Abstract

Pulmonary exacerbations (PEx) in Cystic Fibrosis (CF) are associated with an increased morbidity and even mortality. We investigated whether early detection of PEx in children with CF is possible by electronic home monitoring of symptoms and lung function. During this one-year prospective multi-centre study, 49 children with CF were asked to use a home monitor three times a week. Measurements consisted of a respiratory symptom questionnaire and assessment of Forced Expiratory Volume in one second (FEV1). Linear mixed-effects and multiple logistic regression analyses were used. In the 2 weeks before a PEx, the Respiratory Symptom Score (RSS) of the home monitor increased (p = 0.051). The FEV1 as percentage of predicted (FEV1%pred) did not deteriorate in the 4 weeks before a PEx. Nevertheless, the FEV1%pred at the start of exacerbation was significantly lower than the FEV1%pred in the non-exacerbation group (mean difference 16.3%, p = 0.012). The combination of FEV1%pred and RSS had a sensitivity to predict an exacerbation of 92.9% (CI 75.0–98.8%) and a specificity of 88.9% (CI 50.7–99.4%). The combination of home monitor FEV1%pred and RSS can be helpful to predict a PEx in children with CF at an early stage.

Highlights

  • Pulmonary exacerbations (PEx) in Cystic Fibrosis (CF) are associated with an increased morbidity and even mortality

  • Nine children were excluded from the analysis as no four weeks of home monitor data before a PEx were available in these children

  • This study in children with CF showed an increase in the Respiratory Symptom Score (RSS) from the home monitor, especially in the 2 weeks before exacerbation

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Summary

Introduction

Pulmonary exacerbations (PEx) in Cystic Fibrosis (CF) are associated with an increased morbidity and even mortality. We investigated whether early detection of PEx in children with CF is possible by electronic home monitoring of symptoms and lung function. The combination of home monitor FEV1%pred and RSS can be helpful to predict a PEx in children with CF at an early stage. Treatment of a PEx starts when a patient presents with an increase in (respiratory) complaints, a deterioration in lung function and/or weight loss. It often takes some time before a patient with more complaints seeks medical care. The aim of the present study was to test the hypothesis that electronic home monitoring of respiratory symptoms and lung function is a valuable tool to detect PEx in children with CF at an early stage

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