Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2012 Feb;47(2):135-43.
doi: 10.1002/ppul.21526. Epub 2011 Aug 24.

Lung function decline from adolescence to young adulthood in cystic fibrosis

Stacy L Vandenbranden  1 , Ann McMullen, Michael S Schechter, David J Pasta, Rory L Michaelis, Michael W Konstan, Jeffrey S Wagener, Wayne J Morgan, Susanna A McColley; Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis
Affiliations

Lung function decline from adolescence to young adulthood in cystic fibrosis

Stacy L Vandenbranden et al. Pediatr Pulmonol. 2012 Feb.

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 ≥5 encounters and ≥5 measurements of forced expiratory volume in 1 second (FEV(1) ) spanning ≥1 year during both adolescence and young adulthood, and ≥1 encounter with weight and height and ≥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.

PubMed Disclaimer

Conflict of interest statement

Disclosure of Conflict of Interest

This study is sponsored by Genentech, Inc. Ann McMullen, Michael Schechter, Michael Konstan, Jeffrey Wagener, Wayne Morgan, and Susanna McColley have received honoraria from Genentech, Inc. for serving as members of the Scientific Advisory Group for the Epidemiologic Study of Cystic Fibrosis (ESCF). Stacy VandenBranden, Ann McMullen, Michael Konstan, Wayne Morgan, Jeffrey Wagener, and Susanna McColley have served as consultants to Genentech. No compensation was provided to these authors in exchange for production of this manuscript. David Pasta and Rory Michaelis are employees of ICON Clinical Research, which was paid by Genentech for providing analytical services for this study. Jeffrey Wagener was previously an employee of Genentech.

Figures

Fig. 1
Fig. 1
Timeline and FEV1 assessments.
Fig. 2
Fig. 2
Patient disposition.
Fig. 3
Fig. 3
FEV1 % predicted by age and CF severity.
See this image and copyright information in PMC

References

    1. Parker HW. Transition and transfer of patients who have cystic fibrosis to adult care. Clin Chest Med. 2007;28:423–432. - PubMed
    1. 2009 Annual Data Report. Bethesda, MD: Cystic Fibrosis Foundation; 2010. Cystic Fibrosis Foundation Patient Registry.
    1. Morgan WJ, Butler SM, Johnson CA, Colin AA, FitzSimmons SC, Geller DE, Konstan MW, Light MJ, Rabin HR, Regelmann WE, Schidlow DV, Stokes DC, Wohl ME, Kaplowitz H, Wyatt MM, Stryker S. Epidemiologic Study of Cystic Fibrosis: design and implementation of a prospective, multicenter, observational study of patients with cystic fibrosis in the U.S and Canada. Pediatr Pulmonol. 1999;28:231–241. - PubMed
    1. Ren CL, Pasta DJ, Rasouliyan L, Wagener JS, Konstan MW, Morgan WJ. Relationship between inhaled corticosteroid therapy and rate of lung function decline in children with cystic fibrosis. J Pediatr. 2008;153:746–751. - PubMed
    1. Konstan MW, Wagener JS, Pasta DJ, Millar SJ, Jacobs JR, Yegin A, Morgan WJ. Clinical use of dornase alfa is associated with a slower rate of FEV1 decline in cystic fibrosis. Pediatr Pulmonol. 2011 (in press) - PMC - PubMed

Publication types

MeSH terms

Substances