Cystic Fibrosis Outcome Data

The Cystic Fibrosis Foundation (CFF) now publishes national outcome data from cystic fibrosis (CF) centers across the United States.

Below are pulmonary function and nutritional outcomes from UPMC Children's Hospital of Pittsburgh. Both outcomes are considered important indicators of the health of CF patients. These two aspects have significant prognostic implications and are strong determinants of longevity (length of life) and quality of life. The Forced Expiratory Volume in 1 second (FEV1, the volume of air a person is able to forcefully blow out in a second) is considered a good indicator of lung function. The Body Mass Index (BMI, a ratio of body weight to stature) is considered a good indicator of nutritional status.

Children’s Hospital's data is shown compared to the average outcomes reported by the CFF for all accredited CF centers in the United States and also against the values reported by those centers regarded as top 10 performers. (2020 data)

Measures Level at Children's CF Center National Average

Lung Function in Children*
Median FEV1 percent of predicted in children 6 to 12 years of age.

 96.8 97.5
National Status in Children*
Median BMI percentile in children 2 to 19 years of age.		  55.9 61.8
Screening for CF-related Diabetes
Percent of non-diabetic patients 10 years of age and older screened for CF-related Diabetes (CFRD).		 66 51.3
Guidelines for Care in Children
Percent of children less than 18 years of age who had at least one clinic visits, one lung function tests, and a sputum or throat culture. 		 98.8 87.5

* Adjusted for attained age of patients, gender, pancreatic sufficiency, race/ethnicity, socioeconomic status, and age of diagnosis

The above data is raw data supplied to us by the Cystic Fibrosis Foundation (CFF). There are factors that can influence the data. The CFF has made adjustments in the data (adjusted for attained age of patients, gender, pancreatic sufficiency, race/ethnicity, socioeconomic status, and age of diagnosis) and has publicly reported this on its Web site.