Painting the Picture: HAE Types and Management Approaches

Today on “Swell Conversations: A Promotional HAE Series,” John Anderson, MD, clinical assistant professor at The University of Alabama at Birmingham and partner at AllerVie Health, welcomes Douglas H. Jones, MD, practicing physician at the Tanner Clinic in Layton, Utah. In this episode, they break down the three types of hereditary angioedema (HAE) and share their personal approaches to disease management. Special attention is given to exploring HAE when C1 inhibitor (C1-INH) levels and function are deemed normal (ie, HAE-nl-C1INH). Drs Anderson and Jones also discuss the possible role that RUCONEST® (C1 esterase inhibitor [recombinant]) may play in HAE treatment regimens.

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WHAT IS RUCONEST? 

RUCONEST® (C1 esterase inhibitor[recombinant]) is indicated for the treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE). Effectiveness in clinical studies was not established in HAE patients with laryngeal attacks.

IMPORTANT SAFETY INFORMATION 

RUCONEST is contraindicated in patients with a history of allergy to rabbits or rabbit-derived products and for patients with a history of life-threatening immediate hypersensitivity reactions, including anaphylaxis, to C1 esterase inhibitor (C1-INH) preparations.

Monitor patients for early signs of allergic or hypersensitivity reactions (including hives, generalized urticaria, tightness of the chest, wheezing, hypotension, and/or anaphylaxis). If symptoms occur, discontinue RUCONEST and administer appropriate treatment.

Serious arterial and venous thromboembolic (TE) events have been reported with plasma-derived C1-INH products. Risk factors may include the presence of an indwelling venous catheter/access device, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives or certain androgens, morbid obesity, and immobility. Monitor patients with known risk factors for TE events during and after RUCONEST administration.

Appropriately trained patients may self-administer RUCONEST upon recognition of an HAE attack. Advise patients to seek medical attention if progress of any attack makes them unable to properly prepare or administer a dose of RUCONEST. No more than 2 doses should be administered within a 24-hour period.  

The serious adverse reaction reported in clinical trials was anaphylaxis. The most common adverse reactions (incidence ≥2%) were headache, nausea, and diarrhea.  

Before prescribing RUCONEST, please read the full Prescribing Information including the Patient Product Information. 

DISCLAIMER 

This educational program is sponsored by Pharming Healthcare, Inc. The speakers have been compensated for the presentation of this information. The information contained within this podcast is for educational purposes only and is not intended to be medical advice. Patient experiences may be discussed in this episode. It is important to recognize that these experiences may not be representative, as every patient has a unique disease course. This activity is not intended for Continuing Medical Education credits.

EPISODE REFERENCE LIST 

1. Zafra H. Hereditary angioedema: a review. WMJ. 2022;121(1):48-53. doi:10.1016/j.jaip.2020.08.046 
2. Busse PJ, Christiansen SC, Riedl MA, et al. US HAEA Medical Advisory Board 2020 guidelines for the management of hereditary angioedema. J Allergy Clin Immunol Pract. 2021;9(1):132-150.e3. doi:10.1016/j.jaip.2020.08.046 
3. Jones DH, Bansal P, Bernstein JA, et al. Clinical profile and treatment outcomes in patients with hereditary angioedema with normal C1 esterase inhibitor. World Allergy Organ J. 2022;15(1):100621. doi:10.1016/j.waojou.2021.100621 
4. Jacobs J, Neeno T. The importance of recognizing and managing a rare form of angioedema: hereditary angioedema due to C1-inhibitor deficiency. Postgrad Med. 2021;133(6):639-650. doi:10.1080/00325481.2021.1905364 
5. Riedl MA, Danese M, Danese S, et al. Hereditary angioedema with normal C1 inhibitor: US survey of prevalence and provider practice patterns. J Allergy Clin Immunol Pract. Published online January 30, 2023. doi:10.1016/j.jaip.2023.01.023 
6. Bova M, Suffritti C, Bafunno V, et al. Impaired control of the contact system in hereditary angioedema with normal C1-inhibitor. Allergy. 2020;75(6):1394-1403. doi:10.1111/all.14160 
7. Zanichelli A, Longhurst HJ, Maurer M, et al. Misdiagnosis trends in patients with hereditary angioedema from the real-world clinical setting. Ann Allergy Asthma Immunol. 2016;117(4):394-398. doi:10.1016/j.anai.2016.08.014 
8. Ruconest. Prescribing information. Pharming Healthcare Inc; 2020. 
9. Jones D, Zafra H, Anderson J. Managing diagnosis, treatment, and burden of disease in hereditary angioedema patients with normal C1-esterase inhibitor. J Asthma Allergy. 2023;16:447-460. doi:10.2147/JAA.S398333