Advancing Congenital Heart Disease Care with Dr. Kiona Allen

Experts in cardiac care at Lurie Children’s want to ensure that children with congenital heart disease (CHD) live the healthiest, happiest lives possible. Lurie Children’s unique, comprehensive approach is dedicated to investing in the long-term outcomes of children with CHD. In this episode, Dr. Kiona Allen, attending physician in cardiology and Medical Director of the Regenstein Cardiac Care Unit, discusses key components of a supportive, long-term approach for children with this condition. This includes neurodevelopmental and mental health support, exercise, teaching children to take ownership of their health and helping bridge the gap for those with socioeconomic obstacles. 

“As soon as the surgery is over, physical therapy is back in there helping kids to roll and move and do everything they need to be doing. And more importantly, teaching the parents how to help them. The great thing about our team is that they're really committed to this work. They can see that (post-surgery care) is as important as a life-saving surgery, that the life-saving surgery itself isn't enough unless we're doing these other things.”

Kiona Y. Allen, MD
Medical Director, Regenstein Cardiac Care Unit
Medical Director, Single Ventricle Center of Excellence
Co-Director, NICU-Cardiac Neurodevelopmental Program
Gracias Family Professorship in Cardiac Critical Care
Associate Professor of Pediatrics (Cardiology), Northwestern University Feinberg School of Medicine

Show Notes

• Advancements in treating congenital heart disease in children have come a long way in the past few decades. Dr. Allen notes that in 1983, there was a one hundred percent mortality rate for children with hypoplastic left heart issues, for example. In contrast, today, most children are expected to survive this condition.
• Congenital heart disease, or CHD, is the most common congenital defect, happening in almost one percent of American children. It’s 60 times more common than childhood cancer and twice as many children die from congenital heart defects than all forms of childhood cancer combined.
• CHD can impact children long-term. While more serious cases may be affected by needing multiple operations over many years, others can suffer from developmental and emotional health issues from spending the majority of their early life in a hospital.
• Dr. Allen feels personality invested in the lives of the children she treats, and as a leader, is committed to building an infrastructure at Lurie Children’s to support positive long-term health outcomes.
• When it comes to children with CHD and exercise, doctors in the past have tended to generalize risk protocols, suggesting all children follow the same guidelines of little to no exercise. However, Dr. Allen says protocol should be more individualized to ensure that children who can exercise are not avoiding doing so due to fear.
• Dr. Allen advocates that children with CHD need to learn how to take ownership of their futures when it comes to their health. Children who rely too heavily on their parents will be less prepared as they age, and this can be dangerous.
• Lurie Children’s is one of few programs in the country that offers a neurodevelopmental clinic to support children in their executive functioning skills. The program also works in tandem with schools.
• Dr. Allen is passionate about health equity when it comes to the social determinants of health. At Lurie Children’s, Dr. Allen and her team try to provide as many easily accessible resources as possible to help families bridge the gap between socioeconomic obstacles and the best care for their children.
• While much work has been done to measure the long-term outcomes of children with CHD, Dr. Allen says more work is needed in developing on-the-ground, intervention-focused programs that can truly make a difference.
• Dr. Allen says that her team at Lurie Children’s is truly a family. More than any other place she has worked, she feels she and her colleagues truly try to understand the full journey of the patients and their families.

Transcript

[00:00:00] Erin Spain, MS: This is Precision Perspectives on Children's Surgery from Ann & Robert H. Lurie Children's Hospital of Chicago. I'm your host, Erin Spain. On this podcast, we introduce you to surgeons at one of the country's most renowned children's hospitals to find out how they are transforming pediatric medicine. Advances in surgical innovations over the past several decades have dramatically increased survival rates for children with congenital heart disease. But there is still important care needed after surgery to help these children maximize neurodevelopmental and functional outcomes and optimize their quality of life. Today's guest, Dr. Kiona Allen, is a pediatric cardiac intensivist here at Lurie Children's and joins me today to talk about providing holistic longitudinal care of patients. Welcome to the show.

[00:00:58] Dr. Kiona Allen: Thank you so much.

[00:00:59] Erin Spain, MS: Well, let's start with a little bit about you. What drew you into pediatrics and specifically cardiology?

[00:01:06] Dr. Kiona Allen: I grew up as a gymnastics coach, so working with children from the time that I was very young, since it was a family business. I was able to help out in the gym even before I was old enough to have a real job. And I just always really enjoyed working with kids and I always really enjoyed science and it just made sense to put those things together into medicine. And so I wasn't actually sure what kind of doctor I wanted to be. I just knew it was gonna be something that was kid focused within the field of medicine. And then when I was in college, I actually did research in a cardiac surgery lab. And it introduced me to congenital heart disease and it introduced me to this really incredible population of patients and all the different types of cardiac care that you could get involved in. And I just never looked back. I've been passionate about it ever since.

So as I mentioned there's been so much progress in the surgical treatment of congenital heart disease, but there are still many challenges to congenital heart disease care. So to start off, I really want you to explain the impact of congenital heart defects I mean, I think it's hard to even put this in perspective, but in 1983, every single child with the type of congenital heart disease that I take care of the most, hypoplastic left heart, every single child died. We had absolutely no intervention available to them, surgical or otherwise. Was a hundred percent mortality. These days, it is expected that they will survive. I mean, to go that far in this period of time, I think it's just really incredible and it makes it so that it wasn't until relatively recently that we even had to think about the long-term outcome for our patients. We were just trying to get them to survive childhood, and now they do. And there are actually more adults living with congenital heart disease than there are children. But that means that our responsibility to them is now different. That it's not just enough to say, we did a lifesaving surgery, go be free. That's not what the parents were and trusting them to us to do. It's more than that. It's about. Yes, a life-saving surgery, but with the promise of a good life in the long term. And so we've had to really reconceptualize what our responsibility to the patients and families is. I think very similar to what has happened in cancer care. Right? That in the beginning they just needed kids to survive and now they do. And then we've learned that there's a variety of things that they have to consider in the long term. It's just that unfortunately, cancer care gets about five times the NIH funding that congenital heart disease care gets, even though our problem is more common.

[00:03:34] Erin Spain, MS: Tell me a little bit more about how common this problem is compared to childhood cancer, for example.

[00:03:40] Dr. Kiona Allen: So congenital heart disease is the most common congenital defect, meaning something that you're born with. And it happens in almost 1% of American children. Not every kind of congenital heart defect is severe enough to need surgery, but that means that you for sure know people who were born with a congenital heart defect. Probably more than one actually. It's 60 times more common than childhood cancer and each year, twice as many kids die from congenital heart defects, as all forms of childhood cancer combined. So it's very common and it has a huge impact on American children and children all over the world. So I think it's just that people don't talk about it the same. And it's kind of hidden. You have a scar underneath your shirt. And maybe people don't even know that that's something that you went through earlier in your life.

[00:04:28] Erin Spain, MS: And as you mentioned, yes, like there are scars, there's memories of this surgery, but that's not all that's left behind after a surgery. And as you mentioned, this is something that you're really passionate about, is to help these children have the very best outcomes long-term. Tell me about some of the challenges your patients may face in the months, years and decades after surgery for CHD.

[00:04:52] Dr. Kiona Allen: So as a single ventricle doctor, so I wear a lot of different hats and I think that is sometimes seems a little bit overwhelming, but it actually works really well because I get to see all the different ways our patients and families interface with the healthcare system, because I'm an intensive care doctor, I'm also an outpatient doctor for very complex patients. I help run our neurodevelopmental clinic that follows them long term. And so I'm seeing them in all of these different worlds. And one of the things I love so much about cardiology and congenital heart care is how engaged the community is. So I'm also often at events being sponsored by community organizations and parent organizations. So I even get to see them out in their natural world trying to be themselves with their families. And it gives me a very different perspective about what we've left behind after these life-saving surgeries and what our responsibilities are. So, for example, a patient with complex single ventricle congenital heart disease, which is my primary area of focus, those kids are born with a heart that just is never gonna be, even with surgery, transformable into a typical heart. I can't rebuild missing chambers. I can't make the circulation work the way yours or mine does. And so we kind of, we make do with what we can get and we reroute things so that it can be functional, but it's not perfect. And so then, what we anticipate is that they'll be in the hospital for one to two months right after birth, that they'll have weekly appointments for the first four to six months of life. They'll have a second operation within that first year. They'll need multiple daily medications to control their symptoms of heart failure that we can't totally relieve with surgery. They need feeding tubes. Sometimes they need home oxygen. Most of them will need a third, sometimes even a fourth operation. We believe that in the long term, they may be facing heart transplant, and then that's just the heart piece. Then there's all of the things that come along with having to be in the hospital for a prolonged period of time, and the heart is such a major part of the body that it, you know, is responsible for pumping good, strong blood to all your other vital organs. So you can end up with lung problems and kidney problems. And then there's also just coping. Like if you're in the hospital for the first two months of your life when you're supposed to be snuggled in the comfortable, warm embrace of your parents, like sequestered in your home, right? There's a consequence of that with regard to the long-term sort of developmental outcomes with regard to the long-term, emotional health and resilience of the kids. And certainly it's not hard to imagine how our families could have substantial stress, PTSD, and even beyond that, we actually partner with an organization that has been looking at the financial burden of this, and it's hundreds of thousands of dollars, even with insurance covering a great deal of this due to missed work and the cost of meals out and childcare for your other kids, and just all the things that go into this. And so we really have to build, like, a whole world around them in order to guide them through this journey so that the kids can be the best version of themselves and still have thriving lives despite what they had to go through.

[00:08:03] Erin Spain, MS: So you mentioned that you're able to partner with other organizations, but there are other ways that you're addressing these challenges. Can you just deep dive into that a little bit more for me. How do you and your team address these specific challenges that you just detailed for me?

[00:08:17] Dr. Kiona Allen: So I'm the medical director of the cardiac care unit, and so it gives me a unique opportunity to pounce on some of these challenges from the very first moment. So, as I said, we're already meeting these families. We're providing wraparound services with regards to social work and to mental health support for families, for siblings, even before babies are born. Once they're in the cardiac care unit, we have an incredible multidisciplinary team that automatically consults on every baby that we admit to make sure that while there's a captive audience here, we're working on all of the developmental milestones that we are putting on hold. And that as soon as the surgery is over, physical therapy is back in there helping kids to roll and move and do everything they need to be doing. And more importantly, teaching the parents how to help them with that. The great thing about our team is that they're just really committed to this work. I think they can see that it is as important as a lifesaving surgery, that the lifesaving surgery itself isn't enough unless we're doing these other things. And so that team actually moves from inpatient to outpatient with our kids. And we have an entire dedicated neurodevelopmental clinic that is only focused on the non-cardiac aspects of that care to make sure that they're hitting their developmental milestones, that their family is supported financially and emotionally, and that as they approach school age, that we are partnering directly with the school to make sure they're getting the supports that they need and that we're supporting families through that part of the journey too.

[00:09:54] Erin Spain, MS: You mentioned how personally invested you are in each one of these patients that come through. Tell me about how do you develop these relationships and how do you speak to these young patients and their families in a way that makes them feel cared for and makes them feel like you are invested in their journey?

[00:10:12] Dr. Kiona Allen: I mean, I think that that part isn't that hard actually. So much of this work has been driven by the patients and families because they can't not be invested in it. It's their life, The families have really held us accountable. When we first started doing surgeries, as I said, we thought that we were stamping out disease and saving lives. And even for some of our straightforward, we do this operation, your heart's pretty much back together in a typical way. The research shows that even those children and families face substantial neurodevelopmental and academic and mental health difficulties. Not every child, not every family, but they are certainly at high risk to have that happen. And we just ignored it for a long time and the families came to us and said, no, no, this is not okay. You're missing it. And I think it's been actually one of the benefits of the way social media has evolved, that now families can connect with each other in ways they couldn't before. And so they've really elevated the conversation. Then my job as a leader within the Heart Center is to find people who have that kind of passion. If you find people who want to work hard, who all you have to do is put them in front of the patients and families, the right people will immediately understand the urgency, the need, and I think, they just kind of run with it. I've just been really fortunate to be able to recruit a team that wants to be making a difference. And so they try really hard every day to do that, even when we don't have enough resources or we're tired or it gets hard. I think if you pick the right people, it just happens.

[00:11:48] Erin Spain, MS: Tell me about your relationships with the surgeons at Lurie Children's. How do you interact with the surgeons and how are they able to pass cases onto you and kind of make that full circle happen?

[00:12:01] Dr. Kiona Allen: The cardiac world is different than many of these other fields where there's kind of a surgical team and a medical team. We're just sort of one integrated entity. We are knitted together at essentially every level. Like it's almost hard to tell where they start and stop and where we start and stop. You have to have a tremendously trusting relationship with your surgical team as the medical side. I need them to be totally honest with me about what things went well and didn't go well. What are they worried about? Similarly, then, when I'm managing something postoperatively, I need for them to feel like they can come to me immediately if they don't think things are going the way they need to. And I need to be able to receive that feedback and like, not take that personally. It's, it's like a super weird marriage, honestly. Like, do we always agree? No, but I think we have here in particular with our current group of surgeons, we just have tremendous respect for each other, which makes it easy to navigate what you have to navigate, respect and trust that every one of us is doing our absolute best at all times and working hard, which then even when you don't agree, makes it that you can achieve consensus and move forward together as a team.

[00:13:13] Erin Spain, MS: Another area of research that's opening up is the role that exercise can play in the lives of these children. For many, many years, kids who have CHD and who've had surgery, they were told to kind of tamper down exercise or maybe not work out so hard. But you're finding that, that might not be the best advice. Tell me about that aspect of the care of these children.

[00:13:34] Dr. Kiona Allen: Oh, this is one of my passion projects, so I'm glad you asked about that. There are obviously children who have specific kinds of congenital heart lesions that make it unsafe for them to exercise, but we have been, frankly, historically a little bit lazy about just blanket statementing that for all children with congenital heart disease. We know that a huge part of heart health in all human beings is exercise. It is obviously just as true for this population. You just have to find ways for them to do it safely. My lead physical therapist, her name is Casey Vogel. She's one of the national leaders in rehab and physical therapy for children with congenital heart disease and children who are waiting for a transplant, especially who need special heart assist devices. She's helping to lead a national initiative for the single ventricle population, which is what I primarily take care of about introducing exercise again. And what's hard about it is that we spend years teaching them that like, if your heart feels fluttery, that could be a huge problem. If you feel winded, that could be a huge problem. If you have chest pain, that could be a huge problem. And as a post covid, out of shape, lazy adult, I can tell you that I feel all of those things when running to the bus stop. You have to feel something or you're not exercising. So how do we reframe it? You can't just say no, no, don't be scared of that feeling. You have to a little bit say that. How do we help them understand what's safe and what's not? How do we help them safely push so that they can progress in the journey that is physical activity? For kids, physical activity is their, it's their daily life, play sports and run around outside with your friends, And if part of what's been happening with you is that you're a little bit behind from a motor standpoint because of the things that happened with you when you were young and your coordination's not great and you have some attention problems, which are all things that we see in a much higher prevalence in our population. It's not that easy to play on the team . And so I think that is a huge part of our challenge is to make sure that we're not just saying, go out and exercise. We're helping them to actually successfully ramp up their physical activity and have fun doing that so that they develop a lifelong love of healthy behaviors.

[00:15:47] Erin Spain, MS: I mean, in general, you really want patients to be able to take ownership of their futures. So how do you help cultivate that sense of ownership within them and help them strive to reach goals, especially as they're transitioning into adulthood?

[00:16:01] Dr. Kiona Allen: We know that's a huge risk for our patients. There are a tremendous proportion of patients lost to follow up in that transition and for patients who have important medical needs and medications, they have to stay regular with. The transplant population is a great example of this. It can be a really dangerous time. You don't have the freedom to make the same mistakes that other teenagers and young adults make. It is extra unsafe perhaps, is the way to frame it. And one of the things that we know is a problem for our patients through a lot of the research that we do in neurodevelopmental outcomes is something called executive function. Executive function is like the administrative assistant that lives inside your head. So to get you to things on time and to keep track of your tasks and to triage your day and your working memory and all of that stuff. It often lags behind for our patients. And so that makes that transition even more challenging if that's not one of your strengths. It's harder to take ownership of your health. It's harder to keep track of your responsibilities. And one of the things that's great about the congenital heart community is that the parents are fierce advocates for their kids. We teach them to do that early on. But it means that they may be band-aiding that executive function problem for longer than they should. Eventually mom and dad or grandma or whatever the caregiver is, has to stop being the person who does everything for you. You have to learn to do that for yourself. So we spend a lot of time in our neurodevelopmental clinic trying to objectively measure executive function and to give people strategies and partnership with the school for how to develop those skills. I would say that's probably the biggest thing that we really work on. I think similarly, for things like exercise, one of the things that people always used to say was, don't let them do organized sports, the idea being that, your coach may push you beyond the level that you can be pushed and that the peer pressure of that is potentially dangerous. My argument is that welcome to the world. Like that's what the world's gonna be. Instead of saying don't do organized sports, we should be teaching them how to explain their condition, how to know their limits, and how to partner with the people supervising them to make sure they stay safe and how to be brave enough to stand up to their peer group to do the things they need to do. So I think we have to reframe how we're protecting people from the world that they're still gonna eventually have to encounter. And I think that's part of the problem with it being a largely pediatric disease that now has become an adult one, that we're not prepared for those conversations the way we need to be.

[00:18:33] Erin Spain, MS: Well, it sounds like you are making progress on this front though. How unique do you think the programming that's offered at Lurie Children's is out in the pediatric CHD world?

[00:18:42] Dr. Kiona Allen: There is increasing interest and dedication to this work. But Lurie Children's, I think I've been very proud to be sort of at the forefront of this. We are one of only a few programs in the country that has a neurodevelopmental clinic for child and adolescent patients. So for the cardiac population, our research shows us that they continue to have ongoing issues throughout adolescence and into adulthood, and so we've started with a lot of parent pressure actually developing these clinics for older kids that are focused on a totally different thing, which is now the majority of your developmental progress and your lived life is happening at your school, so we have to partner with the school directly to provide that care. But there's only less than two dozen programs like that across the country, even though there are many, many high level heart centers. We in our child and adolescent clinic, we've seen over 700 patients to date since the clinic opened in 2017, and in about a third of the cases, we have to make a substantial modification to what the school is providing based on our assessment in clinic. So a lot of times kids are getting services, but they may not be meeting their needs. And we've been able to really, I think, have a significant clinical impact on what kids are experiencing. And then also because we have this whole patient population, our ability to do the research that's needed to really describe what's happening with these patients is better than I think almost any other program in the country.

[00:20:09] Erin Spain, MS: That's phenomenal. And the kids that are able to participate in this really are reaping the benefits, but unfortunately there's still a lot of kids who are left behind who aren't able to receive these types of services. And this is something that you're also passionate about. That's health equity social determinants of health. Such as a family's social economic status can impact the outcomes of these children. From your experience, what are some of the most significant barriers to achieving health equity for patients with CHD?

[00:20:38] Dr. Kiona Allen: So much of this is outside of what has traditionally been the medical system's hands. The majority of cardiologists are not doing a deep dive into, tell me about your neighborhood, tell me about your community supports. Tell me about your job stability and housing stability and food security. It's not part of what we've been trained to do. And it's not really part of what most cardiologists have time to do, to be honest. And so we've tried to really pick up the slack of that, if you will. We encourage the Heart Center to funnel that to us because we're developing an expertise in how to provide supports and analyze those things. But I think it's not hard to imagine that, if you're just trying to get through the day, seeking out additional supports for weekly physical therapy appointments or even like just to go to the school and talk to the teacher. If you can't take off work, you can't do that. And it doesn't matter how much you want to, whether or not you even know that you should, that doesn't even take into account language and literacy barriers that the parents may have. And so a lot of what we are doing is just trying to make it so that we have thrown as many resources at you as possible and made them as easy as possible to access. And I think in neurodevelopmental care in particular, the health equity influence and the social determinants of health is such a huge part of the outcomes. We know that that's true for the medical outcomes too, but I think it's more intuitive how it's affecting your quality of life and your neurodevelopmental outcome. One of the things that I talk about a lot when I give talks across the country is that many of us are advocates on a national level for not just congenital heart care and funding and research, but also for in my mind as a pediatrician, the social safety net that our children deserve. And so that's something that is sort of a little bit outside of what people traditionally think of the role of a doctor, but in my mind is part of my responsibility. We know that one of the social determinants of health that people talk about is neighborhood and community. If you don't have family supports and you don't have the same community supports that some of our more well-resourced patients have, at least we can be a part of that infrastructure. Even if I can't change what your actual home looks like.

[00:22:53] Erin Spain, MS: What a fabulous resource. So looking forward, there's a lot happening right now in this space. As you mentioned, there's a lot of new research that's up and coming, and there's more and more of these children who are transitioning into adulthood and living with CHD. What is your hope for the future? What do you wanna see happen in the field and for all of these patients who come through your doors?

[00:23:15] Dr. Kiona Allen: I mean, I think that we have done a lot of measuring of the problem, these sort of long-term problems that I've described, and we need to start moving into an intervention focused phase of this journey where we're not just measuring that children are behind or struggling in school, but we're coming up with concrete interventions to help address that. It's difficult, right? Because there's no magic pill for that. It's gonna take hard work and most of these things are longitudinal interventions that require some funding. You know, if you're gonna do a behavioral intervention, it takes continued reinforcement of that and it costs money and all of that is difficult. So I think. That's where we need to double down on our commitment to that work as a field and continue to seek the funding, both from federal sources, but also from philanthropy and from some of the private organizations that have gotten interested in this. I think we need to continue to build up the workforce that's interested in taking care of our now adults who have survived this journey and are moving out into the world because there's a tremendous shortage of adult congenital heart providers. So that's something that we're working on. And then I think it all just starts from a good operation upfront that sets you up for at least, if not success, sort of the least difficulty along the way. And so just making sure that we're continuing to introduce new technology and new techniques to the surgical world so that we're getting kids on the other side who are starting in a better place and who then we can apply these behavioral interventions to, to optimize their neurodevelopmental and emotional health outcomes. I need for our surgeons to continue to do what they've been doing, which is to grow the field at the incredible rate that they have. Again, we went from a hundred percent mortality to way less than that. So if we can just keep moving forward in that way, then that makes it easier for me for what I have to manage on the backend.

[00:25:07] Erin Spain, MS: Well, as we wrap up today, if you could leave our listeners with some advice, why would you suggest that if they have a child with CHD, that they come to Lurie Children's, not only for surgery, but then for ongoing care?

[00:25:21] Dr. Kiona Allen: I think that more than any other place that I've seen, we really have tried to understand the full journey of the patient and family, and I see that in the way that we navigate them through the system. We're really passionate about roadmaps. We use roadmaps a lot to help people sort of understand what to expect. And so it's not like, oh, the fetal doctors do this and then the ICU doctors do this, and then, you know, the adult congenital doctors do this. We're all one wraparound team that's joined arms together to sort of guide people down the path in a way that I think feels different to me than what I've seen elsewhere, which is why I've stayed. It's why I am here. Our inpatient unit isn't just an ICU or a step down unit. It's just one unit. No matter what acuity you are, that's your home and everyone is working together to shepherd you through. No matter how sick you get, no matter how much better you get, you're still just a heart center patient. That's true inpatient, is true outpatient. It's true before you're born. It's true when you're all grown up. It's true about your siblings. It's true about your parents. It's true about your friends. Like we are truly a family and I think the care is better when that's the way you approach it.

[00:26:37] Erin Spain, MS: It's a great way to end this episode. Well, thank you so much, Dr. Allen. It was a pleasure chatting with you about your work and all the things we have to look forward to in the future.

[00:26:46] Dr. Kiona Allen: Thank you so much. It was a pleasure

[00:26:47] Erin Spain, MS: for more information, including how to make a referral or an appointment, visit luriechildrens.org. 

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