By: Suzahn Ebert
For most people, the simple act of taking a breath is often taken for granted. But for the approximately 30,000 people living with Cystic Fibrosis in the United States, every breath requires effort, not only from the individual, but also from doctors, caregivers, family members, and even researchers here at UNC-Chapel Hill.
Cystic fibrosis (CF) is a genetic disease that causes, among other complications, a buildup of mucus in the lungs. People with CF typically have frequent, persistent lung infections and limited breathing capacity. Because it is a genetic disease, there is no cure for CF; however, there are therapies and medications that target symptoms of the disease to vastly improve patients’ quality of life.
The frequent lung infections that characterize CF are often the result of reduced airway surface liquid volume, or, to put it more simply, airway dehydration. The Cystic Fibrosis and Pulmonary Diseases Research and Treatment Center at the UNC Marsico Lung Institute is the largest center for CF research in the United States. With a team of more than 55 MDs and PhDs, the center is currently working to investigate the mechanism of airway dehydration, a common occurrence in individuals with CF. And if history tells us anything, the future is promising. Now a standard treatment for individuals with CF, the practice of inhaling salty water to reduce infection and rehydrate the lungs originated here at UNC.
Dr. Robert Tarran is one of those 55 researchers, and he has made numerous contributions to the field of CF research and treatment. Much of Dr. Tarran’s research focuses on perciliary liquid (PCL), a thin liquid layer that coats mammalian airways.
“When I started this research, we didn’t know how PCL was regulated, or even what was in it,” said Dr. Tarran. Since then, Dr. Tarran has not only discovered how PCL is regulated and its components, but he has also demonstrated how PCL plays a factor in CF.
In healthy human lungs, the cystic fibrosis transmembrane conductance regulator regulates the amount of ions that flow in and out cells, which helps to maintain normal levels of perciliary liquid (PCL), a thin liquid layer that coats mammalian airways. The PCL is a crucial component of mucus transport, which contributes to the lungs’ natural defense against bacteria. In individuals with CF, the CFTR does not function properly, resulting in inadequate amounts of PCL, and thus dehydration and heightened susceptibility to infection (Figure 2).
In 2013, Dr. Tarran discovered a novel mechanism employed by lung cells to regulate PCL levels that can be pharmaceutically modulated. As a result of his discovery, he founded Spyryx Biosciences, a pharmaceutical company that focuses on developing new treatments for lung diseases. Recently, Dr. Tarran and Spyryx developed a novel peptide based drug that has the potential to be used as an inhalable treatment for CF. The therapy has shown itself to be effective throughout preclinical development, and the next steps for Dr. Tarran include meetings with the FDA and clinical trials. Hopefully, the UNC Cystic Fibrosis Center is once again on the verge of producing what will become a standard treatment for CF thanks to Dr. Tarran’s discoveries.
When he is not researching CF, one of Dr. Tarran’s main areas of interest is tobacco. As you can probably guess, he is not interested in smoking it, but instead prefers to investigate how it affects the lungs of those who do.
As part of the UNC Center for Tobacco Regulatory Science and Lung Health, Dr. Tarran is part of a 50 person team that received a $20 million grant to study the effects of new and emerging tobacco products, including but not limited to cigarillos, hookah, and e-cigarettes. Specifically, Dr. Tarran is interested in the role that new tobacco products play in lung dehydration, which causes disease in more than 12 million Americans. Dr. Tarran feels that this research is of particular importance to public health as the FDA currently has no power to regulate cigarillos; if Dr. Tarran and the rest of the team can provide sufficient evidence to demonstrate the dangers of these new tobacco products, it may be possible for the FDA to place restrictions on their production.
Dr. Tarran believes the best way to get funding is by being diverse, and he certainly sets an example. In addition to CF and tobacco research, he is also part of a project investigating into how epithelia communicate with smooth muscle in individuals with asthma.
“The most interesting thing changes every few years,” said Dr. Tarran. If UNC is lucky, Dr. Tarran’s interests will continue to change, and he will continue to make it a little easier to breathe.