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Universal Access to On-Demand Treatment of Patients with Hereditary Angioedema, the Chilean Experience
Juan J Escobar1,2, Joaquín Aguirre2, Samuel Ibáñez2
1Department of Pediatrics, Pontificia Universidad Católica de Chile, Santiago, Chile.
Insights
Chilean patients with hereditary angioedema (HAE) have access to on-demand plasma-derived C1-INH (pdC1-INH) since 2018. Analysis reveals significant diagnostic delays and high attack rates, suggesting a need for improved awareness and prophylactic treatments.
Area of Science:
- Immunology
- Genetics
- Public Health
Background:
- Hereditary angioedema (HAE) types I and II patients in Chile are covered by Ley Ricarte Soto (LRS) for on-demand plasma-derived C1-INH (pdC1-INH) treatment since 2018.
- The LRS aims to ensure access to essential treatments for specific diseases.
Purpose of the Study:
- To analyze the initial three years of LRS implementation for HAE patients in Chile.
- To assess the impact of LRS on HAE patient management and identify areas for improvement.
Main Methods:
- A retrospective review of the LRS database from 2018 to 2021.
- Analysis of patient demographics, diagnostic delays, attack frequency, and treatment patterns.
Main Results:
- 154 HAE patients were covered by LRS, estimating a prevalence of 0.8 per 100,000 inhabitants.
- A significant diagnostic delay of 22 years was observed, with 50 patients receiving epinephrine pre-diagnosis.
- Patients experienced a mean of 8 attacks annually, with over 40% facing >1 attack/month.
Conclusions:
- Improved disease awareness is crucial to reduce diagnostic delays for HAE.
- Inclusion of long-term prophylactic medications in LRS is recommended for patients with high attack rates.
- Expanding LRS coverage could optimize HAE management and control treatment costs.
Abstract:
In Chile, patients with hereditary angioedema (HAE) type I and type II are protected under Ley Ricarte Soto (LRS), which guarantees access to on demand plasma-derived C1-INH (pdC1-INH) since 2018. We aimed to analyze the first 3 years of LRS. Review of the LRS database between 2018 and 2021. During the study period, 154 patients were covered by LRS, with an estimated prevalence of HAE in Chile at 0.8:100,000 inhabitants. A delay in diagnosis of 22 years was noted, 50 patients received epinephrine during an attack before the diagnosis of HAE. Mean number of attacks per year was 8, with 50% of adults and 42% of children experiencing more than 1 attack per month. Disease awareness must improve to reduce the diagnostic delay of HAE. Long-term prophylactic medications should be included in LRS to treat patients with high attack rates and control the costs of frequent on-demand treatment with pdC1-INH.
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