When we first learned that our son would be born with Hypoplastic Left Heart Syndrome, we were devastated. But once the shock had worn off we were desperate to talk to other families who had been through the experience. We asked our doctor about support groups, but he was less than impressed with what was available.
“They’re out there, but it’s mostly propaganda,” he said, scrolling through a Google search of HLHS communities. “Regardless of what you may read, these kids can’t run and they can’t do sports.”
And while he wasn’t impressed with what Google had to say about the future of kids with HLHS, he was impressed with what Boston Children’s Hospital’s Heart Center could do for children with the condition—and I’m so glad he was. Through his referral we went to Boston, and there we found the hope we needed. It was there that we learned that our son had a good chance of having an extraordinary quality of life under their care. We were also told that other than contact sports, our son could do anything he wanted to.
Over the years Lucas continued to thrive. And for each year he got stronger we participated in the NSTAR walk for Boston Children’s Hospital. It’s our way to say thank you to the hospital that saved him, and to help fund additional research, particularly in the areas of congenital heart defects that affect 1 in 100 babies born every year. Full story »
Kerri Dunn can always tell when her son Peter is determined to do something because when he gets deeply focused his face scrunches up and he squints one eye tightly shut, which his mother says makes him look like a cute, 2-year-old version of Popeye.
It’s a face Kerri sees a lot, because Peter is a very determined little boy. The youngest of six children, Peter is always trying to keep up with his older brothers and sisters—or “my kids” as he calls them. And, despite his smaller size, he still manages to do so—whether they’re walking, running or even climbing trees.
While his determination to keep pace with his older siblings may seem cute on the surface, it’s actually an incredible testament to Peter’s strong will. Not only is he managing to follow in their much larger footsteps, he’s doing so with only fifty percent of a working heart beating inside his chest. Full story »
When Jeffrey Cameron was born in 1996, he seemed to be a perfectly healthy baby. Then, at just ten days old, baby Jeffrey went into acute respiratory distress and was taken by air ambulance to a local children’s hospital where he was diagnosed with Hypoplastic Left Heart Syndrome (HLHS), meaning the left side of his heart was underdeveloped and couldn’t pump blood properly.
After a dizzying round of meetings with doctors, Jeffrey’s parents, Lenore and Tim, were presented with a set of difficult choices: Jeffrey could have a heart transplant, undergo a series of three open heart surgeries, or they could let him die at home.
“The doctors outlined our choices for us, and their exact words were, ‘You have three options and they’re all not very good,’” Lenore says. “We were told that transplant hearts for infants were extremely hard to come by and the that the 3-staged surgery was experimental. They said even if Jeffrey lived he might not have any real quality of life after all the surgeries.”
Based on the information presented to them by doctors, Lenore and her husband were under the impression that there was little hope for Jeffrey. After agonizing over the decision, the Cameron’s brought Jeffrey home with them so the family could enjoy what little time they had together in the comfort of their own house. It was a devastating time for them, but as the days wore on and Jeffrey continued to fight, friends and family asked the grieving couple to consider getting a second opinion. They began researching other treatment centers and learned that Children’s Hospital Boston had been successfully treating HLHS since the early 1980s, and patients with the condition were routinely sent there from all over the world. Full story »
Imagine for a moment that it’s 2001, and you’re Jennifer Miller. You’re pregnant with your second baby, and an ultrasound shows that his heart isn’t developing normally: In fact, the left side of his heart has a problem so significant it will stop developing normally from that point on. Your unborn baby has a severe blockage of the aortic valve, known as stenosis, which will lead to a rare and potentially fatal condition called hypoplastic left heart syndrome (HLHS) at birth.
Now imagine that you’re Jack Miller. As you float in the quiet darkness of the womb, your heart is the size of a grape and that troublesome valve that won’t deliver blood from the left ventricle of your body is only as big as the head of a ballpoint pen.
What happened next put the Millers on the front page of the New York Times and in TIME magazine. Full story »
You may remember Renee Peerless from the ABC documentary Boston Med, which aired last summer and featured several Children’s families and clinicians. After Renee’s unborn son, Sam, was prenatally diagnosed with a congenital heart defect known as hypoplastic left heart syndrome (HLHS), an ABC camera crew followed Renee constantly, highlighting the care required to help a baby born with a heart defect survive the delicate first few days of life. In the following post Renee relives the experience and talks about how the fetal diagnosis of Sam’s condition made an extremely trying situation easier to deal with.
Jake sees the pregnant Renee for the first time in months (photo courtesy of ABC.)
For Renee Peerless, the decision to have genetic testing done during a routine ultrasound had more to do with her husband Jake than her unborn child. Jake, a solider stationed in Iraq, was overseas for most of Renee’s pregnancy, so she initially saw the testing as a chance to have more images to share with him. “Jake felt like he was missing a lot so I wanted more pictures of Sam to send him,” she says. “I knew testing was a good precautionary measure, but my real motivation was getting a few extra ultrasound pictures.”
But as it turned out, the tests revealed that there was something seriously wrong with Sam’s heart. In a matter of minutes Renee went from giddy anticipation to the terror of learning that her unborn son’s health was in serious jeopardy. “It was like my whole world was falling apart,” she says.
The next week Renee met with a pediatric cardiologist in her home state of Connecticut and was told that Sam had a congenital heart defect called hypoplastic left heart syndrome (HLHS), where the left side of the heart is underdeveloped, limiting its ability to pump blood.
Sam had two open-heart surgeries before he was a week old.
“It was so much to take in all at once,” she says. “I didn’t even know what a congenital heart defect was and all of the sudden it was all I could think about.” Full story »
Happy Valentine’s Day. Appropriately enough, this heart-centric holiday falls right in the middle of National Heart Awareness Month, and we couldn’t think of a better way to celebrate than giving our readers a brief update about Parker Bolton, a Children’s Hospital Boston patient born with a congenital heart defect (CHD), called hypoplastic left heart syndrome (HLHS), and the face of Thrive’s Our Patients’ Stories section. (That’s Parker, smiling broadly over there on the left-hand side of this page.)
Since first visiting Children’s, Parker has grown significantly.
Parker first came to Children’s for open-heart surgery two and half years ago. At the time his health was questionable and his mother Casey knew very little about HLHS; it’s amazing how much can change in two and a half years.
Since his first surgery performed by Sitaram Emani, MD, of Children’s Cardiovascular Program, Parker has been growing consistently and his mom Casey has gone from knowing nothing about HLHS to becoming a heart advocate in her home state of New Hampshire. In fact, thanks to a letter writing campaign headed by Casey, New Hampshire governor John Lynch proclaimed February 14, Congenital Heat Defect Awareness day in his state, two years in a row. Full story »
Lindsay had open-heart surgery at Children's a week after birth
Yesterday we shared a patient story, written by a mother whose baby was born with a congenital heart defect (CHD) that wasn’t recognized until hours after birth. Thankfully, once her condition was discovered, baby Lindsay was rushed to Children’s Hospital Boston in time for open-heart surgery. It’s a story with a happy ending, but Lindsay’s first week of life was very touch and go, which caused her parents almost unbearable amounts of stress and worry.
But had Lindsay’s heart defect been recognized prior to birth, much of that anxiety might have been avoided. When a heart condition is diagnosed prenatally, caregivers can proactively provide additional testing, delivery planning and counseling that benefits the baby’s health and parents’ piece of mind.
In some cases, it’s even possible to perform fetal therapy on the child before he or she is even born. Children’s Fetal Cardiology Program can try to repair heart valves the size of a pen point in a fetal heart no bigger than a grape; all while the child is still in the mother’s womb.
Wayne Tworetzky, MD
Fetal or in-utero therapy is a very complex process that brings together cardiologists, obstetricians, anesthesiologists and nurses in a team effort to perform these procedures. In the following video, Wayne Tworetzky, MD, director of Children Fetal Cardiovascular Program discusses how difficult these procedures can be, not just because of the small size of fetal hearts, but also because of complications that can arise from performing invasive surgery on a fetus when the mother carrying the baby is perfectly healthy.
In-utero heart surgery is an amazing feat of modern medicine, but before doctors ever get to that point a problem needs to be recognized. Ultrasounds are essential in monitoring fetus development in the womb, but they’re not perfect.
“Virtually all pregnant women who receive pre-natal care will have a fetal ultrasound during the course of their pregnancy, but that doesn’t mean the ultrasound catches everything,” says Tworetzky. “Congenital heart defects such as hypoplastic left heart syndrome or transposition of the great arteries, and others, can be detected by ultrasound, but research shows that about half the time, they’re overlooked.”
When heart defects go undetected it can produce a host of health problems when the child is born, many of which can have long-lasting effects. But when heart conditions are caught early, caregivers can provide parents with counseling on treatment options and a planned delivery with appropriate pediatric and obstetric staff present.
“With planned and specially managed care before and after delivery of a child with a CHD, caregivers can treat the infant’s heart right from the start,” Tworetzky says. “They can also minimize the risks for future problems.
During a fetal ultrasound, Tworetzky suggests parents ask the technician and the doctor the following questions concerning the fetal heart:
Do you see four chambers in the baby’s heart? (Desired answer is yes)
Are there two upper chambers (left and right atria) with valves controlling blood flow into the heart? (Desired answer is yes)
Are there two lower chambers (left and right ventricles) with valves controlling blood flow out to the body (aortic) and lungs (pulmonary)? (Desired answer is yes)
Do the two valves and vessels (aorta and pulmonary arteries) exit the heart in a crossing fashion? (Desired answer is yes)
Are there any large holes between the lower chambers of the heart? (Desired answer is no)
Is the baby’s heart normal? (Desired answer is yes)
If the answers to the above are as desired, then almost all serious heart defects, and > 90% of all heart defects, can be ruled out.
If the ultrasound shows the possibility of a heart defect, parents should immediately make an appointment with a pediatric cardiologist, who will have access to the most advanced and accurate diagnostic tests. “If further testing show signs of a heart defect your caregivers will help you plan appropriately for your baby’s birth,” Tworetzky says. “This way you’ve given clinicians as much time as possible to make the birth, and early treatment, as smooth as possible.”
One treatment option—and it sounds like something out of science fiction—is repairing CHDs on hearts no bigger than a grape while the baby is still in the mother’s womb.
As you might imagine, it’s an incredibly complex and delicate procedure that brings together doctors, surgeons and nurses. In the video below, Tworetzky discusses how difficult these procedures can be, not just because of the small size of fetal hearts, but also because of complications that can arise from performing invasive surgery on a fetus when the mother carrying the baby is perfectly healthy.
And in this recent interview with NPR, Tworetzky talks about fetal cardiology and we hear from the parents of a Children’s patient who had surgery while she was still in her mother’s womb.