Lung function decline from adolescence to young adulthood in cystic fibrosis

Stacy L. Vandenbranden, Ann McMullen, Michael S. Schechter, David J. Pasta, Rory L. Michaelis, Michael W. Konstan, Jeffrey S. Wagener, Wayne J Morgan, Susanna A. McColley

Research output: Contribution to journalArticle

50 Citations (Scopus)

Abstract

Background Despite improving survival in cystic fibrosis (CF) patients, there is a mortality peak in early adulthood. Defining risk factors that predict significant worsening of lung disease in young adulthood may identify opportunities to improve outcomes in adults. Methods We identified 4,680 patients in the Epidemiologic Study of Cystic Fibrosis 1994-2005 with data in both adolescence (age 14.0-17.4 years) and young adulthood (age 18.5-22.0 years) and analyzed 2,267 who had ≤yen;5 encounters and ≤yen;5 measurements of forced expiratory volume in 1 second (FEV 1) spanning ≤yen;1 year during both adolescence and young adulthood, and ≤yen;1 encounter with weight and height and ≤yen;1 FEV 1 measurement age 17.5-18.5 years. We compared the annualized rates of decline in FEV 1 during adolescence and young adulthood stratified by best FEV 1 around age 18. Logistic regression was used to identify risk factors associated with substantial decline (>20 points) in FEV 1% predicted in young adulthood. Results Annual rate of decline was greater in young adulthood than in adolescence. Risk factors for substantial decline included slower rate of FEV 1 decline, greater FEV 1 variability, faster body mass index (BMI) decline, male sex, chronic inhaled antibiotics, Haemophilus influenzae detection, and absence of multidrug-resistant Pseudomonas aeruginosa in adolescence, and lower than expected FEV 1 and BMI around age 18. Conclusions Decline in lung function accelerates in young adults with CF, especially in those with early stage lung disease. Adolescents at risk for substantial decline in lung function in young adulthood have higher FEV 1 and worse nutritional status, among other identifiable risk factors.

Original languageEnglish (US)
Pages (from-to)135-143
Number of pages9
JournalPediatric Pulmonology
Volume47
Issue number2
DOIs
StatePublished - Feb 2012

Fingerprint

Forced Expiratory Volume
Cystic Fibrosis
Lung
Lung Diseases
Body Mass Index
Haemophilus influenzae
Nutritional Status
Pseudomonas aeruginosa
Epidemiologic Studies
Young Adult
Logistic Models
Anti-Bacterial Agents
Weights and Measures
Survival
Mortality

Keywords

  • H. influenzae
  • nutrition
  • P. aeruginosa
  • risk factors
  • transition
  • youth

ASJC Scopus subject areas

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

Cite this

Vandenbranden, S. L., McMullen, A., Schechter, M. S., Pasta, D. J., Michaelis, R. L., Konstan, M. W., ... McColley, S. A. (2012). Lung function decline from adolescence to young adulthood in cystic fibrosis. Pediatric Pulmonology, 47(2), 135-143. https://doi.org/10.1002/ppul.21526

Lung function decline from adolescence to young adulthood in cystic fibrosis. / Vandenbranden, Stacy L.; McMullen, Ann; Schechter, Michael S.; Pasta, David J.; Michaelis, Rory L.; Konstan, Michael W.; Wagener, Jeffrey S.; Morgan, Wayne J; McColley, Susanna A.

In: Pediatric Pulmonology, Vol. 47, No. 2, 02.2012, p. 135-143.

Research output: Contribution to journalArticle

Vandenbranden, SL, McMullen, A, Schechter, MS, Pasta, DJ, Michaelis, RL, Konstan, MW, Wagener, JS, Morgan, WJ & McColley, SA 2012, 'Lung function decline from adolescence to young adulthood in cystic fibrosis', Pediatric Pulmonology, vol. 47, no. 2, pp. 135-143. https://doi.org/10.1002/ppul.21526
Vandenbranden SL, McMullen A, Schechter MS, Pasta DJ, Michaelis RL, Konstan MW et al. Lung function decline from adolescence to young adulthood in cystic fibrosis. Pediatric Pulmonology. 2012 Feb;47(2):135-143. https://doi.org/10.1002/ppul.21526
Vandenbranden, Stacy L. ; McMullen, Ann ; Schechter, Michael S. ; Pasta, David J. ; Michaelis, Rory L. ; Konstan, Michael W. ; Wagener, Jeffrey S. ; Morgan, Wayne J ; McColley, Susanna A. / Lung function decline from adolescence to young adulthood in cystic fibrosis. In: Pediatric Pulmonology. 2012 ; Vol. 47, No. 2. pp. 135-143.
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abstract = "Background Despite improving survival in cystic fibrosis (CF) patients, there is a mortality peak in early adulthood. Defining risk factors that predict significant worsening of lung disease in young adulthood may identify opportunities to improve outcomes in adults. Methods We identified 4,680 patients in the Epidemiologic Study of Cystic Fibrosis 1994-2005 with data in both adolescence (age 14.0-17.4 years) and young adulthood (age 18.5-22.0 years) and analyzed 2,267 who had ≤yen;5 encounters and ≤yen;5 measurements of forced expiratory volume in 1 second (FEV 1) spanning ≤yen;1 year during both adolescence and young adulthood, and ≤yen;1 encounter with weight and height and ≤yen;1 FEV 1 measurement age 17.5-18.5 years. We compared the annualized rates of decline in FEV 1 during adolescence and young adulthood stratified by best FEV 1 around age 18. Logistic regression was used to identify risk factors associated with substantial decline (>20 points) in FEV 1{\%} predicted in young adulthood. Results Annual rate of decline was greater in young adulthood than in adolescence. Risk factors for substantial decline included slower rate of FEV 1 decline, greater FEV 1 variability, faster body mass index (BMI) decline, male sex, chronic inhaled antibiotics, Haemophilus influenzae detection, and absence of multidrug-resistant Pseudomonas aeruginosa in adolescence, and lower than expected FEV 1 and BMI around age 18. Conclusions Decline in lung function accelerates in young adults with CF, especially in those with early stage lung disease. Adolescents at risk for substantial decline in lung function in young adulthood have higher FEV 1 and worse nutritional status, among other identifiable risk factors.",
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AU - McMullen, Ann

AU - Schechter, Michael S.

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AU - Michaelis, Rory L.

AU - Konstan, Michael W.

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N2 - Background Despite improving survival in cystic fibrosis (CF) patients, there is a mortality peak in early adulthood. Defining risk factors that predict significant worsening of lung disease in young adulthood may identify opportunities to improve outcomes in adults. Methods We identified 4,680 patients in the Epidemiologic Study of Cystic Fibrosis 1994-2005 with data in both adolescence (age 14.0-17.4 years) and young adulthood (age 18.5-22.0 years) and analyzed 2,267 who had ≤yen;5 encounters and ≤yen;5 measurements of forced expiratory volume in 1 second (FEV 1) spanning ≤yen;1 year during both adolescence and young adulthood, and ≤yen;1 encounter with weight and height and ≤yen;1 FEV 1 measurement age 17.5-18.5 years. We compared the annualized rates of decline in FEV 1 during adolescence and young adulthood stratified by best FEV 1 around age 18. Logistic regression was used to identify risk factors associated with substantial decline (>20 points) in FEV 1% predicted in young adulthood. Results Annual rate of decline was greater in young adulthood than in adolescence. Risk factors for substantial decline included slower rate of FEV 1 decline, greater FEV 1 variability, faster body mass index (BMI) decline, male sex, chronic inhaled antibiotics, Haemophilus influenzae detection, and absence of multidrug-resistant Pseudomonas aeruginosa in adolescence, and lower than expected FEV 1 and BMI around age 18. Conclusions Decline in lung function accelerates in young adults with CF, especially in those with early stage lung disease. Adolescents at risk for substantial decline in lung function in young adulthood have higher FEV 1 and worse nutritional status, among other identifiable risk factors.

AB - Background Despite improving survival in cystic fibrosis (CF) patients, there is a mortality peak in early adulthood. Defining risk factors that predict significant worsening of lung disease in young adulthood may identify opportunities to improve outcomes in adults. Methods We identified 4,680 patients in the Epidemiologic Study of Cystic Fibrosis 1994-2005 with data in both adolescence (age 14.0-17.4 years) and young adulthood (age 18.5-22.0 years) and analyzed 2,267 who had ≤yen;5 encounters and ≤yen;5 measurements of forced expiratory volume in 1 second (FEV 1) spanning ≤yen;1 year during both adolescence and young adulthood, and ≤yen;1 encounter with weight and height and ≤yen;1 FEV 1 measurement age 17.5-18.5 years. We compared the annualized rates of decline in FEV 1 during adolescence and young adulthood stratified by best FEV 1 around age 18. Logistic regression was used to identify risk factors associated with substantial decline (>20 points) in FEV 1% predicted in young adulthood. Results Annual rate of decline was greater in young adulthood than in adolescence. Risk factors for substantial decline included slower rate of FEV 1 decline, greater FEV 1 variability, faster body mass index (BMI) decline, male sex, chronic inhaled antibiotics, Haemophilus influenzae detection, and absence of multidrug-resistant Pseudomonas aeruginosa in adolescence, and lower than expected FEV 1 and BMI around age 18. Conclusions Decline in lung function accelerates in young adults with CF, especially in those with early stage lung disease. Adolescents at risk for substantial decline in lung function in young adulthood have higher FEV 1 and worse nutritional status, among other identifiable risk factors.

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KW - transition

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