When is the dyspnea worth it? Understanding functional performance in people with alpha-1 antitrypsin deficiency.

Abstract

Exploratory. People with a severe deficiency of AAT. SAMPLE AND DEMOGRAPHICS: Thirty-three patients (21 men) with a mean age of 47 (SD = 7) years. Data were collected 1993-1996. Activities patients identified as important on the dyspnea subscale of the Chronic Respiratory Disease Questionnaire were categorized and interpreted within the context of an integrity framework (i.e., effectiveness--or connectedness-related). Patients identified over 25 activities. Effectiveness activities, such as bathing, were mentioned most frequently (78%). Connectedness activities, such as playing with children, were mentioned less often (22%) but were ranked more important. Two patients reviewed and supported the content validity of the framework. Results offer insight into the activities patients with genetic emphysema choose to perform and the factors that influence the decision that "the dyspnea is worth it." Identifying the activities people with AAT deficiency choose to perform and understanding why these activities are meaningful can guide interventions to help patients maintain a sense of integrity.