“If she doesn’t make it, don’t wake me up,” Brandi told her doctor. It had only been a short five weeks since she found out that her daughter Jaelyn would be born with a severe congenital heart defect known as Ebstein’s Anomaly. Now, the SSM Health St. Louis Fetal Care Institute team was taking her into the operating room for a specialized emergency EXIT (Ex Utero Intrapartum Treatment Procedure) delivery.
Ebstein’s Anomaly is a rare congenital heart defect that occurs when the tricuspid valve of the heart does not form correctly. Normally, the tricuspid valve has three flaps that allow blood to flow from the right atrium of the heart into the right ventricle. In Ebstein’s Anomaly, one or two flaps are malformed, which causes blood to leak backward into the right atrium. This can cause oxygen-poor blood to be pumped out to the body. It can also cause the fetus’ heart to be enlarged, which may impact lung development.
Brandi was initially referred to the Fetal Heart Program at the St. Louis Fetal Care Institute because her niece had passed away after being born with a congenital heart defect. “Our doctor sent us just to get checked out as a precaution,” recalls Brandi.
During that first visit Renuka Peterson, MD, the director of the Fetal Heart Program, and the SSM Health Cardinal Glennon Children’s Hospital Echocardiography Lab found a slight leakage in Jaelyn’s tricuspid valve that would require follow-up monitoring.
During a follow-up ECHO (echocardiogram) when she was 30-weeks pregnant, Brandi found out that Jaelyn had Ebstein’s Anomaly. “My first reaction was shock, then I cried, then I did as much research as I could,” says Brandi.
To confirm the diagnosis, Brandi and her husband Kaleb sought a second opinion that confirmed the St. Louis Fetal Care Institute was the best place for their care. “After all of our research, I knew the doctors at the St. Louis Fetal Care Institute were the best to care for Jaelyn. The team treated us like human beings and answered every question we had. They would even just sit and hold my hand if that is what I needed. We never felt like we were just another number,” says Brandi.
The team continued to monitor Brandi and Jaelyn, and at 35-weeks they discovered a severe case of hydrops, which is an indication of fetal heart failure. It was decided Jaelyn would make her entrance that day. Because of the severity of Jaelyn’s heart defect, the safest delivery was an EXIT procedure at Cardinal Glennon where Jaelyn and Brandi would have immediate access to the highest level of care.
The purpose of an EXIT delivery is to provide the necessary intervention to the baby before the umbilical cord is cut, and allowing for a more stable transition from placental circulation to newborn circulation. During the procedure, the mother is placed under general anesthesia. The delivery is started similar to a cesarean section, however, a special device is used to open the uterus and prevent uterine bleeding at the same time. The baby’s head and arms are delivered. Monitors are placed on the baby and IV access is obtained. The baby remains attached to the placenta, which serves as life support, while the surgeons, neonatologists, and cardiologists perform the necessary procedures to stabilize the baby.
When she was born, Jaelyn needed assistance breathing on her own, but under the care of the team of nationally recognized pediatric cardiologists and neonatologists at Cardinal Glennon, Jaelyn became stronger and healthier. “We were in the NICU for almost four weeks, and it should have been months. Every person that saw her said she was the true definition of a miracle baby,” says Brandi.
Jaelyn continues to be regularly monitored by Dr. Peterson and the cardiologists at the Dallas Heart Center at Cardinal Glennon but has not required surgery for the Ebstein’s Anomaly.
“This experience taught me to reach out and ask others around for support. Parents shouldn’t try to do this on their own. The best thing I had around me was my family. Whether it was my best friends or my actual family, I had the best support group you could ever ask for,” says Brandi. “I also learned that I am stronger then I thought, and so is my daughter. We should never underestimate how much fight we have in us when we are fighting for something so important.”
Now, two-year-old Jaelyn has a smile that lights up the room. She loves playing outside and in the basketball gym, just like her mom and dad. “She is actually ahead of schedule developmentally and is so smart. She is just perfect,” says Brandi.
“Never in my life, when someone explained to me everything my daughter was going through during my pregnancy, did I ever think she would still be with us today. The amazing team at the Fetal Care Institute made it possible,” says Brandi.
Since opening in 2009, 13 babies have been born via an EXIT procedure at Cardinal Glennon. The team at the St. Louis Fetal Care Institute has performed thousands of fetal ECHOs, and helped families and babies with a congenital heart defect diagnosis.