Cystic fibrosis is the most common inherited life-shortening disease among white populations and is characterized by progressive loss of pulmonary function and exercise tolerance. We have previously shown that exercise programs can increase fitness in these patients, but without apparent effect on lung function. Others have shown improvement in pulmonary function with activity programs but have not examined fitness in patients with cystic fibrosis. No studies have examined the effect that activity programs may have on the quality of well-being in these patients, nor have there ben appropriately controlled long-term studies. We plan to institute a two year at-home activity program which will substantially increase the patients level of habitual activity. Behavioral medicine techniques will be used to promote compliance to this two year program. Fifty patients will be randomly assigned to the activity program and fifty patients to a non-exercise control group. We will examine the effects of this program on pulmonary function, exercise tolerance and quality of well-being of the treatment group compared to the control group. We will also determine if any relationship exists among physical activity (as quantified by two activity monitors and activity surveys) and pulmonary function, cardiopulmonary testing and quality of well- being.
