Published Online: September 21, 2016
Hereditary angioedema (HAE) is a chronic condition that occurs in approximately 1/10,000 to 1/100,000 people. The main symptoms are intermittent, recurrent swelling episodes of skin and mucosa (including the extremities, face, and airway). Given the unpredictable and potentially life-threatening nature of these swelling episodes, emergency care is a significant and essential component of HAE management. However, 70% of HAE patients (n=149) surveyed at the 2013 HAE Association Patient Summit reported that they were not satisfied with their emergency department (ED) care.
In an original article in The Journal of Allergy and Clinical Immunology: In Practice (JACI: In Practice), Otani and colleagues conducted survey research to identify specific areas of emergency care that need improvement from patients’ perspectives. Responses to a 30-question survey were collected from 118 patients with self-reported Type I or Type II HAE in attendance at the 2015 HAE Association Patient Summit. As the survey was anonymous and voluntary, some patients did not answer all 30 questions.
Nearly all patients (99%, 104/105) who had required ED care for an HAE attack reported that knowledge regarding HAE in the ED needed improvement. When asked what specific areas of knowledge needed improvement, 48% (50/105) chose recognition of HAE as a diagnosis, 59% (62/105) chose understanding of effective HAE medications, and 45% (47/105) chose appreciation of the serious nature of HAE attacks. Despite the fact that effective HAE therapies started to become available in 2009, only 65% of patients who had required ED care for an HAE attack before and after 2009 answered that they perceived improvements in ED care. Indeed, 19 patients reported that they did not receive an effective HAE therapy during an ED visit after 2009. This suggests that improving recognition of HAE and administration of effective HAE therapy in the ED are essential to ensure appropriate emergency care for HAE patients.
Having a treatment plan or a written emergency plan provided by an allergist/immunologist were associated with a higher likelihood of receiving appropriate HAE therapy in the ED. There is room for improvement in these areas as 8% (10/118) of patients did not have a treatment plan and 50% of patients did not have a written emergency plan. Furthermore, among 68 patients who reported that their treatment plan was to receive on-demand HAE therapy at home, 26 (38%) required ED care after 2009 due to an inability to access or receive their home on-demand therapy (28%, 26/68). This demonstrates the importance of allergists working closely with patients to ensure treatment plans are effective.
This is the first time a large United States population of HAE patients has been surveyed regarding their experience with emergency care of HAE attacks. This study demonstrates that there is room for improvement in emergency care of HAE attacks with a focus on HAE awareness and administration of effective HAE therapies. These findings highlight the importance of HAE education and care provided by allergist/immunologists and emergency physicians.
The Journal of Allergy and Clinical Immunology: In Practice is an official journal of the AAAAI, focusing on practical information for the practicing clinician.