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.
Authors
- Amod Athavale (ORCID: https://orcid.org/0000-0001-8808-5069)
- ELLIE GOLDMAN
- Abigail Silber
- Shanna Fishel
- Shruti Nambiar
- Kim Wilson
- Catheriine Miller
- Henriette Farkas
- Paula Busse
Institutions
- Semmelweis University (HU)
- Trinity Health (US)
- Intellia Therapeutics (United States) (US)
- Expert System (Italy) (IT)
- Icahn School of Medicine at Mount Sinai (US)
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
- Field-Weighted Citation Impact
- 0.00