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Published on: February 9, 2022
Bronchodilator responsiveness in cystic fibrosis children treated for pulmonary exacerbations
Mordechai Pollak1, Michelle Shaw2,3, David Wilson1
1Division of Respiratory Medicine, Department of Paediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Bronchodilator response testing during cystic fibrosis pulmonary exacerbations is rarely significant and does not predict lung function recovery. Routine testing for bronchodilator response is not indicated during these events.
Area of Science:
- Pulmonology
- Pediatric Medicine
- Respiratory Medicine
Background:
- Cystic fibrosis (CF) pulmonary exacerbations (PEx) significantly impair lung function.
- The utility of bronchodilator (BD) responsiveness testing during PEx treatment to monitor recovery is uncertain.
Purpose of the Study:
- To evaluate the clinical value of measuring bronchodilator responsiveness during PEx treatment in pediatric CF patients.
- To determine if BD response during PEx correlates with baseline lung function or predicts recovery.
Main Methods:
- Retrospective analysis of spirometry with BD testing during hospital admissions for PEx in pediatric CF patients.
- Included repeated events for patients with multiple admissions.
Main Results:
- 249 spirometries with BD testing in 102 patients were analyzed around Day 7 of admission.
- BD response around Day 7 showed poor correlation with pre-PEx FEV1 and no correlation with end-of-treatment FEV1 recovery.
- Only 9% of tests showed a significant BD response (≥12% and 200 ml increase in FEV1).
Conclusions:
- Significant bronchodilator response is uncommon in CF patients during PEx treatment.
- BD response during PEx does not correlate with baseline pulmonary function or predict FEV1 recovery.
- Routine BD response testing is not indicated during CF pulmonary exacerbations.
Background:
Cystic fibrosis (CF) pulmonary exacerbations (PEx) are associated with a significant drop in pulmonary function. The clinical value of measuring bronchodilator (BD) responsiveness during treatment for PEx to monitor or predict recovery of lung function is unclear.
Methods:
A retrospective analysis of spirometry with BD response testing obtained during hospital admissions for PEx in pediatric CF patients. Repeated events were included for patients with BD testing during multiple admissions.
Results:
Two hundred forty-nine spirometries with BD testing in 102 patients were completed around Day 7 (Days 4-10) of hospital admission for treatment of CF PEx. Median (IQR) forced expiratory volume in 1 s (FEV1 ) was 70.6% predicted (58.1, 84.6) before the PEx event (best FEV1 in 6 months before admission), 54.4% (41.5, 66.9) at admission, 62.3% (48.4, 74.7) around Day 7 of admission and 67.1% predicted (53.8, 78.2) at the end of treatment. BD response around Day 7 correlated poorly with FEV1 before PEx (r = -.16, p = .02) and did not correlate with recovery to baseline FEV1 at end of treatment (r = .08, p = .22). Only 23/249 (9%) individual tests had a BD response in FEV1 of ≥12% and 200 ml. BD response was not related to age or severity of lung disease and led to an immediate change in clinical management in only four cases.
Conclusions:
Significant BD response in CF patients treated for PEx is rare, shows poor correlation with baseline pulmonary function and does not correlate with the recovery of FEV1 with treatment. These data suggest that routine testing for BD response is not indicated during PEx.
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