HELPFUL TIPS AND HINTS Vaccinations and shots can be given under the skin instead of into muscles so as not to start a bleed. Talk to your family doctor about the von Willebrand diagnosis. A hematologist should be consulted prior to the administration of any shots. Routine dental visits will help reduce future problems with teeth and gums. Talk to your dentist about von Willebrand. It might be beneficial to use clotting factor medication prior to dental work to prevent bleeds. A hematologist should be consulted before starting dental work.
Diplomat Specialty Infusion Group is a national provider of specialty pharmacy and home infusion services, experienced in the treatment of VWD and related bleeding disorders. Contact your Diplomat representative today to learn more about how our infusion therapy programs might benefit you.
In the event that an operation is needed, the doctor and surgeon should be informed about von Willebrand. A hematologist should be consulted prior to any surgical procedures. Prevention and understanding are very important to maintaining good health with hemophilia. If you have von Willebrand, an annual checkup at a hemophilia treatment center may be beneficial in addition to routine health care. During this evaluation, trained specialists will ask for information and perform tests needed to keep your medical records up to date. This yearly visit will also help determine if any adjustments or changes in medication are needed.
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The information contained herein may not be construed as medical advice. It is for educational purposes only. Diplomat Pharmacy Inc. takes no responsibility for the accuracy or validity of the information contained herein, nor the claims or statements of any manufacturer. Copyright Š 2016 by Diplomat Pharmacy Inc. Diplomat and Diplomat Specialty Infusion Group are either trademarks or registered trademarks of Diplomat Pharmacy Inc. All rights reserved. 005113-0816
VON WILLEBRAND DISEASE Patient Guide
What is von Willebrand disease?
Types of von Willebrand disease2
Von Willebrand disease (VWD) is an inherited bleeding disorder that affects 1–3 percent of the general population.1
TYPE 1
When a person has VWD, their blood does not clot properly due to a decrease in the amount of, or a flaw in the von Willebrand factor. This protein helps platelets stick together and is necessary for blood to clot properly.
This is the mildest type of von Willebrand disease and represents 70–80 percent of all cases. People with type 1 have a decreased amount of von Willebrand factor in their blood. They usually live a normal lifestyle and tend to only have bleeding concerns with severe injuries, surgeries or dental procedures.
TYPE 2
There are four subtypes of type 2 von Willebrand: •
Type 2A: von Willebrand protein is defective and does not glue platelets together like it should.
•
Type 2B: the von Willebrand factor is defective and causes platelets to clot in the blood stream and not at the site of injury. Treatment of type 2B von Willebrand disease differs from the treatment for all other types.
•
Type 2M: von Willebrand factor is defective and not able to bind platelets together to form a clot.
•
Type 2N: the von Willebrand factor is able to bind platelets, but does not carry the factor VIII properly, which is another protein that helps blood clot. Without factor VIII, type 2N patients cannot form clots—much like patients with hemophilia. Therefore, many patients with type 2N are misdiagnosed.
Treatment •
Small cuts and bruises should be cleaned and covered with a bandage.
•
Bleeds inside the body are usually treated by medication or factor replacement therapy. Treatment might need to continue for several days, or until the bleeding stops.
TYPE 3
People with type 3 von Willebrand disease have very little von Willebrand factor in their blood. This can cause severe bleeding episodes similar to those with severe hemophilia. Type 3 is the rarest form, occurring in 1–3 patients per 1,000,000. Centers for Disease Control and Prevention, “Von Willebrand disease, data & statistics,” May 2014, http://www.cdc.gov/ncbddd/vwd/data.html www.ninds.nih.gov/disorders/lambert_eaton/lambert_eaton.htm 2, Lillicrap, D., James, P., World Federation of Hemophilia, “Treatment of Hemophilia,” January 2009. 1
SYMPTOMS: • • • • •
Recurrent nose bleeds Easy bruising Heavy or prolonged menstrual flow Bleeding from digestive or urinary tract Unusual or excessive bleeding from mouth or gums • Unusual bleeding after surgery, dental procedures and injury • Recurrent spontaneous bleeding anywhere