Hereditary Angioedema


“HAE: Learn About It, Talk About It” to Improve Care for Patients with Hereditary Angioedema (HAE)


As  emergency medicine physician assistants,  you may see patients that present with vague symptoms, such as acute attacks of abdominal pain accompanied by nausea, vomiting and/or diarrhea, or unexplained recurrent bouts of these symptoms.  You might diagnose these symptoms as appendicitis, bowel ischemia, inflammatory bowel disease or irritable bowel syndrome, but it could be something else entirely. 

HAE is a rare, severely debilitating, life-threatening genetic disorder caused by a deficiency of C1 inhibitor, a human plasma protein. This condition is the result of a defect in the gene controlling the synthesis of C1 inhibitor. C1 inhibitor maintains the natural regulation of the contact, complement, and fibrinolytic systems, that when left unregulated, can initiate or perpetuate an attack by consuming the already low levels of endogenous C1 inhibitor in HAE patients. Patients with C1 inhibitor deficiency experience recurrent, unpredictable, debilitating, and potentially life threatening attacks of inflammation affecting the larynx, abdomen, face, extremities and urogenital tract.

As front-line providers, it’s vital that you know the signs and symptoms of HAE.  For undiagnosed patients, a lack of awareness can pose a danger because every minute counts in diagnosing HAE and because HAE symptoms can mimic other conditions.  For patients who know they have HAE, it can be frustrating if emergency department diagnosticians don’t know about or understand how to manage the condition.

HAE does not respond to drugs that treat other types of allergic reactions, such as such as antihistamines, epinephrine and corticosteroids, and  if a facial or laryngeal attack is left untreated for too long, patients can experience upper airway edema that may lead to asphyxiation.  Unfortunately, mortality related to laryngeal edema and asphyxiation has been reported in up to 30 percent of HAE patients who were previously undiagnosed and untreated.

SEMPA has joined a growing collaboration of professional organizations in “HAE: Learn About It, Talk About It,” a peer-to-peer clinician education program aimed at improving the accurate diagnosis and appropriate management of patients with HAE.   As part of the collaboration, you now have access to tools and resources to help you identify and manage HAE in your ED:

  • In-depth information  about HAE at www.LetsTalkHAE.com 
  • Podcast episode  with Dr. Sandra Schneider, Professor of Emergency Medicine, University of Rochester, and President of the American College of Emergency Physicians
  • HAE Webinar  
  • iPhone® application called “LetsTalkHAE” 
  • Emergency Medicine case studies

“HAE: Learn About It, Talk About It” is a growing collaboration among the American College of Allergy, Asthma and Immunology (ACAAI), the American Gastroenterological Association (AGA) Institute, the World Allergy Organization (WAO), the American College of Emergency Physicians (ACEP), the Society of Emergency Medicine Physician Assistants (SEMPA), and the American Academy of Nurse Practitioners (AANP).

 

The program is supported by ViroPharma Incorporated.