Cystic Fibrosis Update – PediaCast CME 094

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Description

  • Dr Katelyn Krivchenia and Brittney Haas visit the studio for an update on cystic fibrosis. Long-term outcomes have significantly improved over the past two decades. We explore why and discuss how pediatric providers can support patients and families impacted by the disease. We hope you can join us!

Instructions to obtain CME/CE Credit

  1. Read this information page.
  2. Listen to the podcast.
  3. Complete the post-test at Nationwide Children’s CloudCME.

Topic

  • Cystic Fibrosis

Presenters

Learning Objectives

At the end of this activity, participants should be able to:

  1. Describe the genetic and physiological basis of cystic fibrosis and the symptoms these abnormalities cause.
  2. Explain the importance of early detection and the role newborn screening plays in establishing the diagnosis.
  3. Identify and apply management strategies for cystic fibrosis, focusing on nutritional support, airway clearance and infection control.
  4. Evaluate the long-term outlook for patients with cystic fibrosis in light of recent advances in treatment.

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Disclosure Statement

  • No one in a position to control content has any relationships with commercial interests.

Commercial Support

  • Nationwide Children’s has not received any commercial support for this activity.

CME/CE Information

  • In support of improving patient care, Nationwide Children’s Hospital is jointly accredited by the American Nurses Credentialing Center (ANCC), the Accreditation Council for Pharmacy Education (ACPE), and the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for the healthcare team. (1.0 ANCC contact hours; 1.0 ACPE hours; 1.0 CME hours)
  • Nationwide Children's Hospital has been authorized by the American Academy of PAs (AAPA) to award AAPA Category 1 CME credit for activities planned in accordance with AAPA CME Criteria. This activity is designated for 1.0 AAPA Category 1 CME credits. Approval is valid for 2 years from the date of the activity. PAs should only claim credit commensurate with the extent of their participation.
  • As a Jointly Accredited Organization, Nationwide Children's Hospital is approved to offer social work continuing education by the Association of Social Work Boards (ASWB) Approved Continuing Education (ACE) program. Organizations, not individual courses, are approved under this program. State and provincial regulatory boards have the final authority to determine whether an individual course may be accepted for continuing education credit. Nationwide Children's Hospital maintains responsibility for this course. Social workers completing this course receive 1.0 continuing education credits.
  • Continuing Education (CE) credits for psychologists are provided through the co-sponsorship of the American Psychological Association (APA) Office of Continuing Education in Psychology (CEP). The APA CEP Office maintains responsibility for the content of the programs.

Contact Us

  • CMEOffice@nationwidechildrens.org

 

Episode Transcript

Announcer:     This is Pediacast CME. ♪♪♪ ♪♪♪ Welcome to Pediacast CME, a pediatric podcast for providers. And now, direct from the campus of Nationwide Children's, here is your host, Dr. Mike.

Dr Mike Patrick:     Hello, everyone, and welcome once again to Pediacast CME. It is a continuing medical education podcast for health care providers This is dr. Mike coming to you from the campus of Nationwide at Children's Hospital. We're in Columbus, Ohio It's episode 94. We're calling this 1 a cystic fibrosis update I want to welcome all of you to the program first right out of the gate I want to apologize for my voice it is that time of year and You know, it's virus season and I got you know, the the junk that goes around I don't think it's the flu or COVID, just a cold, but enough to give me a scratchy voice.

Dr Mike Patrick:     But the show will go on, and I just, you know, wanted to mention it in case my voice gives out. Halfway through the interview, you'll know exactly why that is. So we are talking about cystic fibrosis today by means of an update on the disease. And this is really geared toward primary care providers. Our goal is not to prepare you to manage cystic fibrosis by yourself.

Dr Mike Patrick:     Care for cystic fibrosis is of course best given by a comprehensive, multidisciplinary cystic fibrosis center like the 1 here at Nationwide Children's Hospital. So what is our goal? Well, we want to get you up to speed on the latest thoughts about this disease, including newborn screening, what we know about the cause, the symptoms, how it's diagnosed and managed, and long-term outcomes for cystic fibrosis. Now, this is an important update because really a lot has changed with regard to cystic fibrosis management really in the last 10 years and certainly since I completed my pediatric training. I mean, when I completed my training, the median age of death for cystic fibrosis patients was 24 years of age.

Dr Mike Patrick:     And today the median age is 37 years of age with lots of patients living into their 40s and 50s. And really, we think that the young patients who are being diagnosed now are expected to live really pretty much normal lifespans. So what is the difference? Well, a primary difference lies in genomic medicine. You know, with a better understanding of the exact mutations in the DNA that result in cystic fibrosis, we can create novel medications, modulators that really support the function of the abnormal protein that is seen in cystic fibrosis patients.

Dr Mike Patrick:     And work is underway really for gene editing. So, you know, classic gene therapy, which has the potential to cure this disease in the future. We're not there quite yet, but the modulators are really making a big difference for these families as we will soon discover in the course of our conversation. We'll explore these new therapies. We'll talk about the importance of newborn screening and early diagnosis.

Dr Mike Patrick:     And that's really important because the earlier you diagnose CF and get these treatments started, the better outcomes that you're going to see. And so we're going to talk about IRT levels, which is part of newborn screening, genetic testing, and of course the traditional gold standard of CF diagnosis, the sweat test. When are all of these things useful? What are their benefits and pitfalls? We'll talk about all of that along with the management of cystic fibrosis complications, a transition of care to adult providers, and again, the long-term outcomes of this disease.

Dr Mike Patrick:     Again, the goal is not to make us skilled managers of cystic fibrosis, but to understand where we are today so that we can be a trusted resource of information for our patients and families and to help us anticipate their needs, hopefully, and empower us to connect families with local and perhaps distant resources aimed at improving outcomes and quality of life. Of course, in our typical Pediacast fashion, we have a couple of expert guests to help us with the discussion. Dr. Caitlin Krivchenia is a pediatric pulmonologist at Nationwide Children's Hospital, and Brittany Haas is a nurse practitioner with pulmonary medicine at Nationwide Children's. Don't forget, you can find us wherever podcasts are found.

Dr Mike Patrick:     We're in the Apple and Google podcast apps, iHeartRadio, Spotify, SoundCloud, Amazon Music, YouTube, and most other podcast apps for iOS and Android. Be sure to subscribe to the show so you don't miss an episode and please consider leaving a review wherever you get your podcasts so that others who come along looking for free continuing medical education credit will know what to expect. Speaking of that credit, it's really easy to claim yours. Simply listen to the podcast, which you are about to do, and then head over to the show notes for this episode at pediacastcme.org. You'll find a link to the post-test in the show notes.

Dr Mike Patrick:     Follow that link to CloudCME, take and pass the post-test and the category 1 credit is yours. Once you're at Cloud CME, you'll want to click on the materials link. That's where you'll find the post-test. We offer credit to many pediatric professionals, including doctors, of course, but also nurse practitioners, physician assistants, nurses, pharmacists, psychologists, social workers, and dentists. And since Nationwide Children's is jointly accredited by all of those professional organizations, it's likely that we offer the exact credits you need to fulfill your state's continuing medical education requirements.

Dr Mike Patrick:     Of course, you want to be sure the content of this episode matches your scope of practice. Complete details are available at pdacastcme.org. Also want to remind you the information presented in our podcast is for general educational purposes only. We do not diagnose medical conditions or formulate treatment plans for specific individuals and your use of this audio program is subject to the Pediacast CME Terms of Use Agreement, which you can find at pediacastcme.org. So let's take a quick break.

Dr Mike Patrick:     We'll get Dr. Caitlin Krivchenya and Brittany Haas settled into the studio, and then we will be back with an update on cystic fibrosis. It's coming up right after this. Dr. Kaitlin Krivchenya is a pediatric pulmonologist at Nationwide Children's Hospital and an assistant professor of pediatrics at The Ohio State University College of Medicine.

Dr Mike Patrick:     Brittany Haas is a nurse practitioner with the Division of Pulmonary Medicine at Nationwide Children's. Both have a passion for supporting patients and families impacted by cystic fibrosis. That is what they're here to talk about today, to give us an update on the current screening, diagnosis, and management of cystic fibrosis. But before we get into that, let's offer a warm Pediacast welcome to our guests, Dr. Caitlin Krivchenya and Brittany Haas.

Dr Mike Patrick:     Thank you both so much for joining us today.

Dr Katelyn Krivchenia:     Thank you so much for having us. We're really excited to be here.

Brittney Haas:     Thank you so much. We can't wait to chat.

Dr Mike Patrick:     Yeah, absolutely. I'm really excited about it too. And why don't we just start with a reminder, Caitlin, if you can just give us a reminder of what is cystic fibrosis, what is this disease and what causes it just so that we're all kind of starting at the same place.

Dr Katelyn Krivchenia:     Yeah, so CF is a genetic disorder that causes a problem with a protein called CFTR. And this protein helps balance salt and water movement in the body. It is extra important in cells that line tubes or ducts in different organs of the body. So when something goes wrong with this protein, the cells can't move salt normally. And since water follows salt, the water doesn't end up where it needs to.

Dr Katelyn Krivchenia:     And this leads to thick, sticky secretions in all of these ducts or tubes. We have CFTR protein in our lungs and in our intestines and pancreas and liver and our sweat glands. So because of this, a classic presentation of CF includes pancreatic insufficiency, failure to thrive, often starting earlier in life. And then because of the changes in the mucus of the lungs, people with untreated CF can end up with chronic lung infections and changes in the airway that can lead to lower lung function and eventually death.

Dr Mike Patrick:     Now, since this is a protein that does not form correctly, That sounds like a genetic disease, correct?

Dr Katelyn Krivchenia:     Yes, it is a genetic disease.

Dr Mike Patrick:     And then how is that, is it recessive, dominant? How does that transmit from 1 generation to another?

Dr Katelyn Krivchenia:     Yeah, So this is an autosomal recessive disorder. And that's why it can pop up and occur in families that have no history of CF. And so we know that the most common mutation in Caucasian patients is the Delta F508 mutation. And that's the 1 that most of us learned about in our medical training. There's actually more than 1700 mutations of that CFTR protein that can cause disease.

Dr Mike Patrick:     And since there are multiple types of mutations that can do that, I would imagine then that it can actually present in slightly different ways? Like are some of those mutations going to make the secretions thicker than other mutations might or something of that nature?

Dr Katelyn Krivchenia:     Yeah. And so that classic teaching of CF is that Delta F508 mutation, that's, we have got 2 copies of them. And so that combination typically causes the classic things we learn about for CF. But there are several mutations that will just cause mild changes and mild symptoms that may not even present until adulthood.

Dr Mike Patrick:     And as we think about this being an autosomal recessive disorder, we have to break out our Punnett square. Again, I remember those. If 1 parent is a carrier and the other 1 is not, then 50% of the kiddos will also be carriers and 50% of them will inherit the 2 normal genes and not be a carrier for cystic fibrosis. But the issue is if 2 parents are carriers, then 25% of those kiddos are going to have cystic fibrosis, the disease, 50% of them will end up being a carrier and then 25% will be normal, have 2 normal genes and not be a carrier or have the disease. Is that how the inheritance kind of shakes out?

Dr Katelyn Krivchenia:     Yeah, that's correct. And so just being carriers doesn't mean your child is definitely gonna have CF, just sometimes a bit of education that we need to do.

Dr Mike Patrick:     Yeah, but 25% chance that they will. Yes. And then Brittany, how common is cystic fibrosis And are there particular risk factors for inheriting it?

Brittney Haas:     Yeah, so cystic fibrosis is actually a very rare disease. So in the United States, there's about 40,000 people living with CF. There's about 100,000 worldwide. And here in the US, we diagnose about 1,000 new cases of CF each year. And typically that's through newborn screening.

Brittney Haas:     And again, it's a genetic disorder. So we know that in order for a baby to have CF, both of their parents have to be a carrier. So a risk factor for inheriting it would be if there's any carriers of the genetic mutation or any family history of someone living with CF in the family.

Dr Mike Patrick:     Yeah. And of course, we can see cystic fibrosis in any ethnic group, but traditionally, at least through my training, you know, we kind of learned that people of Northern European descent, so Caucasians, it's going to be more common just because there's more of a genetic pool of carriers within the ethnic group. Is that still the case? Although, again, we have to remember it can be any race.

Brittney Haas:     Yes, that's absolutely correct. So thank you for bringing that up. We do know that certain mutations are more common amongst certain race and ethnicity groups. And so there is some truth that these mutations are more common amongst Caucasian individuals. However, we know that cystic fibrosis can happen in any race or ethnicity.

Brittney Haas:     And so we never want to rule out a diagnosis based on race or ethnicity alone.

Dr Mike Patrick:     Yeah, absolutely. And that's why newborn screening is so important because that is not going to differentiate between race because hopefully all babies are getting their newborn screen. And cystic fibrosis is 1 of those diseases that the earlier that you pick it up and start treating, the less sort of more long-term damage that there can be in the body and harm to a particular child. So it's really important. Brittany, tell us a little bit about the newborn screen for cystic fibrosis and what are its benefits and its pitfalls.

Brittney Haas:     So newborn screening for CF is still a relatively new process. So here in Ohio, we started newborn screening in 2006. And each state does it a little bit differently. So here in Ohio, we are what we call an IRT DNA state. So basically that means we have a two-step process.

Brittney Haas:     So the first thing we do when babies are in the hospital before they go home, So within the first couple of days of life, we get a blood sample. And the first thing we test for is something called IRT or immunoreactive trypsinogen. So this is a chemical released by the pancreas and we know that it can be high in babies that have CF. So Most babies with CF have elevated IRT levels. So that's the first thing we test for.

Brittney Haas:     If that comes back elevated, then we send that same blood sample and we do DNA analysis for CFTR mutations. So the genetic screening that we do as part of the newborn screening, it's only testing for about 39 of the most common CFTR mutations. So the newborn screening does not test for all of the possible CFTR mutations. Like Kaitlyn mentioned, there's about 1,700 that can cause disease. That's something that's really important to know.

Dr Mike Patrick:     So this first test that's done, the IRT, you can have false negatives because there may be a different mutation than the most common ones. So a kid could slip through and have a negative IRT test and still have cystic fibrosis. And then you can also have false positives because this particular protein can be elevated not only in cystic fibrosis, but also in prematurity or stressful deliveries. And so that's the reason then to do the genetic testing if it does come back positive. Is that correct?

Dr Mike Patrick:     Yep. Well, you mentioned Ohio is a two-step state. We have a lot of listeners who aren't in Ohio. So it really is state by state in terms of how this is done, correct?

Dr Katelyn Krivchenia:     So every state is a little bit different. And so some states will run the IRT level twice before they send genetic testing. Other states have a bigger panel that they do the genetics on that initial blood spot. So Ohio, I think we have some work to do in our state because I think we need to add some more mutations to our panel. There are some states that do that better.

Dr Katelyn Krivchenia:     And finally, there are some states that they are running full genetic sequencing on that blood test if that IRT is elevated. So there really is the spectrum and so the key is to know what your state does.

Dr Mike Patrick:     Yep, very important. And hopefully if you go to your state's Department of Health and maybe search for newborn screening, hopefully it'll have an explanation there because there are so many different options. But at least here in Ohio, you get 1 IRT. If that's positive, you get the genetic test to look for the various mutations. And that's the end of it.

Dr Mike Patrick:     But there are going to be a lot of kids who could still have cystic fibrosis and kind of pass through that screening. Now, when I was training, the sweat chloride test was how you diagnosed cystic fibrosis. Is there still a role for the sweat chloride test today?

Dr Katelyn Krivchenia:     Yes, and actually, you know, if the newborn screening test is just a screening test, it's not a diagnostic test, Like we've said, you can have missed cases. Kind of that gold standard diagnostic test is that sweat chloride. So that still holds true. And I love the sweat chloride test because it's this genius test that is cheap. It is non-invasive.

Dr Katelyn Krivchenia:     We get results that quickly and it's relatively widely available. So it's this great tool that we have. The way we think about when to use a sweat test is Any child with an abnormal newborn screen, regardless of what it comes back as the mutations, needs a sweat test to confirm that there is a problem with that CFTR protein.

Dr Mike Patrick:     And so you would use it all the time for as confirmatory if you have the IRT is elevated and the genetic test shows a mutation, you're still going to do the sweat chloride to kind of confirm things. Another time that you might use the sweat chloride is if you suspect CF in a kid who passed through the screening, the 2 screening tests, then you would still do it, right? You wouldn't just say, well, no, they were negative on their newborn screen. We don't have to worry about cystic fibrosis. If you are thinking about it, then you really ought to do a sweat chloride.

Dr Katelyn Krivchenia:     And it's Absolutely true. And some of the missed cases that we've had at our center over the past couple of years have involved people relying too heavily on that newborn screen that looks like it's normal or looks like it's low risk. Screen shows, if you have a child with a chronic cough or failure to thrive, certainly with any family history, but even those kids that just have that chronic cough, that should really be raising your suspicion to get this really easy test and rule it out. The other thing I will say is that 1 thing we know about the newborn screening test is it is less likely to catch non-white babies, okay? So we talked about how different mutations can be in different ethnic groups, And those 39 most common mutations we test for are really the most common in white people.

Dr Katelyn Krivchenia:     Okay? And so as a pediatrician and as a general care provider, understanding that just because that black baby is coughing a lot or that black 10 year old is coughing a lot or has sinus disease, I'm actually more worried about that child.

Dr Mike Patrick:     Yeah, and then you would just do the sweat chloride.

Dr Katelyn Krivchenia:     Yep, and then the way that we interpret that is relatively easy. So if it's normal, it's less than 30. A value less than 30, you can really take CF off your worry list. A value over 60, 60 and above, that diagnosis is likely CF until proven otherwise. So that's when we get that child to a CF center who can then do the genetic testing that we need to do.

Dr Katelyn Krivchenia:     And then there's this awful gray area between 30 and 59 where they may have CF or they may not. And so as a CF team, we work with them, do genetic testing and follow them clinically to make sure we're not missing a case that could present later in life.

Dr Mike Patrick:     Yeah. That sounds very frustrating for both the family and the provider. Is that something that happens often or is that pretty rare that you get those gray zone results?

Dr Katelyn Krivchenia:     Yeah, I think for Brittany and I it feels common, but it's not. It is rare. And I think the hard thing for the community physician who's taking care of these patients is they will see kind of CF on a problem list. The fancy term that we use is CFTR-related metabolic syndrome. It really rolls off the tongue there.

Dr Katelyn Krivchenia:     And basically it means we think something's up with that CF protein, but we're not quite sure exactly what it means for the child. So we follow them on an at least annual basis. But that can be a really hard spot to be in. And it's 1 of the side effects kind of of newborn screening, right? Is we catching these babies and these children that may not have otherwise been caught.

Dr Katelyn Krivchenia:     We still think there's an overall value add to the system though.

Dr Mike Patrick:     Yeah, yeah, absolutely. And for folks who want to read a little bit more about newborn screening for cystic fibrosis, we're going to have resources for you in the show notes. So you can head over to pediacastcme.org and this is episode 94 and we'll have a link there for you on a newborn screening for cystic fibrosis that kind of sums up the things that we have been talking about here. I did want to point out too on that sweat chloride, you have to be 4 weeks old to do that, right? You can't do it, you can't say, well, we don't know about the newborn screen and the kid's 2 weeks old.

Dr Mike Patrick:     You can't order the sweat chloride quite yet, correct?

Dr Katelyn Krivchenia:     Oh, Dr. Mike, it's like we planted these questions. So actually, that is a really a common misconception. And that's what many of us were taught about is that the baby needs to be big enough to like get enough sweat, right? Well, 1 of the things that we're discovering is that as we're able to start treatments earlier and earlier in life, and some of the medicines we'll get into later, end up targeting actual specific mutations, and we can actually start some of them as early as 4 weeks of age.

Dr Katelyn Krivchenia:     So if we're waiting until 4 weeks, we might be too late to get things started as early as possible. So the CF Foundation really wants everybody to get sweat chloride testing done before 4 weeks. And we have found kind of The sweet spot at our institution is at about 3 weeks of age. Gives the baby enough time to get a little bit bigger, but we're not past that 30 day window.

Dr Mike Patrick:     Yeah, and if you did a sweat chloride and you really suspected CF and they're less than 4 weeks old and it came back less than 30, would you repeat it when they're a little older or no? You've got the number and it's a good number.

Dr Katelyn Krivchenia:     We got the number and it's a good number. Now sometimes there is an issue with babies not being able to sweat enough. And so that result comes back as QNS, which is we have nightmares about quantity not sufficient, but that means it needs repeated, not, but if I get a good amount of sweat and it is less than 30, I put that away.

Dr Mike Patrick:     And that's very reassuring for families, I'm sure. As we think about cystic fibrosis, I wanna talk a little bit more about the symptoms. And the airway symptoms, I think those are pretty obvious, and those are probably the first ones that come to mind when we think about cystic fibrosis, that persistent cough, recurrent respiratory infections, sinus infections as kids get older, recurrent wheezing, asthma kind of problems. So we've got the respiratory stuff down, but the digestive system is also very involved. And Brittany, remind us, what are some of the common symptoms in kids that would make us think about CF if we're seeing particular GI-related symptoms?

Brittney Haas:     Yes, Mike, you're absolutely correct. The CF affects the whole body, and the GI tract is a big 1. So some of the GI symptoms we can see early on In life, typically what we're looking for is failure to thrive or difficulty growing. The other big thing we're looking for is malabsorptive symptoms because we know that most babies with CF have pancreatic insufficiency, meaning they have trouble breaking down fat. Symptoms we can see with that are greasy or oily stools, and sometimes we can see oil in the diaper or on their skin.

Brittney Haas:     Sometimes this can present like constipation. Sometimes there can be abdominal pain or cramps. Those are kind of the big GI symptoms that we're looking for.

Dr Mike Patrick:     Yeah, And again, just like the secretions being thick in the respiratory tract, GI secretions being thick are going to make it so that pancreatic enzymes are not delivered well to the gut, so we can't break down food and in particular fats. And so that's why you get the greasy stools. Because things aren't broken down, we get the malabsorption and then we get poor weight gain and growth and failure to thrive because these nutrients are not being absorbed correctly because they're not being broken down correctly, which goes back to those enzymes. Is that a good summary?

Brittney Haas:     Yeah, absolutely.

Dr Mike Patrick:     I

Brittney Haas:     think the other big take home point about symptoms as well is just that everyone with CF can present differently. So we actually have some families that have multiple siblings with CF. So they have the same genetic mutations, they're living in the same home environment, and still they can have different symptoms and disease presentations.

Dr Mike Patrick:     So let's say we have a child and we do determine, you know, we've done maybe their IRT was elevated, they had the genetic testing, which did show a mutation that's concerning, then they had the sweat chloride, which ends up being over 60. And so we have the diagnosis of cystic fibrosis. Kaitlin, what then do we do? What are then the first steps of management for this disease?

Dr Katelyn Krivchenia:     Yeah. And so luckily, For most patients, we're finding this out when they're still babies, right? So our very first thing that we focus on is really that nutrition and growth aspect of things. And so the first thing we do is try to replace the enzymes that their body is not likely making. Or Actually, to be fair, they're making the enzymes, it's just not getting to the right spot to digest the food like you described.

Dr Katelyn Krivchenia:     So most people with CF will end up needing to have pancreatic enzyme replacement therapy. A trade name for this would be Creon. Basically, we're giving them amylase, lipase, and pancreatic enzyme before they eat. Now any of us that have had children or any of us as pediatricians understand how crazy busy those first couple months of life are, and eating is a big deal. So we're nursing or having a bottle every 2 to 3 hours.

Dr Katelyn Krivchenia:     Well, the baby is not going to see most of that nutrition unless we're giving him or her the enzymes. So what we actually do is we have parents open an enzyme capsule and put the little enzyme beads on a spoon with some applesauce. We actually teach these babies to eat applesauce before they are able to go to the breast or the bottle. And that allows their body to then digest the enzymes. And in general, when we're seeing these babies come to clinic, they are not growing well, they're failing to thrive.

Dr Katelyn Krivchenia:     And just with the introduction of enzymes, we can get them back on their growth curve and developing appropriately. But you can imagine that that's a lot, right, for a new family to kind of take on.

Dr Mike Patrick:     Yeah, Yeah. So we've talked about pancreatic enzyme supplementation and how important that is for these kids, you know, right from infancy. Are there other things in their diet that we need to think about supplementing other than just the pancreatic enzymes?

Dr Katelyn Krivchenia:     Yeah. And actually it's something that we don't, I think people don't think about immediately. 1 of the really more important things we do for these babies is also make sure they have enough salt. So we know that people with CF sweat out a lot of salt. It's 1 of the ways we diagnose patients, right?

Dr Katelyn Krivchenia:     With our sweat chloride. But babies can have a lot of trouble regulating their salt and water and they can sweat too much. So before we had newborn screening, 1 of the ways babies with CF would present would be in profound hyponatremic metabolic failure. And that's how we discovered that they had cystic fibrosis. This is especially true during the summers in hot environments, babies who are very bundled all the time.

Dr Katelyn Krivchenia:     And so when we first see babies in clinic, not only are we adding those enzymes, but we're also having them add salt to that little applesauce as they take in their medications. So salt's a very important part of it. And additionally, we also will put these babies on an extra vitamin supplement, those fat soluble vitamins that we all know and love, the ADE, and K, we give them extra of those too, because we know that they're not absorbing things 100%.

Dr Mike Patrick:     Yeah, yeah. And so when we see kids who come in with vomiting and diarrhea, like in the emergency department, for instance, we might be quicker to check their electrolytes if they have cystic fibrosis compared to someone who doesn't, correct?

Dr Katelyn Krivchenia:     Yes, That's a really good point. And even kids who are on salt supplements, we have had a couple of patients recently that parents are having a hard time getting the salt in, and that might not be a big thing to worry about in their mind. And then they present with this hyponatremia. So I do think it should be that red flag to check those electrolytes a little bit sooner, worry about it a little bit more.

Dr Mike Patrick:     Yeah, with the with the cystic fibrosis patients for sure. Would you, you know, we do see a lot of kids that are failure to thrive with no other symptoms at all. Should all of those kids have a sweat chloride, do you think?

Dr Katelyn Krivchenia:     So I think it kind of depends a little bit on whether or not you have other warning symptoms. It should certainly, CF should be on your list. But generally kids that are failing to thrive with CF are doing so with really frequent stools, that kind of fatty oily stools, or we've had some babies that they just aren't satisfied so that they're taking 40 or 50 ounces and not growing. They're getting the food in, they're not growing. So then we're thinking about an issue with absorption and that's when you should be thinking, let's get a sweat chloride and make sure that's not going on.

Dr Mike Patrick:     Yeah, absolutely. And then at what point do we start treating respiratory symptoms? Do you wait until there are problems or are there things preemptively that you can do when you first get the diagnosis of CF?

Dr Katelyn Krivchenia:     Yeah, that's a great question. And that tends to be the thing that people are most focused on, right? Is how do I keep my kids' lungs healthy? But the great thing about managing this disease in babies is that they're born with very healthy lungs. So there is actually nothing wrong with the structure or the function of the lungs when they're born.

Dr Katelyn Krivchenia:     It's the constant accumulation of thick, sticky mucus over months and years that can cause damage. And so as long as we can be proactive about keeping those secretions moving nice and thin and moving out of the airways, we can prevent a lot of lung disease. So we're generally introducing what we call chest physiotherapy pretty early in life by about 2 months of age, but we're just doing it a couple times a day, try to get like those secretions moving. We generally do nebulized albuterol and then also mucolytic therapy. So medicines that help break up that mucus, like Dornes-alpha or Palmazyme or hypertonic saline are other tools we use to help the baby keep their airways clear.

Dr Katelyn Krivchenia:     And many of your listeners who aren't taking care of CF regularly may know about the VEST machine when we see patients with CF on a VEST. And that's a really great tool we have for kids when they're closer to a year of age. So the infants, we're still kind of relying on that hand chest physiotherapy, but that vest is a great tool as kids get more mobile to really help with that airway clearance.

Dr Mike Patrick:     Yeah. And the things that we've talked about so far are still pretty much the things that I learned, you know, like 30 years ago when I first learned about CF in medical school. But there's so much more today compared to back then. And a lot of that does come down to that genetic testing. And we've talked many times on this program about genomic medicine.

Dr Mike Patrick:     And so if we know which mutations are present in a particular person, we might be able to tailor their treatment based on the mutation that they have. That's true for lots of diseases. Is that also true for CF?

Dr Katelyn Krivchenia:     It is, and that's a very exciting part of being part of CF right now and CF care and having these new babies come to your clinic and being able to give some hope with some new medicines that are mutation-directed.

Dr Mike Patrick:     And how do those… So if it's an issue with the protein, we have heard about gene therapy being able to treat a malformed protein in order to eliminate or cure a disease. I would imagine that's not quite there with cystic fibrosis, because if it was, that would have been our opening

Dr Katelyn Krivchenia:     topic. But

Dr Mike Patrick:     are we heading in that direction?

Dr Katelyn Krivchenia:     We are heading in that direction. Now, you know, the new medications we have right now are called modulator therapies. And they're modulator therapies because they really help change the protein to function better. And so anybody who's had a medical training in CF can probably has nightmares about the different mutations of the CF protein and the different class of mutations and there's all these things you need to know. But the basics are, in most cases, the protein gets made, but it's folded incorrectly, and it doesn't get transported to the right spot, and then it doesn't open the right way.

Dr Katelyn Krivchenia:     And so we have medicines now that can help the protein fold better and then help the protein open better. And so there's several different medications that have become available over the past 10 to 12 years that have targeted whatever the problem is, either folding or opening.

Dr Mike Patrick:     Yeah. And the exact issue with folding and opening is going to depend on the exact mutation that you have. And so that's the reason why you need to know that in order to pick which medicine, which modulator is going to be best for that particular patient.

Dr Katelyn Krivchenia:     Correct. And the first 1 that really came out that was very exciting was back in 2012 called IVACAFTR. And it was this game changer for about 5% of the CF population. So if you had a gating mutation, 5% of the population, we could basically turn your body into a carrier functioning like a CF carrier. So potentially no symptoms.

Dr Katelyn Krivchenia:     And that was wildly effective for those patients, but we didn't have a way to reach the rest of the population. And so through lots of clinical trials, lots of funding and time and forward movement from the CF Foundation, they came out with true corrector therapies, which help the protein fold better. And the most exciting 1 has been out over just the past couple of years. The trade name of that 1 is Trikafta. And I will say the actual generic once because it is actually 3 medicines altogether, Alexacafter, Tezacafter, Ivacafter.

Dr Katelyn Krivchenia:     And so these are all cafter medications that are folding proteins and making them open up normally. And the cool thing about that is anybody that has a Delta F mutation can qualify. And so we're hitting more than 90%, well above 90% of our patients are able to qualify for this medication. And that we can start as early as 2 years old right now.

Dr Mike Patrick:     Yeah, that is really, really exciting. We've talked about the complications in the lung and with the pancreas. 1 thing that we haven't talked about, you know, the pancreas also makes insulin. Is there an association with cystic fibrosis and diabetes?

Brittney Haas:     Diabetes is actually 1 of the most common complications we see with CF. And when we look at the data in the registry, almost 19% of people with CF have diabetes. And I work with our adults as well. So I see this firsthand. There's a large portion of our adult population that develop diabetes.

Brittney Haas:     So we actually start screening for this in the pediatric population with oral glucose tolerance testing.

Dr Mike Patrick:     And what percentage of patients would you guesstimate will end up having diabetes that have a cystic fibrosis? Caitlin, do you have any ideas about that?

Dr Katelyn Krivchenia:     Yeah, it's in the ballpark of 15 to 20%.

Dr Mike Patrick:     So a significant number, And that's something I think too, that primary care providers can really talk to their patients about because you're seeing them often. And you know, the pulmonary folks are really tuned in. You guys are going to be tuned into everything, But it is an area where primary care docs, you know, put that in your ticker to talk about diabetes with these families and to be on the lookout for it. Because just like everything else, the sooner you catch that, the healthier the outcomes are going to be, right?

Dr Katelyn Krivchenia:     Absolutely.

Dr Mike Patrick:     Yeah. And then I also want to talk about infertility. That's another complication, particularly in boys, because the thick secretions can also impact the delivery of semen and sperm to the eggs. And so you can have infertility problems with cystic fibrosis in boys, correct?

Brittney Haas:     Yeah. So actually the vast majority of males with CF are considered infertile. So the problem actually happens during development in utero, and the problem is with the development of the vas deferens. So sperm is actually made, it just can't get out. So men with CF are still able to have children through IVF, but they can't typically conceive naturally.

Brittney Haas:     And so since this is typically a structural problem, Trikafta does not impact that at all. So we haven't seen any change in fertility for males with CF with these new medicines. However, we also can see subfertility in females with CF. So not that they can't get pregnant, but it can potentially be more difficult for them because of the thick, sticky mucus in the cervix as well. And we actually have seen this improve with Trikafta.

Brittney Haas:     So we've heard reports of women reporting their cervical mucus is more thin. And we've actually had a pretty big pregnancy boom ever since this medication was released in 2019. So it's really exciting that we have females that thought they would never have children and struggled with infertility for years that started this medicine and were able to get pregnant pretty much right away.

Dr Mike Patrick:     Yeah. And then this is, I think, another opportunity for pediatricians to talk with their CF families because you don't wanna say, well, I have CF and so I can't get someone pregnant if you're a boy. I mean, protection is still gonna be important And we have to also emphasize that sexually transmitted infections are also possible. And so that's another reason to use protection. And so the whole family planning talk still needs to happen, right, in cystic fibrosis teenagers.

Brittney Haas:     Absolutely. And that's 1 of our biggest focus in our teenage populations, actually. So we have a formal transition program to help support our teenagers as they transition from our pediatric program to our adult program, and fertility is definitely 1 of those big topics.

Dr Mike Patrick:     And as we think about teenagers, I suppose there's also a pretty big mental health component to cystic fibrosis that we don't want to forget about. Any chronic disease is going to take its toll emotionally in terms of anxiety and depression and balancing disease treatment and your life, you know, your social life and all of those things. So I suspect that you guys are really on the lookout and primary care providers should also be on the lookout for any mental health issues in these kids, right?

Brittney Haas:     Absolutely. That's another 1 of our big focus in this population. It's something we take very seriously in anyone with CF. And actually, we can see this in caregivers of infants with CF as well. So we have a mental health coordinator as part of our team, and we do mental health screening as part of our annual assessments.

Brittney Haas:     So this is definitely something that we're keeping very close tabs on because we know that there are increased risk for depression and anxiety.

Dr Mike Patrick:     Yeah, and we're gonna have some resources in the show notes again over at pediacastcme.org on cystic fibrosis and depression, just some additional materials for folks to take a look at. And then as we also think about teenagers, transitioning from pediatric care to adult care is also going to be an issue. And as we see longer lifespans of folks with cystic fibrosis, that's going to become even more and more of a big deal. I also know in a lot of places, the pediatric folks kind of hang on to these patients. And so they don't necessarily transition to adult providers.

Dr Mike Patrick:     Or I don't know, is there like a cystic fibrosis folks who are becoming more specialized in just adult care, kind of like in congenital heart disease?

Dr Katelyn Krivchenia:     Yeah. And you know, that is something that the CF caregivers have kind of been struggling with across the country. But similar to like how the newborn screen is different in every state, every program has a little bit of a different process. So our program is set up where we actually have our adult providers in our own clinic. And so a transition for our patients is really a different hallway with a different provider team.

Dr Katelyn Krivchenia:     There are definitely some centers out there that have done transition to a true adult hospital very well. And there are adult pulmonologists that have kind of taken the reins on adults CF care as well. As with all of these potential comorbidities, it is multidisciplinary. And so often it's not just finding a lung doctor that's willing to take care of them, but a GI doctor and an endocrinologist and a psychiatrist, psychologist, all of those things. And so it is, we still consider CF a pediatric disease, but you're absolutely right that we're gonna get them living longer and longer, so it's a chronic illness and so we'll have to continue to have chronic care.

Dr Mike Patrick:     Yeah, absolutely. That's an exciting thing.

Dr Katelyn Krivchenia:     It's great. That's great.

Dr Mike Patrick:     That's right. So, Brittany, as we transition teenagers into adulthood and they become adults with cystic fibrosis, Are there additional health concerns beyond just the cystic fibrosis itself? Are there other complications in the adult patient that we should be thinking about?

Brittney Haas:     Mm-hmm. Yep. So we talked through some of the complications of CF, but 1 of the big ones from a GI perspective is actually that people with CF have an increased risk of colon cancer. So the risk for colon cancer in adults with CF is actually 5 to 10 times greater than the general population. So that's a pretty big increase.

Brittney Haas:     So we get colonoscopies earlier in these patients. So we start at age 40 and we actually get them every 5 years if normal. And if there's any polyps or concerns, then we'll follow even more frequently.

Dr Mike Patrick:     Yeah. And I would suspect that's something that we've just been learning about just as these patients reach adulthood and live longer and then we say, oh their rate of colon cancer is way higher, which you know 20 years ago we may not have known that because they didn't reach the age when that becomes a risk. Mm-hmm

Brittney Haas:     And we're still really learning about why that increased risk exists and also learning how these modulators could potentially mitigate that risk. We don't know the answer to that.

Dr Mike Patrick:     Yeah, so more to come. Brittany, are there other ways that primary care providers can support families that are impacted by cystic fibrosis. We've talked about some of the ways, but are there other things that we should really be thinking about, particularly in areas where there might not be as well-developed of a cystic fibrosis program as there are at major children's hospitals?

Brittney Haas:     Yeah. So, a couple of things I think about in terms of the role of the primary care provider, I think a big thing is keeping up with vaccines and screenings. And of course, we always want people with CF to be referred to an accredited CF center to help manage the CF side of things. And the CF center should be able to manage everything related to CF. But I think the big things that we think about for the primary care provider is making sure we're not missing all the routine stuff that we're not thinking about in CF clinic.

Brittney Haas:     I think the other big piece of things is keeping an eye out for the kids that potentially got missed by newborn screening. So primary care providers definitely have a big role in that. If you have a kid that's failing to thrive or chronic pulmonary issues, the PCP definitely has a role and they can order a sweat test and help and then coordinate followup with the CF center if needed. So there's definitely a huge role for the primary care provider in helping care for these patients.

Dr Mike Patrick:     Yep. And I would say education is also gonna be important. Parents have questions and sometimes the questions don't come to mind when they're in the CF center and then they get home, they start thinking about things. Of course, you guys are always fine with folks asking you questions through MyChart and those kind of patient portals, But parents may have questions when they come in to see their pediatrician. And so the more that we understand about the current treatment of cystic fibrosis, the better we're gonna be able to help raise the health literacy and improve outcomes by helping families understand the disease.

Dr Mike Patrick:     So yeah,

Dr Katelyn Krivchenia:     and a lot has changed, a lot has changed, right, in the world of CF. And so what the CF that we learned about 10 years ago, it's very different from the CF today. And it's 1 of the reasons why, you know, Brittany and I are doing a bunch of outreach to certainly the areas in Ohio is that we are hearing a lot of misinformation, right? And people that come with this new diagnosis of CF that are devastated because they think their child's not going to live past adolescence, right? Or their 20s.

Dr Katelyn Krivchenia:     And so part of our education is also around, hey, this is a different disease than it was 10 years ago. Nothing you Google, right, is going to be accurate. And so kind of calling those centers that do exist and trying to get some guidance and help, it can be really, really helpful to help you feel like you got those tools to have that conversation.

Dr Mike Patrick:     Yeah, absolutely. For folks who do live like pretty far out, do you, so how often do you see kids with cystic fibrosis in your clinic? Like let's say a kid's doing well, just sort of the routine rechecks.

Dr Katelyn Krivchenia:     Yeah, it's about every 3 months, right Brittany?

Brittney Haas:     Yeah.

Dr Katelyn Krivchenia:     So if you

Dr Mike Patrick:     live 3 hours away from the closest children's hospital, I mean, do you offer like virtual appointments or do you work in conjunction with a patient's primary care team, like if they're far out? Is that something that comes up?

Dr Katelyn Krivchenia:     It does come up. You know, we're fortunate in Ohio because we have so many CF centers, but we even have, you know, a child that lives 20 minutes away that doesn't have good transportation that we're having to work with the PCP and trying to do some virtual visits and those kind of things to try to at least still keep an eye on them as we try to find the best way to support them.

Dr Mike Patrick:     Yeah. So ultimately, we all want what's best for particular kids and families. And while it's best for sure to be seen physically in a CF center, you know, some folks are going to have difficulties and barriers and things that we all have to kind of work through. You did talk about life expectancy. And so a lot of people, if they Google it, they're going to see, oh, you know, in the median age of death I saw in 1999 was 24 years of age I mean 1999 wasn't that long ago and now we're up in 2020 the number I saw was like 37 as and that's the median age of death.

Dr Mike Patrick:     So median means that there's folks, you know, living in their 40s and 50s. And as these treatments get better, it's gonna, like you said, it's gonna be more of just a chronic disease that you live with. Are there anything else we need to know about long-term outcomes with cystic fibrosis?

Dr Katelyn Krivchenia:     Yeah, I think, you know, our counseling has changed drastically over the past 10 years of what we're telling families to expect, right? And so even the median survival that you're able to find on good sources like the CF Foundation website and those things, they're not taking into account these new modulator therapies and being able to start them in some cases as young as 4 weeks. And so I anticipate that we are going to see that life expectancy move much closer to normal, because lung disease is what kills people with CF. And we have the tools to, in most cases, prevent lung disease, which is so exciting. Yeah, yeah.

Dr Katelyn Krivchenia:     Put ourselves out of a job.

Dr Mike Patrick:     Yeah, well, And then where does, we haven't talked about lung transplant. Is that still something that some folks with CF will end up needing lung transplant or is, are these modulators going to eliminate that from really being a need?

Dr Katelyn Krivchenia:     Yeah. So in, in the past, like lung transplant was definitely on the list of once your lung function got closer to that 30% and you were in the hospital a lot with big exacerbations, we were thinking about transplant a lot more proactively. Now that these modulator therapies cover most mutations, it is not even something that I want to talk about with my family's that asked that same question, right? Am I gonna have to have a lung transplant? We put that so far out of our head They really should not need a lung transplant.

Dr Katelyn Krivchenia:     Some of the, 1 of the risks of getting so excited about these new treatments though, is that they're still like, you know, 3 to 5% of the CF population that don't have access to these drugs. And so those are the kids that, and the adults, right, that we are continuing to treat in that classic CF fashion, and their life expectancy is very different. But that's where some of the new research and kind of the ideas moving forward from the CF Foundation, our goal is to find a cure, right, and not just a treatment. So it's still on the table for some patients, but the vast majority of patients do not need to worry

Dr Mike Patrick:     about that. The small number who don't have access to the medications, the modulators, is that because of not being able to afford them or not being in the healthcare system and getting diagnosed and those kind of things?

Brittney Haas:     Yeah, so it actually depends on which mutation they have, whether they're eligible for these medicines. So we mentioned the most common mutation, Delta F508. If you have 1 copy of that, you're eligible for it. So that's somewhere in the 90% range of our patients are eligible for it. 1 of the other cool technologies that we have available for our patients that have mutations that are not eligible, we actually have ways that we can swab their nose to get a sample of their cells and then in vitro we can actually test how well these medicines work for their specific mutation.

Brittney Haas:     So there have been some rare cases where we potentially are able to get patients on medicines that way if they don't potentially qualify. But the big focus is really finding treatment that is available for everyone. And I do want to comment on the cost of these medicines because these are extremely expensive medicines. And so far, it is a new medicine, so we have not run into access issues a ton yet. But I do worry that that is a problem going forward, that we've been able to get them covered, but if that becomes an issue, the cost could definitely potentially be prohibitive.

Brittney Haas:     So that's something we'll continue to advocate for.

Dr Mike Patrick:     Yeah, yeah, absolutely. And I think that's something too that pediatricians in general would take up the charge because, you know, these are our patients too. And so we definitely want, you know, all kids to get diagnosed and to get treatment right away and to live their best healthiest lives that they can just like the rest of us have that opportunity. So I think that's just so, so important. Kailin, what are some other hot topics then in cystic fibrosis research right now?

Dr Katelyn Krivchenia:     Yeah, so we have these modulator or corrector medications. And so there's research going on to make that, make those medicines easier to take and take them less frequently and have fewer side effects. And so that is happening. But at the same time, the CF Foundation has committed a lot of money and time and really smart minds to try to figure out how we help correct the protein on the genetic level. 1 of the cool studies going on right now is actually inhaled CFTR messenger RNA that we can actually inhale the messenger protein or the messenger RNA so that the correct CFTR protein gets made by the cells.

Dr Katelyn Krivchenia:     And so that is an ongoing trial right now. There's also gene therapy trials in the pipeline, right, that will use that viral vector to try to deliver the CFTR DNA to the cells to then make the normal protein. And then down the road is gene editing, right? Like, can we actually affect this problem at the level of the patient's own DNA? So it's all very exciting and nobody is saying, hey, we've got this medicine, we can just stop, right?

Dr Katelyn Krivchenia:     There's a lot of good time and effort going into it.

Dr Mike Patrick:     Yeah, yeah, absolutely. And I guess this is 1 of the bright sides, if you could call it that, of the pandemic. Because as we think about the COVID vaccines being messenger RNA-based, like our knowledge of this technology and being able to use it in other circumstances, I mean, if we're going to look at a silver lining, I guess that's from living through a pandemic. That is 1 of them.

Dr Katelyn Krivchenia:     There you go.

Dr Mike Patrick:     And then Brittany, tell us about the Cystic Fibrosis Center here at Nationwide Children's Hospital. It really is a multidisciplinary effort, right?

Brittney Haas:     Absolutely, Yep. So it takes a whole village to care for these patients. So not only the parents who are doing the whole work, but we have a whole team of physicians. We have nurse practitioners, nurse clinicians, we have dietitians, social workers, respiratory therapists. We're fortunate enough to have a genetic counselor, not all CF centers do.

Brittney Haas:     We also have mental health coordinators and child life specialists, so truly it is a team effort. Here at our center we have 3 separate CF programs. So we have our infant program and we follow patients from diagnosis to 3 years of age or from birth to 3 years of age, and then we transition them to the pediatric program where they're followed until about age 20 to 21 is when we start that process to transition to the adult program. So here at our center, we actually follow patients throughout the lifespan. And again, we follow these patients at least every 3 months, as long as they're doing well.

Brittney Haas:     But in our infant clinic, we start out following them a little bit more frequently as we're diagnosing them and starting these treatments. So we follow them weekly at first and then kind of space that out. So after about a few years, about after about 1 year of age, we're typically seeing them about every 3 months.

Dr Mike Patrick:     So you really get to know these families. They become like part of your family. I would

Brittney Haas:     imagine when

Dr Mike Patrick:     you're seeing them that often and for that long.

Brittney Haas:     Yeah, and I think that's the reason we all love working with CF so much is that these patients truly become part of the family.

Dr Mike Patrick:     Yeah, absolutely. Well, we really appreciate both of you stopping by and chatting with us about cystic fibrosis today. Once again, in the show notes, we are going to have a lot of resources for folks about cystic fibrosis, newborn screening, the multidisciplinary nature of our cystic fibrosis program here at Nationwide Children's Hospital. So please do check out the show notes over at pdacastcme.org, episode 94, and you will find all of those resources there in the show notes. So once again, Dr.

Dr Mike Patrick:     Caitlin Kravchenia and Brittany Haas, thank you both so much for stopping by and chatting with us today.

Dr Katelyn Krivchenia:     Thanks. This has been a really fun

Dr Mike Patrick:     time.

Dr Katelyn Krivchenia:     Thank

Dr Mike Patrick:     you so much. We are back with just enough time to say thanks once again to all of you for taking time out of your day and making Pediacast CME a part of it. Really do appreciate that. Thank you to our guests this week, Dr. Caitlin Krivchenia, Pediatric Pulmonologist, and Brittany Haas, nurse practitioner, both with pulmonary medicine at Nationwide Children's Hospital.

Dr Mike Patrick:     A couple of other things I wanted to mention about the links that we're going to have for you in the show notes, in addition to the ones that we mentioned during the interview, we also have a couple of helping hands that might be helpful for your families. 1 on the newborn screen blood test and another on CFTR-related metabolic syndrome. And again, remember that is where they don't necessarily meet the criteria for cystic fibrosis, but there is still some problems that may need to be followed, and they just don't necessarily have the same level of risk factors as full-blown cystic fibrosis. So we have a helping hand on that condition as well. And then also coming up on May 9th, 2024 at noon Eastern time, there's going to be a 1 hour Cystic Fibrosis Newborn Screening Virtual Conference that is free.

Dr Mike Patrick:     And I'll put a link in the show notes if you'd like to register for that. It's really aimed at providers and talking a little bit more in depth about newborn screening and with a focus on the state of Ohio but I'm sure that it's similar for other states so you may want to check that out again we'll put a link to that in the show notes for you. Don't forget you can find our podcast wherever podcasts are found. We're in the Apple and Google podcast apps. IHeartRadio, Spotify, SoundCloud, Amazon Music, YouTube, and most other podcast apps for iOS and Android.

Dr Mike Patrick:     Our landing site is pdacastcme.org. You'll find our entire archive of past programs there, along with show notes for each of the episodes, our CME information, that terms of use agreement, and the handy contact page if you would like to suggest a future topic for the program. Reviews are also helpful wherever you get your podcasts. We always appreciate when you share your thoughts about the show and we love connecting with you on social media. You'll find us on Facebook, Instagram, threads, LinkedIn, Twitter, X, all of those places.

Dr Mike Patrick:     Simply search for PDA cast. So you've listened to the podcast. Now be sure to claim your free category 1 continuing medical education credit. Really easy to do. Simply head over to the show notes for this episode at pediacastcme.org.

Dr Mike Patrick:     You'll find a link to the post-test in the show notes. Follow that link to CloudCME, take and pass the post-test and the category 1 credit is yours. Be sure to click on the materials link to find that post-test. Super easy, right? And again, we do offer credit to many pediatric professionals, including physicians, nurse practitioners, physician assistants, nurses, pharmacists, psychologists, social workers, and dentists.

Dr Mike Patrick:     Of course, you want to be sure the content of the episode matches your scope of practice. Complete details are available at pdacastcme.org. Also don't forget about our parent podcast, Plain PDA Cast without the CME. It's an evidence-based podcast for moms and dads. Lots of pediatricians and other providers also tune in as we cover pediatric news, answer listener questions, and interview pediatric and parenting experts.

Dr Mike Patrick:     Shows are available at the landing site for that program, pediacast.org. Also available wherever podcasts are found, simply search for Pediacast. So my voice made it through. It's going to rest now for a few days. Hopefully it'll be a little bit better next week.

Dr Mike Patrick:     Thanks again for stopping by. And until next time, this is Dr. Mike saying, stay informed, keep it evidence-based, and take care of those kids. So long, everybody.

Announcer:     This program is a production of Nationwide Children's. Thanks for listening. We'll see you next time on PediaCast. C-M-E.

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