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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Children with Cystic Fibrosis | Childrens ages 1-18 with a diagnosis of CF based on 2 known cystic fibrosis transmembrane conductance regulator (CFTR) mutations and/or sweat chloride >60 mmol/L, thought to be clinically stable at the time of study consent. |
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| Healthy Controls | Children ages 1-18 with no underlying respiratory of cardiac conditions including chronic cough, CF, asthma, obstructive sleep apnea, or congenital heart disease thought to cause chronic nighttime symptoms. |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Curie Artificial Intelligence (AI) cough monitor | Device | All patients are given nighttime cough monitors for home use for 3-4 months |
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| Measure | Description | Time Frame |
|---|---|---|
| Feasibility of using an in-home cough monitoring device | Percent of nights over the study period during which 4 or more hours of analyzable data are collected over study period | Through study completion, an average of 3 months |
| Measure | Description | Time Frame |
|---|---|---|
| Comparison of nighttime cough between participants with CF and healthy controls | Average cough seconds per hour per night over the study period | Through study completion, an average of 3 months |
| Comparison of nighttime cough in children with CF during clinician diagnosed pulmonary exacerbations |
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Inclusion Criteria for Children with Cystic Fibrosis
Exclusion Criteria
Inclusion Criteria for Healthy Controls
Exclusion Criteria:
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See inclusion and exclusion criteria for both groups above. Our aim to recruit 20 participants in each cohort.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Lilah Melzer, DO | Contact | 7207772934 | lilah.melzer@childrenscolorado.org |
| Name | Affiliation | Role |
|---|---|---|
| Lilah Melzer, DO | Children's Hospital Colorado, University of Colorado | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Children's Hospital of Colorado | Recruiting | Aurora | Colorado | 80045 | United States |
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Assess for changes in cough frequency before and after diagnosis of pulmonary exacerbation |
| 7 days |