Optimism and Bravery Without a Cure

Oct. 4, 2007 — -- Bailey Hunsberger is no different than most teenagers. She loves music and hanging out with her two little sisters. She decorates her room with posters of her favorite heartthrobs like actor Patrick Dempsey, also known as "McDreamy" on the television show "Grey's Anatomy."

What makes Bailey different than other girls her age is that she is fighting for her life. Bailey's mother, Angie McGraw, remembered the moment she found out about her daughter's condition.

When Bailey was born she looked fine at first. She even scored a perfect 10 on the APGAR, a test doctors use to evaluate newborns.

"Fourteen hours later they came in and said, 'You have a very sick baby,'" McGraw said.

Bailey was born with aortic stenosis, a defective aortic valve that allows blood to back up into her lungs. Doctors told Bailey's parents she needed surgery, and her first operation took place when she was only 3 days old.

By the time she was 4, she'd had another one. After that there were little bumps in the road. Simple childhood illnesses such as ear infections or the common cold eventually turned into pneumonia. Bailey and her parents ended up in and out of the hospital. Her father, Scott Hunsberger, said, "You take the ordinary bumps in the road and multiply that by 100."

Even so, Bailey still did ordinary things, like riding her bike and playing ball. "She was happy. She thought she was perfectly healthy," McGraw said.

But in January 2005, the family was reminded of just how sick Bailey really was. Bailey told her parents she felt a little bloated -- it turned out she had retained enough water to increase her body weight by 15 percent. Her doctors said the fluid retention indicated that her heart was weakening.

At age 12, Bailey went into congestive heart failure. A documentary film team followed her, her family and her doctors at Riley Children's Hospital in Indianapolis as they searched for a way to keep Bailey alive.

In the film "Heart to Heart," Dr. Mark W. Turrentine, a pediatric cardiovascular surgeon at the Riley Hospital for Children, said Bailey wasn't doing as well as she appeared. Bailey's doctors wondered if she had scar tissue in her heart. They wanted to avoid a heart and lung transplant by cutting out that scarring.

Dr. Robert Darragh, a pediatric cardiologist at the Riley Hospital, said she would have a much better long-term chance of survival if her lungs were given a chance to heal. "She would be a candidate for just a heart transplant alone," he said.

After meeting with the doctors, Bailey's parents felt positive, but worried about the possibility of a double transplant.

"We knew what that meant in terms of Bailey's long-term viability," Scott said.

Hope Comes Half a World Away

The doctors acted quickly and planned a surgery to thin the lining of Bailey's heart in the hopes that her blood and lung pressures would go down. But they weren't sure her heart was strong enough to work on its own. They needed a back-up plan.

"They kind of told me everything they will do and everything that they might do," Bailey said. "Like I might need an assist device and that'll help my heart rest up so that it will be better."

Bailey's parents felt this device might buy them time while waiting for a donor heart. But the device, known as the Berlin Heart, lived half a world away in Germany.

"The system basically consists of a pump that functions as the main pumping chamber of the heart," Turrentine said. "A valve will close, a valve will open, and blood is ejected out. It allows us to do for children what we basically do for adults."

The U.S. Food and Drug Association, however, hasn't approved its use in the United States.

Dr. Daniel Schultz, director of the Center for Devices and Radiological Health at the FDA, advocated a careful approach when considering devices made overseas.

"We have to maintain a standard that allows people to be assured that those devices have been appropriately studied," he said. "There are instances where we have all seen that devices have been used in other places with not such brilliant results."

McGraw was shocked to learn that the device wasn't yet available in the United States. Bailey's parents would have to get the Berlin Heart through what's called a compassionate use exemption.

Bailey was the ninth American child to apply for the Berlin Heart. The FDA had never denied a request and they approved Bailey as well.

Watching and Waiting

After four hours of surgery the doctors had nearly succeeded in removing the scar tissue from Bailey's heart. But her heart was not responding as hoped. The doctors would have to rely on their back-up plan and attach the Berlin Heart.

They attached the device successfully and watched as her heart began to pump, stronger than before. For the next 30 days Bailey's parents watched hospital monitors, looking for signs that Bailey's pulmonary pressure had come down.

When they cleared that first hurdle, they felt relieved that Bailey wouldn't need a lung transplant in addition to a heart transplant.

From that moment on McGraw and Hunsberger waited anxiously as doctors looked for a heart that would be compatible with Bailey's body. Days turned into weeks and weeks into months. Bailey was still in the hospital, still attached to a 6-foot tube attached to a 200-pound pump and a computer that runs the heart.

"You're not as mobile as you would be without this but that's only because you have this cord so it's not been bad because I can walk around," Bailey said.

Months into her ordeal, Bailey had become friends with the staff at Riley Hospital, the place she called home for the last 100 days. She even had a water gun fight with Turrentine. And then one day a surprise visitor showed up.

The documentary team filming Bailey had pulled some strings and got their friend Patrick Dempsey to pay a visit. But their meeting could only be a temporary distraction before reality set in once more.

Facing New Challenges

"We didn't keep anything from her," McGraw said. "We had lots of those life and death talks about survival and the talk you never want to have with anybody, let alone your own child. And she came up to me shortly before we went to the hospital for her surgery. She said, 'I think I know what's gonna happen. I think I'm gonna need the Berlin Heart and they're gonna put me on it and my heart is gonna get better, and they're gonna take me off it." McGraw started to choke up. "And she said, 'I'm gonna help lots of kids in the process.'"

Secretly McGraw didn't believe that the heart would work. But Bailey's premonition was right. Nearly six months after entering Riley Hospital, doctors said Bailey's heart was well enough to beat on its own.

"In the back of our minds we were always kind of hoping that something like this could happen," Turrentine said.

After 162 days with an artificial heart beat, Bailey went back into surgery to have the Berlin Heart removed. "I can't wait to hug her and not feel the device," her father said.

Bailey's surgery was successful yet again -- her heart was functioning even without the device. She had effectively become a poster child for the Berlin Heart. But Schultz remained cautious.

"What we need to see is the information on all those individual experiences put together in a way we can understand how the Berlin Heart works in different situations in these sick children," he said. "It's one thing to have 100 individual experiences, it's another thing to have a data set to look at so that we can actually define performance in a range of experiences."

Since May, the Berlin Heart has been in clinical trials at 10 pediatric hospitals throughout the United States: the first step in getting it approved for use nationwide.

It has now been two years since Bailey was on the Berlin Heart. She's a healthy teenager, doing things normal 15 year olds do, but there's a certain maturity to her.

"Just all the stuff I've been through, I know there's a lot of kids at my school that are really immature for their age and I think about everything that's happened the past few years and they make such big deals out of little stuff and it doesn't, little stuff doesn't bother me anymore," she said.

She's been sick again lately, and there's the very real possibility that she will eventually need that heart transplant.

"You can't look too far into the future because you don't know what's going to happen," Bailey said. "You just focus on one day, get through that day, and then focus on the next."