IN BRIEF

YO U R G U I D E TO
von Willebrand Disease

If you often have large, lumpy bruises; lead to heavy, hard-to-stop bleeding after
frequent or difficult-to-stop nosebleeds; an injury. The bleeding from VWD can
bleed a lot after a fairly minor cut; or are lead to damage of your internal organs or
a woman who has very heavy or long even be life threatening, but this is rare.
menstrual periods, you should ask your
In VWD, you either have low levels of
doctor about an inherited bleeding
a certain protein in your blood or the
disorder called von Willebrand disease
protein doesn't work the way it should.
(VWD). Named for Dr. Erik von
The protein is called von Willebrand
Willebrand, a Finnish doctor who first
factor (VWF). It is made in the walls
described the condition in 1926, VWD
of your blood vessels and released into
affects your blood's ability to clot and can
your blood.
 Normally, when one of your blood vessels • In type 3 VWD, you usually have no
is injured, you start to bleed. As soon as von Willebrand factor and low levels of
this happens, small cells in your blood that factor VIII. Type 3 is the most serious
are called platelets clump together to plug form of VWD, but very rare.
the hole in the blood vessel and stop the
Von Willebrand disease cannot be cured,
bleeding. Von Willebrand factor acts like
but it can be treated. Early diagnosis is
glue to help the platelets stick together and
important, and with the right treatment
form a blood clot.
plan, even people with type 3 VWD can be
Von Willebrand factor also carries with it helped to live active lives.
clotting factor VIII (8), another important
protein that helps your blood clot. Factor
VIII is the protein that is inactive or What Causes von Willebrand disease?
missing in hemophilia, another clotting
disorder. Von Willebrand disease is almost always
inherited. Your parents pass the gene for
Von Willebrand disease, like hemophilia, is the disease on to you. You can develop
an inherited bleeding disorder, but VWD types 1 or 2 VWD when only one of your
is more common and usually milder. In parents carries the gene for it. You usually
fact, VWD is the most common of all the inherit type 3 VWD only if both of your
inherited bleeding disorders. It occurs in parents pass the gene on to you. Your
about 1 out of every 100 to 1,000 people. symptoms may be different from your
It also affects both males and females, parent’s.
while hemophilia mainly affects males.
Some people develop a form of the disease
There are three major types of von later in life as a result of other medical
Willebrand disease. conditions. This is called Acquired von
Willebrand Syndrome (AVWS).
• In type 1 VWD, you have a low level of
the von Willebrand factor, and you may
have lower levels of factor VIII than
What Are the Signs and Symptoms of
normal. This is the mildest and most
von Willebrand Disease?
common form of VWD. About 3 out of
every 4 people with VWD have type 1 The signs and symptoms of VWD depend
VWD. on the type and severity of the disease.
• In type 2 VWD, the von Willebrand Many people have such mild symptoms
factor does not work the way it's that they may not know they have the dis-
supposed to. Type 2 VWD is divided order. Some people have the gene for the
into subtypes 2A, 2B, 2M, and 2N. disease but don't have any symptoms.
Each type is caused by different gene If you have type 1 or type 2 VWD, you may
mutations and treated differently. This have the following mild to moderate
makes knowing the exact type of VWD symptoms:
that you have very important.

2 Your Guide to von Willebrand Disease

• Frequent large bruises from minor not be diagnosed until they have heavy
bumps or injuries. bleeding after surgery or some other
trauma.
• Frequent or difficult-to-stop nosebleeds.
On the other hand, type 3 VWD can cause
• Extended bleeding from the gums after
major bleeding problems during infancy
a dental procedure.
and childhood. As a result, children with
• Heavy or extended menstrual bleeding type 3 VWD are usually diagnosed during
in women.* their first year of life.
• Blood in your stools from bleeding in To find out if you have VWD, your doctor
your intestines or stomach. will take a complete medical history and
do a physical exam. For the history, he
• Blood in your urine from bleeding in or she will likely want to know about
your kidneys or bladder. your personal and family history -–
• Heavy bleeding after a cut or other in particular:
accident. • Any episodes of bleeding from a small
• Heavy bleeding after surgery. wound that lasted more than 15 minutes
or started up again within the first seven
*Heavy menstrual bleeding is the most days following the injury.
common symptom in women. If it isn’t
treated, it can lead to iron deficiency and • Any episodes of extended, heavy or
anemia. (Not all heavy menstrual repeated bleeding after surgery or dental
bleeding is due to VWD.) extractions that required medical
attention.
If you have type 3 VWD, you may have any
or all of the symptoms listed above, as well • Any episodes of bruising with little or
as the following: no apparent trauma, especially if you
could feel a lump under the bruise.
• Severe bleeding episodes for no reason.
These bleeding episodes can be life • A nosebleed that occurred for no
threatening if not treated right away. apparent reason and lasted more than
10 minutes despite pressure on the nose
• Bleeding into soft tissue or joints, or a nosebleed that needed medical
causing severe pain and swelling. attention.
• Any episode of blood in your stool for
How is von Willebrand Disease no apparent reason.
Diagnosed? • Any heavy menstrual bleeding in
Von Willebrand disease is sometimes women (usually with clots or lasting
difficult to diagnose. People with type 1 longer than 7 to 10 days).
or type 2 VWD may not have major • Any history of muscle or joint bleeding.
bleeding problems; as a result, they may

Your Guide to von Willebrand Disease 3

 • Any medicines you've taken that might Your doctor may order these tests more
cause bleeding or increase the risk of than once to confirm the diagnosis. He or
bleeding – for example, aspirin, other she may also refer you to a hematologist
nonsteroidal anti-inflammatory drugs (a doctor who specializes in treating blood
(NSAIDs), clopidogrel (Plavix ®), diseases) to confirm the diagnosis and for
warfarin, or heparin. follow-up care.
• Any history of liver or kidney disease, Early diagnosis is important to make sure
blood or bone marrow disease, or high you are treated effectively and can live a
or low blood platelet counts. normal, active life.
The doctor will also do a physical exami- How is von Willebrand Disease
nation to look for: Treated?
• Unusual bruising or other signs of Your doctor will decide what treatment
recent bleeding. you need, based on the type of VWD you
have and how severe it is. Most cases of
• Evidence of liver disease or anemia.
von Willebrand disease are mild, and
No single test exists for diagnosing VWD. you may need treatment only if you have
As a result, your doctor will order a surgery, tooth extraction, or an accident.
combination of blood tests to diagnose
Treatments for von Willebrand disease
the disease. These tests may include:
include medicines to:
• Von Willebrand factor antigen. This test
• Increase release of von Willebrand
measures the amount of von Willebrand
factor and factor VIII into the blood-
factor in your blood.
stream.
• Von Willebrand factor ristocetin
• Replace von Willebrand factor.
(ris-toe-SEE-tin) cofactor activity. This
test shows how well the von Willebrand • Prevent breakdown of clots.
factor works.
• Control heavy menstrual bleeding in
• Test for factor VIII clotting activity. women.
Some people with von Willebrand
Specific treatments include:
disease have low levels of factor VIII
activity, while others have normal levels. • Desmopressin (DDAVP). This is a
synthetic hormone that you usually take
• Von Willebrand factor multimers. This
by injection or nasal spray. It makes
test is performed if one or more of the
your body release more von Willebrand
3 tests above are abnormal. It shows
factor and factor VIII into your blood-
the makeup or structure of the von
stream. DDAVP works for most
Willebrand factor. It helps your doctor
patients with type 1 and some with type
diagnose what type of VWD you have.
2 VWD.
• Platelet function test. This test
measures how well your platelets are
working.

4 Your Guide to von Willebrand Disease

Tips for Living with von Willebrand Disease

I t ' s i m p o r t a n t t h a t y o u t r y t o p re v e n t b l e e d i n g V W D ( f o r e x a m p l e, t y p e 3 V W D ) , s o t h a t i n
a n d s t a y h e a l t h y. Yo u s h o u l d : case of serious injury or accident the
d o c t o rs c a r i n g f o r y o u w i l l k n o w y o u h a v e
■ Av o i d o v e r- t h e - c o u n t e r m e d i c i n e s t h a t c a n
V W D.
a f f e c t b l o o d c l o t t i n g, i n c l u d i n g a s p i r i n ,
I b u p ro f e n , a n d o t h e r n o n s t e ro i d a l ■ E x e rc i s e re g u l a r l y a n d m a i n t a i n a h e a l t h y
anti-inflammatory drugs (NSAIDs). w e i g h t . E x e rc i s e h e l p s ke e p m u s c l e s
f l e x i b l e. I t a l s o h e l p s p re v e n t d a m a g e t o
■ A l w a y s c h e c k w i t h y o u r d o c t o r b e f o re
m u s c l e s a n d j o i n t s. A l w a y s s t re t c h b e f o re
t a k i n g a n y m e d i c i n e s.
e x e rc i s i n g.
■ Te l l y o u r d o c t o r o r d e n t i s t t h a t y o u h a v e
S o m e s a f e e x e rc i s e s o r a c t i v i t i e s a re
v o n W i l l e b ra n d d i s e a s e. Yo u r d e n t i s t c a n
s w i m m i n g, b i k i n g, a n d w a l k i n g. F o o t b a l l ,
talk to your doctor about whether you
h o c ke y, w re s t l i n g, a n d l i f t i n g h e a v y
n e e d m e d i c i n e b e f o re d e n t a l w o r k t o
w e i g h t s a re n o t s a f e a c t i v i t i e s i f y o u h a v e
r e d u c e b l e e d i n g.
b l e e d i n g p ro b l e m s. A l w a y s c h e c k w i t h y o u r
■ C o n s i d e r w e a r i n g a m e d i c a l I D b ra c e l e t o r d o c t o r b e f o re s t a r t i n g a n y e x e rc i s e p ro g ra m .
necklace if you have a serious form of

• VWF replacement therapy. This • Fibrin glue is medicine that is placed

involves getting an infusion of a concen- directly on a wound to stop the
trate of von Willebrand factor and bleeding.
factor VIII into a vein in your arm. This
treatment can be used if you:
Frequently Asked Questions
❏ Can’t take DDAVP or need extended
treatment. 1. Is there a cure for von Willebrand
disease?
❏ Have type 1 VWD that doesn’t
respond to DDAVP. No, von Willebrand disease is a lifelong
disorder. However, most people have a
❏ Have type 2 or type 3 VWD.
mild form that causes little or no
• Oral contraceptives, or birth control change in their lives. Even those with
pills, can help women who have heavy type 3 von Willebrand disease can live
menstrual bleeding. The hormones in normal, active lives once they are
the pills can increase the amount of treated.
VWF and factor VIII in the blood-
2. Who should know that I have von
stream.
Willebrand disease?
• Antifibrinolytic drugs help prevent the
People like your doctor, dentist,
breakdown of blood clots. They are
employee health nurse, gym trainer,
used mostly to stop bleeding after minor
and sports coach should be aware of
surgery, tooth extraction, or an injury.
your condition. If you have a severe
They may be used alone or together
form of VWD (for example, type 3
with DDAVP and replacement therapy.

Your Guide to von Willebrand Disease 5

 VWD), consider wearing a medical ID 5. Do all people who have the gene for
bracelet or necklace that states you von Willebrand disease have bleeding
have von Willebrand disease. In case of problems?
an accident or emergency, this will be
No. Some people carry the genes for
very helpful to the health care team
the disease but do not have symptoms.
treating you.
They still can pass the disease on to
Since your parents, brothers and their children.
sisters, and children may also have von
6. How will I know if I have von
Willebrand disease, you should
Willebrand disease?
consider telling them about your
diagnosis and suggesting they consider The first step is a thorough history
getting tested. and physical examination. If you or
anyone in your family has a history of
3. Who should know that my child has
bleeding, your doctor can order a
von Willebrand disease?
number of blood tests to find out
Anyone who is responsible for your whether you have the disease. These
child should know about his or her tests will check how quickly your blood
condition. For example, his or her clots and the levels of clotting factors in
teacher, school nurse, daycare provider, your blood. Your doctor can use these
coach, or any after-school program test results to diagnose your exact type
leader should know, particularly if your of VWD.
child has one of the more severe forms
of VWD (for example, type 3 VWD).
4. Is von Willebrand disease life
threatening?
Most people with von Willebrand
disease have a mild form of the disease,
which usually does not cause bleeding
that is life threatening. But any
bleeding that can’t be controlled can be
life threatening. Some people with
severe forms of von Willebrand disease
need to seek emergency treatment to
stop bleeding before it becomes life
threatening.

6 Your Guide to von Willebrand Disease

For Women Only
Heavy menstrual bleeding is often the main for another reason, it will do away with
symptom of von Willebrand disease for menstrual bleeding altogether and possibly
women. Doctors call this menorrhagia. improve your quality of life. However,
They define it as: hysterectomy carries its own risk of bleeding
complications.
■ bleeding with clots larger than about
1-inch in diameter Pregnancy can be a challenge for women
with VWD. Although blood levels of VWF
■ anemia or low blood iron
and factor VIII tend to increase during
■ need to change pad or tampon more pregnancy, women with VWD can have
than every hour bleeding complications during delivery.
They also are likely to have heavy bleeding
Of course, heavy menstrual bleeding can for an extended period after delivery.
also be a sign of a gynecological disorder, so
it's important to have a complete gynecolog- However, there are things you can do to
ical exam before you seek testing for VWD. minimize the chances of complications in
pregnancy:
If you have VWD and heavy menstrual
bleeding, there are treatments to help you. ■ Consult a hematologist and an
They include: obstetrician who specializes in high-risk
pregnancies before you become
■ Combined oral contraceptives. They pregnant.
increase VWF and factor VIII in your
blood and reduce menstrual blood loss. ■ Consider using a center that specializes
They are the most often recommended in high-risk obstetrics and has a
birth control method for women with hematologist with expertise in bleeding
VWD. disorders on the staff for prenatal care
and delivery.
■ A levonorgestrel intrauterine device.
This is a contraceptive device that ■ Before you have any invasive procedure,
contains progestin. such as amniocentesis, ask your doctor
whether anything needs to be done to
■ Aminocaproic acid or tranexamic acid are prevent serious blood loss.
antifibrinolytic drugs. They can reduce
bleeding by slowing the breakdown of ■ During your third trimester, you should
blood clots. have blood tests to measure VWF and
factor VIII to help plan for delivery.
■ Desmopressin (DDAVP).
■ You should also meet with an
For some women who no longer anesthesiologist to review your
want children, endometrial ablation is choices for anesthesia and to
performed. This procedure, which discuss taking medicine to reduce
destroys the lining of your uterus, has your bleeding risk.
been shown to reduce menstrual blood
loss in women with VWD. With these precautions, most women
with VWD can have successful
If you need a hysterectomy, or pregnancies.
surgical removal of the uterus,

7
For More Information

The NHLBI Health Information Center is a service of the National Heart, Lung, and
Blood Institute (NHLBI) of the National Instititues of Health. The NHLBI Health
Information Center provides information to health professionals, patients, and the
public about the treatment, diagnosis and prevention of heart, lung, and blood
diseases and sleep disorders. For more information, contact:

NHLBI Health Information Center

P.O. Box 30105
Bethesda, MD 20824-0105
Phone: 301-592-8573
TTY: 240-629-3255
Fax: 301-592-8563
Web site: http://www.nhlbi.nih.gov

Copies of this and other publications are available in bulk at discounted rates.

NIH Publication No. 08-5849

January 2008