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.
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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 use 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.
Until the mid-1990s, virtually all fetal procedures were done outside the mother’s body. Virtually all of these patients ended
up being born prematurely. Anyone who’s been around for a delivery can understand the basic problem. When the sac in which
the fetus grows is torn or punctured, the amniotic fluid that surrounds and cushions the baby leaks out. Or pours out, as
the case may be. When a mother’s water breaks, it signals that labor is close at hand. The tearing of the sac, also called
the rupturing of membranes, is more than a side effect. According to Michael Harrison, the amniotic fluid actually triggers
a complex biological response, which itself induces labor. That’s why a physician who wants to induce birth will simply rupture
the membranes.
To do anything to the fetus surgically, there’s no way to avoid this fact of biology. You have to go through the lining of
that sac. Says Harrison, “If you poke this little thin lining with a needle, you make an opening that does not heal. Fluid
leaks through the hole, gets between membrane and muscle, and it’s a metabolically active fluid.” It turns out that even a
tiny amount of that fluid is enough to cause a baby to be born several weeks before it’s due. You might think it’s a soluble
problem, but Harrison says the human anatomy has proven trickier than expected.“We used to say, ‘Oh, just make a smaller hole.’
But it’s a discouraging thing; we’ve found that using smaller instruments doesn’t offer a big advantage.”
A few developments have made fetal intervention far less risky, to both mother and child, than it was in the early 1990s.
As we’ve seen already, sometimes the most important medical breakthroughs are the simplest. In the case of fetal surgery,
the biggest thing making it safer and more effective was the simple ability to see the patient. The first fetal surgeries,
at UCSF Children’s Hospital and a handful of European hospitals, were crude. Surgeons had to open the mother’s abdomen to
reach the fetus. But the mid-1990s saw the development of tiny fiber-optic cameras, which could be inserted on a small instrument
and send back pictures to a viewing monitor that would guide the surgeon. Michael Harrison and his colleagues at UCSF dubbed
the technique Fetendo, because it reminded them of watching their kids play Nintendo video games.
7
(Dr. Hanmin Lee, the current director of fetal therapies at UCSF, smiles at the mention of Fetendo but says he prefers to
talk about fetal endoscopic surgery.
8
)
Another breakthrough came with the improvement of ultrasound imaging, which got to the point where the ultrasound could make
out fine distinctions in a heart the size of a grape. A lot of expectant parents have watched in wonder as an ultrasound technician
sends back pictures of their growing baby. This is the same technology that made possible the operation on Anders Wiley. “This
is where the whole field is going,” Dr. Lee told me. “We are trying to make this a minimally invasive surgery.”
Making fetal intervention minimally invasive hasn’t solved all the problems. Even for procedures like the one that Sally Wiley
went through, the proportion of preterm births is about one in three. Preterm delivery and labor remains the Achilles’ heel
of fetal surgery. Says Harrison, “We’ve been trying for thirty years, and we’ve failed [to prevent preterm birth].”
For such a miraculous-seeming medical procedure, fetal surgery has seen its share of disappointments. One of the biggest involved
early efforts at UCSF to repair congenital diaphragmatic hernias. CDH was one of the first problems identified as something
which might be fixable in utero. Of babies born with the defect, about a third die and many more have lifelong, severe, and
debilitating problems caused by stunted lung development.
Unfortunately, in the first group of UCSF patients, the babies who had surgery did even worse.
9
“We found it was probably too much of an operation,” Lee says now. “It didn’t really work. We just had too many problems
with preterm birth and the delicacy of the fetal body at that stage.”
A group of younger UCSF doctors decided to take another tack, trying a complicated procedure that involves blocking the trachea
of the fetus, which causes the developing lungs to secrete fluid. They hoped that the added pressure inside the chest would
push the lungs to grow. Early results were promising, but a larger study fizzled. It’s not the end of the story; efforts are
now under way to refine the technique, using smaller surgical tools and leaving the trachea blocked for a shorter period of
time. A team in Belgium has found promising results. But even after twenty-five years of research, for a relatively simple
anatomical problem, there is still no reliable fix.
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Of the study that didn’t pan out, Lee told me, “Of course, it’s disappointing that something you put a lot of work into doesn’t
show the benefits, but you still have a group of sick patients you have to take care of. We need to think about ways of making
care better for these patients. It just makes you roll up your sleeves and say to yourself, ‘You still have more work to do.’
”
It’s a pretty tough ledger. On one side, there are modest successes, along with a few patients stolen from the clutches of
fatal illness. On the other side, there is a high risk of preterm birth, not to mention the dangers of any major operation:
bleeding, infection, and complications with anesthesia—for the mother as well as the developing child. With this daunting
calculus in mind, these operations are almost never done unless doctors think there is no other option—if the condition looks
so debilitating that the child can’t wait until he’s born.