Hereditary Angioedema Attack Prophylaxis Management In Australian Patients

Management of Hereditary Angioedema (HAE) has changed substantially in the last decade with the advent of new medications and improved access to replacement therapy with C1 inhibitor concentrate. Improvements in prophylactic therapy have resulted in patients having greater control over their condition and better quality of life. International guidelines are reflecting the shift to greater access to safer and more effective prophylaxis for this potentially life-threatening condition. We have surveyed ASCIA members involved in the care of patients with HAE to gauge the pattern of usage of prophylactic therapy. A questionnaire was sent to members of ASCIA HAE Working Party and to other Immunologists known to have HAE patients in their care (n = 14). The questionnaire was returned, de-identified, by 13 specialists reporting on 102 HAE patients in their care. Usage patterns for prophylaxis with Berinert, Danazol and tranexamic acid were explored and results were tabulated. Six of 13 responding specialists had prescribed Berinert for long term prophylaxis (BLTP) to 18/102 (17%) patients. NBA prescription criteria were followed in all; 11 patients received 20 IU/kg while 2 received less, and 4 more, than this. Only 2/18 patients on BLTP had not received other prophylaxis in the past. No side effects were reported in any on BLTP; 17/18 patients were satisfied with control. Since using BLTP only 1 patient had had a hospitalisation and only 1 had seen no reduction in icatibant use whereas 12/18 had reduced icatibant use by >50%. Danazol was currently used in 36/102 patients and tranexamic acid in 19/102 patients. BLTP is used currently in the minority of HAE patients receiving prophylactic therapy. It is well tolerated with the majority of patients self administering and its use has led to a reduction in acute treatment with icatibant.