Measuring Progression
Measuring Progression
Measuring
Progression
Progression in cystic fibrosis (CF) can be measured in many ways, depending on the organ of interest. Some measurements are used infrequently in clinical practice, while other measurements may be taken at every clinic visit.1,2
Chest X-Rays
Used to detect early abnormalities, including hyperinflation, peribronchial cuffing, or streakiness.2
ppFEV1
Pulmonary outcome measure used both in clinical practice and in clinical trials.3
BMI
Body mass index (BMI) and BMI percentile can be used to assess nutritional status.3,4
FE-1
Highly predictive of pancreatic insufficiency.2
Oropharyngeal Cultures
Routine surveillance of respiratory microbiology is necessary, including eradication regimens, regardless of presence or absence of symptoms.2
LCI
Lung clearance index (LCI) is a sensitive measure of overall ventilation inhomogeneity assessed by multiple-breath washout that is based on tidal breathing techniques.5
Chest X-Rays
Used to detect early abnormalities, including hyperinflation, peribronchial cuffing, or streakiness.2
FE-1
Highly predictive of pancreatic insufficiency.2
ppFEV1
Pulmonary outcome measure used both in clinical practice and in clinical trials.3
Oropharyngeal Cultures
Routine surveillance of respiratory microbiology is necessary, including eradication regimens, regardless of presence or absence of symptoms.2
BMI
Body mass index (BMI) and BMI percentile can be used to assess nutritional status.3,4
LCI
Lung clearance index (LCI) is a sensitive measure of overall ventilation inhomogeneity assessed by multiple-breath washout that is based on tidal breathing techniques.5
FE-1, fecal elastase-1; ppFEV1, percent predicted forced expiratory volume in 1 second.
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References: 1. Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125 (1 Suppl):1S-39S. doi:10.1378/chest.125.1_suppl.1s. 2. Borowitz D, Robinson KA, Rosenfeld M, et al. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155(suppl 6):S73-93. doi:10.1016/j.jpeds.2009.09.001 3. Boyle MP, Sabadosa KA, Quinton HB, Marshall BC, Schechter MS. Key findings of the US Cystic Fibrosis Foundation’s clinical practice benchmarking project. BMJ Qual Saf. 2014;23:i15-i22. doi:10.1136/bmjqs-2013-002369 4. Zani EM, Grandinetti R, Cunico D, et al. Nutritional care in children with cystic fibrosis. Nutrients. 2023;15:479. doi: 10.3390/nu15030479 5. Gustafsson PM, De Jong PA, Tiddens HA, Lindblad A. Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis. Thorax. 2008;63(2):129-134. doi:10.1136/thx.2007.077784