Early life growth trajectories in cystic fibrosis are associated with lung function at age six

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: BMI; Cystic fibrosis; Early childhood; FEV(1)pp; Growth trajectories; Registry; WFL.

Figure 1.

Flow chart of the study cohort to determine growth trajectory classes through group based trajectory modeling

Figure 2.

Growth trajectories of children with cystic fibrosis from birth through age five. Growth trajectories were based on two growth parameters, the weight-for-length percentile during the first two years of life and the body mass index percentile for ages two up to age six. The percentage of children classified in each trajectory class are presented with trajectory class names. The 50^th percentile line is represented in black.