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Dr. Patrick Flume encourages clinicians to think a little differently when presented with a patient who has a chronic cough without an identifiable cause.
Patrick Flume, MD, is the director of the Adult Cystic Fibrosis Center at the Medical University of South Carolina (MUSC), and has been treating patients and studying cystic fibrosis (CF) for almost 30 years. In an interview with HCPLive, Flume spoke about the current clinical and research landscape of bronchiectasis, a rare disease that can often present in patients with CF.
As with many rare diseases, awareness is a key component to progressing research, the development of therapies, and even the ability to properly diagnose the condition. Years ago, when Flume discovered that patients had bronchiectasis, he said oftentimes they've been living with their symptoms for years before finally receiving a diagnosis.
"What we try to teach clinicians is not everybody who has a cough needs a CT scan, but in those patients where the cough just seems to be persistent and you don't have another reason like asthma, or they have certain types of infections that they get—certain bacteria which are really not typical for simple bronchitis or pneumonia—then thinking about it a little bit differently and getting a high resolution CT scan. And then, if they have bronchiectasis, then making the appropriate, either diagnostic tests, therapeutic actions, or even referral."
Bronchiectasis causes a dialation of the airways accompanied with inflammation and scar tissue. In addition to cystic fibrosis, the rare condition is also associated with a multitude of immune deficiencies, autoimmune diseases, genetic issues, infection, injuries, and chronic aspiration.
"What's really difficult, I think, for a lot of clinicians is this is a tough diagnosis to explain to people," Flume explained. "And then some of the therapies that we we treat them with really is better served if they have proper education."
Basic therapies like airway clearance therapies such as nebulizers can be effective in managing the problematic symptoms patients deal with. Part of the treatment process, according to Flume, is being able to properly teach patients how to effectively use these therapies and better understand their condition.
"Think of it like hygiene for the airways," he said, "to help clear this mucus out of the lungs."
Centers that are dedicated to that kind of diagnosis, like a cystic fibrosis center, can be a great resource for clinicians to be able to refer to. The Adult Cystic Fibrosis Center at MUSC provides access to specialists like dieticians, respiratory therapists, a genetic counselors, social workers, and pharmacists for a comprehensive approach to each patient's needs.
Flume shared that his interest in the pathophysiology of airway infections is what led him to working in a cystic fibrosis center before the adult program was initiated. Most of the patients coming to the center were experiencing chronic airway infections.
"I started getting referrals of patients who had bronchiectasis, and decided we really needed to create some greater structure, and try to organize our thinking and how we approach the care of these patients, as well as developing trials and in the like," he said.