What every pulmonologist should know about cystic fibrosis exacerbations

Gretchen Winter, MD

Recent cystic fibrosis (CF) treatment advances have dramatically reduced the incidence and frequency of exacerbations, but pulmonary exacerbations still happen—and they can be life-threatening if not treated promptly and appropriately.

“There will always be cystic fibrosis pulmonary exacerbations,” said Gretchen Winter, MD, assistant professor of critical care medicine at the University of Alabama at Birmingham. “Every pulmonologist should be able to recognize cystic fibrosis pulmonary exacerbations in their patients and institute correct initial therapy.”

Dr. Winter will discuss CF exacerbation signs, symptoms, and treatment options during Cystic Fibrosis Pulmonary Exacerbations: What Should Every Pulmonologist Know? on Tuesday, October 20, from 7:30 am to 8:30 am CT. Prompt recognition and treatment of exacerbations is even more important in the current pandemic environment of overburdened hospitals and more cumbersome processes to transfer patients to an accredited CF center.

Clinicians have seen a dramatic improvement in lung function since the 2019 introduction of Trikafta (elexacaftor/ivacaftor/tezacaftor), the first triple CF transmembrane conductance regulator (CFTR) modulator, Dr. Winter said. Pulmonologists have seen a similarly dramatic decline in pulmonary exacerbations and hospital admissions for exacerbations, but exacerbations have not disappeared.

Exacerbations can be identified by worsened symptoms, she noted, including increased fatigue, shortness of breath, sinus symptoms, cough, or sputum production. A decrease from baseline lung function on FEV1 is also a sign of pulmonary problems.

Patient input can be the clearest sign of an exacerbation in process.

“Our patients know their bodies and their breathing,” Dr. Winter said. “You need to listen to them when they’re telling you that something is not right.”

Antibiotics and aggressive airway clearance remain the mainstay of treatment for CF pulmonary exacerbations, she continued. The real question is which antibiotics to use and for how long.

“Antibiotics are tailored to sputum culture results,” Dr. Winter said. “We tailor treatment to what they have grown in the recent past on sputum cultures and on what they are growing right now.”

She orders sputum culture on every clinic visit—every three months for most patients. Even if the patient is showing no signs of exacerbation, it is helpful to know what organisms are present and which agents will be more effective in case problems develop. That gives the clinician an immediate starting point for treatment if problems do develop.

“And whenever someone presents with an exacerbation, if they are able to express sputum, we do another culture to make sure we’re appropriately covering everything that is growing right now,” Dr. Winter added. “The majority of times, we find what they’re growing now is what they’ve grown in the recent past, but it is not uncommon to find something new that has popped up that we can treat.”

Expect to wait 2 to 3 days for initial culture results on common bacteria, plus another day or 2 for susceptibility results. And it can take weeks to identify less common pathogens, including some molds or fungi, and nontuberculous mycobacteria.

“It is important for providers to be able to institute a good plan for treatment, understanding what types of antibiotics to use at the moment, what types of aggressive airway clearance to employ, and how to manage complications of both cystic fibrosis and its treatment,” Dr. Winter said. “We have to be able to initiate appropriate initial care ourselves to achieve the best possible outcomes for our patients.”

Sign in to CHEST 2020 now!

Not registered yet? Register now to enjoy top-tier education through Wednesday, October 21, and access to the virtual platform until January 18, 2021.