Increased adherence to CFF practice guidelines for pulmonary medications correlates with improved FEV1

Pediatr Pulmonol. 2013 Aug;48(8):747-53. doi: 10.1002/ppul.22665. Epub 2012 Sep 19.


Background: CFF practice guidelines recommend patients ≥age 6 use dornase alfa and hypertonic saline daily, and those ≥age 6 colonized with Pseudomonas aeruginosa use inhaled tobramycin and oral azithromycin to improve lung function and reduce pulmonary exacerbations. A decline in FEV1 was noted in our 2008 CF Center Report. We hypothesized that increasing adherence to prescribing guidelines for these pulmonary medications would improve mean FEV1.

Methods: This was a quality improvement project completed at a US CF center. CFF practice guidelines were reviewed with the center physicians. Patients were identified that were eligible to receive recommended therapies and it was determined whether they were prescribed the therapies. Baseline FEV1 data was collected. Adherence rates and FEV1 were followed quarterly for 1 year. Providers received a quarterly report card with adherence rates, mean FEV1 compared to colleagues, and a list of eligible patients that were not prescribed recommended therapies.

Results: Ninety-two patients were included. At baseline, the overall adherence rate was 59%. Overall adherence increased quarterly (P = < 0.001). Each quarter there was improvement in adherence to prescribing for each medication (P < 0.001). Except in quarter 1, FEV1 increased quarterly (P = 0.092). There was moderate correlation (r = 0.533) between improved adherence and improved FEV1.

Conclusions: Educating clinicians about guidelines, providing feedback on adherence to guidelines, and monitoring prescribing patterns improves prescribing adherence. FEV1 showed improvement after months of sustained adherence, trending towards significance. Longer follow-up is necessary to determine if improved prescribing adherence translates into improved FEV1 or slows rate of decline in FEV1.

Keywords: adherence; azithromycin; cystic fibrosis; dornase alfa; guidelines; hypertonic saline; tobramycin.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Anti-Bacterial Agents / therapeutic use*
  • Child
  • Cystic Fibrosis / drug therapy*
  • Cystic Fibrosis / physiopathology
  • Female
  • Forced Expiratory Volume / drug effects*
  • Forced Expiratory Volume / physiology
  • Guideline Adherence / trends*
  • Humans
  • Male
  • Practice Guidelines as Topic / standards*
  • Quality Improvement*
  • Registries*
  • Saline Solution, Hypertonic / therapeutic use*


  • Anti-Bacterial Agents
  • Saline Solution, Hypertonic