![Professor Scott Bell](https://thelimbic.com/wordpress/wp-content/uploads/2019/10/19_0186_Scott-Bell_3-214x300.jpg)
Professor Scott Bell
France should be the benchmark for management of cystic fibrosis (CF) as their patients have the highest recorded median age of survival.
A comparison of CF survival between Australia, Canada, France and New Zealand found the median age of survival was 53.3 years, 55.4 years, 65.9 years and 54.8 years respectively.
The data, from 14,842 patients across four national registries between 2015 and 2019, predates the widespread availability of elexacaftor–tezacaftor–ivacaftor.
The study found the risk of death was higher in Canada (HR 1.79, 95% CI: 1.44 to 2.22; p<0.001) and Australia (HR 2.04, 95% CI: 1.61 to 2.59; p<0.001) compared with France, even after adjusting for sex, age at diagnosis, newborn screening status, pancreatic status and lung transplant status, baseline ppFEV1 and BMI category.
“The exceptional survival in France is despite having the highest proportion of underweight individuals which has consistently been found in the literature to be strong predictor of worse outcomes in CF.”
The study, published in Thorax (link), said Canada had the lowest percentage of individuals who were diagnosed through newborn screening (13.8%).
France had the lowest proportion of individuals who were homozygous for Phe508del (42%) and the highest percentage of people with ‘other’ mutations (13.9%).
France also had the highest proportion of transplanted patients (16.5%) compared to Australia (11.7%), Canada (13.3%), and New Zealand (6.7%). Age at transplant and age at death were the lowest in France.
“Using contemporary CF data, our study identified a survival gap between people living in high-income countries with similar healthcare systems,” the study said.
The authors, including Professor Scott Bell, from the Prince Charles Hospital and the University of Queensland’s Child Health Research Centre, said it was unlikely that a single factor could explain the differences in survival.