Factor First: An Assessment of Emergency Department Care of Patients with Hemophilia

Background: With the establishment of hemophilia treatment centres (HTCs) and home infusion programs for factor replacement therapy, emergency department use among patients with hemophilia occurs less frequently, with patients often presenting for significant bleeding events only. As a result, the management of patients with hemophilia can represent a challenge to emergency department (ED) physicians and staff. Both delays in diagnosis of a bleeding event, and timely administration of hemostatic therapy have been associated with patient morbidity and mortality. The World Federation of Hemophilia, in addition to other organizations, emphasize the importance of timely receipt of clotting factor replacement therapy, providing treatment at the time a bleed is suspected. If a patient with hemophilia is registered with a HTC, they often carry a wallet card, or have a formalized treatment plan, outlining their diagnosis and emergency treatment of bleeding recommendations to ensure timely access to hemostatic therapy. The aim of this study was to describe ED management of patients with hemophilia at a single Canadian HTC, with a focus on use of individualized emergency bleeding management protocols, and time to receipt of hemostatic therapy within the ED.

Methods: Adult patients with hemophilia A or B (FVIII or FIX ≤40%), of any severity, followed at the Southern Alberta Rare Blood and Bleeding Disorders Comprehensive Care Program, were identified for inclusion. ED health utilization and ED encounter management data for each of the four hospital sites in Calgary, AB was obtained from the electronic health record for the cohort, through to 2022.

Results: A total of 191 patients with hemophilia A and B were identified for study inclusion. 153 patients with hemophilia A (mild 64.7%; moderate 5.9%; severe 28.8%) and 38 patients with hemophilia B (mild 44.7%; moderate 36.8%; severe 18.4%). Males comprised 77.5% of the patient cohort. A total of 393 ED visits occurred over the study period. Less than a third of ED visits were associated with receipt of hemostatic therapy (either desmopressin or clotting factor concentrate). The median time from triage to ordering of hemostatic therapy was 2.9 hrs (IQR 2.3-3.2), and median time from triage to administration of hemostatic therapy was 4.2 hrs (IQR 3.1-4.2). For ED visits due to suspected bleeds, the proportion of patients who received either coagulation factor replacement or desmopressin within two hours of ED triage is depicted in Figure 1. Time to receipt of hemostatic therapy did not significantly differ whether desmopressin or clotting factor concentrate was administered. The number of ED visits in which the individualized emergency bleeding management protocol was accessed is depicted in Figure 2. Among those accessed in 2022, the proportion of protocols that were updated within the last 365 days was 86.8%. Less than 20% of ED visits were associated with a hematology consultation.

Conclusions: Increasing utilization of individualized emergency bleeding management protocols for patients with hemophilia was observed; however, significant delays in timely receipt of hemostatic therapy remains, with infrequent involvement of hematology in consultation. Time to receipt of potentially life-saving hemostatic therapy for our cohort falls outside of hemophilia care standards. This data provides a better understanding of the experience of patients with hemophilia in the ED and provides an opportunity to define further quality improvement interventions to ensure quality care metrics in this population are met.