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.

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Research Project (R01)
Project #
5R01HL103965-04
Application #
8534241
Study Section
Respiratory Integrative Biology and Translational Research Study Section (RIBT)
Program Officer
Banks-Schlegel, Susan P
Project Start
2010-09-27
Project End
2015-06-30
Budget Start
2013-07-01
Budget End
2014-06-30
Support Year
4
Fiscal Year
2013
Total Cost
$706,945
Indirect Cost
$76,665
Name
University of Washington
Department
Internal Medicine/Medicine
Type
Schools of Medicine
DUNS #
605799469
City
Seattle
State
WA
Country
United States
Zip Code
98195
Goss, Christopher H; VanDevanter, Donald R (2016) CFTR modulators and pregnancy: Our work has only just begun. J Cyst Fibros 15:6-7
Heltshe, Sonya L; Goss, Christopher H (2016) Optimising treatment of CF pulmonary exacerbation: a tough nut to crack. Thorax 71:101-2
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
Ramos, Kathleen J; Quon, Bradley S; Psoter, Kevin J et al. (2016) Predictors of non-referral of patients with cystic fibrosisfor lung transplant evaluation in the United States. J Cyst Fibros 15:196-203
Sack, Cora S; Goss, Christopher H (2016) Nature versus Nurture: Does Genetic Ancestry Alter the Effect of Air Pollution in Children with Asthma? Am J Respir Crit Care Med 193:1196-8
Ramos, Kathleen J; Goss, Christopher H (2015) Remarkable long-term survival post-lung transplantation among Canadian patients with cystic fibrosis. J Heart Lung Transplant 34:1131-3
Goss, Louisa B; Ortiz, Justin R; Okamura, Daryl M et al. (2015) Significant Reductions in Mortality in Hospitalized Patients with Systemic Lupus Erythematosus in Washington State from 2003 to 2011. PLoS One 10:e0128920
Sack, Coralynn; Goss, Christopher H (2015) It Starts at the Beginning: Effect of Particulate Matter In Utero. Am J Respir Crit Care Med 192:1025-6
Sawicki, Gregory S; Goss, Christopher H (2015) Tackling the increasing complexity of CF care. Pediatr Pulmonol 50 Suppl 40:S74-9
Goss, Christopher H; MacNeill, Stephanie J; Quinton, Hebe B et al. (2015) Children and young adults with CF in the USA have better lung function compared with the UK. Thorax 70:229-36

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