Early Lung Disease in Infants and Preschool Children with Cystic Fibrosis. What Have We Learned and What Should We Do about It?

Abstract

The past decade has seen significant advances in understanding of the pathogenesis and progression of lung disease in cystic fibrosis (CF). Pulmonary inflammation, infection, and structural lung damage manifest very early in life and are prevalent among preschool children and infants, often in the absence of symptoms or signs. Early childhood represents a pivotal period amenable to intervention strategies that could delay or prevent the onset of lung damage and alter the longer-term clinical trajectory for individuals with CF. This review summarizes what we have learned about early lung disease in children with CF and discusses the implications for future clinical practice and research.

Keywords: cystic fibrosis; early intervention; early lung disease; newborn screening.

Figure 1.

Median FEV₁ has improved over the past two decades in those with cystic fibrosis (CF) by greater than 10% in 2009 (upper curve) compared with 1989 (lower curve). When first measured in school-age children with CF, median FEV₁ is usually within the normal range (>80% predicted), where it remains throughout childhood (1). This indicates that factors such as treatment during the preschool years must have also improved. Despite this generational improvement in lung function, a large majority of children have underlying bronchiectasis, indicative of irreversible airway damage occurring irrespective of relatively preserved FEV₁.