Snowflakes: How Each Heart is Unique (Remastered)

The heart is an extremely complicated organ. The muscle on one side of the heart differs from the muscle on the other side of the heart. There are valves that open and close allowing blood to travel to different parts of the heart, to the body and/or to the lungs. The heart has its own unique electrical system. There are structures inside the heart separating different chambers from one another. In babies with congenital heart defects, something goes wrong and there are a lot of places where something can go wrong.

We will discuss the noninvasive and invasive procedures doctors currently use to obtain a diagnosis for children with congenital heart defects. We’ll talk with the mother of a child with a very unique heart and how having that child has affected her. We’ll also meet a nurse who is working on developing a feeding protocol to help babies born with congenital heart defects survive when they are between surgeries.

Transcript

spk_1:   0:00
welcome friends to heart to heart with the Anna. This is your host damage Dworsky and I'm very excited about today's show Snowflakes. How Each Heart Is Unique. This is the seventh episode of Season one, and this show originally aired on December 24th 2013. Today's guests are Heart Mom, Christy Pina with Crusade for Christopher, Dr Gerald Marx with Boston Children's Hospital, and Cynthia Weston, who's the nurse with the UT Health Science Center in San Antonio. We will talk about how each heart is unique, what current tests are done to identify heart defects and how to help our Children born with congenital heart defects to survive and thrive. Now let's enjoy today show and remember, my friends, you are not alone.

spk_2:   0:45
Welcome

spk_3:   0:51
to heart to Heart with Anna, featuring your host Anna Dorsey. Our program is a program designed to empower the CH D or congenital heart defect community. Our program may also help families who have Children who are chronically ill by bringing information and encouragement to you in order to become an advocate for your community. Now here is an ID Gorsky. Welcome to the

spk_4:   1:16
seventh episode of Hurt Madonna, a congenital heart defect community to empower members of our community with resource of support and advocacy information. Today's episode is called No Flights. How each Heart Is Unique. The heart is an extremely complicated organ. The muscle on one side of the heart is different from the muscle. On the other side of the heart. There are valves that open and close, allowing blood to travel to different parts of the heart, to the body and or to the lungs. The heart has its own unique electrical system structures inside the heart of separate different chambers from one another and babies with congenital heart defect. Something goes wrong, and there are a lot of places where something can go wrong. The American Heart Association, or a used to state on their website that there were 35 different kinds of perfect today, December 2013. They state the word congenital means existing at birth. The terms congenital first effect and congenital heart disease are often used to mean the same thing, but defect is more accurate. Her ailment is defensor abnormality, not a disease. A defect results in the heart or blood vessel near the heart don't develop normally before birth. A now has on their website 17 heart defects that they elaborate upon, some with simple diagrams to help illustrate with the heart defects look like they don't include the most common heart defect in your list bicuspid aortic valve. Nor do they include problems with the heart, beating too fast or too slowly for being in complete heart block. Nor do they discuss some of the genetic conditions, which might lead to heart defects like down syndrome on cuties syndrome, or 22 to 11 dot to deletion syndrome. A really mentioned how each heart is unique and at many hearts like my son's heart, have a combination of some of the heart defect, a mentions and others that are not on that list. A. The Centers for Disease Control and Prevention, the March of Dimes and the National Heart, Lung and Blood Institute have websites with very limited information about the different kinds of heart defects Baby might be born with. One thing all of these undertakes agree upon is that heart defect. They're the number one birth defect that the effect over a 1,000,000 people in the United States alone, some of the types of heart defects in mentioned. Some of the possible causes were mentioned, but no one seems to be talking about how every heart, just like every snowflake, is unique. Which is why our topic today is like how each Kardashian ik is so important to discuss this topic. Our guests today or Christie Pain your doctor, Gerald Marx practitioner Cindy Weston Opinion is currently stayed home mom and caregiver to her youngest son, Christopher. It was born in heart failure and given less than six months to live. It lives under hospice for 15 months until the discovery of a miraculous improvement. He has continued today well, despite the odds. Volunteers for both Syndrome Foundation, the Children's Miracle Network Hospital, Blair a. Batson Hospital for Children and other various causes. She helps me families by hearing her knowledge and resources and advocacy. She has written for a complex child, a magazine, and guess block. She's met with various politicians, advocating for congenital heart defects and rare disease. Prince Family makes various appearances for Children's Heart Network, Children's Miracle Network, traveling to Orlando and Washington, D. C. They've also been in countless news article sharing. Their incredible story will meet Dr Gerald Marx the cardiologists at Boston Children's Hospital and Cindy Weston, a nurse, the universe to the hospital in San Antonio later in our show. Thank you, Christy, for being on the show. I

spk_6:   4:58
thank you for having me, Anna,

spk_4:   5:00
but I'm so excited to talk to you. I want you to tell us all about Christopher and how his heart defects came to be identified.

spk_6:   5:07
Yes, that's an interesting question. We anticipated nothing but a healthy child. And when he was born, we were totally taken off guard that he was found to have a congenital heart defect. We never expected that we would be stepping into this world. It looks perfect us, even though the doctors told us that he was really sick. Ultimately, the pulse ox symmetry called the congenital heart defect, showing that his two levels were down and it alerted the doctors to look further. His father and I, we found nothing but a perfect child. He was just beautiful. But in retrospect, we look back at the pictures. It's all very dusty child, and we remember that the first thing that we saw was that he struggled to eat. So that was our first indicator. We live in Jackson, Mississippi, actually, right here at the blurry bats in hospital for Children were very lucky. I could drop to the Children's hospital within 10 minutes. He was actually born in a smaller hospital, and he had to be transferred to the larger hospital when they realized how serious his heart defect was. No one thinks of rare diseases or heart defect affecting them, but it can happen to anybody. And cardiomyopathy was his initial diagnosis. It was actually a symptom of something much larger,

spk_4:   6:16
right? But you said that it was the pulse ox symmetry. And for our listeners who may not know, that's a very simple noninvasive test that some states now are requiring by law. That's why I was wondering what teacher and I just did an episode on Corners Law. And that's what that whole laws about it trying to get postal symmetry for newborns so they don't go home with the heart defects undiagnosed. And so what the simple test says is it measures the amount of oxygen it's going throughout the body so they can determine if there's a problem or not. And so when your baby had postdoc symmetry, you're saying that the numbers were low enough that it alerted the doctors and the nurses that something could be wrong.

spk_6:   6:55
That's correct.

spk_4:   6:55
But we're

spk_6:   6:56
actually quite lucky, because right now in our state, the legislation hasn't passed. Although we've been in the works and Christine McCormick and Core has been a very big inspiration and and also the fact that much I looking so perfect to me that could have very well been our story. We're trying really hard to try to make that happen for Mississippi, as well as the spike surrounding us that have already adopted that

spk_5:   7:17
long. I'm very

spk_6:   7:18
excited just to see how much progress we've made in that because five years ago there were no states practicing pulse ox imagery. So now we

spk_4:   7:27
have one.

spk_6:   7:28
When he say 17

spk_4:   7:31
I think we have 17 that haven't I think all of the other states Aaron one degree or another of accepting that as protocol, and I'm very excited about that. Texas is one of the ones that has, and I went to the state too. Advocate for that, and I'm very excited to say that that is something that Texan babies can't anticipate is having that simple test. If my baby would have had that test, he wouldn't have gone home undiagnosed. We would have known that something was wrong and he wouldn't have gone into congestive heart failure. But it sounds like this was a lifesaver for you, but initially they diagnose Christopher with cardio myopathy, and then they discovered something else. Can you tell us what other tests they ran on him and how his heart defect was ultimately diagnosis?

spk_6:   8:13
Well, after they pound on the pulse oximeter test, that is, levels were to love the next step cardiology tend to do for any person that needs have their heart. Look at that. You could have called the echocardiogram, and it's a lot like an ultrasound, and it just kind of take a look inside their hearts. But I want to say, looking back on cursor for story. Initially, they found his enlarged heart because as two levels were down, so they x rayed his lungs to make sure that they were actually completely developed. And when they did that excellent, they found his enlarged heart which a larger than looking

spk_4:   8:46
for her. Wow. Hey, had a lot of test stand, but he was lucky They started with the non invasive stuff, and they were able to detect it with that pretty much. And so they went into the echo. What did the echo show them?

spk_6:   9:00
Actually, his clinical diagnosis is cardiomyopathy and the type of cardiomyopathy he has a cardiomyopathy, infants. Nobody thinks about it pretty much a rare thing that you think of cardiomyopathy. Can you hear about that? An adult pulling cursed first Casey home, who not only had cardiomyopathy, but he also had a new form that doctors weren't familiar with that had not heard of it. It's called left ventricular, non compaction, and the easiest way for me to describe to people what that means is just think of your hand is the baby's heart, and when you open the fingers up, you can picture them as this is the baby's muscles and has a baby forms and grows, the fingers closed and they form. That tight muscle will increase the first case or others that had the left ventricular non compaction. Those muscles never form IMAX. It insufficient for the blood to be able to pump

spk_4:   9:47
correctly for the body. Wow, so you can totally relate to our theme today how each heart is unique because that sounds like something very special and very wearier,

spk_6:   9:58
absolutely, very rare. I have to say we feel very rare. We're the only ones here in the state with the type of congenital heart defect he has. But third, networking. We've been able to connect with other people that are like us. That's been a teetering Lee, an extreme blessing for us.

spk_4:   10:16
So tell me why you started working with the Barn Syndrome Foundation and the Children's Miracle Network,

spk_6:   10:22
the Children's Miracle Network. They're part of the hospital that we go to to receive all of our treatment. So that called, is really close to our hearts, of course, and then with the Barr Syndrome Foundation. When the geneticist called us with our diagnosis of Barr syndrome, his advice was the best he could have possibly given me, he said. I don't know much about the disease. This foundation is so you need to contact. And so

spk_4:   10:43
from that

spk_6:   10:43
day forward they have been the most amazing support group. What they taught me is that the research scientists gain insight on the behavior of common diseases that all of us have been touched by, like cancer, diabetes and Alzheimer's. They taught me to learn howto advocate for my child, people writing about Barr syndrome. They would picture a child who is gravely ill. When you look at these boys, they're deceptively healthy and you can actually go to the bar center website at the Meet The Boys section is sacred for and all the other healthy and beautiful looking boys and men with Barson teacher so much about the way the disease behave. They connect us. We have a global connection with all the people, even though there's less than 200 effective families in the world. We've been united. There's a foundation. They've been amazing. They have funded all of this research that has benefited, like I said, all of the common diseases. But in addition to that for my child, they're actually on the brink of a treatment for Barr syndrome. There's no cure. They're currently isn't a treatment, but they're about to start a clinical trial, which has given us a lot of hope for the voice because a couple of decades ago they didn't have a name until 1996. But when you heard of this disease, it was 100% fatal before the age of three. They were gonna where now we're encountering men. We were living on their marrying. They're having Children. I want to say the oldest one that we know of a 67 years old. So

spk_4:   12:09
what denomination

spk_6:   12:10
is given it

spk_4:   12:11
and when was that person dies? Moves. But you just said that before my teen honest there was no you weren't And

spk_6:   12:19
it was it was just described. It was described with all

spk_4:   12:22
the physical

spk_6:   12:22
characteristics like cardiomyopathy in neutral Panya. And what Barr Syndrome is. It's an error in one of the genes, and it calls it all. The cells in the body did not work correctly. It makes it where the energy that our bodies need, they don't have it. So it affects everything in their body, from their heart to their blood system.

spk_4:   12:40
They have

spk_6:   12:41
no trip in you, which is a neutral field efficiency, which is what we all use. The fight bacteria and infections that causes them to be very weak. It just causes a multitude of symptoms, and it's a horrible disease if you look at it on paper. But if you encounter one of these boys, they're gonna inspire you and give you hope and know that the disease is a horrible thing. But that is not then that does not define them.

spk_4:   13:06
Absolutely. I mean, your son is definitely a beautiful estimate to the fact that science is starting to learn more about the heart defects and starting to be able to treat them. Like you said, There's no cure yet, but at least they're able to give them more time. And who knows what? That extra time, they may just find a cure. So what advice can you get? Parents who are just now learning how to cut with their Children's unique art? Mike is your child's heart syndrome.

spk_6:   13:34
In my case, I think the very first thing that helped us cope my husband myself. The number one thing was, we had faith when we gave our hope to the Lord and I asked for him to help us with our strength. Because as I was talking with my husband earlier today, that strength could only come from God, but secondly, but not is important because I think that that's the most important thing. But almost as important is participating. Getting out there sharing your story, helping educate and inspire others. You're making a difference just sharing your story because every life is important. And even though Christopher's rare networking we don't feel isolated, you know we have a again the global community for supporting information. It's a really important thing to do that, to find other people like you so you can get involved. You can get informed because the congenital heart defect world and rare disease world this is a war we're fighting and having a diagnosis. It gives us the weapons to help us fight. It makes the fight more fair, and it empowers us. Help our child and others empowering yourself and teaching yourself the advocate for your child. I cannot stress enough that that is the most important thing that you can do for your child, and I have confidence and that if you are your child advocate and you have the right to speak up for them and say, This is my child and I care about them. And don't question the doctor's but also at be involved. Ask why it's okay to ask why, because it's important that you understand what's going on with your child, because without that, you can't advocate.

spk_4:   15:06
That's a little better. My stuff. He did a great job, Kristi, And thank you so much for sharing Christopher story. And you have a beautiful Facebook page for Christopher, and you're doing a fabulous job of being a great advocate and sharing Christopher story with the community. Thank you so much for sharing all about in your advice with us. Now, unfortunately, I have to go to a commercial break, but don't leave yet. Coming up, we have one of the top cardiologist in the country who is going to help us understand each other's unique and what doctors are trying to do to really identify my heart. Develop in such unusual ways to find out what new tests are available to help doctors and who needs those tests When we return to heart to heart

spk_2:   15:45
with

spk_3:   15:55
Hannah Dworsky has spoken around the world at congenital heart defect events, and she is available as a key note or guest speaker. For your event. Go to heart to heart with anna dot com to learn more about booking Anna for your event. You can also find out more about the radio program, keep up to date with CHD Resource is that information about advocacy groups as well as read Ana's Weakly Blawg. Anna wants you to stay well connected and participate in the CHP community. Visit Heart to Heart with anna dot com Today, Anna Dworsky has written several books to empower the Congenital Heart Defect or C H D Community. These books could be found at amazon dot com or at our website www dot maybe Hart's press dot com. Her best seller is The Heart of a Mother, an anthology of stories written by women for women in the CHD community and as other books, My Brother Needs an Operation, The Heart of a Father and Hypo Plastic Left Heart Syndrome. A Handbook for parents will help you understand that you are not alone. Visit baby hearts press dot com to find out more. You are listening to heart to heart with Anna. If you have a question or comment that you would like addressed on our show, please send an email to Anna Gorski at Anna at heart to heart with anna dot com. That's Anna at heart to heart with anna dot com Now back to heart to heart with Anna.

spk_4:   17:34
Welcome back to our show. Hart to Hart Madonna, A show for the congenital heart defect community. Today we're talking with parents Christie Pina, cardiologists, Dr Gerald Marx and nurse practitioners Cindy Weston. We just finished talking with Christy Opinion about her son, Christopher, into unique heart and how parents can empower themselves by learning about their Children's heart defects and how parents can have their Children to understand about the heart defects themselves. Now we will turn our attention to Dr Gerald Marx. Dr. Marx is cardiologists at Boston Children's Hospital and is internationally recognized for his contributions to patient care and medical education. A devoted physician, he cares for Children with the most complicated heart defects. He's author of over 100 and 80 peer reviewed publications, captures of books, maybes and editorials. A teacher he has lectured nationally and internationally and has taught generations of cardiology fellas the art and science of echocardiography. Dr. Marx is especially renowned as a pioneer in three D imaging of congenital heart defects. Dr. Marx has also been a tireless volunteer for the American Heart Association for A J. A. J. Has given him the Pol Deadly White Award at the 2012 Boston Heart that's named in honor of one of Boston's most revered cardiologists and a founding father of the American Heart Association. It is the most prestigious tributes given by the HHS Founder affiliate and bestowed and legally on a Massachusetts medical volunteer also just found out that he was named the Doctor of the Year for 2000 and 13. So congratulations, Dr Marx, you very much. I'm so proud to be talking Thio sighting, and we will be meeting nurse practitioners in the Western in our next segment. So not a mark. Maybe you can give us a little bit more information on some of the non invasive method that doctor here to help us understand some of the heart defects our Children are born with

spk_7:   19:27
well, and it's a very important question that you're asking. And I think it's been some of the great advances in the care of our Children born with congenital heart defects. Probably the single most important, I think noninvasive test is the echocardiogram. Many of you know it as the term ultrasound, but this is an ultrasound specifically of the heart. Basically, it uses the transmission of sound waves, so the instrument sends out sound waves. It's the heart and great vessels in the blood. Those sound waves air transmitted back to the transducer are the probe, and then the ultrasound machine creates an image of the heart and its ability to measure the flow with the blood in the heart. And it's great vessels. So it's termed non invasive because it really does not cause pain or discomfort, and it has no known harmful effects. And in fact, we use the echocardiogram to examine the fetal heart as early as 16 to 18 weeks of pregnancy, even up to the adult with congenital heart defects. This test, it allows us to see the anatomic details of the most complex forms of congenital heart defects, but also allows us to see the size and function of the heart and great blood vessels. Additionally, we can see the direction and the speed and even the amount of blood flow in the heart. Miraculously, yes, you know, when I started my fellowship, we didn't have the ability to do non invasive imaging and to detect heart disease. It really had to be done in an invasive procedure, even a newborn infant. So you could imagine what a great advance it was to be able to make these detection, even when the baby's first born. And certainly Christie was giving us a very good example of how her baby was born and diagnosed as having a cardiomyopathy within the first hours of life. And that's amazing. Yeah, really is. And really, the beauty of the ultrasound machine of equipment is that it could be taken anywhere. I mean, we can use it in a clinic in the fetal Abbas I mentioned we often times you use it in the intensive care unit and another major venue. Our we take. Your ultrasound machine is into the operating room where, when the patient first comes off bypass, we can look at the results of the surgery, can give immediate feedback to the surgeon. So it's important not only in its non invasive characteristics but in the ubiquitous matter in which it can be used and because it's noninvasive. It's a tested you conduce eerily in the baby and friend, her child, for example. I often see a baby, your child every year, and I can do the ultrasound test on a yearly basis, and it gives me a great way to know how the cardiovascular effects of the baby or child or young adolescent are going. And so it's a great tool for the clinician. I don't want to just take a few minutes and just talk about one other noninvasive test, which has become very important which patient apparent may have actually had. And that's a magnetic resonance imaging study. Then basically, the patient is placed in a very large magnet, and the shifts in the magnetic field causes protons in the heart. And then I know you and I remember back to our science days protons of the positively charged parts of the Adam and IT classes. Any event, these positive charges to shift, and when they shift it creates a beautiful it could be through. Computer analysis provides a very calm, positive view of the heart and the great vessels and even the lung field and both of these tests that normally take 35 to 45 minutes. But again, they give just a wealth of information to help the cardiologists in the cardiovascular surgeon in knowing what the best next steps to take.

spk_4:   23:14
I know, I know that Christie mentioned another non invasive procedure that my son had every year for a long time and sometimes multiple times. And that was it. Chest X ray I've noticed in the last five years or so. Alex isn't getting X rays anymore. Is that because the echoes have improved so much? I love how they have the Doppler and are able to read so much more with the Doppler also, but also the MRI's and CAT scans Is that why they're not doing X rays is much anymore.

spk_7:   23:41
You know that I get a very insightful question. Used to be that before ultrasound and M r I became so advanced and so again useful when a child came to clinically normally got an e k g, which mentions the rhythm of the heart or how many times the hardest beating and in what pattern? The hardest beating. But they'd also get a chest X ray. But it was clearly shown in actually clinical research studies that the echocardiogram provided much more valuable information than a test X ray would. For example, not only can you see the specific anatomic abnormalities, but it allows you to really directly measured the size of the heart and the volume of the heart, and how well that heart is contracting or actually squeezing the blood out to the body. And it's a much more specific and sensitive test on the chest. X ray would be

spk_4:   24:36
well, and can't you, even with the ultrasound, measure the width of the cardiac walls, which sometimes for our kids, could get enlarged?

spk_7:   24:44
Yes, exactly. So it not only measures the cavity, the heart, which is where the volume of blood is, but in measures is you're saying the mass of the heart are the wall thickness of the heart. And that's critically important, having an understanding not only of specific defects such as cardio myopathy, which is what Christie was talking about in her son's case. But it also allows you to measure how well the heart is functioning, even if there's not a cardio myopathy but a heart defect itself.

spk_4:   25:14
I know my son had to be put onto Jackson as an infant because his heart tended to flutter instead of pumping efficiently, and that the Jackson did help his heart to work a little bit better. No, you're sure that was one of the reasons why they were using the ultrasound on him.

spk_7:   25:30
Yes, exactly. So it's mentioned the ultrasound ability to non invasively see how the heart is doing. And since it can be done on a yearly basis, even in the young baby, you can look at the effects of administration of a specific drug and see if it's beneficial or not. Or you can look specifically at a surgery. Did the surgery meet its accomplish goals? And again because it's noninvasive? Because it can give you so much clinical information, it's really an extraordinarily valuable tool.

spk_4:   26:04
Well, I appreciate you explaining four different types of non invasive procedures that doctors can use to help them identify the Children's unique heart. Can you tell us about some of the invasive procedures the doctor shoes

spk_7:   26:15
sure can begin? The great advances of medicine has even changed how we use an invasive tools. Probably the most common one in continual hard defects is the cardiac catherization, and basically that's a procedure that I think many of you are aware of. But where you take long thin plastic tubes and you place him to the vessels, like the vessel in the arm or leg, and then you can feed them up into the heart itself. And with the catheter in the heart, you can directly measure the pressures in the heart, and these pressures and heart could be extraordinarily important information to guide. You know, both medical and surgical therapy, and the echocardiogram gives us a lot of important information, but it can't give us the pressures in the heart, so it gives us extraordinarily important information. But I'd have to say ended today that probably the most important part of a cardiac catherization is what's called the interventional catherization, especially gentle heart disease. There may be a hole there that you want to close, or maybe there's times when you need to have a hole and you can create a hole. Or, if there's an obstruction, you can open up the obstruction either with a balloon catheter, where you dilate the obstruction where you can put like a medal, the thin wire like sort of a tube called the stent, and keep that narrowing open. And probably I think the most exciting notice in advance is now. We can place valves the cardiac valves, which before you could only be able to replace in the heart by open heart surgery. We can now place those into the heart in the catheterization laboratory without ever opened in the chest or going on cardiopulmonary bypass. So cardiac catheterization is probably still our mainstay of invasive procedures, but it's abuse for diagnostic, but again, importantly, for interventional purposes.

spk_4:   28:14
Now this whole hybrid procedure for the cardiologists working with the surgeons is such an exciting area for me to save my son's 19 and I've been watching this change and grow and bloom over time. And it really is exciting, because if our kids don't have to go on bypass and they don't have to have their chest cracked open, that eliminates so many potentials for infection and really long recovery times. Isn't the melody valve one of the valves that they can? D'oh actually, with the catheterization?

spk_7:   28:43
Yes, absolutely. We can place house on the right side of the heart, that pulmonary valve with great success now, and there's even use of placing valves on the left side of the heart in the aortic position, mostly in adults. But we've actually had a few select cases where we've placed it in young Children. So I think the future for putting in valves is going to be extraordinarily important. And when I speak the parents today, I tell him, You know, we'll put in a certain valve now, but our hopes are that in the future the valve can be put in in the catherization laboratory.

spk_4:   29:19
It just gives me goosebumps, hearing you say that because I have a son who has a leaky valve, and I know that Sunday that's probably going to need to be replaced. And I'm hoping that you're right. And this could be something that could be done in the customization lab instead of him having to have get another open heart surgery. Dr. More thinking so much for hearing so much information with my listeners that, wow, I think we could do a whole episode just gone the interventional strategies that are being today in Children. It's really quite remarkable. And I appreciate all the work that you've been doing to help Children like mine.

spk_7:   29:50
Oh, thank you very much for having me on your program.

spk_4:   29:54
Well, unfortunately, it's time for us to go to another break. But don't go far because we will soon have a nurse practitioner from San Antonio who's working to help empower parents to their Children with complex congenital heart vexed, create care teams and your plans to take care of your tunic cart, find out what this nurse feels is important and how parents can be better advocates for their Children. When we return heart to Heart with Anna

spk_0:   30:27
Texas Heart into Street, we're offering us a mechanical hot and he said, now that I've had enough to give it to someone who's worthy My father promised me a golden dressed world held my hand and asked me where I wanted to go. Whatever stripe for conflict that we experienced in our long career together was always healed by humor. Heart to heart With Michael Please join us every Thursday at noon, Eastern as we talked with people from around the world who have experienced the most difficult moments way forever. Baby blues sound collective. I think what I love so much about this is that some of the songs were inspired by the patient. Many listeners will understand many of the different songs and what they've been inspired by. Our new album will be available on iTunes amazon dot com Spotify. I love the fact that the proceeds from TD are actually going to help those with heart defects Enjoy the music tonight forever. You

spk_3:   31:44
are listening to heart to heart with Anna. If you have a question or comment that you would like addressed on our show, please send an email to Anna Gorski at Anna at heart to heart with anna dot com. That's Anna at heart to heart with anna dot com Now back to heart to heart with Anna.

spk_0:   32:05
Back to our

spk_4:   32:06
show. Heart to Heart With Anna A show for the congenital heart defect community Today we're talking with crispy pina cardiologists, Doctor Gerald and nurse practitioners Cindy Weston. We've heard from Christie opinion about how she handled her son, Jimmy cart and what she's doing to empower other parents. And we just start with world renowned pediatric cardiologist Dr George Marks on Childrens Hospital about the invasive and noninvasive test that doctors are using to identify exactly what kind of heart defects the child has and how the doctors and parents can work together to help Children with their congenital heart defects. Now we're about to meet a nurse practitioner who's devoted many years of her life to helping families of Children with complex congenital heart defects to survive the open for surgery and prepare them to lead happy quality lot. Weston began her nursing career in 1989 and Cardiovascular Recovery Room at ST Luke's Episcopal Hospital, Texas Heart Institute and Carry, for instance, Children and adults after cardiac surgery in transplantation to completing her master's degrees in 1995 is a critical care clinical nurse specialist with a research focus on rewarming. After cardiac surgery, 1996 she received the Betty Baker Distinguished Professorship and Cardiovascular Nursing in 2000 and one. She had paid a postmasters family nurse practitioner certification and moved to San Antonio in 2011 where her career has come full circle by returning to the care of infants and Children with congenital heart disease. Your wife is a nurse practitioner with a congenital program and has served as the nurse practitioner for their single ventricle clinics, completing her doctorate of nursing practice with a research focus on improving feeding and outcomes in infants with single ventricle physiology. And I happen to know the Western Personally because she was one of my sons nurses when he had his last open heart surgery that welcome to the show, Cindy Whole thing. Sana. You have an amazing son. While you're very sweet. Well, I am just fascinated by the project that you have chosen to do your doctorate as nursing practice program on. I am so thrilled that you're finding out what each child's unique art needs and how, especially regarding feeding. We parents and professionals can work together. So can you tell us a little bit more about that? I love,

spk_5:   34:22
too, And I love your illustration of snowflakes because every heart is unique. Just like every child, there are all sorts of categories of complex congenital heart disease, and within each category there's really unique nuances for every individual child. So your title is so appropriate. The focus that I have is on feeding and nutrition and with the various forms of complex congenital heart disease. Many of these Children struggle with eating and nutrition before surgery, and even sometimes after surgery, babies grow the most in that first year of life, and helping nutrition is important for that growth. My project is on the single ventricle physiology or those infants that are born with one functioning ventricle. They're the most complex of all of our Children with congenital heart disease and carry the highest mortality and the most challenges for surgical Paley ation. This is theology occurs because as the baby's growing in utero, there's either a ventricular inflow track or outflow tract obstruction, and so that corresponding pumping chamber of the heart fails to fully developed. But 40 years ago, these Children did not survive. But fortunately with surgery advancement and now we have three stage paleo two procedures that we perform with medical advancement. These Children are living and surviving and thriving that even now the most vulnerable stage for them is from the first stage surgery to the second stage surgery and that carry immortality up to 22%.

spk_4:   35:57
Yeah, it's really scary speaking as a matter of a child who fits that description. He was so tiny he was emaciated, and they called him failure to drive. Just about broke my heart. You tell us what we parents can do to get our Children a little bit fatter. Meteors,

spk_5:   36:15
That's right, Will beating for an infant is exercise, and when we all exercise, which is something great for all of us to do it requires physical endurance and coordination. And if you think of when you exercise your own heart, you're breathing increases your heart rate goes up. So imagine these challenges created on a baby born with congenital heart disease. They're trying to learn this complex coordination of sucks, swallow and breathe with a baseline altered circulation. But the focus of what I'm doing my research is called Translation Science. It's taking the evidence that is out there and implementing it into practice for a sustainable program to improve outcomes. So I'm working on a standardized feeding approach, which actually came out of a collaborative initiative from the Joint Council on Congenital Heart Disease. This is a group made up of the American Academy of Pediatrics, American Heart Association, American College of Cardiology and the Board of Pediatrics. With their subgroups of cardiology and cardiothoracic surgery, they developed the quality initiative several years ago to look at building sustainable network throughout the United States and North America to input dated to see what could improve outcomes for this vulnerable population during the inter stage, from the first to second stage surgery. One of the things that they've determined from the data that we've all been putting in different congenital heart programs is that a standardized beating approach did improve outcomes. They saw these improved outcomes with growth, and it didn't matter if you were a small center or large center. Some centers it didn't matter about their size had improved outcomes. And when they started looking at the data, they found that all of those centers that had the best growth outcomes had a standardized approach. So what we have done in San Antonio is look at the evidence in the literature to see what are the elements of the best beating protocol. We've developed our own, and we're implementing that for this vulnerable population to improve their growth.

spk_4:   38:14
Absolutely excellent job with that. I'm so excited to see what you're doing. Because even though it struck hard is unique and its child has unique needs. Beating is universal, absolutely have to be able to grow and gain weight, because otherwise they have no reserves and when they get sick and hit him even harder. So what can we parents do to help our Children with the complex congenital heart defects so they can survive? How can he work with your cure team help our Children have the best odds for survival.

spk_5:   38:45
Well, you're empowered as a parent and my goal as a nurse practitioner and all nurses are to empower parents. And I loved hearing. Christie referred to how she felt empowered, and she was taught to be an advocate for her child and just to hear her story and her child's great outcomes. It excites him, inspires me that we do this one way is through an interstate home monitoring programs that we begin education with. The parents understand the complex city of the disease that their child has of the complexity of the circulation and what that means to them and how Thio do an assessment on their child. They're you know, they're child better than anyone, and so we teach them the signs tow, watch for and encourage them tohave a short threshold to call the cardiology team. If they identify anything concerning, we send them home with pulse oximeter so that they can monitor their child's oxygen saturation daily. We sent him home with a scale so that they come way their child every day at the same time without a diaper on, and they record those weights in a binder that we provide them, and we teach them how much the child should be growing every day and give him a red flag that if they lose more than a certain amount of weight, generally 30 grams in a day, we want him to call us. We teach them how to do the math, even on calculating out those wait, we empower them that if they just see something unusual in their child, if they're not breathing right, are there in console, herbal and irritable and not themselves toe have a low threshold to call us so we can intervene early on these vulnerable Children.

spk_4:   40:22
I love that. And I felt that when we were there, even though Alice wasn't a baby anymore, he was a young adult. I felt the two while we're there for him every step of the way. Thank you so much, Cindy, for being on the show and for sharing your expertise with us, and especially for doing this really important project for your doctoral program. Oh, thank you for having me. And, uh Okay, well, it's time for another commercial break, but don't leave yet. We still have our miracle moment. and today's miracle moment comes from an amazing man who was born with a heart defect. But he didn't realize just how lucky he was until he started doing something that changed his perspective on his own life. Find out what he did.

spk_0:   40:58
Wait. Theo

spk_3:   41:13
Hart with Emma is a presentation of hearts, Unite the Globe and is part of the hug Podcast Network Hearts Unite The Globe is a nonprofit organization devoted to providing resources to the congenital heart defect community to uplift and power and enrich the lives of our community members. If you would like access to free resource, is pretending to CHD community please visit our website at www Congenital Heart Defect. Stop Tom for information about CH D, the hospitals that treat Children with CHD summer camps for CHD survivors and much, much more. You are listening to heart to heart with Anna. If you have a question or comment that you would like addressed on our show, please send an email to Anna Gorski at Anna at heart to heart with anna dot com. That's Anna at heart to heart with anna dot com. Now back to heart to heart with Anna Come Back to

spk_4:   42:18
hurt Dan, a congenital heart defect community, to empower members of our community with resources, support and advocates the information. I want to take another moment to thank my guess. Christy Pain you, Dr Gerald wants and Cindy Western honor to get to know each and every one of them. And I love how each of them are working so hard to strengthen our community and help us all feel more empowered with what we're doing. Everyday Napper on Miracle Moment Today's local moment comes from the heart of a father. Max S. Gerber is one of the contributors to the special book due time restrictions. I have had to take just excerpts from his story. If you'd like to read his entire essay, you can find it on page 232 in the book The Heart of a Father, which is available at www dot baby hurts press dot com, amazon dot com for kids with heart dot org's wrote a wonderful essay entitled Broken Hearts Club, he writes. I take pictures of people for a living, and I spent a lot of time looking at people's faces, paying attention to their lives and their stories. I'm interested in the universality of the human experience. I grew up with a broken heart 13 months premature with a heart rate, half of what it should be. I didn't meet anyone else with a similar heart. For 27 years, when I started work on a book called My Heart versus the Real World and Making a Book, I spent almost five years following 10 families with kids who have congenital heart defects talking to them, photographing bam and at the same time paying closer attention to my own Sam like on my own experience, growing up with a heart defect, I created that both for the same reasons. A lot of you are reading this one. This collection of essays, hopefully monster community of the brokenhearted, looking towards club, if you will. The most universal human desire is to feel like someone understands you to feel like you're not alone to feel like what you're thinking. You're sure by someone else. I certainly wanted to see myself in someone else. I wanted to know there were other people who grew up like me. Mostly, I wanted to make sure other people didn't have to wonder about these things and that they were not going through a singular, isolating experience. Got my first pacemaker when I was eight years old almost 30 years ago. This wasn't a simple and straightforward is it has now become. At the time, it was a major open heart procedure to place the wires. This was before on demand peacemakers, meaning my heart rate was 72 beats per minute. I never went up. It never went down. It was a model of stability during my teenagers in school. I was more self conscious about my condition than anyone else was. Frankly, looking back, I don't think people noticed or cared. I was a skinny kid. Anyway, just like my father, I read a lot of books instead of running around. It was having the pacemaker just caused me to be more careful, more aware, more crushes and how I lived my life. I was never a reckless, headstrong teenager. Of course, the heart is the most important part about having a broken heart. Is figuring out how to get over it kind of move on For most of us, with little broken heart, you keep pain generally comes from some sort of surgery. You wake up from surgery, and while you corrupt your head around, how much it hurts, you focused on trying to regain your appetite, trying to walk down the hall without collapsing. You realize that you need to let your pain be and you need toe. Wait it out. You realize it will get better. It could give it its due. It's so easy to get wrapped up in everything that surrounds a broken heart. It's easy to get lost in the medicine, the doctor's appointments and worrying about missteps, problems and accidents. It's easy to forget about living your life, but sometimes difficult for parents to realize. A broken heart doesn't have to be the entirety of their child's life. They don't have to define themselves by their condition. They don't have to feel isolated. Broken hearts can sometimes feel that concludes this episode apart. Hurt with Anna. I hope you'll come back next week, when our show will be called losing a Child to a congenital heart defect. Until then, please find and like us on Facebook, check out our website heart to heart with Anna, and remember, my friends, you are not alone.

spk_3:   46:17
Thank you again for joining us this week. We hope you've been inspired and empowered to become an advocate for the congenital heart defect community. Heart to Heart With Anna with your host Anna Gorsky can be heard every Tuesday at 12 noon Eastern time. We'll talk again next week.