Lung function decline from adolescence to young adulthood in cystic fibrosis

Abstract

SUMMARY Background Despite improving survival in cystic fibrosis (CF) patients, there is a mortality peak in early adulthood. Defining risk factors that predict significant worsening of lung disease in young adulthood may identify opportunities to improve outcomes in adults. Methods We identified 4680 patients in the Epidemiologic Study of Cystic Fibrosis 1994–2005 with data in both adolescence (age 14.0–17.4 years) and young adulthood (age 18.5–22.0 years) and analyzed 2267 who had ≥5 encounters and ≥5 measurements of forced expiratory volume in 1 second (FEV1) spanning ≥1 year during both adolescence and young adulthood, and ≥1 encounter with weight and height and ≥1 FEV1 measurement age 17.5–18.5 years. We compared the annualized rates of decline in FEV1 during adolescence and young adulthood stratified by best FEV1 around age 18. Logistic regression was used to identify risk factors associated with substantial decline (>20 points) in FEV1% predicted in young adulthood. Results Annual rate of decline was greater in young adulthood than in adolescence. Risk factors for substantial decline included slower rate of FEV1 decline, greater FEV1 variability, faster body mass index (BMI) decline, male sex, chronic inhaled antibiotics, Haemophilus influenzae detection, and absence of multidrug-resistant Pseudomonas aeruginosa in adolescence, and lower than expected FEV1 and BMI around age 18. Conclusions Decline in lung function accelerates in young adults with CF, especially in those with early stage lung disease. Adolescents at risk for substantial decline in lung function in young adulthood have higher FEV1 and worse nutritional status, among other identifiable risk factors.

Inhaled Tobramycin Effectively Reduces FEV1 Decline in Cystic Fibrosis. An Instrumental Variables Analysis

“…Eligible patients included those aged 6 to 21 years who were chronically infected with P. aeruginosa. Age restrictions were chosen to limit the cohort to valid pulmonary function tests (above 6 yr of age), to capture first occurrences of chronic P. aeruginosa infection (below 21 yr of age), and to focus on periods of increased risk for rapid lung function decline (22). Data acquired after organ transplantation were not included.…”

Inspiratory Muscle Strength and Endurance in Children and Adolescents with Cystic Fibrosis

“…13 Once infection is established, there is an accelerated decline in lung function, quality of life, and survival. 14,15 Furthermore, chronic P. aeruginosa infection has been related to decreased maximum inspiratory pressure (P Imax ) and is probably an independent predictor of respiratory muscle compromise in cystic fibrosis, 16 although its relation to muscle endurance remains unclear.…”

ChronicPseudomonas aeruginosaInfection and Respiratory Muscle Impairment in Cystic Fibrosis

“…2 P. aeruginosa infection is associated with gradually declining pulmonary status in children and young adults with CF, as assessed by lung function studies. 3,4 Furthermore, chronic pulmonary infec-tion with P. aeruginosa is associated with deteriorating nutrition state, to the point of malnourishment. 5 Sex differences relating to the natural history of P. aeruginosa infection have been described in the literature: women suffer higher rates of colonization and younger age of conversion to the more aggressive mucoid phenotype, compared to men.…”