title: The humanistic burden of hereditary angioedema: results from the Burden of Illness Study in Europe.
abstract: Hereditary angioedema (HAE) is a rare but potentially life-threatening disease marked by spontaneous, recurrent attacks of swelling. The broad range of consequences of HAE on patients' lives is not well understood. The study objective was to comprehensively characterize the burden of illness and impact of HAE types I and II from the patient perspective. The HAE Burden of Illness Study in Europe was conducted in Spain, Germany, and Denmark to assess the real-world experience of HAE from the patient perspective via a one-time survey, which included items on clinical characteristics and physical and emotional impacts. One hundred eighty-six patients participated; 59% reported having an attack at least once a month, 67% reported moderate-to-severe pain during their last attack, and 74% reported moderate-to-severe swelling. The most common sites of the last attack were the abdomen and extremities; 24% experienced an attack in more than one site. The impact of HAE on daily activities was high during attacks and did not vary significantly by body site affected; patients also reported that HAE impacted their daily activities between attacks. Patients reported substantial anxiety about future attacks, traveling, and passing HAE to their children. Based on Hospital Anxiety and Depression Scale scores, 38 and 14% had clinically meaningful anxiety and depression, respectively. Despite standard of care, HAE patients still have frequent and painful attacks. Patients experience substantial impairment physically and emotionally both during and between attacks. A better understanding of these effects may help in the clinical management of HAE patients.
authors.affiliation: Allergy Department, Hospital La Paz Institute for Health Research (Idi-Paz), Biomedical Research Network on Rare Diseases U754 (CIBERER), University Hospital La Paz, Madrid, Spain.
authors.forename: Teresa
authors.initials: T
authors.lastname: Caballero
authors.forename: Emel
authors.initials: E
authors.lastname: Aygören-Pürsün
authors.forename: Anette
authors.initials: A
authors.lastname: Bygum
authors.forename: Kathleen
authors.initials: K
authors.lastname: Beusterien
authors.forename: Emily
authors.initials: E
authors.lastname: Hautamaki
authors.forename: Zlatko
authors.initials: Z
authors.lastname: Sisic
authors.forename: Suzanne
authors.initials: S
authors.lastname: Wait
authors.forename: Henrik B
authors.initials: HB
authors.lastname: Boysen
mesh_terms: D000203:Activities of Daily Living; D000328:Adult; D017668:Age of Onset; D054179:Angioedemas, Hereditary / Q000453:epidemiology* / Q000523:psychology*; D003718:Denmark; D005260:Female; D005858:Germany; D006306:Health Surveys; D006801:Humans; D008297:Male; D008875:Middle Aged; D011569:Psychiatric Status Rating Scales; D011788:Quality of Life; D013030:Spain