Aortic valve stenosis pediatric patient stories

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Ann Louise's story

The first thing you notice about Ann Louise Atherton (her real name is Hazel, but everyone calls her Ann Louise) is her eyes: playful, brilliant blue and sparkling with humor. When she throws a toy and you pick it up, she beams, knowing she’s hooked you into playing fetch. Sitting in her Princess stroller and playing with her mother’s iPhone, a small catheter tube by her hip is the only sign of the risky, life-saving heart surgery that Ann Louise went through before she was even born.

Early in her pregnancy, Mary Beth Atherton and her husband, Andrew, learned that Ann Louise’s still-developing heart was showing signs of critical aortic stenosis, a congenital defect that prevents the aortic valve from opening completely. If left unrepaired, aortic stenosis causes the left ventricle to work harder to get blood to the body. Over time, the left ventricle becomes thicker and pumps blood at a higher rate, damaging the heart while failing to pump blood efficiently. At home in North Carolina, Mary Beth’s doctors warned her that repairing her baby’s heart after birth would involve several risky surgeries, and would likely result in serious mental delays—if Ann Louise survived at all. Despite the bleak prognosis, they also advised Mary Beth and Andrew against in utero surgery, warning of low survival rates and poor quality of life for the baby. They recommended preparing for the worst.

“We found Boston Children's Hospital on our own,” Mary Beth says. Andrew called Gerald Marx, MD, senior associate in Cardiology, directly and spoke to him for two hours about Ann Louise’s chances if they came to Children’s for in utero heart surgery. Mary Beth’s doctors advised strongly against the trip, but Marx made a convincing case. “After talking to Dr. Marx, we didn’t even need to think about it,” says Mary Beth. “He gave us his pager and said to call him directly when we made up our minds. We ended up paging him on Thanksgiving, but he called back immediately and said ‘come to Boston…now!’” Two days and one Med Flight later, Boston Children’s surgeons operated on Ann Louise in utero, repairing her aorta.

Ann Louise was due on March 24, 2008, but she arrived a month early and spent the first five weeks of her life in the Cardiac Intensive Care Unit. It was there that she first met her brothers, Graham and Reed, who traveled to Boston Children’s to meet their new baby sister. “They had to keep her sedated because she was still healing from surgery,” says Mary Beth. “But when Graham held her hand, she squeezed back.”

Since her first surgery, Ann Louise has returned to Boston Children’s 12 times for additional surgeries and check-ups. She and Mary Beth have spent more than 200 days in the hospital, and Ann Louise now looks forward to seeing the Cardiology team. When given a picture of Marx, she laughs and blows kisses at him. “He’s been so good to us,” she says. “Ann Louise thinks he’s part of our family.” Mary Beth also credits the Boston Children’s Center for Families with “helping keep us sane” through the long visits.

Last month, Boston Children’s surgeons replaced Ann Louise’s mitral valve; her last major surgery, for now. The 18-month-old will need her mitral valve replaced again when she’s 10, and her pulmonary valve will need to be replaced when she’s in her early teens. “The doctors we saw before coming to Boston Children’s said Ann Louise wouldn’t live past 25 if we came to Boston for in utero surgery,” Mary Beth says. “The doctors at Boston Children’s told us there’s no realistic basis for that statistic. This surgery’s only been done for 20 years! For all anyone knows, Ann Louise could live a normal, long life.”

For now, Mary Beth and Andrew are looking forward to bringing home a happy, healthy little girl who loves chewing on her toes, watching the Imagination Movers and flirting with doctors. “When we contacted Boston Children’s, there was such a difference in how they approached things,” says Mary Beth. “We’d been told that Ann Louise had a 25 percent chance of dying. Dr. Marx said there was a 90 percent chance that she’d live. That’s what gives you hope.”

- Mary Beth Atherton

Genevieve's story

“We see something with your daughter’s heart — it is probably nothing, but we would like to refer you to Boston Children's Hospital for an echocardiogram.”

I can still hear those words. After struggling with infertility, we were blessed with our twins and now there was potentially something wrong with them. What does this mean? Did we do something wrong? Did we cause this?  It is probably nothing, right?

We called the Advanced Fetal Care Center to schedule an echocardiogram. The appointment seemed to go on forever — we watched the colors dancing on the screen that represented the blood flow. The ultrasound technician scanned both hearts — that of my son and my daughter — just in case. We found ourselves studying the face of the technician, hoping to see a glimpse of whether things were good or bad. My husband and I were both waiting for them to say that it was nothing.

We then went to another room to wait for the doctor. Dr. Ronald Lacro came in to talk to us. He brought with him a book from the American Heart Association called If Your Child has a Congenital Heart Defect. He showed us pictures and went over our daughter’s diagnosis — aortic stenosis. 

Dr. Lacro took the time to explain the diagnosis and did his best to answer our questions but a lot of it would remain unknown until after she was born. My husband liked his direct, honest approach, while I was in tears, and nothing that he could have said with the exception of “everything looks perfect” would have been a comfort to me. Dr. Lacro left us with the book, his card and an invitation to call or e-mail with anything that we wanted to discuss further. He knew that we were overwhelmed and that a lot of the questions would not come to mind until we had left the hospital.

Hiss recommendation was to schedule a follow-up echocardiogram so they could monitor her as she got bigger. Wait and see. I was a wreck — what did this mean? And what does it mean for her twin brother?

For the rest of the pregnancy, we had regular appointments at the Advanced Fetal Care Center. We also had regular appointments with our OB at Brigham & Women’s Hospital. She seemed genuinely surprised that the spot they had seen at our anatomy scan had turned out to be “something” and worked hard to be reassuring and supportive. It was stressful, but uneventful. No major change with her heart, no complications with the pregnancy.

In the last month of the pregnancy, it was time to start talking about what all of this would mean. Thankfully, we did not require intervention during the pregnancy, but it looked like she would require surgery shortly after birth — a catheterization procedure to widen the narrowed valve in her heart.

We scheduled appointments to tour the Neonatal Intensive Care Unit (NICU) at Boston Children’s as well as the NICU at Brigham & Women’s. It was heartbreaking and inspiring at the same time. The most beautiful tiny children hooked up to machines and monitors while the most dedicated and positive doctors and nurses do all they can to support life. It was amazing and gave us a bit of hope for what was to come.

We scheduled our C-section for August 26, 2008. There was comfort in having a scheduled date. Our families could be there to support us. The cardiac team at Boston Children’s was ready and knew that our twins were going to be delivered that day. During the entire pregnancy, our son was presenting as Twin A and our daughter was Twin B. Even during a quick ultrasound right before we went into surgery, our son was Twin A. At 8:11 a.m., Genevieve Anne Norton came into the world. Determined to be born first, she slipped right past her brother — a sign of things to come with our little girl. At 8:12 a.m, Harry Francis Norton made his appearance — now the second born and little brother.

The first few days were a blur. I saw Genevieve for a moment and then she was gone, transferred to the Cardiac Intensive Care Unit at Boston Children’s. My son Harry and I were recovering at Brigham & Women’s. My poor husband was running back and forth trying to be there for everyone. He was amazing — so strong during all of this. While time seemed to slow for me, he felt like he was in constant motion. At my bedside, holding his son and watching the Red Sox game, then back on his feet running over to Boston Children’s to check on Genevieve and meet with her doctors. And then back again.

At just a day old, Genevieve underwent her first catheterization procedure. They had been able to open the valve more and she was recovering. Every day, I am so thankful to everyone at both Boston Children’s Hospital and Brigham & Women’s Hospital. Dr. Lacro would call me to let me know how Genevieve was doing, the staff at Boston Children’s made sure that my mom, my father-in-law and anyone else who sat at my daughter’s bedside was comfortable — and the team at Brigham & Women’s always happily took Harry to the nursery and ordered a wheelchair so that my husband could take me through the tunnel that connects the two hospitals to see Genevieve.

I was discharged with Harry from the hospital on Saturday and Genevieve joined us at home the following Thursday. That first year was tough — a couple of hospitalizations for bronchiolitis — but we got through it.

Genevieve continues to be monitored by Dr. Lacro and the team at Boston Children’s. She has moderate regurgitation from the valve and will require surgery to repair it. At 15 months old, she had a second catheterization procedure to check the pressure going across the valve. Her heart function continues to look good, so we are putting off the surgery a bit longer. I know that it is best to wait, to give her the chance to be bigger and stronger when going into surgery; but it is still hard to have this hanging over our heads. 

Every day, I am thankful to be so close to Boston Children’s; to have our concern be how bad traffic will be in the morning when we have her next follow-up appointment! We have met families that come from all over the world — Genevieve’s first roommate when she got out of intensive care was a girl who had flown in from Iceland — and it reminds us that we are truly blessed to have such excellent care right in our own backyard.

Genevieve is now 20 months old. There is still the cloud of impending surgery, but you would never know it by looking at her. Genevieve and Harry are inseparable; they are the best of friends, hitting all their milestones together and having a great time getting into all kinds of trouble. We have been blessed.

-- Tina Norton

Stacy's story

My daughter, Stacy, had a brain tumor and was treated by Boston Children's Hospital from 1975–1978. Sadly, she passed away on November 1, 1978, a month before her seventh birthday. She and our whole family received wonderful care at Boston Children’s, and the numerous doctors who treated Stacy went above and beyond the call of duty while attempting to save her life.

My other daughter, Michelle, who is now 35 years old, was born with a hole in her heart and aortic stenosis (narrowing of aortic valve). Again, we were treated with utmost care. Michelle chose to stay at Boston Children's and is currently followed there even into her adult years because of the comfort zone it provided for her.

We had a scare with our “baby” (now 18), who was born nine weeks early and suffered bleeding in the brain. She was brought into Children's and put through various tests, after which they decided it had only been mild bleeding and reassured us that it had resolved. (Ironically, she was seen by the same neurologist and neurosurgeon that our first daughter had 20 years prior).

She had gross motor delays, so was followed by the Early Intervention team for three years. Even though she was delayed with all her motor skills, she went on to become a championship Irish step dancer! She's now in her first year of college at the University of New Hampshire and is doing great.

So, that's our family's history with Boston Children's Hospital. I don't hesitate to recommend it to anyone who's looking for medical help for their child.

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

Boston Children's Hospital 300 Longwood Avenue, Boston, MA 02115 617-355-6000 | 800-355-7944

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