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.