ROBERT SIEGEL, host:
From NPR News, this is ALL THINGS CONSIDERED. I'm Robert Siegel.
MICHELE NORRIS, host:
And I'm Michele Norris.
Today, in our series "Beginnings," how doctors are correcting birth defects before birth. Among newborns in America, one-third suffers from a birth defect. Until recently, the most severely affected babies faced a short life or a life of severe limitations. But that's changing for some.
NPR's Richard Knox takes us into an operating room where surgeons are correcting one of the most common birth defects by operating on a child who is not yet born.
RICHARD KNOX: Sarah White is lying on an operating room table at Children's Hospital of Philadelphia. She and her doctors have given us permission to watch what's about to happen.
Dr. DAVID COHEN (Anesthesiologist, Children's Hospital of Philadelphia): Can you take some real deep breaths? We're going to take very good care of you.
Ms. SARAH WHITE: OK.
DR. COHEN: Nice, deep breaths.
KNOX: She doesn't need surgery, but doctors are about to cut into her abdomen to operate on her 22-week-old fetus.
Ms. WHITE: I've never had surgery, so it's pretty terrifying for me.
KNOX: A routine ultrasound has found her fetus has spina bifida. It's a condition that conjures up images of wheelchairs and leg braces, physical and mental handicaps.
Joe Hensley is Sarah's husband.
Mr. JOE HENSLEY: I did not know much about spina bifida. So when they said, your baby has spina bifida, I knew it wasn't good.
KNOX: In fact, it's a hole in the spine. The spinal column fails to close properly, leaving the spinal cord exposed and vulnerable to injury.
Neurosurgeon Lee Sutton says doctors now realize that most of the damage occurs late in pregnancy.
Dr. LEE SUTTON (Pediatric Neurosurgeon, Children's Hospital of Philadelphia): We were seeing all these kids that were coming in here unborn - you know, little fetuses; 18, 19 weeks - and they were moving their legs beautifully on the ultrasounds. And then when they would get delivered at 38 weeks or something like that, they had club feet and they were paralyzed.
KNOX: So the aim of fetal surgery is to close the hole before the damage is done.
Dr. SUTTON: Because once the damage is done, it's done. There's nothing that any of us can do to reverse the paralysis.
KNOX: Ultrasound tests before the operation show Sarah White's fetus is still kicking, and she's going to have a boy. So if they operate now, there's a chance they can keep those legs moving and her son won't be paralyzed.
Joe says he and Sarah know it's not a guaranteed fix, but they'd never forgive themselves if they didn't give their child the chance to walk.
Mr. HENSLEY: We would probably look back and say, wow. Why didn't we do it? Why didn't we try?
KNOX: Still, it wasn't an easy decision. This is high-risk surgery. They could lose the baby, and Sarah could die.
Dr. SCOTT ADZICK (Director, Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia): How's the mom doing, David?
Dr. COHEN: What?
Dr. ADZICK: How's the mom doing?
Dr. COHEN: Fine.
KNOX: That's Scott Adzick, chief of surgery. He begins the operation.
Dr. ADZICK: We're cutting through the abdominal wall structures now, first the skin with the knife.
KNOX: Through skin, fat and muscle until he gets to Sarah's uterus. At nearly six months of pregnancy, it's the size of a kid's soccer ball.
Dr. ADZICK: And then we're actually going to tip the uterus out of the abdomen. It's still attached, of course, on the bottom part.
KNOX: Once Sarah's uterus is almost entirely outside her body, Adzick takes a marker and outlines where the placenta is. If the surgeons cut into the placenta by mistake, Sarah could bleed to death. Adzick carefully cuts into the pregnant uterus, using a special stapling device.
Dr. ADZICK: ...the other one.
KNOX: A few more staples, and they've opened a small window.
Dr. ADZICK: And the cut is about three inches in diameter, and that allows us to expose the fetus. And here's the fetal bottom.
KNOX: It's an amazing sight. The six-inch-long fetus, his back facing up into the glare of the surgical lights.
Dr. ADZICK: There's the umbilical cord, you've got a glance of that. It's really beautiful.
KNOX: The object of the whole operation is an odd, bluish bubble on the fetus's lower back. It's a fluid-filled sac of fragile tissue over the hole in the spine.
Dr. ADZICK: Can we have the suction tip, please?
KNOX: So here is the enemy. Here is what you're trying to fix, right?
Dr. ADZICK: That's the enemy. It's the myelomeningocele or spina bifida sac, which bulges way out.
KNOX: Spinal fluid leaks out of this thin membrane. That causes the bottom of the brain to sink down and become dangerously wedged in the fetus's neck. And amniotic fluid seeps into the sac, directly onto the developing spinal cord. That's what causes lifelong paralysis.
Dr. COHEN: Heart rate, about 155.
KNOX: At this point, Lee Sutton, the neurosurgeon, steps up to the table. He cuts into the sac. Spinal fluid gushes out. Adzick points out the hole underneath.
Dr. ADZICK: The spinal cord is right there. It's like a little, tiny ribbon of tissue. It's a little bit yellowish. And there's a little blood-staining there.
KNOX: The hole is only about an inch across. Sutton needs magnifying lenses to work. He takes a curved needle and starts the delicate process of closing the defect, using layers of tissue and skin.
Dr. SUTTON: I remember when I did my first one, when this was, you know, just being pioneered. And I mean, I was just awestruck with it. And I was doing it.
(Soundbite of laughter)
Dr. SUTTON: And I still get a real, you know - kind of a shiver, almost, when I do one of these.
Dr. ADZICK: Things are right?
Dr. SUTTON: Yes.
KNOX: The repair has to be watertight so spinal fluid doesn't leak out, and amniotic fluid doesn't get in. The stitches are teeny, tight and even.
Dr. ADZICK: Well, it looks a little bit like the laces of a miniature football.
KNOX: After 10 minutes, the surgeons are satisfied with the repair.
Dr. ADZICK: So we're getting ready now to close the uterus.
KNOX: The surgeons stitch together Sarah White's uterus, and put it back inside of her. Then, layer by layer, they sew her abdomen back together muscle, fat, skin - until only a fine-line scar remains.
Dr. ADZICK: Thanks, everybody.
Dr. SUTTON: Thank you.
Dr. COHEN: Thank you.
KNOX: A little more than an hour after it began, the surgery is finished, and Adzick steps away from the table.
Dr. ADZICK: Yeah, it went very well. I'm very pleased. No drama. Boring. But that's the way we like it.
KNOX: But while the operation is over, the suspense isn't. Sarah White could go into premature labor. That could be disastrous. So she'll have to stay in bed for the next three and a half months, and take drugs to calm her traumatized uterus. If all goes well, doctors will deliver her baby by Caesarean section at 37 weeks - just a little short of a normal pregnancy.
After the baby is born, there's more watching and waiting to see how the child develops. Only then will the family and the surgeons know if it all worked.
Neurosurgeon Lee Sutton says he's hopeful, partly because this spina bifida defect was low on the spine.
Dr. SUTTON: That's good. So, you know, I think this kid's going to do very well.
KNOX: A study earlier this year showed fetal surgery for spina bifida often works. Children who had fetal surgery were twice as likely to walk as those who didn't have surgery until after they were born. But not all of them could walk, and many had bowel and bladder problems common with spina bifida or other difficulties. Still, sometimes, the outcome is truly amazing.
Mr. SEAN MULLIGAN: I'm Sean Mulligan, and I'm 10 years old.
KNOX: Sean Mulligan was one of the first children who had this experimental surgery, more than a decade ago. The only outward sign is a six-inch scar on his lower back.
Mr. MULLIGAN: I had surgery on my back. My spine was open, my back.
KNOX: And they fixed it, huh?
Mr. MULLIGAN: Yeah.
KNOX: If they hadn't fixed it, what would your life be like?
Mr. MULLIGAN: I know I'd be in a wheelchair. I wouldn't be able to walk and play sports, like I do now.
KNOX: You feel lucky.
Mr. MULLIGAN: Yeah. I do. A lot.
Unidentified Soccer Coach: All right, ready? Let's go. There you go. There you go, Sean. Good header.
KNOX: Sean's mother, Katherine Mulligan, and I are standing on the sidelines, watching him play soccer.
Unidentified Soccer Coach: It's also back there. Get it. Get it! Save it. I mean -oh, well done.
KNOX: She says he got honors in school this year. But his first love is sports.
Ms. KATHERINE MULLIGAN: He just keeps going and going. If he's out here playing, he does not give up. And I love that, too. I love the attitude.
KNOX: She says nobody watching these kids could pick out the one with spina bifida.
Ms. MULLIGAN: I challenge anybody to come out here on this field and say, oh, it's Sean. They wouldn't pick him. It just gives me goose bumps.
Unidentified Soccer Coach: Go, Sean! Oh, goal! Nice. Good work, Sean.
KNOX: About 1,500 babies are born with spina bifida in this country each year. Until recently, parents had only two options: Terminate the pregnancy, or wait until their baby is born to do surgery.
But as of this year, fetal surgery for this problem is no longer considered experimental. So soon, more children will have the same chance Sean Mulligan got.
Richard Knox, NPR News.
NORRIS: And you can learn more about fetal surgery, and see photos from Sarah White's operation, at npr.org.
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