Tell Me More about Von Willebrand Disease

Von Willebrand disease (VWD) is a bleeding disorder caused by having too little von Willebrand factor (VWF) protein or VWF that doesn’t work well. VWF makes platelets stick together and carries the clotting factor VIII. VWD is the most common of all inherited bleeding disorders and occurs in males and females.

People with VWD can have prolonged bleeding, easy bruising and bigger bruises, nosebleeds,or bleeding with dental work or surgery. Girls often have heavy menstrual periods. Sometimes the symptoms are very mild, and some people with VWD do not even know they have it.

It can take a long time to diagnose VWD because the lab tests needed can vary with time and can be affected by many things, such as illness, hormones and stress. So, tests may be repeated a few times to be sure whether or not you have VWD.

There are three types of VWD, based on the amount of VW factor protein and how well it works. Often, additional tests are needed to determine the type of VWD a person has. Most people have type 1, which is the mildest type. Type 2 and type 3 are less common.

How is it treated?

Treatment is important to prevent bleeding with any surgery, before pulling any teeth, and to help control heavy periods or nosebleeds. Treatment depends on the type of VWD, type of procedure, and location and amount of bleeding. Here are treatments and how they work:

• DDAVP (Desmopressin) releases the VW factor stored in your cells and raises the level to stop or prevent bleeding. It is given through a vein (IV) or by a nasal spray (Stimate). To make sure that DDAVP works for you, a DDAVP trial is needed to measure VWF levels before and after a DDAVP dose.
• Aminocaproic acid (Amicar) and Tranexamic acid are medicines taken by mouth that work for some types of bleeding by stopping the clot from breaking down too soon.
• Plasma-derived VWF replacement products (Humate P™ and Alphanate™) are given by IV for those with type 3, some type 2, or for those with type 1 for major surgery or who don’t respond to DDAVP.
• Hormonal therapy, or birth control pills, can help girls with heavy menstrual bleeding by increasing the VW factor and factor VIII in their blood and regulating their cycles.

What you should do:

Be sure to ask questions at your clinic appointment, or call the nurses any time. Our team of doctors, nurses and social workers are all here to help you. Just ask us!

1. Learn more about VWD by reading the booklets you received in clinic and check out reputable websites like www.hemophilia.org. Click on the VWD sidebar on the home page.
2. Visit CHOP’s Hematology Clinic at least once a year. Put our phone numbers in your cell phone so you can reach us quickly. The nurse’s number during business hours is 215-590-2199. For emergencies outside business hours, call 215-590-1000 and ask the operator to page the hematologist on call.
3. Talk to the team in clinic if you have questions or any bleeding. Let us know at least two weeks before any procedure so we can develop a plan and help you get the medicines you need.
4. Learn how to prevent and treat bleeding, and about the type and dose of medicine you use.
5. Stay physically active and fit. Participate in sports and gym class. We do not recommend high-risk contact sports like football, but most kids with VWD enjoy many other sports like bike riding (with a helmet!), running and just having fun with friends.