The purpose of this study is to examine the relationships among severity of illness, quality of life and functional ability; and to determine the impact of 02 therapy of functional ability in patients with Alpha 1-Antitrypsin Deficiency. Adult Alpha 1-antitrypsin deficient patients, admitted for evaluation of their lung disease, respond to a questionnaire that measures four aspects of quality of life: emotional function, fatigue, mastery, and dyspnea. Patients walk for 6 minutes with oxygen via nasal cannula and with placebo (measured by the distance walked, the oxygenation and pulse rate during the walk, and the dyspnea intensity at the end of each walk. To date we have collected data on 25 subjects. Preliminary analysis indicates no advantage of oxygen over placebo in terms of functional outcomes. An amendment to this protocol was recently approved to test patients with more advanced disease status using oxygen as compared to paced breathing.
