What Is von Willebrand Disease?
Utah Center for Bleeding & Clotting Disorders
Our Location
Sugar House Health Center
Fourth Floor
1280 E. Stringham Avenue
Salt Lake City, Utah 84106
Von Willebrand disease (VWD) is a genetic bleeding disorder that is passed down from parent to child and affects both men and women. People with VWD do not make enough protein — known as the von Willebrand factor (VWF). This protein is needed for platelets in the blood to form clots and stop or prevent bleeding. When you cut your skin, platelets will move to the spot to plug the hole. The von Willebrand factor allows the platelets to stick to the blood vessel wall and to each other. In a person with VWD, the platelets do not stick together well enough to make a good platelet plug. This results in a longer than usual bleed.
Von Willebrand Disease vs. Hemophilia
People with hemophilia or Type 1 VWD may have low levels of the blood clotting factor VIII (8). However, the big difference between the two bleeding disorders is that people with hemophilia have normal levels of von Willebrand factor. Hemophilia is also more common in men whereas von Willebrand disease occurs in both men and women equally.
Types of von Willebrand Disease
There are three different types of von Willebrand disease:
- Type 1 — This is the most common and mildest form of VWD. People with Type 1 VWD have low levels of von Willebrand factor (VWF) and may also have low levels of blood clotting factor VIII (8).
- Type 2 — A person with Type 2 VWD has normal amounts of VWF, but the factor does not work properly. This type of VWD is broken down into four subtypes based on the particular problem with VWF. Each subtype needs to be treated differently.
- Type 3 — This is the rarest and most severe type of VWD. A person with Type 3 VWD has very little or no VWF and low levels of blood clotting factor VIII (8).
von Willebrand Disease Symptoms
People with VWD experience the following symptoms:
- Nosebleeds
- Easy bruising
- Menstrual periods with heavier than normal bleeding that last longer than seven days
- Abnormal bleeding after surgery (such as having a tooth pulled, getting injured, or childbirth)
However, not everyone with VWD bleeds.
Find a Hematologist
von Willebrand Disease Diagnosis
We will ask you questions about abnormal bleeding (such as nosebleeds or bruising) and your family history of bleeding. We will then conduct blood tests that are specific to different types of bleeding disorders. These tests will tell us if you have normal, low, or no blood clotting factor in your blood.
What to Expect at Your First Appointment
As a team, we want your first appointment at Utah Center for Bleeding & Clotting Disorders at University of Utah Health to run smoothly and provide you with quality education to help guide the treatment of your von Willebrand disease diagnosis.
We treat von Willebrand disease using a multidisciplinary team approach:
- Hematologist
- Nurse practitioner
- Nurse
- Physical therapist
- Social worker
- Pharmacist
- Genetic counselor
- Research coordinator
You will typically see each of these providers during your initial visit to our center. Clinic visits are often 1.5 to two hours in length. Please come with any questions, concerns, or issues written down to discuss with your provider.
Blood Work
We often conduct blood work in conjunction with your first visit. Your blood will be drawn either in the exam room or in the lab following your visit. Some insurance carriers do not pay for labs to be done at U of U Health. If that is the case with your insurance, we will help you arrange labs at a location covered under your insurance provider.
How to Prepare for Your Appointment
Before you arrive to your appointment, please make sure your visit is covered under your insurance. If you have any questions about your coverage, call our office. Most insurance companies work with our center to ensure patients are treated appropriately. Please send our office your past medical records to help the team understand your previous care and guide their decisions for future care.
What to Bring
Please bring the following to your appointment:
- Insurance card
- Medication history, including factor prescription
- Bleeding and dosing logs
- Pair of shorts for your physical assessment
- Any other items that might help us guide your treatment
Please arrive 20 minutes before your appointment to fill out pre-clinic paperwork.
von Willebrand Disease Treatment
Need Surgery?
If you and your provider decide that surgery is the best treatment option, you will need to fill out a pre-surgery form. Please fill out the highlighted patient section on the last page of the form and send it back to your surgeon before your procedure. We require a 4-week notice to coordinate all elective surgeries and procedures.
We treat von Willebrand disease using medications like DDAVP (desmopressin acetate). This drug will release von Willebrand factor into the bloodstream. However, this drug does not work for everyone. Before we prescribe you DDAVP, we will give you a dose of the medication when you aren't bleeding to test its effects on your body. Then we will measure the von Willebrand factor level in your blood to see if the medication released enough of the protein to stop any bleeding.
Most people who respond well to DDAVP use a form that is squirted into the nose. It is known by the brand name Stimate®. From the nose, the medication is absorbed into the blood. Another form of DDAVP can be injected directly into your blood though a vein or given as a shot under the skin. For major surgeries, von Willebrand factor will be given intravenously to increase your von Willebrand levels.
In general, people with mild to moderate VWD will experience minimal bleeding problems except when they have surgery or serious trauma. A person with severe VWD may have many of the same problems that a person with hemophilia has, including bleeding into joints.
Home Remedies to Stop Bleeding & Swelling
PRICE = Protection + Rest + Ice + Compression + Elevation
PRICE* is used as a general first aid treatment for joint or muscle bleeds or other injuries to help stop bleeding, manage swelling, and reduce pain. These recommendations are to be used in conjunction with blood clotting factor infusions as recommended by your medical provider.
PROTECTION — Limit your movements or keep weight off the affected area to prevent further injury and help stop bleeding. An arm sling, brace, or a splint will help support your arm or leg and limit movement. Assistive devices such as crutches or a walker will also help you decrease or prevent putting weight on a painful leg or foot.
If you don't have these items at home, contact the Utah Center for Bleeding & Clotting Disorders at U of U Health for recommendations.
REST — Stop using the affected arm or leg for 24 to 48 hours. However, you may need to limit activity for a few days or longer, depending on the site or severity of the injury or bleeding episode. Rest will help stop bleeding, manage pain, and limit swelling.
ICE — Apply ice/cold packs to the affected area (as needed) for 15 to 20 minutes every two to four hours for up to 48 hours or longer for more severe bleeds. This will help reduce your pain. Wrap a plastic bag of ice or cold packs in a thin cloth or paper towel to avoid direct contact on the skin.
COMPRESSION — This technique is used to reduce swelling and to help stop the bleeding. Wrap the affected area with an elastic bandage. Start wrapping below the area of swelling in an X-cross pattern, NOT in circular layers. The bandage should be snug, but loose enough to easily slide a finger under the wrap.
Muscle bleeds can put pressure on nerves and blood vessels which may require immediate medical intervention to prevent permanent damage.
DO NOT use an elastic bandage if you have:
- numbness or tingling,
- an increase in pain, or
- skin that feels cool below the site of swelling.
Call your medical provider immediately if you have any of these symptoms.
ELEVATION — Elevating an injury will help reduce and resolve swelling. Keep the injured leg or arm elevated (ideally above the level of your heart) as much as possible in the first 24 to 48 hours.
*This information was prepared and reviewed by the Western and Mountain States PT Working Group as a quality improvement initiative to standardize educational materials: Final-1/5/2017
References: GUIDELINES FOR THE MANAGEMENT OF HEMOPHILIA e31© 2012 Blackwell Publishing Ltd Haemophilia (2013), 19. PT PRACTICE GUIDELINES FOR PERSON’S WITH BLEEDING DISORDERS: Joint Bleeds/Muscle Bleeds, MASAC Document #204, National Hemophilia Foundation. Steps for living: Education for all Stages: https://stepsforliving.hemophilia.org/basics-of-bleeding-disorders/treatment-basics/prompt-treatment-is-important#firstaid (retrieved 10/05/2016)
Is There a Cure for von Willebrand Disease?
Currently, there is no cure for von Willebrand disease. However, it is possible for changes in von Willebrand factor levels to occur with age, childbearing, and other factors that should be discussed with your hematologist.
Make an Appointment
To make an appointment with one of our hematology specialists, you will need a referral from your physician. We also accept self-referrals; please fill out our online appointment request form.
Please check with your insurance carrier to make sure this appointment will be covered. In some cases, special permission has been given to our providers to see patients with out-of-network insurance.
Please call us at 801-213-8371 to schedule an appointment. We look forward to meeting you.
