The rain forced Janaya Moscony to slow her usual breakneck pace as she drove her black Audi convertible to Paoli Hospital.

She was nervous about the big needle they would stick in her belly for the amniocentesis. What if it hit the baby? Was it worth taking a chance, even a small one?

But she was 35, an age that technically made the pregnancy "high risk." So she had taken a few hours off that Tuesday morning last September to get the test, just to be sure everything was OK.

Her financial clients could wait. Besides, Janaya didn't have to be at her home office in Phoenixville to stay connected. She always carried her BlackBerry.

Janaya signed in for the test, checked her e-mail one more time, and nervously took a seat in the waiting room.

Within minutes, she was following the receptionist down the hall to the exam room.

She didn't have to undress. She lay down on the examining table and bared her belly. They would do an ultrasound first, the technician told her.

Do you want to know the sex? she asked Janaya.


She was 16 weeks pregnant. The technician looked at the screen and paused. I can't tell. Let me get the doctor to check, she told Janaya.

Moments later, the doctor came in and repeated the ultrasound.

It's a boy, he told Janaya. And then the hammer.

There's a problem.

What? What did he mean?

The doctor was matter-of-fact: The baby had a birth defect. His intestines appeared to have spilled out of a hole in his abdominal wall.

The doctor told her it was probably something called gastroschisis or possibly omphalocele, a similar defect.

She started to shake. She tried to hold back, control her emotions, but she could not.

Janaya began to cry.

You need to see a specialist, the doctor said. He mentioned Children's Hospital of Philadelphia. Some women, he was telling her, consider termination at this point.

Her mind was reeling.

Would her baby live? Should she end the pregnancy? Could her baby have a normal life?


Janaya couldn't sleep.

Instead, she sat at her computer, searching for information, studies, anything that might help her understand what was going on - and decide what to do.

Research was something she was good at.

She discovered that most gastroschisis cases involve younger women - those in their late teens or early 20s.

So maybe it wasn't that.

Older mothers were more likely to carry babies with omphalocele. But those babies were at higher risk of having other birth defects.

She was overwhelmed. Could the baby have even more problems?

Janaya had never contemplated any of this when she and her partner, John Lukan, decided they wanted to have a baby.

It was a remarkable leap, considering their rough start.

They'd met in 2003, when the finance business John ran hired Janaya's newly formed consulting firm to perform a "mock" audit. He wanted to make sure his company was complying with federal securities law.

John insisted on quick black-and-white answers. But Janaya, who lived in Hawaii at the time, explained to him repeatedly that there were only shades of gray.

Janaya thought he was a jerk, just a pushy CEO. She knew what she was doing.

The next year, John hired Janaya again. By then, Janaya, who had grown up in Drexel Hill, had moved back to the area. Again, they didn't get along. But at a business dinner meant to smooth things over, John's interest moved from the professional to the personal.

He had separated from his wife in 1999. Janaya was single.

Six months later, they went on a date: dinner at Buddakan. The relationship grew from there, and when Janaya bought the house in Phoenixville in November 2005, John moved in.

John, 11 years her senior, reversed his vasectomy in January 2007.

When months passed and they still didn't get pregnant, Janaya got a prescription and took a fertility pill.

Problem solved.

But this problem with their baby defied her ability to fix things. She hated feeling helpless. She couldn't find a quick answer, and the questions destroyed any hope of sleep.

Had she done something to cause this? Her hair dye? The stress of running a business? The ups and downs with John?

She'd found one glimmer of hope online, however: Children's Hospital of Philadelphia.

Its doctors specialized in diagnosing and treating birth defects, including gastroschisis and omphalocele. CHOP had recently opened a unit where, for the first time, mothers could deliver babies with birth defects.

Typically, even babies with known birth defects are delivered at regular hospitals. The sickest are then transferred to pediatric hospitals for specialized care. The $20 million Special Delivery Unit made it possible to deliver babies at CHOP.

The first baby born in the SDU arrived June 10, 2008. He had spina bifida - an exposed spinal cord.

Janaya learned that the eight-bed labor and delivery unit brought together CHOP's expertise in research, fetal medicine, and surgery to provide everything from prenatal diagnosis and delivery through neonatal treatment.

She grabbed the first available appointment, 7:30 a.m., Wednesday, Sept. 17.

A week to wait and wonder.

A diagnosis

Janaya felt frazzled but determined as John drove them to CHOP's Center for Fetal Diagnosis and Treatment.

Guards directed them to the elevators. Doctors waited and held the doors for all the patients to enter first. Janaya was struck by the staff's courtesy.

A nurse ushered them into an exam room.

Janaya could see that the ultrasound equipment was newer and looked more sophisticated than what had been used the previous week.

First, the doctors looked at her baby's heart.

It checked out.

Next they ruled out spina bifida. His brain was developing normally. He had no obvious kidney problems.

His lungs looked good, too.

The doctors told her it was gastroschisis. That was bad, she knew, but at least the baby didn't have a host of other problems.

The doctors examined the intestines, floating in the dangerous amniotic fluid that would inflame the delicate tissue not meant for such exposure. That could cause long-term damage.

They checked the blood flow and looked for blockages and lesions.

No one could say what had caused the birth defect. Gastroschisis occurs in one in 2,500 births and is rising markedly, suggesting an environmental trigger for an underlying genetic problem.

At the end of the day, however, CHOP's doctors had a plan based on experience with hundreds of such cases.

Janaya would come for monthly checkups at first. Then, around her 32d week, when the baby's danger of life-threatening complications would grow, twice a week.

And, with the addition of the SDU, they would deliver the baby at CHOP.

The doctors explained that even with gastroschisis, a vaginal delivery would be better than a C-section, as it is for most babies.

Making sure the baby survived to birth would be the first hurdle.

Nationally, about 10 percent of gastroschisis babies die before they are born. But at CHOP, with close monitoring for signs of fetal distress, the risk to Janaya and John's child was lower.

Four decades ago, when IV nutrition was in its infancy, almost all gastroschisis babies died. Moreover, for many years the problem wasn't recognized until the baby was born, complicating care.

Today, with modern ultrasound technology, almost all cases are discovered months before birth.

The doctors at CHOP have developed protocols for taking care of these babies before and after birth. Plus, they treat 30 to 40 such patients a year, yielding a 95 percent survival rate, compared with a national rate of about 85 percent.

Once born, Janaya's baby would be immediately handed over to a team of neonatologists and surgeons, who would start the slow process of putting his intestines back into his body.

Even then, the doctors explained to John and Janaya, the baby would face a long course of care with risks of serious - sometimes fatal - setbacks.

The key would be how well the newborn's inflamed intestines recovered from months of exposure to amniotic fluid. Any severely damaged segments would need to be removed. If too much of the intestines were lost, the prognosis would be bleak.

For gastroschisis babies, blockages and infections are major concerns. Necrotizing enterocolitis, or NEC, happens when poor blood flow damages the intestines, leading to serious infections. It occurs in as many as one in five such babies after birth.

Because the baby's abdominal cavity would be too small to absorb the intestines all at once, it could take weeks. The intestines would be inched in through the same hole they had spilled out of. Then it was a relatively simple "surgical closure" to sew him up.

Janaya was glad to have a plan.

But something nagged at the back of her mind for the hour-long drive home to Phoenixville. She was sure all the doctors at CHOP were very good. She just wanted the best one.

She jumped online as soon as she got home.

The next day she called CHOP's surgeon-in-chief, N. Scott Adzick, a pioneer in fetal surgery. Could she persuade the renowned pediatric surgeon to take her son's case?

He wasn't in, so she left a message.

Still, Janaya was surprised when Adzick called her back that day.

He typically performs extremely delicate in-utero surgery to repair birth defects such as spina bifida and operations to remove lung tumors.

Janaya's insistence amused him. A surgical closure on a gastroschisis baby was simple. He was happy to treat her baby.

A quick trip

Janaya's baby wasn't due until Feb. 28, but by January she was getting antsy.

On the 21st she e-mailed Adzick to check whether he would be in town for the next month or so, in case the baby came early, as most with gastroschisis do.

"I don't mean to be intrusive, but I do prefer that you do the surgery on the baby, and I just would like to know what I can expect if there is a possibility that you will not be available," she wrote.

Twenty minutes later, she got his reply: Not to worry.

"I may be away for a weekend, but the surgical closure is invariably done on a weekday," Adzick wrote.

Still, by the end of the month, Janaya was so anxious she decided she needed to get away.

She threw a few things into an overnight bag and drove alone to her sister's house in Somers Point for a winter weekend at the Jersey Shore.

Sunday night she went to sleep early. She woke up a few minutes before 11 p.m.

The bed was wet.

She didn't think she had felt a contraction. But she was sure that her water had broken.

Was that unusual? Was it a problem? Janaya woke her sister, and they called CHOP.

I'm not feeling contractions. Should I go to the hospital here? Janaya asked the obstetrician.

No, come on in.

Janaya's sister drove.

They shot up the Atlantic City Expressway, over the Walt Whitman Bridge, and into West Philadelphia. It took less than an hour.

John, glad he had watched the Super Bowl at home that night instead of going to a party, was waiting in the CHOP parking garage when they got there.

A nurse escorted them to Room 7 in the Garbose Family Special Delivery Unit - the SDU.

It was a few minutes after midnight on Feb. 2.

A soft cry

Four-and-a-half hours into her labor, Janaya asked for an epidural.

She'd been able to endure the pain, so far. But she wasn't so sure about the next contraction. She was afraid she wouldn't be able to handle what was coming, couldn't control it.

It was 3:30 a.m.

Five hours later, she was at 8 centimeters. The nurse called down to the neonatal intensive-care unit - the NICU - to alert the team of neonatologists, surgeons, nurses, and respiratory therapists.

The team began to assemble in the starfish room, the nickname for the infant-stabilization room next door to where Janaya lay.

At 10:30 a.m., she was fully dilated.

Time to push, the midwife told her. Next door a warmer was prepped for the newborn.

Every few minutes, a member of the NICU team glanced through a window into the delivery room, checking on the progress of baby and mother.

The delivery was easy, for a first child. Janaya felt as if she pushed just once and then heard Makoa John Lukan-Moscony announce his arrival with a soft cry.

It was 10:49 a.m. The quicker the treatment began, the better the chance to limit infection or further damage to the intestines.

Janaya was exhausted. She cast a fleeting look at her baby before the midwife passed him through the window and gently placed him on the waiting scale in the starfish room.

Makoa - which means fearless in Hawaiian - weighed in at 4 pounds, 12 ounces.

John distracted himself by snapping away with his digital camera.

He'd been prepared by the doctors and had seen pictures of babies with gastroschisis on the Internet. Still, he was amazed at the sight of his son. The huge, purplish mass of plump sausagelike intestines looked impossibly large for such a tiny baby.

A nurse quickly lifted Makoa off the scale and moved him into the waiting warmer.


For Janaya, Makoa's delivery was the easy part; it was more painful to watch through the window as the NICU team worked on her son.

A neonatologist quickly wrapped Makoa up to his chest in a plastic bag, dampened with a warm saline solution to protect his exposed intestines - all four feet of them.

Team members cleaned him off, then threaded a thin tube down his throat into his stomach, which had partially come out of his abdomen during the birth. The tube would suction bile to prevent any waste from getting into his already stressed intestinal tract.

At 11:02 they placed an oxygen mask over his nose to help him breathe.

John continued to take pictures, watching the nurses and doctors for any sign of surprise. Since they acted as if everything was normal - at least for a gastroschisis patient - he was able to keep his parental panic in check.

At 11:11 a nurse finally got an IV into Makoa's left arm to keep him hydrated and maintain his blood sugar level. It would likely be weeks before his intestines recovered enough for him to eat anything.

Makoa's breathing had improved, and they reduced the oxygen mix. By 11:30 he was getting regular room air pumped through his mask.

A few minutes later they rolled his warmer out the door and into the delivery room to see his mother.

Janaya stretched her hand slowly, tentatively, toward her newborn son.

She brushed her baby's arm and then rested two fingers gently on his forehead. She was not allowed, of course, to pick him up.

She felt the cataclysmic pull of new parenthood: instant love, and terror.

But this wasn't the time for bonding. Janaya just wanted her baby put right.

After a few minutes, the doctors took Makoa away, down to the second-floor NICU.

It was 11:35 a.m. on Monday, Feb. 2.

88 minutes

Makoa, born a month prematurely, was the 111th baby delivered at CHOP and the 12th with gastroschisis.

The Special Delivery Unit - the only one of its kind in the world when it opened last summer - was built for babies like Makoa.

It would speed the start of treatment and allow mothers and their newborns to stay together.

Makoa had arrived in the NICU within an hour of his birth.

That was faster than would have been possible had he been born next door, at the Hospital of the University of Pennsylvania. It was hours sooner than if he'd come from any other hospital.

Each year about 200 newborns - the sickest in the Philadelphia region - are rushed to CHOP's NICU from other hospitals.

Yet the quicker a distressed newborn like Makoa is treated, the better his chances.

In the NICU, four floors below the room where Janaya lay in recovery, a team of doctors, nurses, and therapists went to work on the baby.

Specialists quickly began the long, inch-by-inch process of putting the intestines back into Makoa's little body.

They inserted a second IV, this one in his right arm.

Then Adzick, assisted by two other surgeons, carefully stuffed the intestines into a soft plastic condom-like tube called a silo. The silo was hung directly above the baby's belly.

At 12:17, Adzick was finished. It had been 88 minutes since Makoa's birth.

Above his bed, a monitor tracked the baby's heart rate, his breathing, and the amount of oxygen in his blood. The warmer was set to come on automatically if Makoa's temperature dipped.

Two hours later, Janaya, still groggy from her labor and dressed in a loose hospital gown, came down to see her son.

She still couldn't pick him up, but she could soothe him - and herself - with a stroke of his forehead, a kiss on the cheek, a soft whisper in his ear.

Hours later, after grabbing some sleep, some food, and a shower, she returned to the NICU.

It was 3 a.m., and the 70-bed unit was quiet.

She pulled a chair up next to Makoa's warmer and looked down at her son.

She didn't want to wake him, but couldn't stop herself from putting a hand on his head. She watched intently, the rise and fall of his chest, the reflexive spasms in his legs, the small movements of his red lips.

Janaya couldn't take her eyes off him.

The fit

She was still at her baby's side the next morning when Adzick came through to check on Makoa. John was with her, having gone home the night before to get a couple of hours rest and pack for a prolonged hospital stay.

He looks good, Adzick reassured the anxious parents.

Another surgeon gently squeezed the top of the silo containing Makoa's intestines. That pressure, aided by gravity, pushed a bit more of the intestines back to where they belonged.

Using a white, blue-striped twist tie, the doctor cinched closed the silo just above the topmost portion of intestine.

Suddenly, everything slowed down.

Janaya and John settled into the small, windowless alcove that would serve as an interim "home" inside the NICU. It was not exactly how they'd dreamed of starting their family, but they were grateful to be at their son's side.

Janaya focused on doing whatever the experts at CHOP told her to do to help her baby. She had already started breast-pumping.

At first came the colostrum, which she would dab on Makoa's lips in hopes he would benefit from its immune-building powers. But within a day she would be pumping every three hours, marking the date and time on each plastic bottle.

She would do it for weeks.

Each day during surgical rounds, the doctors checked Makoa and pushed down the intestines a little bit more. The twist ties marked their daily progress. A couple of inches one day, a bit more the next, less the following one.

At this point, there was no rush.

Makoa's abdomen hadn't expanded properly during gestation to accommodate his intestines. Now it grew bigger little by little as more was put back inside.

On Sunday morning, six days after he was born, the job was nearly done. Adzick scheduled Makoa for surgery the next day at 7:30 a.m.

Inside the operating room, Adzick removed the silo, gently pushed the final bit of intestine into the abdomen, and began to suture up the hole. He made sure to repair each layer from the muscles through the skin, and he was careful to preserve the belly button - because, he says, every child should have one.

The baby was back in the NICU by 9:30, sedated and intubated.

Two days later, when the doctors removed the breathing tube, Makoa's rough breathing and hoarse cries struck Janaya like blows.

Is he OK? Why is he crying? Is he in pain?

She couldn't live with that.

Demetra Exarhoulakos, the 23-year-old NICU nurse who had spent much of the last week with the family, quietly worked to calm Makoa and reassure his parents that he was OK.

Later that morning, the nurse gently suggested it was time for Janaya to hold the baby.

That will calm him, she said.

But Janaya was still worried.

He is too fragile. I might hurt him.

She pumped more milk.

Around noon, the nurse asked Janaya again if she was ready to hold the baby.

No, not yet.

At 1:20 p.m. Exarhoulakos grabbed a robe out of the cupboard and handed it to Janaya.

Skin to skin is best, said the young woman.

As the hesitant 35-year-old mother removed her top and put on the loose-fitting robe, Exarhoulakos untangled the wires and tubes connecting Makoa to the monitors, nutrition, and medications.

The nurse lifted Makoa out of his warmer and handed him to his mother.

The baby howled. His heart rate soared, and the monitor rang out.

"I'm sorry, baby, so sorry. . . . I'm sorry," Janaya whispered in his ear.

"Is he comfortable? Are you sure he's OK? How's he look?" she asked.

"Give him a little time," Exarhoulakos said.

In moments, Makoa quieted, as did the heart monitor.

He laid his head on Janaya's chest, closed his eyes, and fell asleep.

She looked up at John and down at their sleeping baby.

Janaya found herself smiling. He was 7 days old. To her surprise, her breasts filled with milk, a nursing reflex she'd heard about but hadn't experienced yet.

One more thing that was out of her control.

She could live with that.


Four days later, Makoa was cleared to get his first bottle. He sucked down 5 milliliters of his mother's stored milk. She fed him.

After a week, Janaya was able to breast-feed her son.

He was discharged at 1:04 p.m. on Feb. 24, after 22 days.

Today, Makoa is making a steady recovery and eating well.

He weighs nearly 11 pounds.

 More photos of Makoa John Lukan-Moscony and his care at CHOP are at