The goal of this research study is to better understand current treatment practices for pulmonary exacerbations (lung infections) and whether the Cystic Fibrosis National Patient Registry (CFFNPR)can be used for this type of study.
Cystic fibrosis (CF), a life-shortening genetic disease, is marked by acute episodes during which symptoms of lung infection increase and lung function decreases. These pulmonary exacerbations (PEs) are treated with varying antibiotics for varying time periods based on needs determined by individual patients, their families, and the health care providers. Cystic fibrosis pulmonary guidelines for the treatment of PE published by the Cystic Fibrosis Foundation (CFF) in 2009 provided recommendations for treatment and also identified key questions for which additional studies were needed. Standard treatment for PE involves many facets including selection of antibiotics, duration of use, and outcomes that define treatment success. Understanding current treatment practices and measures of treatment success are needed before a study can be designed to define optimal treatment strategies. This is a multi-center, prospective, observational study designed to prospectively follow patients with CF that are initially admitted to the hospital for treatment of a pulmonary exacerbation.
Study Type
OBSERVATIONAL
Enrollment
220
CFF Adult Program University of Alabama
Birmingham, Alabama, United States
CFF Care Center Arizona Health Science Center
Tucson, Arizona, United States
National Jewish Health
Feasibility of using the CFF National Patient Registry
Assess feasibility using the CFF National Patient Registry as measured by the accuracy of data entry
Time frame: 28 days from time of start of IV antibiotic therapy.
Physician and patient level clinical outcomes for their use in comparative studies of CF pulmonary exacerbations to determine the optimal treatment endpoints
Evaluate physician assessment of treatment response as measured by the physician treatment assessment questionnaire. Evaluate change in lung function and patient reported respiratory symptoms and quality of life in response to treatment of an acute pulmonary exacerbation.
Time frame: During hospitalization and during a period following discharge of 28 days from time of start of IV antibiotic therapy.
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Denver, Colorado, United States
Johns Hopkins
Baltimore, Maryland, United States
CFF Care Center & Pediatric Program Rainbow Babies and Children's Hospita
Cleveland, Ohio, United States
Rainbow Babies and Children's Hospital & University Hospitals Case Medical Center
Cleveland, Ohio, United States
CFF Care Center & Pediatric Program Children's Hospital of Pittsburgh
Pittsburgh, Pennsylvania, United States
Medical University of South Carolina
Charleston, South Carolina, United States
CFF Care Center & Pediatric Program The University of Texas Southwestern Medical Center at Dallas
Dallas, Texas, United States
CFF Care Center & Pediatric Program Seattle Children's Hospital
Seattle, Washington, United States
...and 2 more locations