Non-Cardiac Health Issues for Survivors with Complex Congenital Heart Defects (Remastered)

There are over one million adults alive with a congenital heart defect or CHD. Many surgeries performed on survivors are corrective but not curative. The non-cardiac health issues that seem to appear fairly regularly include, but are not limited to: problems with teeth, (i.e. an abscess, needing wisdom teeth removed or needing braces); scoliosis; problems with legs or feet requiring braces or surgery, pregnancy or female problems, male babies with undescended testicles or concern about circumcision, eye problems, protein-losing enteropathy, other intestinal problems, plastic bronchitis, asthma, strokes, seizures, migraines and allergies. These non-cardiac conditions are more worrisome for heart parents and CHD survivors since everything is more complicated when considering a body which has had open-heart surgery. Special considerations must be made for drugs taken and surgical complications endured. What kinds of non-cardiac health issues are common in the heart community and why?

Thanks to our newest HUG Patron, Ayrton Beatty and long-standing Patrons: Laura Redfern, Pam Davis, Michael Liben, Nancy Jensen, Alicia Lynch, Deena Barber, Carlee McGuire, Carter & Faye Mayberry, and Frank Jaworski. We appreciate you!

Transcript

spk_0:   0:00
welcome friends to heart to heart with Anna. This is your host standing Dworsky and I'm honored to have today show non cardiac health issues for survivors with complex congenital heart defects. This is the 12th episode of Season one and this show originally aired on January 28th 2014. Today's guests are Lauren Bed North, David Gibson and Dr Amy Roberts. We will be discussing different issues related to raising a child with a heart defect with non cardiac health issues or being a PhD survivor with non cardiac health issues. Here is our show for today

spk_1:   0:34
Welcome

spk_2:   0:39
to Heart to Heart with Anna featuring your host Anna Dworsky. 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 Dworsky.

spk_3:   1:02
Welcome to the 12th

spk_4:   1:03
episode of Heart to Heart with Anna, a show for the congenital heart defect community. Our purpose is to empower members of our community with resource is support, an advocacy information, according to US News and World Report and the Mayo Clinic. Historically, only a minority of Children born with a congenital heart defect survived into adulthood. However, recent advances in surgical treatments and techniques have reversed thes statistics. Now, approximately 90% of Children born with a heart defect live healthy lives through adulthood. This shift in survival rates has produced a relatively new group of patients with a new set of challenges for the medical community. Adults with congenital heart disease. Today, adults with congenital heart disease outnumber Children with that condition. As a mother of a congenital heart defect survivor, an advocate for the heart community For the last 19 years, I've come in contact with hundreds of parents. We parents often talk about the other kinds of medical problems that frequently appear as our Children grow up. The non cardiac health issues that seem to appear fairly regularly in our heart community include but are not limited. Thio survivors developing problems with their teeth, such as having an abscess needing wisdom, teeth removed or needing braces. Spine problems. I mostly hear about our survivors having scoliosis problems with legs or feet, which require braces or sometimes even surgery. Women getting pregnant or having female problems. Male babies having problems with undescended testicles or parents expressing concern over whether they should have their baby circumcised or not. I problems but more often just Children needing glasses. Protein losing interrupted the other kinds of intestinal problems, which frequently require feeding to you gall stones or problems with the gall bladder. Problems with the liver, especially in survivors who have had the Fontana procedure. Plastic bronchitis, asthma, pneumonia, vocal cord problems, diaphragm problems, stroke, seizures, cancer, especially for our transplant recipients allergies. And the list goes on and on. This is just the kind of list that would go on for any child growing up. What makes these conditions more worrisome for heart, parents or heart survivors is that everything is more complicated when considering a body that has had open heart surgery takes some kind of medication for the heart or blood thinners to prevent strokes has been on paralyzing drugs, a heart lung machine or a ventilator. Nothing is ever simple with congenital heart defect survivors. They need to have special considerations when undergoing any kind of surgical procedure or treatment. The question we have today is what kinds of non cardiac health issues air comin into her community and why today we'll meet three individuals who have firsthand knowledge of non cardiac issues affecting survivors of complex congenital heart defects or C HDs. First, we have single ventricle survivor Lauren Bednarz, who will share her experiences with scoliosis and eye problems. Next, we'll hear from David Simpson, father of a son with protein losing interrupted the European Ellie. And lastly, we'll hear from Dr Amy Roberts, geneticist from Boston Children's Hospital, who will share with us possible genetic causes for non cardiac health issues. Is survivors of complex See HDs, Loren writes. My name is Lauren Bed nards, and I'm 26 years old. I was born with a complex congenital heart defect called Try Cusp. It atresia hypo plastic right ventricle or simply put half a heart. I have had to open heart surgeries, the last being the Fontane procedure 24 years ago. I was also born with two other medical issues. The first was an eye muscle disease, for which I had patched therapy as a young child. I got my first pair of glasses at nine months old and have had three eye muscle surgery so far at ages, 18 months, seven years old and 20 years old. I also have congenital scoliosis, which was not formally diagnosed until about 14 years of age. X rays revealed I have an extra vertebrae in my lower back. My first liver, M r I a few years ago showed I have a small noncancerous tumor on my spine as well. We'll meet David Simpson and Dr Amy Roberts later in our show. Thank you, Lauren, for coming back on our show.

spk_5:   5:03
Thank you for having me again

spk_4:   5:04
when you are on our second episode of Hart to Hart to Anna. When we talked about how you dealt with anxiety over the years, I can't help but think that having ah heart defect plus I problems and scoliosis is partly why you had so much anxiety. Which of these conditions has caused you the most distress?

spk_5:   5:21
Definitely. My heart always calls me the most anxiety, especially with all the things that can happen afterward, especially with being a Fontana. But of the two non cardiac issues, my eye problem is the one that gives me the most just just because I have to wear glasses all the time, and I've had surgeries with them and my ice that it will only get worse, not improve. It will eventually decrease as I get older. Worn more so, actually got a new pair of glasses this year because my eye sight decreased. Moreover, the past probably 56 years. So

spk_4:   5:55
unfortunately, I think that happens with most of us. Let's say, But you're so beautiful in the photos that you've sent me. So it looks like you're not always wearing glasses. Are you able to wear contacts?

spk_5:   6:08
No, I'm not with the eye muscle disease. I have my eyes float all the time so the lenses might get stuck up in my eyes, so they usually plus, with my heart, there's a high risk of infection that I would have to consider.

spk_4:   6:20
Okay, so you take your glasses off before you

spk_5:   6:24
put it before photos, and when sleeping

spk_4:   6:27
Well, yeah, when sleeping. Okay, well, you are a beautiful young

spk_5:   6:33
will end. Well, thank you so much.

spk_4:   6:34
I'm slide that you're talking to us about some of these eye surgeries. My son Alexander has been wearing glasses since he was four years old. But goodness by then you had already had quite a bit done to your eyes. Can you tell us about the eye problems that you've had and the surgeries that you needed to tried to repair those problems?

spk_5:   6:55
Yeah. When I was a few months old, my parents noticed that my eyes were floating like one would go up and one would go in and I wouldn't be focusing very much. So they brought me in. And I have what's called a strop A, which is an eye muscle disease, which means my eye muscles and the communication between the brain and your eyes. There's some miscommunication there, so my eyes have weak muscles because of that and the weak muscles condemn it. Your eyesight, because your eyes have trouble focusing. So, yeah, I had three eye muscle surgeries and they basically were trying to tighten the muscles so my eyes didn't float as much, so my eyesight wouldn't be is compensated.

spk_4:   7:32
Okay. Wow, that sounds kind of complicated. I had a peasant who had problems with her eyes as well, and they put a patch on one eye to try and strengthen that I could. She had a lazy eye and you said something about needing a patch to what were the patches for?

spk_5:   7:48
They did do patches first after I was diagnosed, hoping that maybe my muscle disease wasn't that bad. And sometimes it can be strengthened with patches. But mine was just so severe that the patches weren't really helping. So surgery intervention was the only way.

spk_4:   8:02
Okay, so you've had three surgeries. Does that mean that like our heart surgeries for kids who are like you and like Alex, who have HHS or HR a chest, they have to do it in staged procedures? Was that the same case with your eyes that it had to be done progressively?

spk_5:   8:19
No, the 1st 1 was hopefully a fixed. But as I got older and you get bigger, the muscles kind of on Titan. So they just kind of did, Ah, another fix of it when I was seven and when I was 20. I shouldn't really have had to need 1/3 surgery, but I was kind of rebellious, and I didn't wear my glasses like I was supposed to, which compensate in my eyesight, and my muscles got loose. So I needed the third eye surgery, and that's something that kind of give myself a little bit. So

spk_4:   8:48
now you're much more aware of how significant wearing those glasses is. It's not just to help you read. It's actually to help your eyes stay focused. And for them to not become weaker. Is that true?

spk_5:   9:00
Yes. Yes. So, yes, when you're a teenager, you kind of have a rebellious side anyways.

spk_4:   9:05
Oh, yeah. I, uh, have had to talk. So, yes, I could totally relate to that. And I was a teenager myself. Okay, so you've had three eyes surgeries, and so now are you considered legally blind, or are you okay with your glasses?

spk_5:   9:21
I'm OK with my glasses. Even without him, I'm not considered legally blind yet. But one day I could be so okay. We don't know. But I

spk_4:   9:31
have a feeling that if I meet with you in person after this, you'll be wearing your glasses.

spk_5:   9:36
Yes, I will. Okay.

spk_4:   9:40
Okay. The mom and me is happy. Okay, so let's move on to your other topics. So you talked about discovering that when you were 14 you had scoliosis. And in episode number 10 I interviewed Mark Cummings, who also had scoliosis. He had to have two rods inserted into his back to straight in it. Have doctors done anything to help you with your scoliosis?

spk_5:   10:05
You know, I honestly have only had one back, doctor, and that was the one that diagnosed me. He would crack my back. Sometimes he would show me some moves to strengthen my muscle in my lower back. And I would have periodic X rays to see where it stood and make sure I didn't get worse. He was also checking when my bones fused for when my growth spurt was done so you could see how about it might be afterwards. And then I have an insulin, my shoe toe help level out my back because one hips, it's hired another. But other than that, not really no surgery. And when I moved to California, do not have a back doctor as of right now, but I definitely I'm looking for one.

spk_4:   10:45
Okay, Well, good. I'm glad you haven't actually needed surgery for that. And it sounds like there are some things that you can do, such as those exercises that will strengthen your back So you won't need surgery in the future. Is that right?

spk_5:   10:58
Hopefully not. It's only mild to moderate But you never know With scoliosis, Sometimes it could get worse. But right now I seem okay. But I do get pain so that sometimes I get a daily basis. But, you know, you kind of learn to live with it.

spk_4:   11:12
Advil and talent. All those could be your best friends. Yeah, that's what they're having problems. But I'm sure that you're taking a number of medications, and you have to be careful what other medications you take. Absolutely. Can you think of any extra precautions thatyou're non cardiac doctors had to take with you, Given the fact that you do have a congenital heart defect and you are on some medications?

spk_5:   11:34
Yeah. For all three of my eye muscle surgeries, I had to have Ah, pediatric cardiac anesthesiologist put me to sleep and how to be under a little bit more special observation afterwards because of my heart condition, A little extra cautious and my back Dr. Growing up In my teenage years, there are certain places he couldn't crack, like near my chests and everything because the bones there he didn't he didn't want to risk anything, you know, bad happening. So yeah, definitely. There's some extra precautions that were taken.

spk_4:   12:04
Well, we only have about one minute left, Lauren. So in this last minute, could you tell us what advice you would give to parents of Children with complex heart defects to help them be aware that they might have other non cardiac issues that they'll have to deal with with their Children?

spk_5:   12:19
Oh, well, if you suspect something early on, definitely. Just don't ignore the signs. As for those that could have complications from their surgeries, like the Fontanne and the liver off you're a teenager and on adult, definitely get your liver chucked out at least once year with an M. R I. And make sure you do your research and be proactive because being proactive can help us extend our lives to the fullest.

spk_4:   12:40
That's excellent advice. Thank you so much for being on the show. I really appreciate it, and now we'll have to go to a commercial break. But don't leave yet. Today we have a heart dad coming from England, who will be talking to us about how he felt isolated after his son's diagnosis with protein losing and theropod the find out what happened when he flew to America to find out about the serious condition when we return to heart to heart with the animals.

spk_2:   13:15
He has written several books to empower the congenital heart defect, or CHD community. These books could be found at amazon dot com or at our website www dot baby hearts 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 Hypoplastic 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. Anna 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 and information about advocacy groups as well as read Ana's Weakly Blawg. Anna wants you to stay well connected and participate in the C H D community Visit heart to heart with anna dot com Today

spk_1:   14:30
you

spk_2:   14:31
are listening to heart to heart with Anna. If you have a question or comment that you would like a dress on our show, please send an email to Anna Gorski at Anna at heart to heart with anna dot com. That's Ana at heart to heart with anna dot com. Now back to heart to heart with Anna.

spk_4:   14:51
Back to our show. Heart to Heart with Anna, a show for the congenital heart defect community. Today we're talking to Lauren Bed in Arts, David Simpson and Dr Amy Roberts about not cardiac health issues and complex CHD survivors. We just finished talking with Lauren about her experience with having genital scoliosis and eye problems. Now we will turn our attention to David Simpson. David Simpson, PhD, has had a 20 year career in medical physics. He married his wife, Helen, in 1997. They have three Children. Alistair, Eleanor and Katherine Al was born with aortic atresia, a variant of hypoplastic left heart syndrome, or atrial HS, in October of 2000 and seven. Out developed protein losing and tour apathy, or P. L. E a potentially fatal condition, knowing no one else with P L E Helen and David were frustrated. The heart and intestinal specialist did it work together. Even O P L E is not uncommon in hohs survivors. They felt isolated but slowly make connections with researchers and parent groups to try to find out what they could. David flew to the United States to meet Dr Jack Roy. Chick David asked him questions for two hours about P l E and its significance in the CHD community. He flew to San Diego and met with Dr Had Some freeze. David concluded his trip by flying to New York and meeting with Tom Coulson, another heart debt, who dealt with P. L E and had set up a charity to research for a cure will meet Dr Amy Roberts in our next segment. Thank you, David, for coming on heart to heart with Vienna.

spk_6:   16:27
Well, thank you very much for asking me. Yeah, it's very nice to have the opportunity to chat with someone in Texas when you're sitting in Canterbury, England.

spk_3:   16:36
I know

spk_4:   16:36
you're by second United Kingdom guests, and I'm so thrilled to have you on the show and for those listeners who may not know it. David was also a contributor to the heart of a father. So this is a sort of reunion for David and me, David. First of all, can you tell us about how Alice doing right now and tell our listeners what P. L E is? And how you discovered that your son had that condition?

spk_6:   16:59
Yeah, well, to start with, Alistair is doing very well. I I'm actually sitting in his bedroom on his computer equipment. He's my technical support for this coal, and so he's grown up to be a very able 13 year old. But yes, I mean, if I asked you how Alex Waas there is always part of you that's going to be worried about them that's goes with them being a heart parent. So, yeah, God, ever cold today. But in general, no, we're really pleased with how he's doing. He's does feel time in mainstream school. Kids with P L E are often shorter than their peers and on that street for Alistair, but no, in general, very proud ofhim on very pleased with how he's doing. But we have been through a lot with I think the I see that I did for you for the heart of the father covered the 1st 3 operations for his hyper plastic left heart. Very in. Yeah, I think a lot of your listeners will be aware off the chat you have where the doctors come and tell you what's wrong with your child and what you can expect on. We basically had another one of those chats. Really. Eight of the blue. When Alistair was seven, we noticed that his stomach had been getting a little bit swollen on Dhe after a summer where he really wasn't himself, they did a stool test and discovered that he was losing protein through his gut. It was the first we'd ever heard about it. On we were We were told that it was a very serious condition prognosis up to that point, being quite sure, and that is possible. He would need a heart transplant. So, yes, that was quite difficult news to take. Yes. You want me to say what protein losing interoffice E is? Yes. If

spk_4:   18:55
you would explain it to our listening audience. I know that that's something that a lot of people here about, but they don't know exactly what it means.

spk_6:   19:04
Well, again as your audience. Me? Well, no. If you have a single ventricle condition on, do you have the staged for Anton procedure your body set up to have a single look circulation. So the blood returns to your heart through the lungs instead of having a separate heart, just takes the job of sending the blood from the heart to the lungs and back. That's done by passive dreaming of the blood on the way back to the heart. Much easier with a back of an envelope so I can draw a diagram. But

spk_3:   19:40
I

spk_6:   19:40
guess I could describe it so that circulation works very well. But the pressure's air all wrong because the lungs are in the way of the blood, getting back to the heart, the blood pressure. The other organs aren't know what they're expecting, so the pressure of the gut seems to cause some sort of inflammation to happen on dhe. As far as I can see, it seems to trigger something a bit like an inflammatory bowel condition on that's making the chorus, and it's letting protein aids. So obviously that's bad news. You need protein for bone growth and everything, but more seriously than not, If your protein drops below a certain level, your blood can't retain the liquid in the circulation. Your blood needs a certain level of protein to hold the fluid in. And if it drops below that level, the fluid just accumulates in your tissues on your heart. Finds it increasingly difficult to pump this volume of fluid. Arraigned on dhe. They can get to be very serious on me to quite a nasty downward spiral. Fortunately, we had some parents ahead of us who'd done a fair bit of work on dhe. Alistair was put onto a treatment that had been published maybe four months earlier called sildenafil, and that opens up the capillaries in the lungs on it makes the lungs much easier for the blood to travel through, so that starts to sort the blood pressure. OK, and, um, Alistair responded to that and some steroid treatment to sort out the inflammation. Um, yeah, that seemed to do the trick.

spk_4:   21:25
So it's actually gotten his P L E. Under control. So it's not such a major issue for him.

spk_6:   21:30
Yes, after he started eight on a standard steroid with missile of But My Trip to America uncovered a new steroid that was being used in Children's Hospital of Philadelphia by Dr Reid Check. And that was something that was designed for inflammatory Boyle disease. I'm actually, interestingly, that was suggested by another apparent off child with P L E. That's a potential treatment for Pierrot, so we're really grateful for the work. The parents you've gone before have pertained to helping us deal with this disease. So when I went on my trip in 2010 Alistair was doing well on the standard steroid. But shortly after that, he didn't seem to do so well. But because I'd made those contacts, I was able to persuade his clinicians to try the standard practice in America, and he responded well to that. Then, in June this year, he needed a catheter procedure again. Thio try and equalize the pressure of the gut. It all seems to be hinge on blood pressure. That seems to be the key to this disease.

spk_4:   22:46
So what other organizations have you found particularly supportive since your son's diagnosis?

spk_6:   22:53
Well, it's being quite tricky because it's a rear complication of what's a rare disease. So There are very few people have yet to meet another parent in the UK with a child with this condition. So the organization that we found most useful is cold little heart smarter on that's ah, single ventricle charity beast in Birmingham in the UK And that's been great because we've actually been able to meet face to face. Nothing beats actually spending some time in the same room as other parents that know what you've been through. That what Facebook has also been handy to me. Contacts throughout the year, thirds throughout the world, with people Hey, do have protein losing interest. So there's a Facebook page, post fun town protein losing into apathy J only discovered recently, but that's being very useful as well. I may never meet those people, but they knew the detail of the exact condition that we're dealing with.

spk_4:   23:54
Right? And I went out and looked at that page when you shared it with me. And it does seem to be helpful to talk to other parents who have experienced the same things that you have experienced with your son. So you've been an amazing advocate for Alistair and other suffering with P. L. a. Because I know you're my go to person. Whenever I have a direct contact Me? Who says what has really I always put them in touch with you? Can you tell us what our listeners can do to help themselves or to help others with Paley?

spk_6:   24:24
Yeah, Well, I hope you don't spoil the magic of radio, but you sent me this question in advance, and I've been thinking about it for days now. I don't generally like giving advice. I don't really think of myself as an expert, but I'll pass on the advice from the coasts and family that I got when I visited him there. And that was actually to take my wife for, ah, a weekend away and make sure that we were really talking together. It's really easy to get caught up in trying to fix. This is a technical problem, but hopefully this is going to be something you're gonna be managing for decades. So you have to think of it as a long term condition and you need the support of the people close to you. So my advice would be if you're listening into this, you're probably doing a lot of research, but you probably know a lot of the fact. What you need is the emotional support. Get the people alongside you here close to you to do this with you and not to do it alone. That would be

spk_4:   25:26
That's beautiful advice. You could have been on my show last week where we talked to that. How having a child with a heart defect effects, parental relationships, that a place that was given on that show also was the importance of communication. So I love that, especially being a wife. I love the idea that you feel that what's really important is to continue to foster that relationship. So the two of you could be united to help your son the best you can. I love that. Thank you so much, David, for coming on the show.

spk_6:   25:54
Thanks again, I really enjoyed it.

spk_4:   25:56
Well, now it's time for a commercial break, but don't go far. Stay tuned To find out what a geneticist for one of the leading Children's hospitals in the United States has to say about non cardiac health issues and Children with complex congenital heart defects when we return, take heart to heart with Diana

spk_3:   26:20
Harding chief. We're offering us a mechanical hot, and he said, now that I've had enough to give it to someone worthy, my father promised me a golden dressed twirling 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 those most difficult moments forever by the Baby Blue Sound collective. I think what I love so much about this CD is that some of the songs were inspired by the patient's many listeners will understand many of the different songs and what they've been inspired. Our new album will be available on iTunes. Amazon dot com. Spotify. I love the fact that the proceeds from this CD are actually going to help those with Joe Hart effects. Join music Home Tonight forever, huh? You

spk_2:   27:36
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 Ana at heart to heart with anna dot com Now back to heart to heart with an A

spk_4:   27:57
Heart to Heart With Anna A show for the congenital heart defect community. Today we're talking with Lauren Bed Nards, David Simpson and Dr Amy Roberts about knock cardiac health issues in complex THD survivors. We just finish talking David Simpson about his experience with PL E and with Lauren Bernard's about her experience with congenital scoliosis and eye problems, in addition to having a complex heart defect. Now we will meet Dr Amy Roberts. Dr. Amy Roberts is a cardiovascular geneticist and researcher. She is also an assistant professor of medicine at Boston Children's Hospital. She is board certified, specializing in medical genetics. She graduated from Dartmouth Medical School, completed a residency in pediatrics at the University of Massachusetts Medical Center and then completed a second residency and medical genetics at Harvard Medical School in 2000 and four. Dr. Roberts is a published author, having published articles in peer review journals. She has stunned research specifically addressing new dances, Barth syndrome, dilated and hypertrophic cardio myopathy and potluck lipsky syndrome. She has also done other research into genetic causes of congenital heart disease. She is on the medical advisory board for cardio Fast Get Fruitiness Syndrome International and the Noonan Syndrome Foundation. Welcome to heart to Heart with Anna Dr Roberts

spk_7:   29:18
Thank you. Thank you for having me

spk_4:   29:19
as the geneticists. What kind of non cardiac problems do you commonly see associated with complex, congenital heart defect survivors?

spk_7:   29:28
Well, there are a few broad categories that I commonly encounter, and then any other number of medical problems air certainly seen but the most common things. That idea was particularly in the first few years, usually center around growth and oftentimes, feeding problems. So those two things can be related in that if a child is having trouble taking enough calories, obviously their growth will be affected. And there are other Children for whom they have the mechanics of seating down but still seem to be somewhat growth delayed. And this raises a number of issues, which is to ask whether or not it's actually the child's underlying heart problem, that causing the growth issues or if it seems like the growth issues are independent of the heart problem. Hey,

spk_4:   30:09
I have a lot of friends who have Children with growth issues of my own son was labeled failure to thrive before his heart defect was finally diagnosed.

spk_7:   30:18
Right, that's actually, unfortunately more common. And we would hope or like actually, you find out that because of the extra work of a heart that didn't form as we had, I hoped it would. That can lead to growth issues because so much of the energy is going towards just keeping the heart pumping and the blood flowing. Also, Children who've had prolonged intubation because they needed to be on a ventilator can have a lot of Orel feeding issues. Sometimes they can be quite defensive about having things put into their mouth and develop aural aversion and need alternative ways of being fed either through a nasal gastric tube or a gastric tube. Directly. So those air common issues for Children that I see, I also see a significant variability, but very commonly issues with developmental delays and that can be delayed motor milestone, so learning to sit or stand or walk later than we might expect, starting to talk a little bit later, and then also later on learning issues and learning disabilities. And again, just like with the growth issues that raises the question of whether or not this is secondary to perhaps surgical complications or prolonged periods of time with inappropriate oxygenation that may be leading to developmental issues or learning issues. Or is it something that, even without a heart problem, a child may already have had a predisposition to have developmental delays or cognitive issues? And then, for a child who has one quote unquote birth defect, which would be the congenital heart malformation, they do have an increased chance of having a second birth defect. And so we sometimes see Children who have one kidney instead of two or a malformed kidney. They can have vertebral anomalies like Lauren described in the first segment. They can have extra fingers, are missing fingers or collecting on the lip work lifting of the palate so any number of birth defects can come along also with congenital heart defects.

spk_4:   31:57
That was excellent. That was an excellent description of so many of the conditions that I hear parents talking about on the Facebook pages that I belong to you. Yeah, Can you tell us why some non cardiac conditions seem to present themselves right away, like the eye problems that Lauren discuss. But how other problems don't seem to show up until much later, like Lawrence, congenital scoliosis or Davidson who had pl? E

spk_7:   32:21
sure, I think to some extent it depends upon what exactly you're talking about, why it would take longer to the apparent. And that's because, in the example of Davidson pl e, that was, as he very eloquently described, secondary to the way that his son's cardiac surgery altered the blood flow and subsequently changed the blood profusion of his intestines. It was secondary to all of those things, and that blood flow pattern didn't exist until after he'd had his surgeries. And the damage that it did to the intestines took some time to develop, so he didn't become symptomatic until he was seven. In the case of Lawrence congenital scoliosis. Think I can't specifically talk about it not knowing the details of her case, but I could guess that one issue may have been that it just wasn't noted until she started to get taller, and then maybe the curvature was seen when she was having a physical exam and bending over. However, if she was truly born with a malformation of one of the vertebral bodies in her spine, had specific spine X Ray's been done. It would have been seen as soon as she was born, but it took

spk_4:   33:21
some

spk_7:   33:22
time with her growth and development for it to be seen simply on a physical exam without them looking for it specifically by X ray. So it sort of depends on the issue itself. I problems of the is a trophy A. That Lauren described over the eye that turns in is something that parents note right away because you expect your child to be looking at you directly with their eyes pointing right at you. And if you look at photographs, too often will notice that an eye turns in. That's when it's often noticed. My parents and usually noted by a parent before it's noted by a physician because it can be subtle in the beginning and on Lee scene, for example, when a child is very tired or when they're sick, it may be much more obvious. And so a parent who's spending a lot of time with their child may notice before a physician who really is essentially spending very brief periods of time with the child would noted on a physical exam.

spk_4:   34:05
And that's one of the reasons it's so important for parents not to be afraid to say something to their doctors because they may notice something that a doctor wouldn't simply because they spend so much more time with the child. Isn't it true?

spk_7:   34:18
I think that's absolutely true. And for a child to have a sort of complicated medical history, I think it's always important to really listen to what the parents were saying because they think nobody knows a child as well as the parent does for that very reason, and especially a parent who really has become quite well versed in medical things that are going on and to paying attention to their child and really is in tune with everything that's happening and knows when, even if they don't know the proper terminology. They know when something is different or something feels not quite right, and it's really important to do the best you can to explain that.

spk_4:   34:49
Good. I love hearing doctors say that because as a parent, I actually did come in contact with some doctors who did not treat me with the respect that I felt I should be given. And I even had a doctor tell me it wasn't natural or normal for apparent to research their child's heart defect and that I should just trust the doctors.

spk_7:   35:09
Oh, that's terrible. I'm sorry that happened. That sounds like maybe I'm gonna make an assumption here. But maybe an older physician who might have said that to you. I would hope that that would be a rare circumstance happening now.

spk_4:   35:21
No, sadly, that was a very young person, but I only worked with her once.

spk_7:   35:27
That's a good thing. There are lots of doctors out there, and I think that that's actually, you know, we're laughing. But it is an important point. You have to trust the person who's taking care of your child. And if you don't feel that they're listening to you, then you I can't imagine that you could. You could trust them. So it's certainly worthwhile to spend a little bit of time finding and creating the team that you think listens to you when it comes to any concerns that you have.

spk_4:   35:47
And I love that because that's exactly what we should be. We should all be a team that's working together for the benefit of our Children.

spk_7:   35:54
Yeah, I agree.

spk_4:   35:55
I love that. Thank you. Do you think it's important for Children with complex, congenital heart defects to have genetic testing and cannot testing help us to be alerted to some non cardiac issues that they might have?

spk_7:   36:07
Well, I think before one jumps to the question of genetic testing, the question that I might ask first is whether or not the child needs a genetic evaluation or an evaluation by a geneticist. So the real question to ask is, Is there any evidence early on or even a little bit later that there are issues outside of the heart that are really not explained by the heart? So are the developmental delays outside of or more extensive than one might have expected, based on the child's history of surgery and the extent of their heart defect? Are the growth delays out of proportion with what one might have expected based on their feeding history? Caloric intake? You know, something that indicates that there may be a genetic diagnosis, a larger genetic diagnosis that X claims why they were born with her heart malformation. And certainly anybody who was born with a second birth defects, we would say, should probably have a genetic evaluation with a geneticist to say. Is there a larger umbrella diagnosis that may explain why a child was born with not just congenital heart disease but other issues as well, Understanding that that's not always straight forward. Tease out whether or not the other issue the child is facing are secondary to the heart problem or in addition to the heart problem. And genetic testing is evolving quite rapidly, and we actually have quite a bit of testing to offer families once we've done an evaluation to help. First of all, understand whether or not there are other issues that we need to be on the lookout for. And also what is the chance, for example, that these parents may have a second child with a heart defect? What should we be thinking about for siblings that are already born and for their own future Children, and for the child who was born with a congenital heart? The effect when they're an adult, is there an increased chance that they themselves will have a child born with a congenital heart defect if they decide to have Children of their own.

spk_4:   37:53
That's all excellent advice, and those are all excellent points that you made. We only have one minute left, so I'm hoping in this last minute you can tell me what advice you would offer parents of Children with complex congenital heart defects or even adult survivors if they should feel that something is wrong. How compares? Make sure their Children are receiving all the medical care they need without going overboard.

spk_7:   38:13
Sure, I think it's really important in this sort of echoes the conversation we already had, which is to say that you really need to partner with your primary care provider and your cardiologists. If you're concerned that something seems unusual to speak to them and say, What is your experience? Do you think that this is unusual and warrants further exploration? Or do you think that this is sort of part and parcel with my history of my surgeries, my heart disease? And really, it's something that we should have expected and shouldn't be surprised by, and they can help you with that? If you're not quite sure, as long as you are on the same page and feel as though they're really understanding what it is that you're concerned about.

spk_4:   38:47
That's excellent advice. I think so many of us who are parents. I feel like we become friends with our Children's doctors because they do become part of a care team. You feel a closeness to them that you wouldn't normally feel to your child's pediatrician or pediatric cardiologists. But in the heart world, I feel like we get really close to those who are providing care for our Children.

spk_7:   39:07
Well, I think that that's a natural result of spending, first of all, more than the average amount of time with your child doctors, but also making very important decisions together and that sort of once he's built that trust that breeds that feeling of teamwork by working together

spk_4:   39:20
absolutely well. Thank you so much for being on the show. Dr Roberts, you were awesome, and unfortunately, now it's time for another commercial break. But don't leave yet. We still have today's miracle moment, and that will be coming from the heart of a father. Find out what Jacob's bright smile might mean when we return to heart to heart with

spk_3:   39:37
Anna Thing Heart to heart with Anna 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 the C H T community. Please visit our website at www congenital heart defects dot com For information about CHD, the hospitals that treat Children with CHD summer camps for CHD survivors and much, much more. You

spk_2:   40:35
are listening to heart to heart with Anna. If you have a question or comment that you would like a dress on our show, please send an email to Anna Gorski at Anna at heart to heart with anna dot com. That's Ana at heart to heart with anna dot com Now back to heart to heart with Anna

spk_3:   40:56
Welcome back to the 12th

spk_4:   40:57
episode of Heart to Heart with Anna, a show for the congenital heart defect community. Our purpose is to empower members of our community with resource is support and advocacy information. Now for our miracle moment for a miracle moment today comes from the heart of a father and is written by Tim Bread Hower. Tim's essay is entitled Jacobs Bright Smile and I will be selecting excerpts from the essay to read the essay and full please turn to Page 101 in the heart of a father Tim, writes Jacob Bradley. Brett Hauer was born on August 1st, 1997 August 9th. We were given the worst news of our lives. Jacob had hiked book classic Left Heart syndrome. I remember the doctor pulling us into a side room away from the rest of the family. She told us the news and been drew some crude pictures to show us what was wrong with Jacob's heart. When she completed our education, she began telling us our options. We could go with the three stage open heart surgery, a heart transplant or compassionate care. The third option meant that he would be made as comfortable as possible and allowed to pass away. We were not quite ready to let go. We were given time to think about this decision, but not much. The doctor felt it was necessary to give a statistics and details on the various procedures. The doctor told us that only about 20% of the patients survived all of the scheduled surgeries. We were also told that at that hospital, no child had ever waited more than 10 weeks for a new heart. We were then shown a picture of a child that was doing outstanding. We have learned many doctors have a bias one way or the other. In her portrayal of each procedure, we were sucked into this bias and shown one side of the story. We basically had the choice of 20% survival rate three different times in the poor quality of life, or almost guarantees success as it was portrayed, tow us with transplantation. Given the same choice. Where did the same way most people would choose guaranteed success. We chose that as well. Now we could do nothing but wait. Doctors warn you that while waiting for heart, your child will get progressively worse. Their weight will go down, other organs will fail and other complications will arise. Fortunately, the 1st 2 never happened to Jacob. He gained weight. His other organs never came close to failing. Jacob was being kept alive because he was taking a medication that made his heart function as it does in the womb. The tube called a Peyton Detectives. Arterial, says her Petey A allows blood to pass between the main cardiac arteries. The medication keeps the P D A open when it usually closes anywhere from hours to 30 days after birth. One of the complications we were never warned about was that this whole could close. No one had waited more than 10 weeks before at this hospital, so it had never been an issue. Unfortunately, we got a rude awakening about the danger of this complication. Just before Jacob hit his 10 week point, a little girl waiting for a heart hit her 10 weak point. She had to have a procedure to break open this whole and buy some time. Sadly, something went wrong, and she died as a result of the procedure. Shortly thereafter, Jacob needed the same procedure. Jacobs survived, but the next day he had a stroke. Later, we learned it was a major stroke, affected the entire right side of his brain. The doctors felt he would never crawl here or maybe never walk. Loma Linda was unsure whether they wanted to do a transplant on him. It took over a week before they decided to go ahead with the procedure. With a stroke behind us, we were left with nothing to do but wait. They let us dress him as a pumpkin for Halloween. We started planning for Thanksgiving. We got a call late on a Thursday that the heart had come in. The surgery took eight hours. Jacob was in isolation for two weeks after surgery. We had waited four months for Jacob to get a heart Jacob Progress well after that, and we brought him to our real home in early March of the next year. Since that time, Jacob has had problems with other areas and related to his heart and thought to be other birth defects. The main problem was with one of his kidneys. The kidney problem caused him to take medication to make his kidneys function better and with less strain. He had extreme, a complicated by his knee Orel, which is one of the medications he takes for Hiss heart transplant. However, his heart has never had trouble. It takes them longer to get over a cold. Yet, considering where he was, Jacob is generally healthy. He went to a special education preschool for a while. This was mainly due to limitations from a stroke the stroke weekend, his left side. He had trouble with fine motor skills. The doctors think that Jacob may have been born left handed. He has been forced to become right handed. This made writing especially difficult for him. The doctors who said Jacob would never crawl were right. He didn't. He just learned to scoot around on his bottom. Children are very resourceful in overcoming obstacles. However, Those same doctors were wrong when they said he wouldn't hear or walk. His hearing is fine. He walks and even runs. He went to physical therapy for about four years and had fine motor therapy. He no longer needs therapy of any kind. He is in regular classes, and he is in the upper half of his class. He struggles sometimes to find the right words, to say what he means and stutters or repeats himself until he finds the right words. Yet he speaks at his age level. He can read some and knows his alphabet by sight and loves to display this challenge as much as possible. Jacobs bright smile and never give up personality or an inspiration not only to me and my wife, but to every person who meets him. To read the full essay, please turn to Page is 100 went 107 in the heart of a father that concludes this episode of Heart to Heart With Anna. Today's episode was meant to educate and empower the congenital heart defect community. Although hearing about non cardiac health issues that many of our CHD survivors face could be scary, it's even scarier when our heart warriors grow up, stop taking their medications, stop continuing to see their cardiologists and become lost medical care. When this happens, more severe problems can, and often to crop up later. Instead, we hope that this show has illustrated some possible problems that are not uncommon steps that can be taken to ensure the best quality of life possible and waste for parents and survivors to become strong advocates for themselves wanna thank again my excellent guests today, Lauren Bernard's David Simpson and also Dr Amy Roberts. They did an excellent job in helping us to understand better non cardiac health issues in Children with congenital heart defects. Please come back next week when our show will be called. What is normal child development for survivors of congenital heart defects? Until then, please find unlike a some Facebook, check out our website heart to heart with Vienna. And remember, my friends, you are not alone.

spk_1:   47:31
Thank

spk_2:   47:37
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.