QUALITY OF LIFE AND BURDEN OF DISEASE IN PATIENTS WITH HEREDITARY ANGIOEDEMA AND THEIR CAREGIVERS

Introduction

Hereditary angioedema (HAE) is characterized by recurrent and unpredictable episodes of angioedema. This targeted literature review assessed the burden of disease experienced in HAE patients and their caregivers.

Methods

In January 2024, a search strategy was implemented in Medline and Embase with additional conference abstract screening, using key terms for HAE and burden. Studies describing burden of disease among adults with HAE Type 1 and 2 were included, prioritizing North American and European studies.

Results

A total of 1,975 abstracts were screened for relevance, of which 48 studies met eligibility criteria and were included for analysis. HAE patients had lower quality of life (QoL) than the general population, associated with increased attack frequency, attack severity, depression and anxiety. Anxiety and depression were largely driven by societal perceptions and disease burden, with constant fear of laryngeal attacks. Consistent long-term prophylaxis (LTP) has been shown to alleviate anxiety and depression and improve QoL, compared to no treatment or only on-demand treatment. Uncontrolled attacks and adverse effects were common reasons for treatment discontinuation. Caregiver burden was profoundly influenced by the severity and unpredictability of HAE symptoms, diagnosis delays and negative healthcare provider interactions.

Conclusions

HAE patients and caregivers bear a heavy disease burden, driven by the severity, frequency, location, and unpredictability of attacks. Because of their symptoms, patients experience poor QoL and high psychological distress, the latter of which could also trigger HAE attacks. To improve QoL patients seek LTP that provide less frequent dosing, fewer attacks and less side effects.