Evaluating adherence to long-term prophylaxis treatment with danazol in adult hereditary angioedema patients: A real life study

Abstract

Objective: To investigate the adherence to the prophylactic treatment in hereditary angioedema (HAE) patients as well as the potential factors which may affect this situation. Patients and Methods: In addition to evaluation of their medical records, sixty HAE patients were asked to complete a questionnaire including inquiries about demographic and clinical features of their disease and medications used. Disease severity was determined depending on their age of onset of symptoms, clinical manifestations, and need of long-term prophylaxis. Results: Sixty-five percent of the patients were female, the mean age was 38.07±12.38 years, 93.3% were type 1 HAE, 58.3% had a severe form of the disease, and 71.7% were under prophylaxis with danazol. Fourteen patients were not using danazol regularly due to the fear of side effects (n=11) and forgetfulness to take the medication (n=4). It was observed that the patients who were the only cases in their families, those having few relatives with HAE and having had no excitus due to HAE in their families, were more adherent to prophylactic treatment (P=0.008; P=0.018; P=0.028). Conclusion: The majority of patients were effectively under long-term prophylaxis and the majority adhered to this treatment. The primary cause of non-adherence was fear of side effects.