Tetralogy of Fallot (TOF) with Pulmonary Atresia Pediatric Patient Stories

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From Iceland to Boston

I'm from Iceland.  I am the mother of a boy, Ásgeir Valur, who  was born with a heart defect called Tetralogy of Fallot. During the pregnancy we also found out he only had one kidney. But luckily, the kidney has never been a problem. It works fine, as it should.  We found about the heart defect when I was 7 months into the pregnancy and it was quite a coincidence that it was discovered then, because it wasn't seen in 20 weeks sonar, nor 25th week sonar (which I had because of the doctor wanted to be sure there was only one kidney there). But I‘m glad it was discovered during the pregnancy because then we had two months to prepare for what was coming. Most parents get the news after their baby is born.

He was taken with c-section (after his heart rate dropped during birth) the 11th of February and he was taken immediately to the newborn ICU. But I knew that would happen.  His father went with him but I saw him 2 hours later.  He was christened  the day after his birth. When he was two days old he got  a Blaloc-Taussig shunt in Landspitalinn (The hospital of Reykjavik, Iceland) and he spent his first three weeks there. The shunt was successful and we could take him home.  For the next 7 months he got home-nurse vising us three times a week and in July-August (2004) we had a very difficult 6 weeks as he refused to drink anything. He didn't want his milk and the feeding was really a struggle.  But we survived.  I‘m lucky he was my first child as I didn't have any comparison of how it was to have a healthy child.

In September his cardiologist decided it was time to prepare for the big operation and we had one month to make the passport and everything else ready.  The 29th of September me, my husband and son met the doctor on the airport and took of to Boston. We were lucky as my mother and my mother-in-law had decided to buy their own tickets to Boston to be with us for support. It was good to have their support when we was abroad.  We didn't have to have any financial concerns as the government paid the flight for me, my husband and son, the stay at the hotel and the hospital bill. I don‘t think a can complain about high taxes again!

The flight took about 6 hours, and my sons sO2 dropped to 62% during the flight. I was a bit worried but then I decided to look more on my child than the machine, because he was happy playing with the oxygen mask!  The trip from the airport to the hospital was the fastest one through airport I've ever had.  We had our passport checked inside the airplane, then a few guards took us the shortest way through the airport straight into an ambulance. We were at the hospital with our son in his hospital bed 45 minutes after the plane landed.  That was fast! We didn't have to wait for our luggage  because we had been told, only to take handbags with us.  I thought it was crazy to go to another country for 2 ½ weeks with only clothes in our handbags, and a lot of the luggage was our son‘s! Toys, clothes, bottles, diapers (for the flight) and all the stuff that baby needs! The most problem was finding the right milk formula he would drink because the one he was used to drink didn't exist in Boston. The painkillers were also different. He had Tylenol at the hospital for painkillers, but we were used to give him Parasupp. There was lot of other little things, like that. Nurses in Iceland tell us not to microwave the milk for babies, but it was the only option to heat the milk at the hospital. But those little things didn't bother us too much, we just learned new ways, and the staff at the hospital was very nice and helpful. We felt like our son was in the best hands.

We stayed at the hotel across the street so it only took us 2 minutes to get to the hospital. We switch places in spending the night at the hospital with our son. And the whole time he was sleeping by the Icelandic clock, which meant that he thought it was morning at 4:30 a.m.

The day after we arrived to Boston (the 30th of September) he had catheterization (it‘s very hard for me to remember this word!), which was performed by Dr. Armsby and it was very successful. This was on Thursday and we were lucky that he was allowed to spend the weekend at the hotel. He had a little bit fever, but nothing serious. His father took the most amazing photo of him at the hotel with the hospitalband around his leg.  We call this photo "Go Red Socks" because he looks like a supporter of a team and while we were in Boston Red Socks won the World Series Championship.

The Tuesday the 5th of October he had the big operation, which was performed by Del Nido.  The operation got delayed by few hours, but we kept in contact with our family and friends through his blog, which we tried to update currently.  However, everybody in Iceland had forgotten to tell his great grandmother that the operation got delayed and she was really really nervous for his behalf.  She though something must have gotten wrong, but that was not the case.  The operation was very successful, even more successful than anybody thought.  His cardiologist had expected that he would have had several surgeries while growing, and few catheterizations but in fact he has only had one catheterization since then and no other surgery. The original plan was to put some Gore-Tex patch inside to widen his pulmonary artery, but luckily the surgeon could use living tissue from my son‘s heart instead! 

The first time I saw him after the operation, I really didn't notice all the tubes and stuff, what I saw, was his beautiful pink skin.  He had never been so pink before.  Most other people would probably have noticed the  tubes, but I didn't. He was in the heart ICU until Friday, then he was moved into 6 East (which I believe, has moved to the 8th floor by now...  ).  We had still another culture shock on the ICU. In Iceland, you are not allowed to go into the ICU if you are wearing Fleese sweater, and no shoes or other outdoor clothes.  Children (up to 16 years old) where not allowed to visit the newborn ICU in Iceland. My husband brother who was 15 years old when our son was born, was not allowed to see him until he got home from the new born ICU.  Here, we could would in the ICU in our Fleese sweaters, in our outdoor shoes and everything!   We were so surprised by that. But after we got over the culture shock we found out that the staff at the ICU was really nice and they did really care. We felt like our son was in the best hands.

On 6 East we got a one person room, with our son‘s hospital bed, lazyboy and TV. That was luxury. I don‘t recall of having a one person room in a hospital before.  Usually, you have to share the room with 2-5 persons! But it was good to have this private room, I think we got it because our son was waking up in the middle of the night thinking it was morning (he was the whole time by the Icelandic clock). The first night we had been in a two person‘s room and our son and the other baby kept each other awake.

In 6 East, we kept getting this good service by the people, and when we noticed that he didn't want to sleep in his bed, we spoke to the nurses, and they agreed with us to leave him always alone in the bed, if they needed to take his blood, or do something (he didn't like), he was always taken into another room, which made the bed his safe place.  I really liked that.  He was also terrified of the X-rays so when he had had X-rays or several blood works done, we used to buy him an ice cream. It worked surprisingly well, because the ice cream seemed to make him forget the pain. (Luckily, they had ice cream in the cafeteria!). He is not afraid of doctors after this stay, in fact, he likes doctors very much.

We stayed on the Children‘s hospital until the 15th of October (which was Friday). We left the hospital in the afternoon and got to the airport. I think the flight was in the evening, but due to time difference, we landed in Iceland early Saturday morning.  The grandmothers had left two days earlier (which was our original departure date, but the departure was delayed by two days, because our son had a little fluid around his heart, but when we left we got Lasix with our which he had to take for a week or so), so when we came home, they were waiting at our home with breakfast ready for us. That was nice.

It is difficult to have ones child in a hospital and even more difficult to have the child in a hospital in another country, but the doctors, the nurses and all the other staff, made our stay as pleasant as possible.  I really want to return to Boston someday, and then go there as a tourist, and I hope I can take my son to visit Children‘s hospital, so I can show him the place, he had his life changing surgery. 

He is now 6 years old, he is very happy boy having a totally different life, than expected.  He practices swimming three times a week, he takes dancing lessons and plays piano.  Just the things you expect a normal child to do. His scar is almost invisible, so he doesn't have to worry about people staring at him when he goes swimming.

--- S. Andrea Ásgeirsdóttir

 


Heart2Heart: A legacy of mentoring

Alexa Horwitz and Eliot Greene have a lot in common. They’re both high school honor students. Eliot is his class president at Belmont Hill School, while Alexa is editor-in-chief of the school newspaper at Buckingham Browne & Nichols. Both are varsity athletes, even participating in similar sports—tennis for Alexa and squash for Eliot. They’re friendly, engaging and quick with a smile. And they’ve both undergone multiple open-heart surgeries at Children’s Hospital Boston, making them perfect candidates to pilot the Heart2Heart at Children’s Teen Mentoring Program.

The program—currently available in Cardiology—matches high school or college students who were born with heart defects with patients currently being treated at Children’s. It’s the first program of its kind involving face-to-face mentoring with both patients and parents. “The questions they have are the same questions we had,” says Alexa. “It’s easier to talk to someone who knows firsthand what you’re going through.”

That was the case 17 years ago when Alexa’s mother, Kasey Kaufman, learned that Alexa—still in utero—had an atrial septal defect (ASD), a congenital heart condition that occurs when there’s a hole in the wall between the upper chambers of the heart. “On the spectrum of heart defects, ASD is pretty fixable,” says Kaufman. “At the time though, it seemed so overwhelming.” Jane Newburger, MD, MPH, Children’s associate cardiologist-in-chief—and Alexa’s doctor—suggested that Kaufman reach out to Leslee Greene, whose son Eliot, had recently gone through a similar operation.

Eliot was born with Tetralogy of Fallot, a condition involving four different congenital heart defects, which were repaired by Children’s John Mayer, Jr, MD—the same surgeon who would replace Eliot’s pulmonary valve 16 years later. “I called Leslee, and I just sobbed to her over the phone,” says Kaufman. “She was so incredibly kind. Just talking to her gave me back my hope and strength.”

Alexa’s ASD was repaired at 15 months, but when she was in fifth grade, a second surgery was needed to repair a moderate leak in her mitral valve. Children’s Gerald Marx, MD, was able to pinpoint the exact location of the leak using echocardiography, allowing Cardiologist-in-Chief Pedro del Nido, MD, to repair it without replacing the valve. While Alexa was in the hospital, family friend Cheryl Toole, MSN, RN, nurse manager in the Children’s NICU, visited Kaufman regularly. Toole’s daughter, Avery, had recently been born with critical congenital heart disease. A few years later, the roles were reversed with Kaufman and Alexa visiting with the Tooles' while Avery was awaiting a transplant. After one of these visits, Alexa commented to her mother about how good it felt to spend time with Avery, because she remembered what it had been like waiting for her own surgery. She went home, sat down at her computer and typed up the proposal for Heart2Heart at Children’s.

Alexa sent the proposal to Newburger, and to Sara Conahan, director of Development for the Children’s Hospital Trust, who had enlisted Alexa to speak at the Women in Medicine Luncheon a few years earlier. After a few trips back to the drawing board, a formal steering committee was formed in October 2010, consisting of Newburger, Conahan, Sue Reidy, MS, RN, from Nursing, Beth Donegan-Driscoll, MS, CCLS, director of Child Life Services, and Christine Rich, RN, MS, a nurse with the Center for Families.

With the program close to becoming a reality, Alexa knew it was more than she could handle on her own. She and Eliot had been crossing paths their entire lives, completely unaware of the support that Eliot’s mother had offered to Alexa’s mom 17 years earlier. They went to the same schools and even lived within blocks of each other until Alexa was 5, but since Eliot was a year older, they didn’t really know much about each other outside of their shared history of heart surgery. “One night, I got a Facebook message from Eliot, saying ‘Hey, we should volunteer at the hospital,’ ” says Alexa. “I sent a message back, saying, ‘funny you should mention it…’ ”

With Rich on board as coordinator, Heart2Heart at Children’s entered its pilot stage with Alexa and Eliot as its first two teen mentors. Before they could meet directly with patients, both teens—and both moms—went through weeks of training on hospital policies and procedures, as well as a period shadowing Child Life to see how they interact with patients.

The first time Alexa or Eliot meet with a patient, a representative from Child Life sticks around to ease the transition. After that, if the patient’s family approves, they’re on their own, providing a confidant who knows exactly what the patients are going through, and answering questions kids may not feel comfortable asking their parents or clinicians. “A lot of kids want to know if it hurts when they take tubes out of your throat after surgery,” says Eliot. “When I say ‘I really don’t remember, so it can’t be that bad,’ they know I’m speaking from experience.” One patient was so appreciative of the time Eliot spent with him that he named his stuffed wolf after him. Another told his parents, “I want to do what Eliot does when I grow up.”

Alexa and Eliot have mentored patients as young as four years old. “The experience can actually be less intense with younger patients,” says Alexa. “They’re less likely to ask really tough questions than someone who’s older.” Some patients simply want to spend time with someone close to their own age, like the 14-year-old who told Alexa that the rainy afternoon they spent watching Avatar was the best part of his hospital stay.

With Heart2Heart at Children’s proving to be a success in Cardiology, Alexa and Eliot have already drafted a proposal to expand the program. Just as their mothers passed a legacy of mentoring on to them, Alexa and Eliot are working with the steering committee on plans to begin recruitment and training of additional teen mentors this summer. “This program’s all about creating connections,” says Alexa. “I’d love to see it expand formally to other departments. Even more, I’d love to see it expand to other hospitals.” 

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

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