Editor’s Note: This is the second in a five part series where The Blaze has looked into a newer treatment for children diagnosed with spina bifida. While most would have the hole exposing his or her spine closed after birth, more than a decade ago surgeries that were considered controversial at the time began where the hole was closed in utero in the hopes of reversing some of the disabling effects of the disorder.
The first results of a federally funded human trial were released in 2011, showing the success seen in the new treatment thus far. We’ve talked to families who had the elective surgery before it ever entered a formal human trial and those who more recently have had the surgery since the period trial ended. We’ve timed this series to specifically run close to Glenn Beck’s Restoring Love as the parents of children who underwent the surgery, many of whom still have some disability, want the world to know the blessing of celebrating differences.
Dr. Scott Adzick, the Department of Surgery Chief at the Children’s Hospital of Philadelphia (CHOP), says the best decision he ever made was to head to the University of California-San Francisco 30 years ago in order to be on the cutting edge of fetal medicine. Adzick wanted to research surgery before birth and San Francisco was the mecca at the time.
“I was frustrated taking care of babies with life-threatening problems, and when the baby was born, we were too late,” he said.
It was out of this frustration of not being able to treat disorders early enough that Adzick and a few others decided they wanted to operate on the fetus through the mother’s womb as a treatment — a treatment specifically for spina bifida. At the time, fetal surgeries were reserved for only babies in life-or-death situations. Spina bifida wasn’t necessarily life threatening to the infant, but still very debilitating — and research prior to actually beginning the surgeries on human fetuses showed they could be a benefit.
Spina bifida is a congenital disorder that occurs when the backbone and spinal canal do not close. What causes spina bifida varies, but it can be genetic or result from low levels of folic acid early in pregnancy. The most severe and most common cases of spina bifida where the hole doesn’t close in the lower half of the spine, known as myelomeningocele, occurs in 1 in every 800 infants. More than 1,500 babies are born with spina bifida in the United States each year, making it the most common birth defect of the central nervous system. It is estimated 64 percent of fetuses diagnosed with spina bifida are aborted. With myelomeningocele, the membranes and the neural tube will protrude from the back forming a sack. A nurse described it as looking about the size of a small mellon. The mother can learn of the condition around 18 to 22 weeks, generally when the she goes in for her an ultrasound.
Watch this video from CHOP explaining what spina bifida is:
The treatment — still the most common treatment for those with the disorder today — is closure of the hole after birth. More often than not, depending on the child’s “lesion level” — the location where the spinal canal failed to close in the womb — the child would have hydrocephalus (water on the brain) and could require a shunt (a pump that would drain the fluid from the brain). They could be paralyzed from the waist down or, if they’re lucky, could walk with a walker or braces on their legs. They frequently don’t have proper nerve formation for normal bowel and bladder function and would need to be catheterized first by the parents and at an older age by themselves.
Before fetal surgery could even be considered to correct the spines of human fetuses, research was done to define what Adzick called the disorder’s “natural history.” Essentially, this means tracking the disorder back to when it begins to form and what occurs in the womb after the hole fails to close.
A husband-and-wife team, Dr. Martin Meuli and Dr. Claudia Simmen-Meuli, conducted reviews of aborted fetuses that had myelomeningocele and found the exposed spinal cord would go through varying degrees of injury within the womb, which was the cause of the symptoms after birth. Dr. G.M. Hutchins, who was also involved with these initial reviews, with the Meuli’s conducted an experiment using fetal sheep, where the fetal lamb’s spine was exposed in the womb, creating a spina bifida-like defect. At birth, the lambs exhibited similar symptoms to children with myelomeningocele.
In some lambs though, were operated on again to correct the lesion. Adzick, who was also involved in this research, said the results on lambs that had the spinal correction were “phenomenal.”
“There was some distortion but they were remarkably protected,” he said.
In this video, Adzick and other doctors explain how fetal surgeries came about:
After these findings and strict procedural regulations, Adzick and others began offering this as an option for parents who wanted to prevent some of the effects of the most severe forms of spina bifida. Eventually, a clinical trial was instituted to establish if there was in fact a significant benefit in the outcome of those with spina bifida who had fetal surgery compared to the more traditional, after-birth treatment.
Three hospitals were involved in the trial: the CHOP, Vanderbilt University Medical Center and the University of California-San Francisco. The Management of Myelomeningocele Study (MOMS) was a randomized trial that began in 2001 where some fetus underwent fetal surgery while others had the traditional treatment after birth. Watch an overview video from CHOP on the study here.
Just last year, after 10 years, the trial stopped accepting patients because those evaluating the data found they had enough information to show that fetal surgery was more successful at reducing the need for a shunt, reversing the Chiari malformation and other more minor effects.
“In spite of an increased risk for preterm birth, children who underwent surgery while in the uterus did much better, on balance, than those who had surgery after birth,” Alan E. Guttmacher, M.D., director of NICHD, which funded the study said in a Feb. 2011 press release on the findings. “However, caution is advised. Because the surgery is highly specialized, it is best undertaken in facilities with staff having experience in the procedure.”
“This was very exciting,” Adzick said referring to when the results of the study became apparent. “[The] results basically matched what we had seen at CHOP before the [MOMS study] began.”
The time period from when a woman finds out her child has spina bifida to when she is considered for the surgery and goes under the knife is very short. Although procedures vary slightly among hospitals at the moment, most will not perform the surgery after 26 weeks of gestation. The lesion in the fetuses back needs to be at a certain level, and the fetal candidate cannot have any other genetic abnormalities. These are just a few requirements for the fetus — the mom has some as well.
The mother will not be accepted for surgery if she doesn’t meet a range of criteria. Not to mention the mental and ethical evaluation parents are put through.
Lori Howell, a nurse working with families of children with birth defects for more than 30 years who is currently the executive director of CHOP’s Center for Fetal Diagnosis and Treatment, explains that the process for choosing candidates is rigorous. The candidates tour the neonatal intensive care unit, seeing all the extremely premature and sick babies. A place where their child could potentially end up. They also meet with a ethics board who asks them several questions, many of which are hard to hear but are meant to prepare the mother for every possible outcome.
One mother told the Blaze that the board asks questions like “If you didn’t have this surgery, your child will most likely be born healthy considering their condition and the surgery performed after birth to fix the hole in his or her back is routine. This fetal surgery is elective, there is the potential your child — or you — could die as a result of this procedure. Would you still want to go through with it for this risk?”
Watch this video explaining the surgery and the process:
“It’s not for everybody,” Howell said. “You shouldn’t feel guilty if you can’t have a fetal surgery or if you opt to make another choice.”
Stay tuned for the third installation of this five part series tomorrow where the Blaze will cover many of the positive results of fetal surgeries as explained by parents whose children had the operation years ago.