Every Heart Has a Story

Meet Ben and LJ who were diagnosed with a heart defect before they were born — Ben with hypoplastic left heart syndrome and LJ with transposition of the great arteries — and Reid who was diagnosed with hypertrophic cardiomyopathy shortly after birth. Hear their stories and look at them now.


Hypoplastic Left Heart Syndrome: Ben's Story

Ellen: We went for the routine ultrasound and they checked everything. They said, “We see a heart defect.” They said his condition was hypoplastic left heart syndrome. And it was really a very bleak picture that they painted for us. A part of me gave up hope that I would have a baby to bring home.

Stephanie Fuller, MD: Hi, how are you?

Ellen: Good.

Josue: Good.

Stephanie Fuller, MD: How are you?

Ben: Doing good.

Stephanie Fuller, MD: Good?

Benjamin’s diagnosis is hypoplastic left heart syndrome. It’s essentially a condition where the heart, that normally has two pumping chambers, one to send blood to the lungs, and one to send blood to the rest of the body, only has one chamber which is the right side of the heart. Our main goal is to convert that pumping chamber so it really serves to pump blood to the entire body. So it’s essentially a combination of three surgeries. It’s lifesaving for these babies who otherwise would not survive.

Ellen: I could tell he was a fighter from the very beginning because while I was pregnant with him, he was so active. And I told my husband, I said, “I know that he wants to live. I know he wants a chance.”

Stephanie Fuller, MD: So we’re talking about a very complex surgery. You have one chance to do it right. And after that, hopefully you have two more operations to get this child to a healthy and productive childhood.

Ellen: He was born right on time, full-term. He was taken right away and they put the IVs in and everything. And I was wondering, you know, Am I going to have a chance to hold my baby? Am I going to have a chance to get to know him?

He had his first surgery here with Dr. Fuller at 3 days of age. And his second surgery was at 6 months, and his third surgery he was 2 ½.

Josue: I do remember the first time that, the first surgery. You know, we took a picture, the four of us, the family, because it wasn’t said, you know, and the thought was that we know we have this moment right now, we don’t know after this.

Ellen: And now he’s 6, and he’s awesome.

The fact that Benjamin had the same surgeon every time… and I’ll never forget how special I felt when Dr. Fuller remembered my daughter’s name. To me that made me feel like this is a surgeon who cares specifically for our family. We’re not just another patient.

Stephanie Fuller, MD: We bond very quickly with these families because we spend a lot of time with you. There wasn’t a day when Benjamin was here that I didn't see him.

Josue: A lot of the things that happened here in the hospital, it made me feel better, it gave me more hope. They just made us feel that we were just as important as just any other part of the team, you know. And I just, I didn't… you know, I received encouragement from that through a very hard time.

Stephanie Fuller, MD: I’d like to think that even though I don’t have my own children, I have hundreds of them. And Benjamin is one of them. It’s amazing to see him as just a normal kid. You would never guess he has heart disease.

Ellen: People will tell us, “You know, if we didn't know that he had a heart condition, we would never believe it.” He’s very active, very normal. And you know, he, he’s very bright, has a great sense of humor, is just an amazing kid.

How would you describe Ben?

Daughter: Funny.

Ben: I would describe myself… fantastic.

Ellen: Every heart has a story, and this is my son’s story.

Transposition of the Great Arteries: LJ's Story

Kristin: The first time we heard that LJ had congenital heart defects, we were absolutely devastated. And the ride home, I don’t think Joe and I spoke one word to each other, we just wept.

Joe: We found out the news about our baby, it was the most difficult time for us.

Hello Dr. Rychik

Jack Rychik, MD: Hey Joe, hey Kristin, how are you?

Kristin: How are you Dr. Rychik? Great to see you.

Jack Rychik, MD: Hey LJ. How are you doing?

The first time I met the Spitales, Christine was, I think, about 21 weeks pregnant. After a fetal echocardiogram, we were able to identify that in fact LJ had something called transposition of the great arteries. Normally the right side of the heart, which receives the low-oxygenated blood, gives rise to a vessel called the pulmonary artery, that then delivers blood to the lung. Blood picks up oxygen from the lung, returns to the left side of the heart and then is ejected into a vessel called the aorta that delivers that oxygenated blood to the body.

In transposition of the great arteries, those two vessels are switched. Such babies can get very, very sick very quickly.

Kristin: As you can imagine, it was extremely difficult for us to digest this news.

Jack Rychik, MD: With the arterial switch operation, we can now essentially turn the heart back into something that’s almost identical to a normal heart.

Kristin: When we visited Children’s Hospital and we first met Dr. Rychik, it was the first bit of comforting news that we had received. Learning that we were candidates for the SDU, we relocated to the area.

Jack Rychik, MD: The Special Delivery Unit is a critical aspect of the overall management for our patients who have important congenital heart disease and need to have management as soon as they’re born.

Kristin: I had gone into labor sooner than expected, and we delivered at the SDU. They stabilized him with the breathing apparatus, and once I was in recovery, they let me know that he would in fact need a balloon procedure.

They scheduled his surgery for two days after he was born. That was a tough day, knowing that not only couldn’t we see him for five hours, but just the idea of such a small, little being being opened up like that was very difficult.

Joe: I’ve been in a lot of difficult situations with my job as a detective, but this was the most difficult time ever.

Kristin: It’s one of the most memorable days because after nine months of carrying a baby that I knew would have some challenges, it was so emotional.

The surgeon came into the room and told us that the surgery went very well.

Joe: Dr. Mascio, when he told me that it was a perfect surgery, it was just so much relief.

Kristin: We follow up with our local cardiologist. She’s extremely pleased with everything that she’s seen and she does not see any issues for the future.

Jack Rychik, MD: Typically we see kids once a year, young adults once a year perhaps, or something like that, for general checkups. But when you’re not at your doctor's office, these kids are out doing normal stuff, leading completely normal lives.

Joe: This is Louis James. He was born at The Children's Hospital of Philadelphia on August 17, 2014.

Kristin: And he’s very, very happy.

Jack Rychik, MD: And currently hungry.

Kristin: Very hungry.

Jack Rychik, MD: Every heart has a story, and this is LJ’s story.

Hypertrophic Cardiomyopathy: Reid's Story

Maria: After Reid was born they came in and said that, “Sometimes C-section babies had problems breathing.” And they came back to me and said that they thought that Reid was in heart failure. It was living like the nightmare of just what’s going to happen? Is he going to make it? And it’s truly terrifying to think, you know, life is so precious and I just never thought that we’d have this worry.

He spent about 10 days in the NICU and that’s when we found out that he had hypertrophic cardiomyopathy.

Good morning.

William Bonney, MD: How are you?

John: Good to see you.

William Bonney, MD: You too.

Maria: Hello, how are you?

William Bonney, MD: Hypertrophic cardiomyopathy, Reid’s heart condition, is a disease where the muscle of the heart can become so enlarged that it starts to obstruct blood flow out of the heart. And the way that the electrical signals travel through the heart can become disordered and that can be a set-up for arrhythmias.

Maria: It kind of changes the way you think of everything. And, you know, just changed our lives forever.

We would have to give him his medication, then it was the tiny little dropper, and it was such a stressful time. You know, if he spits up then you can’t re-dose, and you know, if he does it again then you have to go back to the hospital.

John: I’ve flown combat missions, I’ve been in places I never wanted to be, but giving Reid his medicine was the most stressful thing I’ve ever done.

Maria: As he was getting older and it was more adolescence and things started to change. That’s when there were some issues. The cardiologist that we were seeing at the time started talking about a defibrillator.

William Bonney, MD: In Reid’s case it was that his heart was just becoming abnormally thick. And had reached sort of a critical threshold. A defibrillator is similar to a pacemaker in that it’s a device that’s implanted in the chest and it consists of two components. The first is the generator itself, and then the other component is what’s called the lead. And that’s the wire that extends from the device, and attaches to the inside of the heart. And the device serves two functions: it can pace the heart if it gets too slow, and it also monitors the heartbeat for rhythms that are dangerously fast.

Maria: He wasn’t really allowed to do soccer or anything until he got the defibrillator. It gets more difficult as you become a teenager, and you want to be like everyone else and do what everyone else is doing, to face those restrictions.

Reid: I’m a goalie. There’s like a lot of restrictions with running and things like that. So when my team will condition, I’ll have to like sit out. And it really doesn’t bother me, but, I mean, I wish I could do it, but being a goalie is satisfying.

Maria: Reid rides a bike, goes to the gym, likes to exercise as much as he can.

William Bonney, MD: In the event that there’s a life-threatening cardiac arrhythmia, the defibrillator will deliver a shock similar to what you would get externally with paddles.

Colin: The night his pacemaker went off we were just laughing, and it just went off. And we didn't know what it was at first. We thought it was like a cellphone. And we kind of just looked at each other and he looked at his chest, and everything got like serious. It was scary.

Reid: After I came, like, back to my senses and like realized what was happening, I was pretty scared.

William Bonney, MD: Reid had a fracture of his defibrillator lead. The lead needed to be removed and a new lead needed to be implanted. The thing that happens with young people is that the leads that live inside their veins can develop a scar, just like you have scar on the outside of your body, you have scar around that lead. And so pulling it out isn’t just as simple as pulling a key out of a door. It has to be lasered down and removed in a very controlled setting. So again it is something that is not without risk.

John: It was a very difficult procedure. I believe it was eight or nine hours,

William Bonney, MD: Yes.

John: somewhere thereabouts.

Maria: At one point I started to cry, and he came out and reassured, you know, “Look we have the best people in there, and this is what we’re doing.”

William Bonney, MD: Fortunately Reid’s procedure went well and we were able to implant a new lead and a new device.

Maria: Reid is very special. We’re looking at this as a marathon, this is not a sprint. This is the long term.

John: Every night when Reid goes to bed, even now, I kiss him three times and say, “I love you Reid.” And I’ve done that since the day he was born, every night.

Reid: I look at life a lot more precious I guess. I don’t know, I live, I do more exciting things. I don’t really have any worries because of that.

Every heart has a story, and this is my story.

Related Centers and Programs: Cardiac Center