Decline in lung function does not predict future decline in lung function in cystic fibrosis patients

Margaret Rosenfeld, Donald R. VanDevanter, Clement L. Ren, Eric P. Elkin, David J. Pasta, Michael W. Konstan, Wayne J Morgan

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Background Despite the attention paid to minimizing lung function decline among cystic fibrosis (CF) patients, the effect of rate of decline on subsequent disease progression is poorly understood. We aimed to describe the rate of decline of FVC, FEV<inf>1</inf>, and FEF<inf>25-75</inf> and to test the hypothesis that rate of decline of each spirometric variable predicts subsequent rate of decline in that variable and each other variable. Methods Data were from the Epidemiologic Study of CF, an observational study of North American CF patients from 1994 to 2005. For each year of age, patients' best percent predicted FEV<inf>1</inf> and associated FVC and FEF<inf>25-75,</inf> were used to calculate 2-year slopes for each spirometric variable. Pearson correlations were calculated between reference slopes and follow-up slopes up to 8 years later and, for FEV<inf>1</inf>, between reference slopes and level (not slope) of lung function up to 5 years later. Results Twenty six thousand, three hundred and ninety-three patients contributed 427,063 spirometries. Median 2-year slopes of all variables were negative for all ages >6 years and the magnitude varied with age, being greatest among 13-17 year olds, especially for FEF<inf>25-75</inf>. There was no correlation (r<0.10) between reference slopes and subsequent slopes 3-8 years later, either within or across variables. The correlation between 2-year FEV<inf>1</inf> slopes and FEV<inf>1</inf> level even 5 years later was moderate (0.37-0.49) across disease stage categories. Conclusions Contrary to our hypothesis, rate of lung function decline did not predict future rate of decline either within or across spirometric variables. In contrast, FEV<inf>1</inf> slope did have moderate predictive ability for subsequent FEV<inf>1</inf> level. These findings are relevant for clinical care and for clinical trial design.

Original languageEnglish (US)
Pages (from-to)856-862
Number of pages7
JournalPediatric Pulmonology
Volume50
Issue number9
DOIs
StatePublished - Sep 1 2015

Fingerprint

Cystic Fibrosis
Lung
Spirometry
Observational Studies
Disease Progression
Epidemiologic Studies
Clinical Trials

Keywords

  • epidemiology
  • longitudinal
  • spirometry

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine

Cite this

Rosenfeld, M., VanDevanter, D. R., Ren, C. L., Elkin, E. P., Pasta, D. J., Konstan, M. W., & Morgan, W. J. (2015). Decline in lung function does not predict future decline in lung function in cystic fibrosis patients. Pediatric Pulmonology, 50(9), 856-862. https://doi.org/10.1002/ppul.23227

Decline in lung function does not predict future decline in lung function in cystic fibrosis patients. / Rosenfeld, Margaret; VanDevanter, Donald R.; Ren, Clement L.; Elkin, Eric P.; Pasta, David J.; Konstan, Michael W.; Morgan, Wayne J.

In: Pediatric Pulmonology, Vol. 50, No. 9, 01.09.2015, p. 856-862.

Research output: Contribution to journalArticle

Rosenfeld, M, VanDevanter, DR, Ren, CL, Elkin, EP, Pasta, DJ, Konstan, MW & Morgan, WJ 2015, 'Decline in lung function does not predict future decline in lung function in cystic fibrosis patients', Pediatric Pulmonology, vol. 50, no. 9, pp. 856-862. https://doi.org/10.1002/ppul.23227
Rosenfeld M, VanDevanter DR, Ren CL, Elkin EP, Pasta DJ, Konstan MW et al. Decline in lung function does not predict future decline in lung function in cystic fibrosis patients. Pediatric Pulmonology. 2015 Sep 1;50(9):856-862. https://doi.org/10.1002/ppul.23227
Rosenfeld, Margaret ; VanDevanter, Donald R. ; Ren, Clement L. ; Elkin, Eric P. ; Pasta, David J. ; Konstan, Michael W. ; Morgan, Wayne J. / Decline in lung function does not predict future decline in lung function in cystic fibrosis patients. In: Pediatric Pulmonology. 2015 ; Vol. 50, No. 9. pp. 856-862.
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abstract = "Background Despite the attention paid to minimizing lung function decline among cystic fibrosis (CF) patients, the effect of rate of decline on subsequent disease progression is poorly understood. We aimed to describe the rate of decline of FVC, FEV1, and FEF25-75 and to test the hypothesis that rate of decline of each spirometric variable predicts subsequent rate of decline in that variable and each other variable. Methods Data were from the Epidemiologic Study of CF, an observational study of North American CF patients from 1994 to 2005. For each year of age, patients' best percent predicted FEV1 and associated FVC and FEF25-75, were used to calculate 2-year slopes for each spirometric variable. Pearson correlations were calculated between reference slopes and follow-up slopes up to 8 years later and, for FEV1, between reference slopes and level (not slope) of lung function up to 5 years later. Results Twenty six thousand, three hundred and ninety-three patients contributed 427,063 spirometries. Median 2-year slopes of all variables were negative for all ages >6 years and the magnitude varied with age, being greatest among 13-17 year olds, especially for FEF25-75. There was no correlation (r<0.10) between reference slopes and subsequent slopes 3-8 years later, either within or across variables. The correlation between 2-year FEV1 slopes and FEV1 level even 5 years later was moderate (0.37-0.49) across disease stage categories. Conclusions Contrary to our hypothesis, rate of lung function decline did not predict future rate of decline either within or across spirometric variables. In contrast, FEV1 slope did have moderate predictive ability for subsequent FEV1 level. These findings are relevant for clinical care and for clinical trial design.",
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N2 - Background Despite the attention paid to minimizing lung function decline among cystic fibrosis (CF) patients, the effect of rate of decline on subsequent disease progression is poorly understood. We aimed to describe the rate of decline of FVC, FEV1, and FEF25-75 and to test the hypothesis that rate of decline of each spirometric variable predicts subsequent rate of decline in that variable and each other variable. Methods Data were from the Epidemiologic Study of CF, an observational study of North American CF patients from 1994 to 2005. For each year of age, patients' best percent predicted FEV1 and associated FVC and FEF25-75, were used to calculate 2-year slopes for each spirometric variable. Pearson correlations were calculated between reference slopes and follow-up slopes up to 8 years later and, for FEV1, between reference slopes and level (not slope) of lung function up to 5 years later. Results Twenty six thousand, three hundred and ninety-three patients contributed 427,063 spirometries. Median 2-year slopes of all variables were negative for all ages >6 years and the magnitude varied with age, being greatest among 13-17 year olds, especially for FEF25-75. There was no correlation (r<0.10) between reference slopes and subsequent slopes 3-8 years later, either within or across variables. The correlation between 2-year FEV1 slopes and FEV1 level even 5 years later was moderate (0.37-0.49) across disease stage categories. Conclusions Contrary to our hypothesis, rate of lung function decline did not predict future rate of decline either within or across spirometric variables. In contrast, FEV1 slope did have moderate predictive ability for subsequent FEV1 level. These findings are relevant for clinical care and for clinical trial design.

AB - Background Despite the attention paid to minimizing lung function decline among cystic fibrosis (CF) patients, the effect of rate of decline on subsequent disease progression is poorly understood. We aimed to describe the rate of decline of FVC, FEV1, and FEF25-75 and to test the hypothesis that rate of decline of each spirometric variable predicts subsequent rate of decline in that variable and each other variable. Methods Data were from the Epidemiologic Study of CF, an observational study of North American CF patients from 1994 to 2005. For each year of age, patients' best percent predicted FEV1 and associated FVC and FEF25-75, were used to calculate 2-year slopes for each spirometric variable. Pearson correlations were calculated between reference slopes and follow-up slopes up to 8 years later and, for FEV1, between reference slopes and level (not slope) of lung function up to 5 years later. Results Twenty six thousand, three hundred and ninety-three patients contributed 427,063 spirometries. Median 2-year slopes of all variables were negative for all ages >6 years and the magnitude varied with age, being greatest among 13-17 year olds, especially for FEF25-75. There was no correlation (r<0.10) between reference slopes and subsequent slopes 3-8 years later, either within or across variables. The correlation between 2-year FEV1 slopes and FEV1 level even 5 years later was moderate (0.37-0.49) across disease stage categories. Conclusions Contrary to our hypothesis, rate of lung function decline did not predict future rate of decline either within or across spirometric variables. In contrast, FEV1 slope did have moderate predictive ability for subsequent FEV1 level. These findings are relevant for clinical care and for clinical trial design.

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