Hereditary Angioedema: An Updated Experience with Patients with Angioedema in Puerto Rico

Objective: This was a study of patients with hereditary angioedema (HAE) and their responses to new therapies, measured in terms of HAE attack rates, the number of hospitalizations and emergency room (ER) visits, and the impact of HAE on their quality of life (QOL). Methods: Patients that came at a private practice with recurrent angioedema without urticaria from 2013 through 2016. All HAE (types I & II) patients received rescue treatment and prophylaxis for those who had 2 or more attacks per month. Results: Of 48 patients, 22 (45.8%) patients with HAE (I or II) were identified. 45.5% of those HAE patients were on prophylaxis and 77.3% were on rescue therapy. Treatment effects were reported as percentages of the HAE patients in each attack/ month category: Before treatment, 41.2% of the patients had 0 to 1 attack; after treatment, 84.2%. Similarly, 23.5% had 2 to 3 attacks before treatment, fell to 17.6%, after treatment. Finally, 35.3% experienced more than 3 attacks prior to treatment; and none after treatment. The number of ER visits in 6 months decreased from 64 (3.8 per patient) to 7 (0.4 per patient), and hospitalizations in 6 months decreased from 35 (2.1 per patient) to 7 (0.4 per patient) after treatment. The diagnosis delay averaged 4.3 years; patients diagnosed on or before 2012 averaged 8.6 years; patients diagnosed after 2012 averaged 0.4 years. Conclusion: HAE patients showed improved treatment responses as documented by decreased diagnostic delay, attack rates, ER visits and hospitalizations and improved QOL in treated patients.