Early Intervention in Pulmonary Exacerbation in CF

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DESCRIPTION (provided by applicant): Individuals with cystic fibrosis (CF) are plagued by daily respiratory symptoms such as cough and dyspnea. In spite of dramatic improvements in care, their lives are greatly shortened due to progressive respiratory failure. The course of CF is marked by recurrent pulmonary exacerbations characterized by worsened symptoms and declines in pulmonary function. CF acute pulmonary exacerbations result in more rapid decline in lung function and increased. Preliminary data from our groups suggest that delays in seeking care are associated with lack of recovery of lung function after an exacerbation. The primary goal of this proposal is to perform a clinical trial to determine the efficacy of early intervention in the treatment of adolescent and adult CF acute pulmonary exacerbation. The study will assess the integration of two novel approaches to the treatment of CF pulmonary exacerbation: 1) the use of home spirometers to identify and trigger treatment of an exacerbation; and 2) the use of home based assessment of patient symptoms to identify and trigger the treatment of a pulmonary exacerbation, both integrated into one protocolized care plan. This novel approach will be compared to usual care with the primary outcome measure being lung function as measured with forced expiratory volume in the first second (FEV1). We hypothesize that if pulmonary exacerbations are treated earlier with a multi-faceted intervention, the progression of lung disease will be slowed. This approach will utilize state of the art, telehealth equipment to improve the assessment and delivery of care to adolescents and adults with CF. PUBLIC HEALTH RELEVANCE: The primary goal of this proposal is to perform a clinical trial to determine the efficacy of early intervention in the treatment of adolescent and adult CF acute pulmonary exacerbation using remote monitoring with home spirometry and respiratory symptom assessment and protocolized management of CF exacerbation. This approach will utilize state of the art, telehealth equipment to improve the assessment and delivery of care to adolescents and adults with CF.