Page Two

 

Spina Bifida Baby

(the story of Daniel, our #1 open procedure patient)

By Nancy Humphrey

A Nashville infant born with spina bifida may have a more normal life, thanks to a first-of-its-kind surgical procedure performed while he was still nestled in his mother's womb. The groundbreaking procedure — performed at Vanderbilt University Medical Center — involved opening the mother's abdomen and uterus, partially removing the fetus and sewing shut the opening over the fetus's spinal cord. Previous fetal surgery for spina bifida at VUMC and elsewhere had been performed endoscopically.



VUMC is one of only three medical centers in the United States doing open fetal surgery, and it is the only institution in the world performing such surgery to repair spina bifida lesions on a fetus still in its mother's womb. The infant, Daniel Meyer, born June 12 to Cory and Scott Meyer of Nashville, still suffers from marked weakness in his lower body, but is able to flex his hips and appears to have good bladder and bowel function.

Spina bifida is the most common neurologic birth defect in the United States, occurring in 1.5 to 2 out of every 1,000 live births. Babies born with the defect have an opening in their spine, and although it is not considered to be a lethal disorder, in most cases it is severely handicapping. Babies typically have lower extremity weakness or paralysis, sensory loss, dysfunction of urinary or bowel functions, and are prone to develop hydrocephalus, or the increase of spinal fluid in the brain.

About 95 percent of babies born with spina bifida require a drainage shunt placed in the brain to help remove excess fluid. Dr. Joseph P. Bruner, assistant professor of Obstetrics and Gynecology, is optimistic about the results of the operation, even though Daniel did also require a shunt. Bruner is part of a team of VUMC researchers — which includes Dr. Noel B. Tulipan, associate professor of Neurosurgery, and Dr. William F. Walsh, associate professor of Pediatrics — testing the feasibility of the pioneering technique.

The research is a natural progression from animal studies in the mid-1980s at other institutions that looked at the exposed spinal cords of rats and monkeys and the abnormal development of the spinal cord due to those openings. "From these studies, researchers came up with the 'two hit' hypothesis for neurological deficits with spina bifida," Bruner said. "The first hit is the fact that the fetus has spina bifida and the spinal cord is developing in an abnormal fashion. The second hit results from the prolonged exposure of the spinal cord to amniotic fluid. These studies have been repeated in sheep and pigs and show the same thing. If you protect the spinal cord from the intrauterine environment, the baby will develop more normally. If you expose the spinal cord to the fluid, the baby will develop neurologic lesions."

This led Vanderbilt researchers first to the animal labs, then to a human study three years ago for the first attempt at fetal surgery to protect the spinal cord from the possible damaging effects of sustained exposure to amniotic fluid. That technique, now replaced by the new technique performed on Daniel, was used four times during the past three years.

Endoscopic surgery using the earlier technique was done in the second trimester, at 22 weeks of pregnancy. The spinal cord was repaired by covering the opening in the spine with a skin graft taken from the leg of the mother. The graft was sealed in place with "biologic glue" made from fibrin from the mother's blood and thrombin, a substance derived from cows.

During the procedure, Vanderbilt surgeons removed amniotic fluid surrounding the fetus and replaced it with carbon dioxide to allow them to work inside the uterus with an unobstructed view. Of the four repairs using the previous technique, one child is almost three. He suffers from only a mild weakness in his ankles. One child is four months old with weakness in one leg. Two others have died. In the meantime basic laboratory research at VUMC has shown that the major damage from amniotic fluid occurs late in pregnancy.

"We're a long way from knowing that intrauterine surgery improves outcome. Prior studies showed that only 6 percent of children were mildly affected. Compare that to our series where two-thirds have been mildly affected and it's encouraging," Bruner said. "But the numbers are too small at this point to prove anything."

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The following story about Joseph Llerena is by Katie Clark, M.D.

 

Hernan Llerena and Merlith Torres certainly took a 'leap of faith' by coming to Vanderbilt for a spina bifida repair for their baby in utero.

Just imagine yourself...in an unfamiliar country, you don't speak the language, and then you find that your baby, that hasn't even been born, "tiene una enfermedad: bifida del espina. No hay nada que pueden hacer ayudarles hasta el nino es nacido menos una cirugia experimental." Difficult to understand, isn't it? This translates into "your baby has spina bifida and there is really nothing we can do until your baby is born except an experimental surgery." In addition, the surgery was only offered in Nashville--a city far from their new home and it would be done by a surgeon they had yet to meet. Despite all of this, they chose to take that 'leap of faith.'

Every aspect of this experimental experience was relayed to them through me, their translator. The technical details of the surgery, the experimental nature of the procedure, the possible outcomes, and the ethical and religious issues all were intensely discussed. After speaking by phone several times with Hernan and Merlith, they arrived in Nashville for three days of preliminary counseling.

First, we went to the Hospitality House where they would stay until Merlith was admitted to the hospital. The manager of the house was quite worried they wouldn't understand the rules of the house since they only spoke Spanish. They understood with translation and found it humorous that the manager didn't want them to stay in the same room since they had different last names and probably weren't married. Little did the manager know it is customary for Hispanic women to keep and use their maiden name after marriage.

Throughout the long days of discussion, they remained enthusiastic and firm in their decision to proceed despite various doctors vividly describing just how it would be:

Dr. Bruner described the surgery in detail and explained that they didn't know if the surgery would be beneficial or not.
Dr. Walsh described what it might be like to have a baby born at 28 weeks gestation, in the intensive care unit, and on a ventilator for the first few months of its life.
Dr. Zaner, an ethicist, discussed the ethics of such an experimental procedure, thoughtfully questioned their decision and gave enlightening perspectives on the entire situation.
A Catholic priest came to counsel the couple on religious issues at their request. I was pleased that this priest also spoke Spanish, as religious thoughts and ideas are difficult to translate.)
A financial advisor came and reviewed with the couple the economic side of the procedure.

Translating such important details, to help Hernan and Merlith make one of the most difficult decisions of their lies, was a true joy. Despite the language barrier, they were always optimistic and never wavered in their decision to try to help their baby. Finally their faith paid off and they endured a successful surgery at Vanderbilt. The worried lines on their faces seemed to fade each day that Merlith and the baby recovered. This sign didn't have to be translated into any language.

Through phone conversations with the couple in New Jersey, I know that they have had a beautiful son. He is named Joseph after Dr. Joseph Bruner. He was delivered precipitously, "por suerte, por ciencia, or por Dios" (by luck, by science, or by God) and the family are living happily in their new country. It was a true 'leap of faith.'

 

Fetal Surgery Babies Return to VUMC

By Nancy Humphrey

Sixteen-month-old Nicholas Garcia was quite smitten with Eileen Vrabcak, RN., interim manager of gynecological surgery, at a luncheon last week at The Vanderbilt Clinic. He laughed. He stood on his father's legs and bounced. He alternated eating a chocolate chip cookie and laughing at Eileen's big smile. To Vrabcak he's much more than just an adorable, brown-eyed toddler. He's a miracle.

Nicholas was one of the first babies in the world to undergo pioneering fetal surgery to correct spina bifida in utero. He was the third baby to undergo the procedure in October 1997 and was born five weeks later. He and five other babies, ranging in age from 32 months to 22 months, returned to VUMC last week for thorough clinical evaluations by a team of Vanderbilt physicians and other health professionals. Over the next six weeks, 25 families will return.

Vrabcak, who has coordinated the surgeries for most of the fetal surgeries, had seen none of the infants since they were born. Only a small portion of their backs had been visible during the procedures. Seeing them smiling, bouncing and walking last week made her cry. "Seeing these babies is amazing. It touches my heart," Vrabcak said. "To see these babies and their Moms is a miracle. It's both an ending and a beginning. It's a closure for the surgery and a new beginning for their lives."

Joyce Garcia of Jersey City, New Jersey said she is very happy and very optimistic about her Nicholas' progress. He is doing very well. He started walking about two months ago. "We are thrilled."

The babies underwent X-rays, ultrasound tests and exams during the reunion visit. The Spina Bifida Association of America is paying for the travel, housing and meals for the group. "The reunion gives Vanderbilt a chance to obtain a consistent review of the infants' progress," said Delia Nickolaus, RN, case manager of Pediatric Neurosurgery.

Last week's group included Daniel Meyer, now 22 months old, the first baby to undergo the intrauterine myelomeningocele repair in April 1997. The procedure is performed by Drs. Joseph P. Bruner, assistant professor of Obstetrics and Gynecology and director of Fetal Diagnosis and Therapy and Noel Tulipan, associate professor of Neurosurgery.

Zane Gardner, four months old, was the eighteenth baby to undergo the procedure in September. Born Nov. 12, he is doing well, his parents said. "His legs, feet and ankles are all moving. He has no bowel or bladder problems," said Todd Gardner, the baby's father. "My boy is going to walk. He's going to use the normal public bathroom like everyone else. We are helping him be as normal as he can be. What else can we ask for?"

 

Zachary’s Story

Written by Scott and Lisa Ritter

At 19 weeks gestation a routine ultrasound revealed that our first child, Zachary would be born with the birth defect known as spina bifida. My husband Scott and I were crushed. In the two short years that we had been married, we’d suffered through a miscarriage and a near fatal ectopic pregnancy. We had thought that the worst was behind us. Little did we know, the adventure was just beginning.

Three days after the diagnosis my doctor, Diedre Russell called to see how we were doing. I told her that we were coping. After all, my husband and I knew nothing about spina bifida. We collected multitudes of technical information, but we hadn’t digested it yet. We were still in a state of shock. On this day, however, Dr. Russell was the bearer of some good news. She explained that there were specialists at the hospital who had developed a procedure to repair the spinal defect. I was elated! It was almost an answer to our prayers. I listened closely as she briefly described what was involved. We were looking at an endoscopic procedure to cover the defect in-utero. They theorized that the amniotic fluid was causing most of the neurological damage characteristic of spina bifida. Their completed study demonstrated that covering the defect minimized the neurological damage. How shocked we were to find out they had never before attempted this on humans. The statistics we used to base our decision on would be taken from fetal sheep and rats!

When I finished with Dr. Russell, I immediately called Scott at work. He hesitated at the thought of being the first human test case, but remained excited about the chance we had to maybe improve the quality of our son’s life. We were extremely optimistic about the research, but we still hadn’t spoken to Dr. Bruner or Dr. Tulipan (two of the doctors involved). Dr. Bruner was heading the study, but had been out of town. We were anxiously awaiting his call later on that night.

When the phone rang, I wasn’t sure if either of us would pick up the receiver. We were nervous, but we wanted all the details: the good and the bad. After all, this was the reason that I had chosen Vanderbilt for my prenatal care. I wanted the cutting edge in the event that anything went wrong with this, my third pregnancy. I have to be honest, this wasn’t what I had in mind! After a long conversation with the doctor we agreed to meet with him 3 days later in his office. In the meantime, he was having their study on fetal sheep and other supporting studies on fetal rats delivered to our house so that we could look at the research first hand.

After meeting with Dr. Bruner and reviewing the research, we continued having only positive feelings about the whole thing. We took a few days to gather our thoughts. Many long distance calls were made to our families. We needed to hear their ideas and opinions. We spent one whole evening discussing the pros and cons realistically. We literally took a piece of paper and listed the reasons to have the surgery on one side and the reasons not to on the other. In the end, the pros won out and we decided to go ahead with the surgery. But, before we told the doctors, Scott and I made a promise to one another that regardless of the outcome, we were never to look back and say, "Why did we do this?" In our hearts it was what we wanted and the reasons we agreed to it were written right there in our own handwriting. If we ever had a doubt we just pulled out our list and read it over again.

Everything made sense to us. The procedure seemed so plausible and simple. Why hadn’t something such as this been developed sooner? After all, fetal surgery was by no means a new concept. And, spina bifida was (and still is) the leading cause of birth defects. Before the surgery could be performed, however, we had to appear before an ethics committee. It was the University’s way of proving that we understood the process and procedure and that we knew the risks involved. The committee was comprised of a handful of specialists. We passed the screening and I was scheduled for surgery at 22 weeks gestation, just 3 weeks after my initial ultrasound. We would soon become the first human test case for repairing a spinal defect in-utero.

We arrived at the hospital very early on the day of the surgery. We went directly to labor and delivery and I was admitted under an assumed name. I had become close to a few of the nurses on the floor. They had run some of the preliminary tests on me in the weeks prior to the surgery. We took the last name of a nurse that was very dear to me: Betsy Kennedy. Since this procedure was a first, everything was very secretive. They even placed extra security in that section of the hospital. I remember Scott saying later that the floor was unusually empty that day. We don’t think it was a coincidence. After another meeting with the ethics committee and a double check that the surgery was what we were there for, I was prepped and taken to the operating room. If the number of people in the operating room were any indication of history in the making, then we were definitely headed for the medical journals. The procedure took approximately 2 hours and the doctors were very pleased with the outcome. The atmosphere was electric. Scott remembers Dr. Tulipan coming out of surgery with a big grin on his face. He took it as a good sign and relaxed a little.

As it turned out, the rest of my pregnancy was uneventful: no abnormal contractions or infections. Medicine and monitors were quickly put aside as baby and I co-existed remarkably well. All that was left were minor inconveniences. Weekly visits to the doctor for ultrasounds continued for the next 13 weeks. Finally at 35 weeks, an amniocentesis determined that the baby’s lungs were mature. Just what we’d been waiting for. A cesarean was scheduled for the following Wednesday. They needed the weekend to get the doctors assembled at Vanderbilt. Some of them were out of town. That was okay with us, because our families needed a few days to get themselves to Nashville.

On December 7th, 1994 Zachary Kennedy Ritter came into this world crying and biting the doctor’s finger! Delivered five weeks premature, he was by no means a low birth weight baby. At 6 lbs. 6 oz he was a sight for sore eyes. We had waited for him for so long. We laughed at his size and didn’t worry about the spina bifida. Now it was time to be the parents that we had prepared ourselves to be. Zachary was in good hands at Vanderbilt Children’s Hospital. We visited him in the NICU a few hours after he was born. He looked out of place because he was so big, but we knew that was where he needed to be for a few days. Five days after he was born he had a VP shunt inserted to control a mild case of hydrocephalus. Three days after that, he was released from the hospital. We were anxious to get home and prepare for Christmas. We couldn’t think of any better gift than our little pioneer!

Zachary recently turned four and he has already paved the way for an even more successful fetal surgery for spina bifida babies. At first glance, you would see Zack as a typical 4 year old boy. As you got to know him you would see that he battles with some of the common problems associated with an L4/5 level of spina bifida. Incontinence is something we manage for him 5 times a day and low muscle tone below his knees requires him to wear a pair of MAFO braces. But, he walks by himself and he has never had to have a shunt revision. All that put aside, he has made tremendous strides in all aspects of his life. He began preschool this year and he is enjoying every minute of his newly found independence. We are so proud of him and everything he has accomplished. We couldn’t have asked for a more beautiful child!

In closing we would just like to say congratulations to all of the doctors, parents and children involved with the surgery over the past 4 years. Although the surgery is no longer performed endoscopically, great progress has been made toward a more successful open surgery to repair spina bifida in-utero. We wish them all luck as they continue to test the boundaries of medical science.

 

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