by Sanjay Gupta
With advanced medical equipment and the skills that come with repeated practice, surgeons today can fix life-threatening birth defects with a success rate that was almost unimaginable when the specialty was born in the early 1980s. To take it another step, “fix” may not be the right word. The handful of surgeons who perform fetal surgery are nipping deadly problems in the bud and preventing life-threatening conditions from arising in the first place. In doing so, they blur the definition of birth and of life itself.
The pioneer of fetal surgery in the United States is Dr. Michael Harrison, at the University of California, San Francisco, Children’s Hospital. In a published reminiscence, Harrison writes that he had been nourishing “a crazy idea about fixing surgical defects before birth” ever since his first month of surgical training at Massachusetts General Hospital, in the summer of 1969. 1 “As a green and naive surgical intern, I assisted Dr. Hardy Hendren in operating on a newborn baby with congenital diaphragmatic hernia (CDH) who subsequently died.” In a case of CDH, the abdominal organs are located too high in the chest, blocking the development of the lungs. Harrison wrote, “It seemed obvious to me that the baby died because the lung was too small, and the lung was too small because it was not able to grow adequately before birth, and that the only way to save the baby after birth was to fix the anatomic defect before birth.”
Within days, Harrison told me, he had written a protocol for a program of animal experiments that would test this radical notion in dogs. 2 It would be nearly a decade before he could put that plan into action. In 1978, after finishing his surgical training and a stint doing research at the U.S. National Institutes of Health, Harrison found himself in San Francisco, where a small group of South African expatriates was experimenting with fetal lambs. Soon, Harrison was surgically creating birth defects in the lamb fetuses, watching to see what problems developed and then attempting surgery to correct the problem. As he learned more, he started to work with fetal monkeys; these were more challenging because, like humans, they are prone to preterm birth.
This was a fertile time for research. In weekly get-togethers, Harrison would compare notes with several other physicians who were intensely interested in repairing birth defects. Dr. Mitchell Golbus ran a clinic that scanned for and treated fetal abnormalities. Dr. Roy Filly led a group that was establishing a massive fetal ultrasound unit and tracking what happens to babies who develop problems in utero. The weekly meetings, which take place at UCSF Children’s Hospital to this day, came to be what Harrison calls the “heart and soul” of the fetal treatment program.
For the first procedure in a human, Harrison zeroed in on fixing a condition called fetal hydronephrosis, where the output of urine—in a developing male fetus—is blocked. Urine backs up into the kidneys, causing potentially fatal problems, and for complex physiological reasons the fetus’ lungs also won’t develop. The first operation was fairly simple. Harrison took the fetus, still attached to its mother’s womb, and made a tiny hole in the bladder. He inserted a catheter that could drain the urine directly into the amniotic fluid. He then placed the fetus back in the womb and sewed the incision shut. That modest effort launched a specialty that in cases like the one I saw is bearing fruit as a real, lifesaving miracle.
Fetal surgery is a desperate measure. It’s generally performed only after doctors determine that the fetus would not otherwise survive long past birth. Seen in that light, the surgery is a way to cheat death. But here it gets complicated. When you talk about redefining the boundaries of life, it makes people uncomfortable. Questions about life’s starting point underlie some of the most bitter debates of our time. Many modern religions teach that life begins at conception, at the moment a woman’s egg is fertilized by sperm. But in the legal world, it is argued a person isn’t a person until they are born. The forty weeks in between are an uneasy limbo.
As strong as emotions run around the question, in a way it’s like arguing over angels on the head of a pin. No brand-new developing human could survive outside the womb at the moment of conception or for several months afterward. In the majority of cases, survival outside the womb is impossible until about twenty-four weeks of gestation, but it’s equally true that intervention before that point can mean the difference between life and death. Untreated, it was clear that the heart condition of twenty-two-week-old Anders Wiley would mean almost certain doom. And there I was, watching the delicate surgery that would save his life.
None of these philosophical questions were on the mind of Anders’ mother when she laughingly lay on the table for an ultrasound in an Austin, Texas, medical suite. 3 A physician herself, an ob-gyn, she was taking a turn as a patient at the practice where she worked. Known to patients by her maiden name, Dr. Grogono, Sally Wiley was pregnant with her second child. The first, a boy named Dyson, had been born just nine months earlier. When she’d gone for an initial ultrasound two weeks earlier, Wiley had been deeply anxious. At age thirty-nine, she knew full well that her child was at increased risk for birth defects, most notably Down syndrome. The test, however, put her mind at ease. Everything was normal, or so it seemed.
This time, the ultrasound was just for fun, to see how those little fingers and toes were growing. As her favorite technician swabbed cold gel across her belly, Wiley felt a surge of pleasure, thinking of the new life inside her. Now her friend could see as well, peering at the screen as the black and gray images came into focus in the darkened room. But something was wrong. The friendly cooing turned to quiet. Instinctively, Wiley raised her head toward the screen, but of course, she couldn’t see the details from the table where she lay. Her friend, the ultrasound tech, was still quiet, and then said, “There’s something not right about his heart.”
From there, it all went pretty fast. Wiley looked at the films herself, and then she had to show them to her regular perinatologist, but it was mostly a formality. There was indeed something wrong with the tiny growing heart. A healthy heart would be symmetrical, but in this one, the entire left side was shrunken. A valve releasing blood from the heart was narrow, too narrow. As a result, there was barely a trickle of blood flowing to the left side of the heart. Without blood flow, that side of the heart couldn’t grow. The condition is known as hypoplastic left heart syndrome. No one knows what causes it, whether it’s genetically programmed or somehow caused by some idiosyncrasy of development. Whatever the reason, as the condition progresses, blood flow to the left side of the heart is slowly but surely choked off. A serious case is always fatal.
Wiley was in shock. “It was horrible. I had been so focused on being older and Down syndrome, I didn’t even think about the other things that could go wrong. So when [my technician] said there was something wrong with the heart… .” Her voice trailed off.
Along with her husband Jay, a law student and former political consultant, she was faced with an excruciating dilemma. Her own doctor said there was a specialist in Boston who might be able to correct the condition, but there was no time to spare. If it weren’t already Friday afternoon, he would have her on a plane already. But did she want to go? Even if surgery was successful, there could be massive complications down the road. By twenty-two weeks, the fetus relies on its own growing heart to circulate blood to the rest of the body. Wiley knew her baby’s developing brain might already be injured by a shortage of oxygen. There was no way to guess the extent of the damage. Her doctor raised the possibility of an abortion, but Wiley shot that down. She and Jay had already given the baby a family name—Anderton, shortened to Anders—and he felt like part of the family.
“I’ve always totally supported that choice,” she said, “and I always thought I’d known what I’d do, faced with the possibility of serious birth defects. But when I got the news, at twenty-two weeks, there was no question. It was already a baby. Maybe not legally, but to me.”
That wasn’t all. Ethical and legal concerns aside, surgery would be a risk to both mother and developing child. The conservative course would be to sit and wait. If
the syndrome progressed slowly enough, the heart might still grow; if it grew enough, the defect could be corrected at birth. To go in surgically now, on the other hand, might be deadly. The slightest error, the smallest nick of the uterine lining, could trigger premature labor. If she went into labor, Wiley knew she would lose her baby.
Sally and Jay Wiley felt fortunate in at least one way: A friend whose baby had also had a congenital defect advised them to get it fixed right away. “It was such a whirlwind,” Jay told me. “You’re just living your life, and all of a sudden you’re being told you have to hop on a plane that day. But we decided that afternoon to just go all out and do everything we could to save the baby. To just drop everything and go through with all of it.” By Sunday morning, they were on a plane to Boston.
CHILDREN’S HOSPITAL IS part of a huge medical complex, a maze of buildings sprawled haphazardly across the short streets of Boston’s Mission Hill neighborhood, about a mile from Fenway Park. An overhead pedestrian bridge connects it to Brigham and Women’s Hospital, another huge building on the other side. At Children’s Hospital, the staff will tell you pretty quickly that the first operation to fix a congenital heart defect was performed here back in 1938 by a surgeon named Robert Gross. 4 Another first took place here in 2001, when a team led by Dr. Wayne Tworetzky became the first in the United States to correct a heart defect in utero. At the time, Tworetzky called it “the science fiction procedure.” 5
Another first took place here in November 2005, almost exactly a year before the Wileys arrived. This time the desperate parents were a couple from Virginia, Jay and Angela VanDerwerken. Angela, thirty weeks pregnant, had just learned that the baby had hypoplastic left heart syndrome. She faced a stark choice: because the pregnancy was so advanced, it would have to be open-heart surgery. Even then, doctors estimated that there was only a 20 percent chance of fixing the problem; on the other hand, the condition was so severe that leaving it alone for ten more weeks was a death sentence. Undaunted, Tworetzky, a Children’s Hospital cardiologist, told the VanDerwerkens he could do it. And he did. Amazingly, the operation was a success. Grace VanDerwerken was born ten weeks later with a robust, pumping heart. 6
Tworetzky had shared these stories over the phone with Jay and Sally Wiley and that had helped convince them to come, but he had also shared words of caution. There was maybe a 10 percent chance that Anders wouldn’t survive the operation. Just a few dozen infants had had the surgery, so it was hard to know the odds. The long-term prognosis could only be a guess, just an educated guess.
The Wileys landed in Boston on Sunday afternoon. Monday morning, doctors started running a series of tests, and on Wednesday, Sally Wiley found herself lying on an operating table with Wilkins-Haug gently manipulating her abdomen, trying to gently work Anders into the proper position. Out loud, Wiley said, “I can’t believe I’m here.” She thought of her own patients. She had cancelled all her appointments for the week. It felt strange; she felt personally close to many patients, but right now her daily Austin work routine felt very, very far away.
Jay tried to quell the worries running through his head while sitting in the hospital coffee shop with a friend who had driven down from Providence. He avoided saying what was really on his mind: “They told us there was a chance that the baby wouldn’t even survive the procedure. There are inherent risks in any kind of invasive procedure, and if he lived, there was a chance he would still have hypoplastic left heart syndrome. If he survived the operation, they were talking about a series of future heart surgeries.”
But by now there was no turning back. Getting the fetus into an accessible position can take as long as forty-five minutes, but Anders moved easily—it didn’t take more than twenty minutes. As soon as he was turned in the proper direction, the anesthesiologist stepped in, and within minutes, Sally was completely knocked out. The anesthesia drugs were absorbed by Anders, too, quieting his small, jerky movements.
Wilkins-Haug worked quickly. She began by making a small incision in Sally’s bulging belly. Dr. Tworetzky’s description from 2002 was pretty apt. Like a lot of modern surgery, this one really looks like a science fiction movie. The only thing guiding Dr. Wilkins-Haug was the black and gray ultrasound image on a monitor, just on the other side of the operating table. As she slipped the needle through the incision, she kept an intent eye on the picture. Beside it was Dr. Carol Benson, an ultrasound specialist, who watched an identical image on a second monitor over Wilkins-Haug’s shoulder. Tworetzky stood toward the back of the room. Several other nurses and technicians—about a dozen people in all—stood behind either Wilkins-Haug or Benson; among the group were two specialists in cardiac intervention who could jump in if anything went wrong with Anders’ heart. They were there to try and restart the heart if it suddenly failed.
With the point of her needle just outside the uterus, Wilkins-Haug paused, cocked her head, and stared intently at the image on the monitor. Her next motion would slide the needle through the uterine wall and the chest wall of the fetus, all in one smooth motion. It would be crucial to take the proper angle. Just a few millimeters off target might puncture the wall of the heart. A bigger slip, a nick of the uterus, could send Sally into labor.
Any surgery requires precision, but this one was complicated by the fact that Wilkins-Haug would be striking at a moving target. “The baby is floating in amniotic fluid,” she explains. “There’s a lot more movement there than people realize.” Not only was the baby gently bobbing in its fluid-filled home, there also was movement from the mother herself. Even on a ventilator, Sally’s body gently rocked with the pulse of her major arteries. The movement was subtle, but keep in mind that Anders’ heart, at twenty-two weeks, was just about the size of a grape. Wilkins-Haug was aiming for the left ventricle, a space no bigger than a raisin.
It is a skill that is only gained by experience. Wilkins-Haug watched her needle on a flat screen, but she was working in three-dimensional space. The fetus could roll forward or back with the motion. Over time, Wilkins-Haug has come to rely on an almost intuitive sense of touch. She says, “There’s a rhythmic movement. You have to kind of find where your spot is going to be, when there won’t be any countermovement rocking back towards you.” Aside from the risk of puncturing something, if she bumped Anders’ rib, it might be enough to jolt him out of position. That would mean halting the operation.
When she felt her needle in position, Wilkins-Haug leaned forward just a touch and felt the sharp tip slide through the uterine wall, through the surface of Anders’ chest, and through the valve at the bottom of the left ventricle into his tiny heart. It had been less than five minutes since she’d made the first incision.
The needle was like a straw, and a second physician—a cardiac interventionist—threaded a small wire down the narrow channel. It was attached to a tiny balloon. The next step has been compared to fly-fishing; the wire floats in the trickling blood flow while the cardiologist waits for it to catch against the valve. When it does, the doctors inflate the balloon and stretch the opening. Right away it looked good inside Sally Wiley. On the screen, the surgical team could see a rush of blood through the chambers on the left side of the heart.
Wilkins-Haug waited about thirty minutes after removing the needle to make sure there was no bleeding and to make sure that Anders’ heart didn’t go into an abnormal rhythm. Everything was fine. She gave the signal, and the anesthesiologist started to nudge Sally back to consciousness. The entire procedure had taken barely an hour and a half. Now, all anyone could do was wait.
Heart surgery on someone who isn’t quite a person yet is a staggering achievement, but a handful of prebirth interventions are more well established. Every year, surgeons go inside the womb to prevent major birth defects like fetal tumors, urinary blockages, and congenital diaphragmatic hernias. Each procedure is another tool to cheat death.
But as typically happens in a new field of medicine, doctors grow more comfortable with these cutting-edge techniques and look to us
e them more broadly. In recent years, physicians have moved to treat illnesses that are “not life threatening, but life ruining,” as Michael Harrison puts it. A major trial is under way to see if prebirth surgery can prevent a crippling form of spina bifida, where the spinal cord is not fully covered, leading to lifelong incontinence and paralysis of the lower part of the body. Other procedures that are on the drawing board sound even more like science fiction. Harrison, the father of fetal surgery, talks about going into the womb to inject stem cells into the fetus, to prevent common but devastating problems like sickle-cell anemia or genetic disorders in the metabolic and immune systems. “Stem cell biology is wonderful, but the place it will pay off clinically is in the fetus,” he says. “The earlier you can start a developmental change, the better it is. These diseases can be quite damaging, even by the time the baby is born.”
By the time Wilkins-Haug wheeled Sally Wiley to her hospital room, the surgical team had avoided the biggest pitfalls and the operation looked like a success, but there was no way to tell, not just yet. There was still a chance that the valve could close off again. For now everyone could only sit and wait, while Anders grew inside his mother.
Wiley was in no physical pain, but the wait would be agonizing. The biggest concern was preterm birth, with the time of greatest danger being the first forty-eight hours postsurgery. At twenty-two weeks, there was no way Anders could survive outside the womb. Any birth before thirty-four weeks is considered premature, which sharply raises the chance of respiratory problems, cerebral hemorrhage, poor circulation, and a host of other problems—-including a long-term risk of conditions like cerebral palsy. As you might imagine, the earlier a baby is born, the bigger the danger. Anyone born before twenty-eight weeks—when lungs reach a crucial level of maturity—faces especially daunting challenges.
