Transcript
Speaker: Welcome to Critical Matters, a sound podcast covering a broad range of topics related to the practice of intensive care medicine.
Speaker: Sound provides comprehensive critical care programs to hospitals across the country.
Speaker: To learn more about our programs and career opportunities, visit www.soundphysicians.com.
Speaker: And now your host, Dr. Sergio Zanotti.
Speaker: Due to advancements in medical and surgical care, the survival of patients with congenital conditions into adulthood has dramatically increased.
Speaker: However, as these individuals transition to adulthood, their unique physiology, chronic complications, and evolving care needs create significant challenges for their management when they are admitted to the adult intensive care unit.
Speaker: Today's podcast episode will discuss adult congenital disease in the ICU.
Speaker: Our guest is Dr. Cameron Desfoulian, a pediatric and adult critical care physician.
Speaker: He is the director of adult congenital heart disease program development for the section of critical care at Texas Children's Hospital.
Speaker: He is faculty for cardiothoracic critical care at Baylor St.
Speaker: Luke's Medical Center and a senior faculty member at Baylor College of Medicine in Houston, Texas.
Speaker: It's a true honor and pleasure to have him as our guest today.
Speaker: Cameron, welcome to Critical Matters.
Speaker: Thank you, Sergio.
Speaker: Really pleasure to be here.
Speaker: Well, this is a topic that I know fascinates both of us for different reasons, but definitely, I mean, you with your extensive training in pediatric and adult medicine and then critical care obviously have, I think, a very unique perspective on this growing population.
Speaker: But I would like to start maybe with why should other intensivists who don't have your niche and training care about this topic?
Speaker: Yeah, well, great question.
Speaker: I mean, I think the practical answer is because it's coming your way.
Speaker: And if it's not already there, the reality is, is across the spectrum of what used to be called pediatric illness.
Speaker: So that's kids with genetic defects, syndromic children, you know,
Speaker: our preemies, and really I'm talking about our micro preemies, adults who are now, you know, used to be children with congenital heart disease.
Speaker: Our survivals are incredible, right?
Speaker: But what that means is that there's all these patients who had chronic illnesses that would die as children that adult intensivists never dealt with that now all of a sudden are surviving into adulthood.
Speaker: So there's a lot of intensivists who, if they already haven't experienced it, are going to be seeing lots of
Speaker: chronically ill young adults, because these are often 20s and 30s, who have illnesses that started at the moment of birth and have been managed for the first two decades of their life by a pediatric group, often not just one physician, but a group of physicians.
Speaker: And now all of a sudden they're transitioning to adult care.
Speaker: And I think there hasn't been a whole lot of
Speaker: planning, education, or kind of multidisciplinary meeting of minds between the two sides of peds and adults.
Speaker: And so basically, this is a group of patients that's coming to you soon if you're an adult intensivist, but you may or may not be fully aware of what's coming to you.
Speaker: Yeah.
Speaker: And I read it.
Speaker: I don't know if this is a true stat, but in terms of, like you said, they're coming our way.
Speaker: I read that specifically with heart disease, there are more adults with congenital heart disease than kids with heart disease right now, congenital heart disease.
Speaker: So as they live longer and longer, that population keeps growing, right?
Speaker: Absolutely.
Speaker: And I think that, like you mentioned, this also applies, as we'll talk, to many other conditions.
Speaker: So why are these patients different from any other adult patient in the ICU?
Speaker: Yeah, well, I mean, I think the, you know, the chronic illnesses or chronic comorbid conditions that these patients carry are very different than what I think we're used to as adult intensivists.
Speaker: So, you know, our classic chronic conditions that we deal with all the time are atherosclerotic disease, obesity, diabetes, hypertension, you know, things like that, maybe a prior stroke, dementia,
Speaker: that's very different than what the chronic comorbidities of these patients are.
Speaker: And with that, that's a lot of the challenge in critical illness because you've got to then deal with what is essentially the baseline.
Speaker: It's not a normal baseline.
Speaker: It's a different baseline than what you're used to.
Speaker: So you have to understand what's the best case scenario, where are you starting from, which I think most of us would agree that in critical illness, our hope is to get back to baseline.
Speaker: We're not going to improve the patient in most situations, but we want to get them back to the baseline.
Speaker: But if you don't understand what that baseline is and how it impacts your patient, then you really don't know what you're shooting for.
Speaker: And the other question I wanted to ask you before we move on to more specific conditions is, historically, when this group was a smaller group, they just stayed in the care of pediatricians.
Speaker: What's changed?
Speaker: Well, I think the biggest thing that's changed is the sheer numbers and the age of these patients, right?
Speaker: So you're absolutely right.
Speaker: They used to stay within the PETE setting, but that was because, you know, let's take like, you know, congenital heart disease or, you know, in the old days, cystic fibrosis before the care got so good.
Speaker: Chances are they were going to die by the time they got to 30.
Speaker: So the pediatricians would just kind of flex a little and handle the 20, 30 year olds.
Speaker: Now, all of a sudden, you're dealing with adults with congenital heart disease who are in their 50s and 60s who now have the overlay of adult disease, things like atherosclerosis and dementia happening and a variety of other adult problems such that the pediatricians don't feel equipped to handle it.
Speaker: And yet...
Speaker: the adults don't really feel equipped handling it either because they don't understand the pediatric illness.
Speaker: So these patients really start falling through the cracks.
Speaker: It becomes really challenging for them to be managed in either setting.
Speaker: Many pediatric hospitals, as this phenomenon has been going on, have started setting hard and fast rules as to when patients have to transition.
Speaker: And so most places right now are setting numbers between 21 and 25 years of age where they basically tell their clinicians,
Speaker: you have to stop admitting patients after this age.
Speaker: And so it forces the issue of transition because of the problems that were being created by physicians who were holding on a little too long to the patients.
Speaker: And yet what that means is that these patients now are delivered to adult centers where they're not necessarily equipped to handle what the comorbidities are.
Speaker: Let's dive into some of the specific congenital diseases that might result in adults with these diseases presenting to an ICU care.
Speaker: And there's three big categories that I wanted to go over.
Speaker: First and foremost is congenital heart disease.
Speaker: Second is genetic diseases and syndromes.
Speaker: And third, I mean, you talked about the prematurity-related conditions in adults or like the super preemies.
Speaker: which seems to be something that we are younger and more and more premature babies are surviving to adulthood.
Speaker: So that has its own set of problems.
Speaker: And maybe we can start with congenital heart disease, which I understand is the most common congenital defect in newborns, affects thousands of babies every year in the United States.
Speaker: What are some of the common conditions that might end up in the ICU of an adult and also some of the general ICU considerations that you would consider there?
Speaker: Sure.
Speaker: Thanks.
Speaker: So, you know, when we talk about congenital heart disease, first of all, we're talking about roughly nine in a thousand births.
Speaker: Okay.
Speaker: So just short of 1% have congenital heart disease and the vast majority of those.
Speaker: So about seven out of those nine are, you know,
Speaker: forms of congenital heart disease that honestly are not all that impactful in the long term.
Speaker: So when we talk about things like atrial septal defects, ventricular septal defects, patent ductus arteriosus, most of those are handled fairly early in life.
Speaker: The treatment is essentially curative and the comorbidity 10, 20 years later is next to nothing.
Speaker: So that if you have simple congenital heart disease,
Speaker: you know, your life expectancy and your chances of having a cardiovascular death are no more increased than the general population.
Speaker: So I almost put those aside because they don't need specialty care, nor do I think most intensivists need to think much about it beyond just the, you know, like, huh, that's interesting.
Speaker: This guy had an ASD.
Speaker: But then when we start talking about some
Speaker: of the more what would be considered by the Bethesda criteria moderate or high complexity anatomic lesions.
Speaker: Those are the ones that really do have quite a bit of cardiovascular excess mortality and morbidity when they start getting into adulthood.
Speaker: And those are the ones that are going to need some very strong
Speaker: kind of, you know, out of the ordinary planned out care pathways that are not typical and are not something that's going to just come readily to your average critical care physician.
Speaker: So now we're talking about things like Tetralogy of Fallot, transposition of the great arteries.
Speaker: a variety of lesions that essentially end up in a fontan, which means you have a single ventricle, whether that's a right ventricle or left ventricle pumping, and then a variety of lesions that wind up with Eisenbanger's disease, which is essentially pulmonary hypertension taken to the extreme where you have systemic pulmonary pressures.
Speaker: And then finally, Eisenmanger's physiology and Eisenmanger's syndrome, which is a dysplastic tricuspid valve and atrialization of the right ventricle that then leads to lots of problems with RV failure as well as arrhythmias.
Speaker: So that list of lesions ends up
Speaker: having high rates of mortality in adulthood and a lot of very unique comorbidities and complications that your average intensivist is just not going to be used to taking care of.
Speaker: And obviously, as we were discussing earlier, one of the biggest problems with this population is that our adult intensivists like myself just don't know what they don't know, right?
Speaker: And you're treating somebody in their 40s and you are missing things that...
Speaker: are very common in these pathologies.
Speaker: The other thing that I know and maybe you can comment that's very unique about particularly complex congenital heart disease is that the way they are fixed keeps evolving and changing.
Speaker: So then you have all these pockets of patients surviving with different physiology, right?
Speaker: Yeah, absolutely.
Speaker: Absolutely.
Speaker: So, I mean, a great example of that that I think you're very familiar with is transposition of the great arteries.
Speaker: And so in the case of transposition, detransposition of the great arteries, so that particular form, the older surgeries, which are no longer performed or what are known as the sending or mustard shunts, which essentially are atrial switch operations.
Speaker: So
Speaker: Transposition is one where the great vessels, the aorta and the pulmonary artery are not aligned with the ventricles, you know, the right ventricle and the left ventricle.
Speaker: And the circulations are essentially running in parallel, but not connected and going the wrong way, meaning oxygen poor blood is staying on the right side and oxygen rich blood staying on the left side, but they're not communicating, right?
Speaker: And so you have to fix that.
Speaker: And the old way of fixing that was to switch the atrial flow of blood.
Speaker: and ultimately to deliver the blood that was going into the right atrium into the left ventricle and into the left atrium into the right ventricle, which then fixed the kind of parallel circuits, but created the issue that you had a systemic right ventricle, as well as this baffling between the atria that then was susceptible to lots of arrhythmias and clots.
Speaker: For those reasons and all the complications that resulted of those surgeries, now the standard of care has become what's called the arterial switch operation.
Speaker: So now you're switching the pulmonary artery and the aorta and re-implanting the coronaries.
Speaker: Those have their own core morbidities.
Speaker: They're very different than that of the atrial switch operations.
Speaker: if you did not take care of the atrial switch operations, and I'm 50 years old, and the reality is the atrial switch had already stopped by the time I went through fellowship.
Speaker: And so I know these folks only as adults with congenital heart disease.
Speaker: You know, these operations now, you're still going to encounter the patients because they have the disease, but the pediatricians aren't even dealing with
Speaker: it because it's not in front of them.
Speaker: So the only folks that are seeing them are going to be adult intensivists, but it's an operation that historically is no longer happening.
Speaker: And so I think the knowledge of what the complications and all is now becoming isolated to a very select group of people.
Speaker: And the biggest group in this particular disease process is adult congenital heart disease cardiologists, which there's about 570 in the country.
Speaker: So they exist, but it's not plentiful.
Speaker: And what are some of the ICU considerations for these patients with congenital heart disease that make it to the ICU as adults?
Speaker: Well, so there's a variety of issues that need to be dealt with.
Speaker: First of all, arrhythmias is a huge problem, getting up to almost 50% of patients with complex disease by the time they're getting into adulthood.
Speaker: Heart failure is a huge issue.
Speaker: And the arrhythmias and the heart failure don't necessarily look like what we're used to in structurally normal
Speaker: The arrhythmias are, you know, with structurally normal hearts, the most common thing we see on the suprasintricular level is atrial fibrillation and flutter.
Speaker: And that happens in congenital heart disease also.
Speaker: But there's a variety of other arrhythmias, things like intra-atrial re-entrant tachycardias.
Speaker: You have wolf-clurps and white and aberrant pathways.
Speaker: You've got a variety of atrial tachycardias that are from different foci.
Speaker: You have re-entrant circuits that are essentially happening because of scarring and
Speaker: and areas where there's suture lines and things that happen from multiple repairs.
Speaker: And those are much harder to deal with, both in terms of medication and also in terms of electrophysiologic ablations than the standard arrhythmias we have.
Speaker: The heart failure, which results in a lot of patients, is because they have a systemic right ventricle.
Speaker: So that right ventricle is trabeculated.
Speaker: It has two muscle layers instead of three.
Speaker: It's not
Speaker: dealing with the same morphology as what you dealt with in a left ventricle.
Speaker: And so how to handle that and what that ventricle is capable of is very different than what a systemic left ventricle is capable of.
Speaker: You know, when you start talking about considerations of advanced supports and things like putting an impella in or putting a fad in place, that's also very different when you have anatomic constraints such as a diminutive left ventricle or right ventricle
Speaker: common atria, things that really make it challenging to use the technology that we're using right now with our standard structurally normal hearts in this congenital population.
Speaker: So for all those reasons, you have to really give a lot of thought to the approach to these patients.
Speaker: And it's not straightforward and algorithmic.
Speaker: And a lot of what we have is our standard
Speaker: kind of algorithms and protocols for structurally normal hearts just don't apply or won't be very successful in terms of resolving the arrhythmias and the heart failure in these patients.
Speaker: And the last quick thing I'll add is just the issue of endocarditis.
Speaker: You know, these patients, by virtue of their multiple repairs and then the subsequent work that we do now with transcutaneous placement of valves and a variety of devices like AICDs and
Speaker: sequential pacing leads and things like that have a lot of prosthetic material in their body.
Speaker: So the risk of endocarditis is present and it's been growing because we're doing a lot of things such as valve and valve repairs that are creating turbulence and are setting them up for both for thrombosis and also for endocarditis.
Speaker: And so a lot of these patients fly under the radar and we're
Speaker: you know, intensivists will get them and not recognize that this is somebody who has indolent endocarditis.
Speaker: And instead you're treating their heart failure or their arrhythmia and not understanding why they keep having the trouble they're having.
Speaker: And another thing that I think is very important that you can comment on, Cameron, is the reliance on what we usually do for hemodynamic monitoring for our patients might not work.
Speaker: And also maybe our approach with mechanical ventilation needs to be rethought.
Speaker: These are things that we just bread and butter in the ICU, but maybe in these populations, if we're not really thinking about it, we might have a little bit of a misfit.
Speaker: Could you talk about that?
Speaker: Yeah, I mean, you're 100% correct.
Speaker: So let's start with the monitoring.
Speaker: You know, if you take somebody like a Fontan, their IVC and SVC ultimately are converging in their pulmonary arteries.
Speaker: So there's no way to put a standard PA catheter in.
Speaker: If you try to float a PA catheter, you're basically going to most likely go from one vena cava to the other one and not get anything in terms of a reading.
Speaker: At that point, if you're looking for a mixed venous oxygen saturation, the best you're going to do is land an IJ right down around the level of the Fontaine, which is basically where the pulmonary arteries connect to the shunt that, I mean, usually it's a shunt that is connecting the SVC and IVC together.
Speaker: And at that level, you'll get blood that is roughly mixed venous, but it's not 100%.
Speaker: And likewise, the pressures that you're getting there, which we would normally call a mixed venous, I'm sorry, a central venous pressure, that's actually a mean pulmonary pressure.
Speaker: because it's running right into the pulmonary arteries.
Speaker: So I think, you know, understanding that you have these, this very different anatomy, you have to realize that the monitoring is different for that reason.
Speaker: And you have to use very different strategies.
Speaker: From the perspective of
Speaker: Things like mechanical ventilation that you mentioned in the case of single ventricles, that's probably the most extreme case where you're dealing with now no right ventricle pumping blood through the lungs.
Speaker: So the blood flow through the lungs is completely passive.
Speaker: The central venous pressure essentially now has to drive or I should say the mean systemic pressure has to drive pressure that goes from
Speaker: pre-palmonary artery to post-palmonary capillary.
Speaker: And that pressure then has to end in a common atrium that's gonna be pumped by the systemic ventricle.
Speaker: That passive blood flow that's happening through the lungs means that anything that takes up intrathoracic pressure or alters pulmonary vascular resistance is gonna have huge consequences on blood flow.
Speaker: So just intubating and going onto positive pressure ventilation may actually drop your cardiac output dramatically because of the fact that you have risen
Speaker: the intrathoracic pressure and impeded the passive blood flow that's going through the lungs.
Speaker: So you have consequences on the physiology that are not at all straightforward and are not your straightforward heart-lung interactions that you expect when you ventilate somebody or put them on positive pressure with structurally normal biventricular heart.
Speaker: Perfect.
Speaker: And we can talk more about multidisciplinary approaches to these patients, but specifically for this population of congenital heart disease, you did mention there's 500-plus cardiologists who have expertise in adult congenital heart disease.
Speaker: Can you just talk about the importance of collaborating with them when these patients are in the ICU?
Speaker: Yeah.
Speaker: Yeah, I can't underestimate the importance of collaboration when it comes to dealing with with adult congenital heart disease and just generally speaking, congenital processes.
Speaker: You know, the the truth is, is that every one of these patients
Speaker: even if they fall into a specific group.
Speaker: So let's I've talked already about Fontans.
Speaker: Let's talk about Fontan physiology.
Speaker: A Fontan can be a patient who has a shunt running through their atrium or involving their atrium or external to their atrium.
Speaker: They can have a ventricle that's primarily right ventricle or it can be left ventricle or it can actually be a mix of right and left ventricle.
Speaker: They can have pulmonary veins that are all running back into their common atria or perhaps going to opposite atria or perhaps going into a confluence that drains into one of the atria.
Speaker: And so right there, I've already laid out 15 different permutations of what would all be called a fontan.
Speaker: And so you have to realize that they're all different.
Speaker: They're all unique.
Speaker: It makes it very hard to have a protocol, a pathway, and to just have a single approach to the patient.
Speaker: And so this is really one of those cases, I think more than almost anything else, where a multidisciplinary discussion and a thoughtful kind of planning before you start therapeutic interventions is called for.
Speaker: Because if you just launch into it thinking, oh, I've done a couple of Fanteans before, I can take care of this.
Speaker: you're in for some trouble because this Fontan may be very different than the last one you took care of.
Speaker: And I'll tell you that, you know, as somebody who's probably now cared for as many adults with congenital heart disease in an ICU setting as anyone in the world, I don't consider myself an expert.
Speaker: I think it's crucial to sit down with the cardiologists and with the surgeons and talk about every patient because of the heterogeneity and all the impacts that it's going to have on physiology and on their ultimate outcome.
Speaker: Excellent.
Speaker: Let's move to a different category, and maybe we can talk a little bit about genetic diseases and syndromes.
Speaker: And obviously, this is, I'm sure, a very big group of patients.
Speaker: But if you can give us maybe some examples of diseases that might present in the adult ICU, and then maybe we can go into some of the basic ICU considerations for this group.
Speaker: Yeah, great.
Speaker: So I think, you know, when we talk about genetic diseases and syndromes, we really are talking about a broad set of things.
Speaker: But let me try to break it down into a few groups.
Speaker: I think, first of all, you've got the known chromosomal abnormalities.
Speaker: So things like trisomy 21 being by far the most common.
Speaker: That's Down syndrome, as everyone knows it.
Speaker: But there's other trisomies, 18 and, you know,
Speaker: Not all of these trisomies make it into adulthood, but again, increasingly, we're getting successful at surviving them, right?
Speaker: So a trisomy, as one would assume with having an extra chromosome, it doesn't just give you one genetic defect or one thing wrong.
Speaker: Ultimately, there's multiple things that are different than kind of normal.
Speaker: So taking trisomy 21, because it's by far the most common, if you're caring for a Down syndrome patient, you're going to have to worry about atlantoaxial instability.
Speaker: You're going to worry about their thyroid.
Speaker: There's a very good chance they have congenital heart disease.
Speaker: They're going to have early dementia, such that a 50-year-old is like an 80-year-old from a brain perspective.
Speaker: They're going to have some level of immunocompromise where they're much more susceptible to have pneumonia and certain other infectious illnesses.
Speaker: So all of those things,
Speaker: are the expectation of down syndrome and down syndrome is really common actually um and so i think just being prepared for how these patients are different and what the unique issues are is going to be critical moving aside from kind of chromosomal abnormalities there's a bunch of syndromes that are ultimately due to genetic defects but we're only starting to understand what those
Speaker: genetic defects are.
Speaker: And many of them are not a single gene defect, but there might be, you know, small chromosomal deletions or translocations or things like that.
Speaker: And those syndromes, again, very much like the trisomies, have multiple facets, often involving the brain, often involving the heart, maybe involving skeletal features and things like that.
Speaker: One of the things that I think gets underestimated, for example, is if you have severe scoliosis, which is not uncommon with the syndrome in kids, you have a form of restrictive lung disease where essentially one lung is squashed by your scoliosis and there's only one lung that's effectively ventilating and doing all the oxygenation.
Speaker: So if you get pneumonia in that one lung, you got a problem, a much bigger problem.
Speaker: problem than you would in most normal people.
Speaker: There's also limits to what's reasonable in terms of breathing that you can expect and what their FEV1 is going to be and their FRC and things like that.
Speaker: You know, when we then go to other a little bit more straightforward genetic diseases, things like Duchenne's muscular dystrophy or Becker's muscular dystrophy, these are patients who are going to have lots of problems in the case of the Duchenne's population,
Speaker: with progressive muscle weakness leading to respiratory failure and then progressive cardiomyopathy leading to heart failure.
Speaker: And although most of them die from the respiratory, there's a good chunk of them that die from their heart.
Speaker: And so you really have to have an understanding of what those processes are and also a lot of heart-to-hearts with the patient about where their values are and what they want for goals of care.
Speaker: Then there's others such as Marfan syndromes or Ehlers-Danlos, which are collagen vascular diseases, where we're dealing with things like
Speaker: repeated coarctations, I'm sorry, not coarctations, aneurysms of the aorta and problems with their particular aortic valve, which then leads to multiple reoperations.
Speaker: So all of these have various comorbidities.
Speaker: I think it gets more complex when you're dealing with things on a chromosomal level than a single gene defect.
Speaker: But you have to understand what the natural history of disease is for these different genetic syndromes.
Speaker: and then be able to deal with the various comorbidities that come with it.
Speaker: So obviously, a lot of diseases, like you said, with specific issues, but it sounds like in terms of ICU considerations, again, going back to airway challenges with these patients that we should be aware of, right, not just walk into these intubations kind of with a laissez-faire kind of attitude and respiratory issues in terms of how we support them and complications they could have.
Speaker: And then, like you mentioned, cardiovascular complications, which are much more common in this population than maybe some people are
Speaker: think of.
Speaker: Yeah, and I think you said it really well there, Sergio, because it's don't have the laissez-faire attitude.
Speaker: And this is hard, right?
Speaker: Because I think as intensivists, once you've got a lot of experience under your belt, you have a way you handle things, right?
Speaker: A way you handle shock and respiratory failure.
Speaker: And, you know, generally speaking, the approaches that you've learned over years and over, you know, dozens and hundreds of patients are
Speaker: 100% right.
Speaker: You know, I mean, those work and they're efficient and effective and, you know, get things under control.
Speaker: But if you try to apply those same principles to somebody who has a complex genetic disease or adults congenital heart disease, you'll quickly find out that their unique comorbidities, the fact that their C-spine is not stable or they have macroglossia or microanathia or, you know, a variety of other illnesses means you get into airway issues.
Speaker: And before you know it, you have a coat on your hands.
Speaker: And that's, you know, that's the situation nobody wants to be in.
Speaker: So, you know, the biggest thing I would say is these patients require forethought a lot more than most of our ICU patients.
Speaker: And I'm not saying that we don't we don't think I think as intensivists, we think an awful lot in caring for our patients, but I don't think we pre plan on most.
Speaker: of them because the nature of ICU care is that a patient gets sent to us kind of in extremis and we very quickly deal with it, right?
Speaker: And this is one of those situations where short of the things that are life-threatening, I think what we need to do is take a minute and kind of think and plan before acting, which I don't know is always, you know, the usual routine in an ICU setting.
Speaker: Excellent.
Speaker: And the last category that we mentioned that I want to hear a little bit more about, Cameron, is prematurity-related conditions in adults.
Speaker: Now, I would imagine that every five years, I don't know, every five years, 10 years, whatever, the...
Speaker: The number of weeks that are survivable preterm keeps increasing, right?
Speaker: Seems that way, right?
Speaker: And we are seeing things that maybe, I mean, 20 years ago were not something that we would imagine possible.
Speaker: But like you said, these premature newborns are surviving longer and longer, and there might be some consequences.
Speaker: So how do you think that's going to be a little bit?
Speaker: Tell us a little bit about, I mean, this is an area that I really have no, no, no experience with.
Speaker: And I'm just curious in terms of where we are today and how you think about it as a pediatric and adult critical care specialist.
Speaker: Yeah, well, I mean, I think what you said is 100% accurate.
Speaker: I mean, when I was going through residency, you know, we were pushing the limits talking about 25 weekers and, you know, roughly under a kilo and a half was considered to be really low birth weight, right?
Speaker: Now, there, I think, you know, the current discussions and I'm not a neonatologist, so I might be wrong on this, but I think the current discussions are about 23 week viability and 500 grams as very low birth weight.
Speaker: So we're really, we've pushed the extremes of what is survivable.
Speaker: And unfortunately, when a child is born, you know, weighing a pound and, you know, being 23 weeks of gestation, so about half of what's normal, there's a whole lot of complications that often occur within those first couple months of life that
Speaker: go on.
Speaker: I think breaking it into some of the common characteristics, chronic lung disease owing to prematurity and lack of surfactant, which then manifests itself as pretty significant pulmonary hypertension as they become older.
Speaker: The issues that will happen with immature brain circulation, in particular with intraventricular hemorrhage and germinal matrix hemorrhages, which then can leave them neurologically quite devastated or having things like cerebral palsy or other kind of chronic diseases.
Speaker: motor and sometimes cognitive.
Speaker: They don't always go together, dysfunctions moving into life.
Speaker: And, you know, I think those types of illnesses, because of their severity, will then lead to things like being chronically trached, vented, G-tube fed, problems with swallowing.
Speaker: And not all of these problems are always because they're incapable of swallow, but children will actually develop significant symptoms
Speaker: dysphagias that are as much behavioral and because of the complications they had as a child as they are actually physical and, you know, based on muscle and nerve.
Speaker: And so then dealing with those adults who have the lingering consequences of that from their childhood is challenging because, again, it's a very difficult
Speaker: And I think for us to deal with a 20 year old in the ICU who has severe pulmonary hypertension from lung disease and potentially is neurologically normal in the terms of cognition, but has spastic quadriplegia as a result of some of their intraventricular hemorrhages when they're a child.
Speaker: But that's the type of comorbidities that are not uncommon coming out of extreme prematurity.
Speaker: Excellent.
Speaker: And we discussed, obviously, I mean, different categories of disease and some specific considerations.
Speaker: Ultimately, I think the real goal of our conversation today, Cameron, is to bring awareness to the critical care adult community, right, of this growing number of patients and particular differences that their management might require.
Speaker: And we can't cover everything.
Speaker: Like you said, I mean, even yourself who sees this on a regular basis feels that obviously the expertise is hard to grasp.
Speaker: But what about what I would like to do now is maybe talk more general of ICU management and maybe of congenital disease in the adult ICU and talk about pitfalls to avoid and maybe some pearls of management that you can share with us.
Speaker: And going over more of like a systems approach of different areas and start maybe with physiology and recognizing the unique physiology and what is normal for these patients, right?
Speaker: Right.
Speaker: Yeah, absolutely.
Speaker: So, so, um, gosh, very broad, right?
Speaker: So, uh, let, but let's, let's start with kind of in adult congenital heart disease with, um, some of our single ventricles, um, and what is unique about them.
Speaker: So I touched upon the fact that in single ventricle physiology, you, you,
Speaker: have some very unique heart-lung interactions because you have passive blood flow through the lungs.
Speaker: In addition to that, the actual mechanics of the blood flow are quite different in that the IVC and the SVC, which ultimately connect to the pulmonary arteries,
Speaker: the IVC often doing so through some kind of grafting or shunt, they don't always feed the pulmonary arteries to the same degree.
Speaker: So it's not like the SVC blood flow and the IVC blood flow end up 50-50 to the left and right PA.
Speaker: Often there are asymmetries, and those asymmetries may be as much related to the directionality and the positioning of the attachments between the SVC and IVC with the pulmonary arteries as they are by
Speaker: potential stenosis within the pulmonary arteries, both proximally and distally.
Speaker: And, uh, and so you have, um, individuals who actually have differential pulmonary blood flow for their entire life where, uh, most of their, um, um,
Speaker: Pulmonary blood flow goes more to the right or to the left.
Speaker: And so then obviously having a pulmonary embolism, depending on whether you get it on the majority side or the minority side, has a very different impact on them.
Speaker: But also what that means is for the diagnosis of pulmonary embolism, you got to be really careful because if you do what we usually do, which is to give an IV contrast bolus into an arm IV and let it circulate through and shoot your CT scan to try to diagnose a PE, what's going to happen in most cases with these Fontans is
Speaker: is that that SVC, which is going to accept that contrast load, is going to connect to one PA more than the other.
Speaker: So you're going to end up shunting most of your contrast load through your, say, right PA and not through the left PA.
Speaker: And that's going to get read as a massive left PE.
Speaker: And I've had probably a dozen referrals for massive PE, and actually none of them have been pulmonary embolism.
Speaker: They've all been an artifact of the contrast bolus in reading.
Speaker: And it's not surprising because it's not something that the radiologist expects, right?
Speaker: But in that situation, you either need to do a delayed phase study where you give time for an entire circulation of the contrast load to go through the heart and then get pumped out and come back onto both IVC and S.
Speaker: or you need to put IVs both in the upper and lower extremity and be able to time your contrast bolus on both of them, which is not something that a non-congenital center is going to have as a protocol.
Speaker: So it really doesn't happen in most of the world.
Speaker: So that's just one example.
Speaker: The, you know, I think that once you get past that, there's the issue of in congenital heart disease, what is the normal sat?
Speaker: So many of the diseases in congenital heart disease either have residual shunts or the formation of abnormal collaterals.
Speaker: So take the Fontan group.
Speaker: There's a lot of aural pulmonary collaterals, and there's also veno-venous collaterals, where it's essentially a systemic vein, often from the lower extremity that's experiencing high
Speaker: venous pressures that then anomalously forms a collateral to a pulmonary vein.
Speaker: So that's supposed to be an oxygen-rich vein because it's post-pulmonary capillary.
Speaker: So what that equates to is oxygen-poor blood getting shunted into the oxygen-rich blood.
Speaker: And so these patients...
Speaker: normal saturation declines often with age.
Speaker: And so what you'll have is a Fontaine who's 30 years old, who on their best day is going to have a SATA 82.
Speaker: And the patient can often tell you what their baseline is.
Speaker: But if you don't ask and you just look at their SAT, you're going to think they're hypoxic when actually they're at their baseline, which is their day-to-day for the last five years.
Speaker: And so it's really important to be aware of what that baseline is and what's driving it, because things like intubating them and giving them 100% oxygen not only won't help them, but by virtue of compromising the problem of blood flow is likely to hurt them and put them in a worse space.
Speaker: So it's really important to understand the norms and the physiology.
Speaker: And this is something, I mean, we could go on for a long time about this, but hopefully that just that one description gives folks who are listening an idea of some of the
Speaker: and some of the features they need to think about.
Speaker: You know, I want to get back to something you said right at the beginning of this section, which is, you know, it's important that we're talking about it.
Speaker: And I think the only way that we as a field are going to be able to handle the influx of these patients and really give them great care is that we continue to talk about it.
Speaker: And those conversations really involve multidisciplinary conversations between not only patients
Speaker: different adult specialists, but it's actually reaching out between adults and peds.
Speaker: And that can be really hard because as you know, there's a whole lot more adult ICUs and adult medical centers than there are pediatric ones.
Speaker: So I don't know the statistics.
Speaker: You probably know this.
Speaker: The number of adult ICUs, how many of them are in a med center that also has a pediatric ICU?
Speaker: I would guess it's somewhere on the order of 10%, 15%, which means that most adult practitioners don't actually have a pediatric intensivist in their house that they can turn to and say, hey, give me some advice about this process.
Speaker: And that right there is actually a big problem.
Speaker: I agree.
Speaker: And I think that what a lot of pediatric beds get, that get counted are really neonatal beds, which is a different story again, right?
Speaker: Yes, very much so.
Speaker: Yeah.
Speaker: And the neonatologists don't have experience with, outside of prematurity, don't have experience with a lot of this.
Speaker: I agree.
Speaker: Yeah.
Speaker: And so what about the one thing I wanted to ask you here before we move on to airway and ventilation considerations is we like to give a lot of volume in the ICU and that pendulum keeps swinging back and forth with sepsis and we're learning.
Speaker: And despite being one of the most common things we do in the ICU, we still haven't figured it out.
Speaker: But in particular, I think we have to be very careful with some of these patients, right?
Speaker: You do.
Speaker: You know, I think the amount of right heart failure that I deal with in congenitals, adult congenitals, is just so much more common than what I'm dealing with in structurally normal hearts.
Speaker: And it's driven by things that include chronic shunts that for the most part are going left to right.
Speaker: So what you're doing there is you're essentially overloading the right because a portion of the left heart blood is being pumped back into the right.
Speaker: Okay, so if you have an ASD or a VSD, that's essentially what happens, or anomalous pulmonary venous return that dumps into the right side.
Speaker: You're basically taking a portion of what should be blood pumped on the left side and bringing it back to the right.
Speaker: And so the result of that is the right side is volume overloaded.
Speaker: Now, the right heart, as we know, is a more compliant ventricle and it's able to handle higher degrees of volume load and, you know, can continue to pump that without getting into trouble.
Speaker: But there's a limit to that.
Speaker: And if you're chronically overloaded and then all of a sudden you bolus a bunch of fluid, the chances of setting that patient at the right heart failure are substantial.
Speaker: And this is particularly common in some of the Fontans where they don't even have an RV.
Speaker: You're dealing with passive flow and they always have high CVPs and very high EDPs.
Speaker: within their systemic ventricle.
Speaker: This is also common in things such as Eisenmenger's syndrome for a variety of causes and with Epstein's anomaly, because in Epstein's, you've already got an abnormal RV, which often is failing, and a very leaky tricuspid valve, which results in overload of that RV.
Speaker: So in these cases, you quickly get to RV failure if you give too much fluid.
Speaker: That'll be manifested by what looks like liver failure because they already have congested livers and now you fill them even more and quickly the AST-ALT will get into the many hundreds and almost look like an infarct.
Speaker: but it's not.
Speaker: It's just that you've congested the liver.
Speaker: And then the very unique thing about congenitals that you don't see very often in other processes is they can have lymphatic abnormalities.
Speaker: And so these lymphatic abnormalities are really...
Speaker: abnormal lymphatic return into the venous circulation.
Speaker: And normally the thoracic duct dumps into the asagus.
Speaker: And so if you have venous hypertension, you impede lymphatic return as it is.
Speaker: But now if you have abnormal lymphatic connections that may be empty into the pleural space or into the gut, which is what you see over the bronchioles, which is what you see with congenital heart disease,
Speaker: Now, all of a sudden, the lymph starts pouring into their pleural space and they get chylos effusions or dumps into their lungs and they develop plastic bronchitis or dumps into their GI tract and they've got protein losing neuropathy.
Speaker: So these are problems that you rarely deal with when you're dealing with non-congenital patients.
Speaker: And these are exacerbated by being an RV failure or having high venous pressures.
Speaker: Like you said, I mean, a lot of things to consider that can be very nasty surprises if we don't pre-plan.
Speaker: Now, as we move to the next topic, airway and ventilation considerations, I'll share with you very briefly a nightmare experience I had some years ago.
Speaker: I was doing a night call at a large community hospital here in Houston, and there was a 20-something-year-old, late 20s, who got transferred from an outside hospital with what was labeled a STEMI.
Speaker: So it was taken directly to the cath lab, and then they call a code, and they call me emergently to the cath lab because I needed to intubate this patient.
Speaker: When I get there, they tell me he has some congenital issue.
Speaker: That's all he said, the cardiologist.
Speaker: Yeah, right.
Speaker: It was a horrible experience, bad outcome, and the patient had Pierre Robin sequence.
Speaker: And I was trying to intubate this poor patient in a cath lab without support.
Speaker: Anesthesia came.
Speaker: They couldn't do it.
Speaker: It was a disaster.
Speaker: Yeah, I'm sorry about the difficult airway management and how I mean, we I mean, I think our anesthesia colleagues are always very cautious because they, I think, see a lot more airways than we do.
Speaker: And we always get called in emergencies.
Speaker: And I think.
Speaker: And the nature of practice is that by the time you get too cocky, you get humbled, right?
Speaker: So you get, you get, you get, you bring down a couple of notches, but I think it's always important to understand in particular with this population, you can be in for a lot of surprises.
Speaker: Yeah, no, I think you're right.
Speaker: I mean, just hearing your experience, like, you know,
Speaker: gives me chills because I completely understand, you know, I mean, I've dealt with this before and it's, you know, I've dealt with it knowing what all the issues are.
Speaker: So I can only imagine being surprised by it.
Speaker: Right.
Speaker: So Pierre Robin syndrome, which your sequence that you're talking about is a combination of macroclossia, microdaphymia.
Speaker: And so what you're dealing with is a very small
Speaker: jaw and a very oversized tongue.
Speaker: And so as a result, they effectively have complete obstruction of their mouth so that you can't really ventilate them through the mouth.
Speaker: And so now you've got the nose, but often in these situations, you also get coenal atresia, which means that their nasal passages are narrowed or perhaps even completely stenotic.
Speaker: So now you don't have an airway.
Speaker: when you're dealing with somebody who is not conscious and able to keep their mouth open and breathe.
Speaker: You know, you may not recognize it at all, but for a lot of patients who have Pierroban syndrome, when you look at them, at their face, you can tell that
Speaker: something looks a little funny, right?
Speaker: And it's a little hard to necessarily put words on it, but what it is is that their jaw, their bottom, you know, their chin is just too small for the rest of their face.
Speaker: And that's the sign of microanathia, which then, you know, you have to look in their mouth to see they have macroglossia, but that's essentially what brings together the sequence.
Speaker: So these patients are going to need an oral airway introduced very quickly after you induce anesthesia, and still they may be very difficult to bag.
Speaker: And if you can't bag them effectively and because of the kind of short distance to...
Speaker: their larynx, getting a view of their airway may be impossible by standard techniques.
Speaker: And so you may need something like bronchoscopic guidance to be able to intubate.
Speaker: And sometimes you have to move to trach emergently.
Speaker: So it's a lot of challenges.
Speaker: And Pierre Robbin is probably the most extreme, but there are variants of that in all of the other, excuse me,
Speaker: in all of the other genetic diseases that I got to.
Speaker: So for example, Down syndrome kids have huge tongues.
Speaker: I think everybody who's seen a Down syndrome kiddo is used to kind of the tongue sticking out of their mouth.
Speaker: It's not that they love sticking their tongues out of the mouth.
Speaker: It's that their tongues are oversized relative to their oropharynx.
Speaker: And so that's a form of obstruction, especially when you're dealing with somebody who's sick or not fully conscious and not able to regulate their muscle tone the way they normally would.
Speaker: And they're
Speaker: and their ability to swallow.
Speaker: And so kind of preparing for that obstruction, having available adjuncts like oral airways, nasal airways, bronchoscopic guidance, those are all going to be things that are really crucial, as well as quickly calling for support from anesthesia and sometimes from ENT.
Speaker: Because like I said, in some cases, it may be an airway that's just not obtainable and you're going to trach.
Speaker: The other thing I wanted to ask you about in this kind of category is we use a lot of non-invasive ventilation more and more, I think, especially post-COVID and with all the literature coming out.
Speaker: Any particular concerns with particular subgroups of patients with the use of non-invasive ventilation?
Speaker: Yeah, I think they're the only one that, you know, I think is as a class, a consideration is that, again, when you're dealing with Fontans, non-invasive ventilation is in effect going from negative to positive pressure ventilation, although the impact on intrathoracic pressure is not nearly that of intubating and putting them on a mechanical ventilation.
Speaker: But nonetheless, that positive pressure really can result in issues with impeding polypathy.
Speaker: blood flow.
Speaker: And likewise, if that even if they have biventricular physiology, but they have Eisenmangers, so they have essentially systemic RV pressures, putting them on positive pressure may be that little bit of extra that tilts them into not having any flow from their right side to their left and having a cardiac arrest.
Speaker: And so I think you have to be really thoughtful when you go on positive pressure for those reasons.
Speaker: I'd like to talk a little bit about hemodynamic monitoring and support.
Speaker: We did mention some aspects of this in the adult congenital discussion, but just a couple more things to talk about, Cameron.
Speaker: Blood pressure measurements, can you talk a little bit about that?
Speaker: Yeah, I mean, first of all, when you're dealing with the adult congenital population, you have to recognize that some of the surgeries that they have potentially have affected things like their subclavian vessels, because we used to use those for things like blood lactose chunks.
Speaker: And so if you put a radio art line in on a side where the subclavian is compromised, you're obviously not going to get an accurate reflection of aortic blood pressure.
Speaker: You also can have issues with coarctation, particularly at the level of the ductus arteriosus, because that's where most coarcts form, but it can happen anywhere else through their blood.
Speaker: circulation.
Speaker: When you're dealing with some of the mutations like Marfan's, Ehlers-Danlos, actitude mutations, things like that, the propensity for a dissection can make it so that, again, putting in an arterial line on a
Speaker: radial versus ephemeral can give you a very different number because you've got essentially a stenosis or a dissection and you're measuring blood pressure through a false lumen.
Speaker: And so all of those are things that impact inflammation.
Speaker: how reflective your numbers are.
Speaker: And so sometimes we're forced to place multiple arterial lines because we care about, I mean, in the end, the arterial line down below often will reflect perfusion to things like the gut and the kidneys, whereas the arterial line up top
Speaker: is gonna and when I say up top I mean like right radial is gonna reflect brain and coronary perfusion and both are important right and so there there are definitely patients where we have two a lines in because we need to be monitoring perfusion pressures on both sides the other thing are the SATs related to that right so when you have a
Speaker: for example, a ductus arteriosus that's patent, that will happen roughly at the level of the left subplavian.
Speaker: And so if you're measuring the sat on the right finger and you compare that to something in either of the legs, either toe, you may see very different saturations because you have shunting of blood.
Speaker: And, you know, this would have to be a patient who has progressed to Eisenmangers.
Speaker: But if they're having right to left shunting and essentially oxygen poor blood is shunting across the ductus into systemic circulation, then in that situation, the right finger sat is going to be considerably higher than the left toe sat.
Speaker: And so it's just being aware of the fact that they are different.
Speaker: And again, they reflect the oxygen delivery to different parts of the body.
Speaker: So it just depends how concerned you are about that region of the body.
Speaker: Your monitoring should be appropriate to reflect that.
Speaker: Excellent.
Speaker: And we talked about hemodynamic monitoring.
Speaker: You gave examples of the difficulties of placing PA catheters in some pathologies.
Speaker: Can you talk about the importance of echocardiography?
Speaker: Yeah, I mean, I think, you know, echo, point of care ultrasound in general is huge, right, and so helpful.
Speaker: But when you're dealing with critical care echocardiography and congenitals, it gets confusing really quickly.
Speaker: You know, the
Speaker: The most extreme example are going to be your patients who have things like dextrocardia and transition where literally everything's flipped.
Speaker: And, you know, if you're trying to get an apical four chamber on the left, you're going to quickly realize there's only lung there, you know, and so...
Speaker: What the windows are and being able to interpret, you know, what you're looking at in terms of the ventricular pictures really takes some expertise.
Speaker: And I think this is where partnering with an adult congenital cardiologist becomes really critical because they're going to be able to guide you through the echo and tell you what everything looks like.
Speaker: And so it does hamper the ability to get point of care echo, where I think a lot of us working in cardiac setting are doing that routinely on structurally normal hearts because we kind of have a few views where we have imaged so many times that we're comfortable making.
Speaker: making diagnoses like effusions or what the function is.
Speaker: But, you know, diagnosing the function on somebody whose ventricular function ultimately is kind of a combination of the RV and the LV and their transpose is not straightforward at all.
Speaker: Excellent.
Speaker: What are some other anatomic differences that might impact care in general ICU care for this population?
Speaker: Yeah, I mean, I alluded to a few of them.
Speaker: I mean, a lot of these genetic or syndromic children have severe sclerosis.
Speaker: And with that, restrictive lung disease, there's a lot of contractures in the extremities from things like cerebral palsy.
Speaker: And so that makes it very challenging to get, you know, it.
Speaker: atrial, sorry, arterial lines in certain places and PICC lines, things that we use very commonly.
Speaker: And then the lymphatic anomalies that we talked about, particularly seen in the single ventricle population, but really within, throughout congenital heart disease, there's a higher incidence of lymphatic anomalies.
Speaker: And that can result in things like plastic bronchitis and protein losing enteropathy, which are pretty rare to see in structurally normal hearts.
Speaker: And we talked about extensively, I think, about the important multidisciplinary collaboration and consulting specialists early.
Speaker: I mean, obviously, if you don't have access to adult congenital heart disease experts, getting your genetic colleagues involved, getting your pediatric colleagues involved.
Speaker: And when it's complicated, probably trying to refer patients to the right center where they have that expertise, I think, is very important for us in terms of trying to get the best outcomes from these patients.
Speaker: But I think that this is a perfect segue into the last portion of our discussion, which is navigating goals of care to conversations.
Speaker: And this is something that we talked a little bit about before we were recording offline.
Speaker: And I know that a lot of times when adults see some of these patients, there's an immediate nihilist reaction, like, what are we doing here?
Speaker: But that might be very different from the perspective of a pediatric patient and their family who has grown for decades with basically chronic critical illness.
Speaker: Can you talk a little bit about how you approach goals of conversations and maybe give us some pearls on how to do this a little bit better with this patient population?
Speaker: Yeah, and I'm so glad we're talking about this.
Speaker: I mean, just like you said, for parents who have raised children who have chronic illness, so let's take the example of a child who, for example, has a trisomy or some kind of other syndrome.
Speaker: And let's say they're chronically trached, vented, G-tube fed, spastic quadriplegia,
Speaker: may be limited in terms of verbalization.
Speaker: They may be completely non-vocal, right?
Speaker: Over the course of 20 years, which is typically, you know, how old the patient is by the time they show up in adult care, that parent has learned what is happy, sad, uncomfortable, you know, and they've found an interaction with their child that there's no way you're going to pick up on that in two minutes or even an hour.
Speaker: And so,
Speaker: you really need that parent to tell you what is quality of life, what is kind of a good or bad day, happy or sad, in pain or not in pain.
Speaker: And if you break that therapeutic alliance between yourself and the parent, you're really going to be treating that patient out of complete ignorance and not getting good outcomes as a result of it.
Speaker: And
Speaker: One of the biggest things that I see break that therapeutic alliance is that an adult intensivist walks into this room, sees this contractured, trached, vented, you know,
Speaker: patient laying there and just says, what the heck are we doing?
Speaker: Let's just, just DNR, DNI and stop.
Speaker: Right.
Speaker: And, you know, like I get it.
Speaker: Um, I I've taken care of enough chronically ill patients to understand where that comes from.
Speaker: And perhaps if this was somebody who was 65 and demented and all that, um, you know, that would be a very realistic conversation to have about, you know, should we talk about hospice?
Speaker: But when you're talking about somebody who is 20, um,
Speaker: That's just not something that that family's ready to even contemplate.
Speaker: And the reality is, is as they've been going in and out of health care on the pediatric side, they probably have an admission into the ICU once every five, you know, six, seven years.
Speaker: And then they have many years at home of what they consider to be excellent quality.
Speaker: Right.
Speaker: And if you don't recognize that or know that about this patient, you're, you
Speaker: kind of quick two minute overlook and diagnosis that this is a terminal condition and we need to progress to hospice is going to poison any chance you have of getting through to this family and to this patient.
Speaker: And so I think it's just really important when you're dealing with somebody who has chronic illness like this to understand what their history has been, understand what their baseline is, define what is good quality of life and what are the expectations.
Speaker: And then once you understand all that, then you can start making decisions.
Speaker: I'm not saying that withdrawal of life-standing therapy doesn't make sense in some cases.
Speaker: It does.
Speaker: But what I'm saying is you can't just look at the patient and have an idea of what is normal and what is their baseline without having a very deep conversation with the person that's been with them forever.
Speaker: And again, because a lot of these patients are coming from a pediatric center, often the electronic medical records don't carry over.
Speaker: I mean, even at my own center, I'm at Baylor right now.
Speaker: Before this, I was at the University of Pittsburgh.
Speaker: It's crazy, but we had at Pittsburgh Cerner, and here we have Epic.
Speaker: But the peds and the adult hospitals use completely different builds.
Speaker: And so they don't talk to each other.
Speaker: So my login on the adult side didn't get me the records from the peds side and vice versa.
Speaker: Meaning that if you're the adult intensivist, you don't really have the opportunity to go through the medical records from two decades of pediatric history and understand what was the background.
Speaker: And so you need the family member to fill you in on that so that you can understand what the baseline is.
Speaker: And then go from there.
Speaker: And, you know, sometimes this also means talking to the pediatrician who took care of them for the last 20 years and maybe still is considered their primary care physician.
Speaker: And so having those discussions, I think, is is really important.
Speaker: One other thing I just want to throw in before we move on, Sergio, is you talked about the
Speaker: the concept of kind of transferring care if you don't have the multidisciplinary collaboration that you need.
Speaker: And I think that's something that we don't think about a lot.
Speaker: You know, generally speaking, when folks transfer care, it's often because of some capability, some technology that their center doesn't have, right?
Speaker: So, you know, I don't have ECMO.
Speaker: I'm going to send this patient to an ECMO place.
Speaker: I don't
Speaker: do transplants, heart transplants, lung transplants, whatever.
Speaker: I'm going to send them to a transplant center, right?
Speaker: Here in the case of some of these congenital cases, it really becomes, I don't have the expertise of individuals who deal with this chronic illness.
Speaker: Let me get them to a place that has that.
Speaker: And I think that's every bit as valid a reason to transfer a patient as not having a piece of equipment or a particular program like transplant.
Speaker: I agree.
Speaker: And I think it's about ownership, right, of really being responsible for providing our patients with the best possible outcomes and recognizing the limitations that we have.
Speaker: But however, I think this is an important conversation, Cameron, because I'm sure that there are some intensivists who don't have that luxury of transferring to a place that has the expertise, right?
Speaker: There are plenty of places where you just have to take care of these patients and doing it the best way you can is going to be very important.
Speaker: Now,
Speaker: Regarding the goals of care conversations, I think that it's also a very important lesson for all patients, right?
Speaker: Trying to provide patient goal concordant care is about curiosity, asking what's important for our patients, not telling them what we think should be important for them.
Speaker: Yeah, you're so right.
Speaker: I think a lot of times we all fall into that.
Speaker: We all assume that I would never want that, but we don't know what they would want.
Speaker: And we don't know what we would want if we were in their shoes.
Speaker: So asking those questions and understanding more about what a good day looks like for this patient, what has the last 15 years looked like is extremely important.
Speaker: The other part of this topic that I think is very important
Speaker: is being able to differentiate between reversible and irreversible decompensation.
Speaker: I think we might see something that maybe people with expertise have treated a hundred times and got better, and we think, oh my God, this is the end of it.
Speaker: Can you talk a little bit about that?
Speaker: Yeah, no, I think you're right.
Speaker: As I've been talking about all of this, I think if somebody who doesn't deal with
Speaker: might be kind of horrified like oh my gosh I gotta deal with this and this and this and so when you're when you're handling all of these essentially chronic overlays it can seem like a hopeless case right like a just someone who's so sick that they can't possibly get along and yet
Speaker: they can, right?
Speaker: In fact, they have been for, you know, many years.
Speaker: And so I do think that understanding, you know, recognizing what is the natural history of disease and, you know, the fact that there can be quality and longevity to patients who have actually quite a bit of disease burden.
Speaker: And it's surprising if
Speaker: if you just haven't dealt with that particular process.
Speaker: But fortunately, there are people often who have that expertise and kind of can help you.
Speaker: And like I said, sometimes that person is just the family member, right?
Speaker: The parent in most cases who has been dealing with that since their child was born.
Speaker: And so they know firsthand what's realistic and what's not because they've had so many conversations about it.
Speaker: And so I do think that that's really important to kind of establish and to not conclude that the chronic,
Speaker: illness equals acute or irreversible disease, which then condemns them to no quality of life.
Speaker: Excellent.
Speaker: I think we've had a wonderful discussion.
Speaker: And like you mentioned earlier, Cameron, this is a topic that could go on for hours and hours.
Speaker: So I think that this is a perfect place to stop the clinical discussion.
Speaker: We like to close the podcast with a couple of questions that are unrelated to the clinical topic.
Speaker: Would that be okay?
Speaker: Absolutely.
Speaker: So the first question is about books.
Speaker: And have there been a book or books that have influenced you significantly or a book that you have often gifted to other people?
Speaker: Yeah, so I mean, I thought about this and, you know, there's a lot of books I love.
Speaker: But I have to be honest with you, the book that I've read, the only book I've ever read eight now going on nine times is the Bible.
Speaker: The only book I have read
Speaker: given out to, you know, a hundred people or more is the Bible.
Speaker: So yeah, it's the book that's influencing me the most.
Speaker: I mean, it's, it's,
Speaker: book that I read every single day of my life.
Speaker: So definitely it.
Speaker: And I would say that without going deep into religious discussions, I have read many holy books from different religions.
Speaker: I mean, not the whole thing, but I've ventured in reading different things.
Speaker: And I think there's value and learning in all these traditions, right?
Speaker: And I agree.
Speaker: I mean, I think that at the end of the day, the idea of this portion is to get to know people a little bit more as individuals, right?
Speaker: And this is obviously an important part of who you are.
Speaker: So thanks for sharing that.
Speaker: For sure.
Speaker: The second question is, could you share something you changed your mind about over the last couple of years?
Speaker: Yeah.
Speaker: So, and, you know, when we say business,
Speaker: over the last couple of years is probably more like over the last 15 years.
Speaker: Um, but, uh, you know, I have to say that, you know, growing up as a, as a man and, you know, within kind of, uh, you know, I was in a fraternity in college and, you know, I played sports and a lot of locker room stuff.
Speaker: And, um, I always considered that,
Speaker: things like pornography and, you know, sleeping around, you know, and even prostitution to be essentially like victimless crimes.
Speaker: Right.
Speaker: And I just thought to myself, like, you know, who cares?
Speaker: It's consensual.
Speaker: It's between two adults, you know, that nobody's business.
Speaker: Right.
Speaker: And I've definitely changed my mind about that.
Speaker: I think some of it is my religious inclinations, you know, which came on later in adulthood.
Speaker: But I think more than that,
Speaker: It's kind of grasping what's going on in the, particularly in the porn industry with human trafficking and for that matter, prostitution with human trafficking and what has happened to women across the world that has just horrified me and, you know, made me very regretful of my attitude.
Speaker: And I think that the reason why those things happen is because there's a market, right?
Speaker: There is a complicit population that is fostering that.
Speaker: And I think it's definitely worth, I mean, deep reflection.
Speaker: So absolutely.
Speaker: Well, and having been part of that complicit, you know, population, let me say that I did it without thinking about it.
Speaker: Right.
Speaker: Meaning like I didn't think through what was, what was all this leading to.
Speaker: Right.
Speaker: And so that's, that's why I've changed my mind so radically is because now I've kind of seen some of that.
Speaker: Excellent.
Speaker: And the last question is, what would you want every person listening to our podcast today to know?
Speaker: Could be a quote or fact or just a departing thought.
Speaker: You know, I love the idea that, you know,
Speaker: attributed to different folks, but essentially the idea that discovery is an accident meeting a prepared mind.
Speaker: And, you know, it's the thought that as an intensivist, you know, we start a million different therapies and we have the benefit in the ICU of seeing the physiologic response and getting testing multiple times a day and things like that.
Speaker: And so we are able to very quickly see a reaction to something that we've done or prescribed that
Speaker: And I think if your mind is prepared, not just for the expected outcome, but for what are some potential explanations for alternative outcomes, that's when you start to make diagnoses that are, you know, potentially missed by others and can really change the course of the patient's disease.
Speaker: having that underlying knowledge to then be able to see the outcome and judge it and discern what was the result of that.
Speaker: And then adjust your therapies and diagnoses in keeping with that.
Speaker: Perfect.
Speaker: And I think this is a good place to stop.
Speaker: Thank you so much, Cameron, for sharing your expertise and being so generous with your time.
Speaker: I definitely enjoyed, I mean, learning from you on this fascinating topic.
Speaker: I hope to have you back on the podcast to discuss other topics related to critical care.
Speaker: Thanks a lot.
Speaker: I really appreciate your invitation, Sergio.
Speaker: And it's been great talking with you as well.
Speaker: Thank you for listening to Critical Matters, a sound podcast.
Speaker: Make sure to subscribe to Critical Matters on Apple or Google Podcasts and share with your network.
Speaker: Sound's transforming the way critical care is provided in hospitals across the country.
Speaker: To learn more, visit www.soundphysicians.com.
