Von Willebrand disease

Von Willebrand disease is a lifelong bleeding condition that makes it hard for blood to clot. People with the condition either have low levels of von Willebrand factor, a protein that helps blood clot, or the protein they have doesn’t work well.

Most people with the condition are born with it because the gene that causes it can be passed down from one or both parents. But warning signs, such as heavy menstrual bleeding or bleeding for a long time after dental work, might not show up for years.

Von Willebrand disease can’t be cured. But there are treatments and self-care that can help people with this condition lead active lives.

The most common symptom of von Willebrand disease is bleeding more than expected.

There are three main types of von Willebrand disease. The amount of bleeding varies from one person to another, depending on the type of the condition and how bad it is.

If you have von Willebrand disease, you might have:

• Bleeding for a long time from an injury or after surgery or dental work.
• Nosebleeds that last longer than 10 minutes.
• Heavy or long menstrual bleeding.
• Heavy bleeding during labor and delivery or after delivery.
• Blood in urine or stool.
• Easy bruising or lumpy bruises.
• With more-serious forms of von Willebrand disease, there may be sudden bleeding into joints.

Menstrual symptoms might include:

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

Many people with von Willebrand disease don’t know they have it because they have no symptoms or the symptoms are mild.

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

The usual cause of von Willebrand disease is a changed gene passed through families. Conditions that happen in this way are called inherited. The gene manages von Willebrand factor, a protein needed for blood clotting.

When you have low levels of this protein or the protein doesn’t work as it should, small blood cells called platelets can’t stick together well. The platelets also can’t attach themselves to blood vessel walls after an injury.

This gets in the way of the clotting process. Sometimes it causes bleeding that is hard to stop.

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

Rarely, people who didn’t get a changed gene from a parent can get von Willebrand disease later in life. This is called acquired von Willebrand syndrome. The cause is likely another medical condition.

The main risk factor for von Willebrand disease is having a family history of it. Parents pass down the gene that causes the condition to their children.

The condition often is an autosomal dominant inherited disorder, which means you need a changed gene from only one parent to get the condition. If you have the gene for von Willebrand disease, you have a 50% chance of passing on this gene to your children.

The most serious form of the condition is autosomal recessive. This means both of your parents pass a changed gene to you.

Rarely, von Willebrand disease can cause bleeding that won’t stop. This 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 bleeding in the joints or soft tissue.

If you plan to have children and have a family history of von Willebrand disease, think about getting genetic counseling. If you carry the gene for von Willebrand disease, you can pass it down to your children even if you don’t have symptoms.

To diagnose von Willebrand disease, your healthcare professional asks about your medical and bleeding history and checks for bruises or other signs of recent bleeding.

Your healthcare professional may order the following blood tests:

• Von Willebrand factor antigen. This shows the level of von Willebrand factor in your blood by measuring a certain protein.
• Von Willebrand factor activity. Tests can measure how well the von Willebrand factor works in the clotting process.
• Factor VIII clotting activity. This shows whether your levels and activity of factor VIII are too low.
• Von Willebrand factor multimers. This checks the form of von Willebrand factor in your blood, its proteins and how it breaks down. This test helps show the type of von Willebrand disease you have.

The results of these tests can change in the same person over time due to factors such as aging, stress, exercise, infection, pregnancy and medicines. So you might need to repeat some tests.

A healthcare professional who thinks you have a bleeding condition might send you to a specialist in blood conditions, called a hematologist. If you have von Willebrand disease, your hematologist might suggest that blood relatives, such as parents, siblings and children, have tests to see if this condition runs in your family.

Von Willebrand disease has no cure. But treatment can help prevent or stop bleeding. Your treatment depends on:

• The type of your condition and how bad it is.
• Where the bleeding is and how bad it is.
• Your need for procedures such as dental work that may cause bleeding.

Your hematologist might suggest one or more of the following treatments to raise the activity of your von Willebrand factor, strengthen blood clots or manage heavy menstrual bleeding:

• Desmopressin, also called DDAVP. Given as a shot or through a vein, this is a human-made hormone. It manages bleeding by getting the body to release more of the von Willebrand factor stored in the lining of the blood vessels.

  DDAVP often is the first treatment for managing von Willebrand disease. Given before minor surgery, it helps manage bleeding. You might get a trial of desmopressin to make sure it works for you.

• Replacement therapies. These involve getting blood-clotting factors through a vein that have von Willebrand factor and factor VIII. Your hematologist might suggest them if you can’t take DDAVP or it hasn’t worked for you.

  Another replacement therapy for adults 18 and older is a genetically engineered von Willebrand factor product. Genetically engineered means that the DNA has been changed in a lab. Also called recombinant factor, the product has no plasma. So it carries a lower risk of a viral infection or an allergic reaction.

• Birth control pills. Besides preventing pregnancy, these pills can help manage heavy bleeding during menstrual periods. The estrogen in birth control pills can raise von Willebrand factor and factor VIII activity.
• Clot-stabilizing medicines. These medicines can help stop bleeding by slowing the breakdown of blood clots. Also called antifibrinolytics, they include aminocaproic acid (Amicar) and tranexamic acid (Cyklokapron, Lysteda). Healthcare professionals often prescribe these medicines before or after you have surgery or a tooth pulled.
• Medicines to stop bleeding. A fibrin sealant (Tisseel) helps stop bleeding. A healthcare professional may use a syringe or a spray to put it on a cut. There also are products you can get without a prescription to stop nosebleeds.

If your condition is mild, your healthcare professional might suggest treatment only when you have surgery or dental work or when you’ve had an injury, such as from a car accident.

These self-care tips can help you manage your condition:

• Switch pain relievers. To help prevent bleeding, talk with your healthcare professional before you take pain relievers that thin the blood. These include aspirin, ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve). Your healthcare professional might suggest that you take acetaminophen (Tylenol, others) instead.
• Tell members of your healthcare team and your dentist about your condition. Let them know that you have von Willebrand disease before you have surgery or a dental procedure, start a new medicine, or give birth. Also tell them whether anyone in your family has a history of too much bleeding.
• Wear a medical ID bracelet. Make sure it says that you have von Willebrand disease. This can be helpful to medical workers if you’re in an accident or taken to an emergency room. Also carry a medical alert card in your wallet.
• Be active and safe. Exercise is part of reaching or staying at a healthy weight. But stay away from activities that could cause bruising, such as football, wrestling and hockey.

You may start by seeing your main healthcare professional, who might send you to a specialist in the diagnosis and treatment of bleeding conditions. This specialist is called a hematologist.

Here’s some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask about anything you need to do before you come in. For instance, you might need to fast before having blood tests.

Make a list of:

• Symptoms that involve bleeding. Note how often, how badly and for how long you bleed. Include bruising. Let your healthcare team know if you’ve had a history of nosebleeds or easy bruising since childhood. If you menstruate, note whether your periods are long and heavy.
• Key personal information, including major stresses or recent life changes.
• Key medical information, including other medical issues. Note whether anyone in your family bleeds easily. Also list medicines, vitamins and other supplements you take, including dosages.
• Questions to ask your healthcare professional.

Take a family member or friend along, if possible, to help you remember the information you get.

For von Willebrand disease, questions to ask your healthcare team include:

• What’s the most likely cause of my symptoms?
• What treatment do you suggest? How will I know whether treatment works?
• What are the possible side effects of the medicines you’re prescribing?
• Does this condition raise my risk of other medical problems?
• What will I need to do before having surgery or dental work?
• Does this condition increase my risk of health issues during pregnancy and childbirth? Are there ways to lower that risk?
• Do I need to stay away from certain physical activities or types of exercise?
• I have other health conditions. How can I best manage them together?

What to expect from your doctor

Your healthcare professional might ask:

• Have you had bleeding from a small wound that lasted more than 15 minutes or happened again within a week of getting the wound?
• Have you had a nosebleed that lasted more than 10 minutes or needed medical attention?
• Have you had easy bruising? Have you ever felt a lump under a bruise?
• If you menstruate, how many days do your periods last? How often do you change your tampon or sanitary pad during a period? Have you seen blood clots in your menstrual flow?
• Have you had blood in your stool that wasn’t due to a known medical problem, such as a stomach ulcer or colon polyp?
• Have you ever needed medical attention for bleeding during or after surgery, dental work, childbirth or injury?
• Have you had anemia or needed blood from a donor?

What you can do in the meantime

While you wait for your appointment, don’t use pain relievers that can increase your risk of bleeding. These include aspirin, ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve). If you need relief for aches and pains, try acetaminophen (Tylenol, others) instead.

Don’t do contact sports known to have a high risk of bruising or injury, such as football and hockey. Before having medical or dental procedures, tell your healthcare professional or dentist about your history of heavy bleeding from minor injuries.

If you need a procedure that’s not urgent, do not schedule it until after you’ve been checked for a bleeding condition.