When living with a bleeding disorder like von Willebrand disease (VWD), a visit to the dentist’s office or a surgery center can quickly become complicated. People with VWD bleed more easily than others, which can make surgery and recovery harder to manage. Fortunately, you and your health care team can take steps to ensure you’re safe.

This article discusses what to expect when you have surgery with VWD and how your doctor will manage your condition. If you have any questions or concerns, you can speak with your doctor, surgeon, or hematologist (blood specialist).

What Is von Willebrand Disease?

VWD is a bleeding disorder that interferes with how well your blood forms clots. People with VWD have a genetic mutation (change) that affects von Willebrand factor (VWF). This protein works together with sticky cell fragments called platelets to create blood clots. In VWD, your body doesn’t make enough VWF, or it makes abnormal VWF proteins that don’t work like they should.

Most people with VWD have a mild form that doesn’t cause any noticeable symptoms. However, if you have very low VWF levels, you may experience more severe bleeding. Signs of VWD can include:

• Easy bruising with raised, swollen bruises on the skin
• Frequent nosebleeds that last 10 minutes or more
• Heavy menstrual periods that quickly soak through pads and tampons
• Bleeding from cuts or injuries that takes more than 10 minutes to stop
• Blood in your urine or stool
• Anemia (low red blood cell counts) from a lack of iron

Bleeding With Surgery in von Willebrand Disease

Another sign of VWD is heavy bleeding after a dental procedure or surgery. The body can’t create blood clots like it normally would, so incisions from surgery bleed more and for longer. How heavily you bleed depends on the type of VWD you have.

There are three main subtypes of VWD — types 1, 2, and 3. Type 1 VWD is the most common and is associated with mild symptoms. People with this subtype make only 30 percent of the needed VWF, so they don’t bleed as much. Type 2 WVD is more severe than type 1. This is because there isn’t enough VWF and it doesn’t work as it should.

Those with type 3 VWD don’t make any VWF. This is the most severe type of VWD, and it’s also the rarest. Type 3 VWD can look very similar to moderate hemophilia.

What Surgeries Do People Commonly Have With von Willebrand Disease?

The bleeding risk with VWD depends on the type of surgery you have. Major surgeries involve incisions or cuts that expose parts of your body. Examples include:

• Joint replacements
• Surgeries that involving cutting into the abdomen (tummy area)
• Removal of different organs, like the tonsils or uterus
• Cesarean section (C-section) to deliver a baby
• Surgeries on the brain or spine

Minor surgeries include:

• Dental extractions or removing a tooth
• Biopsies — Taking a small piece of tissue to look at under a microscope, which helps diagnose a disease
• Endoscopies — Using a long, thin tube with a camera attached to look inside the body

How Will Your Health Care Team Manage von Willebrand Disease Before Surgery?

Before surgery, you’ll meet with your health care team to talk about your surgical plan. They’ll go over the risks of surgery — which typically include bleeding, infections, and pain at your surgery site. You can also ask questions.

In some cases, your doctor may want to measure your VWF levels. Your VWF levels can change over time, so it’s important to confirm them before surgery. Certain tests can also measure how well VWF works at creating blood clots. Your health care team will use these test results to create your treatment plan during and after surgery.

Medications To Prevent Bleeding During Surgery

Before you have surgery, your doctor will discuss their strategies for preventing bleeding. Research shows that treating people with VWD before surgery lowers the risk of complications. This is known as prophylaxis or prophylactic treatment.

Milder cases of VWD are typically treated with desmopressin acetate (DDAVP), a hormone that tells your body to make more VWF protein. DDAVP is usually the first treatment option doctors use for VWD. It’s also commonly used to control bleeding from minor surgeries. Studies show that DDAVP treatment before surgery is effective in 91 percent to 100 percent of cases, according to the journal Blood Transfusion.

DDAVP isn’t effective for everyone, and some people can’t take it. If this is the case, and if your VWD is very severe, your doctor will use replacement therapy before surgery. This involves injections with clotting factors to replace what your body can’t make. Replacement therapy for VWD typically includes VWF and clotting factor VIII (factor 8 — you will usually see Roman numerals in any discussion of clotting factors). Replacement therapy must be given within 12 to 24 hours before surgery in order to achieve adequate factor levels.

Another option for controlling bleeding with VWD in surgery is tranexamic acid (Cyklokapron). This medication is known as an antifibrinolytic. It helps prevent your body from breaking down blood clots. Your doctor may use it before a dental procedure like removing a tooth or surgery.

How Will Your Health Care Team Manage von Willebrand Disease After Surgery?

Once your surgery is complete, your doctor will continue monitoring your bleeding risk. They’ll keep giving medications as needed to prevent bleeding. It’s important to keep your VWF levels steady after surgery. Treatment can last from one to five days for minor procedures and seven to 14 days for major procedures.

Lowering the Risk of Blood Clots

Surgery always comes with risks. People with VWD taking clotting factor replacement therapy can develop dangerous blood clots. However, this risk is rare. If a blood clot becomes stuck in a blood vessel in the brain or lungs, it can lead to complications.

Your doctor and surgical team will monitor you closely after surgery. Your team may also encourage you to get up and walk after surgery and wear special leg stockings to prevent clots.

Preventing Bleeding After Surgery

When you have an incision or open wounds from a procedure, your blood needs to clot properly to seal them off. People with VWD are at risk of bleeding after surgery. Your doctor can give you medications like antifibrinolytics to stabilize new blood clots.

If you start bleeding too much after surgery, you may need to replace the lost blood with a transfusion. A group of studies found that major bleeding occurred in up to 5 percent of procedures. Between 3 percent and 20 percent of people needed transfusions.

Living With von Willebrand Disease

As you continue healing after your procedure, it’s important to take extra precautions living with VWD. Try taking pain medications like acetaminophen (Tylenol) instead of ibuprofen. Ibuprofen isn’t recommended because it has a blood-thinning effect. Avoid activities that will agitate your incision site or lead to easy bruising or bleeding as well.

Talk To Your Doctor About Surgery and von Willebrand Disease

Surgery can be a significant medical procedure. With VWD, you want to be prepared for surgery and understand the risks associated. Your doctor and health care team can talk with you about any of your concerns. They’ll also cover the precautions taken before, during, and after surgery to keep you safe.

Find Your Team

On MyHemophiliaTeam, the social network for people with hemophilia and their loved ones, more than 6,000 people from around the world come together to ask questions, offer support and advice, and connect with others who understand life with hemophilia and other bleeding disorders.

Do you have experience preparing for surgery with VWD, hemophilia, or another bleeding disorder? Share your thoughts below, or post a comment on your Activities page.