Practice Patterns in Hospitalized Adult Patients with Hemophilia: A Survey Study of US Hemophilia Centers
Authors: Khan Lubna , Elstrott Benjamin K , Raghunathan Vikram , Olson Sven Reid , Shatzel Joseph J 
Published: November 2020

Introduction:The care of patients with hemophilia (PwH) is rapidly evolving, with novel uses for existing clotting factor concentrates (CFC) and the introduction of non-CFC hemostatic agents (e.g. emicizumab) recently approved by the Food and Drug Administration. However, inpatient care of PwH hospitalized for elective surgery or major bleeds does not follow standardized protocols, and the manner and extent to which novel therapeutic strategies are applied is unknown.
Objective:We performed a nationwide survey to obtain information on the practice patterns of adult hematology providers practicing at accredited U.S. hemophilia treatment centers (HTC) in the inpatient care of PwH in the perioperative setting.
Methods:Using a publicly available list of accredited HTC adult providers, 59 providers were contacted by email with the option to participate in a phone call or online survey. We aimed to collect information regarding the perioperative management of patients on emicizumab, postoperative factor level monitoring, type of CFCs routinely utilized in the inpatient setting, hospital length of stay, and use of venous thromboembolism (VTE) prophylaxis in the postoperative setting. All non-categorizable responses were excluded.
Results: Providers from 10 out of 59 hemophilia centers contacted across the U.S. participated in a phone survey (n=5) or online survey (n=5). Responses were collected from providers practicing in the Mountain States (n=2), Southeast (n=2), Mid-Atlantic (n=2), New England (n=2), Northern States (n=1), and undisclosed (n=1).
Of the participating providers, 80% had experience managing patients on emicizumab who had undergone elective surgery or experienced major bleeding. Perioperative management of patients undergoing elective surgery on emicizumab by center was as follows: a single pre-operative bolus of FVIII (n=1), unmonitored weight-based CFC for the same duration as patients not on emicizumab (n=5), and monitored weight-based CFC using a bovine chromogenic assay for the same duration as patients not on emicizumab (n=2). One provider who had not yet cared for patient on emicizumab undergoing elective surgery anticipated that they would preemptively discontinue emicizumab and treat with CFC alone perioperatively.
Ninety percent of providers monitored factor levels in hospitalized PwH after elective surgeries; length of monitoring varied between 1-2 days (n=3), 4-5 days (n=1), daily or every other day while inpatient (n=4), or through utilization of surgical site dependent monitoring (n=1). Of those who monitored factor levels, 67% measured both peaks and troughs, while 33% monitored troughs alone. For postoperative inpatient CFC infusion, 60% of providers used standard half-life products alone, while 30% used both standard and extended half-life (EHL) products for hospitalized PwH, generally with the EHL product given preoperatively. Forty percent of providers employed bolus CFC delivery alone while 60% used a combination of bolus and continuous infusions in their practice.
Mean length of hospital stay after elective surgery varied between centers and ranged from 1-2 days up to 5-7 days depending on factor levels, pain management, and/or physical therapy needs. Length of stay was highly variable for major bleeds and non-elective surgeries depending on severity of illness and location of bleed.
VTE prophylaxis was not given postoperatively by 70% of providers, another 20% of providers reported they may conditionally consider it, and the remaining 10% prescribed either enoxaparin or apixaban to all PwH on CFCs getting elective surgery while hospitalized.
Conclusions:Practice patterns of medical providers caring for PwH hospitalized for elective surgery or major bleeding are highly variable across institutions. While the majority of participating providers reported monitoring factor levels in inpatients, the length of monitoring and measurements of peaks/troughs varied. EHL products are being used occasionally in hospitalized patients. The majority of providers do not prescribe VTE prophylaxis inpatient. Additional studies are required to clarify the optimal management strategy in this complex patient population.

Disclosures
Shatzel: Aronora, Inc.:Consultancy.