The overarching objective of this proposal Is to provide the principal investigator a springboard from which to launch a thriving, independent clinical research program. The initial primary aim of this research program is to advance the understanding of idiopathic pulmonary fibrosis (IPF), particularly its negative effects on patients'lives-and to develop or identify interventions that might improve those effects. The goals of the specific project outlined in this proposal are to characterize the life-altering sequelae of IPF^dyspnea and impairments in functional capacity, cognitive function, mood and anxiety, fatigue, and quality of life (QOL)- and then to assess the impact of pulmonary rehabilitation (PR) on these sequelae. To accomplish these goals, I am conducting a randomized, delayed intervention/cross-over study in which subjects will complete tests of lung physiology, gas exchange, and functional capacity as well as respond to various questionnaires at various timepoints. Idiopathic pulmonary fibrosis is a devastating disease marked by relentlessly progressive lung fibrosis and debilitating shortness of breath. Despite nearly 30 years of intense investigation, effective therapy for IPF remains elusive;median survival rates have stubbornly remained between three and five years from the time of diagnosis, and no medical therapy has been found to improve survival, dyspnea, functional capacity, or QOL. Without therapy to help IPF patients live longer, an important question to ask is whether there is an intervention that might allow these people to live better- to be more active;to maintain their independence;to experience fewer disease sequelae and to improve overall QOL. One intervention that holds great promise, PR, with the goal of increasing functional capacity and improving disease impact, is commonly prescribed for IPF patients, but the effects of PR on key outcomes^and patient characteristics associated with these effects-have not been studied in this disease. This research has broad implications: IPF is a disease of aging-mean age at diagnosis is around 66 years. As the population ages, the burden of this devastating disease will grow;there is reason to believe that PR is an intervention that can greatly benefit IPF patients. Thus, it is imperative for research like this to be performed to clarify how a safe, easily implemented intervention like PR might help IPF patients and to determine what modifications might need to be made to maximize benefits from such a program.
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