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. 2014 Jul 4:9:99.
doi: 10.1186/1750-1172-9-99.

Socioeconomic burden of hereditary angioedema: results from the hereditary angioedema burden of illness study in Europe

Emel Aygören-Pürsün  1 , Anette Bygum, Kathleen Beusterien, Emily Hautamaki, Zlatko Sisic, Suzanne Wait, Henrik B Boysen, Teresa Caballero
Affiliations

Socioeconomic burden of hereditary angioedema: results from the hereditary angioedema burden of illness study in Europe

Emel Aygören-Pürsün et al. Orphanet J Rare Dis. .

Abstract

Background: Hereditary angioedema (HAE) due to C1 inhibitor deficiency is a rare but serious and potentially life-threatening disease marked by spontaneous, recurrent attacks of swelling. The study objective was to characterize direct and indirect resource utilization associated with HAE from the patient perspective in Europe.

Methods: The study was conducted in Spain, Germany, and Denmark to assess the real-world experience of HAE via a cross-sectional survey of HAE patients, including direct and indirect resource utilization during and between attacks for patients and their caregivers over the past 6 months. A regression model examined predictors of medical resource utilization.

Results: Overall, 164 patients had an attack in the past 6 months and were included in the analysis. The most significant predictor of medical resource utilization was the severity of the last attack (OR 2.6; p < 0.001). Among patients who sought medical care during the last attack (23%), more than half utilized the emergency department. The last attack prevented patients from their normal activities an average of 4-12 hours. Patient and caregiver absenteeism increased with attack severity and frequency. Among patients who were working or in school (n = 120), 72 provided work/school absenteeism data, resulting in an estimated 20 days missing from work/school on average per year; 51% (n = 84) indicated that HAE has hindered their career/educational advancement.

Conclusion: HAE poses a considerable burden on patients and their families in terms of direct medical costs and indirect costs related to lost productivity. This burden is substantial at the time of attacks and in between attacks.

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Figures

Figure 1
Figure 1
Types of treatment visits for most recent HAE attack (N = 37 patients). Note: Four patients had more than one type of treatment visit for the attack
Figure 2
Figure 2
Duration prevented from daily activities by attack sitea. aFor approximately how long did your most recent HAE attack stop you from performing your normal activities? Median duration for less common sites: “other” (including joints and low back) = <2 h (n = 7); face = <2 to 2-4 h (n = 6); respiratory/laryngeal = 4-12 h (n = 3). Differences across sites not statistically significant (p = 0.560).
Figure 3
Figure 3
Work/school absenteeism during last attack by pain severity. Percentage who missed time and reported mean days missed. ap-values for difference in percentage who missed time. Percentages based on 72 patients who were employed or in school and provided absenteeism data. Percent of caregivers based on full sample (N = 164). Note: Means based on number of patients (n = 40) and caregivers (n = 86) who missed time. Caregiver time includes leisure time. Data missing for missed time for 31 caregivers.
Figure 4
Figure 4
Impact of HAE on career and education. Note: 84 patients (51%) reported that HAE hindered their educational and/or career advancement.
See this image and copyright information in PMC

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