Baby who received first mechanical heart pump at Dell Children's home after heart transplant
Zaria Grace Jackson loves to blow bubbles and grab people's face masks and pull them down so she can see their smiles.
"She wants to be in the middle of everything," says Olivia Guthrie, the mother of the 6-month-old Austin baby.
On Monday with cheers from the staff and smiles from her family, Zaria left Dell Children's Medical Center of Central Texas after more than three months.
She is part of the continuing story of the growth of the heart transplant program for the Texas Center for Pediatric and Congenital Heart Disease at Dell Children’s and UT Health Austin, the clinical practice of Dell Medical School at the University of Texas.
On Jan. 11, Zaria became the first child to receive a Berlin Heart pump at Dell Children's, and on March 25, she became the third person to receive a heart transplant at that hospital. A fourth heart transplant has since been done.
The Berlin Heart kept her alive for 73 days, until she made it to the top of the heart transplant list.
Never a permanent solution
When Zaria was born last fall, she looked perfectly healthy, but on Jan. 4, the then 3-month-old stopped eating and going to the bathroom. Her health declined that week and she began panting.
When Guthrie took her to the doctor on Jan. 8, her pediatrician sent them immediately to the emergency room at Dell Children's thinking Zaria had pneumonia.
Doctors found that her heart was enlarged and failing, and they didn't know why. Zaria continued to get worse that weekend. Doctors at Dell Children's had a choice: They could put her on a extracorporeal membrane oxygenation machine (ECMO), but patients can stay on that device for only a few days, weeks, maybe a month and they risk other organs failing. Or they could do the center's first Berlin Heart pump and create a bridge to transplant.
The Berlin Heart Excor ventricular assist device sits outside the body and is connected to the heart through two cannulas (tubes) that are sewn into the heart. One cannula sends blood from the body to the pump. Another cannula takes blood from the pump and puts it into the body.
The actual pump has a silicone bladder inside it. One side of the bladder is filled with air and the other side is filled with blood. In one phase, the bladder relaxes; in another phase, it fills with air. This creates the pulsating of blood in and out of the pump, moving it from the heart to the pump, through the pump and back to the heart. The pump is connected to a driver that pumps air in and out of the bottom of the pump.
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Kids with the Berlin Heart are attached to a machine, and because of the care needed to keep it clot free and the patient healthy, it is not FDA approved for a patient with a Berlin Heart to leave the hospital.
As patients grow, the pumps need to be changed out for larger ones. In Zaria's case, she was growing and doctors were looking at going to the next size pump once she had had it for three months.
The average Berlin Heart is in a patient for 188 days, but there are kids who have had them for more than a year. Guthrie says she was told to expect it to take five or six months.
"Some patients can really wait a long time," says Dr. Charles Fraser Jr., the head of the Texas Center for Pediatric and Congenital Heart Disease, but in this case, it happened quickly, he says.
Growing a transplant program
Doing a Berlin Heart procedure was "great progress for our program," Fraser says.
The program began in 2018, when Fraser arrived at Dell Children's to start the center after having led the program at Texas Children's Hospital in Houston. He began building the transplant program team and working with Dell Children's to remodel parts of the hospital.
A year later, Dell Children's opened a cardiac intensive care unit. It also upgraded two operating rooms to get ready for heart transplants.
On Sept. 24, 2019, Dell Children's was able to do its first left ventricular assist device, a mechanical device that sits inside the body and helps the heart pump.
Dell Children's now has seven people listed for a heart transplant and expects another two to be listed this week.
"We're really progressing rapidly here," said Dr. Ziyad Binsalamah, the surgical director of heart transplantation and mechanical circulatory support at Dell Children's. For the number of transplanted patients, people on the waiting list, and clinic visits, "we've already exceeded our expectations for the first year," he says. By the end of June, he predicts the program might reach benchmarks they expected to hit in the fourth year.
The Berlin Heart procedure is more complicated than a heart transplant. "There are a lot of places out there who can do a transplant, who can't do a Berlin," said Dr. Chesney Castleberry, the medical director of the pediatric heart transplant team, after Zaria got her Berlin Heart. Being able to do ventricular assist devices, transplants and the Berlin means "we are able to provide the full service. Any patient listed on our services has a full shot," she said.
Before the transplant program was built in Austin, patients like Zaria would have been put on ECMO and transferred to Texas Children's Hospital in Houston or to Children's Health in Dallas, "which is a very big deal," Fraser said after Zaria received the Berlin Heart. "We don't like transporting on ECMO."
Now, kids like Zaria can stay in Austin and be offered every mechanical assist device and a heart transplant if they qualify to be listed on the national registry.
Dell Children's is also seeing inquiries for its transplant program from patients outside of Central Texas as well as patients who have been rejected at other transplant centers. So far, half of the transplanted patients have tried to get transplants at other centers. Half were already under the care of the Dell Children's team when they needed to be listed.
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Waiting for the heart
"There's a lot of stories in this heart transplant," Fraser says. It's the first Berlin Heart transplant, the first infant transplant, and the first ABO incompatible transplant, he says.
An ABO incompatible transplant happens when a donor's blood type doesn't match the recipient's. It's possible in infants because they have what is called a "naïve immune system." After the transplant they will forever take on the blood type of that donor heart. It makes the surgery more complicated, Fraser says, and not something all new programs would take on.
While Zaria had her Berlin heart, she looked like a normal baby, just with a mechanical heart on the outside of her body that was attached to a machine.
She had become used to having the pump and would use it as a foot rest. (She still looks for that foot rest even after her transplant.) While waiting for a heart, Zaria played in her crib and could be carried a short distance, still tethered to the machine.
When the call came that a heart had been found for Zaria, Guthrie was asleep and missed it, but the hospital called her mom, who woke her up.
She thought there was no way it could be the call for the heart, because it had only been about 2 1/2 months, but it was was the call they had been waiting for.
"We broke down in tears," Guthrie says.
Saying goodbye to her baby at the entrance to the operating room, Guthrie says, "was the scariest moment of my life." Even so, she says, "I knew she was in the best hands possible."
For the staff, who had been part of her care for months, "the transplant was a very emotional time for everyone," Fraser says. "It was such a big time."
Because it would be the program's first infant heart transplant, making the heart more delicate because of its size, Fraser went to retrieve the donor heart himself. He has been doing research in the best protocols for heart preservation for transplant for decades and wrote the recommendations that other hospitals use.
Dell Children's also brings a perfusionist with them to retrieve a donor heart. That perfusionist can monitor the pressures inside the organ for a better outcome. As well, Dell Children's makes its own cardioplegia solution to preserve the heart.
That level of care and precision, Fraser says, "we think distinguishes our program."
While Fraser went to retrieve the donor heart, which he always reminds starts with an unbelievable loss for one family, Binsalamah, assisted by Dr. Ziv Beckerman, was in the operating room at Dell Children's to prepare Zaria and perform the transplant once the heart arrived.
"He brought me the perfect organ," Binsalamah says. "The heart started immediately."
The heart had been out of a body for only four hours, which was the shortest time yet for Dell Children's. The goal is less than six hours.
When Binsalamah removed the Berlin Heart, he saw just how well-supported they had been able to keep Zaria while she had the pump. "From all my experience, I've never seen a kid that had a clean pump until this transplant," Binsalamah says. "They usually develop small clots."
The pump will be sent back to Excor to be recycled for a new pump for another child.
Life after transplant
Zaria recovered quickly from the transplant. By the next day, she was able to be off the ventilator.
"She was awake and smiling and laughing," Guthrie says. "you wouldn't know she had just had open heart surgery."
She would have been able to go home a week after transplant, except her siblings, who are 18 months, 3 and 13, shared a stomach bug with her. The team wanted to support her in the hospital while she recovered and get everyone at home well before sending Zaria home.
This first year, Zaria will have multiple heart biopsies to make sure she's not rejecting the heart, which is harder to tell in an infant than an older child without the biopsies.
She'll be on antirejection medications and have to avoid some foods to prevent food-borne illnesses as well as not have reptiles at home because of the risk of salmonella, and avoid kitty litter.
Anytime she gets sick, her family will immediately call the heart transplant team line to assess whether she needs to come into the hospital for treatment.
Most of her care in the hospital, from emergency room to admission, from Berlin Heart surgery to transplant and recovery, was covered by insurance.
The new heart will grow with her body. "It's quite a miracle," Fraser says.
Most heart transplants last about 15 years, but infant transplants last longer. Fraser says he knows patients who received their hearts as infants who still have the same heart 25 to 30 years later.
Eventually, though, she will need another transplant.
"I always tell patients that a heart transplant is not a cure," Binsalamah says. "We're replacing the disease with another one, but the new disease is well-tolerated."
They still don't know why Zaria's original heart began to fail. They sent it to pathology and confirmed what they knew: She had idiopathic dilated cardiomyopathy. Idiopathic means there's no explanation to what caused it. Dilated cardiomyopathy means the left ventricle is enlarged and can't pump as well as it should.
"It would be nice if we could specifically identify causation in all patients," Fraser says. "We don't always have a diagnosis."
With her new heart, her echocardiogram shows a normal heart.
Guthrie is looking forward to having all of her children under one roof and planned to spend Monday just hanging out as a family in their living room.
She is, of course, nervous. With the Berlin Heart, she says, she could see it working and there was comfort in knowing Zaria was in the hospital with expert care there.
She's been assured the experts are just a phone call away.
The staff, of course, will miss her. Everyone always wanted to pick her up.
"She's as cute as she can be," Fraser says.
American-Statesman reporter Nicole Villalpando has been following the start of the transplant program and the expansion at Dell Children’s Medical Center of Central Texas. She was allowed into the operating room when the new team performed the hospital’s first heart transplant.