Angelo Milazzo, MD (Host): Hello, and welcome to Pediatric Voices, a conversation with the people who make up the healthcare team at Duke Children's Hospital. I'm Dr. Angelo Milazzo. Today, I bring you a very special conversation. Today, I'll be talking with one of our patients, Ms. Adeline Dinin. Adeline has a fascinating story to tell us.

It's a story about a rare and serious diagnosis, how it was discovered, how it can be treated, and how this has impacted not only her life, but the lives of her family members and the lives of the people who've been taking care of her here at Duke Children's. Her father, Dr. Aaron Dinin, is joining us today for the conversation.

Aaron's medical journey is woven closely to his daughters, and we'll explore that in the discussion today. We're also joined by Dr. Salim Idriss, who is a friend and colleague of mine. Dr. Idriss is a pediatric electrophysiologist, and I'm going to let him explain what that is in a moment. And he works here in the Pediatric and Congenital Heart Center at Duke Children's Hospital and Duke Health. He and I have been colleagues for many years, practice partners for many years, and he has taken care of many patients with diseases that impact the electrical properties and the electrical function of the heart. And again, I'm going to let him explain that, because he'll do a much better job than I will.

Welcome Adeline. Welcome Aaron. Welcome. Salim. It's a pleasure to have all of you here on the Pediatric Voices podcast. Aaron, I want to start with you, and then, we'll bring others in to the conversation as things move forward. If you could take us back to the beginning of your family's story, which I know begins quite early in your daughter's life.

Aaron Dinin,PhD: So, we were scheduled to have a baby sometime in November of 2015. And, on Halloween evening, my wife went into labor. My goal was to have a Halloween baby. But it was not to be. Adeline came the day after Halloween. . So, during the pregnancy, there was one nurse thought she heard something weird, in a prenatal visit and we went to a specialist and the specialist checked everything out. Everything seemed perfectly fine, nothing to concern with.

And then we, I said Adeline was born November 1st and she came out and she was a beautiful eight pounds, something ounce looking healthy, great looking baby girl. And it was our first child, you know, being a dad for the first time, all the good kind of dad things. But I guess there was an issue. She wasn't keeping her temperature up.

And they weren't sure why, but, you know, it was a fairly normal thing. And they, but eventually, it seemed like, in the middle of the night, like at 3 o'clock, 4 o'clock feeding, a nurse thought she heard something strange. And so they came in, they brought in a specialist to check on her.

We were at a local hospital. We were at Wake Med in Cary, North Carolina. And they heard an arrhythmia and they kind of told us, it wasn't anything unusual, particularly for a newborn to have arrhythmia. Sometimes it happens. They weren't overly concerned. They wanted to just send her to a bigger hospital where they could watch her a little closer.

And so we said, that's fine. We asked if she could go to Duke because I happen to work at Duke and I'm a Duke alum. Our doctor, attending was from UNC and they were going to send her over there. And, and, you know, with the Duke UNC rivalry, I was like, no, no, let's go to Duke because that's where the good people go. Obviously realizing UNC is a fantastic hospital too, but it was more convenient to go to Duke. So, they sent her to Duke. My wife couldn't go because she had just given birth, obviously. And so I, it was kind of my first fatherly duty. And so, but they took Adeline in an ambulance.

I, I imagined like how else would they get her there. And I didn't think much about it. But, just went over obviously to be with her, but they assured us they didn't think anything major was wrong. And, then I got over to Duke Hospital and they brought me back into the pediatric ICU and I remember walking through the pediatric ICU and seeing all these big machines, all these little tiny preemie babies and thinking, Oh, this is weird. And then there was Adeline, beautiful full term baby girl sitting in her, you know, just a simple bassinet.

And I was like, Oh, I guess everything's fine. And then Dr. Idriss was there and that was when I met Dr. Idriss and Dr. Idriss kind of explained to me what was going on and that Adeline, he explained, had something he suspected, because of course that term, we didn't have a confirmation, but he, you know, kind of used the information he had available to say, I, you know, I think Adeline has something called Long QT syndrome, and Long QT syndrome is, and of course he can explain it much better than I can, but Long QT syndrome is a sudden arrythmetic death syndrome, which means, you know, there are things that can trigger Adeline's heart to go into an arrhythmia.

And we needed to be aware because if she went into that arrhythmia and it wasn't treated, it would kill her. And so he kind of explained this to me and he explained why there was a big, you know, there were the AED shock panels sitting right there and there were so many nurses kind of hanging out.

Uh, because apparently her heart was going in and out of this arrhythmia called Torsades de points, which is very common for Long QT. And so, he explained all this to me and I was trying to take it in. And I, of course, again, this was my first 12 hours of being a parent.

So I had no idea what I was hearing. And then, you know, he asked, you know, uh, Mr. Dinin, do you have any questions? And I had no idea what to say. And you said, well, I got one more bit of news for you. You know, that's, that wasn't the language you used, but you know, there's something else I have to tell you.

This is a genetic condition and based on what we know of you and your history, we think you would've given it to her. I gotta tell you, we should probably get you to a doctor too. And so that's when I discovered that I had the same condition, and it hadn't impacted me yet, but it probably would.

And so they started treating me as well, pretty much in the same afternoon. So it was a big day.

Host: Aaron, you mentioned your relationship to Duke and, for our listeners, Aaron has a PhD. Can you tell us what discipline it's in and how much of that prepared you for understanding what you were learning about her condition and your condition at that early stage?

Aaron Dinin,PhD: Oh, sure. Uh, yeah. So I appreciate that you gave me the fancy doctor title, but I have a PhD in English literature, so I'm pretty much prepared for metaphysical strife, which is probably pretty excellent for this particular disease because it's very much existential, as much as it is anything else.

So, a PhD in English literature, and then I'm an entrepreneur as well, so I teach entrepreneurship at Duke now. I've been teaching entrepreneurship this is my 10th year teaching entrepreneurship at Duke. So, I'm entrepreneurship faculty at Duke and, you know, in a weird way, it kind of perfectly aligned me for this.

I like to think it perfectly aligned me for this because after dealing with the initial push and hit of all this stuff and, and kind of, again, I said the existential crisis of all that, the entrepreneur took over and there's a thing that, you know, and no offense to the physicians on the call here, but doctors have a tendency to make everything sound really terrible. No offense, Dr. Idriss, but you didn't pull any punches, I would say, and I was sure I was going to die within the next 10 minutes as was my child. And so it was kind of a tough few months to get over that, but eventually kind of the entrepreneur side took over and, you know, and the entrepreneurship, we deal with big problems all the time, but the way we treat big problems is we say, you know, big problems are also big opportunities.

And I think a different way of phrasing all this stuff was to say, hey, there's this terrible disease, it's called Long QT Syndrome, and there's no cure, but the first symptom is usually death. And by some miracle, we discovered this in your daughter on the day she was born, so as early as possible, and so even though we can't cure it, we can treat it and because we know about it, that's our best protection.

And so we can give her a much better chance of a long, happy, healthy life because we know about it. Isn't that amazing? And that would have sounded way better. So for future reference, Dr. Idriss, maybe that's how we frame things. and then I would have, then I would have gone, but wait, there's more, here's the big upsell.

Not only does your daughter have this, you know, it actually is a genetic condition, which means one of her parents gave it to you. We think that's you and it means you have it. And by some miracle, it hasn't killed you yet. And thanks to your daughter, we now know you have it, which means we can protect you and put in the infrastructure in place and give you a chance at a much longer, happier, healthy life.

Isn't that amazing? Your daughter is like your guardian angel. And it is. And so I consider Adeline my guardian angel. And so that entrepreneurial opportunity is I think probably what got us onto this podcast, which is Adeline and I have taken this challenge. And we said, well, you know what?

We don't want anyone else to have to discover it this way either. And we want other people to be prepared. And we want to help Dr. Idriss does some incredible work, putting infrastructure in place and AEDs in place and Project Adam. And so what can we do to help spread awareness? And save people because this is a very treatable disease if we know about it.

And so our mission is now how do we get infrastructure in place for ourselves and for more people in the world to, to save lives?

Host: I love the specificity and the expansiveness of that answer. It really gives, I think, an important insight into what this process looked like from your perspective, which, you know, in some respects is the central perspective here because it connects to all the other perspectives as we're going to hear in the rest of this conversation.

So, I want to bring in Dr. Salim Idriss first to introduce himself to tell us a little bit about who he is and what he does here. And then I'm going to, Dr. Idriss, give you an opportunity to connect directly with a little bit of what we heard from Aaron. So why don't we start with an intro? Tell us who you are and what you do.

Salim Idriss, MD, PhD: So, I'm the Director of Pediatric Electrophysiology at Duke. So electrophysiology is the study of the electrical properties of the heart, how the heartbeat initiates, how the heartbeat transmits through the heart muscle itself. Our hearts function in two parts, basically; it's mechanical and it's electrical.

So many of us in Pediatric Cardiology, we take care of a lot of the mechanical parts, but in electrophysiology we have to be taking care of the rhythm and believe it or not in our lives, tenuous balance of making sure our heartbeat is going through our hearts smoothly every moment, when we have over a hundred thousand heartbeats a day, for all of our lifetime. That's what I do. It's, a long training process. I love what I do. It takes a lot of knowledge from the genetics of everything, to what we call the ion channels, which are the little holes inside the sides of everybody's heart cells that let things like sodium in and out that create the heartbeat, all the way up to an electrocardiogram measuring that on the outside of the heart and figuring out what happens, and what was happening with Adeline at the moment. So that's what I do. I've been at it a long time and I'm just hearing and bringing back everything that happened with Adeline and her family. It's, another reminder that we as physicians have a very personal and special role in families that's ongoing. And I'm not an adult cardiologist, so I would just say in pediatrics, that's even more so the fact that, I can be a part of Adeline's life from the moment she was born till now is, an incredible honor and an incredible responsibility that I take very seriously.

So, I think about Adeline all the time. Yes, Adeline, I do. And, as well as many others, related to this.

Host: Salim, thinking about what Aaron was saying, the gravity of this diagnosis, something that, you're dropping in his lap, in his family's lap on the day of their daughter's birth and the significant impact, the psychological ramifications of that. How do you come to terms with that? You know, what is your approach and what do you remember about those first conversations with Aaron and Adeline and the rest of the family?

Salim Idriss, MD, PhD: Yeah, I think, actually I credit you, Dr. Milazzo. You have always reminded me that medicine is an art. It is something that we practice and we practice and we practice. So what I recall from what my discussions were then may be very different from how I approach it now, just with experience with different families.

It's an extremely fine line. There is definitely, in all of us, the importance of getting the message across, that is the no pulling punches part, understanding the gravity of the situation, especially when things, as a newborn, having those types of very life threatening arrhythmias that were happening right then and there, that things could have gone wrong, and, we're in a special, urgent situation of not only preparing a family for something that could happen right then and there, but also going forward with trying to get across without just an absolute fire hose, but in the moment, when you speak to families, when something that has happened, right then, or your child has had a cardiac arrest and they're there in the same situation, in an intensive care unit, unconscious, and you have to get the message across.

And we walk a little line and, you know, we walk a line because there is always a first reaction of somebody in front of you when you're telling them something as serious as this. I fully respect and understand that families undergo and will always have a certain degree of trauma related to that moment.

And it's my responsibility to temper that moment, to temper that trauma, but not ignore it. And so like I said, it's a difficult line. And it takes time on our part. You can train so much, but there's just an experience factor you have to go through, to make sure that we gently approach the message without sugarcoating anything.

Because it is serious and it was serious and I'm thankful where we are. That being said, on the caregiver side, I also say that us as caregivers, we also have a certain degree of memory and I don't want to necessarily say trauma, but, I certainly know exactly where Adeline was in the hospital, the moment I met her, I know exactly what room. I picture it in my mind.

I have a photographic memory. I know exactly in the NICU, for her and, that's how it is. It's imprinting. So it's, a very serious subject, but I'm thankful that I'm sitting here looking at Adeline right now.

Host: I think that's supremely well said. There is a very difficult line that we must tow. And as you said, it is medical practice. We call it practice for a reason. We're always learning how to navigate that line. And, none of us are fully expert in it. We are all still learning as we do. And it's a, it's a privilege to be able to take part in that learning. So I want to bring in our guest of honor. I want to bring in Ms. Adeline. Adeline, when I meet new patients for the first time, and I'm a pediatric cardiologist too, just so you know, I'm not an electrophysiologist like Dr. Idriss, but I'm also a cardiologist and I meet lots of new patients each day in my practice.

I like to start with the basics. So please tell the audience, who you are and how old you are.

Adeline Dinin: I'm Adeline, and I'm nine years old.

Host: And Adeline, what grade are you in in school?

Adeline Dinin: Third.

Host: And the final question I have for you before we get to today's subject is, what's your favorite thing to do for fun?

Adeline Dinin: My favorite thing to do for fun is play with my friends.

Host: Very good. And do you have a lot of friends, Adeline?

Adeline Dinin: Not too many. Two in the neighborhood, and a lot outside.

Host: Very good. Well, thank you so much for that introduction. So Adeline, what I want to ask you first and foremost is what has it been like to be a patient here at Duke Children's? You know, what has it been like to be treated by Dr. Idriss, but also what has your overall experience been like?

Adeline Dinin: I don't exactly remember that because I was a baby, but, I do remember now that I see Dr. Idriss every six months, I do a call in every three months and they help me a lot and that's why I'm here right now today. So like when I hit there, my pacemaker I can call and check in any time I need.

Host: So, I believe what I just heard, Adeline, is that you have a pacemaker. Can you tell us a little bit about what it's like to have a pacemaker?

Adeline Dinin: Having a pacemaker, you can't really feel anything, it's just implanted inside you, but you know it, and you know it helps you, so you feel really nice about it.

doesn't hurt at all, you can't

Host: And there may be people listening who are really curious about what it's like to have a pacemaker. Can you tell those people, whether it hurts or not?

Adeline Dinin: Having a pacemaker doesn't hurt at all. You can't even feel it. I do have some limitations on activities, like no swim team, no gymnastics, and and not getting hit in the device.

Host: So what I think you're saying is that you have some limitations on your activity and you gave us some examples of that. But other than that, would you say that with the pacemaker you can still do most of the things that you like to do?

Adeline Dinin: Yeah, I can do a lot of the things I love to do still, like dance and Girl Scouts. I take medication every day, too.

Host: So the medication that you take every day, does it have any side effects that you don't like? Or is the medication okay?

Adeline Dinin: I've been on it since I've been born, so I don't know. It makes me feel cold really often.

Host: But again, like the pacemaker, even though you're on this medication and maybe there are some minor effects, would you say that it doesn't prevent you from doing most things that you want to do?

Adeline Dinin: Yes.

Host: And the reason I ask is there may be other children listening who either are on medication or may have to go on medication and they may be scared about that. What would you tell a friend or another child who had to take medication every day? What would you say to that person?

Adeline Dinin: It doesn't bother you much. It depends if they're taking like a liquid or a solid.

Host: And what is your medication? Is it a pill or is it a liquid?

Adeline Dinin: Pills. So, I drink some water and swallow with my pills. My dad bought me some Tic Tacs, and I started learning by those. They're a great way to start pills. The mint Tic Tacs, they taste like the pills that you have to swallow. I started with different ones, the good flavors.

Host: Tell me about going to school and being a child that has a medical issue and has a pacemaker and is on medication. How do you feel when you're at school with your friends? Do you feel different or do you feel, like most of the other kids? And, does your medical condition change the way you enjoy school?

Adeline Dinin: No, it doesn't actually, it's normal. I feel like one of my friends when I'm at school, my teachers all have to know about me.

And I take my AED to school, but that's like all of the side effects. The things that happen there.

Host: Well, those were great answers, Adeline. I'm going to talk to some of the other folks and then come back to you in a few minutes, okay? And you actually gave me a really great thing to talk about, and that as you mentioned an AED, So, Dr. Idriss, can you explain to the audience what an AED is, why Adeline has one, and probably most broadly, why she has to bring it to school with her?

Salim Idriss, MD, PhD: Yeah. So, an AED is an automated external defibrillator. So, a defibrillator is a device that delivers an electric shock to our heart. It is meant to reset the heart rhythm to normal. If the heart rhythm is chaotic, out of control, then that electricity will take every one of our heart cells, basically reset it to zero, kind of like your computer reset, and then the normal heartbeat takes over.

 An external defibrillator, and especially when considered automated, means that by putting large sort of patch electrodes on the chest, on the skin, that device will sit there through those patches and record the heart rhythm, analyze it, determine if it's a rhythm that is normal or is abnormal that it can be reset.

And then, if it needs to be reset, then the AED will either automatically deliver the shock to do that, or prompt the person standing there to push the button to deliver that shock and reset the heart. The AEDs right now, it's a sophisticated device, but it's kind of a not sophisticated device because it's there to deliver the shock.

But there are important add ons to it, and one really key is that every AED, as soon as you turn it on, it has to talk to you. So there's an audio that tells you what to do, tells you where to put the patches, there are diagrams, if you have it in the field, if it's anybody, an adult, a child, it tells you what to do and it also tells you when and when not to do CPR.

That is a critical piece of this as well, because if the heart rhythm is out of control, that means it's not pumping and CPR needs to be done until the heart rhythm is reset and all AEDs will tell you when and when not to do that. So it's very important if you use an AED, first thing you do is turn it on and it'll tell you what to do.

Host: So we've heard that Adeline is on medication. We've heard that she has a pacemaker. We've heard that she has to have an AED close by. Can you talk a little bit now about her diagnosis, what it is, what it means, and what her prognosis is?

Salim Idriss, MD, PhD: So, Long QT syndrome is basically a grouping of genetic diseases that all affect those channels that are in all of our cells of our heart. Our channels of our heart, are basically like little pores in the outside of every cell of our heart muscle.

And those little pores are very specific. There's one door basically that opens for what's called sodium, sodium ions that we have in our body, one for calcium and one for potassium. And each of those ions has an electric charge. So basically it's electricity. So when the doors open in that one pore, it's very well timed and well programmed. Sodium goes inside the cell, then calcium goes inside the cell, then potassium goes outside the cell, and all of that in and out, in and out, is programmed by our genetics. Our genetics make those pores happen and those timings. So that's what creates our heartbeat. So the way I describe Long QT syndrome to everybody and families and even to our fellows and such, is basically this, our electric heartbeat is like the wave at the beach.

So, basically, when you're standing at the beach, if you've been there, when that wave comes in, you see the big, tall, sharp part, that's the surfing part. And if you're standing out in the water and it crashes over you, you all know that if you're there, that water level is still up there, the wave goes by, but there's still high water.

And it takes time for the water to go down before the next wave comes in. So that is exactly how the electric wave is in our heart. There's the front part of it, which is really sharp and goes through the heart really quickly, but then there's the back part of it that has to go back to normal and reset.

The timing of that reset is based on our DNA, our genetics, which times those pores to open and shut. So if there is a problem with the DNA, if there's a, what we call a mutation in the DNA in a very singular, specific part of the DNA, just one point of it outta millions, and it happens to be the wrong kind of problem in the wrong spot at the wrong time; it will affect how those pores work and it'll affect how that wave comes back to normal. And if that timing of the wave is very, very long, the water stays high for a very long time, that could lead to disrupting the rhythm of the heart and creating chaos in those waves and just basically choppy water.

And choppy water in the heart of the heartbeat is an arrhythmia that's life threatening. And that's how Long QT syndrome can lead to a sudden cardiac arrest. And that is the reason that defibrillation works, is because it resets that choppy water to smooth it out so the next wave can come in. So, an automated defibrillator is meant to do that from the outside.

In many people, we do that from the inside. We actually do, much like a pacemaker, implant a device under the skin and inside the heart that can do the same detection and deliver the same shock inside without having to carry it along. But, while in an adult, that's one decision. It's a lot easier to make that decision in an adult.

In children, who have a lifetime of having a device; it's a much harder decision of when do you do that because things can happen and batteries need to be changed and things like that. So those are the tough decisions. Now, medication itself in Long QT syndrome, in most Long QT syndromes, the more prominent types, medication reduces risk of a sudden event, in some cases up to over 70%.

So it's critical to have medication every single day for life, to reduce the chance of that, arrhythmia happening. The really hard part is, what predicts the next step? What predicts if an arrhythmia will happen? And that's a very, very difficult thing. Humans we are probabilistic. There's probability involved.

And, one person can go into it and the other person can't. And so, those are the hard parts. So, the best approach is to have multiple types of prevention, including medication and if an internal defibrillator is not appropriate, then an external or an AED is appropriate to have along.

Host: We heard earlier in the show from Adeline's father that not only was she diagnosed, but very quickly you surmised that this was a familial condition and in short order, he was diagnosed. Can you talk a little bit about what that means? What having a genetic cardiac disease means?

Salim Idriss, MD, PhD: Yeah, absolutely. So diseases are categorized when they're genetic. They're categorized by being something called autosomal dominant or autosomal recessive. And for example, Long QT Syndrome, autosomal dominant means all you need is one bad copy. We inherit two copies of our genes, one from mom, one from dad.

And if you inherit one bad copy in Long QT syndrome, for example, that's an autosomal dominant disease, that one bad copy can create the conditions for having the disease of Long QT Syndrome. Everybody's disease is different in how severe it is. Everybody, it varies quite a bit. So when we have a child that's diagnosed, you can have a situation where the DNA it's called de novo.

The DNA is that gene had a mutation on its own. Just happened in that one person. And then going forward, it can be inherited in any prodigy after that on a 50 percent basis. You have two chromosomes, you give one of them. So, that also means that if you have a child who has a disease, that means that there's a chance that one of the parents has the mutation. And for any other children, there would be a 50-50 chance that next child could also inherit it, 50-50 separate from the other child having it. So it is not unusual in this situation, just like what happened with the Dinin family, that we go up the tree. We go up the family tree and I take care of families where I'm taking care of the child and then I have another child, for example, that's been discovered

three levels up and over two levels like second cousins. That is because it's an inherited disorder. So discovery is really important. It saves not only a singular life right there. But it saves so many others in the family and it can do that. So that's why discovery and knowledge of this being present is really important.

Host: As we wind down with each of you, I want to ask you a version of, of this question. I'm gonna start with you, Dr. Idriss, and then bring Adeline and her father back into this conversation. What would you, as a pediatric cardiologist, as a pediatric electrophysiologist, what would you want our audience today to take away from this conversation?

Salim Idriss, MD, PhD: I could go down different realms and I'm just going to go down the realm of all that the Dinan family has done related to what was mentioned with Project Adam and what's out there. And I'll go on my soapbox about it a little bit. Basically, there are multiple diseases, not just Long QT syndrome, that can result in a sudden cardiac arrest in children and adults, but I'm going to deal with children, where it's an unknown, it's a hidden, there are no symptoms, that's it.

And then the first presentation can be an arrhythmia. So, we want to have a situation where we have prevention of sudden cardiac arrest in young people, and that's a passion of mine. And there are two aspects to that. One is called primary prevention, where you discover and then you try to prevent. And in Adeline's situation, the discovery was right there, obviously. And then in Aaron's situation, of course, yours was discovery as well. In secondary prevention, though, secondary prevention is after the fact. Secondary prevention is prevention of sudden death after a sudden cardiac arrest has already happened. And so in the country right now, especially with the HEARTS Act that just got passed in Congress and then the Senate was signed by the President, is dealing with secondary prevention of putting AEDs in schools, but equally important to that, AEDs as a defibrillator, but also having the training of personnel in schools and in other areas where children congregate, training to recognize that a cardiac arrest has happened, to do CPR, to apply a defibrillator. That's called an emergency response plan. And a cardiac emergency response plan is critical and very important as well as the AED. So, that's secondary prevention.

And Project ADAM, I run the North Carolina affiliate for Project ADAM, which is a national program which is promoting that. So in North Carolina, we go around to schools that want to participate. It's voluntary, and help them to do CPR and do emergency response plans and AEDs. And we go out and we go to that school and we run drills from start to finish, to try to make sure that people are ready for something that could happen, even though there are no symptoms.

So I guess that's my message is that understanding the importance. And one last part of that is, this, is that again, it's a soapbox, but it's a reality. What's out there in the country right now is what you see on television. And, there's a very strong, strong message that athletes are the most vulnerable.

And athletes are the only ones that can have a cardiac arrest. And that's absolutely not true. And so anybody can do this, anybody, non athletes and athletes alike. So I don't want anybody to shy off from being an athlete or exercising, but we also have to understand that people can have a cardiac arrest, children, who are playing or not even playing, just walking. So, that's my main message.

Host: Thank you for that perspective and also for the work that you're doing. The work of Project Adam, that you are leading is so important for so many reasons, but I appreciate you taking the opportunity to highlight it for us. Aaron, bringing your voice back into this conversation, what do you want families who are listening to this show, what would you like for them to take away from this discussion?

Aaron Dinin,PhD: The big thing we've embodied as a family, and it's a largely been through, through Duke and Dr. Idriss is, not a very exciting word, but it's infrastructure. And, what I'll never forget, right, is after this happened and, you know, and we're in the hospital, is the infrastructure and for lack of a better word, that was in place to help us and support us.

And, that made us feel like, we understood what was going on. It was to teach us. We still actually have the paper, when Dr. Idriss, my wife, eventually came over from where she was, and Dr. Idriss sat down and explained everything. He did like a, for lack of a better word, a chalk talk with us.

He had a piece of paper, and he, like, out the ion channels. He explained everything. And we felt so much more knowledgeable and, you know, and as being scholars, my wife is a scholar as well. And we just felt so much more knowledgeable once we had information and then the infrastructure of support.

Funny story is that my understanding is Dr. Idriss has clinics kind of all around the state. And so he would be not necessarily in Durham during the day. And we were in the hospital for nearly a month. And, he would come even after, you know, being in Wilmington, hours away, he would come and it'd be, you know, nine, 10 o'clock at night.

And he would come in and he would visit us and he would visit Adeline. And he was just dressed in kind of normal clothes. And the nurses thought he was her grandfather because he was there and, you know, it was like, oh, is this your grandfather? I know this is, this is the doctor. But because he was just there and he cared so much and that's just been ongoing ever since.

Right. And so everything that's happened since then, is the infrastructure. Our goal as parents for Adeline is how do we put the infrastructure in place to let her have this amazing, wonderful life and be a normal kid. And she is a normal kid. Even the times I'm mad at you, Adeline, you're still a normal kid, your dad should get mad at you. And so being able to have those moments and those wonderful things is because we have an AED. As we know, we have it, you know, Duke worked, we have doctors just worked really hard to make sure that we were able to get an AED. And they're expensive devices and we were able to get it on insurance and it's there and shows up and he advocates for that, making sure we always have the medicine and, had a good system for taking the medicine and putting it in place.

And that's all been the amazing infrastructure that Dr. Idriss has given us. And so, going forward, I think is normal and good, as long as you take the moments to think about the infrastructure and put those things in place. And so Dr. Idriss has been the most incredible advocate and Duke Health has been just the most incredible supporters for us.

 And then from there, we've just wanted to go out and spread the message because again, I think just knowledge is the biggest protector.

Host: Well, it's a wonderful answer. It highlights the running theme of the show, which is the people make the place special. And that infrastructure scaffolding that you talked about is so important and it only gets activated when you have people like Dr. Idriss who are so committed to the cause, so committed to their patients.

And that's what makes me so proud to be a physician here at Duke Health. Adeline, I want to end the conversation part of today's show with you. I'd like to ask you, if you were speaking directly to other children, what would you tell them about your experience that might help them if they're dealing with an illness or if they're worried about a medical problem? What would you say to other kids who may be listening today?

Adeline Dinin: I would say, I think the best way to deal with this is take it step by step. And doctors aren't scary.

Host: So you're saying take things one step at a time, which sounds like great advice. And you're also saying that doctors like me and like Dr. Idriss, we're not scary. We may seem scary at times. But we're not, right? And that has helped you. Would you agree with that?

Adeline Dinin: Yes, you really care and you really help people.

Host: Well, I think that's fantastic advice for other children. I think it's fantastic advice for their parents and family members to hear as well. So thank you so much for that. That's really fabulous.

Salim Idriss, MD, PhD: Can I bring up one thing Angelo? I just want to thank Adeline, what didn't come up is that Adeline has been a Children's Miracle Network champion and she has been going around the country and Adeline, you are helping so many people by doing what you've been doing. So many iIt's really special.

And I just want to thank you for doing that. I know it's a lot, it's a lot of stardom and doing, you know, videos and doing promotions and being on Cheerio boxes, which I have many copies at home, thankfully. Thank you all, Dinins, for the boxes of Adelines Cheerios, which are awesome. But just thank you. Thank you for all you're doing. It's amazing and helpful.

Adeline Dinin: You just made my day. So the reason we started AAD Girl, because we knew my story was already going to be out there, so we just wanted to record it someplace.

Aaron Dinin,PhD: And to clarify, AAD Girl is, Adeline's Instagram account that her mom and I help to manage, and it's where we do a lot of the advocacy work and, you know, the power of social media to reach millions of people. And so Adeline has had videos about AEDs and Long QT syndrome go viral and reach millions of people.

And it's such unique power because as Dr. Idriss, you know, will say over and over again, you know, it's a knowledge it's letting people know, it's not trying to scare people. It's just when you prepare people and so like the drills he's doing in schools, right?

If you're prepared, then you know what to do in an emergency. And so we've had this wonderful opportunity to be able to leverage social media to tell people, tell millions of people. And we hope by doing that, we can save lives.

Host: Well, Adeline, we will try to give you a social media boost if we can through this show. We'll put links to your social media presence, we'll put all those links in our show notes. And, you know, I want to thank you all, all three of you again. This has been a fantastic opportunity to hear from you, particularly to hear from Adeline.

It's so nice to get direct feedback and to see the impact that we're having on patients and their families. Thank you all for being part of the show. I hope to have you all back soon to hear more about your journey, Adeline, and to hear about how you're doing. So many, many thanks?

Adeline Dinin: Can I add one more thing? Thank you guys.

Host: You are so welcome. And again, we look forward to having you back on again in the future to be our guest. It was a pleasure and a privilege.

Salim Idriss, MD, PhD: Oh, look at that shirt.

Host: For those who can't see, because we don't have video with today's podcast, Adeline is wearing a pink shirt with red hearts all over it, which is the perfect outfit for today's recording. So thank you.

Pediatric Voices is brought to you by Duke Children's Hospital and the Department of Pediatrics at the Duke University Medical Center in sunny Durham, North Carolina. Thanks to Courtney Sparrow, who keeps us on track and organizes our work. Special thanks to Dr. Anne Reed, and of course, to the wonderful people at Dr. Podcasting. You can find the show and please hit the subscribe button wherever you find your favorite podcasts. You can connect with us on all of the usual social media outlets, and also at our website, which is pediatrics.duke.edu/podcasts. We'd love to hear from you. So please leave us a review at your favorite podcast application.

Thanks for being part of the show. We will see you next time.