Session 3: Living with recurrent angioedema – burden, comorbidities, predictors
This session opened with a presentation by Professor Connie Katelaris from Campbelltown Public Hospital in Australia. She concentrated on the burden of recurrent angioedema, presenting data on the impact on quality of life, psychological and physical burden on patients of various forms of recurrent angioedema. She presented data indicating that even among patients with complete disease control, 28% reported impaired quality of life. She indicated three key parameters in order to address the full burden of disease:
- Recognize the distinct non-HAE subtypes and the burden profiles
- Measure the full burden using validated patient-reported outcomes tools in routine practice
- Treat beyond attack protection, targeting complete QoL restoration as a treatment goal
In the second half of her presentation, Prof Katelaris focused on burden and comorbidities in HAE. She highlighted the many aspects of burden, from prolonged delay to diagnosis, to the unpredictable and potentially severe life-threatening nature of an attack. She suggested that HAE places a significant burden on patients and their carers, and that the consequences cut across the mental and physical well-being of both. She suggested that effective long-term prophylaxis can improve the quality of life in HAE patients.
Highlighting the wider psychosocial impact of hereditary angioedema (HAE) beyond physical attacks: Findings from 4 multi-national patient experience surveys
The first oral abstract in this session was given by Dr Kathrin Schön from Charité – Universitätsmedizin, Germany. She presented data from a series of patient experience surveys. The surveys were conducted in the UK, Spain, Germany, and the Netherlands to uncover the broader impact of HAE beyond attacks and physical burden.
The patients questioned reported a substantial negative impact of HAE on daily living, quality of life, productivity, and psycho-social well-being, with burden remaining, despite more than 60% of respondents receiving long-term prophylaxis. She concluded that while the primary focus of treatment has been to reduce or prevent attacks, achieving complete control requires a more holistic perspective, in which patients are empowered to share the impact of HAE on their whole life and lifestyle, and that this would inform treatment choice.
Suicidal ideation among patients with hereditary angioedema: Early results from the SAFE-HAE BRADILAT initiative
People with HAE are at three times higher risk of depression and anxiety. Dr Carolina Crespo-Shijin from Universidad Espíritu Santo in Ecuador presented her and colleagues’ work to assess the prevalence of suicidal thoughts in HAE. Their research indicated that individuals with HAE are more at risk of suicidal thoughts than members of the general population, and that the risk increases with greater frequency of attacks, pain, and absenteeism from work. They suggest that doctors routinely ask patients about their mental health using standardized assessments, and that ultimately proactive mental screening and optimized HAE therapy may reduce suicidal thoughts.
Characteristics and burden of caregivers of patients with hereditary angioedema: Results from the ECRINS study
Dr Isabelle Boccon-Gibod from Grenoble Alpes University Hospital in France gave a presentation based on data to explore, in greater depth, the experience and burden of caregivers of patients with HAE. Their data examined the lives of HAE caregivers, including the types of people they were, the support provided, and the impact of the disease on their lives, not just on the HAE patient. Their findings, according to Dr Boccon-Gibod, indicated that three out of four patients with HAE (77%) rely on the support of a caregiver, and that, in the main, these people were close female relatives. The burden of HAE on caregivers’ lives was described as substantial, and caregivers considered oral treatments for HAE as representing progress in HAE management.
Hot topic: Hereditary angioedema with normal C1 inhibitor: What do we know?
The final presentation of the session was a dive into the latest knowledge in HAE with normal C1 inhibitor, given by Professor Sandra Christiansen from the University of California, San Diego. Drawing on the latest science and expert consensus, Dr Christiansen focused on:
- Diagnosis
- Clinical characteristics
- Pathophysiology
- Management
She gave the audience an overview of angioedema classifications and where HAE with normal C1-inhibitor fits, and outlined new research into genetics and pathophysiology. In terms of treatment, Dr Christiansen stressed that in the absence of high-quality studies, it has been necessary to rely on case series to make recommendations, such as:
- HAE with normal C1 inhibitor often responds to the same medicines that are useful in HAE due to C1 inhibitor deficiency
- There is some indication that a discontinuation of estrogens and the use of progesterone as a long-term prophylaxis leads to positive outcomes
- Anti-fibrinolytics are more likely to be of benefit than in patients with HAE due to C1 inhibitor deficiency
Dr Christiansen suggested that, in just over 25 years, progress has been made in understanding and treating HAE with normal C1 inhibitor, but that more questions remain about the availability of biomarkers to support diagnosis and to inform controlled trials that identify the best treatments for the condition.
Industry session: Pharvaris
The second industry-supported session was from Pharvaris, given by Dr Giorgio Giannattasio, the company’s Head of Medical Affairs. He started by illustrating the company’s milestones since its founding in 2015, and its focus on advancing medical care for bradykinin-mediated angioedema. Dr Giannattasio walked the audience through the currently available data on Pharvaris’ bradykinin B2 receptor antagonist, deucrictibant, and its potential in a range of angioedema types. He closed by thanking angioedema patients and expert physicians for inspiring everyone at Pharvaris, sharing their knowledge and experience, and partnering to advance medical care.
