A Retrospective Analysis of Emergency Room Management of Individuals with Von Willebrand Disease

Background: While epistaxis and menorrhagia represent the most common symptoms in those with von Willebrand disease (VWD), postoperative and post-traumatic bleeds are urgent problems often managed in emergency settings where evaluation and treatment may be delayed. In order to improve the management of such patients, we sought to determine the scope of the problem by collecting medical record data on VWD patients seeking care in a single large institution’s emergency rooms.

Methods: De-identified medical records from the Medical Archival Record System (MARS) on ICD-9 identified VWD patients in the University of Pittsburgh emergency room (ER) between January 1, 2004 and December 31, 2009 were evaluated. The study was approved as an exempt study by the University of Pittsburgh Institutional Review Board. An honest broker retrieved and de-identified all data, assigned ID numbers, and removed all linkage codes. The initial dataset of 922 notes was simplified by combining encounters into a unique dataset, and conflicting data were resolved in favor of the attending documentation. Continuous data were analyzed by student’s t test, categorical data by chi-square analysis, and significance calculated by generalized estimating equations for repeated measures.

Results: There were 385 ER visits by 183 VWD patients, mean age 34.4 years, of whom 141 (77.0%) were female (p=0.07) and 5.5% African American (p=0.017). Over half the ER visits, 204 (53.0%), were for active or suspected bleeding, while 181 (47.0%) were for non-bleeding causes. Bleeding was post-traumatic in 101 (49.0%), postoperative in 21(10.2%), epistaxis in 18 (8.7%), gastrointestinal bleeding in 18 (8.7%), gynecologic (menorrhagia, ovarian cyst rupture, spontaneous abortion) in 16 (7.8%), dental bleeding in 11 (5.3%), and hemarthrosis, hematuria, hemoptysis, hematoma, central nervous system bleeding, or medication-related bleeding in 3% or fewer. Although 77.8% of those with menorrhagia and 50.0% with epistaxis were treated with DDAVP (IV, IN) or VWF concentrate (VWF), only one-quarter, 25.7%, of all bleeds were managed with DDAVP or VWF, with no difference between groups, active vs suspected bleeds, p>0.05. The likelihood of treatment was related to referral source: among those with active bleeding, 46.4% received DDAVP (IV or IN) or VWF, including 83.3% referred by a hematology specialist, 42.9% by another specialist, 42.5% by self-referral, and 33.3% by a primary care provider. Among those with suspected bleeding, only 18.2% received DDAVP or VWF, including 90.9% of those referred by a hematologist, 21.7% by another specialist, 9.4% by self-referral, and 25.0% by a primary care provider. Few patient records indicated a known VWD type (8.2%) or DDAVP response (19.1%). Further, among those with epistaxis, nearly half, 47.1%, underwent nasal packing, and overall, non-steroidal anti-inflammatory agents were prescribed in 13.6% and aspirin in 5.1%.

Discussion: The most common reason for ER visits in VWD are traumatic or postoperative bleeds, and in most the VWD type and DDAVP response are unknown. Only 75% of active or suspected bleeds are treated with DDAVP or VWF, with significantly lower rates among primary-care, other-specialty, or self-referrals. While aspirin and heparin are commonly used agents, their use in patients with bleeding disorders may increase bleeding, as can nasal packing for epistaxis. These data suggest more information is needed to manage patients with VWD, beginning before an ER visit. This includes an initial evaluation by a hemostasis specialist, confirming the diagnosis, performing DDAVP testing, providing patient education regarding diagnosis, treatment, and formulating a treatment plan, as is standard for those with hemophilia, but not those with VWD.