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ISSN: 2155-9864

Journal of Blood Disorders & Transfusion
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Research Article

Treatment and Management Patterns of Patients with Von Willebrand Disease in the United States

Cindy A Leissinger1* and Diane Nugent2

1Section of Hematology/Oncology, Tulane University School of Medicine, New Orleans, LA, USA

2PSF Hematology, Children’s Hospital of Orange County, Orange CA, USA

Corresponding Author:
Cindy A Leissinger
Section of Hematology/Oncology
Tulane University School of Medicine
1430 Tulane Avenue, Box SL-78
New Orleans, LA 70112, USA
Tel: 504 988-5433
Fax: 504-988-3508
E-mail: [email protected]

Received Date: May 20, 2013; Accepted Date: August 07, 2013; Published Date: August 09, 2013

Citation: Leissinger CA, Nugent D (2013) Treatment and Management Patterns of Patients with Von Willebrand Disease in the United States. J Blood Disord Transfus 4:152. doi: 10.4172/2155-9864.1000152

Copyright: © 2013 Leissinger CA, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract

Background: Few data exist regarding patient management strategies for von Willebrand disease (VWD), the most frequently inherited bleeding disorder. The objective of this study was to retrospectively assess patterns of VWD patient management in the United States.

Methods: 41 physicians were recruited using the Centers for Disease Control and Prevention’s list of hemophilia treatment centers (HTCs) and the American Medical Association’s physician database (16 HTC-, 25 non-HTC-affiliated). Patient charts were randomly sampled by quota method.

Results: Charts of 225 VWD patients treated from 1/2005–12/2007 were reviewed (94/74/57 for VWD types 1/2/3, respectively). The proportion of white patients was higher at non-HTCs than HTCs, and among type 1 than type 2 patients. Females comprised 65% of patients, (77% of type 1, 55% of type 2, and 58% of type 3 patients) However, bleeding episodes requiring treatment occurred in 53% of male but only 36% of female patients. The proportion of patients treated with desmopressin or von Willebrand factor/factor VIII (VWF/FVIII) concentrates for bleeding (148 events) and surgery (140 episodes) within VWD type were: 31% and 47% of type 1, 55% and 36% of type 2, 44% and 39% of type 3 patients, respectively. Of the 140 surgical events requiring desmopressin or VWF/FVIII concentrates, 61% occurred in the non-HTC setting.

Conclusion: In addition to revealing interesting disparities in VWD patient demographics, these data indicate VWD patients receive care generally aligned with treatment guidelines, with the exception of the number of surgeries performed outside of the HTC setting, where access to laboratory monitoring of factor levels may be limited.

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