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Anna's Buzzsprout Affiliate Link
Baby Blue Sound Collective
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I am Anna Torsky and the host of heart to heart with our Thevenin sees it is organ donation and transplantation. I'm very excited for today. Show to feature heart mom Shannon Ariaga. Today's show is titled Heart Transplantation due to protein losing enteropathy. Shannon Arriaga is the mother of Eric Jacob Area Olga, who was born with hypoplastic left heart syndrome, coarctation of the aorta, transposition of the great vessels of ventricular septal defect and tricuspid Atresia. Eric had a Norwood procedure at six days of age, a Bi Directional Glen at six months of age, and Fontana procedure at two. This is a pretty standard when it comes to children born with single ventricle hearts. What isn't typical is what happened next. Eric developed protein, losing enteropathy, a potentially life threatening condition. He battled this with a great sense of humor until he was 15 years of age. After waiting 50 days, Eric received a heart transplant at Texas children's hospital and his life dramatically changed today. Shannon will talk with us about Eric's transformation, how transplantation affected the family, and what advice Shannon has for other parents dealing with protein, losing enteropathy. Welcome to heart to heart with Anna Shannon. Hi, it's good to be here, but I'm happy for you to be here and first of all, I'd like for us to talk about when Eric first started having symptoms of protein, losing enteropathy or ple
Speaker 2:about six months after his Fontana procedure. He had his procedure when he was two years old, right when we came home. I didn't even have to potty train him. He went straight to the toilet. We didn't have any problems whatsoever. His hospital stay was very, very short. The doctors told us setting this kid up, he needs to gain some way given butter. We were giving him plenty of food. We thought he was gaining weight. We had no idea that it was fluid retention and then it was the day before Easter. He had developed a runny nose. He sounded a little wheezy. I wanted to take him to the doctor. I didn't want him to get sick from any of the other kids. I knew he was going to be hunting for Easter eggs the next day, so we took him in to the doctor. They saw that his oxygen level was at a 73. They transferred us from his cardiologist to brackenridge children's. He had so much fluid buildup around his lungs that his right lung was almost ready to collapse. We had no idea. Yeah. We had no idea what was going on. They pulled off two and a half pounds of fluid.
Speaker 1:Oh my gosh. Off a two year old.
Speaker 2:That is huge. Yeah. Then he was a skinny, once again, found out he didn't gain any weight and then they did the stool sample and they came back and they told us that he had protein losing enteropathy. It was very, very early for him to have gotten it. In his cardiologist opinion. She was very nervous for him. She's very scared. We had no idea what to do. So we were all searching for answers in the dark. Well, tell us how old your son is right now. Right now he is 17. He will be 18 on September the first. Okay. So this happened about 15 years ago, 15 or 16 years ago, and so I'm not surprised. Your cardiologist was really concerned because, golly, 15 years ago there really wasn't much known about ple. Yeah, piece, kind of one of the pioneers here to still be alive and to be doing okay and we'll be talking more about his journey, but just right now for talking about the diagnosis of the protein, lucy of neuropathy. I've had friends in the past whose children have also developed ple and when they talked to me about it, they said the first sign that their child had a problem was diarrhea, that they had runny stools and that was the first symptom that they noticed for protein losing enteropathy. But it sounds like for you it was fluid retention. Is that true? Yes. Now, to be clear, he did have runny stools, but we were always told that was just part of him being a heart kid and that he had a super high metabolism. We thought that came with the territory. He never had uncontrollable stools or anything. He just had regular tire area constantly. We didn't feel that it was out of control or anything. We just figured his normal stools were soft and loose, but it was mainly the runny nose, the puffy face, and the wheezing. The wheezing is what actually caught my attention. Wow, okay. And have wheezing was probably because there was fluid backing up on the lungs. Right, exactly. Wow. Okay. P develops this at two years of age. What measures did they take? Gosh, 15 or 16 years ago. What did they tell you to do to try and help combat this condition? Well, the first thing they did was called the make a wish foundation because my goodness. Yeah. They said he's already had surgeries. We don't know how it's going to turn out. Let's grant this kid a wish. They started that application process. They upped his lasix. They upped his oxen, and of course they said no more butter. Don't give them any more butter. Oh No. Let me tell you, this kid really liked his butter at this time. We were constantly in the hospital is a very, very dark time between age two and a half to about age five. He would swell up. He'd be back in the hospital with draining tubes. They would up as lasix again a couple times in an effort to dry them out. He got dehydrated and that was never fun. Well now because then they can get arrhythmias that can be life threatening. That's a lot to try and balance his sister. She's two and a half years older than him. She was with us all the time. I wasn't working at the time. I wanted to be at home to get him through this. I was available for him and uh, thank goodness it was about right before he turned five. The doctors came to us and they talked to us about Ivi, Aig and they were gonna give them a huge dose. They didn't know how he was going to respond. They pretty much said, call your family. This is one of the last things that we could do. The subject of heart transplant had come up, but his father knocked it down. He said, no, absolutely not. And so the Ivi Aig was their last ditch effort at that time for him. Okay. What does ge mean? It's an intravenous immunoglobulin, so it's basically a ton of white blood cells. A ton of white blood to help boost his immune system and they gave it to him over a 12 hour period and there were doctors and nurses in his room the entire time. They gave it to them very, very slow. They checked his vitals every hour and shockingly he came out of it like a superstar and his ple went into remission until he was about 11 years old. Wow. That sounds like a supercharged of white blood cells. Actually caused the ple to go into remission for six years. I have no idea if it was just that timing or the medicine, but it went into remission for six years now during this. Amazing. Yeah. We were very, very, very careful with him. This kid still has not had butter to this day or kid. We were just very careful with them health wise with what he was allowed to do. Now he was 96 percent pacemaker dependent. We don't know if it was just the culmination of everything, the medicine, prayers, everything. He just miraculously went into remission for six years and it was very nice. Six years now. He never grew. He grew very, very slowly. He was always at bottom of the charts, always the skinniest kid, always very, very tiny and he wasn't hitting his milestones, his height milestones or wait milestones or anything like that in our subconscious. We knew, we knew something else was going on and we knew that we would probably see the ple again. They didn't know that it was in remission. They told us he was cured. So we thought it was gone. We thought the to take care of it. Yeah. Wow. Okay. So you have six years where you're really not worried, but you were still cautious enough not to let him have the butter or anything that you were afraid might caused him to return to having problems. Right. And then when he was 11 years old, his pacemaker, the battery needed to be replaced. We noticed that he lost a lot of energy. We didn't understand what was going on. So of course, you know, we took him in and I asked them, can you check his pacemaker? Because sometimes he has a lot of energy and sometimes it's Kinda like, is better. You just shuts off. I don't understand what's going on here. They found that his battery had died and that backup generator had been working and I know it was going to be four days before a new pacemaker got there, so they wanted to keep him in the hospital for four days, but he told them, no, I want to go home. That's one thing. I've always let him call the shots on what he wanted to do medical wise, if you want to stay in the hospital or if you wanted to go home, because it was probably about the time he turned nine. He started kind of showing symptoms but not full on symptoms, so he started being in the hospital a little bit more often. They never nailed it down to the ple. They just said it was a complication of his Fontanne or he needed to be tweaked or he needed a tune up is what they would call it. Wow. A tune out, but what were they planning on doing with that tune up? That sounds really vague. Yeah. Sometimes he would have some fluid retention and they would pull some fluid off and they would change around his medicines and they would call it a tuna, but nobody said that dreaded[inaudible] word until after his new pacemaker was put into place. About three months after that, I knew, I knew the Plu was back in. I took him to the doctor and I told her, just tell me what it is. We all know what it is. That's when it came back with a vengeance,
Speaker 3:were offering us a mechanical heart and he said, no, I've had enough. Give it to someone. My father promised me a golden dressed. He held my hand and asked me where I wanted to go. Drive for conflict that we experienced in our long career together was always healed
Speaker 2:by humor.
Speaker 4:Heart to heart, heartless Michael, please join us every Thursday at noon eastern mass. We talked with people from around the world who have experienced those most difficult moments.
Speaker 5:You are listening to heart to heart with Adam. If you have a question or comment that you would like to address on our show, please send an email to energy water ski at Anna, at heart to heart with Ana.com. That's Anna@hearttoheartwithAna.com. Now back to heart to heart with them.
Speaker 2:So Shannon, you are taking us on this rollercoaster ride with you were first. You have this child who's born with a serious heart defect and he goes through these surgeries and develops ple, but gets this almost magical transfusion and is in remission from the ple only for you. A very intuned heart mom to realize something's not right. Something's not right. The pacemaker gets replaced. You know something's still not right, and then they say the dreaded words protein losing enteropathy again, and I'm sure your whole world just to still for a moment. So tell me what happened. What was the next thing that they could offer you to help your son? They of course does lay six. Again. They told us to keep doing what we were doing with the Diet. Everything just started making sense. Why they would take fluid off of him every now and then and it would happen more often. They started sending him to a gastroenterologist, an endocrinologist. He had all these specialists that he would go to. They would check his bone age. They would check his magnesium, potassium, albumin, and total protein, everything like that. A few times. He got really, really, really, really sick to where they told us, call your family. He's not gonna. Make It. Yeah. Three separate times. He came through every single time. What I started doing was I've always given him control of his medical future, what he wanted done, and we've always been very, very open with him in regards to this is what's going on with you, what would you like to do? These are the options and ever since he was first born, when the doctors were taking notes, I would ask them to hand me the binder. I wanted to read all of the notes they had on him. I would research. I'm a natural researcher, so I would research day in, day out, day in, day out, and I would ask them questions and I would give them situations from other pod cases that I knew of and I would say, have we tried this? Have we tried that? And I reached out thing. I will exhaust all. Lots of. Good for you Shannon. Yeah. You were a strong advocate and him and at a time when it was really necessary because there wasn't that much information. Right? So you were probably throwing a few curve balls to some of these doctors were good for you. Okay. So I love the fact that you are letting eric makes some of his own decisions for care and you're keeping him constantly apprised of what his situation is. So what happened? Well, I earned the nickname momma sister from him. Please do not let him tell it by side map because I'll be a mobster. He's like, you're kind of a monster, but you're a mom so you're my monster. I began having the doctors whenever they would make their rounds every morning, their rounds, if it was about my son, they would take place in his room and I would just almost demand that they talked with him and that they include him and that the answer any questions that he had and I would reach out to other parents whose children had ple and I took this kid across Texas a few times to meet other kids who had ple so he would know that he wasn't alone and I would ask them, what are you doing? What's working for you guys? So you started a support group for people whose children had ple. That's amazing. Okay. So I know that he ended up getting a heart transplant. Tell me what transpired to enable him to go that next step. He was 15 years old. He was four foot six inches. He weighed 64 pounds. His albumin was at a two and his total protein was at a one point seven and those are dangerously, dangerously low numbers. The integrity of his skin was extremely compromised. We couldn't put a bandaid on him without his skin ripping. He couldn't have stitches. Yeah. His skin would rip open. He was pretty much living in the hospital. We would come home now and then, but we would always go back to the hospital. I met Lisa Ball person is seen by Dr. Rutchik and I had my sons medical records sent to Dr Rutchik and I had them sent to Texas Children's hospital. His cardiologist worked with me and we got an evaluation at Texas children's. We went over there to Houston and it was a three day process. It's very, very intricate. So many interviews. They interview the parents, the siblings, the child to make sure that everybody knows what's going on and it's to make sure that you're mentally capable to handle the entire process. They did a heart cath on him. They took all of his numbers, x-rays, blood, urine, stool, and right away they said he definitely needs a heart transplant. He would not survive without it. Wow. Well, he was extremely medically fragile at this point. He wasn't your typical heart kid. He was a kid is it sounds
Speaker 1:to me living day to day. Pretty precarious. Lee. Yeah. Okay, so Texas children's and for those of you who don't know, Dr. Rutchik at that time was at children's Hospital Philadelphia where he still is, I believe, and he is in charge of the largest single ventricle survivorship program in the world. I believe so. He's very well renowned and I've known of other people who have taken their children even from the United Kingdom to chop to get help for PLA. There aren't that many centers that really know how to deal with this, so by this point all of you had to be convinced that the best option for Eric was a transplant.
Speaker 2:It was his last option. Pretty much. We were doing subcutaneous Ivi and so subcutaneous is right below the skin. Every Sunday I would put four needles into his abdomen and the infusions would go right under his skin to try and help him, and we were doing this once a week. He was taking 18 medications and we would still be in the hospital constantly with draining tubes attached to him. It was just a rollercoaster, it was a crazy roller coaster and when you're out of options you go for it.
Speaker 1:Right, right. And so he must have been pretty high on the transplant waiting list than.
Speaker 2:Yeah, he was accepted immediately. He was listed as a status one B. I relocated to Houston with him and my daughter stayed back here with her father so she could finish out high school because she was actually a senior in high school. Yeah. So
Speaker 1:I must have been hard for her to be able to focus and concentrate on all those things are supposed to be fun. Your senior year knowing that her little brother was going through this.
Speaker 2:Yeah, but she's very resilient. She would do her homework on the hospital floor anytime it was in the hospital, she'd constantly be there and she was in orchestra so she would take her cello in there if you would play her cello for all the kids.
Speaker 1:So need. Is that? Yeah. Okay. So I know from having spoken with you before that it was a 50 day wait, which really given conversations I've had with other organ recipients, 50 days is really not that much less than two months.
Speaker 2:Yeah. But I imagine it felt like a lot longer than that for your family. Probably. It actually happened so much sooner than I thought it would. I had gotten an apartment over there. So part of the protocol is you have to live within an hour of the hospital. So once they get that heart you have to show up for it. So I had to relocate to Houston. I was able to transfer my job there. I worked right across the street from the place that I found I had to be within 15 minutes of him at any given moment. So it was good. The team over there, very nice, very accessible. I could call any time, day or night and they would answer any questions I had. They would send doctors and nurses to our home to check on him if he wasn't feeling up to going to the hospital. And during the 50 day wait, he was hospitalized three times before we even got the call for the transplant. He really needed it.
Speaker 1:Absolutely. It sounds like it was a godsend that he got it as quickly as he.
Speaker 2:We did. Yeah. We got the call on November 14th. That was a Saturday morning. The day before that, Friday the 13th. Friday the 13th is always his favorite day. But that Friday the 13th, he said he felt weird all day and I told him, well, changes coming. That's why you feel weird and sure enough the next morning we got that call and we book it on over to the hospital.
Speaker 1:Oh my gosh. I bet you were excited. Scared, nervous feelings.
Speaker 6:All right.
Speaker 7:This is what we showed them. Let's party. Let's go
Speaker 1:forever by the baby blues sound collective. I think what I love so much about this cd is that some of the songs were inspired by the patient.
Speaker 7: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.com. Spotify.
Speaker 1:I love the fact that the proceeds from this cd are actually going to help those with congenital heart defects.
Speaker 7:Join the music
Speaker 2:forever
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Speaker 1:Wow. So it's time to party. Yes. You're in the waiting room. How long was the operation for your son to have the heart transplant? Oh my God. Oh my goodness.
Speaker 2:Oh, well, what had happened was we got the call at 5:00, got there before 6:00 and they did an x ray and they found that his heart had begun enlarging and fusing to his sternum. So yeah. So they actually had to, whenever they went to get the heart, they actually had to go speak with the donors, respective family again and let them know that they needed more than what they initially thought they would need the surrounding tissues. Oh Wow. Yeah. Other components of the heart than what they originally thought that they would need, and thankfully they consented so they were able to come back with everything that my son needed to ensure his survival. It was about midnight whenever they said that the borrowed heart's beating. It was great. I need a second.
Speaker 1:I'm so sorry. I know that just talking about this probably brings back a flood of memories and I can't imagine sitting in that waiting room knowing it wasn't enough for them to get the heart. They had to go back to the donor family and say, can we have more? And knowing that your son was on that table and they were kind of making it up as they went along. I have a feeling seeing what he needed,
Speaker 2:but it all. It all worked out. It all worked out really good. They thought that he was going to be asleep for between three days to a week. This kid woke up 45 minutes later. Oh my goodness. Oh my goodness. We were shocked.
Speaker 1:Wow. Okay. So then that's a little scary because you don't want them to wake up too soon after because they don't like being intubated and having all these things.
Speaker 2:Yeah. So they had to push extra sedation to knock them out, so.
Speaker 1:Oh my goodness. Okay. So he's in the hospital. He has this new heart and they're having trouble. So data you. It sounds like he's an energizer bunny. He wants to get up and go. How long was the recovery period for him in the hospital?
Speaker 2:He was actually only in there for 11 days. Yeah, he was so sick. He was so sick. They didn't know if he was going to come home. They thought it would be about six months before he was rehabilitated enough to come home. He woke up the next day they stopped dating him. They let him start waking up. They took the breathing tube out. He sat up and he started talking.
Speaker 1:Oh my goodness. This must have just made you so joyful. I was shocked. Yeah. I would be shocked to. Okay, so he sits up. He starts talking, which is pretty amazing. When did they dare to get them out of bed? He's the one who said, let's take a walk. Oh my goodness. It was up and walking around the next day. He had all his draining tubes attached on his medications and he was walking. He couldn't get enough of it. Does ple went away before he even woke up? It started resolving itself before he even woke up. Wow. So they could see a difference in his body because when you're in the hospital like this, they're constantly drawing labs on you and so they must have been taking all of these labs and been astounded at the fact that some of his labs were now coming back near normal or normal. Do I dare say normal as of now is 100 percent normal. It took three months for him to have a regular albumin level. Regular total protein level is immunoglobulin levels are perfectly at the normal rate. Everything. Everything is pink, so pink is a beautiful color. This. Yeah. We don't have to worry about 73 percent sex anymore. Wow. Unbelievable. Okay. Well I have to give a shout out to our mutual friend Lisa Bach because she's the one who put me in touch with you and I know that she's a really strong ple advocate and I know that you are too now because you've told me how you helped start a support group and everything that you did. What do families dealing with ple need to know about the decision to go the transplant route for treating ple? They need to know that every case is different. Every child's body is different and your child body will let you know what they need to do, what works for you. Okay. I think that's really good advice. There are some cases that I know of where a heart has been transplanted and sadly the ple has not gone away and I think one of the scary things about pla to me is there. So a lot of questions revolving around what causes it and how to get rid of it. The heart transplant, I may not be the solution to the problem. Right. And it's sad. It's very sad when that happens. Doctors don't know everything and there'll be the first to tell you that they don't know everything. It's the ultimate gamble. It is the ultimate gamble. I've known families whose children haven't survived the heart transplant. It's a very sad place to be in that situation. Luckily for us, Eric survived the heart transplant. He's thriving. He has a second chance at life. Right. And very, very thankful. It's amazing to me how you said you couldn't even put a bandaid on him without it tearing his skin and yet they did a transplant that's so invasive and he was so medically fragile at the time, but it sounds like this is exactly what he needed for his body to heal. Okay, so you told us how tiny he was in 65 pounds. I've got to know how tall is he big? Is he now?
Speaker 2:Although now he is five foot seven and he weighs about 125 pounds now.
Speaker 1:Wow. So He's tall and skinny?
Speaker 2:Yes. Yes he is. And he's filling out, he's getting some muscles on him. Will be. He has a lot of, yeah,
Speaker 1:growing yet to do okay. But now he is growing and that's the big plus here is that this new heart has given him what he needed so that his body wasn't just in a holding pattern, sustaining life, but now it's growing and thriving. Exactly. Unbelievable. Wow. This is such a miraculous story. It's such a beautiful story. I know. I don't want to add anything negative, but I have to ask this. It's been said that decided to go the transplant route is trading one disease heart for a different set of problems. So tell me what you think the advantages and disadvantages of transplantation with dealing with ple are.
Speaker 2:I can only talk about from our situation, the advantage of my son having a heart transplant and it be successful is that his ple is resolved. It is 100 percent resolved. There are no signs of ple. His sats are at 100. He has so much energy and that's good. So no more cardiologist. He sees the transplant team for his regular checkups. He has the biopsies. That's the good part. Okay. The cons. We will forever be looking out for coronary artery disease. You could transplant a heart, but the arteries can wear out fast or they can not wear out fast, so that's one thing that they're constantly looking for. They're constantly looking for rejection and they're constantly looking for coronary artery disease. Also, if the medicines are not exactly correct, these kids can develop cancer, different types of cancer. My son cannot be out in the sun at all. Really? He needs to have sunscreen on all the time because he's very susceptible to skin cancer. He can't be in a lake because his immune system is very suppressed, so he's at risk for getting any type of bacterial infections, so we're very, very careful of all that. You have to be careful. Colds, coughs, viruses, flu. He gets his flu shots every year. Of course, we all have to get our flu shots. We just have to be very, very careful. Thankfully he's done really, really well. He hasn't even had a cold. It gives a new meaning when you have a heart child to live each day like it's their lab.
Speaker 1:Yes, yes, I agree, but it sounds like his quality of life has changed dramatically.
Speaker 2:Yes, it has. He's doing really, really great.
Speaker 1:What advice do you have for other families who are now dealing with protein? Losing enteropathy?
Speaker 2:Listen to your kid. Your child will tell you what they need. When your child is not hungry, it's because they're really not hungry. Their intestines are not allowing them to be hungry, so a lot of these kids are told to eat a lot of food and gained a lot of weight, but the disease won't let them, so seek other options, seek other opinions, reach out to families, ask questions, seek second opinions medically, go above and beyond because that's what these kids need, and that's the only way we're going to get all the information about this disease is by compiling all these different cases and comparing information. Research, research, research, research. That's the best thing you can do is research.
Speaker 1:I know from having had friends whose children had ple that it's very easy to feel isolated and to feel like you're the only one, so reaching out to those support groups. There are pages on facebook that you can join which are also really helpful and I know that you and Lisa or involved with one of those pages and so I think that does help and the research. Absolutely. I think things have changed a lot over the last 15 to 16 years. Don't you think, Shannon?
Speaker 2:Yes, for sure. The social media age allows more families to get in contact with each other and it allows these kids to see that there are more kids out there like them going through the same struggles. Moms, you're not alone, dad. You're not alone. Kids, you are not alone. This is a scary place to be, but it doesn't have to be done alone.
Speaker 1:I love that. Well, thank you so much for coming on the program today. Shannon. You're welcome. This was really informative and I hope that it will give some inspiration and some hope to other families who are living with PLA right now. Well, that does conclude this episode of heart to heart with Anna. Thanks for listening today. My friends find us on itunes and subscribe and remember my friend. You are not alone.
Speaker 2:Heart to heart with Emma, a presentation of
Speaker 4:hearts unit, the globe, and as 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, empower, and enrich the lives of our community members. If you would like access to free resources pretending to the chc community, please visit our website@wwwcongenitalheartdefects.com for information about Chd, the hospitals that treat children with chd summer camps for Chd survivors, and much, much more.
Speaker 5:Thank you again for joining us this week. We hope you have been inspired and empowered to become an advocate for the congenital heart defect community. Heart to heart with Anna, with your hose down. Torsky can be heard every Tuesday at 12 noon eastern time.