They kept her in their cardiac ICU for five days and monitored her. She was doing well, tolerating it well, so they sent us home with her and hoped that she would grow just that little bit bigger before they had to intervene. She was just under a month old, when she was drinking her bottle I would notice that she would get really sweaty on her forehead and she would be gulping her bottle and then stopping for a second, like trying to catch her breath, and then gulping her bottle and sweating and I thought “Gosh, she’s working really hard to drink her bottle,” but that was the only symptom that I noticed.

At that point we went back in for an appointment and that’s when they did her first cardiac cath. They said she was symptomatic, it was severe, and they wanted to try to balloon it out a little bit. They did and it was beautiful and she tolerated it without any complications. They sent us home, and then right before she was 12 weeks old, maybe 11 weeks old, she had another balloon cath. The same thing had happened again, she started to become symptomatic again, and they did her second balloon, and that time it went really well again, however the problem with cardiac caths is when they balloon the valve open, then there’s the risk of aortic regurgitation. So after that cath she started to have issues with aortic regurgitation. Coming in post-procedure and seeing her being transfused with blood was like one of the scariest things—I don’t think that I expected that either. Being a nurse and somebody who gives blood, I didn’t think that would be such a frightening thing to see her getting blood. That’s something that I just can’t forget. So she tolerated that okay, but we were frequently at the hospital—monthly we were there for her echoes and appointments.

When she was 10 months old the aortic regurgitation got problematic enough that she needed to have her first open heart surgery. They were able to repair the valve using a piece of her pericardium which was awesome. She recovered really well. We spent those days in the hospital. The first day you’re in the ICU seeing her connected to all the tubes and the vent and all of the medications all at the same time I think still now, every time I physically enter an ICU of my own hospital and hear the vent machines, I see Isabelle and I can’t even get past that at my own job. But the nurses, everybody who cared for her was great. They always talked to us, they would talk to her.

After all of the surgeries, the hardest thing for her was nausea and vomiting from anesthesia. She would wake up and be thirsty and want to drink and would guzzle, and would vomit. She’s had three procedures, and every one that was like one of the biggest things for her to hurdle. But on the floor the first time she ever pulled herself up from sitting to standing in her crib was post-op after her first open heart surgery. It was the fourth day she’s in the crib and she pulled herself up. I don’t know what kids can do that, but she did. She’s just really strong, she’s really tough. That procedure lasted until she was 3 years old. They thought it might last a year, and it had lasted over two, so that was pretty awesome. And again we had really frequent visits every month or so we’d have our echoes and sometimes they would need to do sedated echoes, and sometimes she would just fall asleep on her own. It’s like she just kind of knows when we’re in Children’s that like, these are things that just have to be done, and she’s so great about it even now. And with her second surgery she was 3, we didn’t have any complications, we spent a day in the ICU, they extubated her rather quickly, she gets to the floor which is all great because the faster they can do that and the faster they can get her out of the ICU, the better she can get. But again, I knew the things she needed to do to be able to move and get home and get better, and I remember standing at the end of the hallway and her not wanting to walk and taking five steps away from her and making her walk to me, and she didn’t want to but it was only going to make her better. She needed it for her heart, her lungs, and to get her GI tract back on track because the medicines constipate, and that was really hard.

But again, when we came home from the hospital that time she was hula-hooping outside in two days, she does crazy things. And she always came home on this Tylenol or Motrin, and never needed more pain medicine. It was always enough for her, and she did great. On the second surgery they used what they described as the core matrix of a bovine valve, so they stripped away all of the DNA and just used the core structure in that for her valve. I guess they had shown that they were working really well, and that her own body would grow new cells over it. It wouldn’t grow with her, but it should function well. But it never really did because each echo just was either the same or a little bit worse.

It was 11 months later when she needed another surgery, and that was really hard because it wasn’t even a year later and I felt like for that whole year, her life was on hold. Every appointment it was just “Are they going to tell us today she needs surgery again?” So after 11 months she had her third surgery, and this time they did the Ross procedure. They took her own pulmonary valve and moved it to the aortic valves place because being her own native tissue, this would have a chance to grow with her, and then they used a cadaver valve in her pulmonary valve’s place. So they were able to put a larger size in, so as she grows she won’t outgrow it so quickly. So that was really good, and the positive part of that procedure is you don’t need any blood thinners, so she was on Aspirin therapy temporarily after, but she did not need to be on any other blood thinner, which is really good. Any time she came home on meds they always seemed to be able to taper any med she came home on very quickly.

 – Erica, mother of Isabelle, age 5, Anomalous Aortic Valve

 

The doctors needed to build a pulmonary system, and he built it using collateral blood vessels, and putting in the valve and the conduit and creating a whole pulmonary system. But it took multiple surgeries to do it. He’s had three open heart surgeries, right in the front of his chest, and then he’s had two through the back, through the left shoulder blade and twice on the opposite side of the back on the right trying to reach all of those collaterals and incorporate them into the pulmonary system. The first two years he had 25 hospitalizations between the surgeries and the catheterizations, it seemed like he’d be coming home from a surgery just long enough for him to recover, get strong, and then we’d have to go back and hand him over again. It was tough. That second surgery was-I mean every time it’s been difficult to hand him over, but that second surgery…it was brutal to hand him over, just knowing what complications had happened after the first one.

 – Grace, mother of Austin, age 10, TOF

 

Richard: There was a second surgery that happened when David was 16 and we didn’t know he was going to need that until he was like 12 or 13.

Courtney: We never knew he was going to need that.

Richard: David decided he wanted to have the surgery sooner rather than later so he could be more competitive in sports. So we knew that there was going to be a surgery at some point. We had thought it was going to be maybe when he was 18 or 20 or something like that. It kind of hit us like a punch that there was going to be another open heart surgery. Back on the heart/lung machine, and all of the trauma for us as a family, and obviously for David as an individual. But the kick part of the story is that David decided he wanted to have that surgery early because he understood that it would improve his heart’s performance dramatically when the purpose of the second surgery was to put a valve in, in fact a pig valve.

Courtney: A pulmonary valve.

Richard: So David decided he wanted to get this surgery sooner rather than later, so I said “David, that’s really not our decision, that’s up to your cardiologist and a conversation that you should have with him.” So we made an appointment with his doctor to go back in and have the conversation about sports and David’s doctor said, “You’re going to have this surgery when I say you’re going to have it. It’s got nothing to do…” of course he was very blunt with him, in his charming way.

Courtney: He said, “There’s no reason, you’re not symptomatic, you’re doing great, there’s no reason to be having the surgery.”

Richard: But they did some tests and he said, “let me look at this.” He ended up calling that night, and Courtney and I were out to dinner, and all of a sudden David calls and his doctor had called on the house phone, and David had answered, and he had said, “This is your doctor, I’ve got some news that you’re going to need this surgery.”

Courtney: “Your heart is enlarged.”

Richard: “And he’s in a dangerous position, you need to have the surgery immediately.” So that was one of the little bumps in the road. I had conversations with his doctor about this subsequently that we wish that he had waited and had the conversation with me or Courtney first.

Courtney: He did apologize to us afterwards, but he probably shouldn’t have given the information to David. So we started out with a little bump in the communication, but it smooths itself out. We got ready for a surgery a week later. In a way, that’s the best way to figure out you’re going to do it, because you don’t have time to be agonizing. He went in—they removed his scar, his original scar, which grows with you. They removed the scar and they saved it to do stem cell research. We had to sign something that allowed them to take the scar and use it for stem cell. Now at this point he’s in biology in school, he’s 16 going on 17 years old, he understands exactly what’s going on with his body, and is very aware of everything that’s about to be done. He was very brave, incredibly brave.  His friends were very supportive, they all signed a poster, “Get back soon David” and school couldn’t have been more supportive.

Richard: And we fortunately had the same surgeon who did David’s original surgery. He was still at it, in fact he had been doing lots of work to come up with new solutions for pulmonary valves. The surgery went extremely well, I think David was in the intensive care unit for maybe an hour and a half. Then he was in a room, they thought it was going to be for a week in the hospital, and I think he was home on the third day.

Courtney: So at this point his brothers are now an integral part of this because they’re young adults, they’re eight years older than David. So they were there when he woke up, and of course he had a pig valve put in so they’re signing his little pillow that they give you at Children’s, “Oink Oink” and making him laugh, telling him he’s never looked better because he’s all swollen after the surgery, looking very muscular. They made light of everything, but they certainly understood the severity of the situation. And he knew that if something happens to us, they’re there and he can lean on them. That was what I saw during that episode is that they’re there for each other. You couldn’t really ask for more, as far as David’s health goes. This is going to go on for a while, you know he’ll need another surgery probably in five years maybe. They’ll go through his groin this time, so he won’t have to have open heart. That’s to replace the valve. They don’t last that long. When they put it in this time, they set it up for that.

 – Courtney and Richard, parents of David, age 23, TOF