both females AND males can carry and be affected by VWD.
For more information about VWD inheritance, click here.
I’m a woman with VWD. Can I have children?
Yes, you can have children. For more information on go to the Women with Bleeding Disorders section.
What is von Willebrand factor and what does it do?
When blood vessels are damaged, platelets (tiny particles in the blood) clump together at the site of injury; von Willebrand factor is like glue that makes these platelets stick to the areas of blood vessel that are damaged. Von Willebrand factor also acts as a protector of clotting factor VIII. This means some people with von Willebrand disease may also have a lower amount of factor VIII.
What are the types of von Willebrand disease?
VWD is divided into “types” according to whether a person has a low amount of von Willebrand factor or has a type of von Willebrand factor that does not work properly, or both. Knowing the type of von Willebrand disease helps the doctor decide what kind of treatment would be best for that person.
- Type 1: This is the most common form of von Willebrand disease. A person with type 1 von Willebrand disease has low levels of von Willebrand factor (VWF) but the von Willebrand factor present functions normally. Symptoms are usually mild. However, some people with type 1 can have very low levels of VWF and have severe bleeding problems. People with type 1 make up more than 80% of cases of this disorder.
- Type 2: A person with type 2 often has a normal amount of von Willebrand factor in their blood but has an irregularity in the von Willebrand factor they produce. This type is divided into sub-types 2A, 2B, 2M and 2N. Certain sub-types might require different treatment, which makes knowing the exact type of VWD you have very important.
- Type 3 is very rare. People with type 3 VWD have very little or no VWF in their blood and also have low factor VIII (8) levels. Bleeding can occur often, be more severe and can also include joint and muscle bleeding.
Nevertheless, it is important to remember that there can be bleeding problems with all forms of VWD and any bleeding that occurs with VWD needs to be assessed and treated.
Why is the condition called VWD?
VWD was discovered in 1925 by, and named after, the Finnish doctor Erik von Willebrand. Professor von Willebrand described this new type of bleeding disorder after observing families living on the Aaland Islands between Sweden and Finland who had bleeding problems. It is also known as von Willebrand disorder.
How is VWD different from haemophilia?
VWD affects both males and females in equal numbers whereas most people diagnosed with haemophilia are males. Most people with VWD do not have bleeding symptoms very often, except during or after surgery or accidents, although females can have heavy menstrual bleeding and prolonged and very heavy bleeding after childbirth. This means that more females than males visit a doctor to be assessed for treatment and at an earlier age.
Bleeding symptoms from VWD can vary from person to person, even in the same family. Bleeding from VWD often involves the mucous membranes, the delicate tissues that line body passages, such as the nose, mouth, uterus, vagina, stomach and intestines. People with haemophilia bleed mainly into joints and muscles.
Does everyone with the gene change causing VWD have bleeding symptoms?
No. Many people who have the gene change causing VWD carry it without symptoms. In fact, they may not even realise they have it. However, the condition can still be passed on to children.
Do I have VWD?
A few bruises do not necessarily indicate the presence of VWD. However, if you think you may have VWD, talk to your doctor about your concerns.
What about surgery?
Surgery, including tooth extractions, can cause bleeding for people with VWD. It is important you tell your surgeon you have VWD well before your surgery date so they can work with your haematologist and other key staff to ensure you receive the best possible treatment and care.
Source: ABDR Annual report 2021-22