Editor’s Note: As the nation marks National Women’s Health Week, PBS NewsHour will share the stories of three women — and their doctors — who refused to allow a cancer diagnosis interfere with a successful pregnancy. These are their stories of hope, perseverance and, ultimately, success.
Once a week, 2-1/2-year-old Cami Santee practices her walking in her grandfather’s Bentleyville, Pa., carpet store, where there’s lots of room for the tiny medical walker she needs for support.
Before Cami was born, a huge tumor began growing from her lower body and injured her hips and internal organs. To save her life, doctors had to operate on Cami en utero, half her body still inside a special incision in her mother Tami Dobrinski’s womb.
Talking about it usually makes Dobrinski cry, but she says it’s not because it makes her sad.
“It’s OK. It’s a happy story,” she said. “I never thought she wouldn’t be here.”
Cami’s tumor, called a sacrococcygeal teratoma, is just one example of an uncommon category of tumors and cancerous growths that can occur in unborn children — a teratoma like hers occurs in only one in 35,000 infants. According to Cami’s surgeon, Dr. Timothy Crombleholme, doctors don’t fully understand what causes teratomas like hers to spontaneously form on fetuses. However, it may have something to do with their location.
“We think that many of these tumors derive from stem cells,” said Crombleholme. Fetuses have a concentration of stem cells on their hindquarters called Hensen’s Node, which on a very small percentage of fetuses can evolve into a tumor like Cami’s.
“But we don’t know why they occur in only some babies, or why some grow more than others,” said Crombleholme, who is now surgeon-in-chief of the Children’s Hospital Colorado and Director of both the Colorado Fetal Care Center and the Colorado Institute for Maternal and Fetal Health.
Once a fetus develops a tumor, it can grow very rapidly — something else that doctors don’t fully understand. “It’s very likely that there’s something about the fetal milieu that supports these tumors,” said Crombleholme.
Dobrinski and Cami’s father Chad Santee found out about the tumor shortly after her first ultrasound. “We had the ultrasound. I got to listen to the baby’s heartbeat, and we left. And like an hour later they called and said we needed to go to the hospital right away,” said Dobrinski. Because of the large size of Cami’s teratoma, Dobrinski’s Pittsburgh doctors immediately advised her to terminate the pregnancy.
The doctors were concerned because Cami’s tumor was highly vascular, meaning that a great deal of the baby’s blood was passing through the tumor. According to Dr. Bill Polzin, co-director of the Fetal Care Center of Cincinnati, such tumors are very delicate.
“By the end of gestation, the tumor is like wet tissue paper: You can blow on it and it will rupture,” Polzin said.
If Cami’s tumor ruptured under the stress of birth, it would put the baby in extreme danger. According to Crombleholme, a seven-month-old fetus might have a total blood supply equal to about a cup and a half of liquid. “If a person loses 20 percent of their blood supply, they can go into shock,” said Crombleholme.
“Two tablespoons of blood loss is a significant danger to these babies,” Polzin said. “They can exsanguinate [bleed out] in under two minutes.”
However, despite the urging of the doctors, Dobrinski said that she never thought about terminating the pregnancy. “Even though they said that, I just never had a terrible feeling,” she said. “We felt that if she was going to be here, she would make it,” Santee said.
Frustrated with their local doctors, Dobrinski, Santee and their families started researching treatments for Cami’s condition. Eventually the couple connected with the Fetal Care Center of Cincinnati, where they met Crombleholme.
Crombleholme said that hospitals that don’t specialize in fetal care are not always familiar with all the possible treatments for rare conditions like Cami’s. His solution — one that is commonly used for large, highly vascular teratomas — was to remove the tumor while she was still in the womb. According to Polzin, operations such as this are only done when the situation is dire because of the danger to both child and mother.
Dobrinski faced the dangers of general anesthesia, infection and possible internal bleeding during the surgery, along with a more unique complication called mirror syndrome, which occurs when a fetus goes into heart failure during surgery. For reasons that doctors don’t fully understand, this can cause the mother’s body to respond by also going into heart failure, a potentially fatal situation.
Though Crombleholme warned Dobrinski of the dangers, she said that she never thought of not going through with the surgery. “It just wasn’t in my head not to do it,” Dobrinski said. “I didn’t think of it like that.”
When Cami was still two months away from full term, Crombleholme performed a procedure called ex utero intrapartum treatment, or EXIT. With Dobrinski heavily sedated, Crombleholme reached through a small hole cut into her uterus and pulled Cami’s legs, lower body and the dangling tumor out of the womb. He left Cami’s upper body inside the womb with the umbilical cord attached to help keep the infant alive. He then delicately cut away the tumor. Once the operation was finished, he completed Cami’s delivery without any complications.
“I love that guy,” Dobrinski said of Crombleholme.
Though the tumor removal was a success, it left its mark on Cami. Hip dysplasia keeps her from walking normally, and because of the way the tumor affected the growth of her kidneys and bladder, she needs a catheter every three hours. However, Cami’s father said there’s no sign of developmental delays and that he’s confident that Cami will walk one day, though she may need leg braces to do so. “She’s a genius in my mind,” said Santee.
Dobrinski said that during a recent physical therapy session, Cami’s therapist took his hands off her leg braces. Cami stood for 46 seconds before she noticed no one was holding her up and fell down.
“That’s a new record,” Dobrinski said.
Photos courtesy of Tami Dobrinski. Sacrococcygeal teratoma illustration by artist Holly R. Fischer. Image courtesy of the University of Michigan’s C.S. Mott Children’s Hospital.