Rethinking the management of hereditary angioedema

<b>Background:</b> Hereditary angioedema with C1INH deficiency (HAE-C1INH) is a rare, debilitating genetic disorder characterized by recurrent, unpredictable attacks. Although treatments exist, patients with HAE still alter their lives to avoid triggers and experience substantial physical, psychosocial, and financial burdens. <b>Objective:</b> To estimate the burden that HAE-C1INH patients experience despite currently approved therapies, aiming to identify unmet needs related to HAE, its therapies and the ability to achieve normalization of life. <b>Methods:</b> A web-based survey was conducted from March to April 2025 among 100 US adults with HAE-C1INH currently receiving long-term prophylaxis and/or on-demand therapies. Responses captured attack frequency, the impact of living with HAE, avoidance of attack triggers, and the patients' unmet needs. Descriptive statistical analysis was conducted. <b>Results:</b> Even with treatment, 80% of respondents reported ≥1 HAE attack in the past year and 61% thought about HAE at least weekly. Mental health was the aspect that respondents felt was most impacted by HAE (54% of respondents), and 73% reported taking ≥2 measures to avoid attack triggers. Several concerns impacted the ability to reach normalization; lifetime use of medication was the most commonly reported concern (68% of respondents). The greatest unmet needs associated with long-term prophylaxis were cost- and access-related. <b>Conclusion:</b> Substantial unmet needs related to disease control and achieving normalization remain for patients with HAE-C1INH, despite existing treatments. This study re-enforces the need to not only assess the frequency and severity of attacks, but also the psychosocial, mental, logistical, and financial burden of lifelong management of HAE-C1INH in clinical practice.

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Publication Details

Journal
Allergy and Asthma Proceedings
Published
2026-02-17
DOI
https://doi.org/10.2500/aap.2026.47.260004
Primary Topic
Coagulation, Bradykinin, Polyphosphates, and Angioedema
Type
article
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article

Rethinking the management of hereditary angioedema

Amod Athavale, ELLIE GOLDMAN, Abigail Silber, Shanna Fishel et al.
Allergy and Asthma Proceedings
Coagulation, Bradykinin, Polyphosphates, and Angioedema
article

Rethinking the management of hereditary angioedema

Amod Athavale, ELLIE GOLDMAN, Abigail Silber, Shanna Fishel, Shruti Nambiar, Kim Wilson, Catheriine Miller, Henriette Farkas, Paula Busse
article en

Abstract

<b>Background:</b> Hereditary angioedema with C1INH deficiency (HAE-C1INH) is a rare, debilitating genetic disorder characterized by recurrent, unpredictable attacks. Although treatments exist, patients with HAE still alter their lives to avoid triggers and experience substantial physical, psychosocial, and financial burdens. <b>Objective:</b> To estimate the burden that HAE-C1INH patients experience despite currently approved therapies, aiming to identify unmet needs related to HAE, its therapies and the ability to achieve normalization of life. <b>Methods:</b> A web-based survey was conducted from March to April 2025 among 100 US adults with HAE-C1INH currently receiving long-term prophylaxis and/or on-demand therapies. Responses captured attack frequency, the impact of living with HAE, avoidance of attack triggers, and the patients' unmet needs. Descriptive statistical analysis was conducted. <b>Results:</b> Even with treatment, 80% of respondents reported ≥1 HAE attack in the past year and 61% thought about HAE at least weekly. Mental health was the aspect that respondents felt was most impacted by HAE (54% of respondents), and 73% reported taking ≥2 measures to avoid attack triggers. Several concerns impacted the ability to reach normalization; lifetime use of medication was the most commonly reported concern (68% of respondents). The greatest unmet needs associated with long-term prophylaxis were cost- and access-related. <b>Conclusion:</b> Substantial unmet needs related to disease control and achieving normalization remain for patients with HAE-C1INH, despite existing treatments. This study re-enforces the need to not only assess the frequency and severity of attacks, but also the psychosocial, mental, logistical, and financial burden of lifelong management of HAE-C1INH in clinical practice.

Allergy and Asthma ProceedingsVol. 47(2)
Semmelweis University (HU), Trinity Health (US), Intellia Therapeutics (United States) (US), Expert System (Italy) (IT), Icahn School of Medicine at Mount Sinai (US)
Openalex Percentile: Top 55%
Coagulation, Bradykinin, Polyphosphates, and Angioedema
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