Early Assessment of Risk Factors
Structural lung damage and early risk factors can be present before abnormal lung function
Structural lung damage may well be under way prior to abnormal lung function
* High-resolution computed tomography.
† Image courtesy of J. Wagener, MD.
‡ Reproduced with permission from Tiddens HAWM. Pediatr Pulmonol. 2002;34(3):228-231.
FEF25-75 = forced expiratory flow at 25% to 75% of forced vital capacity; FEV1 = forced expiratory volume in 1 second; FVC = forced vital capacity.
At-risk patients can be identified even before routine pulmonary function tests (PFTs) are performed
Lower nutritional factors (weight for age and height for age) were also associated with reduced pulmonary function at age 6 in this study. (Ref: 2)
Study design: This prospective, observational study of 931 patients from the Epidemiologic Study of Cystic Fibrosis was designed to determine the relation of growth and nutritional status, along with other risk factors, to pulmonary function in young children with CF.
Risk assessment should go beyond PFTs
High baseline FEV1 was among several other risk factors associated with a steeper rate of pulmonary decline
High lung function should not be clinically reassuring
Across the 3 different age groups (ages 5-8, 9-12, 13-17) in the study, high baseline FEV1 and crackles were significantly associated with a steeper rate of decline. (Ref: 3)
Study design: This was a prospective, observational study of 4866 patients from the Epidemiologic Study of Cystic Fibrosis (ESCF). Its objectives were to characterize the rate of pulmonary function decline over a period of 4 years in 3 different age groups (6 to 8 years, 9 to 12 years, 13 to 17 years) and to identify and compare risk factors associated with pulmonary decline.
Normal pulmonary function did not predict a slower rate of disease progression. (Ref: 4)
Adapted with permission from Corey M et al. J Pediatr. 1997;131(6):809-814.
Study design: This mixed-model regression analysis of a birth cohort of 366 CF patients with 2 or more measurements of pulmonary function (at least 1 of them before the age of 10 years) sought to estimate the rates of decline of pulmonary function on the basis of survival age, sex, pancreatic status, and genotype.
Risk can be assessed prior to lung function loss
Evaluating all disease contributors and risk factors can help assess Cystic Fibrosis lung disease risk prior to measurable FEV1 loss
*Cough, sputum, clubbing, crackles.
Next Steps
