Cystic Fibrosis in Children
Conditions
Brief summary
Cystic fibrosis (CF) is a disease characterized by chronic airway infection and impaired mucociliary clearance, which predisposes those affected to recurrent pulmonary exacerbations (PEx) and progressive decline in lung function. Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) results in decreases in patient-reported cough and PEx. Despite this, increased cough remains the most common symptom associated with acute PEx and worsening lung disease. Cough frequency was historically difficult to measure due to reliance on human input. Recent advances in audio capture and signal processing have made automated cough detection possible. As a result there's been a surge in development of portable cough monitors, as cough is increasingly recognized as a measurable parameter of respiratory disease. The majority of cough monitors have been designed for use in adults, and little is known about the practicality of collecting cough data in the pediatric population. In this study investigators aim to assess the feasibility of using an in-home device to capture nighttime cough frequency in children with and without CF. Investigators plan to compare nighttime cough frequency between children with and without CF and, among children with CF, and determine the association between cough frequency and baseline lung function. Additionally, investigators aim to evaluate the changes in nighttime cough frequency in relationship to respiratory symptom scores surrounding clinician diagnosed pulmonary exacerbations. This study will provide important preliminary data needed for a larger study assessing the utility of home cough monitoring for clinical care and for use of cough as a clinical outcome measure in research studies.
Interventions
DEVICECurie Artificial Intelligence (AI) cough monitor
All patients are given nighttime cough monitors for home use for 3-4 months
Sponsors
University of Colorado, Denver
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
1 Years to 18 Years
Healthy volunteers
Yes
Inclusion criteria
for Children with Cystic Fibrosis * Diagnosis of CF based on 2 known CFTR mutations and/or sweat chloride \> 60 mmol/L * Ages 1-18 years of age * Clinically stable at the time of consent
Exclusion criteria
* Use of nocturnal positive pressure or supplemental oxygen * Shared bedroom with sibling * Use of oral or IV antibiotics within the past 2 weeks except for chronic azithromycin use * Shared custody (i.e., the participant is splitting time between time households) Inclusion Criteria for Healthy Controls * Ages 1-18
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Feasibility of using an in-home cough monitoring device | Through study completion, an average of 3 months | Percent of nights over the study period during which 4 or more hours of analyzable data are collected over study period |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Comparison of nighttime cough between participants with CF and healthy controls | Through study completion, an average of 3 months | Average cough seconds per hour per night over the study period |
| Comparison of nighttime cough in children with CF during clinician diagnosed pulmonary exacerbations | 7 days | Assess for changes in cough frequency before and after diagnosis of pulmonary exacerbation |
Countries
United States
Contacts
Primary ContactLilah Melzer, DO
Outcome results
None listed