QOTW – “Is it possible to build an algorithm for the ED to help improve the recognition and treatment of HAE?”

Sep 15, 2015

Dr. C: This is an excellent question. We continue to hear stories of HAE patients receiving inappropriate care even when they are armed with action plans. Sadly there are still reported deaths in the ED. Given the importance for all patients with angioedema an effort has been initiated to upgrade there cognition and care in the ED. A working group was formed with angioedema experts and ED physicians resulting in the publication ‘A Consensus Parameter for the Evaluation and Management of Angioedema in the Emergency Department’ in 2014. Marc and Bruce you were involved in this effort—do you have any feeling for the guidelines that were published and how this may translate into improved patient care?

Dr. R:This question highlights one of the real difficulties in the clinical care of HAE. Since HAE is a very uncommon condition, it’s sometimes difficult to “keep this on the radar” of the emergency department (ED), a place which is generally very good at dealing with medical emergencies, but not so much geared to deal with uncommon conditions. There certainly are places that do an excellent job of this, but most ED algorithms and protocols are designed to efficiently treat allergic/histamine-mediated angioedema. It’s difficult to fault this approach as it will successfully help >90% of patients that walk through the door with angioedema. Unfortunately, as we all know, this protocol is ineffective for HAE since the medications given (antihistamines, corticosteroids, epinephrine) don’t work for HAE symptoms. Recent publications in the emergency medicine literature have aimed to educate the specialty about HAE. Additionally, efforts are ongoing to provide educational programs for ED physicians on the various types of angioedema, including HAE, to highlight the important treatment differences.

Dr. Z:As Marc indicates, the biggest challenge for the ED is the patient with who has not yet been diagnosed. The guidelines that this joint angioedema expert-ED physician panel developed represent a compromise between the desire of the angioedema experts to initiate a diagnostic evaluation of patients presenting to the ED with angioedema and the reality of ED physicians needing to manage a large flow of patients in and out of the ED. We tried to promulgate a stepwise approach to the treatment of angioedema such that patients without a known diagnosis of HAE who do not improve with “standard” therapy will be treated for possible bradykinin-mediated angioedema. We also strongly emphasized the need to carefully manage the airway and not take any chances that patients would die from their swelling. I’m hopeful that these guidelines will become adopted more and more widely in emergency departments and thereby improve outcomes.

Dr. C:What can patients with known HAE and their doctors do to improve the experience. Should they carry written action plans to the ED?

Dr. R:I think the best course is to build each individual patient’s “algorithm”, meaning they know the steps to treat their HAE symptoms, ideally with self- or home-administration whenever possible. If it’s necessary to go to the ED for airway symptoms or additional care, then they know exactly where to go (ideally a hospital that is familiar with their diagnosis) and have with them a detailed plan of care that outlines their necessary medications, treatment, etc. This can be carried in a letter or USB drive and should include their HAE specialist’s contact info in case additional recommendations are needed. This isn’t foolproof but often works to ensure the patient isn’t “tracked” into inappropriate treatment at the ED.

Dr. C:One of the problems we still face is that even if a known HAE patient arrives at the ED with an action plan, many EDs do not stock any of the newer HAE medicines.

Dr. Z:The US HAEA can help in this situation. They maintain a geographic database that can steer patients towards an ED that stocks these medicines. In addition, the US HAEA patient service representatives can help locate medicines and assist in having them transferred to the ED where the patient is.

Dr. C:I’m hopeful that this situation will improve once the FDA approves specific drugs for the treatment of ACE inhibitor-associated angioedema since ACE inhibitor-associated angioedema and HAE are both mediated by bradykinin.

Dr. Z:Agreed. ACE inhibitor-associated angioedema is much more common than HAE and getting approval for this type of angioedema will probably lead to all EDs having at least one drug available that would work for HAE.

Dr. C:Thank you, Bruce and Marc. This was informative for the progress made and the progress that we need to make in the ED for HAE. We look forward to our next ‘Question of the Week’.

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