Highlights
- •Six distinct trajectories of early growth were identified using group based trajectory modeling.
- •Growth trajectories that met nutritional guideline recommendations by age 6 were associated with higher FEV1pp.
- •Optimizing nutritional status in childhood remains a crucial aspect of early CF management, particularly for those children who do not currently qualify for HEMT.
Abstract
Background
Higher growth percentiles are associated with more favorable lung function in cystic
fibrosis (CF), prompting the creation of CF Foundation (CFF) nutritional guidelines.
Objectives
To describe early childhood growth trajectories within CF, to determine if growth
trajectories are associated with differences in lung function at age six, and to identify
factors that differ between trajectory groups.
Methods
Retrospective cohort study of children diagnosed with CF and born 2000–2011 using
the US CFF Patient Registry. Annualized growth parameters prior to age six were included
in group-based trajectory modeling to identify unique early life growth trajectories.
FEV1 percent predicted (FEV1pp) at age six was compared between trajectory groups
using linear regression. Factors associated with group membership were identified
using multinomial logistic regression.
Results
6,809 children met inclusion criteria. Six discrete growth trajectories were identified,
including three groups that began with growth parameters >50th percentile, termed:
“always high”, “gradual decliner”, “rapid decliner”, and three which began with growth
parameters <50th percentile, termed: “rapid riser”, “gradual riser”, “always low”.
FEV1pp at age six was highest for the Always High trajectory. The Always Low trajectory
was nearly 10% lower than the Always High trajectory. Sex, ethnicity, newborn screening
and pancreatic function were associated with trajectory class membership.
Conclusions
Distinct early life growth trajectories were identified within CF. Trajectories that
met CFF nutritional guideline recommendations were associated with higher FEV1pp at
age six. CF care teams should continue to partner with families to encourage interventions
to support optimal growth to improve lung function in CF.
Keywords
Abbreviations:
CF (cystic fibrosis), BMI (body mass index), NBS (newborn screening), FEV1pp (forced expiratory volume in one second as a percentage of predicted value), CFF (Cystic Fibrosis Foundation), CFFPR (Cystic Fibrosis Foundation Patient Registry), WFL (weight-for-length), GBTM (group-based trajectory modeling), APPC (average posterior probability of classification), OCC (odds of correct classification), ANOVA (analysis of variance), RRR (relative risk ratio), CI (confidence interval), Pa (Pseudomonas aeruginosa), MRSA (methicillin-resistant Staphylococcus aureus), HEMT (highly effective modulator therapies)To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of Cystic FibrosisAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Risk Factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis.J Pediatr. 2007; 151 (e1): 134-139https://doi.org/10.1016/j.jpeds.2007.03.006
- Risk factors for mortality before age 18 years in cystic fibrosis.Pediatr Pulmonol. 2017; 52: 909-915https://doi.org/10.1002/ppul.23715
- Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review.J Am Diet Assoc. 2008; 108: 832-839https://doi.org/10.1016/j.jada.2008.02.020
- Effects of diagnosis by newborn screening for cystic fibrosis on weight and length in the first year of life.JAMA Pediatr. 2017; 171: 546-554https://doi.org/10.1001/jamapediatrics.2017.0206
- Annual data report.Bethesda, Maryland2021
- Wisconsin cystic fibrosis neonatal screening group. Recovery of birth weight z score within 2 years of diagnosis is positively associated with pulmonary status at 6 years of age in children with cystic fibrosis.Pediatrics. 2009; 123: 714-722https://doi.org/10.1542/peds.2007-3089
- Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis.J Pediatr. 2003; 142: 624-630https://doi.org/10.1067/mpd.2003.152
- Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis.J Pediatr. 2013; 162 (e1): 530-535https://doi.org/10.1016/j.jpeds.2012.08.040
- Early life growth trajectories in cystic fibrosis are associated with pulmonary function at age 6 years.J Pediatr. 2015; 167 (e1): 1081-1088https://doi.org/10.1016/j.jpeds.2015.07.044
- The cystic fibrosis foundation patient registry. Design and methods of a national observational disease registry.Ann Am Thorac Soc. 2016; 13: 1173-1179https://doi.org/10.1513/AnnalsATS.201511-781OC
- Clinical practice guidelines from the cystic fibrosis foundation for preschoolers with cystic fibrosis.Pediatrics. 2016; (Published online March 23peds.2015-1784)https://doi.org/10.1542/peds.2015-1784
- Cystic fibrosis foundation evidence-based guidelines for management of infants with cystic fibrosis.J Pediatr. 2009; 155: S73-S93https://doi.org/10.1016/j.jpeds.2009.09.001
- Consensus report on nutrition for pediatric patients with cystic fibrosis.J Pediatr Gastroenterol Nutr. 2002; 35: 246-259https://doi.org/10.1097/00005176-200209000-00004
- National center for health statistics.CDC growth charts, United States2020 (Published May 30Accessed July 19, 2022)
- Multi-ethnic reference values for spirometry for the 3–95-yr age range: the global lung function 2012 equations.Eur Respir J. 2012; 40: 1324-1343https://doi.org/10.1183/09031936.00080312
- Group-Based Trajectory modeling: an overview.Ann Nutr Metab. 2014; 65: 205-210https://doi.org/10.1159/000360229
- Trajectories of Boys’ physical aggression, opposition, and hyperactivity on the path to physically violent and nonviolent juvenile delinquency.Child Dev. 1999; 70: 1181-1196https://doi.org/10.1111/1467-8624.00086
- Group-based trajectory modeling in clinical research.Annu Rev Clin Psychol. 2010; 6: 109-138https://doi.org/10.1146/annurev.clinpsy.121208.131413
- Association between insurance variability and early lung function in children with cystic fibrosis.J Cyst Fibros. 2021; (Published online June 24)https://doi.org/10.1016/j.jcf.2021.06.006
- The GRoLTS-Checklist: guidelines for reporting on latent trajectory studies.Struct Equ Model Multidiscip J. 2017; 24: 451-467https://doi.org/10.1080/10705511.2016.1247646
- A note on a stata plugin for estimating group-based trajectory models.Sociol Methods Res. 2013; 42: 608-613https://doi.org/10.1177/0049124113503141
- Gender differences in outcomes of patients with cystic fibrosis.J Womens Health. 2014; 23: 1012-1020https://doi.org/10.1089/jwh.2014.4985
- Effect of CFTR modulators on anthropometric parameters in individuals with cystic fibrosis: an evidence analysis center systematic review.J Acad Nutr Diet. 2021; 121 (e2): 1364-1378https://doi.org/10.1016/j.jand.2020.03.014
- Nutritional outcomes following gastrostomy in children with cystic fibrosis.Pediatr Pulmonol. 2012; 47: 743-748https://doi.org/10.1002/ppul.22507
- Nutritional effects of long-term gastrostomy feedings in children with cystic fibrosis.J Am Diet Assoc. 1999; 99: 191-194https://doi.org/10.1016/S0002-8223(99)00046-2
- A Pre-Post Retrospective study of patients with cystic fibrosis and gastrostomy tubes.J Pediatr Gastroenterol Nutr. 2011; (Published online May): 1https://doi.org/10.1097/MPG.0b013e3182250c43
- Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project.Thorax. 2002; 57: 596-601https://doi.org/10.1136/thorax.57.7.596
- Factors affecting clinical outcome in gastrostomy-fed children with cystic fibrosis.Pediatr Pulmonol. 2004; 37: 324-329https://doi.org/10.1002/ppul.10321
- Percutaneous endoscopic gastrostomy feeding in patients with cystic fibrosis.Gut. 1999; 44: 87-90https://doi.org/10.1136/gut.44.1.87
- Post-2000 growth trajectories in children aged 4–11 years: a review and quantitative analysis.Prev Med Rep. 2019; 14100834https://doi.org/10.1016/j.pmedr.2019.100834
- Risk indicators of food insecurity in the CF population.Health Policy Manag Issue Briefs. 2021; (Paper 59)
- Mealtime behaviors in families of infants and toddlers with cystic fibrosis.J Cyst Fibros. 2005; 4: 175-182https://doi.org/10.1016/j.jcf.2005.05.015
Article info
Publication history
Published online: February 28, 2023
Accepted:
February 20,
2023
Received in revised form:
February 11,
2023
Received:
November 8,
2022
Publication stage
In Press Corrected ProofIdentification
Copyright
© 2023 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
