Overview

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.

Eligibility

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

Exclusion Criteria:

• Shared bedroom with sibling
• Underlying chronic respiratory or cardiac conditions including chronic cough, CF, asthma, obstructive sleep apnea, or congenital heart disease or other condition felt by the investigator to cause chronic nighttime symptoms
• Shared custody (i.e., the participant is splitting time between time households)