Congenital Heart Disease – PediaCast 483

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  • Dr Thomas Glenn and Tawanna Nishibayashi visit the studio as we consider congenital heart disease and its impact on children and families. You’ll hear two difficult (and inspiring) journeys through hypoplastic left heart syndrome and its many challenges. We hope you can join us!

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  • COVID Vaccine
  • Congenital Heart Disease
  • Hypoplastic Left Heart Syndrome

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Announcer 1: This is PediaCast.

 

[Music]

 

Announcer 2: Welcome to PediaCast, a pediatric podcast for parents. 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. It is a pediatric podcast for moms and dads. This is Dr. Mike coming to you from Nationwide Children's Hospital. We are in Columbus, Ohio.

 

It's Episode 483 for February 9th, 2021. We're calling this one "Congenital Heart Disease". I want to welcome all of you to the program. We have another important topic for you this week as we consider congenital heart disease.

 

0:01:00

 

Congenital, you'll recall, means something you are born with. So congenital heart disease refers to heart problems that are present at birth. And these are typically structural problems with the heart.

 

For example, there might be a valve that does not form properly. There could be a hole between pumping chambers. There could be a single ventricle. So, there's usually two pumping chambers, the right ventricle and the left ventricle but maybe there's only one. Or there could be an issue with the big blood vessels that come and go from the heart, or there could be some combination of these defects. And the resulting conditions can range from mild to immediately life threatening.

 

Congenital heart disease can be caused by genetic issues, also prenatal infections, medication or toxin exposures. Or there may not be any known reason at all. It just happens.

 

Now, fortunately, congenital heart defects are not crazy common but they're also not rare. So they affect about 1% of all births which amounts to about 40,000 babies each year in the United States of America that are affected by congenital heart disease. And, of course, if you are one of these 40,000 families affected each year in the United States then this is a very big deal for you and your family.

 

0:02:24

 

We're going to cover lots more details today regarding congenital heart disease and we have two terrific guests with us. Dr. Thomas Glenn is a pediatric cardiology fellow at The Heart Institute at Rady Children's Hospital in San Diego. And he not only cares for kids and families with congenital heart disease, he has experienced one of these disorders, a very serious one up close and personal, a condition known as hypoplastic left heart syndrome which he was born with. So he will not only bring medical expertise but also the personal patient perspective as well.

 

And then, also visiting us today, Tawanna Nishibayashi. She is a volunteer coordinator of The Children's Heart Foundation, Illinois Region and also chair of the Diversity and Inclusion Taskforce for The Children's Heart Foundation nationwide and also the founder of Race Equity Solutions.

 

0:03:21

But she's also a parent who's child was born with the same congenital heart condition, hypoplastic left heart syndrome. So, she'll bring the parent and family perspective. She sheds light on the many disappointments and frustrations these families face, but also the encouragements and bright spots that are part of the package for children and families impacted by congenital heart disease.

 

And all of this is extra relevant right now because February is American heart month and this week right now, February 7th to the 14th, is a Congenital Heart Defects Awareness Week, which always takes place this second week of February.

 

0:04:02

 

Our guests will be here soon. Before we get to them, I do have an update for you on my personal experience with the COVID vaccine. I had mentioned back in Episode 480, right after the New Year that I had received dose number of the Moderna vaccine which, of course, I was very grateful to receive as a frontline health worker.

 

Because in addition to this podcast, I'm a real pediatrician as well, working in the emergency department and close-to-home urgent care centers at Nationwide Children's Hospital. So I'm exposed to COVID-19 on a fairly regular basis.

 

Now, of course, by and large, kids do pretty well with COVID-19. But even when they have mild symptoms or no symptoms at all, children and their parents can certainly transmit the virus to others including healthcare workers who are in close proximity.

 

So, there has been risk for all of us working on the frontlines of healthcare during this pandemic. And speaking for myself and I'm sure many of my frontline colleagues, we've really just been so grateful to receive this protection as we anxiously await getting everybody in America and around the world protected from this very serious disease.

 

0:05:18

 

So, I received vaccine number one on Christmas Eve. And as I previously mentioned, other than a sore arm for about a day, it was really an easy experience. Now, you may have heard that dose number two tends to be a little rougher for some. Which we would expect because the first dose primes the immune system and then when we receive the second dose, the immune system is ready to go to work.

 

And so side effects, including fever, chills, headache, body aches and fatigue, these are much more common with vaccine number two. Not because of the vaccine but as a side product of our immune system in action, which is actually a good thing as we build protection against the SARS-CoV-2 virus which causes COVID-19 disease.

 

0:06:06

 

Now, I have to tell you because I want to level with you and be transparent, that was indeed my experience with dose number two. So, I spent the following day on the couch with pretty much all of the symptoms I mentioned. So fever, chills, headache, body aches, fatigue. 

 

The symptoms started about 12 hours after receiving vaccine number two and they lasted for about 12 to 24 hours. So by 36 to 48 hours after the vaccine, I was pretty much back to baseline. Maybe a bit tired for one more day and that was it, so about two days of feeling a little achy.

 

But seriously, folks, I would do it again in a heartbeat. And there is no question in my mind about that at all. And I tell all of this not to frighten you from getting the vaccine but really just the opposite. To encourage you but to also be transparent with the truth. 

 

So you probably can expect, and I have talked by the way to colleagues who have gotten both vaccines who really just had a little bit of a sore arm with both vaccines. So, their number two was sort of the same as happened with their number one.

 

0:07:15

 

And there's no evidence to suggest that those who have more  severe symptoms after the second one has better immunity. You can build just fine immunity and have very mild symptoms. So that might be your experience.

 

But if you are one of the ones like me who have more significant symptoms for a couple of days following vaccine number two, that is so much better than experiencing severe COVID disease, getting sick with the real illness and then having a hard time with it. Maybe you end up with pneumonia and you end up in the hospital, or in the intensive care unit or on a ventilator, or dead from COVID-19, as so many Americans have experienced.

 

0:08:01

 

So feeling a little achy, and in our family, we always call a feeling viral, for a day or two after vaccine number two really was nothing compared to what a lot of folks experienced and what the risks really are. Or even getting sick with the real illness in a mild form and then transmitting it to someone who has a much higher risk of severe disease or death. We make it less likely that we're going to have mild symptoms from it and pass it on the others.

 

We are going to talk much more on about the COVID vaccine on this podcast a little bit later this month. But I wanted to put it out there that I have had both vaccines. The second dose did produce some mild symptoms for a day or two which again are simply a byproduct of my immune system working properly and as expected. But in the scheme of the big picture, it was definitely worth some mild and short-lived discomfort.

 

And I encourage all of you very very strongly to follow my lead and get your COVID vaccine when the time comes because that is the quickest path back to normalcy.

 

0:09:09

 

All right, couple of quick housekeeping items before we get our guests on this week. I do want to remind you, you can find PediaCast wherever podcasts are found. We are in the Apple and Google Podcast apps, also iHeartRadio, Spotify, SoundCloud, Audible, Amazon Music, and most other podcast apps for iOS and Android.

 

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There's also a handy Contact link at pediacast.org if you would like to suggest a future topic for the program.

 

And then, I do want to remind you the information presented in every episode of PediaCast is for general educational purposes only. We do not diagnose medical conditions or formulate treatment plans for specific individuals. If you have a concern about your child's health, be sure to call your healthcare provider.

 

0:10:10

 

Also, your use of this audio program is subject to the PediaCast Terms of Use Agreement, which you can find at pediacast.org.

 

So, let's take a quick break. We'll get Dr. Thomas Glenn and Tawanna Nishibayashi connected to the studio. And then, we will be back to talk about congenital heart disease. It's coming up right after this.

 

[Music]

 

0:11:00

 

Dr. Mike Patrick: Dr. Thomas Glenn is a pediatric cardiology fellow with The Heart Institute at Rady Children's Hospital in San Diego, California. He not only treats children with congenital heart disease in his clinical practice. He also grew up with congenital heart disease, so he is very familiar with these conditions from the medical and the family perspective.

 

Let's give Dr. Glenn a warm PediaCast welcome. It's really great having you here today.

 

Dr. Thomas Glenn: Thank you so much for having me today.

 

Dr. Mike Patrick: Absolutely. Tawanna Nishibayashi also joins us. She too is familiar with congenital heart disease from the family perspective. Her daughter was born with the heart condition that required five open-heart surgeries before the age of two, at which point she received a heart transplant, all of which, of course, is a lot to handle for a young child and her family.

 

Tawanna currently serves as volunteer coordinator of the Illinois Region of The Children's Heart Foundation. She's also the chair of the Diversity and Inclusion Taskforce for The Children's Heart Foundation. She's a writer, speaker and certified life coach and serves as founder and lead consultant for Race Equity Solutions. So let's give Tawanna a warm PediaCast welcome. It's really a privilege having you here today as well.

 

Tawanna Nishibayashi: It's great to be here. Thank you for having me.

 

0:12:10

 

Dr. Mike Patrick: Yeah, great having you here. So Dr. Glenn, let's start with you. Let's sort of lay a groundwork for congenital heart disease. Remind us, what exactly is that?

 

Dr. Thomas Glenn: Congenital heart disease is really the spectrum of cardiac disease that children are born with. It's something that in some cases are detected after just a few hours of life, in the case of myself. Or there's other cardiac lesions that some children are not found to have until they're in their teenage years.

 

So there's really this big spectrum of severity. And sometimes, you don't even know you have one. You can even be an adult actually and find out you have some lesion.

 

Dr. Mike Patrick: Absolutely. So adults with the heart disease, we think about like coronary artery disease or congestive heart failure. But in congenital heart disease, these are usually more structural problems of the heart, kind of plumbing problems, right?

 

0:13:04

 

Dr. Thomas Glenn: Exactly, that's very easy way to think about it. Whether it's the valves that are abnormal or different tubes that are basically plugged in to the wrong places, that's a very good way of thinking about it. So, it's not so much things that were lifestyle, that were caused by lifestyle choices. They're just things that you were born with that are just not really in the right place.

 

Dr. Mike Patrick: Right. And then, who's affected by these disorders? Are they common? How many kids are we talking about?

 

So actually, congenital heart disease, 1 in 100 children are born with congenital heart disease. And that's actually the highest number for any congenital issue that anyone can be born with. So they are very prevalent in the community. Like I said, there's probably people walking around that you don't even know have a congenital heart disease and that's one of the interesting things about it.

 

There are many genetic factors that sometimes can play into congenital heart disease. There's a lot of genes that we don't quite understand that may be causing congenital heart disease, but there are some that we do understand.

 

0:14:10

 

 

 21 or Down syndrome, for example, is often associated on a higher percentage with different forms of congenital heart disease or something called DiGeorge syndrome. That can also be associated with congenital heart disease.

 

So there's these so many syndromes that have a higher instance of having congenital heart disease. But largely, there's a lot of genes that we don't quite understand at this point. And, hopefully, over the next 15 to 20 years, we can really hone in on that and figure out exactly what's causing all these issues for these children.

 

Dr. Mike Patrick: So, 1 in 100, that�s comes out of 1% of births each year in the United States, which is about 40,000 kids each and every year born with a congenital heart disease. 

 

Those two disorders that you had mentioned, Down syndrome, we did a PediaCast on that for those who'd like to hear more about it. It's episode 453. And then, DiGeorge syndrome also called 22q Deletion syndrome, we did an episode on that back in PediaCast 226. And I'll put links to both of those in the show notes for folks over at pediacast.org.

 

0:15:13

 

And then, in addition to genetic issues, there's also environmental risk factors that can be associated with congenital heart disease. What are some of those?

 

Dr. Thomas Glenn: Yeah, absolutely. So there are certain diseases that mothers could have that can lead to a congenital heart disease or at least increase the risk of developing congenital heart disease. For example, maternal diabetes can be associated with some forms of congenital heart disease. If someone has rubella, that's another thing. We don't see that as much anymore because of vaccination but it's always something that we have to think about.

 

There are certain medications, drinking alcohol, things like that can always cause congenital heart disease as well. Again, this is not something that we see as much right now because most women have really good prenatal care. I mean these things are stressed during the pregnancy. But it's something we still think about.

 

0:16:03

 

And even if someone didn't have these exposures or these risk factors, there still a lot that we don't understand that can cause congenital heart disease. And who knows, maybe just the part of the country that you're living, you might be expose to something that we don't even understand at this point that might be causing it.

 

So, there's a lot to be learned still but, yeah, those are some of the things that we do know that can cause different forms of congenital heart disease.

 

Dr. Mike Patrick: That's very interesting to me. At least here in Ohio, there's sort of clusters of communities that seems like where kids with congenital heart disease sort of come from which would suggest that perhaps there are environmental toxins that we're just not completely aware of exactly what those are. And of course, we can see it clustered in families too, since there's that genetic component to this.

 

What are some specific examples of congenital heart disease? What exactly are we talking about?

 

Dr. Thomas Glenn: There's dozens of different forms of congenital heart disease but there some things that we might not catch initially or things like VSD or ventricular septal defect, an ASD or atrial septal defect. These are holes  between different chambers of the heart.

 

0:17:10

 

And then, some of the more severe things like hypoplastic left heart syndrome. That's the form of congenital heart disease that you're probably diagnosed with in the first few days of life and need surgery within the first week.

 

Truncus arteriosus, tetralogy of Fallot, interrupted aortic arch, those are just some of them but I can go on for probably an hour on the different forms of congenital heart disease.

 

Dr. Mike Patrick: Well, one we're going to talk about in a much more detail today because of the fact that it has impacted both of your families, is hypoplastic left heart syndrome. Tell us about that one in a little more detail.

 

Dr. Thomas Glenn:  Hypoplastic left heart syndrome, like a lot of other form of congenital heart disease,  there is a spectrum within that diagnosis. But essentially, what it means is that the entire left side of the heart doesn't quite developed normally in utero. And as a result, the left ventricle which is the main pumping chamber of the heart is not able to sustain a systemic outflow or pump blood to the rest of the body to  deliver oxygen.

 

0:18:11

 

In addition to the left ventricle often being hypoplastic or small, the aorta which is the name kind of candy cane looking structure that leaves the heart that delivers oxygen to the body is also underdeveloped. And with that, sometimes, the mitral valve, there's a shred or absent or very small. But the entire left side of the heart is essentially underdeveloped and you need some forms of surgeries in the first few days of life to kind of fix that and repair it, so that children can live, essentially.

 

Dr. Mike Patrick: Absolutely. And as we recall from biology class in high school, the heart there's really two separate circulations, one that goes from the heart to the lungs and then back to the heart. And then from the heart to the rest of the body, back to the heart.

 

And so the left ventricle is the one that goes to the entire body. And so folks with hypoplastic left heart syndrome, you really have to rely on the right ventricle, which normally would only have to go the lungs really covering everywhere.

 

0:19:12

 

And then, instead of two separate circulations, you're just going to have one circulation, correct?

 

Dr. Thomas Glenn: Yeah, exactly. So the stage repairs for hypoplastic left heart syndrome, it usually includes, at minimum, three surgeries. You basically turn the right side of the heart into that systemic side of the heart.

 

So the right heart is now pumping to the body which, like you just mentioned, is not designed to do. So the right side will pump the blood to the body, send all the oxygen to where it needs to go. But then, instead of the deoxygenated blood coming back to the heart, after all the repairs, it actually bypasses the heart and goes directly to lungs because now you don't have a chamber to send it to the lungs. So it relies on kind of passive flow where gradually, essentially, it gets to the lungs.

 

Dr. Mike Patrick: Since we mentioned these are kind of pumping problems, surgery is often required to treat these. But there's also medical management as well. Can you just give us an overview of what the medical management that these kids go through, both surgery and medication-wise?

 

0:20:15

 

Dr. Thomas Glenn: So for some of the more serious congenital heart diseases like hypoplastic left heart, truncus arteriosus, tetralogy, often, those require surgery. Some of them, like I mentioned, in the first week of life, some of them within the first six months of life and it really depends on the severity.

 

And, like I said, it's a spectrum. So you kind of have to watch the child and see when they need the surgery . But a lot of them do require surgery at some point. There are some congenital heart lesions like a VSD, a ventricle septal defect, where you can medically manage with diuretics or medications to help you remove fluid from the body.

 

And sometimes, you need some like ace inhibitors which lower the blood pressure and kind of balance the flow within that hole that's in the bottom two chambers. And often, you can manage those kinds of lesions in most kids up to about six months of age. But once they start losing weight and demonstrating signs of heart failure, it's usually when you have to go in for surgery.

 

0:21:12

 

But there are some lesions like an ASD or atrial septal defect that you actually might not need surgery at all. Actually, on the last, I'm not sure exactly how long, but last 50 years I'd say, we've really developed some strategies where we can use less invasive ways of repairing these lesions.

 

And you can actually use something like cardiac catheterization procedure. We actually use the veins and then the arteries in the legs to gain access to the heart using these very small catheters and wires. And you can actually close holes that way. And that prevents you from having doing an open-heart surgery. Usually, those kids are in and out within a day or two.

 

So it's pretty amazing what we can now and everyone doesn't need surgery, but there are decent number of lesions where you do have to have open-heart surgery.

 

Dr. Mike Patrick: And there's really been a lot learned over the past few decades with regards to treating these. And I remember early on in my medical training, kids that had hypoplastic left heart syndrome oftentimes did not fare well. 

 

0:22:12

 

And so, we've really made a lot progress in terms of being able not only to technically perform the surgeries, but then also caring for these kids post-surgery. Just talk a little about how that's changed over the years.

 

Dr. Thomas Glenn: Yeah, it's actually pretty incredible how fast because if you think about medicine as a whole, it takes hundreds of years sometimes for things to change in medicine. But within the last 30, 35 years, hypoplastic left heart syndrome is completely different than it was 35 years ago.

 

And I was born 30 years ago. So 1990, when I was born, my surgeon in San Francisco actually never had a survivor with my lesion. He told my parents that there was no reason to do the surgery and that, essentially, it was futile. Luckily, my parents said, "No, we want you to do the surgery," knowing the risk and everything. But they want to give me a chance and it turned out I was his first survivor of many after that. 

 

0:23:08

 

So it was really not until late 80s, mid-80s, that these surgeries, the hypoplastic left heart syndromes specifically, were even being offered because Dr. Norwood who designed the first surgery for these patients have kind of come about in Boston. He's sort of traveling around and all the surgeons will visit him and learn how to do this and bring it back to their institutions.

 

So notice, in 35 years, we have a disease that was essentially 100% lethal to something where now, you can get to the first three-stage surgeries and I think it's like 15% lethal. So much different. And then that's only for the surgery. But everything in the ICU afterwards is completely changed.

 

There are medications like milrinone now that wasn't used back then that can be life-saving therapies for these patients.

 

Dr. Mike Patrick: Yeah, really, really amazing progress. My roommate from college and still very good friends with him to this day, he and his wife had a baby with hypoplastic left heart syndrome who did not make it. And that was around that same time, that kind of early 90s.

 

0:24:12

 

And even today, losing a child to congenital heart disease is really tough and a big hole in that family. So even we had amazing progress but even families today who go through this and have a child who survives and does well, it's still really a challenge for individual families. We have progress but we still have a lot of challenges and obstacles and hard times that families go through.

 

Tawanna, I wanted to bring you in and talk a little bit about what this looks like from the family's perspective. We're talking about this like it's something we talk about every day, with all these complicated things with the heart and surgeries and putting things back together using medicines to manage hypertension and all of these.

 

0:25:00

 

But from someone without an initial medical background, this is really tough for families, right? Tell us a little bit about what it's been like for your family?

 

Tawanna Nishibayashi: Without a medical background, it's lion sighting, to say the least. I count myself fortunate that I am a researcher and a type A personality and I have an affinity to learn. And I'm very thankful for that because I now think and move like a clinician, even though I'm not a clinician.

But that's not everyone's strong suit. Everyone doesn't have that capability and they will still have a child that has a CHD and, unfortunately, perhaps not know how to advocate for such child because there's such an extreme learning curve. And you're getting hit with so many things from so many different directions, from developmental standpoint, from a germ standpoint. And in our case with HLHSC, low oxygenation and lethargy standpoint to hypertension standpoint.

 

0:26:01

 

My daughter was born with a complication of HLHS. As Tom alluded to, no CHD is simple. You have a diagnosis but no two cases are alike and you might have different complications along the way. You might be born with different complications. And so, Avery Grace was born with a small ascending aorta, one that they couldn't always find but more importantly, with a leaky tricuspid valve.

 

And so, when you have HLHS with tricuspid valve regurgitation, the likelihood of making it through the Fontan is not very likely. And if you do, you're generally going to need some sort of assistance. You're going to need intervention in terms of a tricuspid valve annuloplasty. They're going to try and repair it.  They are going to perhaps bring in like a mechanical valve or talk about a valve replacement.

 

So, these are things where in her case not only did she had HLHS but that tricuspid valve regurgitation from the beginning made her path a little bit different.

 

Dr. Mike Patrick: I imagine you're throwing around terms now that you had no clue whatever this was in the beginning.

 

Tawanna Nishibayashi: Yes.

 

0:27:05

 

Dr. Mike Patrick: And I want to point out too that as you've mentioned, you've been a researcher and you looked this stuff up. And from a primary care pediatrician standpoint, I just want parents to know, most of us when you have kids that come in with things that are unusual and not common, we really do trust the parents if they feel like something's not right or something's going on.

 

Because oftentimes, you actually know more about that disease process than the primary care pediatrician does because living it day in and day out and doing all of this research. And we just see it every now and then. So I would encourage young pediatricians to really listen to families when you have these kids. And I think that those of us who've been around the block a few times, we definitely listen to parents. 

 

But I suspected that can be really frustrating from the parent's standpoint when you feel like something is not right or going on or different or you have concerns and then feel like people aren't listening to you?

 

0:28:01

 

Tawanna Nishibayashi: Yes. I have learned how to make people listen. And I've always been one to utilize my voice. It maybe, I live in the  mid-West now, I live in Chicago. But I'm from New York and so I've been told that I have strong personality, right?

 

But that may just mean that I've always known how to use my voice. And again, these are just innate qualities that I had that I didn't have to foster. The one I am mentoring, families that are just entering this world, that's a huge huge point. And I thank you Dr. Mike for making that point.

 

Because when you have a child, especially if you're a first-time parent, you've got to learn just the general parenting things. And Avery Grace was our first and she is our only child. And so, not only do you have that curve, but you're looking to your pediatrician, right, as the person that can tell you what's going on.

 

And in our instance, even though I was careful to choose a pediatrician who have been around for a while and who had experience with some complex cases and medically complex and medically fragile cases, that particular pediatrician wasn't always in when we had a question or when we need to bring Avery Grace in.

 

0:29:02

 

And we were told some things by younger pediatricians who honestly gave us the okay and I was like, "No, we've got to call our team." And subsequently, Avery Grace ended up being in heart  failure, right, when we are told, "Oh, it's just a virus and let's give her some antibiotics." And that's no fault of theirs. But it just goes to your point to research and to ask questions and to listen and to not take anything for granted.

 

And so, for a while, especially during that interstage period and pause for her first year or two of life, we really just went directly to our team even if she had a common cold because heart failure and issues, the symptoms are similar. And so, it was always like, "Well, does she have a cold? Or is there something deeper going on?" And kind of going down that.

 

And so I took notes every day as if I was a nurse. I charted from my child. It's a little extreme, but I mean, I had to make sure she was hydrated. I had to make sure that she was intaking enough calories when she has a bland physiology. She had a feeding tube that we're trying to wean her from without support from anywhere. It's just kind of us and what we were trying to do.

 

And so literally, from the diapers and what she ate and if she had MSS and how much, all of these things were things that I track every day during that first year of life, really to help me keep the pulse on what her behaviors were and if she was healthy.

 

0:30:14

 

Dr. Mike Patrick: Yeah, absolutely. And that goes back to kids who don't have congenital heart disease. I mean, parents have a lot of questions and if you're up at night worried about your child, it is okay to call the pediatrician. I mean, that's where we're here for and we do want to hear. In your situation, with the congenital heart disease, your heart team was your doctor. And so just going straight to them with those issues, I'm sure that that's what they would prefer as well.

 

But I always feel like parents kind of get this idea that they're bothering us when they ask questions, that then to us, they're simple questions. But if they're keeping you up at night, you definitely want to talk to your doctor and get some reassurance. So that's great.

 

How do you advocate now then for families to take care of their kids and to contact their doctors when they have concerns? What advice do you have for them?

 

0:31:10

 

Tawanna Nishibayashi: To be honest, we choose our teams, we choose our doctors. We have the power of choice, right? And so, we're working together but technically, you're working for us, and for our child. And so, try and kind of take, there is a level of respect that all clinicians and anyone in the medical profession, and not that, every day, individual, you give respect and you give deference and you recognize the skillset. But on the same vein, as you said, you are the expert in your child.

 

And clinicians, especially once you get inpatient, they see so much, so many serious cases. And it's very easy for burnout to happen, or for you to miss a thing, or for you to just look at it from the clinical standpoint instead of the wellbeing of the whole child. But you, as a parent, the wellbeing on top of the medical is what you're focused on daily.

 

And so, you get someone that you feel comfortable with, and you go to them and you ask the same question three times to three different individuals. And you'd be amazed that sometimes, you get three different answers and you need a tie breaker, right? So then, you got to ask the fourth person, because a lot of it is you're only as good the pediatrician that's on call, the intending that's on service that week.

 

0:32:18

 

They have different philosophies. Some people are more conscientious and thoughtful. Some people try and foster care. Some people what I like to term is data heads, and they just want all the data and all the labs all the time, right?

 

And so, there's just different styles and you'll find what works best for you and what works best for your child. And just never be afraid to use your voice for the wellbeing of your child because you can make all the difference. What your eyes see, what your instinct says, that question that you asked about three or four times, the fourth time might be the charm where you get something that's like, "Oh, can we just try?"

 

The worst thing someone can say is no, but I learned that the team appreciates the input that you give and recognizes that your remember the team. And the children who have parents that are true advocates do better. They have better outcomes. It makes a difference when the parent is involved, because the doctor or your team can only do what they can do when you're in their office or when you're inpatient in the hospital.

 

0:33:12

 

When you go home, it's the parent that has to keep all of that up, right? It's the parents who are up with the feeding tube every three hours and cleaning up the emesis and getting medications and cleaning that, it's you. I was giving Lovenox injections when Avery was six weeks old. I was dropping NGs, I was doing blood pressure. Just all of these things that were never things that I anticipated that I would be doing, but I was doing them. 

 

And so, your voice matters and don't ever be intimidated to speak up and to ask questions, and if you don't understand, then have someone explain it to you. And then, have a different person to explain it to you. Because there are people, that that's their specific job, and they'll do it, and they'll do it gladly to help you understand what's going on with your child.

 

Dr. Mike Patrick: Yeah, really, really good points. We're going to have a lot of listeners out there for this particular episode who do have kids with congenital heart disease. The title of the episode kind of stands out to them.

 

0:34:08

 

What advice do you have for families that are going through this journey, especially those kind of early on in the journey? Maybe they have a baby who is just recently diagnosed or maybe they're going to give birth to a baby that's been diagnosed with a congenital heart disease prenatally.

 

What advice do you have in terms of the challenges, frustrations, disappointments? What can these families expect? And then, how can they overcome those things?

 

Tawanna Nishibayashi: There's so much to expect. You gave us the questions beforehand, right? And so, I looked and I couldn't even jot down all the points. Really, I think the most important thing is some sort of faith and some sort of trust in the process, to allow the process to happen. Because otherwise, your energy makes a difference and your child feeds on your energy. And so if you're fighting, then you have to be in the fight.

 

0:35:01

 

I think you have to make a decision in some terms. I was offered termination of my pregnancy because of the mortality rate of HLHS. I was offered to have her and to not go through with that first surgery, the Norwood surgery, that she subsequently had when she was five days old. And now, five open-heart surgeries and a heart transplant later, I have a four-and-a-half year-old who has some issues, sure, but she's here and she's thriving, she's healthy.

 

She's in school three days a week in pandemic with her team's approval, right? And so, she's living a life. And she's joyful and she's happy and she's affectionate.

 

And so, to know that, CHD is a place for miracles. CHD is a place where there are things that happen that surpass expectations and reasoning. Your center matters, your team matters, especially if you have a child that's diagnosed with a severe CHD. Unfortunately, I would say and for us, there were ten centers across the United States that we interviewed five of in terms of where we were going to have Avery Grace to give here the best chance.

 

0:36:04

 

It's not just about surgical outcomes. It's about how many surgeries they did. It's about the team. It's about the follow-up with the team. Multidisciplinary rounds help. Are there intensivist? Where do they come from? What are their philosophies? Are parents allowed to be inpatient in the hospital or not and those rules?

 

So really, it's quite a rabbit hole, but just know that the place that you trust to go along and fight with you matters. That the clinicians matter and their lives matter because they're now our family. They really are, Avery Grace's team has become our family. We love seeing one another and we fought for this child together.

 

And we know about each other's personal lives. And just to recognize that they're people and they have the ideas, too. And understand that when you get close to them and you understand that they care about your child not only on the clinical level, but on the personal level, they start to really care about your child. And the more people that care about your child, the better when these things are happening.

 

0:37:07

 

And I would say that it's not just about the CHD. And I struggle with this because Avery Grace is going through so much in such a short period of time. She's a child and we did all this so that she could have a life. And so that she could have some sort of normalcy, right? And it's really, really hard to let her go, especially in a pandemic, which is on immunosuppressed and she's battled rejections and some other things. It's really hard to let her limp.

 

And there are some things, I don't say I'm a germophobe now, being in the hospital, inpatient, outpatient, all the different things. There are some things that aren't going to be safe for them to do. But the things that you can compromise on and make a happy medium, or do safely, you ask your team about and you make it happen in the ways that you can.

 

For example, this is the first year that Avery Grace was healthy enough and stable enough to go outside and play in the snow, right? So people would say, "Oh, it's snow, we're going on a sleigh and we're going to do this. We're going to build a snowman." And this past week, with this big snow, which I am not enthused about now, but I was enthused about that first day because Avery Grace was able to go outside and go play in the snow.

 

0:38:13

 

Avery wasn't able to be socialized the first couple of years of her life because of everything that was going on. So now she is a year and half behind about mentally and in a class that is technically a year and a half behind. And socially, she's just kind of coming to terms with not being afraid of people. And so, the more things that you can do to have your child find some joy and your family find some joy is huge.

 

And then, I think the last big thing is people outside of the world are likely not going to understand. No matter however they try, no matter how compassionate they are, no matter if they are your parent, right, if they haven't gone through it or been adjacent to it or aren't the provider in the world, your challenges are very very unique.

 

And even if they don't understand, it doesn't mean that they love you any less, right? It just means that they don't get it. And sometimes, it's really difficult for them. And it's really laughable as the person fighting for your child's life and watching her cold and be attached to a Berlin heart and all these things that like, "Oh, this is difficult for you." Really, is this difficult for you?

 

0:39:19

 

But nobody should have to go through the things that some CHD parents are asked to go through. And the children shouldn't have to go through the things that, you know. So just don't think less of them, let them have their space and try not to get bitter if they're not there for you.

 

But there's a heart community that knows how, there are heart parents that understand. There are children who are experiencing the same things and your team can help you. And there are organizations across the United States that can help you. And your hospital will sometimes have a local page or a Facebook page.

 

And those become your people. People you may never meet in person and some that you won't. Those become your people that will get meals for you. And when you tell everyone that you don't know need anything because you don't have wherewithal to say what you need in that particular moment, they'll send you things and they show up, and they'll show up for you.

 

0:40:08

 

And so, if people's relationships with you change, it's okay, it's part of the process. And it doesn't mean that the relationship was as valid. What's important is that there is a community that's there for you. And so, if you chose to go through this road and you're fighting this road with your child, make sure that you seek that community because it will make all the difference going forward.

 

Dr. Mike Patrick: That's really, really good points. And I love the way you put it, it's not just about the congenital heart disease. It's also about being a person and being a family, and being a parent and all of the difficulties that these weighs in. It's not just the child that's the patient. There's really hardship and need on the part of families as well.

 

I know one of the things that you are passionate about is quality and the problems with racism that we have in America. And I just wanted with your expertise in that to speak on the issue of racism as a public health crisis and how that particularly affects children of color who have congenital heart disease and the special problems that these families face.

 

0:41:18

 

Tawanna Nishibayashi: It's huge. I live in a major metropolitan city out of Chicago, Illinois. And we're seen at a hospital within the city that's amazing. We love our team, we love our hospital, we love our program. There is no diversity within the program, so there was no one that looked like me or my child, treating my child from a provider standpoint, from a nurse standpoint.

 

There was a surgeon and there was one attending physician. But initially, when we started, we didn't have that. And so, there's the unique lack of representation. It's going into a world where you feel like you have to be a little bit on guard.

 

The mortality rates are different. Treatment can be different. With racial and ethnic minorities, we received lower quality healthcare across the board than white peers and that's one.  Insurance and status and income, age, comorbidities and severity of the conditions are all comparable, but were less likely to be given appropriate cardiac care.

 

0:42:20

 

And so, I knew that when I found out about Avery. And so, automatically, not only am I fighting something where you're telling me the first surgery, there is 15% mortality rate. And you know, and as you go forward, and this is just all things created equal, but I knew I was going into a hospital system that is not always kind to  people who look like me.

 

And so, it just was an extra added layer of stress. And I saw the difference in the way families were treated. I experienced, I've overheard people working, when you live in the hospital, you'll hear a lot of things. I've lived in hospitals for long periods of time.

 

0:43:02

 

I am what I call myself a double negative because I'm a woman. So I'm a minority in that stance and then I'm a black woman. So there’s a double negative here. And so, it's very easy to fit into the stereotype of angry Black woman, right? Or harsh words that you will call a woman who you think are.

 

And so, I would be doing the same thing that my Caucasian counterparts were doing in terms of advocacy for my daughter, but nurses didn't want to take us, right? Because I was too involved or I said too much. And I overheard them making comments when there was finally another nurse of color, like "Oh, we should pair them together then, so that way, they can be comfortable." So there's like microaggressions that happened.

 

And then, the other thing is that I felt and you feel, like tokenism, you feel as if you are the spokesperson then and the representation for your race because there isn't any around you. And so, the onus is on you to kind of answer these questions and to ask the right questions. And you have to try harder than others do because of the fact that you're Black and I'm a Black woman.

 

0:44:13

 

And so, I got up every day at 5:00 in the morning on the hard hospital couch when I didn't go to bed until after her last assessment at midnight. I fought really hard to hand off assessments overnight when I could. And so I had that 12 to 6 window. I'd wake up at 5 and I'd get dressed business casual, as if I was going to work every day.

 

And I would attend rounds, but I had to do that in order to be taken seriously. And I got a lot of surprise at the knowledge that I had and a lot of "You speak so well," or "I'm surprised you're aware of this," or "You articulate that and I didn't know."

 

And rather than just kind of speaking to me as a peer, as a parent, who had an affinity to learn and the ability to learn, it was the microaggression of "Oh, I'm surprised." But I had to do that to be taken seriously. Whereas other moms were in slippers and sweatpants or whatever, and they're like, "Why are you dressing like this?" And I'm like, "If I'm dressed like that, I'm not going to get the respect that I need. And I need this respect in order to properly advocate for my daughter."

 

0:45:12

 

Dr. Mike Patrick: We really appreciate your transparency and vulnerability in this. And there's so many things that families go through that other families don't understand. And really, the more we hear about these things and kind of try to put ourselves in other people's shoes and develop empathy, the more it is that we understand.

 

Well, we did a PediaCast on racism as a public health crisis, that was Episode 464. So folks, if you'd like to hear more about that, please do check out that episode. We'll put a link to it in the show notes.

 

So many difficulties and challenges, and yet part of the congenital heart disease story, there's also encouragement and rewarding times as well. What has your family gotten out of this experience that you've really appreciated?

 

0:45:59

 

Tawanna Nishibayashi: Gratitude. There are things in life that you take for granted a lot of times. My grandmother has like a very grandparent-ly thing to say, like, "When you're old, you should be happy and thankful that you got out of bed this morning," right? You got your two lungs, and you're breathing and your heart is breathing and all these things.

 

And I think you find gratitude and joy in everything. And you have such an appreciation for every moment in life that you get with your child, that it also spurs you to look deeper in yourself and to take a look at your life and say if we're going to have a short period of time because to be clear, HLHS, there's not a cure. And when you get a transplant, that's not a cure either.

 

And so, it's really important to know that a lot of times,  you feel like you're kicking the bucket down the road, right? Or you've got one foot in the air and you're  waiting for the other shoe to drop sort of situation. And so, there's this idea that you may or may not have a lot of time when you're on this clock. That maybe if you have a healthy child or you're a healthy individual, you're not quite aware of that.

 

0:47:05

 

But what gives you is perspective. And with that perspective, you really kind of tackle life, right? And you pull in everything that you can and also how  strong these kids are. They're so much stronger that you, right?

 

As parents and as clinicians you, have to have to be strong and you have to have constitution, and you have to be smart and all these things. But these children, they're just kids. And to see a child with their chest open or who just got their chest closed or who had open-heart surgery last week, like, my daughter was attached to a Berlin heart as a bridge to transplant and she was walking.

 

And she was trying to escape the Berlin heart because she wanted to climb up and go and see in the window and do things. And she was laughing and eating McDonald's french fries.

 

And so, it's like the strength and the joy that the children had teaches you and the hope that they give you for humanity and belief in science that they give you as you see these miracles from Dr. Norwood and Dr. Glenn and Dr. Fontan and everyone who's kind of come together in the community to learn these surgeries to make sure that these kids have a chance.

 

0:48:13

 

So there's so much joy. And there's so much knowledge and there's so much strength that you get from them. And it's a perspective that I know my family will keep all of our lives. And the connections that I alluded to earlier, the connections aren't always just when things are not going great. The connections in times of joy and to see these children grow up together that were hospital neighbors that you've seen.

 

You've heard the codes and all these things. It's really the relationships and those friendships are your friendships that are bonds, that are forever as well.

 

Dr. Mike Patrick: What advice do you have for us medical providers. So having kind of gone through the system, seeing the challenges and the joys that you've experienced, how can we do our jobs better?

 

Tawanna Nishibayashi: Seeing the child as a whole child is a big one. It's I don't want to say it's easy to keep easy these children alive because it's not. It's not. But it's easy to make that the focus.

 

0:49:06

 

In the hospital, I always had saying, just in general, that it's not just about longevity of life. Although that's huge and that's what we're fighting for. We're fighting for quality of life. And so quality of life in patient means your child can get ICU psychosis. And that the person doesn't need to poke the kid at three in the morning if it's not an emergent situation. And they don't need to change the garbage or keep the hallway light so brightly.

 

Kids need sleep, right? And kids need a schedule and kids thrive off of your team. And so I think really just focusing on making an inpatient experience, if you're a hospitalist, one that is as close to outside life as possible. And if you're a pediatrician working with your patient's cardiac team, cardiology team, to see what things they can do. It's not about what they can't do, it's about what they can do. And it's about keeping them healthy, wholly.

 

0:50:02

 

And so, with HLHS, sure, I agree that my daughter needed to have a feeding tube at the beginning, especially in her state because of the way that the kids lose weight and all of that stuff. But somewhere along the line, the feeding tube became a crutch and everyone just like the control of how many ounces she was taking in this for hydration or whatever. And it's not like "Okay, so now we're out of the hospital."

 

She has to be live and she has to be amongst people. And is a feeding tube or another surgery for a G-tube necessary? And so how do we kind of transition from them? So I think our program has gotten really, really good over the past last couple of years about making sure all the therapies are happening from speech and OT because developmental issues are going to happen when your child's in the hospital, when they have low oxygenation and just other things.

 

And so, from wherever your standpoint is, as a clinician, if you focus on the whole wellbeing of the child and not just the defect, then that's not going to make a better quality of life for everyone for the time that the child is here. Let that child experience and let that family experience as much as they possibly can safely, safely, right?

 

0:51:09

 

So in the middle of pandemic, we're not going to go on a trip and stay in a hotel or be around a bunch of people or eat a meal without masks, never again. I'm a germophobe. We haven't seen anyone since March.

 

But what are the things that can happen and help you facilitate those things? And I think that and just really including the parent as a partner but more and more clinicians are doing that now, trusting the parent as a partner.

 

And then, parent education is the last one. I think that handling someone a pamphlet is nice, having someone really explain it and asking some questions and having somebody regurgitate something back to make sure they get it. Because a lot of time, you get this medicalese days and you'll just say yeah, yeah, yeah to get out of there. So it's really just making sure that the parent understands because the better a parent understands, the better partner they're going to be in their child's health.

 

Dr. Mike Patrick: Tawanna, I think that your family, it was not one way that your family benefited from your medical team. I think your medical team benefited from having your family with them.

 

0:52:10

 

Tawanna Nishibayashi: Thank you.

 

Dr. Mike Patrick: Oh, absolutely. And just in terms of the growth of that program and you being such an outspoken advocate for your child and family has probably, I won't say probably, I am sure that it has made a difference in families that have come through that program behind you, in terms of really improving the care that those kids get. And so, thank you. Thank you very much.

 

Dr. Glenn, I want to bring you back in. You've kind of heard Tawanna and her family story. And of course, their experience is a little more recent than yours was 30 years ago. But do you remember those trying times with you and your family as you experience congenital heart disease from the patient side and again, with hypoplastic left heart syndrome?

 

Dr. Thomas Glenn: Yeah, I don't remember all that much. I think the first memory I really have of my hypoplastic left heart syndrome, I think it was when I went back for my Fontan or maybe with the cardiac cauterization afterwards. So it was probably around, if it was my Fontan, I was four and a half, five.

 

0:53:20

 

 If it was later, I was probably like six or seven, but I remember going back for one of my procedures and they were rolling back to the OR. I just remembered giving my dad a thumbs up. And it was something we always I guess did before I went for any procedure and it's something I kept doing after that. But I, for some reason, remember that memory. I remember nothing else until I was like nine or ten. But for some reason, I do remember that right before anesthesia.

 

I've heard a lot for my parents about what it was like back in the 90s being born with this. Back then, it was very different, there was no support group, there was no Facebook, there was no social media. There is probably no one in the whole Northern California who had this.

 

0:54:01

 

So I was born in a small community hospital in Northern California and within a few hours, they noticed that my color is off. I was cyanotic. And no one was having an idea what was going on. So they just sent me to the biggest hospital that was around which was UCSF. And that's where they did an echo which was a lot less detailed as what they can do now. And that's when they found I had this condition.

 

And like I mentioned,  they gave my parents the options of just letting me go home without any intervention and let me pass away or doing the surgery. And at that point, they weren't really doing transplants or there were very few transplants. And the only place I think that was doing  them any time at that point was Loma Linda, which is obviously further south. So there weren't many options but luckily my parents chose the options they chose and it paid off well for our family.

 

0:55:00

 

Growing up, I was lucky enough to have no very serious complications which back then was pretty rare. I count my blessings every day because I don't know how I came out with this without serious complications. I really am one of luckiest. People always say I must be like really unlucky to be born with something so rare. But I'm actually very lucky to come out of this without any serious complications because as a provider, I see all the different complications and other things that children are born with and have to deal with. And I'm just incredibly blessed to not have that.

 

And as result, my parents were able to let me live a pretty normal life. I had cardiologists growing up that were very very motivating and wanted me to, they said that since I had gone through this and I was living normal, they wanted me to pursue all my dreams and do everything I want. And that was really the reason that my parents decide to do surgery, was because they wanted me to have as normal a life as possible.

 

0:56:01

 

And growing up, I had an interest in working with children. I had an interest in science, and then that's how I ended up going to pediatric residency after med school. And then, it was a decision of whether I wanted to be a cardiologist or not. It was a very tough decision. For a long time, I said no, because it was too close to home. I would be too emotional and I would know too much.

 

And I didn't know if that's what I wanted for my life. But the more I tried to stay away from it, it just pulls me back in. And there's a special place in my heart for these kids with congenital heart disease because I was one of them at one point.

 

Dr. Mike Patrick: I would imagine that your experience would be very reassuring to families and patients to have someone who have been in their shoes and can sort of get it from their perspective. Do you find that families and kids appreciate that when you talk about your story?

 

Dr. Thomas Glenn: Well, I actually don't really tell my story to most of my patients. I haven't. And that's something I struggled with going through this, training on whether that's something I want or not. And I still don't know what the right answer is. I really don't because, obviously, I'm going to provide the same level of care no matter what, whether they know or not.

 

0:57:14

 

But I just, I don't know, for me, I want to keep my professional life separate enough from my work life because it can get a little hairy. I don't know, I don't have a good answer. But there have been some families that recognize me from there's a lot of social media posts in different groups I've been a part of online. There are talks I've given. They have recognized me.

 

So there's a few families that have recognized me in San Diego. But for the most part, I don't really talk about it. I'm a fellow, so maybe that will change when I'm an attending. I'll have a closer relationship with these families. I'm following them every month, two months, six months. That might be something that's going to change.

 

There's been a few instances where there is a patient who's going through some stuff and it was just me and him, and I chose to tell him that. I have to get a different level of trust for him and I think it did help. But it's something I don't do with most of my patients. And that's something I'm still trying to figure out, my identity.

 

0:58:13

 

Dr. Mike Patrick: I get that. I understand that, especially with families who maybe they are having a really tough time with it, and they've had multiple surgeries and things aren't going well. And then, here's this person in front of them who did well. I mean, I can see that that could also cause other feelings in there.

 

Tawanna, yeah.

 

Tawanna Nishibayashi: It's tough one. So not only did my child has a CHD, but I would meet people within the community, and they'd be like, "Oh, my child had to be a stay-in." But my child had one of the most complex CHDs, right? And then, not only did my child have one of the most complex CHDs, she had one of the most complex cases of her particular complex CHD.

 

And so, you layer all that on and so all experiences aren't created equal.

 

0:59:03

 

And sometimes, it would be hard. It's like, okay, she was supposed to have these three stage surgeries, not the two or three unplanned because we never had the Fontan. So we have three unplanned surgeries, just trying to keep her alive where I see other children who I know that were her age have had the Fontan at that point.

 

And they were in school. They were running, they didn't have, they were speaking. They were doing all sorts of things. And so don't begrudge them that. But it is difficult when your reality is so different.

 

And then, Tom, I think I understand where you are coming from, because I've never wanted Avery to defined by her CHD. And I've never wanted to be people like, "Why don't you just do this as heart mom?" And you know, heart mom, heart mom, heart mom, and it's like before I was a heart mom, I was Tawanna, right?

 

And before I was Tawanna, I was a Black woman. So I'm a Black heart mom, so that's a different experience because that's just the way our world is. And you put all that together and I don't want to be defined as heart mom. And so, from your standpoint, Dr. Mike, of not wanting to hear he's healthy and then my child's struggling, and it's like why couldn't, definitely, sometimes, there may be that thought process.

 

1:00:07

 

And then, just from that one, to be the leading factor and the care that you provide to the person that you are, "Oh, you know, there's Glenn and he had HLHS so I want him being the doctor of HLHS" just versus a really, really strong provider and a really good doctor aside from all of that would be my thinking.

 

Dr. Thomas Glenn: Yeah, I think there are different things that I will bring to the table, that they can get out of the experience of me being the provider that doesn't have to be said that way. And that's just what I want to try to do. But there'll be probably some point down the line where those patients where I do tell or I'm sure there'll be experience where that happens.

 

Dr. Mike Patrick: Yeah, definitely makes sense. And how you take it kind of one day at a time and one family at a time as you figure it out.

 

So as an adult with a history of congenital heart disease, what does long-term follow-up look like for kids as they transition and travel through adulthood.

 

1:01:05

 

Dr. Thomas Glenn: Yeah, so very recently within the past few years, this field of adult congenital heart disease has really grown and kind of blossomed into its own field now. Before, the last five, ten years, pediatric cardiologists were just seeing these kids as long as they could. And I was one of these patients where I was in New York for my residency. I was going in to a children's hospital as a 28-year-old.

 

And I was with these kids in this room and I could probably have been the parent for most of these kids. I was a lot older. And luckily, they now have these programs and it's still new but there's a lot of programs around the country that are specially trained to treat adults with congenital heart disease. And it's a whole new board exam that physicians have to take now. It's not something you just grandfathered in.

 

So there's a whole process and I think it's a very good thing because there's now more adults with congenital heart disease than there are children. And I think that speaks to what we've done over the last 30, 40 years with the care. And think that's a good thing, but it also means that we have to keep up with it.

 

1:02:08

 

And there's a lot of pediatric cardiologist in the world, but there's not a lot of adult congenital cardiologist in the world then. A lot of adult cardiologist don't get trained in this lesion. They don't know what a Fontan circulation is. They don't know all the complications, the ins and outs of the physiology that comes with this lesion. So it's important to have this extra field then.

 

With that, it's also important for patients to follow up as they become adults. So I think it's very easy for these patients to just kind of "Oh, I've had my Fontan 20 years ago. I'm doing great. I don't need to follow up with anyone. I don't need any more surgeries. This is all they told me I needed."

 

But that's not the truth. Like Tawanna was referring to, a Fontan or a transplant is not a treatment. It's not the end-all of your therapy. You're going to have things down the line and you need to be monitored and followed up in medications. And for a patient with a Fontan, the liver becomes, in addition to the heart, the liver becomes the biggest issue because you have all this passive blood flow coming up past your liver into your lungs. But it's going against gravity, so you have a lot of congestion of the liver. And as a result, there's a lot of complications that can happen down the line.

 

1:03:19

 

The further you get out from your Fontan, the higher those risks go out without having issues. Now, I'm 25 or 26 years past my Fontan, so I'm right in that range that needs follow up. And a lot of patients don't realize that, so I think it's important to encourage them to follow up and they should be seen at least yearly.

 

So I think in addition to your having a normal cardiac check-up, a lot of these patients for the Fontan end up following up with a hepatologist. We usually work hand in hand with these cardiologists to follow up on these things. All the different lesions have their own things that long term you have to watch, but specifically with hypoplastic left heart. Any of the single ventricle physiologies which can be left or right-sided heart lesions, if you have a Fontan, you really do need a lot of follow up long term.

 

1:04:08

 

So there's a lot of uncertainty with it, too. Like my heart will not forever be able to, my right ventricle will not forever be able to sustain what it's doing, that's just the fact. This physiology is not supposed to be doing it. It's amazing it's been doing it for 30 years. But it's not going to be something that's going to be probably till I'm 100. So that's going to be something that has to be watched.

 

I think mental health is another thing that is never talked about. I want  point out that out because a lot of these patients don't ever get plugged in to the mental health professionals. And I think personally, anybody with the congenital heart disease, especially critical congenital heart disease should probably be plugged in with the psychiatrist or therapist or something before the age of ten, I think at very young age, because you went to all these open-heart surgeries, you have PTSD.

 

Parents, I guess speak about that. That's a whole thing in itself. But for these children, they're going through all these procedures. They've been stuck for IVs and procedures, imaging. It's never ending.

 

1:05:08

 

And I think getting them plugged in early is better because I'm 30. I've never seen a psychiatrist or a therapist. Luckily, I haven't had any of the serious complications like depression and anxiety, PTSD, any of that.

 

But there's a lot of people out there that do and they're not getting the help that they deserve or need. And that's I think almost as big of an issue as not following up with the cardiologist. So I just like to raise attention to that, but that needs to be model of care.

 

Dr. Mike Patrick: Yeah, absolutely, very important. For those who have adult congenital heart disease or know someone who does, we also have a podcast that we did on that. That was PediaCast 307. That was with Dr. Curt Daniels who really is a trailblazer in adult congenital heart disease. I mean, he's internal medicine in pediatrics and kind of one of the pioneers in this emerging field of taking care of adults with history of congenital heart disease. So Episode 307, and I'll put a link to that in the show notes.

 

Tawanna, you had another point you wanted to make?

 

1:06:11

 

Tawanna Nishibayashi: Yeah, just to piggyback on what Tom was saying. So Avery is four and a half. And I think we're going to get her in with a behavioral specialist. And our program now has a neurodevelopmental program starting five that we're going to plug her into, to help with everything from being late in jobs to school by getting her in with the psychologist.

 

She definitely has an executive functioning issue which a lot of these children who experiences this does. She potentially has ADHD. She goes from 0 to 100. She was never allowed to cry. So she never learned how to cry it out.

 

So now, I've got a four-and-a-half-year-old who gets really frustrated very easily and cannot dial down because she doesn't have those coping mechanisms. But now, she gets to function like in school and in real-life circumstance and even just day-to-day with her parents, learning how to listen and follow directions and not have these insane meltdowns and get so angry.

 

1:07:09

 

And so, we need to meet her where she's at developmentally and also partner in and get her some assistant so that she can learn to navigate these things before she's well into her school years and gets older. And definitely, I think for the parents, and it's got to be for the patients, it goes beyond PTSD to CTSD, which is like that chronic. It's chronic what we go through and what we experience.

 

And so, for Avery, she is living her normal life but we got labs this morning. And we're missing school on Friday because she's got a clinic. And then, a couple of months later, she's got a car. And sometimes, she's not able to do things. At one point, she had medications at school. And so I was taking her into school a little bit later so that I can give her medication at the door so she could get that on time, and those differences on things.

 

And sometimes, there's bullying that comes along with that, kids not understanding someone that's different. And so, the kids really do need the support level, as early as you can get even if it's play-based kind of therapy would be my recommendation. So be on the lookout for those things that transcend beyond normal traditional toddler tantrums, to really kind of look at the differences with your child and partner with someone as soon as you can which is usually about the age of three.

 

1:08:22

 

Dr. Mike Patrick: Really great points all around. And this has been a fascinating conversation. I feel like we could go for another hour pretty easily. But it is time to call things to a close. Just really appreciate both of you and your honesty and openness about all of this, really both of you are incredible people.

 

Dr. Thomas Glenn, again, pediatric cardiology fellow at the Heart Institute at Rady Children's Hospital in San Diego, California. And Tawanna Nishibayashi with the Children's Heart Foundation and Race Equity Solutions. Thank you both so much for being here today.

 

Dr. Thomas Glenn: Thank you very much. I had a great time.

 

Tawanna Nishibayashi: Thank you.

 

1:09:00

 

[Music]

 

Dr. Mike Patrick: 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 a part of it. I really do appreciate that.

 

Also, thanks to our guests this week, Dr. Thomas Glenn, pediatric cardiology fellow at The Heart Institute at Rady Children's Hospital in San Diego, California. Also, Tawanna Nishibayashi, Volunteer Coordinator with the Children's Heart Foundation and Race Equity Solutions.

 

Don't forget, you can find PediaCast wherever podcasts are found. We are in the Apple and Google podcast apps, iHeartRadio, Spotify, SoundCloud, Amazon Music, and most other podcast apps for iOS and Android.

 

1:10:06

 

Our landing site is pediacast.org. You'll find our entire archive of past programs there, also show notes for each one of our episodes, our Terms of Use Agreement and that contact page if you would like to suggest an upcoming topic for the program.

 

Reviews are helpful wherever you get your podcast. 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, Twitter, LinkedIn, and Instagram. Simply search for PediaCast.

 

Also, don't forget about our sibling podcast, PediaCast CME. That stands for Continuing Medical Education. Similar to this program, we do turn the science up a couple of notches and offer free Continuing Medical Education Credit for those who listen. Category 1 Credit by the way. And that includes credit for doctors, nurse practitioners, physician assistants, nurses, pharmacists, psychologists, social workers, and yes, even dentists.

 

And since Nationwide Children's is jointly accredited by many professional organizations, it's likely that we offer the exact credits you need to fulfill your state's Continuing Medical Education requirements.

 

1:11:08

 

Shows and details are available at the landing site for that program, pediacastcme.org. You can also listen wherever podcasts are found. Simply search for PediaCast CME.

 

Thanks again for stopping by. And until next time, this is Dr. Mike saying stay safe, stay healthy and stay involved with your kids. So, long, everybody.

 

[Music]

 

Announcer 2: This program is a production of Nationwide Children's. Thanks for listening. We'll see you next time on PediaCast.

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2 thoughts on “Congenital Heart Disease – PediaCast 483”

  1. At first glance, I wondered why representatives from the NCH Heart Center were not interviewed but it didn’t take long to see why Tawanna & Dr. Glen were selected. They are amazing! Thank you for your work and selection!

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