Von Willebrand disease


Von Willebrand disease is a lifelong bleeding disorder in which your blood doesn't clot well. People with the disease have low levels of von Willebrand factor, a protein that helps blood clot, or the protein doesn't perform as it should.

Most people with the disease are born with it, having inherited it from one or both parents. However, warning signs, such as heavy bleeding after a dental procedure, might not show up for years.

Von Willebrand disease can't be cured. But with treatment and self-care, most people with this disease can lead active lives.

Symptoms

Many people with von Willebrand disease don't know it because the signs are mild or absent. The most common sign of the condition is abnormal bleeding.

There are three main types of the disease. The amount of bleeding varies from one person to another, depending on the type and severity of the disease.

If you have von Willebrand disease, you might have:

  • Excessive bleeding from an injury or after surgery or dental work
  • Nosebleeds that don't stop within 10 minutes
  • Heavy or long menstrual bleeding
  • Blood in your urine or stool
  • Easy bruising or lumpy bruises

Menstrual signs and symptoms may include:

  • Blood clots greater than 1 inch (2.5 centimeters) in diameter in your menstrual flow
  • The need to change your menstrual pad or tampon more than once an hour
  • The need to use double sanitary protection for menstrual flow
  • Symptoms of anemia, including tiredness, fatigue or shortness of breath

When to see a doctor

Contact your doctor if you have bleeding that lasts a long time or is hard to stop.

Causes

The usual cause of von Willebrand disease is an inherited abnormal gene that controls von Willebrand factor — a protein that plays a key role in blood clotting.

When you have low levels of this protein or it doesn't work as it should, small blood cells called platelets cannot stick together properly nor attach themselves normally to the blood vessel walls when an injury has occurred. This interferes with the clotting process and can sometimes cause uncontrolled bleeding.

Many people with von Willebrand disease also have low levels of factor VIII, another protein that helps in clotting.

Factor VIII is involved in another inherited clotting disorder called hemophilia. But unlike hemophilia, which mainly affects males, von Willebrand disease affects males and females and is usually milder.

Rarely, von Willebrand disease can develop later in life in people who didn't inherit an abnormal gene from a parent. This is known as acquired von Willebrand syndrome, and it's likely caused by an underlying medical condition.

Risk factors

The main risk factor for von Willebrand disease is having a family history of it. Parents pass the abnormal gene for the disease to their children. Rarely, the disease skips generations.

The disease is usually an "autosomal dominant inherited" disorder, which means you need an abnormal gene from only one parent to be affected. If you have the gene for von Willebrand disease, you have a 50% chance of transmitting this gene to your children.

The most severe form of the condition is "autosomal recessive," which means both of your parents have to pass an abnormal gene to you.

Complications

Rarely, von Willebrand disease can cause uncontrollable bleeding, which can be life-threatening. Other complications of von Willebrand disease can include:

  • Anemia. Women who have heavy menstrual bleeding can develop iron deficiency anemia.
  • Swelling and pain. This can be a result of abnormal bleeding in the joints or soft tissue.

Prevention

Because von Willebrand disease is usually an inherited disorder, consider having genetic counseling if you have a family history of this condition and you're planning to have children. If you carry the defective gene for von Willebrand disease, you can pass it on to your offspring, even if you don't have symptoms.