:Cystic Fibrosis (CF) is one of the most common inherited fatal diseases. Over the past thirty years, the median age of survival has improved from 14 years in 1969 to 29.1 years (95% Cl's 27.5 to 31.6) in 1999 in the United States. With this improved survival, this disease has been transformed from a disease with very high infant mortality to a disease that must now be considered a chronic and progressive lung disease. Improving the survival in these patients has been the primary goal over the last 30 years, but now clinical researchers must broaden their assessment of outcome in this disease to include patient-oriented outcomes such as health-related quality of life. Unfortunately, there is very limited research on the utility and relative strengths of different methods to measure health-related quality of life in patients with CF, especially for that group with the most severe disease. A better understanding of the best instruments, the minimal clinically important difference of these instruments, and the course of quality of life in these patients is needed in order to assess the value of treatments and interventions and to counsel patients and their families about their prognosis and the morbidity associated with this disease. This application proposes a prospective cohort study of patients with severe CF. Four different health-related quality of life instruments will be assessed, including a recently developed CF specific quality of life instrument. Patients will be evaluated at 6 month intervals f or a total of three years. The overall object of this proposal is to advance the state of the art in the measurement of health-related quality of life in adolescent and adult patients with severe cystic fibrosis in order to facilitate development and assessment of treatments that improve the quality of patients' lives and help predict the best time for evaluation for a lung transplant. In addition, this application proposes to compare the baseline differences health-related quality of life in patients not listed for lung or heart lung transplant, listed for lung or heart lung transplant, and transplanted and how health-related quality of life changes through time in these patients.

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Mentored Patient-Oriented Research Career Development Award (K23)
Project #
5K23HL072017-04
Application #
7052013
Study Section
Special Emphasis Panel (ZHL1-CSR-J (O1))
Program Officer
Rothgeb, Ann E
Project Start
2003-05-01
Project End
2008-04-30
Budget Start
2006-05-01
Budget End
2007-04-30
Support Year
4
Fiscal Year
2006
Total Cost
$125,253
Indirect Cost
Name
University of Washington
Department
Internal Medicine/Medicine
Type
Schools of Medicine
DUNS #
605799469
City
Seattle
State
WA
Country
United States
Zip Code
98195
Quon, Bradley S; Patrick, Donald L; Edwards, Todd C et al. (2012) Feasibility of using pedometers to measure daily step counts in cystic fibrosis and an assessment of its responsiveness to changes in health state. J Cyst Fibros 11:216-22
Cecere, Laura M; Rubenfeld, Gordon D; Park, David R et al. (2010) Long-term survival after hospitalization for community-acquired and healthcare-associated pneumonia. Respiration 79:128-36
Bennett, Antonia V; Patrick, Donald L; Lymp, James F et al. (2010) Comparison of 7-day and repeated 24-hour recall of symptoms of cystic fibrosis. J Cyst Fibros 9:419-24
Goss, C H; Edwards, T C; Ramsey, B W et al. (2009) Patient-reported respiratory symptoms in cystic fibrosis. J Cyst Fibros 8:245-52
Saliman, Joshua A; Benditt, Joshua O; Flum, David R et al. (2008) Pulmonary function in the morbidly obese. Surg Obes Relat Dis 4:632-9;discussion 639
Goss, Christopher H; Burns, Jane L (2007) Exacerbations in cystic fibrosis. 1: Epidemiology and pathogenesis. Thorax 62:360-7
Goss, Christopher H; Quittner, Alexandra L (2007) Patient-reported outcomes in cystic fibrosis. Proc Am Thorac Soc 4:378-86
Goss, Christopher H; Rubenfeld, Gordon D; Ramsey, Bonnie W et al. (2006) Clinical trial participants compared with nonparticipants in cystic fibrosis. Am J Respir Crit Care Med 173:98-104
Goss, Christopher H; Rosenfeld, Margaret (2004) Update on cystic fibrosis epidemiology. Curr Opin Pulm Med 10:510-4