INTRODUCTION: Limited data exist on the burden of hereditary angioedema (HAE), its variability among patients, and impact on quality of life. This study focused on gaining a better understanding of these issues among US patients.
METHODS: US-HAE Association members were invited to participate in a cross-sectional web-based survey. Respondents with HAE type I/II were recruited if ≥18 years old, reported ≥1 HAE attack or prodromal symptom within the last one year, and received HAE medication within the last two years. Descriptive analyses were conducted.
RESULTS: 2,961 members were invited. Of 707 members screened, 445 (62.9%) completed the survey. Most were 18-64 years (92.8%), white (90.3%), and female (78.2%). Mean (SD) age at first symptoms and at diagnosis was 12.5 (9.1) and 20.1 (13.7) years, respectively, with mean (SD) delay in diagnosis of 8.4 (10.6) years. Family history of HAE was reported in 78.4%. Mean (SD) number of attacks in past 6 months was 11.1 (13.9) and 78.6% reported an attack during the past month. The most recent attack lasted 6-72 hours (74.7%), primarily affecting abdomen (58.0%); extremities (46.1%); and face, throat and mouth (23.8%). During attacks, moderate to severe pain (73.9%), abdominal swelling (57.0%), swelling (55.1%), tiredness (53.1%), nausea (38.9%), and diarrhea (25.4%) were experienced. During the past 12 months, 68.5% reported ongoing use of HAE prophylaxis (C1-INH [60.7%] and androgens [27.2%]). The most commonly reported comorbidities were anxiety (35.3%) and depression (20.9%).
CONCLUSION: Despite advances in HAE therapies, findings from this US study suggest HAE poses a substantial burden.
