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


METHODS
Exploratory.


METHODS
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.


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).


RESULTS
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."


CONCLUSIONS
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.
Knebel
A
A
National Institutes of Health, Clinical Center Nursing Department, Bethesda, MD 20892-1506, USA. aknebel@nih.gov
Leidy
N K
NK
Sherman
S
S
D000203:Activities of Daily Living; D000328:Adult; D002755:Choice Behavior; D004417:Dyspnea / Q000235:genetics* / Q000503:physiopathology / Q000523:psychology*; D004646:Emphysema / Q000235:genetics* / Q000503:physiopathology / Q000523:psychology*; D005260:Female; D006801:Humans; D007389:Internal-External Control; D008297:Male; D008875:Middle Aged; D009042:Motivation; D015404:Nursing Methodology Research; D011788:Quality of Life; D011795:Surveys and Questionnaires; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications*