Emergency department (ED) providers are faced with unique difficulties relevant to achieving optimal factor therapy dosing with their hemophilia patients. While certain ED-related barriers might appear inherent and intractable given the clinical setting, investigators at the Nationwide Children’s Hospital (NCH) in Columbus, OH sought to identify the key challenges and develop remedies for enhancing outcomes.The lead author of the study was Amy Dunn, MD, director of the hemophilia treatment center at NCH.

Dunn and her colleagues looked at pediatric hemophilia patients who presented with bleeds at the ED of NCH’s main campus. To establish baseline data, they conducted a chart review of all hemophilia A and B patients who visited the ED for injuries that necessitated the use of factor from the period of September 2015 to August 2016. Injuries were classified as either minor or major, requiring 50% factor level correction in cases of the former and 100% correction in cases of the latter. Because of the variation in vial sizes for a myriad of factor products, the optimal dosing range was defined as 90%‐120% of the “ideal dose.” Investigators then compared the ideal dose with actual doses administered during the study period.

The baseline results showed that the NCH ED achieved the ideal dose range of 70% during this time period. They found no difference between those patients with hemophilia A or B in frequency of out-of-range dosing. Further, there was no difference in frequency of out-of-range dosing dosing between patients treated with standard factor VIII or IX products and extended half-life factor VIII or IX products. The authors observed a trend of under dosing that took them somewhat aback. “Surprisingly, of the out‐of‐range encounters in the baseline period, there were more encounters where under dosing <90% (six encounters, 66.7%) occurred than over dosing >120% (three encounters, 33.3%) and the frequency towards under dosing persisted throughout the study period.”

After reviewing the baseline data, Dunn and her team set out to increase the number of patient encounters between encounters where factor dosing was outside 90-120% of the ideal dose from 4 encounters to 15. A multidisciplinary team of hematologists, an ED provider and a pharmacist ultimately identified three “key drivers” that affect whether optimal doses are administered in the ED, including injury categorization/dosing challenges, ED workflow challenges and product limitations.

Uncertainty relevant to bleeding categorization (major vs. minor) was found to be closely linked to dosing challenges. While there exist institutional guidelines on such classifications from the World Federation of Hemophilia, the NCH team found that these documents were not readily accessible outside the hematology/oncology (hem/onc) department or to additional staff outside the hospital. To improve access to the existing guidelines, investigators conducted three main interventions: 1) In-service overview on both injury types/optimal dosing for hem/onc fellows and pharmacists 2) Electronic posting of institutional guidelines in easily accessible areas for hem/onc fellows; these would not require remote access, or a hospital issued computer 3) Staffing the ED with an emergency medicine clinical pharmacist daily between 7:30am-1:30am. Because the remaining time is covered by a general pharmacist, the guidelines were strategically placed in the pharmacy near where factor products are stored.

EDs within academic institutions face inherent workflow challenges, often related to regular trainee rotations in-and-out of the department. The frequent turnover can make it difficult to sustain the impact of educational messaging over time. The NCH team addressed this first by developing a hemophilia-specific injury order set for the ED, as an additional reference and support for providers making clinical decisions in an emergency setting. The order included links to institutional guidelines which were previously unavailable on a hem/onc shared drive. Once the order sets were implemented, follow up communications were carried out to reinforce dosing’s clinical significance and financial impact to both the ED and hem/onc departments. In addition, hemophilia guideline reviews were added to ED staff meetings and in-services. Pharmacists took the lead on product limitations, first by endeavoring to provide a factor dose as close as possible to that prescribed. Due to inherent challenges of dosing with varying vial sizes, they also tracked the inevitable wasted factor in cases where available dose “significantly exceeded” the dose prescribed.

Ultimately, upon identifying key drivers and implementing tools for enhancing dosing outcomes in the study period (November 2016 to July 2017), Dunn and her team surpassed their goal by reaching 16 encounters between non-ideal dosing, up from the baseline of 4 encounters. They also improved “ideal” range average from 70% to 83%.

Study limitations were cited, including a reliance on EMR documentation to determine the type/severity of a bleed. This can paint an incomplete picture as it does not account for the varying ways in which a provider might document a bleeding episode, which could in turn inform dose determination. Further discussion of limitations centered both on a lack of sustainability – success did not carry over after study period – and future opportunities for education/collaboration.

“Future goals to reinvigorate ED staff and capture new hires include, continued education at quarterly ED in‐services and ED staff meetings. In addition, Plan‐Do‐Study‐Act cycles are planned to target education for rotating ED house staff as well as implementing annual education for the haematology fellows to capture new trainees,” concluded the authors.

Drayton Jackson M, Dunn M, Storey M, Dunn A. Increasing Optimal Coagulation Factor Dosing in the Paediatric Emergency Department: A Quality Improvement Study. Haemophilia. 2019 Feb 12. doi: 10.1111/hae.13693.

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