The Art of Healing
Even before he was born, Austin Ball had a heart defect that was considered fatal. The Heart Center Team at Children's Hospital of Pittsburgh of UPMC stepped in with a daring alternative and advanced technology. Today, Austin's celebrating his first birthday.
In a dim room at Georgetown Hospital in January 2008, Cher Ball eagerly awaited the results of her sonogram. The 34-year-old mother wanted reassurance that her second baby wouldn’t share the problems of his 6-year-old sister, Symone, who had been a "micro-preemie," born at just 26 weeks’ gestation. But as the technician scanned this 26-week infant in utero, a shadow on the screen threw a shadow on Cher’s hopes.
"They found only three chambers in his heart. The left ventricle was shadowed by a tumor," she recounts. The defect had enlarged the baby’s heart and blocked normal blood flow. Doctors told his mother the problem was inoperable. "I don’t do normal pregnancies," she says wryly. Ten weeks later, on April 29, 2008, Austin Jason Ball entered the world. Though he weighed more than 9 pounds, he was at immediate risk of cardiac arrest. Four hours later, he was transferred to Children’s National Medical Center in Washington, D.C. "I didn’t even get to hold him," Ball remembers.
With an incredibly rare condition found in fewer than one in 50,000 babies, Austin was a candidate for a heart transplant. But few hospitals, even in the nation’s capital, have the expertise to treat these daunting and complicated cases. The concentration of talent required to handle all aspects of their care – from engineers to surgeons and helicopter crews – exists in only a few U.S. cities. But the Heart Center at Children’s Hospital of Pittsburgh of UPMC, now an acknowledged world leader in pediatric transplantation, held out that hope – and ultimately, more.
Infants can accept organs from a wider array of blood types than adults. That improves the chances of finding an organ in time to perform a transplant. Over the next four months, Ball waited at her home in the nearby suburb of White Plains, Md., taking Austin for tests and treatments and interviewing with transplant teams in Baltimore and Philadelphia. (Health insurance provided by Cher’s employer would completely cover the cost of the procedure.) On Sept. 24, Austin underwent cardiac catheterization, a procedure to look inside his heart to gather data for the expected surgery. It did not go well.
After 72 hours, Ball knew her baby was sicker. "He wouldn’t sleep; he had a high-pitched scream; his color wasn’t good, and his nostrils were flaring. I put him down and ran to talk to the doctors. No sooner than I left, he was in cardiac arrest." For 90 minutes, 20 staffers surrounded the crib, packing the baby’s lifeless body with ice and attempting to restart his heart. "After four minutes, babies are brain-dead," Ball says. "I asked them to keep going."
Meanwhile, 250 miles away, the Heart Center Team was fielding requests for admission from around the country – and the world. Since performing the first pediatric heart transplant in 1982, Children’s Hospital has completed more than 250 others. Eighty-five percent to 90 percent of its young patients survive at least three years following surgery, a rate significantly higher than the 80 percent national average.
"When you compare us to other institutions, why do we do so well?" asks Dr. Victor Morell, Children’s chief in the division of pediatric cardiothoracic surgery. "It’s not just the docs, not just the engineering, not just nursing. It takes a whole team of people to make this work. That’s what makes us special. And we do [procedures] all the time – in institutions that do one every five years, the patient is not well-served."
A participant in dozens of national research studies, Children’s was also one of a handful of U.S. hospitals authorized to test a pediatric ventricular-assist device – a tiny mechanical pump for infant hearts. For babies like Austin, the device offered a fighting chance.
"If you break a leg or arm, you rest it. But the heart and lungs can’t rest," explains Dr. Peter Wearden, a broad-shouldered pediatric cardiothoracic surgeon and director of pediatric mechanical cardiopulmonary support at the Heart Center. "So if we have devices that can allow them to rest, they may have a chance to recover."
Transplantation, not recovery, was the next step his mother expected for Austin. The Washington cardiac team had put him on an extra-corporeal membrane oxygenation device (dubbed ECMO) that sustained his heart-lung function but required sedation and narrowed the options for transplant. Without the right experience, the hospital was unwilling to transplant Austin and referred the Balls to Pittsburgh.
On Oct. 1, the Children’s Transport Team of Pittsburgh flew to D.C. and picked up Austin for the 90-minute flight. With the infant on ECMO support, and a physician, nurse, respiratory therapist and perfusionist aboard, the cabin was full. As the flight departed, Cher Ball watched, walked to her car and began the five-hour drive to follow her son.
"I had 24 hours to pack and get loaded for Pittsburgh, walk away from my job and my life as I knew it, with a child who was almost dead," Ball calmly recalls. "But there was no other way."
The next morning, on the second floor of Children’s Hospital, Cher and Austin met the team in charge of their future. Armed with a model of a normal heart, Joanne Snyder, a 29-year-old physician assistant, welcomed Cher to the heart center.
"We see the patient first thing," explains the cheerful Snyder. Working with 10 to 15 transplant patients at a time, she introduces families to "the tubes and the wires," she says. Next, Cher Ball met with Drs. Morell and Wearden, who suggested a new plan: Give Austin a Berlin pump. Manufactured in Germany, The Berlin Heart Excor, the child-sized external mechanical heart, is approved only for experimental use in the United States. Surgeons Wearden, Morell and cardiac chief Steven Webber had already used the device eight times and had made an exciting observation. In several cases slated for transplant, the device was not just a bridge to a transplant – it allowed the child’s own heart to recover proper function instead.
With a professional background in physical therapy and months of on-the-spot learning, Ball immediately understood the implications of the offer. "She was well-educated, quick to get online and learn," observes Joanne Snyder.
"I did not learn about the [Berlin pump] until we got to Pittsburgh," says Ball. "But I understood that while we waited for a donor heart, this would allow him to be awake and responsive – because we didn’t know how long we would have to wait. So the assistive heart being available, because of the research being done in Pittsburgh – that was a godsend."
Snyder dived into the permissions and paperwork required to fly the device in from Europe. Meanwhile, Dr. Morell proposed a new approach. The surgeon believed that, supported by the device, Austin could survive the tumor removal and recover his own heart function – eliminating the long-term risks of rejection and failure of a donor heart.
"Whatever you can do with a patient’s old heart, surgically or medically, is the first goal," explains transplantation chief Webber.
Cardiac surgeon Morell agrees. "We dared to say, let’s not transplant," he recalls. "We were giving him the chance to maybe live without transplantation."
On Oct. 7, the Heart Team assembled to remove the fibrous tumor from Austin’s heart wall. The operating room was crowded; in addition to Drs. Morell and Wearden, scrub nurses, surgical residents, anesthesiologists and perfusionists, observers from the ICU and cardiology service gathered to watch the unusual procedure. Austin was removed from the ECMO device; during the four hours of surgery, a heart-lung machine supported his breathing and circulation. At the end of the procedure, "his heart wasn’t happy," Morell says. "I wasn’t sure his heart would recover." The team attached the Berlin pump to Austin’s heart with cannulae, or tubes, that allowed the blood to circulate outside the body.
As Austin woke and gained strength, Children’s social workers kept a watchful eye on his mother. With 23 years of experience helping cardiac-patient families, Children’s Laura Stabile had practical ideas and a sympathetic ear. "She was a constant help," recalls a grateful Cher Ball. "For a time I considered relocating our family to Pittsburgh – I did not want to be so far away from my daughter."
Stabile talked to local schools about admission and helped with options for housing. She connected Ball to the nearby Family House for UPMC families on Neville Avenue to defray housing expenses. (Cher came to jokingly call it her "storage space," as she spent all her waking hours at the hospital.) Stabile also suggested local charities, including the Ryan Scott Kappes Foundation and Gia Nicole Angel Foundation, that could cover other expenses. And she spent hours helping Ball sort through medical information.
Physician assistant Joanne Snyder became another friend. "I still call and e-mail her all the time," says Ball. As their friendship grew, Snyder shared a bit of encouragement: She, too, had been born with a serious heart defect and had been treated successfully at Children’s.
"What I went through doesn’t change things for [the families], but sometimes, you can tell – they need to know there’s hope," she says. And she emphasizes that the full Heart Center Team provides constant support. "From ICU nurses doing dressing changes, to attending [physicians] watching him 24 hours a day, the social workers, the nutritionists, the occupational and physical therapists – everyone focused on getting Austin out of bed and meeting his development milestones," Snyder says.
As fall turned to winter, Austin became a familiar face at the center: "They picked him up and held him; they’d ask how was he eating – that’s amazing for surgeons," Ball notes. "They would find me in the hospital and talk to me personally."
There were sobering issues and long, heartfelt discussions. Austin underwent three more surgeries. The second procedure resized and patched his left ventricle. The third, on Oct. 16, marked a major success; the doctors removed the Berlin pump. As the doctors had hoped, the assistance of the pump had allowed Austin’s heart to heal and beat on its own.
He was discharged to Squirrel Hill’s Children’s Institute, and Cher Ball began to hope for a return to Maryland.
"The most difficult thing to work out with Austin was when Cher felt she was getting to a breaking point and felt he could be well-served at home," says social worker Laura Stabile. "There are lots of people at the beginning of a medical crisis. But it’s when [a patient] reaches a plateau and there aren’t as many people around that the family really needs you."
In early November came another shadow and another setback: An image of Austin’s aorta showed a bulge. The spot where a cannula had been attached was in danger of bursting. Dr. Morell, on vacation for the birth of his own son, raced back to the hospital to do the repair.
"Just when Austin would be getting better, he’d need another surgery. It was discouraging for Cher and frustrating for us," says Snyder.
After a final surgery to repair the baby’s leaking mitral valve in December, Ball pressed the hospital to discharge Austin to home. The decision was both exciting and terrifying. Though Austin had weathered his first cold without incident, he was still on 13 medications. With Ball’s commitment to return to Pittsburgh for any cardiac surgeries, the team readied the infant, now a wiggly 18 pounds, for departure.
"By the time he went home on March 2, he looked great, smiling and cooing. He is resilient," says Morell. "And Cher, let me tell you – she went through hell and back, and that lady never lost her cool. She was always concerned, but always had a positive attitude. She’s a very loving mother."
"I never gave up. I didn’t believe I would lose him – I held on to the idea he would live," says Ball. "God has a plan – I can’t wait to see what he’ll turn out to be. Now he’s in his jumper jumping around. He’s drinking from a sippy cup. He still doesn’t like to be on his tummy because of the incision. And after five months of being with me day in and day out, he’s spoiled rotten."
Austin’s long-term prognosis is still unclear. "Austin is still struggling, as a lot of heart tissue had to be removed," says transplant chief Webber. He may continue to require treatment in Pittsburgh, and was briefly re-admitted to the Washington hospital in March.
Though Austin’s care is still arduous, his mother feels fortunate. "I learned a lot as a person and as a mom – you pull together strength you didn’t know you had. Something like this halts your life. I’m taking time to appreciate it. Right now, I’m home with my son."
Children’s Hospital Develops the Next Generation of Heart Devices
The Heart Center doctors at Children’s Hospital of Pittsburgh are collaborating on two tiny technologies – one internal, one external – that will advance the science of pediatric ventricular-assist devices.
While a number of the devices, known as VADs, have been approved for adults, the U.S. Food and Drug Administration has not yet approved any for use in infants and toddlers. Until one is developed, Children’s relies on case-by-case permission to import The Berlin Heart Excor.
Children’s and medical-device manufacturer Levitronix LLC received a $2.3 million National Heart Lung and Blood Institute Small Business Innovation Research (SBIR) grant to complete development of, and to clinically test, the first external centrifugal pump designed specifically for infants and small children in heart failure. The Children’s team, led by Dr. Peter Wearden, hopes to begin clinical trials of the device, dubbed PediaVAS, this year.
Wearden has also led efforts to develop a totally implantable pediatric VAD. That effort with partners including Carnegie Mellon University and the University of Pittsburgh has also received NIH funding. It will develop a magnetically powered device the size of a AA battery dubbed PediaFlow, which could support a child for as long as six months.
Christine H. O’Toole, a Mount Lebanon freelance writer, last wrote for the magazine about Pittsburgh’s green buildings.
