About von Willebrand Disease—What is von Willebrand Disease?

Von Willebrand disease (VWD) is the most common inherited coagulation disorder in humans.¹ It occurs in about 1% of the general population.²

What is VWD?
VWD is an inherited bleeding disorder caused by an insufficient amount of von Willebrand factor (VWF) protein in the blood, or VWF that is defective. Because VWF is essential in primary hemostasis, an insufficient amount or a defective supply of VWF interferes with proper platelet adherence or aggregation.³

Facts about VWD

VWD is the most common inherited coagulation disorder—affecting approximately 1% of the world’s population^1,2
It occurs at the same rate in males and females¹
The disease is marked by an absence of or defective VWF³
In most cases, VWD is genetically passed from parent to child; in rare cases, VWD can be acquired⁴

Important Safety Information

Humate-P is indicated for treatment and prevention of bleeding in adult patients with hemophilia A (classical hemophilia). Humate-P is also indicated in adult and pediatric patients with von Willebrand disease (VWD) for (1) treatment of spontaneous and trauma-induced bleeding episodes, and (2) prevention of excessive bleeding during and after surgery. This applies to patients with severe VWD, and patients with mild and moderate VWD for whom use of desmopressin is known or suspected to be inadequate. Humate-P is not indicated for the prophylaxis of spontaneous bleeding episodes.

Humate-P is contraindicated in individuals with a history of anaphylactic or severe systemic response to antihemophilic factor or von Willebrand factor preparations.

Monitor for intravascular hemolysis and decreasing hematocrit values in patients with A, B, and AB blood groups who are receiving large or frequent doses. Also monitor VWF:RCo and FVIII levels in VWD patients, especially those undergoing surgery.

Thromboembolic events have been reported in VWD patients receiving coagulation factor replacement. Caution should be exercised and antithrombotic measures considered, particularly in patients with known risk factors for thrombosis.

Humate-P is derived from human plasma. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, cannot be completely eliminated.

In patients receiving Humate-P in clinical studies for treatment of VWD, the most commonly reported adverse reactions observed by >5% of subjects are allergic-anaphylactic reactions, including urticaria (hives), chest tightness, rash, pruritus (itching), and edema (swelling). For patients undergoing surgery, the most common adverse reactions are postoperative wound or injection-site bleeding, and epistaxis (nosebleed).

Please see full prescribing information.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

The information provided herein is solely for use by physicians and healthcare professionals in the United States. The CSL Behring product listed may not have been approved in other countries and may not be available everywhere.

References

National Heart, Lung, and Blood Institute Diseases and Conditions Index. Who gets von Willebrand disease? Available at: http://www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_WhoIsAtRisk.html. Accessed April 29, 2005.
Rodeghiero F, Castamon G, Din F. Epidemiological investigation of the prevalence of von Willebrand disease. Blood. 1987;69:454-459.
National Heart, Lung, and Blood Institute Diseases and Conditions Index. What is von Willebrand disease? Available at: http://www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_WhatIs.html. Accessed April 29, 2005.
The “Other” Bleeding Disorder. Available at: http://www.haemophilia.org.za/Vonwil2.htm. Accessed April 15, 2005.