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Home-spirometry exacerbation profiles in children with cystic fibrosis
Benoit Bouteleux1, Fabien Beaufils2,3,4, Michael Fayon2,3,4
1Cabinet Resp'Air, Talence, France.
Insights
Home spirometry aids early detection of pulmonary exacerbations (PEx) in children with cystic fibrosis (CF). A lung function threshold can identify patients with a poor response to intravenous antibiotics, guiding earlier interventions.
Area of Science:
- Pediatric Pulmonology
- Cystic Fibrosis Research
- Respiratory Medicine
Background:
- Pulmonary exacerbations (PEx) significantly impact cystic fibrosis (CF) progression in children.
- Persistent lung function decline may follow acute PEx management.
- The role of telemonitoring in PEx detection and management remains debated.
Purpose of the Study:
- To identify symptoms and telemonitoring spirometry characteristics linked to outcomes after PEx management.
- To establish predictors of treatment response in children with CF experiencing PEx.
Main Methods:
- Retrospective analysis of severe PEx in children with CF (CwCF) treated with intravenous antibiotics (IV ATB).
- Utilized home telemonitoring data, including symptoms and spirometry (FEV1), collected pre- and post-treatment.
- Defined patient response profiles based on FEV1 recovery after IV ATB.
Main Results:
- A significant FEV1 decline preceded IV ATB initiation by 8 days.
- 41% of IV ATB courses did not restore baseline FEV1.
- Greater FEV1 drop and delayed treatment correlated with poor response; <94% FEV1 recovery by day 14 indicated a non-responder profile.
Conclusions:
- Home spirometry can enable earlier PEx recognition and intervention in CwCF.
- A specific FEV1 recovery threshold identifies patients with suboptimal response to IV ATB.
- Findings support integrating telemonitoring for proactive PEx management in CF.
Background:
Pulmonary exacerbations (PEx) are strong predictors of respiratory disease progression in children with cystic fibrosis (CwCF) and may be associated with persistent decreased lung function after acute management. Telemonitoring devices can be used for early detection and monitoring of PEx, but its utility is debated.
Research Question:
Which symptoms and telemonitoring spirometry characterics are related to outcome dynamics following initial PEx management?
Methods:
This retrospective study included CwCF followed at Bordeaux University Hospital, France. All severe PEx episodes treated with intravenous (IV) antibiotics (ATB) between 1 January 2017 and 31 December 2021 in CwCF using home telemonitoring were analyzed. Symptoms and home spirometry data were collected 45 days before and up to 60 days after each IV ATB course. We defined three response profiles based on terciles of baseline forced expiratory volume in 1 s (FEV1 ) recovery.
Results:
A total of 346 IV ATB courses for PEx were administered to 65 CwCF during the study period. The drop in FEV1 became significant 8 days before IV ATB initiation. Forty-one percent of IV ATB courses failed to restore baseline FEV1 . The magnitude of FEV1 drop and a greater delay in the initiation of treatment correlated with a low response level. On the 14th day of the IV treatment, a FEV1 recovery less than 94% of baseline was associated with a nonresponder profile.
Interpretation:
Home spirometry may facilitate the early recognition of PEx to implement earlier interventions. This study also provides an outcome lung function threshold which identifies low responders to IV ATB.
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