What is Von Willebrand Disease? And what is a bleeding disorder?

Von Willebrand Disease (vWD) is the most common genetic condition which causes a bleeding disorder (a condition which affects the body’s ability to clot blood properly). It is typically caused when the body cannot properly make a certain blood protein called Von Willebrand Factor (vWF).

Von Willebrand Factor works together with platelets to form the initial clot when a blood vessel is broken.

What are the most common types of vWD?

Von Willebrand Disease is the most common bleeding disorder, 1-2% of the population have a form of this disorder. There are four main types of vWD: Type I, II, III, and Platelet-type. About 75% of all cases of vWD are Type I which is also often the mildest form. Type II is the second most common type and accounts for about 20% of people with vWD; Type II is also the most variable and has several forms which require different treatment. Platelet type and Type III are very rare. Type III is the most serious form of vWD.

Is vWD serious?

Forming a blood clot is very important, and most people take this for granted. People with vWD cannot properly clot their blood. Most forms of vWD range from mild to moderate and only rarely require treatment.

However, some types, like Type III vWD, are a severe bleeding disorder which can be a very serious condition that requires treatment and some lifestyle modification.

Who get’s vWD?

All types of people can get vWD, it occurs equally frequently in males and females and in all races. Type I, II, and Platelet wWD are all dominant genetic disorders which means that only one parent must have the defective gene in order to pass on the disorder, these types can also occur in a person due to a spontaneous mutation. Type III vWD is a recessive gene and for someone to be affected they must have inherited a two defective copies of the gene (one from each parent).

This also means that two people who do not have Type III vWD can each carry the gene and pass on the disease to their children.

Can vWD be cured?

Currently there is no cure for vWD, but it can be treated.

How is vWD be treated?

Each form of vWD is treated differently so it is very important to know what type you have. In many cases it is typically treated with desmopressin (DDAVP). However, in certain forms of Type II and Platelet-type desmopressin can be ineffective and in some cases harmful and should not be used. In these and in severe forms, intravenous (IV) injection of the the missing blood factor (Von Willebran Factor (vWF) or a Factor VIII/vWF compounds) can be required. This type of treatment is called “factor replacement treatment.”

With care, people who receive modern and appropriate treatment for their bleeding disorder won’t be held back by their disorder, they can expect to live an active and high quality life.

Can you die from vWD?

Most people with vWD do not have life threatening symptoms. For most people with vWD childbirth, surgery, and extremely severe traumas may be the only events that must to be treated. However, in some people vWD is a severe bleeding disorder which can be life threatening. People severe vWD who receive modern and appropriate treatment live a near normal lifespan with a high quality of life.

However, even with modern treatment people with severe vWD should attempt to avoid trauma that can cause bleeds whenever possible.

How does vWD affect a person’s life?

Most people with vWD will have symptoms such as nosebleeds, bruising, and heavy menstrual periods which are uncomfortable or inconvenient but which do not present risks of disability or death. Many people with vWD are only at serious risk and should seek treatment when getting teeth extracted, surgery, childbirth, or in the case of severe trauma. However, for people with severe Type III vWD it is very important to avoid bleeding into their joints. Joint bleeds can lead to severe and permanent joint damage causing life long pain and disability. Prompt treatment or prophylactic treatment is very important in minimizing bleeding into joints.

Staying in shape, and having strong and toned muscles can also help prevent joint bleeds. Most doctors recommend that people with severe vWD avoid activities with high likelihoods of injury, joint stress, or trauma. Activities that should be avoided include: football, hockey, rugby, skateboarding, and motorcycle riding. Other activities with less physical contact but high rates of injury should only be practiced in consultation with a doctor; these include soccer, downhill skiing, basketball, baseball, and tennis.

Can my family doctor manage vWD?

Mild and moderate vWD can often be managed effectively by a patient and their primary care physician. In certain cases and in severe forms of the disorder it should be managed with an appropriately trained hematologist.

Is vWD contagious? / Can I catch vWD from someone else?

Von Willebrand Disease, like all genetic disorders, is something a person is either born with or not born with. No bleeding disorder can be passed from person to person. Some very rare forms of vWD are not genetic and can be acquired later in life due to other health complications.

How do I know if my child has a bleed that needs treatment?

Every child is different so only you and your doctor can best decide when to seek treatment. In non-severe types of vWD most common bleeds and bruises do not need treatment, but when in doubt contact your doctor. In severe forms where joint bleeds are a risk a good rule of thumb for joints is if the joint is swollen, feels warm, or the child avoids using it or complains of pain in the area then you should probably contact your doctor to seek treatment. And in the soft tissue, if a hematoma is large and keeps getting bigger or doesn’t begin changing shape and getting smaller after a day or two then you might need treatment.

If the hematoma is in the neck or an area of vital blood flow you should probably consult your doctor as soon as possible. If you suspect a bleed in the head because of symptoms such as vomiting, disorientation, or grogginess consult your doctor immediately as head bleeds are the most serious type of bleed.

If someone with vWD gets a cut or scrape will there be a lot of bleeding?

People with vWD don’t bleed more than people who do not have vWD so a small cut or scrape can often be stopped from bleeding without treatment. But occasionally cuts can re-bleed and can require treatment. Bleeding may be somewhat prolonged in most people with vWD. Even in severe vWD only very serious cuts and scrapes typically turn out to be issues.

For people with severe vWD internal bleeding into joints, the head, or soft tissues like muscles are typically much more serious.

Are there any medicines someone with vWD shouldn’t take?

People with vWD can take most medicines, however, some medicines have side effects which can complicate vWD and should be avoided. Any medicine which thins the blood and has a side effect of prolonged bleeding should be avoid. These include any medicine containing common painkillers, fever reducers, and anti-inflammatory drugs such as aspirin (i.e Bayer), naproxen sodium (i.e. Aleve), and ibuprofen (i.e. Advil). It is important to read the label and make sure medicines don’t contain these ingredients. Acetaminophen (i.e. Tylenol) is typically the primary over the counter fever reducer and pain relief medicine recommended for people with vWD. Bismuth subsalicylate (i.e. Pepto-bismal) is closely related to aspirin and should be avoided for similar reasons. Some doctors recommend against cough syrups containing guaifenesin.

Drugs that are prescribed as anticoagulants such as heparine and warfarin should also be avoided. Ask your doctor if you’re unsure about what medicines are safe to take. If you are prescribed a new medicine it’s a good idea to ask your pharmacist if the drug has any anitplatelet effects. People with certain kinds of Type II vWD should avoid Desmopressin (DDAVP) as it can cause additional bleeding complications.


The Way A Bleeder's Life Should Be, Ayuh!