Early Childhood Risk Factors for Decreased FEV1 at Age Six to Seven Years in Young Children with Cystic Fibrosis

Don B Sanders, Julia Emerson, Clement L Ren, Michael S Schechter, Ronald L Gibson, Wayne Morgan, Margaret Rosenfeld, EPIC Study Group, Paul Comber, Terese Evens, Magdalen Gondor, Kathy Hosler, Stasia Lehmann, Peter Hiatt, Omalee Lopez, Charlene Hallmark, Karen Daigle, Camille White, Blakeslee Noyes, Vikki Kociela, Henry Dorkin, Jonathan Greenberg, Ashish George, Erin Leone Thakkallapalli, Jane Solomon, John McNamara, Sandy Landvik, Mary Sachs, Frank Accurso, Meg Anthony, Shelley Mann, Arnold Platzker, Gregory Omlor, Deborah Ouellette, Ibrahim Abdulhamid, Catherine Van Wagnen, Diana Quintero, MaryEllen Freeman, Tami Miller, Hector Gutierrez, Heather Hathorne, Gina Sabbatini, David Orenstein, Caitlin Clarke, Judy Fulton, Elizabeth Hartigan, Sandra Hurban, Allen Dozor, Ingrid Gherson, Madelint Heydendael, Robert Fink, Sandy Bartosik, Philip Black, Rose Thompson, Karie Robinson, Candy Schmoll, Thomas Lahiri, Joan Lippmann, Sandra Diehl, Joan DeCelie-Germana, Lynn Bonitz, Susan Galvin, Maynard Dyson, Trudy Morris, Sara Scott, H Worth Parker, Alix Ashare, Jennifer Helm, Dana Dorman, Pamela Hofley, Lisa Moulton, Aaron Chidekel, Sandra Budd, Michael Schechter, Arlene Stecenko, Monica Haughton, Eric Hunter, Jeannette Peabody, Joy Dangerfield, Susan Millard, Mary Flanagan, Hollie Bonnema, Teri Crumb, Barbara Chatfield, Heather Oldroyd, Jane Vroom, Gregory Shay, Julie Lee, Erika Marmolejo, Robert Schoumacher, Barbara Culbreath, Kathleen McKie, Heidi Stapp, Gavin Graff, Lisa Allwein, Diane Kitch, AnneMarie Cairns, Harmony Renna, Mary Ellen Corrigan, Carrie Milliard, Robert Zanni, Bridget Marra, Karen McCoy, Barbara Butera, Diana Gilmore, Mary Terri Johnson, Veronica Lowell, Patricia Olson, Laura Raterman, David Schaeffer, Elizabeth DeLuca, Rena Sprinkle, Adrienne Prestridge, Jennifer Milam, Catherine Powers, Michael Wall, Alexandra Cornell, Aaron Guzik, Ben McCullar, Michael Konstan, Colette Bucur, Ellen Divoky, Kate Hilliard, Jeanne Krenicky, Cheryl Velotta, Michelle Howenstine, Terry Barclay, Lisa Bendy, Margaret Rosenfeld, Sharon McNamara, Judy Gabrysiak, Alan Genatossio, Laurie Varlotta, Marcella Aramburo, Thomas Ferkol, Mary Boyle, Patricia Burks, Jane Quante, Richard Moss, Zoe Davies, Colleen Dunn, Cassie Everson, Ran Anbar, Donna Lindner, Valoree Suttmore, Brian O'Sullivan, Karen Longtine, Dennis Nielson, Courtney Moreno, Richard Ahrens, Jean Frauenholtz, Tom Santacroce, Mary Teresi, Jamshed Kanga, Brittany Fuller, Tammy Taylor, Tammy Watts, Samya Nasr, Dawn Kruse, Suzanne Miller, Michelle King, Joseph Majure, Kim Adcock, Linda Bonham, John Colombo, Diane Acquazzino, Shandalle Fertig, Sandra Strizek, George Retsch-Bogart, Carol Barlow, Paul Jones, Kelly Moormann, Caroline LaFave, Clement Ren, Nancy Jenks, Mary Platt, Deborah Froh, Mary Alice Blackwell, Patricia Moss, Michael Rock, Linda Makholm, Rebekah Brown, Alice Bray, Daniel Sheehan, Jameelah Ali, Nadine Caci, Don B Sanders, Julia Emerson, Clement L Ren, Michael S Schechter, Ronald L Gibson, Wayne Morgan, Margaret Rosenfeld, EPIC Study Group, Paul Comber, Terese Evens, Magdalen Gondor, Kathy Hosler, Stasia Lehmann, Peter Hiatt, Omalee Lopez, Charlene Hallmark, Karen Daigle, Camille White, Blakeslee Noyes, Vikki Kociela, Henry Dorkin, Jonathan Greenberg, Ashish George, Erin Leone Thakkallapalli, Jane Solomon, John McNamara, Sandy Landvik, Mary Sachs, Frank Accurso, Meg Anthony, Shelley Mann, Arnold Platzker, Gregory Omlor, Deborah Ouellette, Ibrahim Abdulhamid, Catherine Van Wagnen, Diana Quintero, MaryEllen Freeman, Tami Miller, Hector Gutierrez, Heather Hathorne, Gina Sabbatini, David Orenstein, Caitlin Clarke, Judy Fulton, Elizabeth Hartigan, Sandra Hurban, Allen Dozor, Ingrid Gherson, Madelint Heydendael, Robert Fink, Sandy Bartosik, Philip Black, Rose Thompson, Karie Robinson, Candy Schmoll, Thomas Lahiri, Joan Lippmann, Sandra Diehl, Joan DeCelie-Germana, Lynn Bonitz, Susan Galvin, Maynard Dyson, Trudy Morris, Sara Scott, H Worth Parker, Alix Ashare, Jennifer Helm, Dana Dorman, Pamela Hofley, Lisa Moulton, Aaron Chidekel, Sandra Budd, Michael Schechter, Arlene Stecenko, Monica Haughton, Eric Hunter, Jeannette Peabody, Joy Dangerfield, Susan Millard, Mary Flanagan, Hollie Bonnema, Teri Crumb, Barbara Chatfield, Heather Oldroyd, Jane Vroom, Gregory Shay, Julie Lee, Erika Marmolejo, Robert Schoumacher, Barbara Culbreath, Kathleen McKie, Heidi Stapp, Gavin Graff, Lisa Allwein, Diane Kitch, AnneMarie Cairns, Harmony Renna, Mary Ellen Corrigan, Carrie Milliard, Robert Zanni, Bridget Marra, Karen McCoy, Barbara Butera, Diana Gilmore, Mary Terri Johnson, Veronica Lowell, Patricia Olson, Laura Raterman, David Schaeffer, Elizabeth DeLuca, Rena Sprinkle, Adrienne Prestridge, Jennifer Milam, Catherine Powers, Michael Wall, Alexandra Cornell, Aaron Guzik, Ben McCullar, Michael Konstan, Colette Bucur, Ellen Divoky, Kate Hilliard, Jeanne Krenicky, Cheryl Velotta, Michelle Howenstine, Terry Barclay, Lisa Bendy, Margaret Rosenfeld, Sharon McNamara, Judy Gabrysiak, Alan Genatossio, Laurie Varlotta, Marcella Aramburo, Thomas Ferkol, Mary Boyle, Patricia Burks, Jane Quante, Richard Moss, Zoe Davies, Colleen Dunn, Cassie Everson, Ran Anbar, Donna Lindner, Valoree Suttmore, Brian O'Sullivan, Karen Longtine, Dennis Nielson, Courtney Moreno, Richard Ahrens, Jean Frauenholtz, Tom Santacroce, Mary Teresi, Jamshed Kanga, Brittany Fuller, Tammy Taylor, Tammy Watts, Samya Nasr, Dawn Kruse, Suzanne Miller, Michelle King, Joseph Majure, Kim Adcock, Linda Bonham, John Colombo, Diane Acquazzino, Shandalle Fertig, Sandra Strizek, George Retsch-Bogart, Carol Barlow, Paul Jones, Kelly Moormann, Caroline LaFave, Clement Ren, Nancy Jenks, Mary Platt, Deborah Froh, Mary Alice Blackwell, Patricia Moss, Michael Rock, Linda Makholm, Rebekah Brown, Alice Bray, Daniel Sheehan, Jameelah Ali, Nadine Caci

Abstract

Rationale: There are limited objective measures of the severity of lung disease before children are able to routinely perform spirometry, generally at age 6 years. Identifying risk factors for reduced lung function at age 6 provides opportunities to intervene and slow the progression of cystic fibrosis (CF) lung disease.

Objectives: To evaluate early childhood predictors of lung function at age 6-7 in a large U.S. CF cohort in the current era of widespread early eradication therapy for Pseudomonas aeruginosa (P. aeruginosa).

Methods: Participants were children with CF enrolled before age 4 in the Early Pseudomonas Infection Control (EPIC) Observational Study, a multicenter, longitudinal study that enrolled P. aeruginosa-negative children not exceeding 12 years of age. Linear regression was used to estimate the association between potential early childhood risk factors and the best FEV1% predicted at age 6-7 years.

Measurements and main results: Four hundred and eighty-four children (of 1,797 enrolled in the EPIC Observational Study) met the eligibility criteria for this analysis. Mean (SD) age at enrollment was 2.0 (1.3) years. In a multivariable model adjusted for age at enrollment, the following risk factors were significantly associated with lower mean (95% confidence interval) FEV1% predicted at age 6-7: weight percentile less than 10% during the year of enrollment (-5.3 [-9.1, -1.5]), P. aeruginosa positive during the year of enrollment (-2.8 [-5.7, 0.0]), crackles or wheeze during the year of enrollment (-5.7 [-9.4, -1.9]), mother's education of high school or less (-4.2 [-7.3, -1.2]), and mother smoked during pregnancy (-4.4 [-8.8, 0.1]).

Conclusions: In this large U.S. cohort, we identified several early childhood risk factors for lower FEV1 at age 6-7 years, most of which are modifiable. Clinical trial registered with www.clinicaltrials.gov (NCT00097773).

Keywords: cystic fibrosis; lung function; microbiology; tobacco smoke pollution.

Figures

Figure 1.
Figure 1.
Study flow chart of cohort for the current analysis. EPIC OBS = Early Pseudomonas Infection Control Observational Study; Pa = Pseudomonas aeruginosa.

Source: PubMed

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