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Open Access Study protocol

The hereditary angioedema burden of illness study in Europe (HAE-BOIS-Europe): background and methodology

Anette Bygum1*, Emel Aygören-Pürsün2, Teresa Caballero3, Kathleen Beusterien4, Shadi Gholizadeh4, Patience Musingarimi5, Suzanne Wait6 and Henrik Boysen7

Author Affiliations

1 HAE Centre Denmark, Department of Dermatology and Allergy Centre, Odense University Hospital, 5000 Odense C, Denmark

2 Department of Pediatrics, Pediatric Hematology, Oncology, Hemostaseology and Cardiology, University Hospital, Johann Wolfgang Goethe University, Theodor-Stern-Kai 7, 60596 Frankfurt, Germany

3 Allergy Department, Hospital La Paz Health Research Center (IdiPaz), Biomedical Research Network on Rare Diseases U754 (CIBERER), University Hospital La Paz, Paseo de la Castellana 261, 28046 Madrid, Spain

4 Oxford Outcomes Inc, 7315 Wisconsin Ave. Ste 250W, Bethesda, MD 20814 USA

5 ViroPharma, Chatsworth House, 29 Broadway, Maidenhead, SL6 1LY, UK

6 SHW Health Ltd, 40 Lena Gardens, London, W6 7PZ, UK

7 HAEi–International Patient Organization for C1 Inhibitor Deficiencies, Lindeparken 33, DK-6230, Roedekro, Denmark

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BMC Dermatology 2012, 12:4  doi:10.1186/1471-5945-12-4

Published: 26 April 2012

Abstract

Background

Hereditary angioedema (HAE) is a rare but serious disease marked by swelling attacks in the extremities, face, trunk, airway, or abdominal areas that can be spontaneous or the result of trauma and other triggers. It can be life-threatening due to the risk of asphyxiation. While there have been major advancements in our understanding of the immunogenetics of HAE, there are significant gaps in the literature regarding understanding of the humanistic and economic impact of the disease, particularly in Europe. The purpose of the HAE Burden of Illness Study-Europe (HAE-BOIS-Europe), the development and methodology of which is described here, is to better understand the management and impact of HAE from the patient perspective in Europe.

Methods/Design

This is a cross-sectional study in which retrospective data were also collected being conducted in Denmark, Germany and Spain. The study is open to patients ages 12 and older with a diagnosis of HAE-I or HAE-II. Data collection includes: (i) a survey on individuals’ health care resource use, direct and indirect medical costs, impact on work and school, treatment satisfaction, and emotional functioning (via the Hospital Anxiety and Depression Scale); and (ii) one-on-one interviews to collect detailed descriptive data and patient testimonials on the impact of HAE on patients’ health-related quality of life.

Discussion

The present manuscript describes the development and plans for implementing a multi-country European study with the aim of characterizing the humanistic and economic burden of HAE from the patient perspective. This study will help raise awareness of HAE as a rare but debilitating condition with wide-ranging impacts.