In an Internet message to family and friends on July 12, Leigh Bills described her 3-month-old son, Miles Coulson: "He is very pale, almost gray and his extremities are cold. We sit and hold his little hands but as soon as we let go they get so cold."
"All the doctors agree there is no time to waste," she wrote.
Miles needed a heart transplant. Though he was born healthy to Ms. Bills and her husband, Adrian Coulson, his heart began to fail when he was only a few weeks old, possibly because of a viral illness. By late June, he was on the transplant list.
But donor hearts from infants are so scarce that doctors feared Miles would not live long enough to receive one. Older children and adults waiting for transplants can be kept alive by mechanical pumps implanted in the chest, but none of the pumps approved in the United States is small enough for an infant. There has been little incentive for companies to develop pumps for babies, experts say, because the market is not large enough.
In a desperate move to save Miles, his doctors sought a device they had never tried before: a miniaturized pump called the Berlin Heart, which has been widely used in Europe but not approved in this country.
"I don't think there was any other prospect for keeping him alive," said Dr. David Rosenthal, director of the pediatric heart failure program at Lucile Packard Children's Hospital, at Stanford University.
On July 8 and 9, Dr. Rosenthal asked Stanford's ethics panel and the Food and Drug Administration to let the hospital import the Berlin Heart for Miles. Both agreed, within hours. The device was flown to the United States from Germany on July 12, accompanied by Dr. Peter Göttel, a heart surgeon who works for the pump's manufacturer in the city for which the heart was named. Surgery to connect Miles to the device was scheduled for the next morning.
The history of Miles Coulson's illness is a reminder that the boundary between health and sickness is fragile, and a family's fortunes can turn swiftly from joy to sadness. It is also a story of parents pinning their hopes on a piece of medical technology, wondering whether it will save their child's life or merely prolong his suffering.
The number of infants who might be saved by pumps like the Berlin Heart is not known, but is probably in the hundreds, said Dr. Tracey Hoke, a pediatric cardiologist and medical officer at the National Heart, Lung and Blood Institute.
"It's definitely an orphan product," Dr. Hoke said. "It's a tiny market, but high profile. These are people's babies. It won't help a lot of people, but it will help a few people a lot."
She said that the institute, hoping to fill the gap in the United States, awarded five contracts in April, with a total value of $22.4 million, to companies and universities to develop heart-assisting devices for infants. But it will take five years before any is ready even for testing in humans. And Dr. Hoke warned that even if more pumps did become available they would not solve the organ shortage and some infants might die anyway. In those cases the pump may just give families more time, she said, "to cope and lose their child."
Dr. Göttel said that the Berlin Heart was made in a range of sizes, and since 1989 had been used in more than 950 people, including about 50 babies in Europe. The longest an infant has survived on the pump is three months, he said.
Over all, Dr. Göttel said, about two-thirds of patients who receive the pump survive, going on either to recover or receive a transplant.
An F.D.A. Application
He said the company is preparing an application to the Food and Drug Administration for a "humanitarian device exemption," which is not a full approval, but a special arrangement for devices for which there is no alternative. The exemption would allow hospitals to keep the heart pump in stock - which they are not permitted to do now - and use it when needed without having to request special permission and import it every time.
Three other children in the United States have been treated with Berlin Hearts. Two received transplants and a third recovered, so the pump was removed.
Dr. Mark Turrentine, who treated two of the children at Indiana University's Riley Hospital for Children, said that in similar cases, he would definitely use the Berlin Heart again. He said that heart surgery for children was a decade behind that for adults and "this is something that helps bridge that gap."
Born on March 30, Miles was healthy at first, robust even, at 11 pounds 6 ounces and 221/2 inches in length. He came down with a viral illness in mid-April, a seemingly minor one that had also affected his mother and 2-year-old brother. Miles went on to develop heart failure. The reason is not known, though in rare cases viruses and other infections can attack the heart.
Initially, Miles was treated at a hospital in Sacramento. His heart was so weak that, to keep him alive, doctors connected him to a large device nearly identical to the heart-lung machines used in the operating room to take over the work of a patient's heart and lungs during heart surgery. He stayed on the machine, called ECMO, for extracorporeal membrane oxygenation, for eight days.
Back home, he seemed stable for a while, but his heart continued to deteriorate. His lungs weakened as well, and he needed a respirator. On June 12, he was flown by helicopter to Stanford, where doctors soon concluded that his heart was unlikely to recover, and he would need a transplant - if he could survive long enough to receive one. They sought the Berlin Heart to keep him alive rather than turning to ECMO, because it cannot be used for more than a month or so; patients' rates of bleeding, clotting, infection and organ failure are too high.
Struggle Over Suffering
Ms. Bills, 33, who works with crime victims for the California Office of Emergency Services, and Mr. Coulson, 37, a public school music teacher and band director, said they had struggled to decide what would be best for Miles. At times, especially on the oxygenation machine, his suffering seemed unbearable. Sometimes, Mr. Coulson said, they saw his face contort as he cried silently, unable to make a sound because of the tube in his throat.
Ms. Bills said: "There were times when I thought the best thing would be for Miles to pass. Adrian said, 'Whatever's beyond this life has to be better than this.' "
"We said goodbye a few times," Ms. Bills said.
They swung between hope and despair, and did not always agree whether to continue treatment. They decided if one wanted to continue, they would, Ms. Bills said. Otherwise, she said, there would be regrets, and one might resent the other for having given up too soon.
Lucile Packard Children's Hospital provides a Web site that parents can use to post messages about their children's health for family and friends, and Mr. Coulson and Ms. Bills have in essence recorded Miles's life story on it.
July 11, Adrian: For a few weeks there has been talk of a device called the Berlin Heart. This was presented to us as a possible method of keeping Miles alive long enough to get a donor heart. The problem was this device is not F.D.A. approved and has only been used in the United States a handful of times, never here at Stanford. We received word Friday that F.D.A. has approved this one-time use on the basis of compassionate care. They have tentatively scheduled Miles for surgery on Tuesday.
The feeling is that although there is great risk with the implantation of this type of device they cannot wait for Miles to get any worse before getting him on it. We are both very nervous but hopeful this will keep Miles going until he gets a heart. In case you are wondering there is no way to know how long we'll have to wait.
In a 21/2 -hour operation on July 13, surgeons connected Miles to a Berlin Heart. Before the surgery, his limbs were cold and his skin a dreadful bluish gray, because his heart was pumping so little blood. He came out of the operating room pink, to his parents' delight.
Dr. Bruce Reitz, one of the surgeons, said, "It's exciting, to do it in a baby like this after not having this option before and seeing that it really could work; that was exciting for our team."
Best Feature of Device
The beauty of the Berlin Heart is that it does not have to be jammed into a baby's tiny chest. The bulk of it, including the pump itself, is outside the body. Only tubes are implanted. One collects blood from the left ventricle, the heart's main pumping chamber, which is not working properly. The blood flows into the pump, which pumps it through another tube into the aorta, the main vessel that feeds the rest of the circulatory system.
The two tubes emerge from small openings on the left side of the abdomen and enter the pump, a small, round chamber with a clear window. It rests quietly on the lower abdomen. It is driven by compressed air, and so the pumping chamber is connected by another tube to a compressor about the size of a small filing cabinet. The system is run by a laptop computer.
Miles's pump, which holds about two teaspoons of blood, is set to beat 100 times a minute.
All patients with mechanical heart pumps face risks; the greatest are linked to bleeding, clots and infection. Whenever blood flows through foreign materials and artificial valves and chambers like the ones in a heart pump, it tends to form clots, which can be disabling or fatal if they reach the brain. Drugs can prevent clots, but also increase the risk of bleeding, and so doses must be fine-tuned to keep a patient on the thin line between two potential disasters.
Miles has had bleeding, clots and an infection in his bloodstream. At times, his incisions kept bleeding long after they should have stopped, and he needed several transfusions a day. When clots occur, they form inside the heart pump and are visible in its clear chamber, especially with a flashlight. Doctors and nurses check for them regularly, hoping they will dissolve gradually rather than breaking off in clumps that can lodge in blood vessels in the brain and cause a stroke.
Twice Miles has had neurological problems, possibly related to clots. Once, he became weak on one side, and another time his gaze seemed to be fixed to one side.
The symptoms went away, and CT scans showed no brain damage, but the episodes troubled his parents and doctors.
'A Sinking Feeling'
"It was awful," said Dr. Stephen Roth, director of the cardiovascular intensive care unit at the children's hospital. "We had a sinking feeling, to think we were causing more damage."
He went on, "All this would be for naught if we couldn't deliver him to transplant with good central nervous system and organ function."
July 16, Leigh: a family we know came in and told me the doctors just told them they think they have a liver for their son! So, it does happen. I am overwhelmed with emotion for them; I can only imagine how I will feel when we get that call.
July 18, Leigh: Maybe tomorrow will be the day a heart becomes available for Miles.
One thought that Miles's parents dread is that something could go wrong, making a transplant no longer possible, and leaving them no choice but to turn off the pump.
"We talked to the doctors about what his death would be like," Ms. Bills said.
They were relieved to hear that it would come almost instantaneously when the pump stopped, she said. They talked, too, about the possibility that Miles would die during a transplant operation. In that case, he would be sedated and would not suffer.
"They said they would clean him up and let us hold him," she said.
When Ms. Bills arranged an extended leave from her job to take care of Miles, she told colleagues she would return sooner if he died. Some tried to reassure her that would not happen. But, she said, she and her husband must face the possibility that their son will die.
"We're inundated with it," she said.
It is impossible to say when a heart might become available for Miles.
"It could happen in one day," Mr. Coulson said.
"Or never," said Ms. Bills.
The last three infants of Miles's size who received heart transplants at Stanford waited 10, 20 and 200 days, Dr. Rosenthal said.
"We've certainly had babies die waiting," he said.
One thing in Miles's favor, he added, is that in babies younger than 18 months, blood type does not matter. Any heart the right size will do, and Miles is at the top of the regional transplant list. But the area from which the transplant can come is limited by travel time: hearts are short-lived compared to livers and kidneys, and even on ice last only four to six hours after being removed from the donor.
Infant organ donors are few and far between. Many donors are babies who are brain dead as a result of child abuse, said Mary Burge, a social worker at Lucile Packard Children's Hospital.
Leigh, Aug. 12: It is getting harder and harder to hope. I feel stranded in sadness and the only way out is the tragic gift of a stranger.
For now, Miles is stable. He no longer needs a respirator and is free to suck his fingers like normal babies. He is lively, bright-eyed and often smiling, and has begun to reach out and swat at the toys that hang over his bed. He is in perfect condition to receive a transplant, doctors say.
Ms. Bills said, "Right now we just need a heart."
ALO ALTO, Calif.
Published: August 22, 2004
New York Times