This protocol applies to patients with hereditary C1 inhibitor deficiency presenting with high hereditary angioedema (HAE) disease activity — specifically, those whose attacks are not adequately controlled with on-demand therapy alone.
Long-term prophylaxis is indicated and should be individualised, accounting for disease activity, quality of life, availability of healthcare resources, and failure to achieve adequate control through appropriate on-demand therapy.
The prophylactic strategy in this setting involves antifibrinolytic therapy as a pharmacological option — particularly relevant when first-line prophylactic treatments are unavailable or contraindicated.
DOI: 10.1111/all.15214
Long-term prophylaxis should be individualized and considered in all HAE-1/2 patients taking into consideration the disease activity, patient's quality of life, availability of health care resources, and failure to achieve adequate control by appropriate on-demand therapy.
They are primarily used where first-line prophylactic treatment options are not available and androgens are contraindicated.
The doses of tranexamic acid used range from 30 to 50 mg/kg body weight daily divided into two or three doses to a maximum of 6 g per day.