Something about the way Shayla Jones’ pregnancy came about made her and her husband, Jonathan, more shocked than most by what they learned at her eight-week ultrasound.
Shayla started on fertility medication in 2016, after the couple had tried for more than a year on their own. Four months later, still no baby on the way, they decided to take her off the pills and go forward with an intrauterine insemination (IUI), which is like giving the sperm a penalty kick rather than making it run the length of the field through the defense. All Shayla’s fertility specialist was waiting on was for Shayla to call and say her cycle had started—that the goal, so to speak, was in place. He received a call, just not the one he expected: Shayla had gotten pregnant the old-fashioned way.
Nobody expects twins, but when you’ve just snuck in a conception during the few weeks between fertility boosters—which have been credited with rising twin rates—that outcome floats somewhere near Pluto in your galaxy of expectations. Jaws hit the floor.
Shayla and Jonathan would’ve been just fine had the surprises stopped there. Instead, the doctor delivered more news. Although at the time the fetuses were the size of raspberries, he already suspected they were in a category referred to as monochorionic-diamniotic, or MoDi. That meant they were identical. It also meant they shared a placenta and were in separate amniotic sacs, a circumstance that comes about when the embryo splits about four to eight days after conception. Ideally, it would have split within the first four days, giving each side its own placental home. MoDi twins are at the greatest risk for what is known as twin-to-twin transfusion syndrome, or twin-twin.
With twin-twin, the blood flow between the two is uneven, and one side ends up without enough blood to support normal development while the other’s tiny heart struggles to keep up with too much. There can also be complications caused by an unequal sharing of the placenta and its life-giving nutrients. Without intervention, the disparity will usually be fatal for both, and survival often comes with developmental delays, although the recipient—the one with the excess—sometimes has a slightly better outlook.
Shayla was sent to a maternal fetal medicine specialist, but it wasn’t until week 16, when the babies were the size of avocados, that a slight imbalance appeared in the amount of fluid reaching each. Her doctors took a watchful waiting approach. At a Tuesday checkup a few days shy of 23 weeks—a milestone that would put the babies, little grapefruits then, on the preferred side of their highest-risk window—things appeared favorable.
“We left that appointment ecstatic and thought, Yay, we’re almost in the clear. Things will be fine,” she says. “And then on Friday, I went into preterm labor.”
Triggered by the excess fluid with the recipient baby pushing against the uterus, preterm labor is an enormous risk with twin-twin. It’s a major cause of death associated with the syndrome. Shayla went to a hospital in Abilene, where doctors confirmed she was in labor. After definitively diagnosing her with twin-twin, they transferred her to Medical City Dallas Hospital. There, she was stabilized and referred for fetal surgery, a highly specialized field in which surgeons operate with tiny instruments on the most delicate of specimens, and to a man named Dr. Timothy Crombleholme.
Back when Crombleholme was a student at Tufts University School of Medicine in the 1980s, the roots of American fetal surgery were just starting to take hold. For some reason, he was hooked by a case report published in the New England Journal of Medicine. In it, Dr. Michael Harrison, now known as the father of fetal surgery, lays out his attempts to fix a structural abnormality that was throwing off a fetus’s amniotic fluid volume by cutting directly into the uterus. Harrison was successful in a way—fluid volumes were restored. The baby, however, had already suffered too much damage and died. Regardless, Crombleholme’s interest was piqued. He set off for a residency at the University of California, San Francisco and, in due time, for a fetal surgery research fellowship there, studying under the tutelage of Harrison himself.
“I think my demeanor and faith kind of wavered after the first surgery. What if that’s the outcome again? Luckily, it wasn’t.”
The field of fetal surgery is small enough that specializing in it means one of your top job prospects is working to strike up a new program for a big city hospital. Crombleholme has now been in on the ground floor of five of them. His first was at his alma mater, Tufts. He joined Children’s Hospital of Philadelphia and the University of Pennsylvania next, helping found the Center for Fetal Diagnosis and Treatment. Eight years later, he was founding director at the fetal care program at Cincinnati Children’s Hospital Medical Center. Eight years after that, he became the surgeon in chief at Children’s Hospital Colorado and founded its Fetal Care Center.
Seven years from there takes us to 2018, when Crombleholme accepted an offer to bring open fetal surgery to North Texas. There are only about 12 places in the country that do this type of surgery—wherein a surgeon “exteriorizes” the glowing-red-dinosaur-egg uterus through an incision in the mother’s abdomen, and then operates on babies that have been developing for as little as 15 weeks. The Fetal Care Center at Medical City Dallas—which opened this summer—also gives Dallas access to Crombleholme’s expertise in closed fetal surgery, things like fetoscopic surgeries that require only a small incision in the uterus.
Before he moved to Texas, Crombleholme had been working as a consultant helping Medical City Dallas start its new Fetal Care Center. At first, there’d been no stated plan that he would run it. He was unsure when maternal-fetal medicine specialist Dr. Kevin Magee broached the subject.
But Medical City Healthcare is under the umbrella of national hospital operator HCA Healthcare, which encompasses about 180 hospitals. Crombleholme says there are a whopping 220,000 deliveries within the HCA system each year. The pitch to Crombleholme became a pitch to develop a fetal surgery network, wherein Medical City Dallas will serve as HCA’s national center for the most intense fetal surgeries, while Crombleholme helps establish standards for Level 2 and Level 3 centers elsewhere across HCA. That’s when he got excited.
That setup, he says, will allow for better continuity between a patient’s close-to-home hospital and the hospital of their surgery. Some fetal surgery centers require a family to relocate for the period between the operation and birth, a disruption of four months or more. But under Crombleholme’s vision, even the most intense fetal surgeries, such as procedures to correct spina bifida, would take families to Dallas for only a couple of weeks before a return to a lower-level center.
“Ideally, we would have these geographically distributed across the country with requisite expertise and resources to take care of patients close to home when possible and, if not, they would come here,” Crombleholme says.
It was Magee who referred the Joneses to Crombleholme. Had the couple’s timeline been pushed back about a year, they would’ve been able to stick around Dallas for their fetal surgery. But Crombleholme was still in Colorado. Which meant that five days after Shayla entered preterm labor, she and Jonathan were climbing aboard one very uneasy flight to Denver.
After meeting with Crombleholme’s team, it was settled: Shayla Jones, now at nearly 24 weeks gestation, was the right candidate for a fetoscopic laser ablation. Using a fetoscope and ultrasound as a guide, doctors would go in and burn the ends of select blood vessels to close them off, thus restoring the balance between the two babies.
Before Shayla went under, they ran through every possibility. “It’s either no babies, one baby, or possibly both,” she says.
The first attempt did not go as planned. Because of the placenta’s positioning, Crombleholme couldn’t sufficiently map it. To make matters worse, amniotic fluid that had stockpiled with the recipient baby spilled out when they inserted the fetoscope, and with the release in pressure came a collapse of the uterus. Visualization became impossibly low. Rather than shoot off the laser blindly, Crombleholme opted to drain some of the amniotic fluid, end the surgery, and hope that it would be enough to buy some time while they reevaluated the situation.
Shayla and Jonathan waited a long five days, Crombleholme’s team on close watch. “I think my demeanor and faith kind of wavered after the first surgery,” Shayla says. “What if that’s the outcome again? Luckily, it wasn’t.”
Fetoscopic surgeries can occasionally have an odd side effect: the placenta and the amniotic sac separate. That complicates things. Crombleholme had done only a handful of fetoscopic surgeries with the two separated, and he says they’re considerably riskier and more involved. But with the mapping he did during the first surgery, he went in without puncturing the amniotic sac and was able to fire the laser—which measures just 600 microns in diameter, a little more than half a millimeter—and re-balance the scales.
“It’s a privilege,” he says, his voice suddenly a rasp. “There aren’t many people that get to do what I do.”
The next 24 hours was a critical period. There was a high risk for complications with the babies and, again, for preterm labor. The next day, they performed an ultrasound: two heartbeats. Shayla and Jonathan were ecstatic, even as several more weeks remained along their path. “You’ve made it that far, and you still have two babies,” she says.
Crombleholme was able to problem-solve Shayla’s complications because he has so much experience in the field. The challenge with starting a fetal surgery center—why there are so few of them across the country—is that the types of procedures these surgeons perform are both exceedingly specialized and relatively rare, says Dr. Sean Blackwell, a Houston-based maternal-fetal medicine specialist who serves as president of the Society for Maternal-Fetal Medicine. “You want to have people that are higher-volume surgeons,” Blackwell says. “It’s no different than doing a heart transplant or a brain surgery—if you do six in a year, that’s different than if you do 60 in a year.”
The operations are not without controversy in the medical community. While in Cincinnati, Crombleholme pioneered an “amnioport” procedure, where a surgeon places a catheter inside the amniotic sac, attaching it to a port that remains on the mother’s abdomen throughout the pregnancy. That way, doctors can control the fluid volume in a baby that is otherwise, for whatever reason, deficient—cases that previously had no course of action.
With Crombleholme’s amnioport surgery, you can save the baby, but you can’t save the baby’s kidneys, and the newborn will be on dialysis from the second he or she enters the world. It’s not easy to find a pediatric dialysis specialist, so families often face permanent relocation. Reasonable minds wonder whether a family and expectant mother could truly give informed consent about the circumstances ahead. In a study published by Crombleholme and others that looked at 15 cases in which patients were identified as good candidates for amnioports, six families chose to forgo treatment and seek comfort care. Meanwhile, Crombleholme says about half the babies who have benefited from his amnioport procedures have gone on to successful kidney transplants.
The field of fetal surgery got a boost in 2011 when the public learned about results of the MOMS trial, a multi-program clinical trial that compared the outcomes of spina bifida repairs made in utero versus those made after birth. Fetal surgery won. The study showed, among other things, that kids who had been operated on prior to birth developed better motor skills and were twice as likely to be walking on their own by the time they were 2 and a half years old, although complications could still arise as they grew into adulthood. Crombleholme prides himself on the fact that his surgeries have exceeded MOMS benchmarks, and he and other specialists have been able to tweak their surgical approaches based on specific findings. But the positive results had an unexpected consequence, leading to a wave of new centers purporting to perform the procedures to which Crombleholme has devoted his life.
“That has been controversial, because when you look around the country, there are way more centers than we need,” he says. “And the vast majority of those centers are—what’s the politically correct way to say it?—undertrained.”
Even with all our advancements in technology, the womb appears to be the best place for a baby to recover from surgery. At around 32 weeks, by now back home in Abilene, Shayla Jones’ babies were the size of summer squash, one of them a little larger than the other. She got a call and heard good news: several weeks after Crombleholme relieved the disparity in blood flow, the recipient daughter’s enlarged heart had returned to normal.
On August 24, 2017, one day before 34 weeks gestation, the Joneses met their girls. Maebry came out at 4 pounds, 15 ounces, a normal weight for that stage of development.
Madelyn, the selfless donor, was 3 pounds, 6 ounces. Both babies were intubated the day of the C-section and extubated the day after. They have hit all their developmental milestones relative to the day they were born. Shayla and Jonathan will stay vigilant to make sure nothing appears out of the ordinary as the girls continue to grow older. That’s what parents do. But so far, so good.
It’s fair to say they owe that outcome to Crombleholme, a 60-year-old with rounded brown glasses that poke out from his sterile blue surgical cap. On a recent Thursday, he arrived in the OR for what became one part interview, two parts medical school, his laptop propped on a stainless steel table. As he flipped through a PowerPoint, explaining as he went, he got to the first video. Legs popped out from an exteriorized uterus, slimy and red, the size of a frog’s. Toes were like the tip of a pen. They were dwarfed by a gloved thumb and pointer.
Crombleholme still gets Christmas cards from mothers he helped a decade back, with photos of high school graduates he operated on in utero.
“It’s a privilege,” he says, his voice suddenly a rasp. “There aren’t many people that get to do what I do.”