What is von Willebrand disease?
Von Willebrand disease is a bleeding disorder. It’s caused by a deficiency
of von Willebrand factor (VWF). This is a type of protein that helps your blood
to clot. Von Willebrand is different from hemophilia, another type of bleeding
Bleeding happens when one of your blood vessels breaks. Platelets
are a type of cell that circulates in your blood and clumps together to plug
broken blood vessels and stop bleeding. VWF is a protein that
helps platelets clump together, or clot. If your levels of functional VWF are
low, your platelets won’t be able to clot properly. This leads to prolonged
According to the Centers for Disease Control and Prevention, von
Willebrand disease affects up to 1 percent of the general population in the
How many types of von Willebrand disease are there?
Three main types of von Willebrand disease exist:
Type 1 is the most common type of von Willebrand disease. It causes
lower-than-normal levels of VWF to occur in your body. You still have small
amounts of VWF in your body to help clot blood. You’ll likely experience mild
bleeding problems but be able to live a normal life.
If you have type 2 von Willebrand disease, you have normal levels of VWF but
it won’t work properly due to structural and functional defects. Type 2 is
divided into subtypes, including types:
Type 3 is the most dangerous type of von Willebrand disease. If you have
this type, your body won’t produce any VWF. As a result, your platelets won’t
be able to clot. This will put you at risk of severe bleeding that’s difficult
Symptoms of von Willebrand disease
If you have von Willebrand disease, your symptoms will vary depending on
which type of the disease you have. The most common symptoms that occur in all
three types include:
- easy bruising
- excessive nose bleeds
- bleeding from your gums
- abnormally heavy bleeding
Type 3 is the most severe form of the condition. If you have this type, you
have no VWF in your body. This makes episodes of bleeding difficult to control.
It also raises your risk of internal bleeding, including bleeding in your
joints and gastrointestinal system.
Men and women develop von Willebrand disease at the same rate. But women are
more likely to experience symptoms and complications due to the increased risk
of bleeding during menstruation, pregnancy, and childbirth.
What causes von Willebrand disease?
A genetic mutation causes von Willebrand disease. The type of von Willebrand
disease that you have depends on whether one or both of your parents have
passed a mutated gene on to you. For example, you can only develop type 3 Von
Willebrand if you’ve inherited a mutated gene from both of your parents. If
you’ve only inherited one copy of the mutated gene, you’ll develop type 1 or 2
von Willebrand disease.
How is von Willebrand disease diagnosed?
Your doctor will ask you questions about your personal and
family history of abnormal bruising and bleeding. Type 3 tends to be the
easiest to diagnose. If you have it, you’ll likely have a history of severe
bleeding starting early in life.
Along with taking a detailed medical history, your doctor may also use
laboratory tests to check for abnormalities in your VWF levels and function.
They may also check for abnormalities in clotting factor VIII, which can cause
hemophilia. Your doctor can also use blood tests to learn how well your
Your doctor will need to collect a sample of your blood to conduct these
tests. Then, they’ll send it to a laboratory for testing. Because of the
specialized nature of these tests, it may take up to two or three weeks to
receive your results.
How is von Willebrand disease treated?
If you have von Willebrand disease, your treatment plan will vary, depending
on the type of condition you have. Your doctor may recommend several different
Your doctor may prescribe desmopressin (DDAVP), which is a drug recommended
for types 1 and 2A. DDAVP stimulates the release of VWF from your body’s cells.
Common side effects include a headache, low blood pressure, and fast heart
Your doctor may also recommend replacement therapy, using Humate-P or
Alphanate Solvent Detergent/Heat Treated (SD/HT). These are two types of
biologics, or genetically engineered proteins. They’re developed from human
plasma. They can help replace the VWF that’s absent or working improperly in
These replacement therapies aren’t identical and you shouldn’t use them
interchangeably. Your doctor may prescribe Humate-P if you have type 2 von
Willebrand disease and are unable to tolerate DDAVP. They may also prescribe it
if you have a severe case of type 3 von Willebrand disease.
Common side effects of replacement therapy with Humate-P and Alphanate SD/HT
include chest tightness, rash, and swelling.
To treat minor bleeding from small capillaries or veins, your doctor may
recommend applying Thrombin-JMI topically. They may also apply Tisseel VH
topically after you undergo surgery, but it won’t stop heavy bleeding.
Aminocaproic acid and tranexamic acid are drugs that help steady clots
formed by platelets. Doctors often prescribe them to people who are undergoing
invasive surgery. Your doctor may also prescribe them if you have type 1 von
Willebrand disease. Common side effects include nausea, vomiting, and clot
Drugs to avoid
If you have any form of Von Willebrand disease, it’s important to avoid
drugs that can increase your risk of bleeding and complications. For example, avoid
aspirin and nonsteroidal anti-inflammatory drugs, such as ibuprofen
is the long-term outlook for people with von Willebrand disease?
Most people who have type 1 von Willebrand disease are able
to live normal lives with only mild bleeding issues. If you have type
2, you’re at an increased risk of experiencing mild to moderate
bleeding and complications. You may experience worse bleeding in the case of
infection, surgery, or pregnancy. If you have type 3, you’re
at risk of severe bleeding, as well as internal bleeding.
No matter what type of von Willebrand disease you have, you should let your
healthcare providers know about it, including your dentist. They may need to
adjust their procedures to lower your risk of bleeding. You should also let
trusted family members and friends know about your condition in case you have
an unexpected accident or need life-saving surgery. They can share important
information about your condition with your healthcare providers.