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Stanford Children’s uses innovation and bravery to provide a second heart transplant in a timely manner
Two hundred and ninety five. That’s how many times Josh Cole’s donor heart was passed over by other centers before Stanford Medicine Children’s Health accepted it for transplant. Today, it’s beating strong and giving Josh another shot at life.
Josh, a 24-year-old from Discovery Bay, California, has Danon disease—a very rare genetic condition that weakens muscles in the body, along with the heart. A number of his relatives have it, including his mother and his brother. It resulted in two heart transplants for Josh.
“We have hypertrophic cardiomyopathy, where the wall of our hearts thickens faster than normal,” says Josh.
Cardiomyopathy is a serious condition that may lead to heart failure. Josh was just 17 years old when he had his first heart transplant at Stanford Medicine Children’s Health because of the severity of his heart disorder. It was a traumatic time for him. Before transplant, he had to be put on a ventricular assist device (VAD)—a heart pump—to help manage his heart failure and give him time while he awaited a donor heart. After his heart transplant, he had a long recovery where he couldn’t get out of bed for months due to muscle loss from his Danon disease.
Josh’s troubles didn’t end; within a few years he developed a coronary condition that caused his blood vessels to narrow and his heart to become sick. All of this resulted in a less successful outcome for his first heart transplant and a need for a second one, from the renowned Pediatric Advanced Cardiac Therapies (PACT) program (heart failure/heart transplant) and the Pediatric Transplant Center at Stanford Medicine Children’s Health.
“My first transplant was really scary and hard. This time, I went in with a positive attitude, feeling better, and ready to do it,” Josh says. “I’ve kept a great mindset about everything, and it’s paying off.”
An unusual second heart transplant with unique use of cutting-edge technology
Josh received his second heart transplant this past winter from Stanford Children’s. A repeat heart transplant is not very common at the large, busy Betty Irene Moore Children’s Heart Center, which has performed 500+ heart transplants to date—a higher volume than found at most pediatric heart centers in the United States.
While waiting for his second donor heart, Josh was extremely sick. Time was of the essence. To get Josh to transplant more quickly, his doctors reconsidered a repeatedly declined heart and applied a novel use of a cutting-edge heart transport technology, referred to as Heart in a Box.
“Josh is a great example of us pushing boundaries and being innovative. While we’ve used Heart in a Box before, he’s the first one to benefit from our clinical innovation where we keep the heart beating during transplant surgery,” says Michael Ma, MD, thoracic and cardiovascular surgeon.
Heart in a Box enables the heart to beat during transport, using a pump that circulates oxygen and nutrients, rather than stopping it and transporting it on ice as is done traditionally. With cold preservation, a transplant must occur within four to six hours. With Heart in a Box, the window of time increases to 10 or more hours, allowing heart doctors to consider hearts from all across North America rather than just neighboring states.
Heart in a Box was FDA-approved for adults, not kids, so using it in teens and young adults is unique, yet safe. Lucile Packard Children’s Hospital Stanford is likely one of a handful of hospitals worldwide that do so. And because the heart doesn’t have to be awakened from a cold sleep, it often arrives healthier. Heart in a Box is a remarkable industry breakthrough, but Stanford Children’s doctors found a way to make it even better.
Normally, with Heart in a Box, the heart is stopped twice—once when it’s hooked up to the pump in the box prior to transport, and once again when it’s reconnected to the recipient’s arteries during transplant surgery. Heart surgeons at Stanford Children’s and Stanford Healthcare (the adult hospital) teamed up to explore ways to improve this process.
“We were brainstorming about how we could avoid stopping the heart twice, because any amount of time without oxygen or blood is not ideal for a heart. We discussed ways to keep the heart beating during the surgery. Ultimately, one of my longstanding mentors, Joseph Woo, MD, FACS, FACC, FAHA, pioneered the technique and has performed it more than a handful of times with adults,” Dr. Ma says.
When it came time to perform Josh’s heart transplant, Dr. Ma consulted with Dr. Woo and teamed up with the lead donor surgeon, who delivered the heart to perform their novel modification to give Josh the best outcome—a healthier heart. Using clamps, Dr. Ma rerouted blood flow within the heart without stopping it. The heart transplantation surgery was a great success.
“Generally, it takes a transplanted heart several hours to come off bypass (a heart-lung machine), but his heart came off right away,” Dr. Ma says. “We will probably keep performing Heart in a Box transplants this way. The heart recovers better and has better durability, and I imagine this may lead to improved outcomes in the long term.”
Josh’s recovery from his second heart transplant was like night and day compared with his first one. He was up and walking within three days, which helped him avoid muscle loss due to Danon disease. His heart doctors were extra-careful with his steroid use, which can directly affect his muscles.
“All heart transplants require steroids to avoid rejection early on, but with Josh, we knew we had to navigate not only what the heart wanted, but also what the muscles wanted. We gave him the smallest dose possible to protect his heart,” says Seth Hollander, MD, heart transplantation director with the PACT program at Betty Irene Moore Children’s Heart Center.
Josh is extremely grateful for his new heart and his skilled team of caregivers at the Stanford Children’s Heart Center. He appreciates how the team was responsive to any concern he had over the years, no matter how small, and responded immediately.
“Everybody is so nice—from the doctors, surgeons, nurses, and everyone else in the cardiac division. They all know me, and we have a strong bond. I’ve adopted them as family far from home,” Josh says.
Saying yes to a seemingly imperfect donor heart
Josh’s donor heart was turned down 295 times because the donor had damage to the chest and had COVID-19 at the time of death. Yet cardiologists at the Betty Irene Moore Children’s Heart Center took a second look to see if indeed the heart was damaged or just the chest bones and muscles, and to see if COVID-19 was still active at the time of transplant.
The American Heart Association points to studies that show hearts from donors with COVID-19 may not need to be passed over for transplantation. There have been no known cases in the United States where COVID-19 has been transmitted from a heart transplant, so rejecting hearts of people with COVID-19 may be overly conservative. After close consideration, Josh’s Stanford Children’s doctors deemed the heart just fine.
“We consider every offered heart a gift,” Dr. Hollander says. “In Josh’s case, we made good out of a tragedy.”
The PACT program at the Moore Children’s Heart Center has created a culture of approaching donor hearts with the intention of saying yes rather than no, even for those that do not seem ideal. Not all hearts can be accepted, but many can. As with Josh’s heart, the team’s philosophy is that every donor heart should be given real consideration.
“As a heart transplant community, we can’t accept every heart, but by striving to say yes and truly consider every heart that’s offered to us, we can accept a lot more. If we all embrace this philosophy, we could potentially double the amount of heart transplants we do in the United States and possibly cut the death rate of those waiting for transplant in half,” Dr. Hollander says.
Stanford Children’s has found ways to bring more donor hearts to children more quickly with innovative techniques. These include size-matching adult hearts to older children with advanced 3-D cardiac imaging in a unique donor heart matching program, accepting hearts from people with active hepatitis C and COVID-19 infections—for which there are effective treatments—and creatively using Heart in a Box.
Since 2017, Dr. Hollander and his colleagues have been reviewing every pediatric heart offer they’ve received and rejected. By scrutinizing their past choices and by implementing innovations, heart doctors at Stanford Children’s are improving their skills at determining which hearts to accept and limiting those they reject.
“While each innovation has made a small difference, in total they’ve made a big difference,” Dr. Hollander says. “Today, one of these methods is used in about one-third of our heart transplants at Stanford Children’s, helping more patients get hearts sooner and live until transplant.”
A rejected heart that’s proven itself to be just fine
When asked what he thought of receiving a heart that had been rejected by nearly 300 other heart centers, Josh was unruffled.
“I mean, I’m here, so it worked. If it wasn’t a good fit, my doctors wouldn’t have done it,” he says.
Today, Josh is feeling good and getting back to everyday life. He recently started a job with his dad’s company as a drone operator, and he’s a car enthusiast who enjoys tinkering with his pickup truck. His philosophy about his two heart transplants and life in general is to never give up, never give in.
“I’ve known Josh and his family for a long time. He’s a very jovial, likable guy. And he’s motivated to do what he needs to do to stay healthy,” Dr. Hollander says. “He’s taking really good care of his heart, and it’s working great.”
Learn more about PACT, our heart failure and heart transplant program>
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