Non-invasive ventilation and clinical outcomes in cystic fibrosis: Findings from the UK CF registry

Abstract

Background: Non-invasive ventilation (NIV) for respiratory failure and airway clearance is an established intervention in cystic fibrosis (CF), but its therapeutic benefit on lung function and survival remains under-investigated.

Methods: Using data from the UK CF Registry between 2007 and 2015, we explored the patterns of NIV use, and assessed changes in mean percent predicted FEV₁ (ppFEV₁) prior to and after NIV use, and the survival of patients on NIV.

Results: Among 11,079 patients, 1107 had at least one record of NIV treatment. Incidence and prevalence of NIV was lower in children and followed non-linear temporal patterns. Adjusting for other risk factors, ppFEV₁ rose by 0.70 (95%CI: -0.83, 2.24) after first NIV use in children. In adults with a low ppFEV₁ (<40%) at initiation of treatment, NIV increased mean ppFEV₁ by 2.60 (95% CI: 0.93, 4.27). Our analysis showed that NIV initiation is associated with an increased risk of death/transplant in both children (HR = 2.47; 95%CI: 1.20-5.08) and adults (HR = 1.96; 95% CI: 1.63-2.36) but effect was attenuated in children with low ppFEV₁ (<40%).

Conclusions: NIV usage in CF improves spirometric values but does not benefit survival. Further studies are required to better understand survival outcomes and ultimately improve NIV outcomes in CF.

Keywords: Cystic fibrosis; Non-invasive ventilation; Survival.