hich we established more than 40 years ago to track progress, trends and areas for improvement in the treatment of CF," said Robert J. Beall, PhD, President and CEO of the Cystic Fibrosis Foundation. "The registry offers insights and the ability to analyze treatments in a real-life setting. We are pleased the registry can offer this kind of important information to people with CF and their care providers."
The analysis included patients aged six years and older with FEV(1) predicted* of 25-75% and four or more Pa cultures, who were identified from the Cystic Fibrosis Foundation's Patient Registry between 1996 and 2008. Patients were followed from the first year after 1998 in which they met these criteria until death, or until continuous data were no longer available. Reported use of TOBI was obtained from annual questionnaires.
The results of the analysis were adjusted for demographics, comorbidities, reported use of other medications (i.e. dornase alfa, high-dose ibuprofen and pancreatic enzyme), FEV(1) % predicted, and use of medical resources.
CF is a life-threatening genetic disease that affects the internal organs, especially the lungs and digestive system, by clogging them with thick mucus making it hard to breathe and digest food. A total of 70,000 patients have been diagnosed with CF worldwide, of whom an estimated 30,000 are in the US.
Over the past few decades, significant advances have been made in the treatment and management of CF. In the 1950s, few children with CF lived to attend elementary school. By 2008, the median lifespan for people with this disease in the US was 37.4 years.
Pa is the most common bacterium contributing to CF mortality, with up to 80% of adults between the ages of 25 and 34 chronically infected with Pa in their lungs. On average, patients with CF and Pa experience a 2% annual decline in lung function.
TOBI was first launched in 1997 and is now approved in 46
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