Von Willebrand disease

Von Willebrand disease is a lifelong bleeding disorder in which your blood doesn't clot properly. 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.

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
• Frequent nosebleeds that don't stop within 10 minutes
• Heavy or long menstrual bleeding
• Heavy bleeding during labor and delivery
• Blood in your urine or stool
• Easy bruising or lumpy bruises

Menstrual signs and symptoms might 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

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

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 affected gene from a parent. This is known as acquired von Willebrand syndrome, and it's likely caused by an underlying medical condition.

The main risk factor for von Willebrand disease is having a family history of it. Parents pass the 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 a mutated gene from only one parent to get the disease. 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 a mutated gene to you.

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

• Anemia. Heavy menstrual bleeding can cause iron deficiency anemia.
• Swelling and pain. This can be a result of abnormal bleeding in the joints or soft tissue.

If you plan to have children and have a family history of von Willebrand disease, consider genetic counseling. If you carry the gene for von Willebrand disease, you can pass it on to your offspring, even if you don't have symptoms.