Cystic Fibrosis is a complicated, multi-organ disease with a plethora of functional defects, although most of the morbidity and mortality associated with CF results from the progressive loss of lung function. CF lung disease is due to chronic inflammation, persistent infection, impaired mucociliary clearance, and susceptibility to damage from oxidative stress.
Although substantial improvements in quality and longevity of life for CF patients have been achieved over the seven decades since the disease was first described, the median predicted age at death remains less than half of that for the non-CF population; children and young adults are still dying from this disease. What will it take to solve this problem? We need to find new ways to think about CF, new ways to focus on wellness, to turn this “disease” into a “condition”.
This is the mission of the Center for Cystic Fibrosis and Airways Disease Research (CF-AIR) at Children’s Healthcare of Atlanta, established in collaboration with the Emory University School of Medicine: to build a comprehensive program that will lead to new breakthroughs in CF research that will advance the quality and longevity of life for our patients. CF-AIR is a component of The Emory+Children’s CF Center of Excellence, also known as CF@LANTA, which seeks to become the best comprehensive CF program in the country, excellent in research, clinical care, and education.
CF patients are living longer, fuller lives, thanks to the continued development of therapies that target the symptoms associated with progressive lung disease, although this a profound burden that encompasses hours of daily treatment. Quality of life decreases dramatically as lung function declines due to irreversible damage. As patients age, other organ systems also show defects in function.
Due to the establishment of CF newborn screening nationwide in January 2009, new patients are being identified at a few days of age – an age when the lungs are healthy, without damage and thus likely to greatly benefit from a new model of Prospective Healthcare for CF.
We need to bring new advances in clinical care to the forefront soon in order to have the greatest impact on the lives of these new patients.
