“It’s a pretty crazy story,” recalls Zheni Colon. It was early 2015. Zheni was a third-year recruit in the U.S. Army, stationed in Alaska, and her then-fiancé Johnathan was stationed at the Army base in Fort Drum, NY, when they learned they were pregnant.

Despite the distance between them, the couple was thrilled to begin building a family. When she was 18 weeks pregnant, Zheni went to her anatomy scan, excited to learn the sex of the baby. She was shocked when the doctor said her baby girl had a sacrococcygeal teratoma (SCT), a potentially life-threatening tumor located at the base of her tailbone.

SCTs can develop unpredictably — and dangerously — so it is critical that mothers whose fetuses are diagnosed with the condition go to a center with extensive experience. Johnathan researched the condition online and discovered the Center for Fetal Diagnosis and Treatment (CFDT), an internationally recognized leader in fetal diagnosis, fetal surgery and fetal care, and one of the most active maternal-fetal centers in the nation.

The team at the CFDT has the greatest experience in the world with fetal management of SCT, having counseled or cared for more than 300 cases. Center Director N. Scott Adzick, MD, MMM, a world-renowned pioneer in the diagnosis and treatment of birth defects, performed the world’s first successful prenatal surgery for SCT in 1996, in which the mother’s uterus is opened, the tumor removed and the fetus placed back in the womb to continue the pregnancy for as long as possible. Only 15 such surgeries have ever been performed, and Adzick has done nine of them.

Finding hope despite insurance obstacles

Johnathan called the CFDT and spoke with advanced practice nurse and lead clinical coordinator Sue Spinner, MSN, RN. Sue contacted Zheni to set up an initial consultation, but the referral to the CFDT was denied by her military insurance. Knowing that SCTs can rapidly become life threatening to both mother and fetus, Spinner remained in contact with Zheni throughout the pregnancy, checking in on her every two weeks or so.

“We were worried about her because she couldn’t get a referral,” says Spinner. “She didn’t seem to have access to the medical support she needed.”

“Sue wanted me to be at CHOP because she knew if I didn’t get seen there, something bad could happen to my child,” says Zheni.

Zheni managed to get an honorable discharge from the Army and moved to Fort Drum to be with Johnathan, whom she married soon after. She made an appointment at a nearby maternal-fetal medicine center and hoped the physicians there would refer her to the CFDT. But her new providers believed the tumor wasn’t large enough to warrant a referral and were confident they could manage the pregnancy and remove the tumor after birth.

The standard of care for SCT at Children’s Hospital of Philadelphia includes regular monitoring throughout the pregnancy — at least every two weeks — to track changes in the tumor’s size and the baby’s heart function, but Zheni went months without an appointment.

When she finally returned to the MFM center in upstate New York three months later, she was nearly 38 weeks pregnant and already showing early signs of labor, and the tumor had doubled in size, from 6 to 13 centimeters. Zheni recalls nearly fainting when she saw the ultrasound.

“It was humongous,” she says. “I said, ‘Something’s not right. This tumor is almost as big as her head.’”

Frightened, Zheni and Johnathan packed their things and drove to her parents’ house in New Jersey to be close to CHOP. They arrived in the area on a Friday and underwent a comprehensive evaluation the following Monday.

“I got more care in one day at Children’s Hospital than I got in my entire eight months of pregnancy,” says Zheni.

Saving Ava

The tumor had grown to roughly the size of a softball and was causing strain on the baby’s heart, a detail missed by the center in New York. The CFDT team scheduled an emergency c-section for the next day and baby girl Ava was born in CHOP’s Garbose Family Special Delivery Unit, the world’s first birth facility exclusively for healthy mothers carrying babies with known birth defects. A specialized fetal/neonatal surgical team was standing by in case immediate tumor resection was needed.

Ava was stabilized, and the next day Adzick surgically removed the tumor. Were it not for the CFDT’s expert management, delivery and surgical skill, she would not have survived.

Zheni’s health was threatened as well. She lost a large amount of blood during the delivery, her left lung collapsed and she contracted pneumonia. She had to be admitted to the Hospital of the University of Pennsylvania, adjacent to CHOP. Fortunately, she and Ava both recovered quickly and were discharged home within a week.

Today, Ava, now 4 months old, is “doing amazing,” says her mom. She will be monitored every three months for the first year of her life and may need reconstructive surgery on her bottom because the tumor took up most of her backside, but she’s not limited in any way.

“She’s perfect,” says Zheni. “Had Sue not kept in touch with me the whole time I wouldn’t have known how serious this was. My daughter wouldn’t be alive. They saved my daughter’s life and how can you forget that? You can’t and I never will.”

Originally published: January 2016