SPACE PROVIDED. 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.

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Mentored Patient-Oriented Research Career Development Award (K23)
Project #
5K23HL092227-04
Application #
8286927
Study Section
Special Emphasis Panel (ZHL1-CSR-R (M1))
Program Officer
Colombini-Hatch, Sandra
Project Start
2009-07-15
Project End
2014-06-30
Budget Start
2012-07-01
Budget End
2013-06-30
Support Year
4
Fiscal Year
2012
Total Cost
$130,053
Indirect Cost
$9,634
Name
National Jewish Health
Department
Type
DUNS #
076443019
City
Denver
State
CO
Country
United States
Zip Code
80206
Swigris, Jeffrey J; Streiner, David L; Brown, Kevin K et al. (2014) Assessing exertional dyspnea in patients with idiopathic pulmonary fibrosis. Respir Med 108:181-8
Belkin, Amanda; Albright, Karen; Fier, Kaitlin et al. (2014) "Getting stuck with LAM": patients perspectives on living with lymphangioleiomyomatosis. Health Qual Life Outcomes 12:79
Strand, Matthew J; Sprunger, David; Cosgrove, Gregory P et al. (2014) Pulmonary function and survival in idiopathic vs secondary usual interstitial pneumonia. Chest 146:775-85
Swigris, Jeffrey J; Lee, Hye-Seung; Cohen, Marsha et al. (2013) St. George's Respiratory Questionnaire has longitudinal construct validity in lymphangioleiomyomatosis. Chest 143:1671-8
Fernandez Perez, Evans R; Swigris, Jeffrey J; Forssen, Anna V et al. (2013) Identifying an inciting antigen is associated with improved survival in patients with chronic hypersensitivity pneumonitis. Chest 144:1644-51
Fernandez Perez, Evans R; Krishnamoorthy, Mahalakshmi; Brown, Kevin K et al. (2013) FEV1 over time in patients with connective tissue disease-related bronchiolitis. Respir Med 107:883-9
Fischer, Aryeh; Brown, Kevin K; Du Bois, Roland M et al. (2013) Mycophenolate mofetil improves lung function in connective tissue disease-associated interstitial lung disease. J Rheumatol 40:640-6
Swigris, Jeffrey J; Olson, Amy L; Huie, Tristan J et al. (2012) Ethnic and racial differences in the presence of idiopathic pulmonary fibrosis at death. Respir Med 106:588-93
Pillai, Manju; Olson, Amy L; Huie, Tristan J et al. (2012) Obstructive sleep apnea does not promote esophageal reflux in fibrosing interstitial lung disease. Respir Med 106:1033-9
Swigris, Jeffrey J; Fairclough, Diane L; Morrison, Marianne et al. (2011) Benefits of pulmonary rehabilitation in idiopathic pulmonary fibrosis. Respir Care 56:783-9

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