Progress in Cystic Fibrosis over 18 Years

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:

2 thoughts on “Progress in Cystic Fibrosis over 18 Years

  1. Pingback: N2U -Part 3: EoE, IBD, and Cystic Fibrosis | gutsandgrowth

  2. Pingback: Characterizing Severe Liver Disease with Cystic Fibrosis | gutsandgrowth

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