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Multicenter Study
. 2020 Oct 15;10(1):17421.
doi: 10.1038/s41598-020-74502-1.

Lung function over the life course of paediatric and adult patients with cystic fibrosis from a large multi-centre registry

Arul Earnest  1 , Farhad Salimi  2 , Claire E Wainwright  3   4 , Scott C Bell  3   5   6 , Rasa Ruseckaite  2 , Tom Ranger  2 , Tom Kotsimbos  7 , Susannah Ahern  2
Affiliations
Multicenter Study

Lung function over the life course of paediatric and adult patients with cystic fibrosis from a large multi-centre registry

Arul Earnest et al. Sci Rep. .

Abstract

A key measure of lung function in people with Cystic Fibrosis (CF) is Forced Expiratory Volume in the first second FEV1 percent predicted (FEV1pp). This study aimed to address challenges in identifying predictors of FEV1pp, specifically dealing with non-linearity and the censoring effect of death. Data was obtained from a large multi-centre Australian Cystic Fibrosis Data Registry (ACFDR). A linear mixed model was used to study FEV1pp as the endpoint. There were 3655 patients (52.4% male) included in our study. Restricted cubic splines were used to fit the non-linear relationship between age of visit and FEV1pp. The following predictors were found to be significant in the multivariate model: age of patient at visit, BMI z-score, age interaction with lung transplantation, insulin dependent diabetes, cirrhosis/portal hypertension, pancreatic insufficiency, Pseudomonas aeruginosa infection and baseline variability in FEV1pp. Those with P. aeruginosa infection had a lower mean difference in FEV1pp of 4.7 units, p < 0.001 compared to those who did not have the infection. Joint modelling with mortality outcome did not materially affect our findings. These models will prove useful for to study the impact of CFTR modulator therapies on rate of change of lung function among patients with CF.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Figure 1
Figure 1
Restricted cubic splines showing non-linear relationship between FEV1 percent predicted and age at visit.
Figure 2
Figure 2
Decline in FEV1 percent predicted by key subgroups based on the final multivariate model.
See this image and copyright information in PMC

References

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