Grace Jennings returned to school earlier this month. The 12-year-old from San Antonio spent last semester in Austin at Dell Children’s Medical Center of Central Texas.
"The students are really cool," mom Jamie Jennings says. She’s also the school librarian, so she knows what she’s talking about. They’re very curious about Grace and the left ventricular assist device that now sits beside her heart and pumps Grace’s blood for her.
Grace has exceeded all of her doctor’s expectations. Most kids who have an LVAD go back to school after a year, says Dr. Chesney Castleberry, a cardiologist at Dell Children’s. Grace went back four months later.
"She didn’t appreciate our rules," Castleberry says. "She set her own goals and passed them and made us look silly."
Grace is the first kid to have such a device implanted at Dell Children’s. It’s part of the growing list of surgeries that now can be done at Dell Children’s since the hospital brought in Dr. Charles Fraser Jr. in 2018 to create the Texas Center for Pediatric and Congenital Heart Disease, which is a partnership of Dell Children’s, Dell Medical School at the University of Texas at Austin and UT Health Austin.
Grace came to Dell Children’s on Sept. 3 after noticing more and more symptoms of her heart's decline, including exhaustion and fluid in her abdomen and legs.
"Everything was swollen," Jennings says. "You didn’t look like Grace."
"I looked like a watermelon," Grace says.
"We were heading toward heart failure," Jennings says.
Grace remembers being nervous because she knew she was going to have surgery. She remembers telling her mom, "I just don’t want to die. ... I’m afraid I’m going to die."
Her heart valve was leaking a lot when she came to Dell Children’s. Doctors hoped to fix it surgically, but "we had to do so much more," Fraser says.
While preparing for her surgery, Grace went into cardiac arrest after having an irregular heart rhythm on Sept. 18.
Jennings remembers it was 4 a.m., and she was awoken by a nurse calling Grace’s name. She thought he was just there to give Grace a shot, but when he flipped on the lights and she saw the look on his face, she knew something was wrong.
He called the code.
"I can’t watch this," Jennings remembers thinking. She had to leave the room.
They performed CPR on Grace for two hours.
Jennings, who had fallen out of religious practice, called for the priest.
Doctors were able to hook up Grace to an extracorporeal membrane oxygenation machine, known as ECMO, which pumps the blood for her heart.
If the cardiac arrest hadn’t happened at Dell, "we would be talking about her in memory," Fraser says.
People can’t be on ECMO forever. "There are all kinds of complications," Fraser says, including bleeding and infection, and effects on the brain and other vital organs.
Grace would not have been able to survive without ECMO, and she couldn’t stay on it.
"The clock is ticking," Fraser says.
The planned repair to her heart wasn’t going to work. She now needed a heart transplant, but while Dell Children’s is in the process of getting qualified for heart transplants, it is still months away from its first heart transplant.
The other option was Grace staying on ECMO and being transported to a heart transplant hospital in Dallas or Houston to wait for a heart. Fraser says she had a low probability of surviving that trip.
Or they could try something different. Fraser knew about inserting left ventricular assist devices to do the work of pumping blood for the heart through his previous work at Texas Children’s.
The challenge with kids is that most devices are designed for adults and then made to fit children in what’s considered off-label usage of an FDA-approved device.
One device designed for kids is the Berlin Heart ventricular assist device, but it sits outside the body and kids can see their blood being pumped back into their bodies. It’s OK for little kids who don’t know any differently, but for preteens or teenagers like Grace, it can be off-putting.
Using the HeartWare ventricular assist device, Fraser could insert the device and the only thing that would be visible outside her body would be a tube that runs from her abdomen to the battery pack and controller that rest inside a backpack that Grace can carry with her. Each battery lasts six to eight hours.
The device has a magnet that spins the blood around to move it throughout the body. Doctors can control the speed of the pumping by adjusting the rate at which magnet spins. The ventricular assist device is probably doing 80% to 90% of the work pumping the blood. The heart is doing the rest.
With the concern about the danger of staying on ECMO, Fraser says, Dell Children’s committed to paying for this operation through community donations before it got full insurance approval. The device was overnighted to the hospital, and doctors and nurses trained quickly on how to do the operation and how to care for Grace afterward.
Because of Grace’s unique anatomy and a multitude of past operations that rerouted her circulation, inserting this device in Grace would be even more complicated.
There may only be about 20 to 30 kids like Grace in the United States, Fraser estimates, who have had the kind of defect Grace had and then had an LVAD inserted.
"As I told her mother, ’You are a pioneer,’" Fraser says. "We need a Grace in the world."
Jennings knew from the time she adopted Grace from China in June 2009 that she had a heart condition. "I was told that it wasn’t as severe as it really was," she says. "They couldn’t have known."
When Jennings met Grace, her skin had a blue tint, which revealed how sick she was.
"You were Smurfy," she tells Grace.
Her Chinese name was Fang Bing Quing, which meant "blue ice," because she was blue and cold. Jennings named her Grace, she says, "because I never knew a Grace who was not kind."
Although Grace was 18 months old, she was the size of a 9-month-old. And she cried a lot.
Grace had a hole in her heart, and the right ventricle was very small. She also had pulmonary stenosis, which means that the blood flow from the right ventricle to the pulmonary artery was obstructed.
It’s the way her heart was formed, Fraser says. The typical treatment for kids like Grace is a whole series of operations to reroute the way the blood flows.
The first few weeks Jennings had with Grace were marked by a kid who was miserable and also had chickenpox, which she gave to Jennings. She didn’t like to be held by Jennings, but she liked being held by Jennings’ mom, who looked more like the older caretakers in the orphanage.
Grace had her first surgery that July in San Antonio. That surgery, known as the Glenn, redirected blood from the upper body to the lungs.
It was during the recovery of that surgery that Grace and Jennings really bonded.
That surgery worked for a while, but two years later, she had a surgery called the Fontan, which allows the blood to move from the lower part of the body directly to the lungs without having to go through the heart.
As the years went by, Jennings noticed that Grace’s health was declining. She wasn’t satisfied with her doctor in San Antonio, so she got a second opinion from a different cardiologist, who sent her to Fraser in Austin.
The first appointment in July was in preparation for an eventual surgery to repair what was no longer working. But in September, when it became clear that Grace was worsening, Fraser told her to come to Austin the day before her next scheduled appointment to be admitted.
The cardiac team inserted the LVAD on Sept. 24. It wasn’t without complications. Three weeks later, Grace had a brain bleed, which might have been from being on ECMO or from the blood thinners she’s now on. She needed another surgery to repair that.
Grace still has some lasting effects from the bleed. Her brain just gets very tired, which is why she had built-in brain breaks during her school day and a scheduled nap instead of first-period classes.
Grace doesn’t remember much of those first few weeks in the hospital. Her first memory is walking down the hall in a walker on Nov. 4.
Grace was able to leave the hospital on Dec. 23 and see doctors for outpatient care. They headed home to San Antonio on Jan. 3.
Fraser says these devices "tend to be very reliable." He has patients from Texas Children’s who have had them for eight or nine years.
Grace has learned how to take her own blood pressure using a Doppler monitor. Jennings keeps a logbook of all the numbers the LVAD’s controller records and sends files to doctors at Dell Children’s.
Grace still comes to Dell every few weeks for doctors to do lab tests and to check her LVAD and calibrate how fast the magnet is spinning the blood. Grace has decorated the bag for her controller and battery packs with different pop-culture pins such as the Deathly Hallows from "Harry Potter" books.
An LVAD can be considered a "destination" procedure, meaning that the patient lives with this as their final solution. The device can be replaced as it ages. Or it can be a procedure to buy time before a heart transplant.
"In Grace’s case, it’s working great," Fraser says. "She’s doing incredibly well on it."
If she wanted to keep the LVAD, she could, but the real problem in Grace’s mind is that there are limitations. She can take a shower using a special plastic cover to keep the external parts safe, but she cannot take a bubble bath or go on a water park ride or go to the beach with it and stand in the ocean.
"I want to do kids stuff," she says.
The Jenningses also learned that there are fears with the LVAD. Earlier this month, Grace didn’t fully plug the battery into the controller. A loud beep alerted Jennings, who saw her daughter’s eyes get really wide and her feet fly up. Jennings figured out what was wrong and plugged everything back in again, but it was scary.
"I think she’s doing better than me," Jennings told Castleberry, who will be heading the transplant team at Dell Children’s.
Grace plans to get put on the heart transplant list once Dell Children’s has been approved to do transplants.
"Now we wait for a new heart," Jennings says.
Grace is realistic, though: "It isn’t like hearts are just lying around somewhere."
Since her surgery, Grace has grown and is getting stronger.
"My goodness, she’s strong," Castleberry says as she examines Grace. "You are rock solid."
Strength is what all her caregivers notice about Grace. They pop in to see her whenever she’s in the hospital.
"She’s got an intrepid spirit," Fraser says. "She’s positive, tough and determined to get better."