Increasing life expectancy in cystic fibrosis: Advances and challenges

Pediatr Pulmonol. 2022 Feb;57 Suppl 1(Suppl 1):S5-S12. doi: 10.1002/ppul.25733. Epub 2021 Nov 11.

Abstract

Since the first description of cystic fibrosis in 1938, there have been significant advances in both quality of life and longevity for people living with this disease. In this article we describe the milestones of the last 80 years and what we perceive to be the remaining barriers to normalcy for this population.

Keywords: CFTR modulators; cystic fibrosis; lung function; survival.

Publication types

  • Review

MeSH terms

  • Aminophenols
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Cystic Fibrosis*
  • Humans
  • Life Expectancy
  • Mutation
  • Quality of Life

Substances

  • Aminophenols
  • Cystic Fibrosis Transmembrane Conductance Regulator