Changing Lives and Helping Families (before birth)

by Carole Bartoo

At a time when most new parents are picking out paint for nurseries and getting ready for baby showers, Amber and Dustin Stewart were faced with decisions about surgeries and treatments for their unborn child.

Four months into the pregnancy, an ultrasound image of their daughter’s tiny chest revealed a life-threatening growth. Malformed lung tissue was crowding the development of her airway and putting pressure on her heart.

The Stewarts say they were not given much hope. A high-risk pregnancy expert told them that she was not likely to survive; that she might not might cry or even be able to draw a breath in the delivery room.

But a referral to Vanderbilt offered hope. Ultrasounds at the Junior League Fetal Center at Vanderbilt suggested there was a possibility their baby had enough lung tissue to survive birth. It was still difficult to tell if the Stewarts should seek experimental surgery before birth to boost her chances, or if she could wait to have surgery after.

The Stewarts longed for something so simple and expected: to hear a healthy cry after the birth of their daughter. They faced a long road to get there, but they had the staff and faculty of the leading regional fetal center at their side for the journey.

A Hub of Services

For 15 years, Vanderbilt’s maternal-fetal medicine experts have been providing guidance to parents like the Stewarts who must navigate the murky waters of decision making well before birth. The highly-trained pediatric surgical specialists offer a top-level neonatal intensive care unit (NICU) and high-quality ultrasound technology for women with high-risk pregnancies.

Vanderbilt formed the center to deliver a hub of services to women with complicated, high-risk pregnancies, and to provide the best possible outcomes for their babies. Pediatric surgeons had worked for decades to repair congenital heart defects, malformations of the gut, and lung problems at birth.

The center moved to the forefront of fetal medicine when, in 1997, Vanderbilt pioneered fetal surgery repair of myelomeningocele, or spina bifida, when a baby’s developing spine fails to close during development. The surgery could be performed while the infant was still in the womb.

At the same time, Vanderbilt made a name for itself developing the bio-ethics and evidence-based medicine surrounding the complex field of fetal medicine. It became one of only a handful of centers in the nation to offer full-service fetal care and fetal surgery for spina bifida.

A major gift from the Junior League in 2006 helped launch a fully coordinated center with an administrative staff to connect experts all over campus. The Fetal Center could draw from expertise in genetics counseling, obstetrics and gynecology, pediatric surgery, neonatology, adult surgery and specialized support staff including nursing and social work.

One of the Fetal Center’s co-directors, William Walsh, M.D., professor of Pediatrics and chief of Nurseries in the Division of Neonatology, says a remarkable thing about maternal-fetal medicine has been the speed of its growth. He recently published findings showing that, for example, heart defects have gone from an alarming diagnosis made shortly after birth, when a baby turned blue, to one of the most common defects picked up well before birth. At Vanderbilt, the rate of diagnosis of heart defects prenatally rose from 25 percent in 2002 to 65 percent in 2010.

“It helps immensely to have a diagnosis in pregnancy,” says Walsh. “The heart specialist can become involved early on, the Fetal Medicine specialists can monitor both the mother and the baby, and the family can meet us and tour the NICU. It often allows for a better outcome, but perhaps most striking is the difference we see in families who are allowed time to adjust and plan.”

A Healthy Cry

Stewart, a registered nurse at Horizon Medical Center in Dickson, Tenn., understood all too well the seriousness of her baby’s lung malformation, called Congenital Cystic Adenomatoid Malformation (CCAM), but she was not ready to accept the early prognosis that survival was unlikely.

When the referring physician called Vanderbilt, the Fetal Center fit the Stewarts in for an appointment the next day.

The Stewarts quickly learned that Vanderbilt’s skilled surgeons could remove the malformation after birth. But the family worried their baby would develop fluid build-up, a condition called “hydrops” that makes survival during delivery uncertain.

The Fetal Center team often works with other fetal centers to offer the best option to patients, so in Ashley’s case, they took advantage of a timely visit from a fetal surgeon from another hospital. The surgeon’s center in Houston, Texas, had recently used a partial delivery technique, called “EXIT” to remove a baby’s CCAM just before delivery, and the baby and mother had done very well.

Amber said moving to Houston to be followed by the fetal team there was a possibility, but the couple decided to stay at Vanderbilt. They would wait to have surgery after the baby was born, a less aggressive option that even the Texas surgeon ultimately agreed was preferable.

“It sounds strange, but we were optimistic. I felt at Vanderbilt, these were my friends; they were looking out for my baby and me. It was a very personal decision and experience for me,” Stewart said.

Vanderbilt educated the Stewarts about what to expect. Because doctors didn’t know how much viable lung tissue their daughter had, they were told that she could need an ECMO (extracorporeal membrane oxygenation) machine after birth to support her until she was strong enough for surgery.

Vanderbilt is one of the leading institutions in the country for providing ECMO support to critically ill babies.

Gracyn Stewart was born July 11, and much to everyone’s delight, let out a healthy cry in the delivery room. Her first breath of air opened up enough functional lung for her to breathe on her own. Four days later, pediatric thoracic surgeon Dai Chung, M.D., successfully removed the malformation, allowing her remaining lung tissues to fully inflate.

“After surgery Dr. Chung said ‘I promise you she will be fine.’ For the first time in my pregnancy I actually believed it. My husband reached over and hugged him – and for my husband to do that is a big deal,” Stewart said.

From Beginning to End

The Fetal Center team hopes that innovation and teamwork can find solutions for babies with life-threatening conditions and serious birth defects.

John W. Brock III, M.D., surgeon-in-chief for the Monroe Carell Jr. Children’s Hospital at Vanderbilt, says the Fetal Center is dedicated to science, research and teamwork. The center is also driven both by a deep sense of caring for these families and a desire to find more answers.

“I think the critical factor is the fact that we have a team in place that understands the process from beginning to end,” Brock said. “It’s a multi-specialty effort, and our emphasis is taking care of the whole family, from the first consultation and the explanation of the risks and benefits, to the fetal or post-natal surgery and the post natal care for both the mother and the infant.”

The center is led by three directors: John B. Pietsch, M.D., associate professor of Pediatric Surgery and surgical director of the ECMO Program; Kelly A. Bennett, M.D., perinatal director of the Fetal Center and associate professor of Maternal Fetal Medicine in the Department of Obstetrics and Gynecology; and Walsh. Each brings a different perspective to patient-centered care and science.

Walsh is part of an effort to review the existing literature and evidence about invasive fetal procedures including surgery. Findings published in the journal Obstetrics & Gynecology in May show there are too few high-quality, large studies to inform the regular use of a number of fetal procedures. The authors urged all fetal centers, including Vanderbilt’s, to proceed with caution and work to build the evidence for use of such procedures, as was done in the well-designed Management of Myelomeningocele Study, MOMS trial.

Making a Difference

The MOMS trial, or the Management of Myelomeningocele Study, showed that early closure of the spine in utero could reduce spina bifida effects, including paraplegia, bowel and bladder problems, brain swelling and even developmental delays.

Vanderbilt’s fetal surgery team includes: Noel Tulipan, M.D., director of Pediatric Neurosurgery; Mary Anne Carroll, M.D., assistant professor of Maternal Fetal Medicine; anesthesiologist Ray L. Paschall Jr., M.D.; plastic surgeon Stephane A. Braun, M.D.; diagnostic sonographer Alicia Crum and pediatric cardiologist Ann Kavanaugh-McHugh, M.D.

Since the results of the MOMS trial were made public, requests for fetal surgery have increased. The fetal surgery team now sees one-to-two new cases a week.

“It’s important not to leave families hanging,” said Pietsch. “They come from all over the country, but our goal is to see them within a week of their diagnosis.”

Ashley Hodge was one of those who contacted Vanderbilt shortly after the MOMS trial ended. She learned from her local obstetrician/gynecologist in her 21st week of pregnancy that there was an opening at the base of her baby’s spine. A search online revealed the many disabilities her unborn son faced.

“It was frightening,” Hodge recalls.

She was relieved to find the Fetal Center, just a few hours’ drive from her home in Bristol, Va. She and her baby’s father, Travis Andrews, traveled immediately to Vanderbilt.

Because success rates are best when the spine is closed between the 21st and 25th week of pregnancy, Hodge had to decide quickly. She opted for fetal surgery, so on June 15, at 23 weeks gestation, Tulipan closed the baby’s spine and Carroll safely closed the baby boy back into his mother’s womb.

After surgery, Vanderbilt allows women to return home if the pregnancy is progressing safely. This is not offered at many fetal surgery centers, but the Vanderbilt Center believes strongly that at home stress is generally reduced and there is a sense of normalcy for families. There also may be other children, like in Hodge’s case, to look after and jobs to retain.

The Fetal Center watches each pregnancy by working with specialists in the patient’s hometown. Each week, doctors contact the patient and review ultrasounds, and if any problems are detected, patients are advised to return to Vanderbilt.

Six weeks after her fetal surgery, Hodge began to experience premature contractions. Vanderbilt sent a special transport plane to bring her, 30 weeks gestation, back to Nashville as quickly as possible.

Hodge spent five long weeks on bed rest at Vanderbilt.

“It was difficult for her,” Bennett said. “She had to remain on bed rest so that her son would not have to deal with both the spinal defect and underdeveloped lungs.”

Camden Ace Andrews was born healthy and kicking on Aug. 23 via cesarean section.

“We made it to 35 weeks,” Hodge says with pride. “Camden did great. He was only in the NICU for four days before we were able to take him home.”

Hodge says there is no evidence of fluid build-up in her son’s brain, or of a commonly coinciding defect of the brain called Chiari malformation. Camden was born with clubfeet, so he has casts, and the casts have offered an encouraging observation.

“He’s been able to kick and raise his legs even with those heavy casts on. His physical therapist has cared for other babies with spina bifida, and even she is amazed how well he’s doing,” Hodge says.

She, like all who have come through the doors at the Fetal Center in the last 15 years, is well aware babies are not cured of their defects and there will be hurdles to cross as the babies grow. But Hodge says the efforts made by Vanderbilt’s team to keep her fully informed and a part of the decision-making process created a level of trust absolutely required by parents, and it made the team a part of her family.

“Finding Vanderbilt made a big difference and it will through his whole life,” Hodge said.

To see a video about one family’s experience with the ground-breaking fetal repair surgery for spina bifida, visit www.childrenshospital.vanderbilt.org/hope