Related Experiment Videos
When is the dyspnea worth it? Understanding functional performance in people with alpha-1 antitrypsin deficiency
A Knebel1, N K Leidy, S Sherman
1National Institutes of Health, Clinical Center Nursing Department, Bethesda, MD 20892-1506, USA. aknebel@nih.gov
Abstract:
OBJECTIVE AND SIGNIFICANCE: To examine functional performance in people with emphysema because of alpha-1 antitrypsin (AAT) deficiency. A severe deficiency of AAT affects 1:3,500 to 1:1,670 Americans who can develop debilitating emphysema in the third to fifth decades of life.
Design:
Exploratory.
Population:
People with a severe deficiency of AAT. SAMPLE AND DEMOGRAPHICS: Thirty-three patients (21 men) with a mean age of 47 (SD = 7) years.
Years:
Data were collected 1993-1996.
Methods:
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).
Findings:
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.
Conclusions:
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."
Implications:
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.