Editor's note: This four-part series originally published in 2000. Second of four parts.
NASHVILLE, Tenn. -- Susan Williamson looked small, almost frail, in the hospital bed; her pregnant belly only slightly plumped the bedsheets.
She was just over 22 weeks pregnant, and today, May 17, 2000, her baby would experience a sort of birth.
No one in the room felt like celebrating. After a grueling five days, Susan and her husband, Jason, had decided to proceed with an experimental operation on their unborn baby, Anna, who had been diagnosed with spina bifida. The birth defect, in which the spinal column fails to close, meant Anna could be paralyzed below the waist and would suffer complications affecting her brain.
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Surgeons at Vanderbilt University Medical Center believed that by opening Susan's womb and repairing the hole in Anna's back, they could give Anna a chance for a better life.
But the surgery carried enormous risk. It would inevitably prompt Anna to be born early, perhaps dangerously so, and it would endanger Susan's future pregnancies.
Susan tried to push those thoughts from her mind. The night before, fitful from lack of sleep, she opened a devotional prayer book, and the Scripture for the day was Philippians 4:13: "I can do all things through him who strengthens me."
She kept coming back to the passage.
"I could not do this on my own strength," Susan told her mother, Joyce McNeil, and sister Anne McNeil. "I would have backed out a long time ago."
Susan and Jason placed their trust, and their daughter's life, in the hands of two surgeons at Vanderbilt University - Joseph Bruner and Noel Tulipan - who pioneered the procedure for spina bifida babies. Bruner was an obstetric surgeon - "a fetal kind of guy," he said - and so he emerged as the front man for the team. Whenever the Williamsons began to worry, began to question the laws of probability, they called him. Or paged him. Or had somebody run him down.
Bruner had a way of assurance. He had his serious side, but he was quick with a quip, and the laugh lines around his eyes creased deep. Reared in Nebraska, Bruner managed his brilliance behind a gusty, wide-open plain of confidence. It made for a disarming bedside manner: Call Bruner; feel better.
Tulipan was the pediatric neurosurgeon, wry but reserved next to Bruner's brashness. Tulipan was well-qualified for the procedure. Before he began operating on fetuses, he built expertise closing spina bifida lesions in babies after they were born and then implanting shunts, mechanical devices to drain the buildup of brain fluid in their tiny heads.
He averaged 150 cases of shunting a year, and spina bifida was the main culprit.
"Spina bifida is a pretty miserable disease, and the treatment hadn't changed in 20 or 30 years," Tulipan said. "That's the underlying reason we got into this."
The two doctors linked up shortly after they both arrived at Vanderbilt in 1990. Each had eagerly followed the strides scientists had made in fetal surgery, primarily at the University of California at San Francisco. One group of researchers there had conducted operations on sheep fetuses to correct spinal lesions, and those lambs had been born with near-perfect leg function. Another group operated on a human fetus to correct a lethal defect that prevented the lungs from developing.
Still, fetal surgery on humans remained controversial because it put the mother and the baby at such risk. The medical establishment accepted it, often grudgingly, for conditions that were otherwise fatal. Spina bifida didn't qualify.
Bruner and Tulipan, though, believed the birth defect was serious enough, and all too common, to warrant the drastic surgery. They pointed to the sheep experiments that had shown such promise in restoring leg function, and they reasoned that human children might be spared wheelchairs.
In 1994, they became the first to try it, using a robotic device common for abdominal surgeries. The approach, though minimizing the size of the incision, was disastrous. Two of the babies died; the other two were born dangerously premature.
That, of course, set off an avalanche of criticism within the medical community. At seminars and forums, the doctors were blasted for gambling with the lives of babies who would otherwise have been born alive, though disabled.
But Bruner and Tulipan felt certain some good could come of the procedure. They theorized that spina bifida delivered a double whammy. The first, and most obvious, struck when the spinal cord failed to close in the early days of pregnancy, creating the paralysis and brain complications; the second, more subtle, came during the final trimester of pregnancy, as the lesion - and all that vulnerable nerve and bone - bathed in the increasingly toxic soup of amniotic fluid.
So the doctors tried again in 1997, this time using a new approach in which the mother's womb was actually removed from her body.
Their procedure, and a similar one done by a team of doctors in Philadelphia, seemed to work, particularly for babies like Anna, whose spina bifida lesion was low enough on her back that she would probably be able to walk, even without the surgery.
The most intriguing result of the procedure - and the reason the Williamsons chose to go forward with it - was evidence that it reversed one of spina bifida's damaging neurological complications, a condition known as a Chiari II malformation, where the hindbrain slips back along the neck.
When that happens, the natural channel for cerebrospinal fluid gets dammed, causing a buildup, or water on the brain. Doctors must then perform brain surgery to implant a draining device called a shunt. Ninety percent of spina bifida babies need shunts and, as Tulipan said: "Shunts are bad news." They fail, get infected. Pulling them out and replacing them can involve a lengthy hospital stay, a big block of time when a baby's life is measured in weeks.
All the babies who had the operation at Vanderbilt experienced a reversal of the Chiari II malformation, and about half of those babies didn't need shunts at all.
That was the chance the Williamsons sought for Anna.
The surgical team briefs Susan
The first doctor to drop in to Susan's room was Nancy Chescheir, peeking her head around the door.
"Hi, guys!" she said. "Everybody decent?"
Chescheir had flown in the night before from Chapel Hill, where she is a professor of obstetrics and gynecology at the University of North Carolina medical school. Susan and Jason had consulted with Chescheir before they decided to investigate the Vanderbilt procedure.
Chescheir had, in fact, served as Bruner's first assistant on eight operations, learning, through apprenticeship, how to perform the in utero surgeries in the hope that she could begin doing them at UNC Hospitals in the fall. She would scrub in for Susan's operation.
Susan, especially, was grateful for Chescheir's role. Where Bruner's naw-don't-worry approach bolstered confidence, Chescheir had a more soothing presence.
Bruner arrived five minutes later, and he was all business. After greeting everyone, he sat on the edge of Susan's bed, offering a form for her to sign in which she acknowledged that she understood the risks. He was compelled to lay them out: The surgery, he said, would consist of a laparotomy (a hole in Susan's abdomen) then a hysterotomy (a hole in Susan's uterus), then the neurosurgical repair of the lesion.
Bruner continued: "The risks include pain, bleeding, infection, trauma, preterm labor and delivery, loss of fluid, heart failure and, of course, death. Any one of these may require blood or medicine or both, and require a longer hospital stay, now or during the pregnancy, or require further surgery. We had one patient that had bowel obstruction. And you could have a hysterectomy."
Susan signed the forms.
"Is there anything you want me to tell Anna?" Bruner asked on his way out.
"We just want to tell her we love her, and be still, and know God," Susan said. "And tell her to hang in there another 15 weeks."
Preparing mother and baby
The nurse prepped Susan in a methodical routine, hooking her up to an automatic blood-pressure cuff, strapping her belly with a monitor to amplify Anna's heartbeat. Shortly after 11 a.m., she started an intravenous line to drip its potions: muscle relaxants to keep Susan's uterus from contracting and antibiotics to ward off infection.
At 12:50, the anesthesiologist arrived to set up an epidural to numb pain below Susan's waist. Susan was ready. Woozy, her big brown eyes drooping from medicine, Susan lost fear. As they wheeled her out of the room, she blew a kiss to her mother. Jason held her hand down the hall until, at 1:20 p.m., the technicians pushed her behind the double doors toward the operating room, leaving everyone behind.
Jason stood at the windows until she was out of sight. He, among everyone, wasn't worried. It wasn't in his nature. "I'm not in control of anything that's happening today, " he explained.
Making the incision
Bruner and Tulipan kept the operating room at temperatures above 80 degrees, hot for the surgical team but cool for a baby conditioned to lounging in a 98-degree bath.
The anesthesiologist fed a tube down Susan's throat and began delivering the general anesthesia. When Susan was completely out, Bruner began. He started with a large incision into Susan's abdomen, peeling back the layers of skin, fat and muscle. This took time: slice and yank, slice and yank. Chescheir held the flesh back as Bruner worked.
Finally, they got to the uterus. The magnesium sulfate that dripped through the IV relaxed the uterus, making it possible for Bruner to lift it completely out of Susan's body and place it on her stomach.
"It looks like a red soccer ball," Tulipan said.
The doctors took ultrasound readings to figure out how Anna was positioned inside. She weighed less than a pound, and stretched only 7 inches from crown to rump. And she was fast asleep; the general anesthesia that knocked Susan out passed through the placenta and put Anna under, as well.
Bruner wanted to make his incision in Susan's uterus close to the lesion on Anna's back, so all that Tulipan would have to do was sew the hole shut. But Bruner also needed to avoid the placenta; grazing it would compromise Anna's lifeline to Susan and force an immediate delivery. He also didn't want to nick any hidden blood vessels in Susan's uterus. Profuse bleeding would endanger both the mother and the baby.
The doctors maneuvered Anna in the womb, watching the ultrasound and gauging, from the image, the best spot.
Then Bruner used a tool he and Tulipan designed specifically for these operations, and it cut, with one certain motion, a 1-centimeter incision in which Bruner inserted a suctioning device to drain the amniotic fluid into a sterile container, saving it for later.
Finally, it was time to open Anna to the world.
Waiting and wondering
Jason returned to Room 5 on the labor and delivery floor to wait with Joyce and Anne. The room, aspiring to hominess, achieved only hotel-level comfort; it wasn't a place to relax.
Joyce and Anne slipped out briefly to grab some lunch in the hospital cafeteria and returned with a burger and fries for Jason. He scarfed them down, sitting on a small, round stool common to doctors' offices.
The wait wore on. With Susan gone, conversation swung from her childhood to her courtship with Jason, to her work - as if in a eulogy. From the window, way off in the distance, a church steeple poked up from a cushion of trees, and Anne remarked that it made a fitting image for the day.
What was going on, they wondered.
At 2:23 p.m., the phone rang. Susan had been in surgery for more than an hour, and the nurse, as promised, was calling with an update.
"Hello?" Jason answered. "Oh! They found her! Great. Snap some really cool pictures."
Jason hung up and turned to Joyce and Anne.
"Anna's being worked on right now," he told them.
"The hardest part about this surgery is that it's not like it's a relief and it's over," Anne said. "You're on pins and needles from now until Anna is born."
"Yeah," Jason said, "it's hard. It's hard knowing my baby is right there, and I can't see her. I really want to see my baby."
Closing Anna's spine
Using a tool that cut and stapled at once, Bruner opened a 3-inch incision in Susan's womb. There, at last, was Anna. And there was her defect. Unlike most spina bifida lesions, Anna's was not a cyst that Tulipan had to lance before folding the nerve and bone into the hole and sewing it shut. Hers gaped open, exposing the nerves and bones to the amniotic fluid.
That sort of lesion, the doctors knew, was especially vulnerable to toxins. Had Anna not undergone the surgery in utero, she would have been susceptible to dangerous infections.
Tulipan made cuts along both of Anna's hips so he could pull the skin together over the lesion and stitch the hole closed. That left two open wounds on Anna's haunches, and Tulipan covered them by sewing on skin grafts cultured from cadavers.
Tulipan - wearing a neon pink headband to capture the sweat beading from the heat in the operating room - worked swiftly, starting and finishing in less than 25 minutes.
With Anna mended, Bruner returned the amniotic fluid, sewed up the uterus, put it back into Susan's belly and stapled her incision shut.
They were done.
At 3:07 p.m., the nurse phoned Jason.
Joyce and Anne, sitting on the small sofa under the window, fell silent at the ring.
"Hello," Jason answered. "How is everything? ... All right. ... Everybody's happy? ... Good deal."
Jason hung up and looked at Susan's mother and sister.
"It's over. Now the hard part."