All babies enter the world with their own special flair.
But for Genesis Boss, the entrance came with a most dramatic EXIT, as well.
Genesis arrived through a rare kind of Cesarean section in which only her head was delivered at first―providing doctors precious time to open up her blocked airway before she needed to draw breath.
The EXIT procedure (ex-utero intrapartum treatment) required a host of experts, many of whom waited in the wings ready to step in if Genesis or her mom needed extra support. But Genesis sailed through it beautifully.
“I think it’s kind of cool,” said Annie Boss, cradling her baby girl in the neonatal intensive care unit at Spectrum Health Helen DeVos Children’s Hospital. “I think it’s especially neat that everything went together so well.”
Although Spectrum Health doctors have performed it before at Butterworth Hospital, Genesis was the first baby to undergo the procedure at Helen DeVos Children’s Hospital.
That’s just one more reason little Genesis, now a chubby-cheeked 6-month-old baby, is “the Boss” in her mother’s eyes.
Annie Boss is a high-energy mother with three older children. She lives in East Jordan in northern Michigan and works as a waitress to support her family.
At first, her fourth pregnancy seemed much like the others. But after a 20-week ultrasound showed Genesis was much smaller than average, Boss turned to Spectrum Health’s maternal fetal medicine specialists in Grand Rapids.
A more detailed ultrasound revealed Genesis had an undersized jaw―a condition called micrognathia. The small jaw pushed the tongue back into her throat, obstructing the airway.
I think God picked a good one to take care of her.
The condition also prevented Genesis from swallowing amniotic fluid, as babies do in the womb. Twice, Boss had to have a couple of liters of excess amniotic fluid drained from her uterus.
The ultrasound also showed an atrioventricular canal defect in Genesis’s heart. Eventually, she would need surgery to repair the hole in her heart.
But first, doctors addressed the most pressing concern: making sure Genesis could breathe after birth.
If a baby were born with a blocked airway―and the medical team was unaware of the condition in advance―the child likely would start gasping for air immediately, said Vivian Romero, MD, a maternal fetal medicine specialist with Spectrum Health. In the time it could take to open the airway, a lack of oxygen could cause brain damage or even prove fatal.
Knowing Genesis’ condition in advance, Boss’ physicians worked to ensure a smooth transition from the shelter of her mother’s womb to a world where she must inhale oxygen into her lungs.
As Boss approached her due date, the fetal medicine specialists began to assemble a team for an EXIT procedure.
At the 38th week of her pregnancy, Boss was wheeled into an operating room for her C-section and saw two dozen doctors and nurses waiting for her.
“I was OK with it. I’m not really afraid of surgery, especially if it’s best for her,” she said.
The EXIT is designed to keep the child on placental circulation, even though they are outside of the mom.
But in the last moments, she became nervous. She looked up at Dr. Romero, who held her hand.
“Don’t worry,” the doctor said. “Sweet dreams. It’s going to be fine.”
Dr. Romero and Marcos Cordoba Munoz, MD, also a fetal medicine specialist, performed the C-section.
Dr. Cordoba made an incision in the lower abdomen and went through the layers to get to the uterus. He ruptured the membrane to break the water, and amniotic fluid poured forth.
As he eased out the baby’s head and shoulders, Dr. Romero slid catheters between the baby and the uterus to infuse fluid into the placenta.
The goal was to prevent the uterus from contracting and causing the placenta to separate―as it typically does after a baby is born.
“The EXIT is designed to keep the child on placental circulation, even though they are outside of the mom,” said John Girotto, MD, a pediatric craniofacial plastic surgeon. “Physiologically, they are still in the mom. It’s that balance between delivery and not.”
Dr. Romero placed an ultrasound probe on the baby’s chest. It showed the heart steadily beating.
With the baby stable—and still halfway in her mother’s womb, Dr. Cordoba stepped aside to make room for Chad Afman, MD, a pediatric otolaryngologist.
Dr. Afman intubated Genesis, placing a flexible plastic tube into her windpipe to keep the airway open.
If the intubation did not work, he was prepared to perform a tracheotomy, creating a hole in the windpipe through her neck. But that step was not needed at that moment.
The doctors delivered Genesis. They quickly cleaned a finger to put on a device to measure the oxygen level in her blood. When they felt confident her airway was clear and she had a healthy blood oxygen level, Dr. Cordoba clamped and cut the umbilical cord.
Genesis, a 4-pound, 14-ounce baby girl, made her official arrival into the world.
Dr. Cordoba handed her to a neonatologist. His reaction at that moment: “Relief.”
With Genesis in good hands, the fetal medicine specialists could focus their attention on her mother.
“This is a very complex surgery,” Dr. Cordoba said. “It does carry risk for the baby and the mom.”
Mothers are at increased risk of bleeding because of the continued blood flow to the placenta. Blood transfusions were available in the operating room, although Boss did not need them.
Ready for challenges
Crucial to the EXIT procedure were the efforts of two anesthesiologists–one each for mother and child.
During the operation, Dr. VandenBerg “had the unique job of preventing contractions in the uterus until Genesis was fully delivered and safe in terms of her airway,” Dr. Afman said. “Dr. Huntington assisted me with obtaining the airway and monitoring for any trouble with Genesis, ready to give extra anesthesia and care if needed.”
Other physicians on hand included the neonatal intensive care team and a pediatric surgeon ready with a heart-lung machine, in case Genesis needed it. Gynecologic oncologists were on hand in case Boss needed an emergency hysterectomy.
The physicians had done a trial run before the surgery to consider and prepare for any potential challenges.
“It was a lot of coordination and prepping for a worst-case scenario, and we never got there,” Dr. Afman said. “And that’s a good thing.”
“The baby is the best person to tell you what’s going on,” Dr. Romero said. “We never saw any drop in the heart rate.”
The EXIT procedure is rare, in part because few babies are born with conditions that require it.
“It’s also rare because it’s fairly new,” Dr. Afman said. “This is the result of emergent technology. We can do more diagnostic things on kids before they are born and anticipate problems before they happen.”
In a room in the NICU, Boss lay on a floor mat with her daughter after a physical therapy session.
“How big is Genesis?” she chanted, holding the baby’s arms over her head. “Sooooo big.”
Genesis reached for a stuffed bear. She brought her hands up under her chin, twirling the fingers like a thoughtful scholar.
Her plump arms and legs revealed how much she had grown in the six months since birth―her weight reached 13 1/2 pounds.
In those months, Boss and her mother, Shirley Boss, worked as a tag team to keep someone by Genesis’ side nearly every day
Boss spent the weekdays with Genesis, staying nearby at the Ronald McDonald House of Western Michigan.
Her mother spent the weekends with Genesis, allowing Boss to head home to spend time with daughters Halah, 16, Sophia, 15, and Ava, 5, and to wait tables at the Torch Lake Café.
Genesis faced several more challenges after birth. She underwent a procedure to lengthen her lower jaw. And because her lungs are underdeveloped, she had a tracheostomy.
Boss also consulted with the congenital heart specialists about the next steps to repair her heart defect.
She laughed as she talked about her goal for 2017―she had planned to learn a new language.
“I didn’t, but I learned how to take care of a trach,” she said. “I can change a trach now. I can suction a baby. I learned a lot of medical words I never would have learned otherwise.”
She looked forward to the day she could take Genesis home and put her to bed in her own crib, one covered with pink and black and white hearts. She longed to bring her outdoors, to let her feel the wind and see trees and animals.
In the meantime, she celebrated each milestone Genesis reached. And she treasured friendships with the doctors and NICU nurses.
“I have a pretty good faith in God, and that helps,” she said. “And I am probably the most laid-back person on the planet. I just kind of roll with things.
“I think God picked a good one to take care of her.”
Dr. Romero agreed.
“Annie is wonderful,” she said. “She is just amazing.”
Boss pulled off Genesis’ socks, revealing tiny toenails painted a deep pink. Genesis stared at the eye-catching toes.
“She loves it when you play with her feet,” Boss said. She bent to plant a kiss on her baby’s forehead. “You are so pretty.”
Taking pictures and videos, she has documented each step of Genesis’ life, from the day she made her unusual EXIT and entrance into the world.
“I can’t wait to tell her the story someday,” she said.