About von Willebrand Disease — What Is von Willebrand Disease?

An underdiagnosed condition

According to the Centers for Disease Control and Prevention (CDC), von Willebrand disease (VWD) is the most common bleeding disorder, affecting up to 1% of the US population, or more than 14 million Americans.1,2

  • Because most cases of VWD are mild, many people are asymptomatic and do not know they have a defect in their von Willebrand factor (VWF) gene3
  • VWD is equally likely to affect men and women, thought because of reproductive factors, women are more likely to be diagnosed. Nevertheless, according to a CDC survey (N=75), it takes an average of 16 years from the onset of symptoms for women to receive an accurate diagnosis1

Diagnostic considerations for von Willebrand Disease

Each patient needs individualized assessment based on his or her specific history, signs, and symptoms.4

  • One diagnostic approach is based on a personal bleeding history that is translated into a bleeding score, which can be used with laboratory tests to help diagnose von Willebrand Disease 5
  • A family history that is positive for an established bleeding disorder is frequently not present for persons who have milder forms of von Willebrand Disease 2,6

Proper testing for early diagnosis of VWD can optimize treatment and help patients live normal active lives.2

  • Identification of VWD type and subtype requires multiple laboratory tests for adequate management of the condition7

Start with an accurate assessment2

Initial evaluation for VWD

Adapted from The Diagnosis, Evaluation and Management of von Willebrand Disease. Full Report. National Heart, Lung, and Blood Institute. NIH Publication No. 08-5832. 2007.

  1. If the initial clinical evaluation suggests a bleeding disorder, initial hemostasis tests should be ordered, followed by or along with initial VWD assays indicated in the algorithm above. Referral to a hemostasis specialist is appropriate for help with interpreting and ordering repeat or specialized tests.2
  2. Correction in the PTT mixing study immediately and after 2-hour incubation removes an FVIII inhibitor from consideration. Investigation of other intrinsic factors and lupus anticoagulant may also be indicated2

CBC: complete blood count. PT: prothrombin time. PTT: partial thromboplastin time. RIPA: ristocetin-induced platelet aggregation. TT: Thrombin time. VWF:Ag: VWF antigen. VWF:RCo: VWF ristocetin cofactor activity.

www.MySourceCSL.com provides valuable information and resources for people with von Willebrand Disease and their caregivers

Important Safety Information

Antihemophilic Factor/von Willebrand Factor Complex (Human), 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 risk factors for thrombosis.

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

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

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 here is primarily intended for use by physicians and other 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.

Treatment for VWD patients

© CSL Behring 2018. The product information presented on this site is intended for US residents only. 11-HUM-082

References

  1. Centers for Disease Control and Prevention (CDC). Facts about von Willebrand disease. http://www.cdc.gov/ncbddd/vwd/facts.html Published March 20, 2015. Accessed April 27, 2015.
  2. National Heart, Lung, and Blood Institute. The diagnosis, evaluation and management of von Willebrand Disease. 2007. NIH Publication No. 08-5832. Available at www.nhlbi.nih.gov/guidelines/vwd/. Accessed July 20, 2015.
  3. Kleinert D, Orto C, Gioia K, Hannan M. Von Willebrand disease: a nursing perspective. J Obstet Gynecol Neonatal Nurs. 1997;26(3):271-276.
  4. Rodeghiero F, Castaman G, Tosetto A. How I treat von Willebrand disease. Blood. 2009;114:1158-1165.
  5. Bowman M, Mundell G, et al. Generation and validation of the condensed MCMDM-1VWD Bleeding Questionnaire for von Willebrand disease. J Thromb Haemost. 2008;6:2062-2066.
  6. Katsanis E, Luke K-H, Hsu E, Li M, Lillicrap D. Prevalence and significance of mild bleeding disorders in children with recurrent epistaxis. J Pediatr. 1988;113:73-76.
  7. Federici AB. Classification of inherited von Willebrand disease and implications in clinical practice. Thromb Res. 2009;124(suppl 1):S2-S6.
  8. Werner EJ, Abshire TC, Giroux DS, Tucker EL, Broxson EH. Relative value of diagnostic studies for von Willebrand disease. J Pediatr. 1992;121:34-38.