Quality of life Impairment in 7 patients with Hereditary Angioedema [HAE] in Cayre IPS and foundation, Bogotá, Colombia.

Abstract

Event Abstract Back to Event Quality of life Impairment in 7 patients with Hereditary Angioedema [HAE] in Cayre IPS and foundation, Bogotá, Colombia. CARLOS E. OLMOS1 and CATALINA GOMEZ PARADA1* 1 Cayre IPS y Fundacion, Colombia Objective: Hereditary angioedema (HAE) is a primary immunodeficiency of the complement system characterized by cutaneous or mucosa swellings caused by a deficiency or dysfunction of C1 esterase inhibitor. It is inherited as an autosomal dominant trait. Acute attacks of HAE are characterized by recurrent, unpredictable, rapidly localized swelling. These attacks can be life threatening when airway is involved. HAE has a considerable impact on quality of life [QOL]. This report describes the QOL assesment in 7 patients diagnosed with HAE in Cayre IPS, Bogotá, Colombia. Methods: Clinical, demographic and laboratory data were obtained from medical records. A survey with QOL questionnaire was performed during consultation and by telephone call in 7 confirmed patients with HAE. We recorded information in our database and the results are analyzed. Results: Four patients were female and 3 male, and 2 were minors. All 7 patients have a confirmed diagnosis of HAE type 1. They answered 17 questions contained in a QOL questionnaire that measure four dimensions: [1] functionality, [2] mood, [3] fears and [4] impact in food and frequency in the last 4 weeks. The four questions related to functionality are about impairment at work, school or physical activity, and spare time activities 7/7 answer very often. The five mood questions are realted to waking up during the night 1/7, feeling tired during the day 5/7 , difficulties in concentrating 1/7, and feeling downhearted 7/. The six fears questions are about feeling burdened at having swellings 7/7, fear of new suddenly appearing swellings 7/7, fear of increased frequency of swellings 7/7, ashamed to visit public places 3/7, embarrassed by the appearance of swellings 7/7, and fear of long-term negative drug effects 7/7. Finally, two last questions are related to general limitations in eating and in the selection of food and beverages 1/7. The most affected dimensions were those related to functionality and fears, followed by the mood, and eating limitations had the lowest impact. Conclusions: HAE is a major condition in patients that severely impacts their quality of life. Although it is not often evaluated, QOL evaluation should be an integral compoment of the holistic management of these patients.