When the new year dawned, Rachel Robinson and Bill Thompson were looking forward to the birth of their twins in March. Like all expectant parents, they imagined how their lives would change. They anticipated a double dose of diaper duty and midnight feedings in the kind of fractured schedule that blurs nights and days. But they weren’t prepared for what happened in mid-January, when their twin girls were born eight weeks prematurely, one with a life-threatening heart defect. Suddenly they found themselves in a Mobile, Ala., hospital, in a twilight world between birth date and due date, full of scary technology and questions with no clear answers, sitting frighteningly close to the line between life and death.
One of the girls, Callie, improved quickly and was off her respirator in a week, then out of the hospital in a month. But Emma was not doing well. Her heart had developed without a valve and an artery needed to carry blood to the lungs. Steroids could keep open the fetal circulatory system for weeks—perhaps months—allowing some oxygen to get into her blood, but eventually even that would shut down.
A frantic search of major research hospitals throughout the East Coast and the South yielded only rejections—no cardiac surgeon would risk operating on a 500-gram preemie (just over 1 pound). “Tulane said she had to get to 1,400 grams,” Rachel says. She and Bill passed anxious weeks with hope but little advice from the doctors—until a visiting cardiologist remembered reading a paper about Stanford surgeons performing heart surgery on a 600-gram infant. Soon a private jet was rushing Emma 3,000 miles west.
Rachel and Bill joined the more than 1,500 families who come to the neonatal intensive care unit (NICU) of the Lucile Packard Children’s Hospital each year. They enter not eagerly, but gratefully. In the process, they get an up-close view of one of the country’s fastest-growing medical specialties, in a unit that has long pioneered pediatric research. Under the leadership of newly recruited pediatric cardiac surgeons Robert Hanley and V. Mohan Reddy, Packard Children’s has become the only hospital in the country that will perform heart operations on babies as tiny as Emma.
There is a rising demand for neonatal care for premature infants, partly because there are more of them. Multiple births have always carried a high risk of premature delivery, and today’s sophisticated fertility treatments have increased multiple births. But the main reason doctors are now treating many more preemies is that they can.
Research over more than three decades has pushed back the limits of survivability for babies born far before nature intended, including those with other medical problems, like Emma’s heart malformation. More effective ventilators, a better understanding of drugs and new therapies are among the advances. As a level-3 facility, Packard Children’s Hospital can provide long-term care with advanced technologies like the neonatal heart/lung machine called ECMO (extracorporeal membrane oxygenation), which can keep babies alive when their lungs cannot deliver enough oxygen.
“When I was a resident, babies who had gone through 25 to 26 weeks of gestation did not have a good chance of survival,” says Louis Halamek, a physician in the Packard NICU. “But now their chances of survival are 60 to 70 percent.”
April 19. Emma’s admission to Stanford comes just in time. “They [the doctors in Mobile] called us and told us she was getting worse fast,” Rachel recalls. “They used some terrible word like she was ‘decompositing’ or something.” Bill and a nurse speed Emma to the Mobile airport, where a Learjet is waiting to take them to California. Throughout the flight, Emma’s oxygen saturation keeps falling critically low, even though she is breathing pure oxygen. She makes it to Stanford—but once there, her heart stops, and doctors have to perform cardiopulmonary resuscitation for 10 minutes to bring her back.
Meanwhile, Rachel, Callie and Bill’s parents have taken a commercial flight to San Francisco. Only when they get to Palo Alto do they learn how close Emma came to dying.
In many ways, the world they enter at the children’s hospital is similar to the one Emma left in Mobile. In the NICU, tiny infants are arrayed around a ward, ensconced in clear plastic boxes called isolettes or cradled in open beds where they can be exposed to lights that warm them or treat jaundice. The youngest babies are connected to vermicelli-thin tubes that deliver medications, hormones and nutrients, and to colored wires that carry out information about their vital signs to banks of computer-operated machines at their bedsides. The infants seem living jewels in a high-tech setting. And although they are exceedingly young, the absence of baby fat normally put on in the last weeks of gestation gives them some resemblance to wizened old men and women.
By this time, the difference between Emma and Callie has become marked. Although she and her sister have been out of the womb for 12 weeks, Emma still looks thin and small like the other premature newborns. Callie is completely swathed in baby fat and has grown to the size of most other 3-month-olds. The twins’ parents and nurses also note personality differences. Callie is easygoing and spends a lot of time sleeping or quietly observing her world. Emma is “feisty,” says her mother. “She lets you know how she feels; she gets mad and her arms and legs turn red and flail. Every nurse who has had her remarks on how ‘bad’ her attitude is—which is good, because she has to fight for everything she has.”
The Stanford doctors decide not to schedule surgery until Emma has gained a little more weight. The stress of waiting magnifies personality differences between Rachel and Bill, too. Rachel tends to take things harder, Bill says, while he is more matter-of-fact. “I just feel that the way it is, is the way it is,” he says.
But Rachel observes that her husband, a veterinarian, is also burdened by the extra knowledge his medical training provided. “He sits by Emma’s bedside for hours watching the machines, because he knows what they mean,” Rachel says. One day he notices that Emma’s heart rate has risen slightly; after he alerts the doctors, they find the beginning of a small infection where an IV enters her skin. The infection is easy to treat, but it delays surgery further.
April 29. With the surgery postponed, Bill must return to Alabama to keep his practice running. He has no partner to cover for him, and he can’t put his clients on hold for weeks without losing them, especially since there is a competitor in their small town. Rachel and Callie stay on at Ronald McDonald House, a godsend for parents awaiting pediatric surgery. The house provides accommodation near the hospital and an opportunity to meet and commiserate with each other. It also eases their financial burden, however slightly—parents pay just $10 a day, and charitable organizations provide nighttime meals.
Though Rachel is not alone—her in-laws have remained in California—she is now in sole charge of her daughter’s care. Wherever she goes, Rachel carries a spiral notebook crammed with writing. “This is Emma’s baby book,” she says wryly. “I scratch out notes on everything, and then in the evening I copy it all over into another book and we talk about what questions to ask.”
For Rachel and most parents around the NICU, the experience is a crash course in medicine that they never signed up for. Shannon Rohrer, a social worker in the unit, eases their way through the bewildering medical maze and helps them coordinate between doctors, nurses and hospital administrators. “Shannon is a miracle worker,” Rachel says.
Rohrer also tries to help families deal with the emotional trauma of merely being in the ward. She points out a father visiting for the first time, looking unsure where to stand, his face tight, lips pressed thin and bloodless. “For most people, it just rocks their world to be here, because this is the opposite of what they imagined their birth experience would be like,” she says.
One of Rohrer’s hardest jobs is to help parents let go when it looks as if a baby won’t make it. Since doctors at Stanford are dealing with the smallest, sickest infants, it is not uncommon for operations like the one scheduled for Emma to fail, or for babies to be born so young they simply can’t survive. Infants born at about 22 to 23 weeks of gestation (a full term is 40 weeks) are generally considered at the outer limit of survivability. Their tissues are so fragile that they are prone to uncontrolled bleeding in the brain, and their skin is so thin that fluid easily evaporates out of the body and infectious organisms easily move in.
In such cases, physicians like Halamek discuss the options and the odds of success. “When a baby is born after only 22 to 23 weeks of gestation, the odds of survival are only 5 to 10 percent,” Halamek says. If things are going really badly for the baby, the doctors have to bring up the possibility of not resuscitating if breathing ceases or the heart stops. In cases where survival odds are very low, or if bleeding in the brain will cause permanent damage, doctors work with the family to decide when to stop taking extreme measures to prolong life. “We take an individual approach with each patient and each family,” Halamek says. “Frequent communication is the key.”
The odds of survival rise significantly for babies born after a slightly longer gestation. Catherine Nink was helicoptered to Stanford from Santa Cruz, Calif., when her water broke in the 25th week of pregnancy. She gave birth to her daughter, Fiona, in a normal delivery on April 13, but that has been the last normal experience so far. “Right after her birth, I remember hanging over the bed trying to get a glimpse of her, and then she was intubated immediately and taken down to the NICU,” Catherine says.
Despite the 1-in-3 chance Fiona would not make it, her mother says, “I just knew I would be bringing her home.” Her hopes were bolstered when Fiona was able to come off a ventilator after five days. “They expect babies this young to be on the ventilator for three weeks,” she says. Though things are going well, Fiona Nink will have to stay at Packard for months to come. “They have a book of ‘graduates’ in the parent’s lounge, and I just keep looking at that book and the date they go home, which is usually around their due date,” Catherine says.
Early May. Emma’s surgery is at last scheduled for May 8. The prospect of bringing both sisters home to Alabama seems more real than ever. But first Emma will have to come through a difficult, risky operation.
Reddy will be Emma’s surgeon. “I love working with children,” he says. “Each case is different and challenging—and when you do a good repair, a child has a whole life ahead of them.” Reddy and fellow pediatric surgeon Hanley were recruited at the same time from UC-San Francisco. Reddy says they were drawn to Stanford by the vision of making Packard Children’s one of the top two or three pediatric centers in the country. The 42-year-old surgeon has a calm and calming air about him, but he has a reputation for intensity and dedication. He is excited about meeting the challenges of the demanding surgical procedure.
May 7. Bill flies in from Alabama. The hospital staff stops feeding Emma that night in preparation for surgery at 6:30 the next morning. Bill and Rachel also get ready, hoping for the best but preparing for the worst. They follow Rohrer’s advice and sit with Emma, thinking about the fact that she might not survive the surgery, that this might be their last night with her, and telling her all the things they would want to have told her if she didn’t make it. They don’t sleep at all.
May 8. In the dark hours of the morning, Bill and Rachel learn that the surgery will be postponed until later in the day because of a shortage of space in the recovery rooms. By 6:30 they learn that the operation will not take place that afternoon either, for the same reason. Surgery is rescheduled for the next morning. The family tries to get some sleep.
May 9. Arriving at the hospital at 5:30 a.m., Bill and Rachel learn that surgery is again delayed until the afternoon, this time because of an overbooked operating room. Later, it is put off again, because there is still no time slot for the operation. Shannon Rohrer lets the doctors know that the family is “going crazy,” and Reddy promises to do the surgery at midnight that night, if necessary. But his contingency plan isn’t needed: a spot opens up in the operating room the next morning.
Then a horrible complication arises. Rachel learns that their insurance won’t pay for the operation. She and Bill go downstairs to talk to the hospital’s financial people and find out that Packard has known this from the beginning but didn’t want to burden them with the information. Coverage was denied on the grounds that surgery could have been done closer to home, but once the insurance company verifies that no one else in the country would do the procedure, it relents and agrees to pay.
Still, the medical costs will be huge. Before Rachel and Bill are through, they will have exceeded the $2 million lifetime cap imposed by the insurance company and will turn to Medicaid to make up the extra. Hospital costs can run over $10,000 a day in the NICU, and with surgical costs on top of that, the sums grow quickly. Still, Rachel says her attitude is “fix my daughter first, and then we’ll worry about paying for it.”
May 10, 9:30 a.m. Bill and Rachel walk with Emma as staff wheel her down the hall to the operating room. Then the couple lets her go as the nurses take the gurney through the double doors. They camp out in the hallway with blankets and pillows they have brought. When they aren’t taking turns going to the cafeteria, they sit anxiously eyeing their pager. If all is going well, they will hear nothing until that afternoon, when they will be paged about 45 minutes before the end of surgery.
In the operating room, Reddy and his research fellow, Malcolm MacDonald, prepare Emma for surgery, securing monitoring lines and, after anesthetizing is induced, cleaning the surgical field on her chest. The operation begins like any open-heart surgery—they cut through Emma’s sternum and spread the ribs to provide room to work, then stop her heart while a heart/lung machine keeps her alive. But this surgery is far different from others because Emma’s chest is only a couple inches across, and the opening in the rib cage is far smaller. Her heart is the size of a thumb. With binocular magnifying lenses fitted over glasses, Reddy looks like a jeweler preparing to cut the most precious stone in the world.
Guiding the surgical instruments with only his hands and his magnified vision, he cuts into the heart to close up a hole between its two chambers that shouldn’t be there. He also has to create a space for a new heart valve and attach it and a new artery that will carry blood to the lungs.
3:30 p.m. After six hours of minute, painstaking work, the surgical team successfully completes the hardest part of the operation. They start the heart and observe it beating normally, squeezing blood up through the lungs, back to the heart and out into the body.
They page Emma’s parents and begin closing up. Often the tissue swelling after such extensive surgery requires the doctors to wait a few days to completely close the chest, but the surgery has gone so well that Reddy decides to knit the chest up now. Then a problem occurs in the operating room before Emma can be moved to the ICU. Her lungs are stiffer than expected, and she has trouble with ventilation. The team brings Emma back onto the operating table, where they reopen her chest to give the lungs room to expand. This procedure and stabilizing her takes another two hours.
6:30 p.m. Having heard nothing since 3:30, Bill and Rachel begin to think something is drastically wrong. Finally, they learn about the second trip to the operating room and that Emma is doing fine. She moves to the cardiovascular ICU at Packard, and in the critical night after surgery, her parents finally get a fitful sleep in the lounge next door. Rachel is grateful and impressed that Reddy sets up a recliner at the foot of Emma’s bed and spends the next two nights there. “He was doing everything—tweaking machines and tapping IVs, messing with drainage tubes, doing things that you would normally see nurses do,” Rachel recalls.
May 11. By afternoon, Rachel feels confident enough to go shopping for a present for one of Emma’s nurses, Linda Torres, who has been especially kind. “I just needed to breathe,” she says. As Rachel starts to relax a little, she realizes how far away she has been from everyday concerns. By the end of May, she says, “I called a friend at her home and she mentioned that a girl from down the street was over, and I said, ‘Shouldn’t she be in school?’” Rachel was surprised to hear that school was out for the year. “It’s like we’re still in January, like we’ve been in this totally different world,” she says. “We might as well have gone to Mars for four months.”
BOTH EMMA THOMPSON AND Fiona Nink finally go home the last week of July. Rachel and Bill bring a 4-pound, 10-ounce Emma to the long-empty crib in Brewton, Ala., from the Mobile hospital she transferred to in early June. Seven-pound Fiona makes the short trip from Palo Alto to Santa Cruz. Emma will need two more heart surgeries, one before she is 5 and another when she is a teenager. Fiona’s doctors have done an MRI to spot any bleeding in the brain, but one of them says Fiona “has the best-looking brain I’ve seen in a long time.” Her eyes are still weak but healing, and she has to stay under lights around the clock to stimulate blood-vessel growth. “But she’s breastfeeding really well,” Catherine Nink says, “and that is extremely important to me.”
Catherine sums up the ambivalence many parents feel about the twilight world they’ve emerged from. “I have spent some of the worst days in my life in this hospital, so I don’t have a warm feeling when I walk in,” she says. “But my husband feels some wonderful things have happened here, and he probably brings me to somewhere in the middle.” She isn’t conflicted about the research that leads to the NICU’s successes, though. “Sometimes I sit and marvel at who figures these things out, the research that they do.”
Christopher Vaughan is a science writer based in Menlo Park.
