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:
Menstrual signs and symptoms might include:
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:
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.
Mild forms of von Willebrand disease can be difficult to diagnose because bleeding is common, and, for most people, doesn't indicate a disease. However, if your doctor suspects you have a bleeding disorder, he or she might refer you to a blood disorders specialist (hematologist).
To evaluate you for von Willebrand disease, your doctor will likely ask you detailed questions about your medical history and check for bruises or other signs of recent bleeding.
Your doctor will also likely recommend the following blood tests:
The results of these tests can fluctuate in the same person over time due to factors such as stress, exercise, infection, pregnancy and medications. So you might need to repeat some tests.
If you have von Willebrand disease, your doctor might suggest that family members undergo tests to determine if this condition runs in your family.
Even though von Willebrand disease has no cure, treatment can help prevent or stop bleeding episodes. Your treatment depends on:
Your doctor might suggest one or more of the following treatments to increase your von Willebrand factor, strengthen blood clots or control heavy menstrual bleeding:
Desmopressin. This medication is available as an injection (DDAVP). It's a synthetic hormone that controls bleeding by stimulating your body to release more of the von Willebrand factor stored in the lining of your blood vessels.
Many doctors consider DDAVP the first treatment for managing von Willebrand disease. It can be used before minor surgical procedures to help control bleeding. You might be given a trial of desmopressin to make sure it's effective for you.
Replacement therapies. These include infusions of concentrated blood-clotting factors containing von Willebrand factor and factor VIII. Your doctor might recommend them if DDAVP isn't an option for you or has been ineffective.
Another replacement therapy approved by the Food and Drug Administration for treating adults 18 and older is a genetically engineered (recombinant) von Willebrand factor product. Because recombinant factor is made without plasma, it can reduce the risk of a viral infection or allergic reaction.
If your condition is mild, your doctor might recommend treatment only when you're having surgery or dental work or when you've had a trauma, such as a car accident.
These self-care tips can help you manage your condition:
You'll likely see your family doctor if you think you have a bleeding problem. You then might be referred to a doctor who specializes in the diagnosis and treatment of bleeding disorders (hematologist).
Here's some information to help you get ready for your appointment.
Ask about pre-appointment restrictions when you make the appointment. You might need to follow dietary restrictions or fast for 8-10 hours if blood tests are scheduled.
Make a list of:
Take a family member or friend along, if possible, to help you remember the information you'll be given.
For von Willebrand disease, questions to ask your doctor include:
Your doctor might ask:
While you wait for your appointment, avoid pain relievers that can increase your risk of bleeding, such as aspirin, ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve). If you need relief for aches and pains, try acetaminophen (Tylenol, others) instead.
Steer clear of contact sports associated with a high risk of bruising or injury, such as football and hockey. If you have medical or dental procedures scheduled, tell your doctor or dentist about your history of heavy bleeding from minor injuries.
If you're having a procedure that's not urgent, reschedule it until after you've been evaluated for a bleeding disorder.
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