Yolanda Stone comforts her newborn son, Kendarious, who underwent a newly developed fetal surgery procedure to attempt to correct a rare birth defect. (photo by Neil Brake)
Infant’s brief life may give others a fighting chance
The Advanced Maternal-Fetal Care surgical team performs the newly developed fetal surgery procedure on patient Yolanda Stone. (photo by Neil Brake)
Yolanda Stone's son, Kendarious, wasn't supposed to survive birth.
He did, but his mother only had three weeks to hold and love her son before he died. To most, that would be an achingly brief span, but for Stone, it was time that she said she wouldn't trade for anything.
Kendarious' short life was valuable, his mother said, because it may show doctors how to help other babies with his condition live even longer.
The Lauderdale County infant's condition was a rare birth defect called congenital high airway obstruction syndrome (CHAOS). Diagnosed early in Yolanda's pregnancy, CHAOS meant Kendarious' airway, or trachea, was missing just about halfway down his throat. For CHAOS babies, birth is usually fatal.
Members of the Vanderbilt Center for Advanced Maternal-Fetal Care team performed a newly developed fetal surgery procedure, called ex utero intrapartum treatment, or EXIT, to help Kendarious survive delivery so that the baby might have a chance at survival.
“Kendarious was here for a reason, to show this procedure can save lives,” Stone said. “I hope news about this procedure gets out to other mothers whose babies have this condition.”
“I have seen several babies over the years with problems like this. Once these babies are separated from the mother's placenta in the delivery room, everyone works so hard to save them. Seeing these babies die in 30 minutes is just tragic,” said William Walsh, M.D., chief of Nurseries at the Monroe Carell Jr. Children's Hospital at Vanderbilt.
“Babies with CHAOS always suffer from (loss of oxygen) at birth and 90 percent or more die,” said Katharine Wenstrom, M.D., instructor in Obstetrics and Gynecology at Vanderbilt University Hospital. “With EXIT, the mother's womb supports the baby throughout surgery. That's the point. At no time would the baby be without oxygen.”
Arguably the only answer for babies with CHAOS is the newly developed EXIT procedure, one that had never been performed in Tennessee. With EXIT, a pregnant woman's body is held in a state of suspension to offer a crucial bridge of time for doctors to perform fetal surgery just before the baby is delivered.
Ed Yang M.D., fetal surgeon at Children's Hospital who co-directs the Center for Advanced Maternal-Fetal Care with Wenstrom, had been involved in a number of EXIT procedures for a myriad of delivery issues at other hospitals, but this would be a first for the team at Vanderbilt. They agreed to try it when Stone came to them and asked them to consider her case.
Yang said this was only the second CHAOS case he has seen. “I think there has only been a handful around the country. It's a really bad disease.”
In a normally developing fetus, amniotic fluid flows in and out of the lungs during development, but in CHAOS, the flow is blocked because the windpipe is sealed, or blocked, at the neck. The lack of an airway itself usually makes birth fatal, but even if they survive, most CHAOS babies die within days from lung problems.
“It's like the lungs are a balloon in-utero, constantly blowing up with fluid that cannot escape,” Yang said. “The weakest part of the balloon blows up the most, so these babies have these large, cyst-like pockets in the lungs that may never function normally.”
Yang, Wenstrom, Walsh, and many others, including social workers and an ethicist, talked with Stone about her choices. She opted to undergo the EXIT procedure when the baby was full term. On Aug. 29, she was showing early signs of labor, so she came in to Vanderbilt for the procedure.
“The team approach is so important,” said Yang. “During Ms. Stone's delivery, our anesthesiologist, Dr. Ray Paschall, was able to fully relax the uterus, which is what makes the EXIT procedure — or any type of fetal surgery — possible because the placenta and umblical cord continue to supply the baby's oxygen.
“Then once Dr. Wenstrom brought out the baby's head, neck and arm, the other teams went to work, including neonatal nurse practitioners to start an IV, pediatric pain specialists, and ear, nose and throat expert, Dr. Shelagh Cofer, used a scope to examine the airway,” Yang said.
The baby's airway was successfully reconnected to the outside at his neck. Because Kendarious' lungs were not functioning at birth, he was immediately placed on Extracorporeal Membranous Oxygenation (ECMO).
“But the key was that there was time to do it,” Yang said. “We were able to make sure the baby was stable and well oxygenated before we cut the cord and delivered baby Kendarious.”
His recovery seemed amazingly good. Kendarious' lungs quickly began to function. He came off of ECMO after only four days and was discharged just three weeks after his delivery, on Sept. 21. But sometime the evening of Sept. 23, he went into respiratory distress. Kendarious stopped breathing, and medical personnel were not able to resuscitate him.
“I am sad, but my faith tells me Kendarious served an important purpose while he was here,” Stone said.
“I think it's important for other mothers like me to know about the EXIT procedure, because there will be more mothers who are told their baby will not surivive birth and Kendarious was proof that it is possible to save these babies and give them a chance at life.”