Transcription:
From Open-Heart Surgery to Ironman: Le Bonheur Provider Shares His CHD story

Bill Klaproth (Host): Ryan Stephens is a Nurse Practitioner at Le Bonheur's Adult Congenital Heart Disease Program. He himself had heart surgery as an infant to repair a congenital heart defect. He's now training towards completing a full Iron Man race. So, let's talk with Ryan Stephens, Nurse Practitioner in Adolescent Adult Congenital Cardiology and get his story from open-heart surgery to Iron Man.

This is the Peds Pod by Le Bonheur Children's Hospital. I'm Bill Klaproth. Ryan, thank you so much for your time. It is great to talk with you. So, let's start with this. Can you explain to us what is a congenital heart defect and how does it impact individuals into adolescence and adulthood?

Ryan Stephens, DNP(c), FNP-C, MBA (Guest): Yeah, it's a great question. For one, you'll often see it as CHD or congenital heart disease. Basically what that means is it's an abnormality of the heart that happened at birth. It's not something that somebody did or over the course of their life, they either you know, smoking or drinking or just sedentary lifestyle something like that that naturally this progression of heart disease occurs. We see that a lot in adults. What congenital heart disease is, baby is born into the world, has something structurally wrong with the heart, most of the time requiring surgery or some kind of intervention to fix it and then that ends up requiring care for the lifespan.

Host: So, you have firsthand experience with this Ryan. So, can you share with us your journey of having a congenital heart defect and what happened as you grew older?

Ryan: Sure, so I was born in mid eighties and had a diagnosis that's called Tetralogy of Fallot. My parents didn't know anything about it. I was born and then started having some issues and was referred to a Pediatric Cardiologist as a young infant and it was determined after some imaging that I had this diagnosis. For most tetralogy patients, that requires at least one surgery if not multiple surgeries. And so I was sent to Birmingham Alabama and had an open heart surgery at nine months of age. I've been fortunate to this point in my life, that that's been really the only surgery that I've had to have. It was a pretty extensive surgery and obviously I still have risks of developing issues later but as a kid, I participated in T-ball and I played Peewee football for a couple of years as a little guy four and five-year-old.

But mainly just played baseball and basketball and then went through my young adolescence going through middle school and getting into high school. Really my only more or less kind of tragedy related to having a congenital heart defect, was when I was in seventh grade I wanted to go out for football. And it was the first time in my life that I actually had to kind of confront the fact that I had this diagnosis because the school wouldn't clear me to play. And for a seventh grader in the state of Alabama wanting to play football, that was pretty catastrophic. Obviously there were tears. I was angry. I was frustrated. My parents were really sad and they did a wonderful job of walking me through that to the best of their ability. I mean how do you do that well, other than just telling somebody that you love them and that you care about them. My experience at that point was more or less I had to realize at that point that I had this diagnosis that was really my first outside of having a scar on my chest. And I have an extra belly button for the chest tube. I never really had to think about it until then. Fortunately that didn't just knock me totally down. I was able to go through the rest of high school and briefly played baseball in college for a year. Stayed active and have been involved in Cardiac Care as a Nurse and now as a Nurse Practitioner. And so for me my journey with congenital heart disease has been for the most part, at this point in my life, you know, knock on wood an uneventful journey as far as surgeries go. But I'm very much involved an around it as part of the way that my career has gone. So.

Host: Yeah that's what you want uneventful. I'm sorry your dreams of being an SCC football star were dashed.

Ryan: Yeah, I would like to think that that was the only reason but have a feeling there were other reasons that that didn't happen.

Host: Right. And I think you made an interesting point earlier, with most children, you don't know that they have a congenital heart defect when they're born. Is that right?

Ryan: Right. Exactly. It's one of those things that for a lot of our patients in the first probably 10, 10 to 15 years of life, the care is as much for the parents as well, when they come in to be seen these Pediatric Cardiologists and our Heart Institute is just loaded with uber talented men and women that are caring for these kids and their families. And a lot of the education and teaching is going to these families because most of the parents and grandparents and aunts and uncles, they have very limited knowledge of what these defects are and what they mean and really it's in the teenage years that these patients start getting asked to kind of learn a little bit themselves to digest some of this material and understand a little bit more about their own care. So, it's definitely one of these processes that is lifelong and ongoing. It's a continual education.

Host: Yeah. You said you were operated on as a nine month old. That's gotta be terrifying for parents. So, we totally understand that. And you talked about as a teenager ages, the time for them to start understanding this. So, then why is it so important to have lifelong cardiac care for a congenital heart defect?

Ryan: One of the things that kind of happened, there was right or wrong or indifferent, I think there was some language used some decades ago that these patients would be quote cured after they had these surgeries. So, that obviously creates a narrative that once someone has surgery that they're better, that they don't have any problems anymore. And to an extent yeah, that's true. Their primary issue is it has been intervened or surgically repaired. However that language is very misleading because after having a surgery, it's not like having a knee replacement or a hip replacement or having your tonsils removed. Once you've had surgery on the heart where the heart muscle has been cut on and where structures within the heart, true open-heart surgery, that increases your risk for having issues later as you grow and develop. And many of our patients have had to have secondary surgeries related to valve replacements or heart rhythm issues that commonly occur as we age after having heart surgery. So, it is critically important that our patients understand that their diagnosis while it might have been treated and corrected, they have not been cured and that any pause in their care can be very very detrimental to their long-term health. And so we as an Institute at Le Bonheur have really, really tried to reinforce and emphasize the importance that yeah you're doing okay right now, and you're 18 but we want you to be doing this well when you're 58, 68, 78 and want to reinforce that, that requires continual followup and staying in regular care.

Host: Yeah that makes sense. So, do you go in for yearly heart checks then?

Ryan: I do. So, I'm seen and managed at Le Bonheur by our Adult Congenital Heart Program.

Host: Okay. So, let's talk about that program. When it comes to parents and children in the process of transitioning from pediatric to adult heart care for CHD, can you explain to us what that process looks like and how does Le Bonheur's ACHD program facilitate that transition?

Ryan: Yes. So, the American Academy of Pediatrics recommends that transition education, this is across the board not just in congenital heart disease; that transition education should begin at the age of 12. Within our Heart Institute, we kind of hold that line of 12 to 14 as our initial starting age. And what I tell our Pediatric Cardiologists most of the time and what we reinforce within our adult program, is we really want to see them one time face-to-face in the clinic between the ages of 16 and 18. And the reason why we do that and the reason why it's so important is for one, as you alluded earlier, having surgery as a small child, these parents for the most part, and patients have developed really, really strong trust with their Pediatric Cardiologists. They've been through the hard times, the good times. They've trusted and developed this rapport that is really hard to replace and really hard to move away from.

And so what we really want to do is for one, just kind of let them know who we are, create some trust, create some recognizability to let them know hey this is the new person that you're seeing. This is the new team that's taking care of you. And also at that time too, they're at a developmental age were talking about some of the harder realities of long-term, lifelong cardiac care can be internalized a little bit more effectively, than trying to do that with a 10 or 11 year old or a 12 year old kid. A patient is 10 or 11 years old, we're not going to be able to try to engage them the same way we can when they're 16 to 18 years of age. So, one of the things we really like to do is meet them. And I do a lot of these encounters with our teenagers and what I do when I go in as I ask them do they know what their diagnosis is? Do they know what their medicines are? Do they know when they had their surgeries? Do they know what kind of surgery they had? And one of the main reasons I'm doing that is not to create stress or to worry the patient or give them extra anxiety; but I really want to kind of get a feel for what they understand. Because that really helps us when we start assuming their care to know where they are maybe just from what they understand and also kind of what's been communicated to them, what their parents have been telling them, all those types of things.

But then also to kind of empower them a little bit too, because a lot of our patients at this time, you know, within this country are in this dynamic phase of movement. Many of our 18 to 19 year olds are going to college or moving to different cities and they're going to be around totally different social circles and away from these secure places with their parents or loved ones that are here, that understand their care. So, I really like to be able to meet with them and make sure that they understand at least enough where if they were in a bad spot or they end up feeling a certain way or worried about something and they're three four or five hours away from us here, that they have the resources and the capacity to be able to provide the self-care they need to keep them safe. We really like to start that, obviously the American Academy of Pediatrics 12 years of age.

Within our Heart Institute, we really focus on getting that started around 14 years of age. And then we teach them progressively in a structured way from ages 14 to 22. And then what we try to do is if they're going to be here locally, we try to transition them into the adult clinic by the end of that 22 year and or if they go off to school and come back, many of those patients will be 24, 25, 26, we'll kind of assume their care at that point. And that's done in tandem with the Pediatric Cardiologist and the family. It's a very collaborative process really are trying to avoid that bandaid kind of oh well here you go. You got to go down this hallway and we'll never see you again type deal. We really want it to be a progressive, staged, planned, understood process so that it's not traumatic for the patient, traumatic for the family. And we keep that kind of continuity going without it being lost to care.

Host: Yeah that's really important. It sounds like this program to facilitate that transition at Le Bonheur is fully thought out and this person is cared for in that transition from being a child into an adult.

Ryan: Yeah it's very strategic. I mean that's one of the things that when Dr. Waller, Dr. Rush Waller who's our Medical Director, he was basically doing a lot of the adult congenital care within the Le Bonheur Heart Institute kind of by himself for several years. And when he hired me in 2017, that was one of the main things that he foresaw me doing is bringing me in to make sure that our kiddos that were in the pediatric clinics are getting the education they need and getting directed, kind of air traffic controlled into the right clinics whether they're in Memphis if they're here and this or helping them find these clinics in other states and other cities to make sure that they stay in good care. He's brought on our other ACHD cardiologist, Dr. Benjamin Hendrickson and then our electrophysiologist, Dr. Gary Joshi and Dr. Hendrickson. They're both a part of that process as well and understand the importance of making sure that these patients are getting into our clinics in a coordinated and kind of planned and strategic way. Our Chief and Co-Chief Dr. Towbin and Dr. Johnson have been very very instrumental and supportive in helping us make that a reality. And we've been acknowledged by a lot of different programs across this country and then Canada for our process as being a very novel approach that many people are trying to figure out a way to reproduce. So, we feel really good about what we're doing. And we feel like it's helping our patients and families to have the care that they need.

Host: Yeah, what a great program. And then looking forward into adulthood, what types of precautions do adults need to take when participating in extreme activities like sky diving or mountain climbing or can you tell us about your Iron Man training?

Ryan: Yeah. So, I'll start with just the basic stuff that we tell our patients. So, the unfortunate thing is each patient's different. When we look at adult patients kind of in a general pool, a lot of our patients, what we are really reinforcing with them is you, myself, the people that have gone through what we've gone through, you've gone through that to have life. And we want you to not just have life, we want you to live, right. And so that looks very different for a lot of people. For some people it's simply being able to walk their dog up and down the street and maybe go on a trip driving trip or something like that. For others of us that looks a little different. Some of us want to skydive. Some of us want to scuba dive. Some of us want to do silly things like an Ironman triathlon. So, but what we tell each of our patients is, if you're going to do something like that, it requires some workup and some strategy. So, we don't like to just totally remove spontaneity but many of our patients need to have some type of evaluation or some type of conversation with our team before they consider doing something like that. Because there might be things that they haven't thought of. The stress related to the adrenaline that you get from falling at altitude. I'm not necessarily worried about a sternum or something breaking from that or pressure from that but it's more about the dynamic things that happen within the body and then also altitude creating opportunity for blood clots and other things like that.

So, the short answer to a much bigger conversation is, is that what we want our patients to know, is we want them to be able to do the most that they possibly can with the body that they've been given. But we also like to be able to give them the information on the front end so that we're not having to talk to them about it later when they're in the hospital because of a problem with it. For me I have just learned and understand for myself that I think that I'm capable of doing more than I think I can. And I think for a lot of us, whether you have a congenital heart defect or not, a lot of this I can't do this or I don't think I can do this, really is between the ears. It's not between the ribs. And so one of the things that I've taken upon myself in one way not because of external pressure but just because of personal desire, is I really want to push it a little bit. And I've talked with Dr. Waller about this and talked to Dr. Hendrickson and talked to Dr Gary Joshi. I have the benefit of being in constant contact with my actual primary team because of my working relationship.

But I just floated this idea to them a year ago and they all kind of looked at me like I was crazy but not because I had a heart condition, but just because why would you put yourself through something like that. For me, I'm married and my wife has obvious concerns about me doing things like this. I have two little kids. The one thing that Dr. Waller tells me all the time is you understand what's going on with you. You're going to have to be your best advocate and understand how to self-limit and how to report things that are happening. And so that's the same thing I would tell our patients is we want you to be active. We want you to exercise. We want you to experience life. And experience life to the fullest, but you also have to tell us if while you're experiencing life to the fullest, something happens so that we can address it quickly and make sure that there's not something kind of creeping in that we didn't know about.

Host: Right. Well when you say addressing something that's happening, you mentioned you are your own best advocate, what are the warning signs that adolescents and adults with CHD should look for that means hey something's quite not right here, time to see the cardiologist?

Ryan: That's a great question. So, the thing we talk about each time that when somebody comes in would be the same thing I'd want to know about whether they're in the clinic or not. So, if you have any kind of chest pain, if you're having any type of shortness of breath or trouble breathing at all, especially with simple things like you just walking around the house or normally you don't get short of breath going up one flight of stairs. That's obviously kind of something that's different or new. If you're getting dizzy. If you're getting lightheaded. One of the biggest things we know about is your heart rate racing. You feel like your heart is beating way too fast for somebody that's just sitting down doing nothing and if your heart is racing, is something else happening? Are you getting lightheaded? Do you have chest pain with that and then if you pass out at all, like if you faint for any reason, that is a really, really good thing to tell your cardiology team. It might make perfect sense you know, you might've had a blood draw donating blood or blood shortages all over the country donating blood because of COVID. And you get up from donating blood and you pass out and it makes perfect sense, right? But that's still something that we would want to know about. And then the other things that we talked about too is if somebody is laying down and they're having trouble breathing that's another thing that kind of would tip us to say hey this is what's going on.

The only thing that we tell our patients about and this is primarily for our female patients is pregnancy. So, if that's anything that's on the horizon at all, birth control, pregnancy, you're in a committed relationship or you're just wanting to have a baby; that's something also that would not necessarily be a warning sign, that is something that definitely requires a little bit of forethought and planning for our patient population compared to someone else who doesn't have congenital heart disease. So, those are the main things that we would really want to know about if somebody is experiencing those as new symptoms as a teenager or an adult patient.

Host: Right. Yeah really good stuff. And thanks for sharing all that Ryan. Really important. So, then last question and thank you for your time. So, what encouragement or advice would you give to children and teens who are learning to manage CHD for the rest of their life?

Ryan: One of the most helpful things that the Pediatric Cardiologist told my parents, and I think it's really true even today; is he told my parents to not make me a mental cripple. And what he meant by that was he wanted my parents to not treat me like I was a piece of glass that would just shatter if I did anything. And for our patients, one of the things I want them to understand is that yeah you've been through a lot and you will go through a lot, but you have a lot to live for. And so, when you are planning your life or thinking about the things you want to do, don't put limits on what those goals are. Talk to your team about it. Talk to your family about it. But don't just assume that you cannot do that because you have this scar here. Ask questions. Be curious about those types of things. Be engaging. The big thing that really would emphasize for our teenagers is start getting curious about your diagnosis. Just like you're curious about everything else in the world at that age. Don't just come in and arbitrarily sit and let your parents answer all the questions or ask all the questions. Engage your team. Ask about your diagnosis. Ask about your medicines. Why am I taking this? Why do I need this test? Why do I need to see this other doctor and also to the parents, allowing the teenagers to kind of experience the tension by themselves is critical. To allow them to maybe have a visit with the cardiologist or the NP or the PA by themselves and kind of have to feel that tension of oh man I don't know the answer to that question or oh I don't know where my pharmacy is or I don't know my emergency contact. All those types of things that adults we just kind of have to learn how to do. It's a great way to kind of create a simulated experience. So, yeah it's hard. Yeah, it's lifelong. Yes, it has a lot of negative things related to it, costs, pills, tests, follow ups, all those types of things. But there's also a tremendous amount of opportunity to impact the world positively and to engage your community in a positive way, despite the fact that you got a scar in the middle of your chest.

Host: Yeah, really good thoughts Ryan. Thank you for passing those along. Don't put limits on your goals. As you said, talk to your team, talk to your family. Don't just assume you can't do it. And then get curious about your diagnosis and what's happening. Be engaged with it. Ask a lot of questions. Go see your cardiologist on your own. So, really get into it and understand what is happening to you. Right that's kind of what you're saying.

Ryan: Absolutely. It's invaluable at that age. It would be invaluable for anyone no matter if they had a heart condition or not but to be able to do that in a safe space, with people that you're familiar with would be invaluable in the adult years.

Host: That really would inform you on what you can and can't do or what you can expect as you live through this. So, that makes a lot of sense. So, really good discussion. And thank you for sharing your story. Good luck with the Iron Man and thank you for sharing this great information with us today, Ryan. We really appreciate it. Thanks again.

Ryan: Absolutely. It was a pleasure.

Host: That's Ryan Stephens and please visit lebonheur.org to learn more about the Adult Congenital Heart Disease Program. And be sure to subscribe to the Peds Pod in Apple podcasts, Google podcasts, or wherever you listen to your podcasts. You can also check out lebonheur.org/podcast to view our full podcast library. And if you found this podcast helpful, please share it on your social channels. This is the Peds Pod by Le Bonheur Children's Hospital. Thanks for listening.