Paige's recovery began in the intensive care unit at Children's Hospital of Wisconsin in Wauwatosa. To keep swelling down, her chest remained open after the surgery, covered by a thin sheet of medical material. Doctors closed the wound two days later.
Her parents, Jim and Gina, soon would become fixtures in the ICU. They spent most of their days there bonding with their daughter and reading her books such as "Peter Rabbit" and "Walter the Farting Dog."
They had pizzas delivered to the hospital and went to nearby Mayfair Mall to kill time.
As a paramedic, Jim felt at home around monitors and medical flare-ups. Gina acted like her very presence in such situations could only make matters worse.
When Paige aspirated food and a doctor needed to suction it out, Jim was right there wiping off the baby and ready to jump in and perform the procedure himself. Gina jumped to the back of the room.
For about two weeks after the surgery, Paige's recovery seemed to be going as expected.
In early March, she began having problems breathing.
Doctors first tried to circulate warm, moist air around her. Then they used a device that forced air into her nose. But fluid was building up in her right lung. She was put on a low-fat formula and a tube was inserted through her chest into the lung.
About two ounces of a fatty, yellow substance eventually drained off.
After a few tense days, Paige started to breathe normally and the crisis passed, but Gina remained wary.
"I'm really goofy about holding her now," Gina said. "I'm just nervous about it."
Wide-eyed and alert in her chrome crib, Paige wrapped her tiny hand around Jim's little finger. She would not let go.
"This is the most content I've seen her," Gina said. "This is really promising."
Refining procedures
Children's Hospital of Wisconsin began performing the complicated surgery for hypoplastic left heart syndrome in 1992. Babies with the condition, which occurs in four out of 10,000 births, essentially are born with half a heart. Without the surgery, they die within a matter of days or weeks.
"Solving this problem was the Holy Grail of pediatric heart surgery," said Pedro del Nido, chairman of cardiac surgery at Children's Hospital Boston, the center that pioneered the surgical technique in the 1980s. "It's been rapidly evolving."
The surgery used today is done in three stages - an operation a few days after birth; another at four to six months; and a third at about two years. As doctors refined the process through the 1990s, Children's Hospital in Wisconsin saw its survival rate increase to more than 40%. But that meant that nearly 60% of babies still were dying. Doctors searched for ways to improve survival, as well as the quality of life the children would experience years down the road.
They eventually hit on a couple of medical management techniques - one used during and immediately after surgery; the other after the babies go home - that improved survival to more than 95%.
"No one has better survival in the world than we do," said Nancy Ghanayem, a critical care physician at Children's Hospital of Wisconsin.
Doctors at other hospitals acknowledge Children's Hospital's effectiveness, but say the techniques pioneered at the hospital have yet to be subjected to the kind of rigorous scrutiny that could prove their effectiveness.
Some also suggest that the hospital's success is the result of operating on a higher percentage of low-risk babies, a contention the hospital rejects.
Either way, the hospital is making a reputation for itself.
"They probably do have the lowest mortality rate," said del Nido, a professor of surgery at Harvard Medical School who got his medical degree at the University of Wisconsin Medical School in Madison. "They have focused on this disease and this approach for many years."
The breakthrough that dramatically improved survival and led to the operating techniques used on Paige came several years ago. Doctors were experimenting with a device that is placed in a major vein to measure the oxygen level in blood returning to the heart. Prior to that, doctors had measured oxygen saturation in blood coming out of the heart.
The new technique gave them a better idea of how much oxygen actually was in blood getting to tissues and organs.
The technique, along with the use of the drug phenoxybenzamine, a powerful blood vessel dilator that helps balance circulation, resulted in survival between the first operation and the second increasing from 44% to more than 81%.
"It was the thing that really changed everything else," said James Tweddell, chief of heart surgery at Children's Hospital.
George Hoffman, chief of anesthesiology at the hospital, recalled how he and Tweddell were in the operating room in 1996 when they first discussed the possibility of using the oxygen monitoring technique, which had been used in adult surgery.
"We started doing it, and it worked, so we kept doing it," said Hoffman, an associate professor of anesthesiology and critical care at the Medical College of Wisconsin.
It would take six more years before they had the data they needed to support their belief. In 2002, Tweddell, Hoffman and several other doctors at Children's Hospital and the Medical College published their findings in Circulation, a journal of the American Heart Association.
'Not thriving'
At the end of March, Paige took a turn for the worse. An X-ray showed that her heart was enlarged. Her heart rate was elevated.
"They don't know why and they don't know why it happened so fast," Jim said.
After a series of diagnostic tests, it appeared that her heart was sending too much blood into the lungs and not enough out to the body.
"Because too much blood was going to the lungs, there was more strain on the right ventricle," said her doctor, Stuart Berger, a professor of pediatric cardiology at the Medical College and medical director of cardiology at Children's Hospital.
As a result, she was breathing faster and she was not eating enough, even with a new feeding tube that had been placed in her stomach.
In addition, the artery sending blood to her lungs had narrowed and there was a small leak in the valve between her right ventricle and the right atrium, the smaller chamber that sits above it.
"Paige really was not thriving," Berger said.
The solution was to move up the second operation, which normally is not done until about four months after the first surgery.
On April 18, Robert Jaquiss, the doctor who performed the first surgery on Paige, opened her chest again.
The difficult, eight-hour ordeal lasted until 10:30 p.m. Jim and Gina left the hospital at 2 a.m.
The shunt that was put in during the first surgery was disconnected. Also, a major vein that brought upper body blood back to the heart was disconnected from the right atrium. The vein then was connected to the pulmonary artery, allowing for a kind of passive flow of deoxygenated blood directly into the lungs.
The changes created a more stable circulation, putting less strain on the right ventricle.
Paige had swelling and a buildup of scar tissue and adhesions from the first surgery that made the repairs painstakingly tedious.
"Things were stuck together," said Jaquiss, an associate professor of heart surgery at the Medical College. "It's like taking wallpaper off. Sometimes you have to chisel away millimeter by millimeter without taking off the plaster."
Even more worrisome was the fact that Paige's heart was dilated and pumping at about two-thirds to one-half of the ideal capacity of a single-ventricle heart.
"She's holding her own, but she still is quite ill," Jaquiss said a week ago. "I'm hopeful over time it will get better."
If she recovers as expected, she could go home in early May. If she does not improve, a heart transplant may be her only option.
On Saturday, Paige's condition showed noticeable improvement.
Her breathing tube was taken out, as well as two drainage tubes in her abdomen. On Monday, two drainage tubes in her lungs also came out.
"We are going to start trying to improve her nutrition," Berger said. "She's very comfortable, and things are looking good."
An echocardiogram that will show how well her heart is functioning and whether there are any leaks in her heart is planned for today.
Paige got a shot of morphine Monday afternoon. Still tethered to several wires and tubes, she was placed in Jim's arms so he could bottle feed her. She gazed up at her dad for a while and then closed her eyes and drank a little longer.
She'll be weaned off the morphine over the next week.
"This is good . . . awake and happy," Gina said. "Yesterday, it was awake and cranky."
At-home treatment
Even with the improved survival that occurred at Children's Hospital in the late 1990s, one in five babies still died between the first and second operation. Typically the babies are at home recovering and suddenly go into cardiac arrest or some other extreme state.
More often than she cares to remember, Ghanayem, the intensive care unit physician at Children's Hospital, was the person who tried to save them.
"Some of them died at home," said Ghanayem, an assistant professor of pediatric critical care at the Medical College. "Some of them would die in the hospital after aggressive resuscitation."
Often the problem started out with something that parents might not notice or that might seem minor, such as the baby not eating enough, diarrhea, dehydration or vomiting.
"They would go from OK to near death in very short order," said Ghanayem.
Ghanayem and other doctors and nurses at the hospital decided to try to set up a home monitoring program that might catch the problems before they became life-threatening.
They began giving parents precise table scales that could be used to weigh their babies on a daily basis to detect slight decreases in weight or the failure to gain weight, both signs of potential heart problems.
At the same time, the parents were instructed on how to use a pulse oximeter, an infrared device that rests on the surface of the skin and monitors oxygen saturation in arteries.
The measures appear to have improved survival another 10 to 15 percentage points.
Now other hospitals are developing innovations that could further revolutionize treatment of the condition.
At Children's Hospital in Boston, doctors are experimenting with an in-utero technique.
Between 20 and 23 weeks into the pregnancy, doctors insert a needle through the mother's abdomen, through the uterus, through the baby's chest and into the left ventricle, the heart's main pumping chamber.
Once inside the heart, a balloon is inflated in the aortic valve, allowing the left ventricle to squeeze out blood.
"We are teaching the left ventricle to grow by having it carry more blood flow in utero," said James Lock, chief of cardiology at the hospital and a professor of pediatrics at Harvard Medical School. "We can do it in the cath lab without (open heart) surgery."
So far, the hospital has tried the technique on 30 fetuses with suspected hypoplastic left heart. Seven of them did not need to undergo the full surgical repair regimen after they were born, but they did need less extensive surgery, Lock said.
"My guess is that in 10 or 15 years we'll be doing something very different for this disease," said del Nido. "I have no doubt about it."
Anticipation
As Paige recuperated in the intensive care unit, one of the family's two dogs sat in her nursery back home in the Town of Vernon, eyeing the stuffed animals neatly arranged on her dresser.
The windows were open and a refreshing spring breeze blew into the room.
The changing table was stocked with unopened packages of diapers.
Covering their bases, the parents placed a porcelain guardian angel on the windowsill and, next to it, a baby monitor.
Dozens of shirts and outfits hung in the closet.
"I started getting stuff before we even knew she was a girl," Gina said. "Everything is ready to go." |
