'You can see it beating': Baby gets another chance with 1st mechanical heart pump from Dell Children's
When Zaria Grace Jackson was born last year, she weighed 9 pounds, 10 ounces.
"My pregnancy was pretty normal," said her mom, Olivia Guthrie. "Everything was fine."
There were no signs of the heart failure that would follow three months later.
"She was a very easy baby," said Guthrie, and was adored by her siblings, who are 15 months, 3 and 12.
She would smile and follow you around with her eyes, Guthrie said.
"The hardest part about Zaria was waking up every two or three hours to feed her," she said.
Things changed. On Jan. 4, Zaria began to stop eating. She was constipated and not urinating. Then she began panting.
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When Guthrie brought her to the pediatrician Jan. 8, she was told to take her immediately to the emergency room at Dell Children's Medical Center, where doctors have been building a new heart program more quickly than most children's hospitals have.
There doctors discovered her heart was enlarged and moved her to the cardiac intensive care unit.
On Jan. 11, Zaria became the first child to receive a Berlin Heart pump at Dell Children's Medical Center of Central Texas.
It's the latest first 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 Sept. 24, 2019, the program implanted its first left ventricular assist device, a mechanical device that sits inside the body and helps the heart pump.
Then on Oct. 3, 2020, the program performed its first heart transplant. Last week, a second heart transplant was performed.
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Program created at ‘breakneck’ speed
Being able to do a Berlin Heart means that the program can offer the full compendium of heart care.
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.
"It's pretty gruesome," said Dr. Charles Fraser Jr., the head of the center.
Dr. Chesney Castleberry, the medical director of the pediatric heart transplant team, said that in some ways she's more excited about Dell Children's being able to do a Berlin Heart procedure than she was about the first transplant.
"There are a lot of places out there who can do a transplant, who can't do a Berlin," Castleberry said. 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 Dell Children's and Dell Medical School recruited Fraser from Texas Children's Hospital in 2018 to build the center, children with heart conditions that required a mechanical assist device such as the Berlin Heart or a VAD or who needed a transplant would have to travel to Texas Children's in Houston or Children's Health in Dallas for care.
A patient like Zaria would have been put on an extracorporeal membrane oxygenation machine and transported by helicopter to Houston or Dallas, "which is a very big deal," Fraser said. "We don't like transporting on ECMO."
Dr. Ziyad Binsalamah, the surgical director of heart transplantation and mechanical circulatory support at Dell Children's, said of Zaria, "There's a good chance that she would not have made the transfer to another center."
The speed at which Dell Children's has gone from no heart transplant or mechanical device program to a full program has surprised even those involved.
"If you look at other new programs that have started ... we've done more in six months than most of those centers. They are getting to this point after two years," Castleberry said. "The pace is actually quite breakneck."
The patients Castleberry has been following since she arrived in 2019 have typically been very complicated, like Zaria.
"Over and over again, what we keep seeing is so much need in this region," Castleberry said.
‘Her heart just got sick’
What happened to Zaria is "every parent's nightmare," Fraser said.
A week before, she looked normal, but once at Dell Children's her case escalated quickly, Fraser said.
When Zaria arrived at Dell Children's at 4:25 p.m. Jan. 8, doctors thought maybe she had pneumonia or bronchitis. When they realized it was heart-related, they looked for infection.
That wasn't the case with Zaria.
They still do not know what caused Zaria's heart failure.
"As frustrating and as crazy as it sounds, it's unknown," Fraser said.
Her heart looked structurally normal, but the squeeze function in the left ventricle was terrible, he said.
"She was just unlucky," Guthrie said of her daughter. "Her heart just got sick."
Friday night into Saturday, Zaria's condition worsened, and she was put on a ventilator. The team discussed putting her on ECMO, but that's a temporary solution that can last for only days, weeks, maybe a month, with the risk of other organs failing. ECMO can work well for kids when doctors believe their heart can regain function quickly and begin to recover.
That wasn't the case with Zaria.
The Berlin Heart provides a longer bridge between heart failure and either the heart recovering or a transplant. The average Berlin Heart is in a patient for 188 days, but there are kids who have had them for more than a year, Fraser said.
Zaria's heart recovering does not seem likely because it was in such bad shape, he said.
When the team made the decision Saturday that most likely Zaria would need a Berlin Heart, Dell Children's first had to get the device and all of its machinery to Austin.
Berlin Heart is based in The Woodlands, and luckily, Richard Owens, Dell Children's lead perfusionist and director of mechanical circulatory support, had worked there previously. He made the call to have a company representative in San Antonio drive everything to Austin on Sunday evening in time for surgery Monday.
Guthrie remembers telling Zaria on Monday that she loved her and that she would be there when she was done.
"It was awful," she said of saying goodbye to her baby outside the operating room area. "It was super scary."
But she had faith because even though this would be the first Berlin Heart at Dell Children's, the team had a lot of experience with the device. She knew all eyes would be on her baby.
"It's a whole lot of people very interested in what's going on," Guthrie said.
Beyond the Berlin Heart
Zaria is now listed for a heart transplant, one of two patients currently listed through Dell Children's. Another six are in line to be listed when their conditions progress to that point.
Because of the Berlin Heart, Zaria is a better candidate for a transplant, Fraser said. "This keeps all her vital organs in good condition," he said.
Berlin Hearts were not new to Fraser. While at Texas Children's, he ran the clinical trial that compared kids who received the Berlin Heart with kids who received ECMO as the bridge to transplant.
"The outcomes were striking," he said. "Ninety percent of the kids enrolled effectively bridged to transplant. In ECMO, no patient lived more than a month."
Before that trial, Fraser said, the choices for doctors and patients "was really terrible. Do you keep them in the ICU on a breathing machine giving them medicine or do you put them on ECMO?"
That trial helped the Berlin Heart get Food and Drug Administration approval in 2011.
Zaria was expected to be taken off a breathing machine late last week, but she has been awake on and off and has sat up in a baby chair.
"She can look around, her eyes are open, and she looks at me," Guthrie said.
"I talk to her a lot and say, ‘What are you doing, pretty girl?’ ‘I’m here.’ I rub her head, and she grips my fingers, for sure."
Doctors have been talking to her about what's next for Zaria, which can feel overwhelming.
She will be at Dell Children's until she gets a heart transplant and is well enough to leave the hospital.
In Europe, patients have left the hospital with a Berlin Heart, but it's not FDA-approved in the United States to do so.
The medical management of it is also more than families could be expected to do, Fraser said.
The Berlin Heart has a risk of clots in the pump or bleeding, infection or stroke. The management of the pump and medications to avoid clotting have gotten better, Castleberry said.
Pumps can malfunction and need to be replaced with a new one. Zaria also has the smallest model, and as she grows, she might need to move up to a bigger pump.
The search for a better pump for kids like Zaria is one of the reasons Binsalamah came to Dell Children's.
Binsalamah, who trained under Fraser at Texas Children's and arrived for his first day at Dell Children's on Jan. 9, said he wasn't expecting to do a Berlin Heart procedure two days in, but it's a sign of how quickly this program is growing and the potential he sees for it.
"This program in the next five years can be one of the leading programs in the nation," he said.
Binsalamah is planning to work with the University of Texas' biomechanical engineering program and Dell Medical School to develop new devices. One goal is to overcome some of the problems of the Berlin Heart, such as not being able to go home with it, or it being used as a bridge to a heart transplant instead of a more permanent solution.
"There's not many centers that work on innovation like that," he said. Most of the innovation is coming from companies improving what's already out there, he said. "No one else is collaborating with their own university to develop their own new device."
Zaria's family, though, is grateful for the miracle of that Berlin Heart sitting outside her body.
When Guthrie first saw it, it was a bit hard to look at, but now she has a different view.
"It is kind of cool. I know it's keeping her alive," she said. "You can see it beating. It's doing its job."
Nicole Villalpando writes about health for the American-Statesman. She can be reached at email@example.com.
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.