Family faces multiple transplants

Markian Hawryluk / The Bulletin

Palo Alto, Calif. —

Stacy and Jason Bingham have been here before.

Six years ago, it was their oldest daughter, Sierra, now 12, lying in the bed at Lucile Packard Children's Hospital in Palo Alto, Calif., waiting for a heart transplant.

Now it's her younger sister, Lindsey, 8, in the cardiovascular intensive care unit on the very same floor, kept alive by a type of artificial heart until a donor heart becomes available. For a while, their brother Gage, 3, was in the room next door, his heart beating irregularly. And tests have revealed that the Binghams' other two children, Megan, 10, and Hunter, 5, have heart abnormalities as well.

There is a possibility that all five of the Bingham children will need heart transplants.

If that happens, it would likely be the most heart transplants ever for a single family, and a sign of the remarkable progress that has been made in keeping children with failing hearts alive.

For the Binghams, who live in North Powder in rural Eastern Oregon, it represents a lifetime of waiting — waiting for donor hearts, waiting for test results, waiting for the next biopsy to rule out organ rejection. Contemplating five transplants at once is too overwhelming. They must focus on one child, one transplant, one crisis at a time.

“They're not all going to need transplants at the same time,” Stacy, an obstetrics nurse, said pragmatically. “Whoever is the sickest gets the most attention.”

Family affair

Sierra, Lindsey and Gage have all been diagnosed with dilated cardiomyopathy, or DCM, an enlargement of the heart that causes the heart muscle to weaken. In most cases, it results from a heart attack or other damage to the heart.

In children, it's often due to a genetic mutation.

It was only a few years before the Binghams started their family that the medical community began to realize those mutations might be inherited. In the mid-1980s, it was thought that only

1 to 2 percent of DCM patients had other family members with the condition. By the 1990s, family histories suggested the rate might be as high as 10 percent, but cases of DCM were often misdiagnosed. Different relatives developed different symptoms at different times in their lives, and some DCM deaths were merely attributed to heart attacks.

It wasn't until 1998 that two large European studies confirmed that 35 to 50 percent of DCM cases were familial, and the hunt for genetic links ensued. Today, researchers have uncovered some 30 gene mutations linked with the condition.

When Sierra was diagnosed with DCM in 2006, the rest of the family was also tested. Neither Jason nor Stacy showed any signs of heart problems. Megan and Lindsey also checked out. (Hunter was born in Palo Alto, just weeks after Sierra's transplant, and Gage three years later.)

“Six years ago, there were not really any ways of testing for those genes,” said Dr. Daniel Bernstein, a pediatric cardiologist at Packard treating the Bingham children. “Even today, the best genetic testing can only pick up about half of the abnormalities.”

There was no reason for the family to suspect that any of their other children were affected. And while Sierra had ongoing brushes with organ rejection, the Binghams thought the world of heart transplants was pretty much behind them.

“I always thought that the Sierra events six years ago was the end of that trial,” Jason said. “Maybe that was preparing us for something bigger.”

In many ways, Sierra's experiences have served as a blueprint for Lindsey. It was more than a year ago that Lindsey started feeling tired and lacking in appetite. Doctors attributed it to stomach issues or asthma, maybe a virus. For months, they treated Lindsey for those conditions, and with each treatment she seemed to get better. But soon, she would decline again, leaving the family searching for answers. Yet all those months, the family never considered that Lindsey could be facing the same issues as Sierra.

“It never crossed our minds, it never crossed the doctors' minds that it might be a heart issue,” Jason said. “It looks so silly now. You would think, 'For crying out loud, you have a kid with a heart transplant.' But then Sierra had such an obscure disease, one in a million, that no way another kid would have that. And maybe there's a little bit of denial going on.”

Then in May, Lindsey woke up one morning with her face swollen. It was a symptom the Binghams had seen before with Sierra. They could deny it no longer. Lindsey's heart was failing too.

“When we saw that, it's like somebody hits you in the gut,” Jason said. Stacy felt sick to her stomach as they took Lindsey to the hospital and requested a chest X-ray. They told the doctor about Sierra and her heart transplant. He returned with the X-rays and asked to hear more about Sierra's heart condition. He confirmed the Binghams' fears. Lindsey also had an enlarged heart.

“Right then everything changed,” Jason recalls. “Now we know it's genetic. Now we know it's our entire family. Now we know the road we're going to have to go down, a road we don't want to go down, a nightmare we don't want to relive. We both broke down right there uncontrollably.”

Desperate to comfort her parents, Lindsey told them, “My tummy doesn't hurt anymore. My tummy doesn't hurt.” It would be the last time they would cry in front of their daughter. They had to be strong for Lindsey.

Treatment begins

The child's denials aside, within 24 hours Lindsey and Stacy flew to Lucile Packard, meeting with the same doctors, the same nurses who had treated Sierra. Doctors prescribed a heart failure medication that would help the heart contract, and Lindsey rebounded. They discharged her days later but asked the family to stay near the hospital so they could continue to monitor her progress.

The doctors hoped they might be able to manage Lindsey with outpatient medications, allowing her to return home. But Stacy was not as optimistic. She had seen the same thing with Sierra, who rebounded for a short while after treatment but soon worsened.

“The doctors told us that every case was different,” she said. “We said, 'That's true, but this is the only thing we have to go on.'”

For two weeks, mother and daughter rode their bikes around the Stanford campus and for a while it seemed like maybe the doctors were right. Lindsey still lacked energy and wasn't eating much, but she wasn't getting any worse. Stacy moved up an echocardiogram test in hopes that they could go home. But the test revealed a blood clot in Lindsey's right ventricle, and doctors readmitted her to the hospital.

The rest of the family came down to Palo Alto, and on June 8, all were tested again. Megan and Hunter both showed abnormalities that doctors believe might be precursors to DCM. Gage's heart, although the youngest of the five, already showed signs of enlargement. Doctors put him on an aggressive medication regimen hoping to postpone heart failure as long as possible.

Jason returned home with Megan, Hunter and Gage. By June 21, Lindsey's condition worsened, and she was placed on the transplant list. Her heart was failing quickly. A week later, doctors told Stacy they wanted to use a Berlin Heart, a device that would help the left side of her heart pump blood. Six years after Sierra, Lindsey was following the exact same path.

Six years, however, allowed for major strides in treating kids with heart failure. Sierra was only a day away from getting a Berlin Heart before a donor heart was found. At the time, the Berlin Heart was considered experimental and required a compassionate-use exemption from the Food and Drug Administration. Even Packard, which has a long history of breaking ground in pediatric heart transplants, had only used the device in a handful of patients.

Packard doctors have used the device with 25 patients, and the FDA approved the device for use in children last year. Without the Berlin, Lindsey might not have made it.

“Until the Berlin Heart was developed, we really didn't have good options for kids her size,” Bernstein said. Other equipment could have kept Lindsey alive a few more weeks, but not the months it could take to get a donor heart. The longer she would have waited, the more kidney, liver and lung damage she would have endured, until a transplant might no longer help.

“Lindsey is right on the borderline of being able to handle another kind of pump,” Bernstein said. “The problem is the pump is made for adults, and so little kids can have trouble with it because it's too big. The Berlin was really a major advance, and thankfully there was a company willing to produce pumps in smaller sizes.”

With the Berlin surgery scheduled for 8:30 the next morning, Stacy spent a long, brutal night by Lindsey's bedside. Lindsey would close her eyes and fall asleep, only to wake up minutes later with stomach pain or vomiting.

“I kept looking at the clock and it's not moving,” Stacy said. “It was like, 'Oh good, I dozed off, it's probably been an hour or two,' and it's been five minutes. 8:30 couldn't get there fast enough.”

Side by side

The Berlin Heart is a mechanical pump that sits outside the body. It's connected to the heart with large tubes that carry blood out to the pump and back into the body. It rests upon the patient's stomach, the clear casing showing the bright red blood pulsing through it.

The lifesaving device carries its own risks, mainly blood clots that develop inside the pump. If those break loose, they could cause a stroke or other serious problems. And once Lindsey was on the Berlin, there was no going back. Her only hope was a heart transplant.

Lindsey came out of the Berlin Heart surgery on the afternoon of June 30, just hours before Jason arrived at the hospital with Gage and Megan. Gage had been taking the medications aimed at preventing his heart from deteriorating but was not tolerating them well.

Stacy took his blood pressure and, finding it extremely low, had the nurses at Packard check him out. His heartbeat was irregular, and they sent him immediately to the emergency room. There, doctors diagnosed a complete heart block, a condition in which the electrical signals to contract don't pass from the heart's upper chambers, the atria, to the lower chambers, the ventricles. Within hours he was admitted to the hospital, and the next day, surgeons implanted a pacemaker.

On July 1, the brother and sister were recovering in adjacent rooms, Lindsey in room 2337, Gage in room 2335.

“What's up with that?” Jason asked, exasperated. “A pacemaker the same day she gets a Berlin Heart? That's just a freak of nature.”

The pacemaker did its job, and within two days, Gage could leave the hospital. Lindsey was up and walking. It looked for a while like the cardiovascular ICU might finally be free of Binghams.

Walking was difficult for Lindsey, but Jason, Megan and Gage did their best to entertain her, playing hide-and-seek in the halls of the ICU, jumping out to scare her when she approached.

“We got her giggling a few times,” Jason said.

If she could regain her strength, walk on her own, eat solid food, she could be moved from the ICU to the cardiovascular wing on the third floor of the hospital.

Doctors and nurses were continually monitoring Lindsey's Berlin Heart to see whether blood clots were forming. To guard against clots, Lindsey was given blood thinners. There's a delicate balance between preventing clots and thinning the blood too much, and when Lindsey's blood got too thin, she started bleeding internally. That led to her right lung collapsing and forced doctors to put her on a ventilator.

She lost valuable ground with the bleeding problem that doctors would have to work hard to reclaim. She stopped eating and couldn't tolerate the feeding tube. Within a week, she lay in bed, a faint wisp of the child that had come to the hospital two months earlier.

“She was 45 pounds when she got here. She was always skinny,” Jason said. “I'm scared to death to ask how much she weighs now. She's pretty sick. It doesn't take much.”

Throughout Lindsey's ordeal, the Binghams have relied on family and friends for support, leaning on their community, their church and each other. Lindsey has relied on her big sister, Sierra, to guide her through the process Sierra can barely remember.

During the two weeks in Palo Alto before she was readmitted, Lindsey had convinced herself she wouldn't need a heart transplant after all. Stacy refused to let her believe that.

“Oh no, you are. You are going to have a transplant,” she told her. “It might not be this year, but that's the reality.”

Lindsey broke into tears and refused to talk to her mother about it. It was Sierra that calmed her down. The girls talked on the phone and Sierra comforted her sister, telling her they could schedule their biopsies together, like they were planning on haircuts or pedicures.

“Sierra wants to be here really bad,” Jason said. “She relates so much to what is going on, she wants to be here.”

Before Lindsey went in for a cardiac catheterization, a procedure in which doctors test the pressures inside the heart, Sierra gave her three pieces of advice: Be brave, hold the nurse's hand and ask what is going to happen next.

The long wait

Megan, the middle sister, does her best to comfort Lindsey as well. If she's concerned about her own heart, Megan doesn't let on.

“I'm worried (about Lindsey),” she said. “It pains me to see how frustrated she is sometimes, like when we don't understand what she's saying because she can't talk. She gets frustrated.”

At other times, there is no one who will do, other than her parents. When the phone rings in the middle of the night, the Binghams know Lindsey is having a rough night. They leave their clothes set out, so they can get there quickly.

Every telephone ring sets off panic alarms for Jason and Stacy. The call about Sierra's donor heart came at 1 a.m. six years ago, and for a moment, they had braced for the worst news. Now every time the phone rings at the Ronald McDonald House where they are staying, Stacy says her heart skips a beat.

Waiting times for a heart for someone Lindsey's size average two to four months. Sierra waited only three weeks. Other kids wait two years.

“The time waiting for a donor heart to become available I think is the worst, the hardest to deal with,” said Mary Burge, a transplant social worker at Packard who has worked with the Binghams since Sierra's transplant. “There's no light at the end of the tunnel. You don't know how long the tunnel is, and then you're facing a whole other set of challenges — big challenges — but at least that phase is over.”

Even if Lindsey gets a heart transplant, she'll still face a lifetime of testing and the risk of organ rejection. The Binghams said they have good insurance, although it doesn't cover all their medication costs or the nonmedical costs, such as travel to and from California. They had already been setting aside money for Sierra's ongoing care.

Sierra is doing well with her heart but has had multiple rejection episodes. Doctors can adjust her medications to dampen the immune system to prevent rejection. They are now monitoring her for coronary artery disease, a narrowing of the arteries that commonly plagues much older patients.

The Binghams are still waiting on the results of their genetic tests to determine what mutations might be causing the heart problems. Doctors have also ordered tests of the well water on the family's land. It's possible that heavy metals such as lead, mercury, arsenic or copper in the water could be damaging the young hearts.

“That would be highly unlikely,” Bernstein said, but worth checking out.

Jason threw out some of the old furniture at the home and tossed the old paint cans they had around, just in case any of those were contributing to the problem.

“As a dad, I've got to do something,” Jason said. “Dads, we're fix-it people. It's your kids, and all your kids are getting sick. You just start throwing things out.”

He's told Lindsey he won't shave his chin hair until she gets a heart transplant. So far it's been 26 days on the transplant list.

“No matter how long it takes, I'm not going to shave it,” Jason said. “Hopefully it won't get too long and ugly.”

Doctors don't know how long Lindsey can stay on the Berlin Heart waiting for a transplant.

“There isn't an artificial time or point where we have to stop,” Bernstein said. “But the risk of complications start adding up.”

Packard kept one child alive on a Berlin for 81⁄2 months.

On Thursday, doctors removed Lindsey's breathing tube and switched out the Berlin pump, disconnecting it from the tubes connected to her heart. The switch takes less than two minutes, while everyone holds their breath.

Bernstein said they've seen other families with multiple siblings with DCM. They've transplanted two siblings before, even a father and son. But doctors doubt anyone has ever transplanted five siblings.

“We have other families, some from many years ago, where in the early days of transplant, some kids didn't make it to transplant,” he said. Tools like the Berlin Heart, however, can now buy families extra time for a donor heart to become available.

But the waiting takes its toll on Lindsey, Stacy and Jason. Stacy said she's surprised at how exhausted she is at the end of a day in which all she did was sit by Lindsey's side. Jason is haunted by the sight of other families going about their daily business.

“I saw kids and families walking around, biking, shopping, playing, fighting, and just living their lives,” he said. “They have no idea what a blessing that is. That seems like such a fantasy.”

A heart transplant would fulfill a lot of fantasies, offering a chance to resume a normal life, to go home, to go back to school, to resume the family vacations. Lindsey has told her parents she has one wish after her transplant.

She wants all four of her brothers and sisters there by her bedside when she wakes up.

— Reporter: 541-617-7814

mhawryluk@bendbulletin.com

Heart transplant costs

A recent analysis by researchers at Seattle Children's Hospital found that hospital charges for a pediatric heart transplant in 2006 averaged $451,738 nationwide in 2006. These costs included all of the pre-transplant care, the transplant surgery, the organ procurement and the in-hospital recovery.

According to the magazine Nursing Spectrum, the use of a Berlin Heart adds a minimum of $92,000 for the device and materials needed to implant it. The drivers that power the device are rented at a cost of $15,000 per month. If the pump needs to be replaced due to blood clots, the cost is $37,000.

After the transplants, patients incur ongoing costs for medications, biopsies and other tests. A 2006 analysis by researchers at Emory University School of Medicine found that patients pay on average $18,480 per year.

From diagnosis to transplant

1 Dilated cardio-myopathy is diagnosed through echocardiogram, a sonogram of the heart.

2 Initial treatment involves medications that help the heart muscles contract more strongly.

3 If the condition worsens, it can lead to heart failure, where the heart is not able to pump adequate amounts of blood to the body. Symptoms include shortness of breath, fatigue, swelling of the feet and legs, and difficulty sleeping flat in bed.

4 If heart failure becomes severe, the patient may be placed on the transplant list.

5 Wait times for a heart transplant vary but can last for months. If doctors can't slow the rate of heart failure before a donor heart becomes available, they may turn to other technology, including the Berlin Heart, to keep a patient alive.

6 Once a donor heart is found, one team of doctors will prepare the patient for the transplant, while another team gets the donor heart. The two teams work in coordination to minimize the time the donor heart is outside the body.

7 Following transplant, the patient will take drugs to suppress the immune system to prevent organ rejection. In the first 30 days, infection is the greatest risk.

8 The patient will need to get regular biopsies, at least every four months, to monitor for organ rejection. If the biopsy shows any level of rejection, doctors can increase the immuno-suppressive drugs.

9 Donor hearts can last indefinitely, growing with the child. But many hearts will not last more than 15 years, so patients often face a second transplant.