Early detection of pulmonary exacerbations in children with Cystic Fibrosis by electronic home monitoring of symptoms and lung function

Marieke van Horck, Bjorn Winkens, Geertjan Wesseling, Dillys van Vliet, Kim van de Kant, Sanne Vaassen, Karin de Winter-de Groot, Ilja de Vreede, Quirijn Jöbsis, Edward Dompeling, Marieke van Horck, Bjorn Winkens, Geertjan Wesseling, Dillys van Vliet, Kim van de Kant, Sanne Vaassen, Karin de Winter-de Groot, Ilja de Vreede, Quirijn Jöbsis, Edward Dompeling

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.

Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Figure 1
Figure 1
Flowchart of included study population.
Figure 2
Figure 2
Course of the estimated mean (A) FEV1%pred or (B) Respiratory Symptom Score 4 weeks before and after a pulmonary exacerbation for the E group (n = 28; filled squares) and NE group (n = 9; open circles). Bars represent corresponding SEs.
Figure 3
Figure 3
(A) ROC curve of predictive model of pulmonary exacerbations and (B) combination threshold of mean FEV1%pred and mean RSS to predict a PEx. Filled squares correspond to children in the exacerbation group, open circles correspond to children in the no exacerbation group.

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