BACKGROUND: Hereditary angioedema (HAE) is a rare, potentially life-threatening disease characterized by recurrent angioedema attacks. Abdominal symptoms of HAE typically mimic numerous abdominal emergencies, which may result in a delay of correct diagnosis and inappropriate treatments. OBJECTIVE: We aimed to evaluate the difficulties experienced by patients with HAE in Turkish emergency departments (ED). METHODS: We conducted face-to-face interviews with patients with HAE by using a questionnaire regarding the clinical and demographic data of the patients and difficulties encountered during ED admissions. RESULTS: The mean (standard deviation) age of 34 patients with HAE (25 women, 9 men) was 36.2 ± 11.5 years and the mean (standard deviation) delay in diagnosis was 17.2 ± 9.7 years. Inappropriate treatment for HAE attacks was administered to 88.2% (n = 30) of patients in the ED, despite their diagnosis of HAE. The most frequent difficulty was "not knowing how to administer C1 inhibitor concentrate" (n = 11 [32.4%]). Other difficulties encountered were as follows: ED staff being unaware of HAE (n = 6 [17.6%]), lack of C1 inhibitor concentrate in the ED (n = 3 [8.8%]), and kept waiting for the appropriate treatment in triage despite their having angioedema in the head-and-neck region (n = 2 [5.9%]). CONCLUSION: Patients with HAE encounter difficulties in the ED, and the delay in diagnosis could be due to a low level of awareness regarding HAE. Therefore, the management of HAE in EDs requires improvement.
BACKGROUND:Hereditary angioedema (HAE) is a rare, potentially life-threatening disease characterized by recurrent angioedema attacks. Abdominal symptoms of HAE typically mimic numerous abdominal emergencies, which may result in a delay of correct diagnosis and inappropriate treatments. OBJECTIVE: We aimed to evaluate the difficulties experienced by patients with HAE in Turkish emergency departments (ED). METHODS: We conducted face-to-face interviews with patients with HAE by using a questionnaire regarding the clinical and demographic data of the patients and difficulties encountered during ED admissions. RESULTS: The mean (standard deviation) age of 34 patients with HAE (25 women, 9 men) was 36.2 ± 11.5 years and the mean (standard deviation) delay in diagnosis was 17.2 ± 9.7 years. Inappropriate treatment for HAE attacks was administered to 88.2% (n = 30) of patients in the ED, despite their diagnosis of HAE. The most frequent difficulty was "not knowing how to administer C1 inhibitor concentrate" (n = 11 [32.4%]). Other difficulties encountered were as follows: ED staff being unaware of HAE (n = 6 [17.6%]), lack of C1 inhibitor concentrate in the ED (n = 3 [8.8%]), and kept waiting for the appropriate treatment in triage despite their having angioedema in the head-and-neck region (n = 2 [5.9%]). CONCLUSION:Patients with HAE encounter difficulties in the ED, and the delay in diagnosis could be due to a low level of awareness regarding HAE. Therefore, the management of HAE in EDs requires improvement.