Kelli Sheffield sat with her 21-year-old daughter, Amanda, in the courtyard outside Starbucks at Children's Hospital Central California. They both wiped tears – even as they laughed together. They had driven from their home in Santa Maria for Amanda’s annual checkup with the cardiologist she calls “Dr. Pro.”
“Having a new doctor is going to be weird,” said Amanda, looking at her mom with tear tracks on her face.
“It’s sad,” said Kelli, using a napkin to dab her eyes. “I don’t like it.”
The Sheffields were on their way to Amanda’s last appointment with Dr. James Prochazka, medical director, pediatric cardiology, Children's Hospital. Pediatric patients regularly transition to adult medicine when they reach age 21.
“I’ve been the oldest one in the waiting room for a long time,” said Amanda, smiling again. “Once when I was waiting to get my echocardiogram when I was 16, there was a kid my age, and then after my surgery there was another one in intensive care my age. But that’s only twice I can think of.”
Amanda has undergone two heart surgeries at Children's Hospital Central California. The first occurred shortly after she was born.
“Amanda got really fussy when she was 8 days old,” said Kelli. “She wouldn’t sleep. I was up with her all night long and I knew something was wrong. Then my sister-in-law noticed her lips were blue, so we called the doctor.”
The worrisome health problems surfaced two days before Christmas 1990. Amanda’s pediatrician was not on call, but Dr. David J. Ikola, a pediatrician at the Pediatric Medical Group in Santa Maria, learned Amanda’s symptoms by phone.
“He said it was probably a heart defect and sent us to Marian Medical Center to wait for an air transfer to Children’s,” said Kelli. “The transport team took a picture of Amanda and gave it to me before they left.”
Kelli followed her daughter by car. “The first thing Dr. Pro said when I got to Children’s was ‘I know what’s wrong and we can fix it.’”
Dr. Ikola had made a correct preliminary diagnosis. Amanda was born with a rare congenital heart defect known as coarctation of the aorta, often referred to as “coarc.” Of all children born with heart defects, less than 7 percent have coarc, characterized by a narrowing in the aorta, the main artery leading away from the heart. In many coarc cases, patients also have a bicuspid aortic valve as Amanda did.
“Heart defects can present on the first day,” said Dr. Prochazka. “But typically these babies are born fine and go home from the hospital.”
In the developing fetus, a blood vessel called the ductus arteriosus connects the pulmonary artery to the aortic arch to bypass the unborn baby’s nonfunctioning lungs. Upon birth the vessel closes to allow proper circulation through the pulmonary system. If the baby has a heart defect, symptoms can begin to occur.
“They usually present with an ashen, dusky color from poor circulation,” said Dr. Prochazka. “Often there’s grunting as they struggle with breathing difficulties. At this point the babies quickly go into congestive heart failure.”
Today, screenings are performed on all newborns to detect certain cardiac problems before the baby leaves the birthing center, but Amanda was born before they became routine.
“At this age, coarctation would be suggested by the symptoms,” said Dr. Prochazka. “With coarctation the pulse is easy to find in the right arm, but it’s difficult to find in the legs. The heart is working hard, like it’s trying to pump against a garden hose with a kink in it.”
An echocardiogram confirmed the suspected diagnosis. “In 1990 we had all the modern equipment for diagnosis,” said Dr. Prochazka. “Amanda benefitted from our excellent cardiographic capabilities, which were strong even then. These cases are very rewarding because we can fix it.”
While the bicuspid aortic valve would simply need monitoring over the course of Amanda’s life, the coarc required immediate surgical repair. “She would’ve died without surgery,” said Kelli.
The procedure took place the following day – Christmas Eve. “They went through my back, behind my left shoulder to repair the aorta,” said Amanda. “They took an artery out of my left arm to patch the narrow spot. They described it as shaped like an hourglass.”
After a 14-day stay in the pediatric intensive care unit (PICU), Amanda returned home to Santa Maria. “I still have that bicuspid aortic valve and might have to have surgery, but Dr. Pro said it’s nothing to worry about now,” said Amanda. “He said by the time I need surgery they may have come up with a new procedure to repair it.”
The Sheffields visited Dr. Prochazka every three months for the first year of Amanda’s life, every six months the following year, and by age 2 they began making their annual trip to Children's Hospital Central California for follow-up care. At one of those yearly exams Dr. Prochazka found a weak pulse, ordered an MRI and discovered an aortic aneurysm at the site of her coarctation repair. The blood-filled, balloon-like bulge stretched her main artery and posed the threat of rupturing at any time. In addition to severe pain, a ruptured aorta causes massive internal hemorrhage and, unless treated immediately, death.
“It’s amazing because I was in a car accident right before that and it didn’t cause the aneurysm to burst,” said Amanda.
Amanda had her second surgery at age 16 – on Halloween. Malcolm MacDonald, cardiac surgeon, Children's Hospital Central California, performed the aneurysm repair.
“In the days when Amanda had her coarctation there was a small but real risk of developing an aneurysm at the surgery site,” said Dr. MacDonald. “The way we do it now it’s fairly rare.” Today’s method involves removing rather than enlarging the narrow section of the aorta. “We went back through the left chest where there was a lot of scar tissue from the previous surgery, which is a little like cutting through a cement wall to get to a spaghetti noodle,” he said. “It’s a time-consuming process, but we were able to get to the dilation in the aorta and patch it.”
Amanda spent 24 hours in PICU post-surgery and another seven days in acute care while her family stayed at Ronald McDonald House. “I met Billy Ray Cyrus when I was here,” she said. “His daughter was doing a concert as Hannah Montana and he was meeting with kids, but I couldn’t leave my room. So my mom went and talked to him.”
“I did,” said Kelli. “I said that my daughter just had heart surgery and she couldn’t leave her room. He was already planning on visiting a few rooms in her unit and 20 minutes later he showed up in her room.”
“We made a sign that said, “Hi, Billy Ray. Come on in!’” Amanda grinned.
As the time for their last appointment drew near, Kelli reflected on the many trips she’s made to Children’s over the past 21 years. “We’re really grateful we were referred here,” she said. “I know of families from Santa Maria that were referred all the way up to Stanford.” After a pause she added. “And we never would’ve met Dr. Pro.”
Once again, mother and daughter grew misty eyed. “I’ve always loved Dr. Pro,” said Amanda. She sighed and prepared to leave the courtyard for her appointment. “I don’t think I’ve ever trusted a person like I trust Dr. Pro.”
Dr. Prochazka has practiced pediatric cardiology with Children’s for more than 30 years and has made a profound impact on Central California’s pediatric population. He turned tears to grins when he told Amanda she could continue her annual visits right up until he retires.
In the meantime, Amanda will remain the oldest patient in the waiting room.