Advancing HAE Management: Mastering Personalized Care with Guideline-Driven Strategies

  Challenges in the Management of Difficult-to-Treat HAE

Transcript

Announcer:

Welcome to CME on ReachMD. This episode is part of our MinuteCE curriculum. Prior to beginning the activity, please be sure to review the faculty and commercial support disclosure statements as well as the learning objectives.”

Dr. Buttgereit:

This is CME on ReachMD. I'm Dr. Thomas Buttgereit, and I'm not alone today. Here with me is Dr. Anna Valerieva. Our topic today is challenges in the treatment and management of difficult-to-treat HAE.

So, Anna, I think that it’s a very challenging question I will ask you now. What do you understand by the definition of difficult-to-treat HAE? 

Dr. Valerieva:

Thank you, Thomas. I think this is a great and really challenging question because we really do not have a clear definition on the topic of difficult-to-treat HAE.From my perspectiveI wouldargue that difficult-to-treat HAE is not always severe HAE. So difficult-to-treat HAE surely, are the patients with severe HAE who tend to have severe and frequent HAE attacks, and they might be put on prophylaxis. But for me, difficult-to-treat HAE are those patients who would have breakthrough attacks, and they will have, also, challenges inrecognizing the attacks, or challenges in treating those acute breakthrough attacks under prophylaxis.

But I think difficult-to-treat HAE might be, also, patients who tend to have certain comorbidities, and those comorbidities might also beinfluencing the course of HAE. So something in my approach, usually, is to check the patients with difficult-to-treat HAE for underlying diseases and to control those diseases, and whether they might have some benefit fromoptimizing their patient carewith regards to another disease.

Difficult-to-treat HAE, I wouldalso add, are the patients who tend to not recognize the HAE attacks.I don't know if you wouldalso agree from your practice, that no matter how much we discuss with the patients from time to time, I tend to have a patient in the clinic that do not recognize the abdominal attacks or are not always sure whether this is an HAE attack, or something related to gastritis or some other abdominal crisis.And this is indeed a true challenge in our HAE practice on a daily basis, that we really do lackthebiomarkers that can help in such situations, to recognize whether this is an HAE attack or something else.

I also would say that difficult-to-treat HAE are the patients who have low complianceto the disease therapies. Andfrom my practice,I always see some patients thattry to negotiate the need for a blanket treatmentunder acute circumstances. Or the patients who tendto skip their prophylactic agents, and this is increasing the risk for having a breakthrough attack. 

Dr. Buttgereit:

Yeah, really, really, a lot of points you mentioned already, what you understand under this for difficult-to-treat HAE. So I think, really, it has a lot to do with compliance, with the nature of the disease itself, but also, with the treatments we have available to treat HAE. And we know that we have modern treatment options available, and many of them work perfectly, but there are still patients who faceattacks, are not happy with their treatmentbecause they still have the symptoms of HAE.

So the next question would be, then, Anna, for you. Are there any guideline recommendations on how to treat these patients with difficult-to-treat HAE?

Dr. Valerieva:

So as far as I'm aware, the guidelines are not really clear on defining what is difficult-to-treat HAE, and we really do lackspecific recommendations in those patients. So this is something that we, as HAEspecialists, should be looking forward to defining how to better manage patients withdifficult-to-treat HAE.

Dr. Buttgereit:

Yeah, I can completely understand this, dear Anna. So I think it's a work in progress.

So maybe from my point of view, many years ago, we had a different definition on how to treat HAE, what is difficult-to-treat HAE. So with the modern treatments we have now available, we have probably another more different definition of what is difficult-to-treat HAE. And I think future guidelinesshould then, more focus on this, especially on the patient populations who still face attacks. What could be the reasons, and what could be the measures, too. Yeah, find out what the reason for the attacks is, and especially, what the recommendations and how to treat and manage these patients are.

So unfortunately, our time is up now. So, Anna, it was great to have you here in this episode. Thanks for the great discussion, and I will hand over the final words to you. 

Dr. Valerieva:

Thank you, Thomas.I think something thatI would like to also underline here, is for patients with difficult-to-treat HAE,as HAE specialists, we should always be supportive to our patients and if we face such patients,maybe it isup to us to schedule more frequent visits, to always have a plan, and try to guide the patient to finding a solution.

Becausewe have the more therapies,we have different options,and I think this is something that we canadd on to their treatment.

Dr. Buttgereit:

Great. We hope this information was helpful for you and useful for your practice.

Announcer:

You have been listening to CME on ReachMD. This activity is provided by MEDCON International and is part of our MinuteCE curriculum. To receive your free CME credit, or to download this activity, go to ReachMD.com/CME. Thank you for listening.

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Overview
Hereditary angioedema (HAE) is a rare, potentially life-threatening genetic disorder characterized by recurrent episodic swelling of the skin, gastrointestinal tract, and airways. The core approach to treating HAE prioritizes the availability of effective on-demand acute therapy, early treatment to prevent attack progression, treatment of attacks, and long-term prophylaxis. Early treatment of all breakthrough attacks, regardless of severity and site, is critical to maximize efficacy and reduce morbidity and mortality. Ongoing management of HAE should include evaluation of breakthrough attacks and any concerns with the disease course and current therapy to decide if a therapy adjustment is warranted. In this educational series, expert faculty discuss the importance of early treatment and personalizing therapy, best practice for managing breakthrough attacks, and current and emerging therapies for HAE.
Disclosure of Relevant Financial Relationships
In accordance with the ACCME Standards for Integrity and Independence, MEDCON International requires that individuals in a position to control the content of an educational activity disclose all relevant financial relationships with any ineligible company. MEDCON International mitigates all conflicts of interest to ensure independence, objectivity, balance, and scientific rigor in all its educational programs.

Chair:
Danny M. Cohn, MD, PhD
Department of Vascular Medicine
Amsterdam UMC
Amsterdam, The Netherlands

Dr. Cohn has reported the following relevant financial relationships or relationships with ineligible companies of any amount during the past 24 months:
Research: Ionis, Kalvista and Pharvaris
Consulting: Astria, CSL, Ionis, Intellia, Kalvista, Otsuka,Pharvaris, Takeda

Faculty:
Thomas Buttgereit, MD
Institut für Allergieforschung IFA
Charité – Universitätsmedizin Berlin
Berlin, Germany

Dr. Buttgereitt has reported the following relevant financial relationships or relationships with ineligible companies of any amount during the past 24 months:
Consulting: CSL Behring, Kalvista, All,irall, BioCryst, Takeda
Anna Valerieva, MD, PhD
Department of Allergology
Medical University of Sofia
Sofia, Bulgaria
Dr. Valerieva has reported the following relevant financial relationships or relationships with ineligible companies of any amount during the past 24 months:
Consulting: AstraZeneca, Berlin-Chemie/Menarini Group, CSL Behring, KalVista, Novartis, Pharming, Pharvaris, Sobi, Takeda, Pharvaris
Learning Objectives
After participating in this educational activity, participants should be better able to:
Evaluate best practices in personalized hereditary angioedema (HAE) treatment approaches that consider disease severity, attack frequency, and patient lifestyle and preferences as factors in individualized treatment protocols
Demonstrate understanding of best practices in the management of acute breakthrough attacks, including consideration of underlying causes and patient-specific factors
Integrate novel therapies into existing HAE treatment plans by comparing recent advances and trial data to optimize long-term outcomes and patient care
Target Audience
This activity has been designed to meet the educational needs of allergists, hematologist and dermatologists as well as all other healthcare providers involved in managing patients with hereditary angioedema.
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This enduring activity is accredited by the European Board for Accreditation of Continuing Education for Health Professional (EBAC®) for 60 minutes of effective education time.
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Today’s healthcare environment is constantly evolving and advances of medical science occur at an accelerating pace. CME/CE plays an important role in the clinical environment and is an essential element of physician training, learning, and improvement, thereby importantly contributing to optimal patient care. Since 2000, MEDCON’s mission is to deliver high quality within the world of medical education by creating forums like PACE-CME, organizing live meetings, and providing online education. We aim to stimulate the review, exchange, and assimilation of key scientific findings to improve patients’ health, to raise awareness of new science underlying various disease states, and to accelerate the translation of this information into clinical practice.
Commercial Support
This activity is supported by an independent educational grant from Kalvista Pharmaceuticals, Inc.
Disclaimer
The views and opinions expressed in this educational activity are those of the faculty and do not necessarily represent the views of MEDCON International and PACE-CME This presentation is not intended to define an exclusive course of patient management; the participant should use his/her clinical judgment, knowledge, experience, and diagnostic skills in applying or adopting for professional use any of the information provided herein. Any procedures, medications, or other courses of diagnosis or treatment discussed or suggested in this activity should not be used by clinicians without evaluation of their patients’ conditions and contraindications or dangers in use, review of any applicable manufacturer’s product information, and comparison with recommendations of other authorities. Links to other sites may be provided as additional sources of information. Once you elect to access a site outside of PACE-CME you are subject to the terms and conditions of use, including copyright and licensing restriction, of that site.
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Publication Dates
Release Date: 11/08/2025
Expiration Date: 11/08/2026