COMMENTARY

Cystic Fibrosis: An Update With The Cribsiders

Christopher J. Chiu, MD; Justin L. Berk, MD, MPH, MBA

Disclosures

September 21, 2020

This transcript has been edited for clarity.

Christopher J. Chiu, MD: Welcome back. I'm Christopher Chiu, here with my esteemed co-host, Dr Justin Berk.

Justin L. Berk, MD, MPH, MBA: We are excited to be here. We are The Cribsiders, a pediatric podcast bringing you knowledge on pediatric topics, which we recap on Medscape. All of these recap videos are related to a podcast. Go check them out.

Chiu: This recap focuses on our episode on cystic fibrosis with Dr Whittney A. Warren, a pulmonologist and specialist in cystic fibrosis in adults. The first pearl I took away from this discussion was the fact that you should really think about undiagnosed cystic fibrosis in your adult patients with recurrent pancreatitis, GI tract abnormalities, recurrent sinopulmonary infections, or male infertility.

Justin Berk, MD: These are great insights into how patients with cystic fibrosis can present even in adulthood. I also enjoyed talking about the airway clearance regimens — how nebulized albuterol, nebulized hypertonic saline, inhaled antibiotics, chest physical therapy, and [recombinant human] DNase can all be a part of keeping the airway clear of the mucus that comes from cystic fibrosis. Another pearl is that hypertonic saline can actually cause some bronchospasm, which is why it's almost always paired with nebulized albuterol.

Chiu: The next really interesting pearl was that a higher body mass index is correlated with better lung function. This is why it's so important to emphasize good nutrition in your patients with cystic fibrosis — lots of calories, but also a smart diet. So, good follow-up in a cystic fibrosis clinic and multidisciplinary approach that includes a nutritionist is important.

Berk: In the hospital we're always giving these patients double calories or double-portion meals, and I guess that's why. For patients in the hospital who are having exacerbations, treatment is just based on old culture data. So the antibiotic selection is based on the patient’s previous resistance patterns and what bacteria they're growing, and this can be drawn from old cystic fibrosis flares that we might have data from or the records from routine maintenance of sputum cultures that are collected in the outpatient setting.

Chiu: One of the most optimistic pearls I took away from this discussion was that more than 90% of mutations in cystic fibrosis can now be treated with CFTR modulator therapy.

Berk: We also talked a lot about some of the cystic fibrosis–related complications outside the lungs. You mentioned a few in addressing diagnoses in adults. One of the big takeaways for me was cystic fibrosis–related diabetes and how it's a consequence of both insulin deficiency and insulin insensitivity, so we treat it with insulin. That being said, it's more difficult to diagnose in patients with cystic fibrosis. We don't use A1c like we do in the general population because there's such a high turnover of red blood cells in patients with cystic fibrosis. Instead, we test these patients with an oral glucose tolerance test.

Chiu: That's all the time we have today. I encourage our viewers to check out this episode on cystic fibrosis with Dr Whittney Warren as well as our other episodes on core pediatric topics.

Berk: If you want to find out more about us, just go to our website or send us an email. Thanks for joining us.

Justin Berk, MD, MPH, MBA, is assistant professor of medicine and pediatrics at the Warren Alpert School of Medicine at Brown University. He is a clinical educator active in ambulatory and inpatient patient care on medicine and pediatrics. He enjoys coffee, thinking about hiking, and being a generalist. Follow him on Twitter

Chris Chiu, MD, is assistant professor at The Ohio State University, where he is also the physician lead at OSU's Outpatient Care East Clinic and serves as the assistant clinical director for the internal medicine residency. He is an Air Force veteran and a self-proclaimed gadget geek. Follow him on Twitter

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