By the time many patients find their way to Johns Hopkins pulmonologist Keira Cohen, they have seen doctor after doctor to treat their lung ailment, but they only seem to get sicker.
“Many of our patients have been misdiagnosed and mismanaged,” she says. “They have bounced from specialist to specialist and have almost gotten whiplash from the different messages and treatments they have received.”
The outlook for these patients has improved dramatically with the launch of a multidisciplinary center at Johns Hopkins – co-created by Cohen, fellow pulmonologist Mark Jennings and infectious diseases specialist Jonathan Zenilman, Lavinia Currier CIM Scholar – that is specifically aimed at managing and studying two lung conditions that doctors are seeing with more frequency: bronchiectasis and nontuberculous mycobacterial infections.
Nontuberculous mycobacteria (NTM) are a category of organisms occurring in the environment, such as in water and soil. In most people, these bacteria do not cause health problems, but in some cases, these bacteria can infect the airways and lung tissue or organs outside the lungs. It can take years and multiple antibiotics to clear the infection. While related to tuberculosis (TB), these infections are not thought to pass from person to person.
“We hear a lot about TB, but we have more than 10 times as many cases of NTM disease than TB cases in the United States these days,” says Cohen.
“We hear a lot about TB, but we have more than 10 times as many cases of NTM disease than TB cases in the United States these days” – Keira Cohen
Bronchiectasis, a scarring of the airways, can develop from multiple causes. If not managed well, bronchiectasis can lead to worsening lung function, repeated lung infections and increased hospitalizations. While patients with bronchiectasis are at risk for all sorts of infections, one that occurs frequently is NTM, which is why the new center focuses on both conditions.
Crucially, says Cohen, “when patients come to our multidisciplinary center, it’s a one-stop affair.” At the Friday morning clinic in Johns Hopkins Bayview Medical Center’s Asthma and Allergy Center, they’ll see a pulmonologist (Cohen or Jennings), an infectious disease specialist (Zenilman or Chris Lippincott) and a physical therapist knowledgeable about airway clearance techniques. The center’s nurse practitioner, Meghan Ramsay, sees patients in clinic, acts as a bridge between these different specialists and communicates closely with patients between visits. The center also works with a chest radiologist and has relationships with specialists in rheumatology, allergy and immunology, gastroenterology, and otolaryngology. The in-house Clinical Mycobacteriology Laboratory at Johns Hopkins, directed by Nikki Parrish, allows physicians to quickly and accurately assess test results.
“As a multidisciplinary team, we meet to come up with a unified treatment plan that is individualized according to the needs of each patient,” says Cohen, who notes that the center has been designated a Johns Hopkins Precision Medicine Center of Excellence.
“Even though we’re seeing more patients with these lung conditions, there’s so little known about these diseases, so we are striving to develop new and better diagnostic strategies and treatments. It’s truly bench to bedside.” – Keira Cohen
Many patients coming to the center have sustained lung damage over the years, making it difficult for them to naturally clear their lungs of mucus. So an important part of their treatment plan is to learn to use methods – such as devices that can be held by hand or strapped on to vibrate the chest, in conjunction with inhaled saltwater – to keep airways clear.
“I reassure our patients that it usually takes several months before they’ll feel comfortable using these new techniques. But once they do, it can make a dramatic improvement in their quality of life,”
Research is a crucial element of the multidisciplinary center. “I’m very proud of the research program we have built in parallel with our clinical program,” says Cohen. “Even though we’re seeing more patients with these lung conditions, there’s so little known about these diseases, so we are striving to develop new and better diagnostic strategies and treatments. It’s truly bench to bedside.”
Patients who come to the clinic have the option to donate biospecimens (blood and mucus) to the team’s biobank and to have data from their medical chart added to an ever-growing database.
“We can look at patient outcomes in a truly rigorous way,” says Cohen. One goal, she says: “to see if we can determine blood-based predictors of who might respond better to a particular medication or treatment plan.”
“Our goal is to develop a standard benchmark of antibiotic efficacy to serve as a guide the development of new drugs for NTM, which are desperately needed.” – Keira Cohen
In her laboratory, Cohen investigates drug resistance mechanisms in NTM and blood-based biomarkers of NTM lung disease. One of the ultimate goals of her research is to improve diagnostic strategies for NTM. These research efforts dovetail nicely with those of others at Johns Hopkins with an interest in NTM. In particular, Gyanu Lamichhane, a basic science researcher in infectious diseases, has recently developed a novel mouse model to study NTM lung disease.
Moreover, patients treated at the center often have the option of joining one or more clinical trial for either NTM or bronchiectasis. “In addition to our translational research program, I’m very proud of one of our pilot clinical trials that will look at early bactericidal activity of azithromycin for Mycobacterium avium lung disease. Our goal is to develop a standard benchmark of antibiotic efficacy to serve as a guide the development of new drugs for NTM, which are desperately needed,” Cohen says.
Since the clinic opened in July 2018, the Johns Hopkins team has seen about 275 unique patients, “and our numbers are growing quickly, with people coming from all over the country,” says Cohen. Lippincott recently joined the clinic, which will make it possible to expand the number of patients who can be evaluated, she says.
Excited about what the future holds, Cohen says that the center never could have opened its doors in summer 2018 without crucial startup funding from David Hellmann and the Center for Innovative Medicine. “Dr. Hellmann’s support and enthusiasm for our program were instrumental to our opening and to our success today,” she says. “We couldn’t be more grateful.”