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.

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