Approach to treating cystic fibrosis pulmonary exacerbations varies widely across US CF care centers

Pediatr Pulmonol. 2011 Sep;46(9):870-81. doi: 10.1002/ppul.21442. Epub 2011 Apr 4.


There is no standard definition of a CF pulmonary exacerbation universally accepted by clinicians. We aimed to investigate the variability of clinical practice among US CF clinicians in the diagnosis and treatment of exacerbations. Using clinical vignettes, we examined if variation in the identification and treatment of CF exacerbations is common, if practice patterns differ between CF care centers and what clinical factors determine treatment. Twenty-eight clinical cases were developed by varying five clinical factors. Participants were given four options for treatment of the patient described in each vignette. Cases were sent via email to a convenience sample of 112 CF clinicians from 13 US CF centers, with 109 clinicians participating (97.3%). 2,792 of the 3,052 cases received a response (91.5%). ANOVA demonstrated variation in rater scores was explained by case scenario and by care center (P < 0.0001). Examining the frequency of each treatment strategy demonstrated no absolute treatment consensus for any given scenario and variability within and between care centers. Direct logistic regression revealed that systemic symptoms (OR = 5.95), decreased O(2) saturation (OR = 4.99) and decreased FEV(1) (OR = 3.78) had a greater effects on the decision to treat a case with IV antibiotics than increased cough/sputum (OR = 2.19) and crackles present on physical examination (OR = 2.10). Similar findings were demonstrated with a cluster analysis. There was surprising variation in the identification and treatment of pulmonary exacerbations by CF clinicians. Variation was present between CF Centers, within each CF center and at the individual clinician level. This study provides additional evidence for the need of a standard definition for a CF pulmonary exacerbation.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Anti-Bacterial Agents / therapeutic use*
  • Cough / diagnosis
  • Cough / drug therapy
  • Cystic Fibrosis / complications*
  • Disease Progression
  • Female
  • Forced Expiratory Volume
  • Health Care Surveys*
  • Humans
  • Lung Diseases / drug therapy*
  • Lung Diseases / etiology*
  • Male
  • Oxygen / blood
  • Respiratory Sounds / diagnosis
  • Sputum
  • United States
  • Young Adult


  • Anti-Bacterial Agents
  • Oxygen