von Willebrand Disease

von Willebrand disease is the most commonly-known bleeding disorder among males and females alike.

vWD is 100 times more prevalent than hemophilia. 60 to 120 million worldwide (one to two percent of the world’s population) has vWD . It is the most common bleeding disorder affecting 1 in 100 individuals, it is also the most under diagnosed bleeding disorder.

von Willebrand Disease is

Our body’s blood contains multiple parts. One part called platelets, is a round, sticky cell that helps plug or seal off tears in blood vessels. When a blood vessel is injured, platelets rush to the tear in the blood vessel. They stick to each other and to the wall of the blood vessel forming a platelet-plug that helps stop the bleeding. While there are many different bodily substances involved in forming the platelet plug, the most important is the vWD protein that works like a “platelet glue.”

A person will have vWD when there is not enough von Willebrand factor (protein) present in the body or when there is a malfunction of the von Willebrand factor (protein).

TYPES OF vonWILLEBRAND’S DISEASE

Type 1 von Willebrand Disease

• Affects about 70% to 80% of the people with vWD.
• Type 1 represents a low level of vWD factor.

Type II von Willebrand Disease

• Affects 15% to 30% of people with vWD. In Type II, the blood produces enough von Willebrand, but it cannot be used properly. There are four subtypes of Type II. (2A, 2B, 2M and 2N)

Type III von Willebrand Disease

• Type III is the rarest and most severe form. In Type III the body essentially produces no vWD factor.

Symptoms of von Willebrand Disease

• Easy Bruising
• Frequent nosebleeds that last a long period of time
• Heavy or prolonged menstrual bleeding
• Prolonged bleeding following surgery, dental work, childbirth or injury.

TREATMENT

Frequently used drugs to treat vWD include:

DDAVP
DDAVP is a hormone treatment that comes in both injection and nasal form.
(The nasal form is called STIMATE.)

DDAVP stimulates the release of vWF in the blood vessel linings.

Female hormones are often prescribed to control menstrual bleeding.

Topical agents such as Gelfoam or Surgicel are used to assist with clot formation.

Amicar helps prevent clots from being dissolved.

Factor VIII Replacement Products.
Humate –P, Alphanate and Koate DVI in severe situations.

Current Recombinate VIII products are not effective in the treatment of vonWillebrand Disease, as they do not contain the vWF; therefore plasma-derived products are most commonly used.

DIAGNOSIS

vWD is complicated and takes many forms with different subtypes, and not all laboratories can perform the required testing.

FactorHealth Management recommends a thorough evaluation by a trained hematologist who specializes in bleeding disorders.

SUPPORT

LadyBugs
www.ladybugsupport.com

Connect with your local Hemophilia Treatment Center for comprehensive medical services.

Join your local bleeding disorders chapter

allaboutbleeding.com (your on-line vWD resource)

The information provided on this website in no way replaces the information and advice provided by a health care professional. A doctor, nurse or Hemophilia Treatment Center should be contacted regarding the diagnosis and treatment of any medical condition.

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