A recent study (J Pediatr 2014; 165: 1091-7) showed significant improvement in lung function among 6-year-olds with Cystic Fibrosis (CF) between 1994-2012.

Using the Cystic Fibrosis Foundation Patient Registry with a total of 11,670 children, the authors found that the mean FEV1 and FVC z-scores increased significantly over the period in the entire cohort.  In addition, the height-for-age (HFA) also improved.  These results are easy to see graphically in Figures 1 & Figure 2.  The authors note that in 2012, children who were identified by screening had improved HFA, FEV1, and FVC compared to children who were not identified by screening.

Take-home points:

• These data show impressive improvement in lung function and growth over the past two decades
• These values are going to improve further now that all 50 states mandate newborn screening for CF
• While there is improvement, 6-year-olds with CF still have measurable pulmonary dysfunction; thus, more work is needed, perhaps with novel therapies.

Related blog posts:

• Ivacaftor for Cystic Fibrosis | gutsandgrowth
• Fluctuating Elastase Levels in Infants with Cystic Fibrosis …