Even though the field of fetal surgery has been around for more than 30 years, the most common question about it remains this: “Wait, you can do surgery on a fetus?”
In fact, it is not only possible to perform surgery on a child still in the womb, but CHOP’s Center for Fetal Diagnosis and Treatment (CFDT), opened in 1995, is the largest and most experienced center in the world, having performed more than 1,000 fetal surgeries, a quarter of the prenatal surgeries ever attempted worldwide.
Home to the Garbose Family Special Delivery Unit, the world’s first comprehensive birth facility exclusively for mothers carrying babies with known birth defects, the CFDT offers powerful hope to families from all 50 states and more than 50 countries around the globe.
Every family at the CFDT is on a unique, and often complex, journey.
It was Jan. 28, 2013, when Catalina Agosto got the best ever Valentine’s Day present for her husband, Jose: the news that she was pregnant with their first child.
She planned to keep it a secret until Feb. 14, but four days later, she gave in to her own excitement. Much to his confusion, Jose came home that evening to find a video camera pointed at him.
Catalina presented him with a box labeled “Early Valentine’s Day Gift” wrapped in red tissue paper. When he opened it, a helium baby balloon floated out. A baby? Jose was in disbelief. But then Catalina showed him a paper from her doctor — she had even framed it.
Once he got over the shock, the couple hugged, shed a few happy tears and started calling their families to share the good news.
At their 12-week ultrasound, they finally got to see their little bean. They could not have been more excited. Catalina began to make a list of the baby things they would need. At week 19, they went together to their anatomy ultrasound. The technician told them it was a boy, but then she got quiet.
She left the room and a doctor came in with some bad news: There was something irregular on the baby’s lung. The doctor referred them to a maternal fetal medicine specialist nearby their home in Jacksonville, FL.
For several days, the Agostos waited nervously for the appointment, alternating between the joy of having a son and the heartbreak of potentially losing him. The specialist told them their son had a congenital cystic adenomatoid malformation, or CCAM. The Agostos, like most people, had never heard of it.
A CCAM is a type of growth on the lung. What makes it so dangerous is that if it grows large enough, it begins to take over the chest cavity, pushing the heart to the side. That in turn makes the heart unable to fill, backing up fluid. In severe cases, the fetus goes into heart failure, which will be fatal to the baby and even can be life-threatening to the mother.
Doctors in Florida presented a stark set of options.
If they wanted to terminate, they had until 24 weeks. That was out of the question for the Agostos.
Alternately, they could try a course of steroids to stabilize or shrink the tumor. “The way he was talking, the reality sounded negative,” says Catalina. “You get the steroid and it works or it doesn’t. That was our last hope in a way.”
It didn’t work. The tumor kept growing.
The doctor repeatedly advised them to terminate. But when the Agostos refused, the doctor brought up another possibility: a hospital in Philadelphia with expertise in CCAMs.
Fetal surgery — something else the Agostos had never heard of — might be an option. They were willing to try anything. Catalina placed a call to the Center for Fetal Diagnosis and Treatment (CFDT) at The Children’s Hospital of Philadelphia.
The journey to Philadelphia
It was May 30 when Catalina dialed the number and connected with nurse coordinator Susan Spinner, MSN, RN. Together, they reviewed the diagnosis and Spinner explained that CHOP is the most experienced center in the world for treating babies with CCAM before birth.
“One of the most important things we do in the beginning when we get the first call,” says Spinner, “is just to let the mother know that somebody is there for her.”
It was Thursday, and Catalina needed to be in Philadelphia on Monday. The baby’s lesion was already far advanced. Catalina had never been to Philadelphia, and Jose, who had just started a new job, couldn’t take time off work to accompany her.
Jose’s mother, who lives in Puerto Rico, volunteered. She and Catalina had never even met before.
Last-minute flights are expensive, and so Spinner turned to the Addison’s Hope for Fetal Families Fund to fly both Catalina
and her mother-in-law to Philadelphia and book them a hotel for those first few days. This fund was started by Mary and Jed Kelly, whose daughter, Addison, had lifesaving fetal surgery at the CFDT in 2006.
The fund is used in exactly situations like these — to pay for travel expenses that might otherwise be a barrier to a family getting the care they need at the CFDT. Catalina just wanted to get to Philly as quickly as possible and find out if there was any hope for the baby they had decided to name Joseph.
Making a plan
Tuesday, June 4, was a blur to Catalina. As with all new patients to the CFDT, she underwent a full day of tests and consultations, including an ultrafast fetal MRI (which was developed at CHOP), a detailed high-resolution ultrasound, a fetal echocardiogram and genetic counseling.
All of her tests took place in one central area — a deliberate effort to simplify a long day.
At the end of the day, Catalina and her mother-in-law met with a large part of her core team: Spinner, maternal fetal medicine specialist Julie Moldenhauer, MD, and pediatric surgeon Holly Hedrick, MD.
Together, they reviewed Catalina’s diagnosis and her options. They would try another course of steroids, they told her, to see if the tumor might shrink. If it stabilized, perhaps it could be removed after Joseph’s birth.
But they warned her it was already pretty large, and the baby already had fluid buildup in his abdomen, a sign of distress. If that progressed further, surgical options would be on the table. While survival rates are only about 60 percent, it’s something they would consider if they had to.
“It’s always a challenge to figure out when to intervene,” explains Hedrick, who has 15 years of experience with fetal surgery. “There is a balancing act to intervene only when absolutely necessary, deciding when no other option will work and soon enough to make a difference. You have to be watching very carefully. Timing is everything.”
That meant Catalina would have to remain in Philadelphia until Joseph was born. She had packed for a week or two but was now facing a months-long stay at the Ronald McDonald House.
Catalina took the steroids and came to CHOP for appointments every other day. But the tumor only grew. Fluid accumulated in Joseph’s belly, skin and scalp. His heart was being pushed to the right side of his chest.
On June 21, Catalina sat down with her entire team and learned that it was time for fetal surgery. It was so urgent, in fact, that it had to be done the very next day. She would be 24 weeks and six days pregnant.
With a survival rate of about 60 percent, Catalina needed to be prepared for some pretty awful possibilities. If Joseph died during surgery, she might wake up from the surgery and not be pregnant anymore. Or Joseph might need to be delivered and might live only a short time.
How would she want to spend that time with him? Would she want to hold him? To have him baptized? She cried when she heard the news, but she accepted that this really was Joseph’s last hope.
Catalina arrived at CHOP at 6 a.m. on Saturday, June 22, feeling numb and scared.
After receiving IVs and an epidural, she was wheeled into the operating room. The anesthesiologist asked her to count to three. She didn’t even make it that far. The next thing she knew, she was waking up.
Meanwhile, the team of almost a dozen people — surgeons, specialized fetal operating room and obstetric nurses, a fetal cardiologist, anesthesiologists, and maternal-fetal specialists — sprang into coordinated action.
This would be an “open” fetal surgery, meaning that there would be a long horizontal incision across Catalina’s lower abdomen, the uterus would be opened and part of Joseph’s body would be exposed so that the surgeons could remove his CCAM.
Once finished, they would return him to his mother’s womb, replace his amniotic fluid with a sterile solution, sew up their incisions, and with any luck, he would return to his mother’s belly for 13 more weeks.
N. Scott Adzick, MD, CHOP’s surgeon-in-chief and director of the CFDT, assisted Hedrick with the surgery.
As complex as they are, these are fast procedures — by design. Fetal surgeries must be done quickly to minimize risk. Hedrick estimates that fewer than 30 fetal surgeries for CCAM removal have ever been done, and most of those have been done at CHOP.
After all the incisions were made, Joseph’s tiny left arm was pulled out into the open, exposing his left chest. An IV was put into his tiny hand, and a pulse oximeter was wrapped around his tiny hand and covered with sterile foil. A pump maintained warm fluid levels around Joseph.
A cardiologist was constantly monitoring Joseph’s heart, while other physicians constantly monitored Catalina and Joseph.
The team was prepared for the worst: Doses of powerful resuscitation drugs were nearby on the table, already measured for Joseph’s 1.8-pound body weight, in case his heart stopped beating during surgery.
Surgeons removed Joseph’s CCAM — while saving the compressed but normal portion of the left lung — and then layer by layer, they stitched closed both child and mother.
Pregnancy, round 2
The surgery had been a success. The doctors had removed a CCAM the size of an adult fist. “I can’t imagine how something so big could be in someone so small,” says Catalina.
After four days in the Garbose Family Special Delivery (SDU), Catalina returned to the Ronald McDonald House on strict bed rest to help prevent preterm labor. She would Skype with Jose every night and spend the day watching TV and chatting with other patient families.
Her weekly ultrasounds at CHOP revealed that the fluid around Joseph’s scalp and belly was disappearing. What had been a tense situation was now a surprisingly uneventful waiting game. The doctors hoped Joseph would stay put until 37 weeks, when he could be delivered by cesarean section. And he did. At 37 weeks and one day, Catalina reported to the SDU.
Happy birthday, Joseph
Jose flew up to Philadelphia to be there for the birth. The enormity of the day hit Catalina just before the procedure, and she began to cry and shake uncontrollably. Her nurses held her hands as she got the epidural, and she went to the OR.
At 9:56 a.m. on Sept. 16, Joseph Manuel Agosto was born, weighing 5 pounds, 10 ounces and measuring 18 inches. He was the 1,573rd birth in the SDU.
Doctors hurried Joseph into an adjoining neonatal stabilization room to make sure he was OK. As they assessed him and brought him to his incubator, Jose finally got to see his son. That’s when Joseph opened his eyes.
“We made eye contact and that was the greatest moment of my life so far,” says Jose.
In the N/IICU, Joseph was being hooked up to various monitors. He also received supplemental oxygen as he learned to breathe, and at one point, his little face was covered by a mask.
Jose had planned to stay one week, but he extended the visit to two. They had hoped to take Joseph home in that time, but he wasn’t ready.
“The baby calls the shots in these cases,” says N/IICU nurse Annabelle Rose, RN-NIC, one of the neonatal surgical registered nurses who often cared for Joseph. “We can only push him as far as he’s ready to go.”
Every day, Catalina would visit Joseph, taking only Sunday off. She would sit in his dimly lit three-walled pod, listening to the constant beep of monitors. She would pump and store breastmilk, which was being fed to Joseph through a tube into his stomach.
After 34 days of oxygen, Joseph was breathing on his own and taking a bottle by mouth. He would cry only if he needed food or a diaper change.
Soon, Rose found herself doing less and less medical care. “It was nice to see the transition from Catalina just sitting there to pretty much being an independent mom, with us there only as backup,” says Rose.
Their journey at CHOP, which started on May 30, ended on Oct. 26 in the best way possible: The Agosto family of three went home together. Jose drove through the night to pick up his wife and son so that Joseph wouldn’t have to endure the stress of a flight. Joseph slept the whole way home. On his first day in Florida, he met both of his grandfathers and Skyped with his uncle in Colombia.
Joseph’s doctors in Florida have given him a clean bill of health. There is even good news about his partial left lung.
“The amazing thing about babies is that even though we took out some lung, the remaining lung will grow to fill the space,” says Hedrick. “And that’s not true with older children or adults.”
There are small reminders of his surgery — a white dot on his left hand where the IV had been placed during surgery, and of course a long linear scar across his left chest — but his story continues to amaze all who meet him.
“He’s gone through so much and he looks like a normal baby,” says Catalina. At 4 months old, he weighs 12 pounds and will soon transition to solid baby foods.
In April, he will return to CHOP for a 6-month checkup with the Pulmonary Hypoplasia Program. Return visits not only help ensure that Joseph is getting all of the services he needs but also help give future fetal surgery families a better idea of what to expect.
The Agostos know how lucky they have been and how many families don’t have as happy an outcome. But despite 60/40 odds, they never lost their optimism.
“I never really thought about him not making it,” says Jose. “I was really positive the whole way through. Especially when Catalina went to Philly and CHOP. That was our last hope, but I knew she was in good hands and that he was going to be fine.”