Progression of Lung Disease in Preschool Patients with Cystic Fibrosis
Sanja Stanojevic1,2, Stephanie D Davis3, George Retsch-Bogart4
11 Division of Respiratory Medicine, and.
Insights
The Lung Clearance Index (LCI) effectively tracks cystic fibrosis (CF) lung disease progression in young children. This measure, unlike spirometry, detects worsening lung function over time, aiding early intervention.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Medical Diagnostics
Background:
- Early intervention is crucial for preventing lung damage in cystic fibrosis (CF).
- Objective outcome measures are needed to track lung disease progression in young children with CF.
- The Lung Clearance Index (LCI) is a potential measure for assessing early lung disease.
Purpose of the Study:
- To evaluate the Lung Clearance Index (LCI) as a tool for tracking disease progression in preschool children with CF.
- To compare the utility of LCI with spirometry in identifying early lung disease in CF.
Main Methods:
- A longitudinal study involving 156 preschool children (2.5-6 years) with CF and age-matched healthy controls.
- Multiple breath washout (MBW) tests were performed at multiple time points (baseline, 1, 3, 6, 12 months) to measure LCI.
- Generalized linear mixed-effects models were used to analyze LCI changes and differentiate CF progression from normal development.
Main Results:
- Both LCI and spirometry distinguished between healthy children and those with CF.
- Only LCI demonstrated significant deterioration in lung function over time in children with CF.
- Increased LCI values correlated with cough episodes and pulmonary exacerbations in CF patients, but not in healthy controls.
Conclusions:
- The Lung Clearance Index (LCI) is a valuable marker for monitoring early lung disease progression in young children with CF.
- LCI may serve as a sensitive tool to guide therapeutic strategies in pediatric CF patients.
- LCI's ability to detect subtle lung function changes offers advantages over traditional spirometry in this age group.
Rationale:
Implementation of intervention strategies to prevent lung damage in early cystic fibrosis (CF) requires objective outcome measures that capture and track lung disease.
Objectives:
To define the utility of the Lung Clearance Index (LCI), measured by multiple breath washout, as a means to track disease progression in preschool children with CF.
Methods:
Children with CF between the ages of 2.5 and 6 years with a confirmed diagnosis of CF and age-matched healthy control subjects were enrolled at three North American CF centers. Multiple breath washout tests were performed at baseline, 1, 3, 6, and 12 months to mimic time points chosen in clinical care and interventional trials; spirometry was also conducted. A generalized linear mixed-effects model was used to distinguish LCI changes associated with normal growth and development (i.e., healthy children) from the progression of CF lung disease.
Measurements And Main Results:
Data were collected on 156 participants with 800 LCI measurements. Although both LCI and spirometry discriminated health from disease, only the LCI identified significant deterioration of lung function in CF over time. The LCI worsened during cough episodes and pulmonary exacerbations, whereas similar symptoms in healthy children were not associated with increased LCI values.
Conclusions:
LCI is a useful marker to track early disease progression and may serve as a tool to guide therapies in young patients with CF.
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