171 Ashley Ave.
Charleston, SC 29425
843-792-1414
800-424-MUSC
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April 2009
A Big-Hearted Story: The Search for a Small Heart
Angela Greenwood still keeps up a blog on her son's website (www.helpjojo.com) which she started when the five-year-old hovered on the brink of death awaiting a heart transplant.
"People from all over still keep up with him and want to know how he is," she says. "Recently another little boy asked what he got for Christmas."
Angela and her husband, Stephen, were happy to report that Joseph, now seven, got a "D-Rex" robotic dinosaur from Santa. They're happy to report any everyday occurrence: "Our family will never take another normal day for granted again," Angela writes in her blog.
Joseph's rare condition was one that seemed to fall out of a perfect sky - much like the one the seemingly healthy child was playing under on the beach when he first fainted -and which worsened just as quickly.
"I had just said that day, watching him having a ball in the water, that this must be the happiest day in his life," recalls Angela.
The collapse resulted in an emergency room visit and EKG that showed nothing. Another fainting episode two months later, and Florence cardiologist Dr. Charles Trant determined what he thought was a heart rhythm problem.
"We left that day shocked and devastated, with all kinds of heart medications," says Angela.
After another near collapse, Joseph was transferred from his local hospital in Florence to MUSC. There, pediatric cardiologist Dr. Phillip Saul and cardiac surgeon Dr. John Reed made the decision to implant a cardiac defibrillator in an effort to protect Joseph from a life-threatening abnormal heart rhythm.
Though the preschooler seemed to improve, a check-up found his condition had worsened. The family was referred to MUSC pediatric cardiologist Dr. Jeremy Ringewald for a transplant evaluation.
But before they could make it, Joseph collapsed on the family porch and Stephen had to administer rescue breaths to revive him.
Joseph was flown to MUSC, where his defibrillator fired more than a dozen times and the medical team had to put him on a breathing machine and intravenous medications.
Joseph suffered from dilated cardiomyopathy, which meant his heart had become enlarged and couldn't squeeze properly, explains Dr. Ringewald. "We suspect it was caused by a common virus that usually causes things like the flu, but occasionally attacks the heart muscles."
The abnormality of his heart caused abnormal rhythms; the defibrillator kept shocking him out of these bad rhythms. "Every time it shocked, his heart squeezing got worse and the blood flow slowed," says Dr. Ringewald. "We felt we were going to lose him unless we did something drastic."
The team put him on an Extracorporeal Membrane Oxygenation machine (ECMO), a heart-lung bypass device. "ECMO is good for a short time but carries risk if you need it for more than few days," says Dr. Ringewald. Within days, Joseph's heart was not improving and he began to suffer damage to his lungs and other parts of his body.
He was given top status on the transplant list.
His parents agonized. "We knew the average wait time for a small heart for a small boy was months, up to eight," says Angela. "Joseph was hanging by a thread on the ECMO. Every day that he made it was a miracle."
Dr. Ringewald talked to the parents about the Berlin Heart, a ventricular assist device that uses external pumps to pump blood out of the heart and into the body. Invented to be a bridge to a transplant, the Berlin Heart was "beyond experimental at this stage," explains Dr. Ringewald. An option in Europe since the early 1990s, the device had been used in the U.S. a few dozen times, but only with strict approval from the Food and Drug Administration.
"There are similar pumps used commonly in adults, but this is the only one produced in multiple small sizes that can be used in children under age 14," says Dr. Ringewald.
The parents gave their consent and within days so did the FDA. On April 4, 2007, after a 10-hour surgery performed by a team of 14, Joseph became the first child in the Carolinas to receive the Berlin Heart.
In less than a week, the boy was talking and walking, all the while attached to a huge console by tubes coming out of his heart and body.
"The Berlin Heart worked so well that he went from critically ill to being off breathing machines, off intravenous meds," explains Dr. Ringewald. "That made him a much better candidate for transplant, because he was able to eat, sit up and move."
A week after Joseph received the Berlin Heart, a new heart became available.
"You know you've been waiting and praying for it, but you also know it's a sad outcome for another family," says Angela, whose blog is full of messages of thanks and gratitude for Joseph's donor family.
Dr. Bradley and Dr. Ty Hsai performed the surgery, removing the Berlin Heart before implanting the donor heart. A few days later, Joseph celebrated his sixth birthday in the hospital with water guns and a game of Operation.
"When they rolled him out after his transplant surgery, he was awake and talking over the breathing tube," exclaims his mother. Two weeks later he was released from the hospital, and after nearly two months of therapy, he returned home to Florence.
Now seven and a second grader, Joseph enjoys biking, jumping on his trampoline, swimming and tae kwon do. He loves to go to the cath lab at MUSC and visit with the doctors and nurses, and sometimes out of the blue he'll share a memory of those days with his family.
"I remember making a chart of how many steps I would make every day to the bathroom," he remembers. He celebrates the two-year anniversary of his new heart this spring.
"I can't say enough about the team at MUSC," says Angela. "It was the many decisions made all along the way that saved Joseph. There is hardly a specialist in the cardiac department that he didn't need. They took great care of us and continue to be on top of things."
She knows that eventually Joseph will need another transplant, that this was not a cure but a second chance. "I pray and hope for research and advances," she says.
Although the process of approval for the Berlin Heart is still difficult, things are improving. Clinical trials with the device are in place at 15 centers around the country.
"MUSC has had a transplant center for more than 20 years, so we have the infrastructural support and are used to taking care of really sick kids," says Dr. Ringewald. "We make sure every technology is offered to every kid that comes in here, even an experimental device like the Berlin Heart."
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