June 5, 2013 at 8:34 AM ET
When Jason and Stacy Bingham’s 6-year-old daughter, Sierra, got a transplant to replace her failing heart, the couple thought with relief that their lives could finally go back to normal. But six years later, they watched in anguish as 8-year-old Lindsey started showing some of the same symptoms that had plagued her older sister before doctors diagnosed dilated cardiomyopathy.
Stomach pain and shortness of breath soon gave way to more frightening symptoms. When Lindsey’s breathing became labored and her face and belly swelled, the Binghams rushed her to the emergency room where doctors X-rayed the little girl’s chest, the Haines, Ore., parents told NBC’s Dateline.
As they waited for the results, Jason recalled thinking: “Oh, please. Oh, please. Oh, please. Oh, please have a normal chest x-ray. Please have a normal chest x-ray. Please. We’ll take anything, you know? We’ll try our hand at something else. I don’t want to go down that road again.”
Then one of the doctors came over to talk to the family. “He said, ‘Tell me more about Sierra,’” Stacy said. After the couple told him about Sierra’s condition, he said, “She’s got the same thing.”
“I think I lost it,” Stacy remembered. “I couldn’t keep my composure and I remember just sobbing.”
The Binghams had just embarked on a traumatic and terrifying odyssey. With two out of five children already diagnosed with a rare genetic heart disease, odds were that the rest of the kids were at risk, too.
But at the time, Jason and Stacy’s focus was on Lindsey, who was going to need a heart transplant, just as Sierra had. She was already so sick that she would need the help of an artificial heart to survive until a donor heart became available. The next day she was flown from their home in Haines to the Lucille Packard Children’s Hospital at Stanford University in Palo Alto, Calif.
Approximately 6 out of every 1 million kids younger than 18 are diagnosed each year with dilated cardiomyopathy, a disease in which the heart becomes increasingly weaker – and larger, as it tries to compensate.
While the disease can be sparked by a virus, about 70 percent of cases can be traced to genetic mutations, said the family’s cardiologist Dr. Daniel Bernstein, a professor of cardiology at Stanford University and a pediatric cardiologist at the Lucille Packard Children’s Hospital.
“At the time when the older sister was first diagnosed, not much was known about the genetics that can cause these defects,” Bernstein told TODAY.com. “Today there are genetic tests.”
But even with genetic testing, it can be difficult to predict what will happen with an individual child, Bernstein said. Gene mutations boost the risk of having an enlarged and weakened heart, but other genes can modify that risk, he explained.
Though there were no genetic tests back when Sierra had her transplant, the Binghams asked doctors to evaluate the heart function of all their other children – including Lindsey.
“And they all came out normal,” Stacy said. “So we thought, good. Then we can rule that out. That’s not an issue.”
But then Lindsey’s heart started to fail. And when the Binghams had their other kids rechecked, they found more evidence of trouble. Their son, Gage, now 4, had begun to develop such severe problems with his heart’s electrical system that he needed to have a pacemaker implanted -- and could require a transplant in the future.
And while their other two children weren’t showing symptoms of heart disease, 10-year-old Megan did have a minor abnormality on ultrasound. Hunter, age 6, hasn’t shown signs of any problems, but the family history of heart disease is enough to say he’s at risk, Bernstein said.
The average wait for a donor heart for a child is about three months. But in Lindsey’s case the months passed relentlessly: three, then four, then six, then seven. Still no heart became available. Finally, after 239 days, the family was told that a heart was on its way.
For Lindsey, the news was good – but a little frightening.
“I’m just scared a little,” she said. “They’re going to be cutting up my chest and then putting out a heart and putting another one in.”
Meanwhile, the family had tough news about Sierra. Tests showed that she was starting to reject the heart she’d been living with for nearly 7 years.
“I’m sick to my stomach,” Jason said. “To be honest, it’s like someone hit me in the gut.”
While the doctors worked on Sierra’s rejection issue, the Binghams learned that Lindsey would have her heart surgery in the wee hours of Valentine’s Day.
Lindsey, crying, hugged her parents and then was wheeled off to the operating room.
At 6 a.m. Lindsey’s new heart arrived, packaged in a cooler marked “Human Heart.” The organ came from a child who died at a hospital in Nevada, the parents learned.
The surgeons meticulously stitched together all the connections to the new heart and then slipped it inside Lindsey’s chest. Then came the moment of truth. They jolted the heart with a defibrillator. Immediately the organ began to pump in a regular rhythm.
As the weeks went by, Lindsey was doing well, but Sierra’s body was still attacking her heart. The first set of treatments failed.
“That light at the end of the tunnel is so close,” Stacy said. “It’s right there and then the tunnel collapsed.”
But then Sierra’s doctors told the family they had another treatment option. Four more weeks went by with the family nervous, but hopeful. When the tests came back, they showed that the therapy worked. For now, at least, her body had stopped trying to attack her heart.
“That means … We can go home?” Jason asked. “Is that what I’m hearing?”
“I think so,” said Stacy.
The Binghams know that their joy came at a cost to some other family.
“It really is a two-edged sword ‘cause you know what it meant,” said Jason. “And as much as we love our child and want them to live, I can’t imagine the grief that [the other family has] to deal with because they actually had to lose theirs.”
As they get ready to head home, the Binghams are counting their blessings.
“We’ve walked away twice now with two children still alive and that’s more than a lot of people can say here,” Stacy says. “We can go home with five children.”
Watch more of the Bingham’s story Wednesday night on Dateline at 8/7c.