Editor's note: This four-part series originally published in 2000. First of four parts.
NASHVILLE, Tenn. -- The pressure was wearing on Susan and Jason Williamson as they walked into the surgeon's office for yet another meeting.
Susan, who was 22 weeks pregnant, eased into one of the hard-back chairs, smoothed the smock over her belly and instinctively reached for Jason's hand.
"This is so hard," she said, sighing.
Four days before, Susan and Jason had arrived at Vanderbilt University from Holly Springs, N.C. Now they had just 24 hours left to make a decision that would change their lives and the future of their unborn child.
Four weeks earlier, Susan and Jason learned that the baby they so eagerly awaited - a girl, her name would be Anna - had spina bifida.
Since then, they had taken a crash course in a difficult subject, dutifully gathering all the reports, all the numbers, all the odds that had somehow hit: One newborn in 1,000 is afflicted with the paralyzing defect. It meant Anna might never walk; that she would probably need brain surgery, perhaps several times; that she would be susceptible to constant urinary infections and kidney disease.
Doctors explained that many parents in the Williamsons' position decide to end the pregnancy. Susan and Jason dismissed that idea right away; abortion was simply not an option.
But was doing nothing the only alternative?
That was the question that had led them here - to Vanderbilt, to this surgeon's office. They were exploring a third path, and it was a medical experiment.
Two of the university's doctors proposed operating on Anna in the womb, cutting open Susan's uterus and repairing Anna's defect in the tender months before birth. The operation, the doctors said, would give Anna a chance for a more normal life. But the doctors wanted to operate now, during Susan's 22nd week of pregnancy, because the earlier they went in, the better the results. So the Williamsons needed to decide by the next day.
The procedure was risky. Only 89 other women had dared to undergo the Vanderbilt procedure. One baby had died. Others had been born prematurely, some dangerously so. One mother's uterus broke open during labor.
Susan and Jason recited the results like scientists at a symposium. They folded the reports and pamphlets - a small library of data - into the black vinyl backpack Jason carried with him everywhere. He set it on the floor of the surgeon's office, plopped himself into the chair and reached for Susan's hand.
Twenty-four hours left to decide. One day, a lifetime.
"All we can do is go with our feelings," Jason said, "and trust that it's the right thing."
The first of four
Susan and Jason were both 27, and for as long as they had known each other, they had wanted four children. They decided to start trying for the first of those children last year. Finally, on the morning of Jan. 8, 2000, they passed the results of a home pregnancy test back and forth, double-checking against wishful thinking that it really showed a plus sign.
Sure enough, they were pregnant.
Days before that morning - before they had any reason to suspect Susan was pregnant - the cells that formed Anna's body had been busy dividing, and dividing again. Somewhere around the 21st day after conception, when Anna was no larger than a grain of rice, a virtual explosion of cells created her spinal column and brain.
As the cells formed, they were already programmed to become bone, nerve, muscle, skin - and they began moving into their assigned places, starting at the midpoint in her back and working in both directions to form the neural tube.
The cells stitching toward the brain did fine, but something happened along Anna's lower back. Where the spinal cord was supposed to knit shut, it didn't. What resulted was a hole, exposing bone, nerve and skin.
Doctors have a term they use when they don't know what causes something: They say it's multi-factorial. Spina bifida is multi-factorial. It may result from some unknown environmental pollutant. It may run in families, particularly those whose ancestors came from the British Isles (the United Kingdom has one of the highest rates of spina bifida in the world). It may just be rotten luck.
Whatever the causes, they seemed far removed from the Williamsons. The couple had only recently moved to North Carolina, where the incidence of spina bifida is the highest in the country, occurring in one in every 500 pregnancies - twice the national rate. Neither Susan nor Jason knew of any cases in their families. And Susan knew to take a vitamin supplement that included folic acid before she and Jason tried to become pregnant.
Since 1992, the government had been recommending that young women take at least 400 micrograms of the B vitamin, which is important for cell growth, because it had been found to prevent up to 70 percent of neural tube defects, of which spina bifida is the most common.
Susan had been on the supplement for a year before conceiving - far surpassing the experts' advice to take it for three months before becoming pregnant.
The Williamsons had done everything right. They had no reason to worry.
A simple, routine blood test
Susan had an appointment with her obstetrician on April 7, when she was a few days shy of her 17th week of pregnancy. She was feeling great - reveling in her body's changes, maintaining her running routine of three miles, four times a week, switching from regular clothes to maternity clothes.
It was time for her to have an AFP test, a simple blood test that measures the alpha-fetoprotein, a substance that helps cells communicate during development. A tiny amount of AFP normally transfers into the amniotic fluid and across the placenta into the mother's bloodstream.
A baby with spina bifida leaks larger amounts of AFP through the open hole in the spine, elevating the protein levels measured in the mother's blood and prompting a series of more precise tests to confirm the diagnosis.
And it's such a devastating diagnosis, with disabilities often so profound, that half the parents end up choosing to terminate the pregnancy.
But for Susan and Jason, such a choice was no choice. They were so adamant on this point that they almost opted to forgo the AFP test altogether. What was the use, they reasoned, of having such information if its only purpose would be to encourage an abortion?
Susan talked with her girlfriends, asking if they had had the test, noting that she and Jason didn't see any reason for it. One friend mentioned that she had read about a procedure at Vanderbilt University in which doctors had operated on spina bifida babies to some benefit, and that caught Susan's attention.
So she and Jason discussed it. Susan had the AFP test.
Within a week, the doctor's office left a message on the Williamsons' answering machine for her to call the next day, a Friday. Susan couldn't catch up with the doctor that morning, and the office closed at noon.
Curious, but not concerned, she thought little of it that weekend.
"I knew it would be normal," she said.
It was anything but. The news stunned Susan, who took the call Monday morning. Jason was playing in a charity golf tournament - a marathon 100 holes for Campus Crusade for Christ - and he called to find out the results during a break on the 40th hole.
"One minute," he said, "I'm expecting a baby. The next minute, my baby has a disability."
Susan called her mother, Joyce McNeil. They cried. She called her older sister, Anne McNeil, and her younger sister, Martha Fisher Raterman. They cried.
When Jason got home, he tried to keep Susan focused on the odds. Ever optimistic, ever confident, he kept telling her that the doctor calculated a one in five chance of spina bifida, which meant there was an 80 percent chance that nothing was wrong. Eighty percent. Those were good odds.
Romance rooted in faith
Jason's confidence is what attracted Susan in the first place. She had never met a guy who had such total assuredness - especially at her high school in Richmond, Va. As a cheerleader, a member of the honor society, a bona fide member of the popular crowd, Susan McNeil had dated her share of the cocky, stuck-up type.
"He wasn't like other boys," Susan said. Jason exuded calm. Balance.
He had only moved to Richmond their sophomore year, and while he and Susan were classmates, they ran in different circles. Funny and outgoing, he had unusual priorities, inspired by a calling to God he had answered at the age of 4.
Instead of spending his summer breaks working, or partying, or playing, Jason traveled overseas with a Christian ministry to smuggle Bibles into the Soviet Union, or build churches in Africa.
Susan might never have paid Jason much mind but for a bit of soul-searching of her own. Her senior year, a close friend battled cancer and eventually died, and another friend fatally shot himself. She distanced herself from many of her in-crowd friends, and found solace in church.
It was Susan's grandmother who brought the two together. The spring before graduation, she was sitting in the bleachers, watching Susan's cousin play a Little League game. Jason was there to watch his little brother. He struck up a conversation.
"My grandmother was so taken that this high school guy would talk to her," Susan said. "So I started looking for him in the halls."
Their first date was June 2, 1991. He kissed her on the second date, four days later, prompting Susan to confide to a friend that Jason was the man she would marry.
They carried on a commuting romance during college - Susan at the University of Virginia in Charlottesville and Jason at Virginia Tech in Blacksburg and then Virginia Commonwealth, back home in Richmond.
They were married on July 1, 1995. In a gesture of equality, he changed his middle name from Patrick to McNeil - Susan's maiden name. They share both names, no hyphen.
Four years later, the couple moved to Holly Springs, drawn to the area by friends. Jason went to work as a software engineer for a start-up company in Cary, and Susan kept her job selling credit-card accounts with Capital One in Richmond, working from home and commuting back to Virginia once a week.
About the same time, the Williamsons felt settled enough to start a family, the first of the four children they had long dreamed of having.
Reaching a diagnosis
The AFP test was by no means definitive. Sometimes the elevated protein level simply meant the baby was further along than anyone suspected. Maybe Susan and Jason had just miscalculated. Maybe it was a simple mix-up.
To confirm the diagnosis, Susan needed a detailed sonogram for a closer look at Anna's anatomy. The lab agreed to work her in the next day.
From her vantage point on the examination table, Susan tried to make sense of the ultrasound pictures she would one day be able to read almost as well as a doctor. The technician tried to keep things light - heart is normal, hands are normal.
It's a girl, she announced. Susan and Jason were elated. They had already picked out her name, Anna Fisher, honoring Susan's sisters.
Then the technician grew quiet and the whoosh-whoosh-whoosh sound of Susan's womb, amplified by the imaging machine, filled the room. Finally, Dr. Steven R. Wells, who had viewed the scan from a computer link in his office, came in with the news: Anna had spina bifida.
The doctor gave the Williamsons some time alone, and the technician quietly retreated. For 10 minutes Susan and Jason held each other, sobbing and praying.
The baby they dreamed of was gone. In her place was a different baby, a baby with spina bifida, and Jason and Susan struggled to know and understand her.
They knocked on Wells' door and sat down to learn more, to acquaint themselves with this new baby.
The doctor told them that this baby, Anna, had a hole in the lower lumbar region of her back that would require a surgical repair once she was born. The repair would only mend the hole, not fix the damage.
But Anna, the Williamsons were told, was actually pretty lucky. The location of the lesion on the spinal cord determines how much mobility - and bladder control - a child will be able to develop. The higher the lesion, the more paralysis.
Anna's lower lumbar lesion meant she would have some leg function, possibly even be able to walk, and have some degree of bladder control.
But Anna had already developed other problems. Spina bifida lesions typically have an insidious effect on the brain, pulling it back along the neck in what's known as a Chiari II malformation - named for the German pathologist who first described the defect more than 100 years ago. Already, Anna's hindbrain had begun to shift.
The malformation would likely begin to dam the flow of cerebrospinal fluid from the ventricles in her brain, causing hydrocephalus, or water on the brain. So Anna faced another operation after she was born - this one to implant a mechanical system, called a shunt, deep into her brain to drain fluid.
Eighty to 90 percent of spina bifida babies need shunts, and although they have been lifesavers, they can get infected or fail. Shunt revisions are costly - up to $30,000 an operation if it requires a long hospital stay. Some children endure two, three, four such procedures. Children with spina bifida can incur medical expenses of $150,000 even before they enter kindergarten, and lifetime expenses range from $500,000 to $1 million.
Beyond money, shunt revisions keep children in the hospital and rob them of time spent in normal activities. And if the devices fail, causing fluid buildup, brain damage can occur; studies show that the more shunt procedures children endure, the lower their average intelligence.
Susan and Jason were reeling ...
"When you start having a baby, you make plans," Susan said. "It's like you're going to Italy. And you make all these plans of where you will go and what you will see, and you even start to learn the language a little. And then the day comes, you get on the plane and you land, and the pilot says, 'Welcome to Holland.' And you're like, I was going to Italy. It's not that Holland is bad, it's just that you have to adjust your thoughts, learn a new language. You know, everybody else got to go to Italy. It's hard to get over the fact that you dreamed of going to Italy and you never got there."
The promise vs. the risk
Before she left Wells' office, Susan mentioned that she had heard about an operation at Vanderbilt. Yes, the doctor said, he knew about the procedure, but it was controversial. He believed the benefits didn't justify the risks, because the procedure inevitably caused a premature birth. And it didn't appear to give the babies any extra mobility.
But, he conceded, he had a colleague who thought differently. He suggested that the Williamsons seek out Dr. Nancy Chescheir, a specialist in high-risk pregnancies at UNC Hospitals, because she knew more about the surgery.
Three days later, Susan and Jason sat in Chescheir's office. Chescheir, an amiable woman who inspired a devoted following among her patients, knew, in fact, quite a bit about the operation. A few weeks earlier, she had begun assisting the Vanderbilt doctors - Joseph Bruner and Noel Tulipan - in the hope that she would perform the in utero surgeries at Chapel Hill.
Even with her hands-on experience, though, Chescheir was cautious in her assessment of the procedure. It showed promise, but it was definitely risky. One baby had died, and 18 percent of the babies who went through the lesion repair in utero were born before 30 weeks of gestation, some on the fringes of viability.
The mothers, too, put themselves in peril, Chescheir explained. The incision in the uterus could rupture during labor, and that could kill Susan and Anna, or prompt an emergency hysterectomy. At the very least, all Susan's future pregnancies would be considered high-risk.
But among the benefits, which had only recently been reported, was a reversal in the Chiari II malformation that had already shown up in Anna. Somehow, closing the lesion in the second trimester caused the brain to shift back into a more normal position, so that when many of the babies were born, they didn't need shunts - at least not right way. Such an outcome, though, depended in large part on the surgery's being performed early enough in pregnancy - before the 24th week.
The Williamsons were intrigued. They could come to terms with the idea of a smaller family, if it meant sparing Anna from a shunt. They drove home and called Vanderbilt.
A maze of misgivings
The university didn't make anything easy. It had set up a rigorous screening program because it recognized that the people who sought the surgery, who flew at whatever cost to Nashville, were, by virtue of their very presence, predisposed to having the operation. Fourteen percent of couples decided, after going through the gauntlet, to opt out.
Among the most daunting hurdles was a long, difficult conversation with the university's ethicists, Richard Zaner and Mark J. Bliton. They were brutal.
You believe in God? It was the standard question they asked of everyone. And everyone, the ethicists said, answered yes. Zaner and Bliton probed: What kind of benevolent God would do this to you?
The Williamsons responded without doubt. They were chosen to be Anna's parents. This adversity had meaning. They were led to Vanderbilt and the alternative it offered.
"Keep in mind how thoroughly American you are and how thoroughly American medicine is," Zaner warned them. "We have the idea of doing. Going forward. All of these are metaphors endorsing the idea that when you have options and you choose action, that's the ideal. The idea of having an option and not choosing action, that's the moral dilemma. So keep in mind your tendency to want to act. Choosing not to go that route is a powerful moral force, even though in our society we tend to underplay it."
The Williamsons endured the probing of the ethicists. In fact, they withstood almost everything the university threw at them: the meetings with surgeons to explain, blow by blow, the experimental procedure; the $30,000 cost, half of which had to be paid upon admission. Here, the Williamsons were lucky. Susan's company carried Aetna U.S. Healthcare, which paid the total cost of the procedure - a decision the company made on the basis of Vanderbilt's record of reducing the number of expensive shunt operations. Other insurers weren't so willing to pay, prompting exhaustive battles that the university won in two-thirds of the cases. And some families borrowed money or maxed out their credit cards to pay.
Such issues didn't dissuade the Williamsons.
On their second day at Vanderbilt, however, their resolve was shaken by a tiny baby. On a tour of the neonatal intensive-care unit, Susan and Jason saw firsthand the consequences of a premature birth. There in the neonatal unit, in a Plexiglas container that maintained a temperature of 94 degrees, a baby born in the 24th week of gestation fought for his life.
His skin was red and incapable of keeping infection out, he sucked air spastically through a tube, he fed unconsciously through intravenous lines. He was smaller than the Beanie Baby that someone, perhaps his mom or dad, had placed beside him in the incubator.
Modern medical science, noted Dr. William Walsh, head of the neonatal unit, was dedicated to preventing premature birth. Babies born before 23 weeks couldn't be kept alive; it was touchy between 23 and 26 weeks.
"The real question for you as a parent is, 'Are they better off for this surgery?' " Walsh said. "If they are born at 23 weeks, and as a result they have cerebral palsy, and blindness, and disease, but no shunt, is that better?"
For Susan, such a fate for Anna was too much. Whatever else, she couldn't inflict additional pain and suffering on this baby. What should she do? What would Walsh do - would he have this surgery if it were his baby? She was shocked at his response.
"He said it was a reasonable choice, " Susan said. "I didn't think he would answer, but when he said that, it made all the difference."
The Williamsons met Susan's mother, Joyce McNeil, and sister, Anne McNeil, at the Nashville airport with a decision that had taken four days of deliberation, prayer, tears. The next day, Wednesday, May 17, at 1:30 p.m., Susan would undergo surgery.
An emotional last mile
Susan hadn't slept well, but she still wanted to walk the mile from the hotel to the hospital.
She had a sense that such a simple pleasure would become forbidden after the day's events, and she wanted to enjoy the small physical feat of it.
Before she was pregnant, and even in the early months of carrying Anna, she had been a dedicated runner, but she quit after Anna's diagnosis; Susan worried that running would jostle Anna and irritate the lesion on her back.
Now, on the morning of surgery, Susan walked along, holding Jason's hand.
"This feels like a wedding procession," her mother said.
"Yeah," Susan said, "but at least in a wedding, you can back out."
"I have such a heaviness in my heart," Susan finally confided. "I just want her to be OK." And then she gave in to tears. The procession stopped in the middle of the sidewalk. Joyce hugged Susan, whispered, "I love you," into her ear, and Jason engulfed them both.
"You're going to be all right," Anne said, tears welling as she stroked Susan's back.
"That's what I have to believe," Susan replied, forcing a smile.
In the hospital admissions office, the perfunctory forms seemed ominous, and Susan deliberated over her responses:
If she died, Jason would get everything.
If she suffered brain death, she wanted machines to keep her body functioning only until Anna could be born. And then Jason would have to pull the plug.
If Jason couldn't bring himself to do that, she wanted her sister Anne to take action.
The admissions clerk strapped a plastic identification bracelet on Susan's right wrist, and then the Williamsons headed upstairs to her room.
Only a last-minute panic could stop the process now.