Treating The Tiniest Patients

Samuel Armas, a chattering, brown-eyed 3-year-old, has no idea what "fetus" means. Nor does he realize that he was one of the most celebrated in medical history. At a mere 21 weeks of gestational age--long before it was time to leave his mother's womb--Samuel underwent a bold and experimental surgical procedure to close a hole at the bottom of his spinal cord, the telltale characteristic of myelomeningocele, or spina bifida. Samuel's parents, Julie and Alex, could have terminated Julie's pregnancy at 15 weeks when they learned about their son's condition, which can result in lifelong physical and mental disabilities. But the Armases do not believe in abortion. Instead, in August 1999, they drove 250 miles from their home in Villa Rica, Ga., to Nashville, Tenn., where Dr. Joseph Bruner, of Vanderbilt University, performed a surgery bordering on the fantastical. Bruner cut into Julie's abdomen, lifted her balloonlike uterus out of her body, made an incision in the taut muscle, removed the fetus, sewed up the spinal defect and tucked him back inside. Fifteen weeks later Samuel Armas "came out screaming," says Julie.

That scream became a rallying cry for fetal-rights groups, which seized on a stunning photograph of Samuel's tiny hand emerging from his mother's uterus during surgery. Since then, anti-abortion activists have posted the image on dozens of Web sites to show just how real human fetuses are--even those that aren't yet viable. And that's just fine with the Armases. "We're very glad it's gotten visibility," says Alex. "That wasn't our fetus, that was Samuel."

No matter what legislators, activists, judges or even individual Americans decide about fetal rights, medicine has already granted unborn babies a unique form of personhood--as patients. Twenty-five years ago scientists knew little about the molecular and genetic journey from embryo to full-term fetus. Today, thanks to the biomedical revolution, they are gaining vast new insights into development, even envisioning a day when gene therapy will fix defects in the womb. Technology is introducing parents to their unborn children before they can see their toes. Expecting couples can now have amazing 3-D ultra--sound prints made in chain stores like Fetal Fotos. "Instead of some mysterious thing inside her belly, a mother and her family can now identify a little human being," says Bruner. In any other field of medicine, the impact of these dramatic improvements in treatment and technology would be limited largely to doctors, patients and their families. But 30 long and contentious years after Roe v. Wade, science that benefits fetuses cannot help but fuel ongoing political, moral and ethical debates.

Fetal surgery has raised the stakes to a whole new level. The very same tools--amniocentesis and ultrasound--that have made it possible to diagnose deformities early enough to terminate a pregnancy are now helping doctors in their quest to save lives. While fetal surgery is still rare and experimental, the possibility that a fetus that might have died or been aborted 10 years ago might now be saved strikes at the core of the abortion debate. And these operations also raise a fundamental question: whose life is more important--the mother's or the child's? While reluctant to take a stand in the political arena, doctors know they are players, like it or not. "We can be a lightning rod used to further a cause, either pro or con," says Diana Farmer, a fetal surgeon at the University of California, San Francisco, "but you can't let that deter you from your mission as a physician."

For decades pediatric surgeons like Michael Harrison, head of UCSF's Fetal Treatment Center, agonized over their inability to save babies from deadly defects after birth. Since 1981, when Harrison performed a pioneering in utero procedure to treat a fetal urinary-tract obstruction, hundreds of fetuses have undergone treatments ranging from tumor removal to spinal-cord repair. Some operations have been dramatic successes, saving the lives of babies who would otherwise have died. Others have been heart-wrenching failures. In no other medical area are the stakes--two patients, not just one--so high. Now the first rigorous National Institutes of Health-sponsored trials will put prenatal medicine, including the spina bifida procedure, to the test. "If there's not a clear advantage," says Farmer, "it's not worth putting the mother at risk."

On the streets, the womb has become a political battlefield. In the OR, it is a medical mine field. Fetuses are moving targets--just locating and positioning them is like trying to catch fish underwater. The placenta, the fetal lifeline, can develop anywhere in the uterus, obstructing access to the fetus. A single nick in the tissue can put the lives of both fetus and mother in danger. Amniotic fluid, the liquid that cushions the growing fetus, can leak to perilously low levels. And preterm delivery, which inevitably occurs because of the disruption to the uterus, is the Achilles' heel of fetal surgery, increasing a baby's likelihood of everything from lung problems to learning disabilities down the road. Bold and entrepreneurial by nature, fetal surgeons have endured the skepticism, even hostility, of colleagues for years. Early on, "folks thought we were nuts," says Dr. Scott Adzick, head of the Center for Fetal Diagnosis and Treatment at the Children's Hospital of Philadelphia (CHOP). "Some still do."

Fetal medicine has been tangled with politics from its inception. Harrison's predecessor in the field, New Zealander A. William Liley, is credited with the first successful fetal intervention in 1963, when he performed an in utero transfusion to treat Rh disease, a deadly blood incompatibility between mother and baby. But over the course of his career, he became as much activist as physician. Ardently opposed to abortion, Liley described the fetus as "a young human," and rallied for fetal rights until his death in 1983. While most doctors keep their beliefs private, the volatile confluence of politics and medicine has led some to join politically aligned groups like Pro-Life Maternal-Fetal Medicine, or Physicians for Reproductive Choice and --Health. But those views can sway. In the past, right-to-life groups criticized surgeons for violating the sanctity of the womb. Now many support medical efforts to treat fetuses as patients. Such oscillations mean little to UCSF's Harrison. His goal from the beginning has been to deal with "the practical real problems of real people."

Susan and Jeff Dezurick are two such people. In October 1999, the couple learned that the twins Susan was carrying had a potentially deadly condition called Twin-Twin Transfusion syndrome, in which one fetus floods the other with fluid through a shared blood vessel in the placenta. In a procedure called amnio reduction, the Dezuricks' doctors in Oakland, Calif., removed excess fluid from the saturated twin, hoping to ward off a buildup of pressure on his heart and lungs. But the technique, performed repeatedly on Susan, ultimately failed. With one of the twins on the verge of death, the Dezuricks arrived at UCSF. In a minimally invasive procedure, doctors located the culprit vessel, zapped it with a laser and cut off the faulty connection. (The two techniques--amnio reduction and laser--are now being compared in trials worldwide.) For the Dezuricks, the outcome was happy. Ten weeks after surgery, Sean and Christopher were born, premature but healthy. Today, lightly freckled 4-year-olds with impish grins, the boys spin around their living room, hugging each other on their tippy-toes. "Thank God for technology," says Susan.

As fetal science advances, the critical question remains: is the benefit to the fetus worth the risk to the mother? Ethicists can debate it all they like, but for Kristin Garcia, the answer was undeniably yes. In her 20th week of pregnancy, Garcia's doctor told her that her baby had a severe defect called congenital diaphragmatic hernia. "He told me that most women choose to terminate, because there's absolutely no way the baby would survive," says Garcia. At UCSF, doctors warned, as they do routinely, that they could not guarantee a positive outcome. In any procedure, risks to the fetus--which is usually operated on between 18 and 26 gestational weeks--include brain damage, physical deformities and death. The mother can suffer excessive blood loss or a permanently scarred uterus that could rupture in future pregnancies (C-sections are done to avoid the problem), and, as with any major surgery, there is always the risk of death. For Garcia, the treatment was a success, but it was by no means easy. Fluid backed up into Garcia's lungs after the operation. "I couldn't breathe," she says. "I felt like I was dying." And little Analisa needed several surgeries to patch up her diaphragm. Today Analisa is "perfect and full of life," says Garcia. The physical and emotional tolls, however, were enormous. "I'm glad I did it for my first baby," says Garcia. "But I don't know if I could do it again."

As a lone female voice among fetal surgeons, Farmer is working hard to put the health of the mother front and center. Although fetal surgeries are rare--no more than about 600 patients are candidates in the United States in a given year--interest in the field is growing around the globe. At this year's annual fetal-surgery meeting, Farmer presented the first guidelines for maternal health, including a strict patient-consent process, counseling on nonsurgical alternatives and a fetal-oversight committee.

Even the best intentions for mother and baby cannot always save lives. Sherry Nicholson was almost 29 weeks pregnant when she and her husband, Phil, learned that their baby, Sean, had a lung mass that would almost certainly kill him in utero. --The decision to try experimental surgery was "the only thing we would do," says Sherry. "I never wanted to wonder later 'what if?'" The operation went well, but 10 and a half weeks after delivery, Sean's healthy lung gave out and he died. Despite the tragic loss, Sherry is grateful for the outcome. "We got to know him and his personality," says Sherry. "He touched a lot of lives."

Of all of the fetal surgeries performed, the spina bifida operation is the most controversial. The disease, which affects one in 1,000 births every year, is the first and only condition surgeons have attempted to treat that is not life-threatening. And the advantages to going in so early are still unclear. Vanderbilt's Bruner has performed about 200 procedures since 1997 and his own results are mixed, but encouraging. Bladder and bowel function, vexing lifelong problems, do not appear to improve after intervention. But he says babies' brains show a clear benefit. Only about half need shunts--implantable devices that divert fluid from the brain--after birth, compared with the majority of babies operated on as newborns. Samuel Armas, who will soon have bladder surgery, cannot move his feet and toes, but he gets around fine with small leg braces. Not having a shunt, says Julie, "made the surgery worth it." The NIH trial, launched this spring at UCSF, CHOP and Vanderbilt, will recruit some 200 patients who will be observed until the age of 3. Doctors hope key questions will be answered: Is fetal surgery better than an operation after birth? And just how much benefit justifies the surgical risk?

In the end, no matter what the data show or the politicians decide, some fetuses will still turn out to be imperfect, and some parents will make choices they never thought possible. "I've had patients say to me, "I marched up and down in front of [abortion] clinics, but I'm terminating my pregnancy," says Dr. Mary Norton, head of prenatal diagnosis at UCSF. Andrea Merkord and her husband, Sean, do not believe in abortion. But last year Andrea had laser surgery to cut off the blood supply to a pair of conjoined babies in her uterus. The twins were unviable, but were threatening the life of a healthy triplet. That baby, Thomas, is now 7 months old and healthy. Andrea doesn't doubt her decision for a minute--but it continues to overwhelm her. "Obviously the twins were terminated and that is hard to say," she says in tears. "Until you've been in the situation, you don't know what decision you'll make."

For Vanderbilt's Bruner, operating on the tiniest patients has had a profound effect on his professional and personal life. Initially the experience was one of pure wonderment. "We would open the uterus and everything in the OR would stop. Everyone was just standing there looking." Now, Bruner says, he feels a deep and personal connection with every fetus. "I'm the first human being who will ever touch them," he says. "I speak to every one."

Treating The Tiniest Patients | News