Conjoined twins with unbreakable bond learning to live apart
“You have strong sisters, don’t you?”
Ernesto Martinez pulls his 4-year-old son Carlos onto his lap as he comes running from the playground. Carlos returns his dad’s smile, and follows his gaze to the building just a couple hundred yards away: The University of Minnesota Masonic Children’s Hospital.
Masonic Children’s Hospital became home for Paislyn and Paisleigh Martinez before they even were born. A routine ultrasound revealed that Paisleigh and Paislyn were conjoined twins, and their mother Paris Bryan was quickly referred to the University of Minnesota Health Maternal-Fetal Medicine Center, which cares for women experiencing pregnancy complications. Conjoined twins are extremely rare, accounting for approximately one in 200,000 live births.
University of Minnesota Masonic Children’s Hospital springs into action
“When I was pregnant with them they were basically saying they weren’t sure they would make it,” said Paris.
But Bryan put trust in the Medical School and her care team at The Birthplace at the University of Minnesota Masonic Children’s Hospital. Bryan was put in the care of Katherine Jacobs, D.O., who specializes in caring for women with high risk pregnancies. As the babies grew and developed, so did the team designated to care for them.
“Caring for Ms. Bryan and her twins highlights how well our institution functions as a team,” said Jacobs. “The communication between the various teams was outstanding, and each team respected the other specialists’ unique roles.”
That team included sonographers, nurses, care coordinators, surgeons and physicians. They cared for Paris and closely monitored the girls’ growth and development. To prepare for their birth, the team conducted a fetal MRI to better define the girls’ anatomy.
The more detailed images revealed they were thoraco-omphalopagus conjoined twins. The girls were connected from their chests to their belly buttons, their livers were fused in the middle and their hearts were connected. Complicating matters even further, the care team determined that one of the girls, Paislyn, had a serious heart condition called tricuspid atresia with transposition of the great vessels and a ventricular septal defect.
“One has heart disease, one doesn’t, but how do you know which is which? From the outside you don’t,” explained Pediatric Cardiologist Matthew Ambrose, M.D.
“After birth it can be challenging to distinguish identical twins, even for parents,” said Jacobs. “The same thing applies during pregnancy and delivery.”
So, Paislyn and Paisleigh became “Baby Red” and “Baby Blue” to their care team to help differentiate the two.
Jacobs and her team went to great lengths, frequently recording measurements and anatomic details for each baby throughout the pregnancy. Details like this were planned during frequent care team meetings, and then put to practice during an obstetric simulation. Delivery was planned for the month following that simulation, but Paisleigh and Paislyn had other plans, and were born that same day- February 10, 2017.
“It worked out perfectly to have run through the birth scenario just prior to the real event,” recalled Jacobs.
Beating the odds
Paris remembers first moment she met Paisleigh and Paislyn and it was unlike anything she could have imagined.
“When I first got to hold them I broke down crying,” she said. “It was the most amazing feeling ever because I never thought I would get that.”
The girls had beat incredible odds, but there were more hurdles ahead. Doctors determined the best scenario for their survival meant they had to be separated, and soon. Physicians from the University of Minnesota Medical School formed a care team, representing multiple specialties including: critical care, neonatology, nephrology and surgery. They had already been meeting weekly to plan for the surgery, even before the girls were born.
“It was wonderful working with the collaborative team in the NICU setting, each with a focus on getting the girls bigger and more mature in preparation for their separation,” said Neonatologist Thomas George, M.D.
[Paisleigh] was working overtime, or doing double the work to support both children.
The girls were getting more acquainted with one another, too. Because of the way Paisleigh and Paislyn were connected, the girls naturally faced each other. As they slept and played and snuggled, they put their arms around one another in a hug.
They learned more details about Paislyn’s heart defect, too. Her heart was too weak to pump blood on its own. There was also a risk of too much blood flow, too quickly, from her heart into her lungs. Paislyn's heart condition also led to a severe kidney impairment. Pediatric Nephrologist Christian Hanna, M.D., M.S., was called in to help. But the girls were about to surprise everyone again. Paisleigh was actually helping her sister.
“Paisleigh was essentially acting as a dialysis machine for Paislyn,” explained Hanna. “It was fascinating to see.”
But that wasn’t the only way Paisleigh was helping her sister.
“Paisleigh’s circulation, and Paisleigh’s heart, actually helped support Paislyn and her circulation,” explained Pediatric Cardiothoracic Surgeon Tony Azakie, M.D., Ph.D. “She was working overtime, or doing the double the work to support both children in some ways.”
“I honestly didn’t expect this to happen, that her sister would be helping her with everything she’s struggling with,” Ernesto said about his daughters, “I really don’t even know how to explain it. Lot of emotions, but mainly happy and proud. I’m glad that she’s doing all this stuff for her sister.”
Preparation and planning had been a staple in the months leading up to the separation surgery. Part of that involved in depth imaging, courtesy of state of the art technology. The care team worked with the Earl E. Bakken Medical Devices Center at the University of Minnesota to create a 3-D reconstruction of the babies’ hearts. One of the devices they used is one of only 5 in the world, and was actually developed by graduate students inside that very center.
A bump in the road, a first of its kind procedure
Despite the constant monitoring and preparation, unforeseen complications still found the girls less than a week before the scheduled separation surgery.
Paislyn started getting very ill. Doctors noticed a drop in her blood pressure and changes in her urine output. If she was to going survive the upcoming surgery, the medical staff needed to act quickly. Pediatric Cardiologist Gurumurthy Hiremath, M.D., stepped in to help.
With Paislyn’s form of congenital heart disease, it’s important for there to be a hole between the two upper chambers of the heart in order for her to survive. The hole had gotten too small and needed to be opened up, otherwise, it was likely Paislyn would not survive after separation. That had to be done through a procedure called a cardiac catheterization.
We were in unchartered territory. - Dr. Hiremath
“We were in unchartered territory,” said Hiremath. “There has been no report of a transcatheter intervention of any kind being performed in conjoined twins before separation.”
Despite several challenges, Hiremath was able to insert catheters into the heart through the femoral vein (the blood vessel in the leg) and reach across the hole in between the upper chambers of the heart. Then he inserted balloon catheters across the septum, pulling the balloons across the wall between the upper chambers to create a bigger hole. The procedure, called cardiac catheterization with balloon atrial septostomy, was successful in essentially ripping a large hole between the two upper chambers. It stabilized Paislyn’s heart for surgery.
“We all worked so smoothly, effectively and efficiently. It did not feel like it was our first time,” said Hiremath. “The whole experience was planned and executed to perfection.”
A moment worth waiting for
“We have a lot of people in the room, and it would be really nice to have everyone tell us, who is with what and what we’re doing.”
In a moment that almost seemed out of place in the operating room that Thursday morning, May 25, more than 30 medical professionals took a moment to introduce themselves to each other. Even through the scrubs, and surgical caps, color coded red and blue dependent on which twin the team member was assigned to, “Red Baby” or “Blue Baby,” most people were also familiar with each other. They had, after all, been preparing for this moment for months.
In a way then, what was taking place in the Masonic Children Hospital’s third floor OR in that moment seemed to be the turning point from preparation to separation. Three anesthesiologists had already done their job, while four general surgeons, two cardiac surgeons, three cardiologists, four nurse anesthetists, four surgical techs, two heart pump techs, four operating room nurses, and two nurse managers stood by. And now, it was their turn.
“One of the most memorable experiences from the operating room for me was when Dr. Azakie put a clamp across the bridge- their hearts beat separately for the first time. That for me was a huge. It was really evocative,” shared Ambrose.
Azakie opened the chest wall and divided the ribs. He then separated the “bridge” that connected the girls’ hearts.
After a total of nine hours, Paisleigh and Paislyn were separated.
“We had watched them connected for so many weeks, and now the final separation had occurred, so there was something surreal about it,” said Azakie.
After the separation, Azakie performed a procedure on Paislyn’s heart called pulmonary artery banding.
A great distance covered, a long journey ahead
Both girls still have a long road ahead of them, including a few more operations and procedures. They will both need surgery to get chest wall coverage to cover their hearts. Paislyn will also need one or two more heart surgeries due to her single ventricle anatomy.
As they recover, they are in the care of the pediatric critical care team. Ashley Loomis, M.D., is one of the doctors focused on their care at this stage, particularly Paisleigh’s.
“She loves to get attention from folks,” said Loomis, a smile immediately stretching across her face as she talks about the now 6-month-old Paisleigh. “You can just go on in and love on her when you’re having a bad day.”
Loomis and the team pay special attention to nutrition and lung development. They also make time to have “playdates” for the girls- bringing them together as often as their conditions allow, now that they are recovering in separate rooms.
“It takes a village. For all of our patients here in the ICU, but even more so for Paisleigh and Paislyn. It’s been a team effort to get them where they are today,” said Loomis.
Bryan says she’s excited for all the little things - for nail polish, and matching outfits, and to continue to see how their personalities grow and help shape them into the people they will become, both together and apart.
“It’s been amazing to watch them grow, and see how far they’ve come,” said Bryan. “It’s been a really great journey.”