Improving HAE Patient Care through an Interactive Patient Simulation Platform

Initiative Rationale

Among 39 patients who required emergency department care within the last year, 6 did not receive any HAE-targeted therapy, and treatment with corticosteroids (n = 3), epinephrine (n = 2), and antihistamines (n = 7) was reported. The emergency department (ED) is lacking in terms of a structured approach to distinguishing the different etiologis of angioedema. specifically differenting histaminergic-mediated angioedema from bradykinin-mediated angioedema. Based on data from the National Hospital Ambulatory Medical Care Survey (NHAMCS), there are as many as 80,000 to 112,000 ED visits for angioedema annually. About 18% of ED visits coded as angioedema result in hospitalization. 

(A consensus parameter for the evaluation and management of angioedema in the emergency department. Moellman JJ, Bernstein JA, Lindsell C. Acad Emerg Med. 2014      Apr; 21(4): 469–484.doi:  10.1111/acem.12341; Emergency Department Management of Hereditary Angioedema Attacks: Patient Perspectives. Otani IM, Christiansen SC, Busse P, Camargo CA Jr, Zuraw BL, Riedl MA, Banerji A. J Allergy Clin Immunol Pract. 2017 Jan - Feb;5(1):128-134.e4. doi: 10.1016/j.jaip.2016.06.029. Epub 2016 Sep 21.)

Regarding the long-term care of a patient diagnosed with HAE, it is critical that physicians and patients work together to design individualized treatment plans that optimize care for each patient. Over the past several years, multiple novel medications have been approved in the United States for the treatment of HAE all with very different modes of action, methods of administration, and risk-to-benefit profiles. The complexity of the available treatment options combined with the potential morbidity and mortality of HAE necessitate that the physician managing the long term care of their HAE patient develop an evidence-based plan that reflects the appropriate standard of medical care. 

(Hereditary Angioedema Association Medical Advisory Board 2013 recommendations for the management of hereditary angioedema due to C1 inhibitor deficiency. Zuraw BL, Banerji A, Bernstein JA, Busse PJ, Christiansen SC, Davis-Lorton M, et al. J Allergy Clin Immunol: In Practice 2013;1:458-67.

Elements of the Identified Educational Gap include:

  1. Clinicians in the emergency department setting are frequently unaware of HAE as a disorder
  2. Clinicians in the emergency department setting do not include HAE in their differential diagnosis of patients presenting with angioedema
  3. Clinicians in the emergency department setting frequently do not view HAE as a life-threatening disorder if not recognized and treated appropriately.
  4. Clinicians in the primary care setting lack awareness and a working understanding of managing HAE on a long-term basis.