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No Womb for Error
At Johns Hopkins’ Center for Fetal Therapy, specialists are pushing the boundaries of prenatal treatments to give mothers and their at-risk unborn babies the best shot at life.
Months after surgery performed in utero saved the lives of babies Lily, top, and Grace, bottom, Shawn and Katie Tingle share a rare quiet moment at home with their triplets, including Mason, center.
While surgical interventions are sometimes necessary, fetal therapy can just as often be a matter of simply watching and waiting, notes Baschat.
Katie and Shawn Tingle had seen quite a few specialists leading up to that day in the fall of 2014, when Katie met with a doctor in Silver Spring, Maryland, who advised her to make an appointment for the next day with yet another specialist—this time at the Johns Hopkins Center for Fetal Therapy.
Katie was 16 weeks pregnant at the time. Two months had passed since she and Shawn had received the “big shocker”—news that Katie was carrying triplets, a pair of identical girls and a fraternal boy. Such triplet pregnancies are rare, accounting for just 7,000 of the nearly 4 million deliveries of babies each year in the United States.
“Katie had seen so many specialists by this point that we didn’t really think anything of it,” Shawn says. A nutrient management research associate at the University of Delaware, Shawn went ahead the next day with a planned business trip.
With Shawn on the road to Kentucky, Katie’s mother made the trip with her from the Tingles’ home in Seaford, Delaware, to the second floor of the Nelson/Harvey Building at The Johns Hopkins Hospital. Katie’s appointment with Ahmet Baschat, the center’s director, began in the same manner as so many of her previous visits with other specialists, with a careful and painstakingly long look via ultrasound at the health of the three fetuses in her womb.
“Right from the beginning, all the doctors we saw were upfront with us about all of the risks and everything, how hard it is to carry multiples and how all three of them might not make it,” Katie says. “What we tried to do was just pray to the man upstairs and take things as they came along. Every time we went in for an appointment, we were both like, ‘Do we still have three?’”
This time around, the focus of the ultrasound was an imbalance of fluid levels between the two identical girls. At one point, Baschat pointed out on the scan the place where that imbalance was playing out.
“There were these two little circles,” Katie says. “One looked like a golf ball. The other looked like a softball. It was very extreme.”
The technical term of this imbalance is twin-to-twin transfusion syndrome, or TTTS. Left untreated, it would likely have ended in the death of both twins. Baschat told Katie that she needed surgery—and that she should have it that very afternoon.
“One of the things that remains difficult to ascertain in TTTS is how quickly a case might deteriorate,” Baschat says. “Once you get to stage 3 TTTS, which is where this one was, you can end up losing a baby between the time you make the decision to have surgery and the time you actually do the surgery.”
Fetal therapy is a young field, with a history that dates only to the 1970s, when a few physician-scientists began looking for ways to identify birth defects early on, in the womb.
The field’s focus has grown much wider in the years since. Cases that land in fetal therapy centers today can range from urinary tract problems, diaphragmatic hernia and growth restriction to anemia, cardiac issues, spina bifida, tumors and more. Many of these complications are quite rare and complex.
“This is high-risk obstetrics and fetal medicine combined,” Baschat says. “We need to be experts in both managing the health of the mother and in diagnosing and dealing with these rare fetal problems. What we’re always trying to do is give the babies that we treat the same start in life that babies have in normal pregnancies.”
There are only a couple of dozen facilities in the United States on the membership roster of the North American Fetal Therapy Network. Johns Hopkins joined its ranks in the summer of 2014, when Baschat arrived in East Baltimore from across town at the University of Maryland. Later that same year, he brought aboard a second fetal medicine specialist, Jena Miller.
In Baschat’s experience, the general public has only a slight understanding of what the practice of fetal therapy entails. While surgical interventions to correct abnormalities are a staple of practice, fetal therapy can just as often be a matter of simply watching and waiting, Baschat says. He points here to certain types of fetal growth restriction where not enough nutrients make it through the placenta to support a healthy growth rate over the course of a full nine-month term.
“You really can’t do anything about this before birth,” he says. “So what we do instead is monitor, looking for signs that the problems associated with that lack of nutrients are accelerating.” The goal in such cases is perfect timing—delivering that baby at the moment that best maximizes fetal growth potential and minimizes the risk of irreversible damage.
For the problem that Baschat diagnosed in the Tingle case, the proper course of treatment is quite well-established. Fetoscopic surgery was first employed to spur a rebalancing of fluid distribution between a pair of twins with TTTS in 1995. The literature surrounding the procedure is rich enough today to give physicians and patients a clear picture of risks and benefits. At the Johns Hopkins Center for Fetal Therapy, the double survival rate in TTTS is better than 80 percent.
“But for many other fetal diseases, that sense of the risks and the benefits is not so well-established,” Baschat cautions. “The way I look at it, TTTS is kind of a poster child for the steps that we will need to take to develop prenatal treatments in other kinds of cases.”
When Greg and Kate Kiley headed out of their Capitol Hill rowhome to take in the fireworks show in the nation’s capital on July 4, 2014, they were not in a celebratory mood. Instead, they were a bundle of nerves.
The first six months of Kate’s pregnancy followed a predictable and problem-free path. But as that June gave way to July, the movements that Kate had felt so often in her womb had slowed noticeably and then stopped altogether.
“Before then, the baby was always moving in predictable ways,” Kate recalls. “It would be 10 minutes after I ate, things like that.” Loud noises had been one of the things that triggered movement, and the Kileys were hoping that the fireworks would spark a reaction.
The next morning, a Saturday, the couple headed to a local hospital in search of answers. They learned that their daughter-to-be was now measuring at 29 weeks in size, bigger than the expected 27 weeks. They heard talk from physicians about “parvovirus” and “anemia” and “hydrops,” but no one gave a definitive answer. They were advised to come back on Monday and see a specialist.
“It’s a horrible, horrible thing to have the Internet at your fingertips when you have no idea what’s happening with your baby,” Kate says. “We spent the rest of that weekend on our phones, looking up hydrops and the other things we’d heard about, and basically reading about how she wasn’t going to make it.”
Things got even scarier on Monday morning, when the 27-week-old fetus weighed in at 34 weeks.
“That’s five weeks’ worth of growth in two days,” Greg says. “Someone said later on that she looked like the Michelin baby.”
There were still no answers. By noon, the Kileys were on their way to see Baschat at Johns Hopkins.
An obstetrician working in a traditional practice might see 30 or more patients in the course of a typical workday. At the Center for Fetal Therapy, Miller and Baschat see just a handful.
“Not only are we dealing with rare cases, but we are dealing with cases that arrive in unpredictable ways and at unexpected times,” Miller says. “As a result, the way we practice needs to be organized in a different way.”
Initial consults at the Center for Fetal Therapy are penciled in at two hours. The sessions generally include at least 60 minutes of face-to-face time between physician and patient. “We really get to know our patients,” Miller says. “There is never a situation where you walk into a room without knowing why the patient is here.”
Looking back on their experiences at the center, the Tingles and the Kileys both express amazement over the amount of time and the length of the conversations they had with physicians and nurses alike.
“It didn’t matter how many questions you had; they always stayed and answered them,” says Shawn Tingle. “They never once in all that time let us see how busy they were.”
“There is always talk about taking a sort of holistic approach to things, but I feel like we really witnessed that firsthand,” Greg Kiley says. “It wasn’t just words—they really did seem to have the whole patient in mind every time they were dealing with you.”
The technical terminology for Katie Tingle’s identical twins is monochorionic diamniotic. The mono in the first word means the twins shared a single placenta. The di in the second means that there were two amniotic sacs, one for each twin. (The third fetus, a boy, was harbored at a safe distance from all the action, in his own sac and with his own placenta.)
Roughly one in five of these twin pairs develop TTTS, and the resulting fluid imbalance creates a situation where a so-called donor twin does not keep enough blood in its circulation, while the “recipient” sibling is getting overloaded. The recipient starts producing more urine than it should, which leads to excess amniotic fluid that shows up on an ultrasound in the form of an oversized bladder and a bulging amniotic sac. The donor twin, meanwhile, produces less urine than it should, which results in a dry sac that looks like it’s collapsing.
This is the softball and the golf ball that Katie saw on her scan. Her first reaction to the recommendation that she undergo immediate surgery was to ask whether she could put it off. She wanted more time to think things through. She wanted to wait until Shawn returned from his trip.
“I sat there going back and forth, for at least a couple of hours,” she says. “Dr. Baschat would pull his chair up and sit with me for as long as I wanted to talk. Then he would get up and give me a break. And then he would come back in after a while and we would sit and talk some more.”
At one point, Baschat asked Katie to try and take her husband’s absence out of the equation and then start thinking things through again. That suggestion made all the difference. She talked things over with Shawn, and they both agreed to go ahead with the surgery that day.
That sort of intense and drawn-out decision-making dynamic is common in fetal therapy. Baschat likes to say that his is a field where “sometimes you have to rush into treatment, but you can never rush into a decision.”
“The natural tendency for most parents in most pregnancies is to tell us doctors to do everything possible for the child and to just forget about themselves,” he explains. “But ultimately, in many of these cases, there is a real need to get past that. Because if there is no healthy mother, then there is no healthy baby.”
“Patients are coming in and hearing these difficult diagnoses,” Miller adds. “There is a lot of information to process. It’s just not fair to give a little speech and then say, ‘OK, do you have any questions? Are we ready to go?’”
One possibility that sits in the back of Baschat’s mind during such counseling sessions is the ever-present chance that a course of treatment will fail. He points to congenital diaphragmatic hernia as one condition where the fetus often faces daunting survival prospects. He adds that the risk is significant even in conditions where the treatment option is well-established—nearly one in five TTTS cases ends in the loss of at least one twin, for example.
“There can be bad outcomes in fetal therapy, and that’s just a fact,” he says. “It’s important for both the physician and the patients to get to a place where everyone feels certain that you’re doing the right thing. Because if there is a bad outcome, you will all need to be able to look each other in the eye and say: ‘We made the right decisions. We tried our best. It’s just that this turned out to be something we could not achieve.’”
When the Kileys arrived for their appointment at the Center for Fetal Therapy, Baschat was still sitting through his employee orientation session. That July Monday was his first day on the job.
Kate had already been through an ultrasound by the time he showed up in the exam room. The other doctors involved hadn’t found any answers. They were about to send Kate out for lab tests when Baschat asked for a last chance to look things over.
“Right away, I was like, ‘Man, something about that baby’s heart looks funny,’” Baschat recalls. “Sometimes you can just tell by looking when a heart has been under stress.”
Just at the moment when he started to measure the fetal heartbeat, it went into overdrive, in a familiar pattern that’s a sure sign of a cardiac irregularity called supraventricular tachycardia, or SVT, which can be triggered by an abnormal conduction pathway in the heart. The Kiley fetus had the paroxysmal variation of the condition, in which the irregular beat is intermittent rather than constant.
Baschat attributes his quick diagnosis that day to a bit of luck. “We would have found it in any case,” he says. “I would have put her on a monitor overnight, and it would have shown up. But it just happened to kick in right when I got there. It was funny, the way everybody was looking at me like they were thinking, who is this new guy?”
Moments after his arrival, then, Baschat was reassuring the Kileys that their daughter’s SVT would likely come under control on a regimen of the heart medication digoxin, either alone or in combination with other medications. Baschat himself was lead author on one of the definitive SVT papers in the literature. Like TTTS, SVT also ranks among the conditions where fetal therapists stand on reasonably solid ground when it comes to prognosis and treatment options.
“It was all such a roller coaster,” Kate says. “We were way down here at the time he came in the room and then two minutes later, we’re all the way up here.” Kate stayed in the hospital for several days after that, with a heart monitor keeping track of how the fetal heart rate was responding to different doses and combinations of medications.
Today, the Kileys marvel at how early and how easily Muriel Renée, now 9 months old, started sleeping through the night and how few and far between were the times when she’s been in a difficult mood.
“Considering all the drama she caused us that summer, well, we haven’t had any drama from her since,” Kate says.
The Tingle triplets are arrayed in a triangle pattern around the living room floor of their Seaford home, each sprawled comfortably on his or her own pillow while their mother and father recount the adventure of the pregnancy that culminated in their birth this past Feb. 10.
Lily, Grace and Mason spent most of their first month in the neonatal intensive care unit at Johns Hopkins, growing under supervision to weights safer than their birth rates, which ranged between 3 pounds, 12 ounces and 4 pounds, 11 ounces. The three were released together on March 3, and it fell to Shawn to drive that new minivan back to Delaware on an overcast day that took the family through stretches of sleet and rain.
“I was like, ‘Please, God, just let me get home with everybody in one piece,’” Shawn says.
The fetoscopic surgery that Katie underwent on Oct. 7, 2014, is a minimally invasive affair called laser ablation. Using a tiny camera to find his way around in the womb, Baschat located and closed all of the blood vessels in an area of the placenta called the vascular equator, which is where the vascular connections between the twins are located.
“What that does is set up a strict line that separates one twin’s part of the placenta from the other twin’s part,” Miller says. “Both of them will then have to sort out their own hemodynamics. It usually takes two weeks to resolve, and it’s usually successful.”
By the time Shawn returned from his business trip two days later, Katie had the all-clear to return home. The couple than returned to the center once a week for four weeks while Baschat monitored the twin girls as they found their way to their new and healthier fluid equilibriums.
The Center for Fetal Therapy at Johns Hopkins is engaged on several fronts in projects that aim to help give physicians in their field a level of confidence in treating other diseases that matches the confidence they have with TTTS and SVT.
Baschat and Miller are now tracking and analyzing clinical outcomes associated with the fetoscopic surgeries they perform for a condition called congenital diaphragmatic hernia, in which the contents of the abdomen move up through a hole in an underdeveloped fetal diaphragm and get in the way of proper lung development.
The intervention in question—inserting a balloon into the trachea of the fetus to help expand the lungs—has been around only since 2008, so the level of data available pales in comparison with that available from two decades of experience with TTTS.
Definitive data is even harder to come by in fetal bladder outlet obstruction, which arises when an errant flap of skin or some other developmental abnormality blocks the flow of urine. In the most serious of these cases, Baschat and Miller can end up discussing with patients a risk of renal failure after birth that ranges between 15 and 75 percent.
“How are patients supposed to make a decision with numbers like that?” Baschat asks.
Two different types of surgery are options here. In one, surgeons look to repair the problem by removing the cause of the obstruction. In the other, they insert a shunt that eases the effects of the obstruction.
“The problem is that when you take a look inside, it turns out that the obstruction can’t be fixed in about 60 percent of the cases,” Baschat says. “Then you need to do a second operation to put a shunt in.”
Here, he and Miller are working with colleagues in pediatric urology on an innovative surgical technique that gives surgeons both options in a single procedure, eliminating the need for those second surgeries—and the risks associated with them.
Spreading the news about such innovations is another key step in the development of this young field.
“Too many times in fetal therapy, you get patients sent to you and it’s just too late—there’s nothing you can do,” Baschat says. “That is why we encourage early inquiry or referral if there are concerns about the health of the fetus.”
When he looks now to the future of fetal medicine, Baschat finds himself looking around the Johns Hopkins campus at the field of general medicine.
“Ideally, I’d like there to be a sort of fetal hospital available to these patients,” he says. “The fetus is a fundamentally different being. These are not babies. They are not breathing. They are sitting in a bag of water, getting supplies through the placenta. Think of everything that is available in medicine in general—the diagnostics, the imaging, the blood tests, nanomedicine, genetics—I would like to see this field get to the point where we can make them all available to the fetus. It’s a lofty goal, but you never know.”
Miller nods her head in agreement. “There is a lot of potential to move beyond what we are capable of right now,” she says. “It’s all about being able to open up our minds enough so that we can figure out how to get there.”
“Patients are coming in and hearing these difficult diagnoses.There is a lot of information to process. It’s just not fair to give a little speech and then say, ‘OK, do you have any questions? Are we ready to go?’”
fetal medicine specialist