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What is hypoplastic left heart syndrome? 

Often the best advocates for increasing public awareness for a particular disease are those directly affected by it. Tracey Bryan became one of th0se people in her fourth month of pregnancy, when she was told her unborn son had a life-threatening heart defect, hypoplastic left heart syndrome (HLHS). Bryan faced a decision: either terminate the pregnancy or deliver a baby who would need immediate surgery. She chose the latter, and because Children's Hospital Boston specializes in the surgery her son needed, she moved there temporarily.

  Today, after three operations and hundreds of hours of therapy, Bryan's son Jack is a happy, spunky 3-year-old, but his battle is far from over. Just 20 years ago, babies born with HLHS would not survive more than a month, therefore doctors can't predict how long Jack's surgical correction will last.

  As she's already demonstrated, Bryan takes adversity head on. She and her husband, Steve, have founded Jack of Hearts Foundation (www.jackofheartsfoundation.com), which they hope will bring attention to HLHS and raise money for research into the condition.

  In the following interview, Bryan discusses the syndrome and why fundraising is so critical — and difficult — for rare conditions such as HLHS.

Q: What is hypoplastic left heart syndrome?

A: It is the underdevelopment of the left side of the heart, specifically the left ventricle, which is the chamber of the heart responsible for pumping blood from the heart to the body. It means it's impossible for your heart to pump blood to your body, and therefore impossible for your heart to deliver oxygen to your body without surgical intervention to correct the problem. If a baby doesn't have the surgery, they will die within a relatively short time, within a couple of days.

Q: How prevalent is this condition in children?

A: It is relatively rare. The reported incidence is between one or two babies in 10,000 births. It's hard to get exact figures on it, because not enough children have survived this syndrome long enough for there to be a (large-scale) study. A recent study in Great Britain showed that they averaged 200-260 babies a year with HLHS. Unfortunately, there aren't good statistics kept on this syndrome.

Q: Do survival rates vary quite a bit depending on where the child is born?

A: If you have a prenatal diagnosis like I did, you're expecting to have a child that is cardiac compromised, so you handle them differently at birth than you do a healthy infant. Parents who have a child with this condition that do not know and deliver at any hospital anywhere, and they only find out when the child goes into cardiac arrest — those kids have a worse outcome because they're compromised already. The problem [only becomes known] when they crash, so they sustain all the damage anybody sustains if you have a heart attack and you're not resuscitated properly.

  This surgery is big and so specialized that it's one of the most difficult surgeries pediatric cardiovascular surgeons do, so not just any doctor can do it. First the kid has to be fortunate enough to be born in a place where the surgery can even be done. But if you know in advance and you can have the child delivered in a place where they do the surgery [often], that's even better.

Q: Jack has undergone three surgeries to correct the abnormality. Will he now lead a normal life?

A: Yes and no. He has a 30 percent chance of needing a heart transplant sometime in the future. And there's no one who can tell me, or any parent of a kid with this disorder, if he's going to be alive when he's 30 or 40. There are no 40 year olds (with HLHS) right now in the world. They don't exist, so nobody knows what happens to these kids for the long term. He's having a pretty normal life now. He goes to preschool, plays with his brothers and sisters, and he can do what most 3-year-olds can do. The overall survival rate to age 5 is 65 percent, and the overall survival rate to age 10 is 55 percent. There aren't any statistics for how many kids (with HLHS) are alive that are older than 10, but we do know that the oldest kids are just in their early 20s.

Q: You've dedicated a lot of time studying this syndrome with a great result — your son is alive and thriving — but that's not enough, is it?

A: No, he's basically alive because of children before him whose parents allowed them to have surgery. Each time the surgery is done, the method improves. Doctors learn things, improve their techniques and you can see an upward arc in the overall survival rates over the last 20 years. That's all because of research.

Q: HLHS is considered a rare condition, so it's difficult to obtain research dollars, isn't it?

A: Cardiac defects are the No. 1 birth defect in children. This cardiac defect is one of the rarer ones, but there is a paltry amount of money that is devoted to pediatric cardiology in general, which comes out to less than one cent of each research dollar. And we get way less than one percent (for research into HLHS). In practical terms, it means fewer kids survive. If you put more money into more research, the survival rate would continue to increase.

Q: What's the goal of your foundation?

A: Our goal is to fund one major research study a year, and a research study costs in the neighborhood of $100,000, so our goal is to raise $100,000 a year. Obviously, we'd like to do better than that, but I think a reasonable goal is to fund one study a year.

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