Heart to Heart with Anna

Embracing Life's Pulse Amidst HLHS and PLE Hurdles with Hollie Stephenson

April 30, 2024 Hollie Stephenson Season 19 Episode 445
Embracing Life's Pulse Amidst HLHS and PLE Hurdles with Hollie Stephenson
Heart to Heart with Anna
More Info
Heart to Heart with Anna
Embracing Life's Pulse Amidst HLHS and PLE Hurdles with Hollie Stephenson
Apr 30, 2024 Season 19 Episode 445
Hollie Stephenson

Send us a Text Message.

Witnessing your child's bravery and resilience as they navigate a life-altering condition like hypoplastic left heart syndrome (HLHS) is both harrowing and inspiring. Hollie Stephenson joins Anna to share the profound story of her son Tom, who not only fought through the early challenges of HLHS but also the complex battle with protein-losing enteropathy (PLE) in his later years. Her vivid accounts from Tom's diagnosis in utero, through his surgeries, and onto his impressive transition into adulthood, working in politics, is nothing short of remarkable. Their journey underscores the unpredictable nature of congenital heart defects and the critical importance of vigilant medical care.

Through Tom's story, we uncover the myriad of long-term treatments and daily hurdles that come with managing PLE. We discuss the innovative treatments at the Children's Hospital of Philadelphia (CHOP) that led to significant improvements in his health and share insights from another patient's experience where a medication for Crohn's disease offered unexpected relief. These narratives illuminate the evolving field of pediatric cardiology and the crucial balance of medication management, driving home the message that children with heart conditions can and should lead full, joyous lives.

Parenting is an art—especially so when your child has a heart condition. Our conversation traverses the delicate art of letting go, allowing our children to taste independence and embrace life's experiences, from participating in sports to taking on careers. We explore the shift from advocacy to supporting our children's autonomy, the complexities of medication management into adulthood, and the emotional journey that comes with each milestone. Hollie and Anna share their personal challenges and triumphs, offering a heartfelt insight into what it means to parent through uncertainty with hope and courage. Join us for an episode that's not just about the struggles, but also the extraordinary victories that come with raising a child with a heart condition.

& so much more
A bi-monthly podcast where we share the stories of our Caregivers, patients and...

Listen on: Apple Podcasts   Spotify

Support the Show.

Anna's Buzzsprout Affiliate Link

Baby Blue Sound Collective

Social Media Pages:

Apple Podcasts
Facebook
Instagram
MeWe
Twitter
YouTube
Website

Heart to Heart with Anna +
Become a supporter of the show!
Starting at $3/month
Support
Show Notes Transcript Chapter Markers

Send us a Text Message.

Witnessing your child's bravery and resilience as they navigate a life-altering condition like hypoplastic left heart syndrome (HLHS) is both harrowing and inspiring. Hollie Stephenson joins Anna to share the profound story of her son Tom, who not only fought through the early challenges of HLHS but also the complex battle with protein-losing enteropathy (PLE) in his later years. Her vivid accounts from Tom's diagnosis in utero, through his surgeries, and onto his impressive transition into adulthood, working in politics, is nothing short of remarkable. Their journey underscores the unpredictable nature of congenital heart defects and the critical importance of vigilant medical care.

Through Tom's story, we uncover the myriad of long-term treatments and daily hurdles that come with managing PLE. We discuss the innovative treatments at the Children's Hospital of Philadelphia (CHOP) that led to significant improvements in his health and share insights from another patient's experience where a medication for Crohn's disease offered unexpected relief. These narratives illuminate the evolving field of pediatric cardiology and the crucial balance of medication management, driving home the message that children with heart conditions can and should lead full, joyous lives.

Parenting is an art—especially so when your child has a heart condition. Our conversation traverses the delicate art of letting go, allowing our children to taste independence and embrace life's experiences, from participating in sports to taking on careers. We explore the shift from advocacy to supporting our children's autonomy, the complexities of medication management into adulthood, and the emotional journey that comes with each milestone. Hollie and Anna share their personal challenges and triumphs, offering a heartfelt insight into what it means to parent through uncertainty with hope and courage. Join us for an episode that's not just about the struggles, but also the extraordinary victories that come with raising a child with a heart condition.

& so much more
A bi-monthly podcast where we share the stories of our Caregivers, patients and...

Listen on: Apple Podcasts   Spotify

Support the Show.

Anna's Buzzsprout Affiliate Link

Baby Blue Sound Collective

Social Media Pages:

Apple Podcasts
Facebook
Instagram
MeWe
Twitter
YouTube
Website

Speaker 1:

If you have complications like PLE, do exactly what I did and many other people have done Get online, figure out who the experts are and reach out.

Speaker 2:

Welcome to Heart to Heart with Anna. I am Anna Jaworski and the mother of an adult with a single ventricle heart. That's the reason I am the host of your program. Today's guest is Holly Stevenson. Holly is the field placement coordinator for a university's school of education and the mother of Tom, who was diagnosed in utero with hypoplastic left heart syndrome in 1999. Following his initial three-stage surgeries, he led a fairly normal life until protein losing enteropathy, or PLE, struck when he was 10 years old. This diagnosis led them to Children's Hospital of Philadelphia, also known lovingly as CHOP, where he was monitored and treated for over a decade. He is now a college graduate who lives independently and works in politics. Yay, I just love any time I hear one of our kids make it to independent living. It's like that's so amazing. So welcome to Heart to Heart with Anna Holly. Thanks.

Speaker 1:

I'm excited to be here and to have the chance to talk with you today.

Speaker 2:

Oh, I'm so excited about it too. So you and I know each other from our Facebook group. We belong to a group that is for parents of adults with CHDs, and I know that Tom was diagnosed with HLHS and had the Fontan. So can you tell us briefly about when he had his Fontan and any other open heart surgeries he's had since the Fontan?

Speaker 1:

Sure. So I was actually thinking about this this morning because next month it will be 25 years since Tom was diagnosed. I was four or five months pregnant at that time and he was diagnosed in utero, which was a good thing, and we were not able for him to have his surgeries here in West Michigan where we live, but fortunately University of Michigan, which is on the east side of Michigan, is only a couple of hours away. So we were referred to University of Michigan, to the amazing Dr Beauvais.

Speaker 2:

Dr Bouvier Correct.

Speaker 1:

And so Dr Bouvier Tom was actually born at University of Michigan in September of 1999. And he had his Norwood there returned for his hemiphantium had a few complications. After his hemiphantium he had an infection, and so he had to return to University of Michigan to have a debriding, I think it was called then, where they just cleaned out the infection in his chest. Yep Right.

Speaker 2:

Yeah.

Speaker 1:

Yeah, and then returned for his Fontan the summer before he turned two and recovered from that one. So after having had a few issues after the Norwood, he was not a good eater as a baby and after that infection after the honey Fontan, we were nervous for the fontan but within a week he was home and running around just doing his thing and really after that had a pretty easy life. He's never had another open heart surgery. He's had a few different heart casts over the years but it's never required another surgery.

Speaker 2:

Wow, so he has not had a fontan revision.

Speaker 1:

He has not. No, in fact, his fontan. When we would go to shop which you know we're going to talk about in a few minutes they would say, oh, this is a Beauvais Fontan. It looks a little different than what we do here, but it's very steady. His heart is in great shape all the way through. That's never been an issue. It's kind of a puzzle, right, like why do you get PLE when you have a really good Fontan? But that is Tom's situation.

Speaker 2:

What events transpired that led to that PLE diagnosis?

Speaker 1:

We had a great cardiologist. So the very same cardiologist that diagnosed Tom in utero was his cardiologist all the way through until he aged out of pediatric cardiology and he was decently proactive about things. So once we had surgeries and transferred back to his care in West Michigan he would do labs and different things. Through back to his care in West Michigan he would do labs and different things. And so when Tom was nine he did labs and he reached out and said hey, I'm seeing something funny with this albumin number and I'm concerned. And the time had no symptoms and I'm concerned that it could be that it could be the beginning of PLE. And so then we went ahead with the stool tests the 24 hour stool test and then he was diagnosed with PLE. But again we went ahead with the stool test the 24-hour stool test and then he was diagnosed with PLE, but again he had no symptoms at that time.

Speaker 2:

It's so bizarre. Yeah, wow, I'm amazed that they decided to go ahead and test him even though he was symptom free.

Speaker 1:

Yeah, it was just a routine. I don't know if it's a CBC, whatever. You just have routine blood work is where the albumin showed up, and then the puzzlement about why are we seeing low albumin? And so that's how they found it. And it was another year after that, before he showed any symptoms at all of PLE. And his biggest symptom he's going to be embarrassed if he hears you talk about this was scroll swelling. Yeah, that was his biggest symptom.

Speaker 2:

You're the first person who's taught to me about that. I've had a number of shows on PLE and nobody has talked about that. Fascinating, okay. So wow, now he was 10. So he tell you, because we moms aren't normally looking in that area when our kids are 10.

Speaker 1:

Yeah, he did. My recollection at the time was he was playing Little League and it was hard to run and that was what had him telling us what was going on. And because we had had that blood work and that testing done a year ago, it didn't take too long to figure out that this was the beginning of PLE actually manifesting. And so then doing some labs and things, you could see that when his albumin would drop below two, then that would be the symptom that he would have.

Speaker 2:

Wow.

Speaker 1:

Yeah, obviously our first step was we went back to University of Michigan because I think our doctor in Grand Rapids had one patient with PLE.

Speaker 2:

Yeah, it's fairly rare, friends, correct? Don't think that this is definitely going to happen to your kid, no, but it is important to know about it because it does happen, and we know that, just like hypoplastic left heart syndrome is fairly rare. And yet here we know a whole community of people whose children were diagnosed with that.

Speaker 1:

So yeah, right.

Speaker 2:

Okay, so you went back to Michigan, to the U of M, and what?

Speaker 1:

did? They say they didn't have a whole lot. They were doing some work with a doctor who was doing some trials with heparin therapy for PLE, but they were having mixed results. So we were in touch with that doctor. Tom was actually impatient for a while because his albumin had dropped pretty low and the swelling was pretty pronounced and albumin infusions would take care of it. That's the protein that's missing. So they put the albumin back and you get better.

Speaker 1:

But it didn't last for very long and we were sitting there in the hospital. He'd had a heart cast. There was nothing to fix, nothing to change. Everybody was scratching their head, and I happened to read an article that Dr Jack Reitchick had written about PLE and their single ventricle program. So this would have been in 2009. Okay, and so I thought, okay, I'm just going to email this guy, and he called me when I was sitting at the hospital in Grand Rapids wondering what on earth we were going to do with this kid, and he said I think there's some things we could do out here that just aren't being done yet where you are. And so he invited us to come out and be part of their single ventricle clinic and it just made a huge difference, a huge difference to be in a center where they saw so much more of it.

Speaker 2:

Right, right, and they knew it, they had some experience, they were already doing some research. I mean, jack Roychick is just amazing. So, how did that affect your family? It sounds like some of you were in Michigan, where you normally live, and you were with him while he was in the hospital, and now we're talking about having to go to Philadelphia, that's a lot of adjusting.

Speaker 1:

Yeah, we're very lucky. All the way through I think we had good health insurance so we didn't necessarily have to worry about the cost of going out there. The cost of going was covered by our insurance. We did have to pay for travel and we had to pay to stay, although the Ronald McDonald House was helpful and we were just lucky. We live in a small town in Michigan, so we live near Grand Rapids, but we're actually in a small town that throughout Tom's life had surrounded us. My mom was here and so we were able to leave, and our daughter, who is four years younger than Tom, could stay with my mom, could stay with friends and was supported. When we first started going out to Philadelphia, we were going and staying like a week or more at a time, sometimes longer if Tom had to be impatient.

Speaker 2:

Wow.

Speaker 1:

But I was able to leave my school and my students in good hands and our daughter was in good hands and we worked it out. Tom was nine or 10 when he went for the first time. It took four or five years to get the PLE under control. So it wasn't a magic. You go to CHOP and they give you some medicine and then it just goes away. That was not how it went for Tom. It took a lot of trial and error Again. Initially we were going every three months. We were having labs done every couple of weeks. Nothing was changing. Nothing was improving.

Speaker 2:

I've had a couple of friends, Holly who have had the Fontan and have developed PLE, and the doctors recommended a heart transplant as a way to deal with the PLE, since PLE is life-threatening. Did Tom's doctors ever talk to you about a heart transplant?

Speaker 1:

They did. And I think if we had not gone out to Children's Hospital of Philadelphia, out to CHOP, we probably would have gone down that route. We did get to a point out there. We had been working on getting it under control for three or four years and it was just still this roller coaster up and down, up and down.

Speaker 1:

So there came a point where they had said, ok, we're going to do one more cast and we're going to try to open his inferior vena cava and his leg had been blocked since he was young, and so they talked about we're going to try one more cath, see if we can get that open, and if that doesn't work, then we're going to send you back to Michigan and down the heart transplant path. So we actually left that time with the packet to take to Detroit Children's and to University of Michigan, because it was back before the computer necessarily was playing nice. Everywhere they were packets to take to them so that we could consult with them for transplant, and then he got better. For him it really was something about the blood flow and that inferior vena cava being blocked.

Speaker 2:

On a Fontan patient. You would think that's critical, because that's part of the way that they get their blood to their whole body and it's not like a person who doesn't have a Fontan, that inferior and superior vena cava connections they're critical.

Speaker 1:

Yes, Something had happened when he had his cath before his Fontan. That's when they first noticed it was blocked. They just had never used it or tried anything with it up to that point. But we used Dr Rome at Children's Hospital of Philadelphia, who was also amazing, and he was able to get it open. The other thing with PLE, every kid is different. I know a lot of kids with PLE and nobody's path is the same. But for Tom that was the ticket and really since then, almost 10 years ago this fall, he's had a few little blips on the radar, but he's really not had too much trouble with it since that time.

Speaker 2:

That's just amazing to me, and that they were able to figure out. Let's try this. I mean, even if it didn't improve the PLE, I would still think it would be better for him to have that inferior vena cava open. And the thing is he was born in the 90s, so we're looking at the early 2000s and it doesn't seem like it's that long ago when we're talking about us and how old we are. However, in pediatric cardiology it was so relatively young. At that time they were just starting to do all of these stents in kids and try experimental things where they had seen some success elsewhere first. Our kids are groundbreaking pioneers in this area, so that's pretty amazing. The treatment for him over the years really hasn't changed much then.

Speaker 1:

Not a lot. I don't know if you've ever interviewed Glenn Barnard. I don't know if you know who that is. Glenn is a PLE Fontan patient from Children's Hospital of Philadelphia, but he's like 30 or something now. He's not a kid anymore.

Speaker 1:

But he was struggling with PLE and his Fontan. He wasn't eligible for transplant so they weren't getting the PLE under control. He wasn't eligible for a transplant. To get the actually 100% accurate story you should talk to him or to his mom, but my understanding of the story is that his mom said what if we tried this steroid for Crohn's disease?

Speaker 1:

Diarrhea is such a huge piece of PLE. It wasn't really for Tom, but for most people it is. At CHOP, dr Rychik and then Katie Dodds was his amazing nurse practitioner they put Glenn on the steroid for Crohn's disease called Budesonide and it worked. And so Tom came through things at the time when that was the standard therapy. Was this Budesonide therapy? I know it's not necessarily anymore. There are different things that they're trying now, but that was what he used and he still takes that. I think he takes three milligrams a couple of times a week, so a very low dose, and that was his treatment protocol, along with just lots of diuretics. Like there's this really fine balance with Tom. I was like you got to keep him dry but you don't want to dehydrate him.

Speaker 2:

So he doesn't get albumin treatments anymore, like he did at the beginning. No, he does take this drug, but otherwise he's living a normal life and he's got his PLE under control.

Speaker 1:

He does?

Speaker 2:

Do they ever say that it's cured, or are they careful and just say this is something that he'll always live with? It's something they always have to pay attention to, but it's treated right now.

Speaker 1:

They use the word remission, like they do almost with cancer patients, so they talk about it being in remission, Okay, and he's had a long-term remission. I think they go back and forth about well, should we just take him off the steroid? Was that really what was helping, or was it this cath that opened the IBC? Dr Jack Rychuk, if you've ever talked to him, is huge for risk-benefit analysis. I've sat with that man many times and had him talk about risk-benefit analysis. So at this time the risks of being on a steroid long-term they feel like the benefit of not having PLE return. That's a good risk for Tom to take and again, it's a very, very low dose that he's taking. At this point. He used to be at one point on nine milligrams every day and now he takes like three milligrams a couple of times a week, so it's a much lower dose.

Speaker 2:

Treatments for PLE and HLHS have changed over the years, which we've already been discussing, but the challenges that the parents face, that's the same. So what have you learned over the years and what wisdom would you like to share with our listeners who are possibly being told that Galey diagnosis for the first time?

Speaker 1:

There were some people that were pretty instrumental in looking my husband and I in the eye and saying we didn't do all this work to bring this kid to where he is, for you to take him home and put him in a glass box. Yeah, we were encouraged to let him run, let him play, let him get banged up, let him fall, let him try. Our cardiologist, Dr Jeffrey Schneider in Grand Rapids, who we love, would say he will figure out what he can and cannot do and you just have to trust that he's going to figure it out. And yet I know a lot of people and I'm sure you do too who the fear. They just can't. So then the heart becomes the whole of the kid. It permeates everything about their family, permeates everything about the child's life, and I think that's probably my best advice is you've got to set that fear aside and let your the child's life. And I think that's probably my best advice is you've got to set that fear aside and let your child live their life.

Speaker 2:

Yeah, I think that's so critical. Yeah, absolutely yeah, I worry being an advocate and going out and talking about it and all that might somehow negatively impact my children, and I actually took a break from doing all of that for about 10 years just to let my child grow up and live without that overshadowing. Even though I felt it was important, I did still have my books out there, I did still write to people, but I did take about 10 years off just to, like you said, be there, be present. I was a robotics coach. I did all kinds of things that moms of children without a heart defect would do, because that's what I wanted. I wanted my child to grow up feeling as normal as possible, and in a Jaworski household that's not easy, I think that's excellent advice.

Speaker 2:

That is such good advice. I wouldn't necessarily expect that with a PLE survivor. That, yeah, just still let them live their lives. Don't let this be the focal point of what they're dealing with, because everybody's dealing with something, right? Yeah, some of my friends had kids with asthma. Some of my kids had friends with ADHD and they were on drugs for that. We're all dealing with something, but not letting that overshadow everything. I think that's excellent advice.

Speaker 2:

Yeah, also, I think what you've done and you're not giving yourself enough credit for this, holly is you've been that powerful advocate. You've been that parent who was out doing the research who reached out to Dr Rychik. There would be some people who would be too afraid to reach out to such a noted figure. You know they would think, oh, I can't do that. But you're complete evidence that, yes, you can reach out to these doctors and if it's a good fit, they will reach out to you and they can provide help.

Speaker 2:

But had you not reached out to Dr Rychik, he wouldn't have known that he could help you. So I think being that advocate and not being afraid to reach out and ask for help that makes a huge difference in the lives of our children. Think about how different your life would be right now if your son had had a heart transplant instead. It might still be a great quality of life, but it certainly would be different, wouldn't it? Yeah for sure. And we know that with transplant, you're just trading one set of problems for another, and I actually had a friend whose child had PLE got the transplant and it did not reverse the PLE, so transplant is not a definitive fix for.

Speaker 1:

PLE. Yeah, when I make that little spiel to people about letting them live their life and letting them fall down, letting them play baseball, letting them do all these things like we were never negligent with him, of course, because we know people that fall into that camp too, where they just ignore all of it, right, we were never negligent about appointments or medications and we had to provide monitoring that he might not even have been aware of. You better believe this. It was a sport my husband was coaching. Yeah, there was some behind the scenes things that we had to do, but, yeah, just letting him do his thing, and I think as an adult, sometimes that's even harder. I've even less control now over what he does.

Speaker 2:

That was my next question to you was oh my gosh, it's hard enough to parent a child with a heart condition and with PLE. As soon as you add in those complications, it makes parenting so much more challenging, especially when you have other children, which both you and I do. But now, as an adult, oh my gosh, I find I'm having a whole new set of problems being the parent of an adult, because I have zero control, and control is an illusion. It's not like I had tons of control when my child was younger, but I did have control over taking my child to the appointments, talking to the doctors, knowing what was going on, making suggestions. As a parent of an adult, it's totally different. So tell me about how you are viewing parenting an adult with PLE versus parenting a child with PLE.

Speaker 1:

Tom's really good. He's really good about taking care of himself. But I think to get to that point, there were some times that I may or may not be 100% aware of where he did let some things go and then he didn't feel very good. But he had to do that to understand that this is how it was going to go. And there's a lot of different stories I can tell, but I feel like he's probably going to listen to this, so I'm going to respect his privacy a little bit. But the one thing that we really had to work on was he's on Coumadin. So another thing that we didn't talk about was Tom. He had a stroke oh, I don't know if it was two or three years ago, it was during COVID a very mild stroke out on the golf course on Mother's Day.

Speaker 2:

Oh no.

Speaker 1:

Yep and landed in the hospital and they believe that he'd gone on to Xarelto and they believe that that might not have been the right medicine for him. There's not a lot going on as far as research on that medicine with kids, so he went off of that and back on Coumadin, but it requires a lot of monitoring.

Speaker 2:

Right right, coumadin is so different. My kid is on Eliquis and that is not as monitoring dependent as Coumadin is. But now I'm finding that that has its own set of issues as well. My mom was put on Eloquist and she didn't have any issues with it that I know of. Do you remember the days? And, holly, maybe I'm older than you, so maybe this wasn't the case for Tom, because my kid will be 30 this year, which I just can't believe but all we had to give them for anticoagulants was baby aspirin, just baby aspirin. That was so easy and that didn't have to have this strict monitoring of Coumadin or Eliquis or any of these other drugs do. And it just seems so easy. And now it seems so scary. So he had a stroke.

Speaker 1:

He did.

Speaker 2:

Yeah, did he just fall on the ground what happened To let you know, because strokes can affect people very differently.

Speaker 1:

So we were golfing. We got to the eighth hole and we started to talk about well, where were we going to go, get something to eat or whatever? And I don't remember if he dropped a golf ball or if he dropped his phone on the ground and he leaned over from the golf cart to try to pick it up. And he couldn't quite pick it up. He just said I just feel a little bit strange, mom, and I thought, well, that's weird. And then we kept going.

Speaker 1:

Tom calls it the stroke hole now, when we play on the golf course, I'm not, I had no clue that that might be what it was, but something was off. And we got to the end of the hole and we were debating because it's Mother's Day, do we just wait and call the doctor in the morning, or do we go up to the emergency room? And finally we decided better safe than sorry, which is how we've generally been. And man, I've never seen people just around somebody quite so fast, and they really didn't think that's what it was. He landed in the hospital. He had to wait like a couple of days before they could get an MRI on him. It was kind of a weird deal. And when they did the MRI, then they could see that in fact he'd had a small stroke. It didn't really leave any lingering effects.

Speaker 2:

That's good. Was it a TIA, a transient ischemic?

Speaker 1:

I'm not sure what they ever called it. Ever called it. But the issue became he wasn't being great about going and having his blood tested. They would call me and they'd say we know we're not supposed to call you, but it's been six weeks since he had his blood tested and he's not responding to us. It took a little trial and error but now he's good about it. But that's the hardest thing with adults is letting them try and fail a little bit, because we know if they fail, it could be fatal.

Speaker 2:

This is what's so scary, and here's that tightrope I feel we're walking. As parents, okay, we want our kids to live a normal life where they're not constantly focused on their heart condition. What that requires is that we have to let go, we have to trust, we have to believe in it, but they're going to test their limits. My heart warrior told me, mom, I'm sick of this, I'm sick of having to take the medicine. I feel fine, I don't need this. And when I tried to explain, you feel fine because you are taking the medicine.

Speaker 2:

You feel fine because you are seeing your doctor every six months. This is what's enabling you to feel fine. She had to figure it out on her own. Fine, she had to figure it out on her own. She had to feel bad and luckily she didn't have a stroke and I told her I'm worried you will have a stroke or something horrific is going to happen to you, but when they are in their teens and early twenties they are invincible, just like all teens and 20 year olds are invincible.

Speaker 2:

So I think that's one of the hardest parts about being the parent of an adult is watching our children, knowing something horrific could happen, but we can't be hovering over them or else they may push us away permanently.

Speaker 1:

Yeah, yeah, and he's really come around. Now he finally got to the point where he called me or he texted me. He'd say, hey, I got my blood done today and this was my level and I have to do it again in six weeks or four weeks or whatever, because then I would put in my calendar. So that is a system that has worked, but I get it. I was a high school teacher before I went to university. Developmentally, I got where he was at. He had to figure out, but it was hard to watch it and hard it's so hard.

Speaker 2:

So, parents, if you are out there and you're in the same position, we know, know, we know how hard it is. But I don't know about you, holly. But I did eulogize my own child at times in my head when I let my child take that black belt test and it was a really hot day in central Texas and they were warning people with heart conditions and asthma not to be out. And here my kid was going out to run the mile to earn the black belt and I wasn't allowed to be there because they didn't want parents hovering over them. And I remember walking around in my house with tears thinking, oh my gosh, but if I don't let my child do this black belt test, then I'm denying them that childhood that every child has a right to have.

Speaker 2:

Did you ever go through that too, where you had to let go because who deserved the right to do that more than our kids, who have? Suffered and struggled so much just to be alive. I think psychologically it's so hard on us because no parent should have to eulogize their child and I remember a story and I think things happen for a reason.

Speaker 2:

So there was another mom who was a taekwondo mom like me and she had a child who also had special needs and when that child went to do the black belt test he sat in a parking lot with binoculars and I remember the instructor not being happy about that and letting us know that's not our role, we're not supposed to do that. He couldn't do anything about it. But everybody knew she was there with the binoculars and I said to myself I am not going to be that, mom. What about you? Did you have any experiences like that?

Speaker 1:

I don't know, gosh, I wish something was sticking out in my head. I don't know that. I think there's something was sticking out in my head. I don't know that. I think there's one experience sticking out my head, but I do think that I've been the binocular mom sometimes, right Like I'm going to let you feel like you're getting to do this just like everybody else, but I'm going to put these little safety nets in place for you that you don't even know are there, just in case you need them. I do think I was kind of that mom.

Speaker 1:

I'm trying to think, especially in his adult life, because honestly, sometimes for me I think it's a relief that he's not right here anymore in terms of risky behavior and he wasn't huge for risky behaviors. But maybe I don't want to know if you're having more than one drink and maybe I don't want to know if you're staying up too late and getting up too early and you're eating too much junk or you're drinking too much Coke. And because he does really like a good Coca-Cola Sometimes I think maybe it's easier not to be able to have a front row seat to it anymore. But definitely when he was younger and he was here, there was a lot of go play baseball. But here are all the safety nets we're going to put in place just in case, and you know, go do this.

Speaker 2:

Safety nets we're going to put in place just in case. And you know, go do this, go do that and, like you said, some of them are not known by the child. I mean, we have to do that. That's doing our due diligence. So, I feel like in some ways we have to kind of be a helicopter parent more than what we would like to be, just because it's that risk benefit that you were talking about before.

Speaker 2:

We have to do that, but our children don't always have to be aware of us doing that I think that is something that all of us have to do and I agree with you. There is a little bit of a sense of relief that I am not waking up every single morning, afraid I wake up checking on my child to make sure my child made it through the night I don't know how long. I went through that years years.

Speaker 2:

It'd be the first thing I think about every morning and the last thing I think about every night. Now that my child's an adult and has made it, that's not something I worry about every single day. I do not text my child every single day to make sure they're alive. I kind of take for granted that they are, which probably is smart, but I have been able to finally let go a little bit. As far as all of that's concerned, we do touch base weekly but, I don't feel like I have to be there every single day.

Speaker 1:

Yeah, Hockey. I mean I'm sitting here thinking about that. That's probably the big thing now as an adult. So Thomas plays hockey. Oh yeah, Like on a we call it a beer league here in Michigan. I don't know what you call it in Texas, but we call it a beer league. It's just a bunch of guys that maybe they played in high school and he plays hockey and he has talked it over with his doctors. You said on Coumadin.

Speaker 2:

Yeah, okay that surprises me On Coumadin, because I just went to my very first hockey game. Last week. I went to a minor game in Round Rock, my husband's work. He had a. I went to a minor game in Round Rock my husband's work. We had our own box. I saw my first hockey game and I've heard about hockey over the years so I thought there would be lots of blood and fights. There wasn't. It was a nice game. There was shoving and you know people did get pushed into the wall, but I didn't see any blood so I was happy about that. So is it kind of like that where it's not as horrific as you might hear some of those stories on the news?

Speaker 1:

Well, it's a no contact situation too. So I think that's a piece of it, and they tag out a lot. So he'll come out on the ice for a little while and then he just goes back and somebody else comes in and I think Tom's big thing like. I remember him as a little guy like five, six years old, and he would find ways to be part of the games the kids were playing. So if that meant he stood in the middle of the circle and everyone ran around him, that's what would happen. I see the adult version of that with the hockey. So he gets out there, he does what he can for a little while and then he tags out.

Speaker 2:

The biggest fear for me is him getting hit with the puck Right. Those pucks, they're going fast. Yeah Right, he would bruise terribly. Oh, he has, that would be scary.

Speaker 1:

Yeah, the first time I went to watch I thought I don't think I'm going to be able to come watch this again, but I do go occasionally and watch and it's okay. It just goes back to. I have to trust the people who care for him, who have studied this and know this and understand this. I don't know better than they do what's okay for him and they're telling him it's okay as long as he wears all his protective gear, especially the helmet, and he gets to go, be on a team and enjoy Right, which is so important.

Speaker 2:

So important. I'm so glad that he has that. Yeah, and that is just amazing. So you've been dealing with this PLE for over a decade. What's the most valuable lesson you've learned? That might help other parents today who are dealing with a Fontaner who has PLE.

Speaker 1:

I think getting to a center that sees it Right. Yeah, I don't know if you're in the PLE group on Facebook that I'm part of, but I still see a lot in that PLE group when kids are diagnosed by a doctor who is well intentioned but doesn't see very much of it and their care is mismanaged. Far be it for me to point it out, but that's what's going on. If you have complications like PLE, do exactly what I did and many other people have done Get online, figure out who the experts are and reach out and, if you can, get yourself to those centers where it's being dealt with more. Chop was the key to Tom's success and to not having a heart transplant.

Speaker 1:

To this point.

Speaker 2:

And I have a friend who lives in England and they came to CHOP to help manage their child's care and their child is in his late twenties. Yeah, yeah, it does make a huge difference, because almost 30 years ago a lot of these kids wouldn't survive that long. So it really is miraculous, I agree. I think being in the right place at the right time makes a huge difference, and sometimes that does mean that you have to travel, even across the pond, as they say.

Speaker 1:

Yeah, there came a point where Dr Reitschik said remember when I said you should come here because we could do things for you here that people couldn't do other places. That's not where we're at anymore. And he said it's okay, it's time you can go back to Michigan now and go into their adult clinic and we're going to make arrangements for you there because it's okay, what we can do for him as an adult, they can do just as well in Michigan. So I think knowing when to put the gas pedal down and really go after it and when to be okay with the care that you can get locally, that's tricky. Things have changed.

Speaker 2:

Now we have some research so they know what's going on and other facilities can duplicate what's being done. The problem is when you're the first and only and you're trying to get that large cohort of people so you can study them and you can see what works and doesn't work, like you said. First of all, being a Fontaner, it's different for every Fontaner, and then to add the complication of PLE on top of it, there are probably no two people who are exactly alike, even if you have twins.

Speaker 2:

There's still going to be some differences, but you get a large enough cohort together and you can find some commonalities and you can find some things that work for most of the group or some of the group, and then you can even delineate oh, it works with this subset of the group that have these conditions, whereas this other protocol works for people who have these other conditions, and that just makes a huge difference. You can't do that if you only see two kids. You'd need that larger cohort, even if larger means 20, which 20 is still a very small number.

Speaker 2:

But when you have a situation that's as rare as this, 20 is pretty big and it gives you a lot of information, so much more than you would have if you only saw two yeah, for sure, for sure, yeah I cannot believe our time is up already. Holly, you have been absolutely amazing and I feel like I've learned so much and I feel like I have a kindred spirit there being a mom, so thank you so much for coming on heart to. I have a kindred spirit there being a mom, so thank you so much for coming on Heart to Heart with Anna today.

Speaker 1:

Well, you're welcome. Thanks for having me. It was great to be able to talk with you.

Speaker 2:

Oh, this has been so much fun, friends. That does conclude this episode of Heart to Heart with Anna. Thanks for listening today. Please consider becoming a patron of our program by visiting wwwpatreoncom. Slash, heart to heart or hearts unite the globe is our website and we have pages there where, if you don't want to join the patreon group, we have other ways that you can be a donor. And please remember my friends, you are not alone. Thank, you.

H2HwAnna Hollie Stephenson
Segment 2
Segment 3
(Cont.) Segment 3

Podcasts we love