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

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.

Discrepancy between Lung Function Measurements at Home and in the Hospital in Children with Asthma and CF

“…We performed a re-analysis of prospectively collected data of children with asthma or CF who were included in two previous one-year observational cohort studies [ 12 , 13 ]. In the ‘Reduction of Asthma exacerbation rate in Children by Non-invasive Monitoring of Inflammatory Markers in Exhaled Breath (Condensate)’ study (RASTER) (clinicaltrials.gov, NCT 01239238), the aim was to investigate the predictive properties of markers in exhaled breath to predict an asthma exacerbation.…”

“…However, these studies compared a home spirometer with a hospital spirometer (mostly pneumotachograph) in the clinical setting, there are no studies directly comparing lung function measurements at home to lung function measurements in the hospital. Other studies have evaluated home spirometry in combination with electronic symptom assessment as a tool to assess pulmonary exacerbations in children with CF, and to measure asthma control in children with asthma [ 12 , 13 , 14 ]. The accuracy of lung function measurements at home in these studies was unclear.…”

Recent advances in the understanding and management of cystic fibrosis pulmonary exacerbations

“…A multi-center study from the Netherlands examining electronic home monitoring of symptoms and lung function for early PEx detection confirmed a change in symptoms at least 4 weeks prior to PEx diagnosis in most patients, and further symptom deterioration in the 2 weeks prior to PEx diagnosis 6 . However, a large multi-center randomized trial recently conducted in the US demonstrated that although electronic home monitoring of symptoms and spirometry is feasible and leads to earlier and more frequent diagnoses of PEx compared with usual care, this strategy does not lead to less lung function lost over 1 year 7 .…”

Using digital technology for home monitoring, adherence and self-management in cystic fibrosis: a state-of-the-art review

“…In a condition where treatment burden is already high, home monitoring adds an additional task for the patient to complete. Home monitoring studies have required data entry from once a month to three times a week 28 29. Adherence to telemonitoring in the eICE trial over 52 weeks was suboptimal; 50% of patients transmitted data once per week and only 19% twice per week as per the protocol, with an increased treatment burden score in the early intervention telemonitoring group 26…”