Abstract

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.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Research Project (R01)
Project #
5R01HL103965-05
Application #
8690951
Study Section
Respiratory Integrative Biology and Translational Research Study Section (RIBT)
Program Officer
Smith, Robert A
Project Start
2010-09-27
Project End
2015-06-30
Budget Start
2014-07-01
Budget End
2015-06-30
Support Year
5
Fiscal Year
2014
Total Cost
Indirect Cost

  Institution

Name
University of Washington
Department
Internal Medicine/Medicine
Type
Schools of Medicine
DUNS #
City
Seattle
State
WA
Country
United States
Zip Code
98195

  Publications

Ramos, Kathleen J; Somayaji, Ranjani; Nichols, David P et al. (2018) Comparative Effectiveness Research in Pediatric Respiratory Disease: Promise and Pitfalls. Paediatr Drugs 20:1-7
Lechtzin, Noah; Mayer-Hamblett, Nicole; West, Natalie E et al. (2017) Home Monitoring of Patients with Cystic Fibrosis to Identify and Treat Acute Pulmonary Exacerbations. eICE Study Results. Am J Respir Crit Care Med 196:1144-1151
Sack, Cora S; Goss, Christopher H (2017) Yet Again, Air Pollution Impacts Lung Health. Ann Am Thorac Soc 14:1761-1762
Stephenson, Anne L; Sykes, Jenna; Stanojevic, Sanja et al. (2017) Survival Comparison of Patients With Cystic Fibrosis in Canada and the United States: A Population-Based Cohort Study. Ann Intern Med 166:537-546
Muhlebach, Marianne Sponer; Beckett, Valeria; Popowitch, Elena et al. (2017) Microbiological efficacy of early MRSA treatment in cystic fibrosis in a randomised controlled trial. Thorax 72:318-326
Heltshe, Sonya L; Cogen, Jonathan; Ramos, Kathleen J et al. (2017) Cystic Fibrosis: The Dawn of a New Therapeutic Era. Am J Respir Crit Care Med 195:979-984
Ramos, Kathleen J; Quon, Bradley S; Heltshe, Sonya L et al. (2017) Heterogeneity in Survival in Adult Patients With Cystic Fibrosis With FEV1 < 30% of Predicted in the United States. Chest 151:1320-1328
Ramos, Kathleen J; Sack, Coralynn S; Mitchell, Kristina H et al. (2017) Cystic Fibrosis is Associated with Adverse Neonatal Outcomes in Washington State, 1996-2013. J Pediatr 180:206-211.e1
Ramos, Kathleen J; Somayaji, Ranjani; Lease, Erika D et al. (2017) Cystic fibrosis physicians' perspectives on the timing of referral for lung transplant evaluation: a survey of physicians in the United States. BMC Pulm Med 17:21
Crull, Mathew R; Ramos, Kathleen J; Caldwell, Ellen et al. (2016) Change in Pseudomonas aeruginosa prevalence in cystic fibrosis adults over time. BMC Pulm Med 16:176

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