Everly Now Thriving Years After Lifesaving Surgery Before Her Birth

The youngest of five siblings, 2-year-old Everly Cooper rules the roost at home. A daddy’s girl, Everly tags along behind her older sister and three brothers, trying to mimic everything they do.

“Everly bosses us all around,” said her mother Roxanne, laughing. “She wants to do whatever the older kids are doing and thinks she can do anything they can do.

Though Everly is a thriving toddler now, doctors discovered a rare, life-threatening condition during the early weeks of her mother’s pregnancy. The Fetal Care Center team at Cincinnati Children’s performed lifesaving surgery on Everly before she was even born.

Routine Ultrasound Reveals Rare Condition – TRAP Sequence

Roxanne and David Cooper of Townsend, Tennessee, weren’t planning to have a fifth child. But they were overjoyed to find out about the surprise pregnancy in November 2019.

A routine ultrasound at 15 weeks gestation revealed another surprise: Roxanne was pregnant with twins. But her maternal-fetal medicine (MFM) doctor in Knoxville discovered an alarming complication. The expectant mother’s monochorionic twins—meaning they are identical and share a single placenta—were diagnosed with twin-reversed arterial perfusion, also known as TRAP sequence.

This condition occurs in about 2.6% of monochorionic twin gestations (or 1 in 10,000 pregnancies). It means that one twin cannot survive because it lacks a heart and defined anatomical features such as a head, torso or limbs. While in utero, this twin (called the acardius) receives its blood supply from the twin with a working heart, known as the “pump” twin (Everly, in this case).

“We experienced a range of emotions,” said Roxanne, whose father is an identical twin. “At first, we were in shock. Then, we were excited to learn that we would have twins, and then we felt let down that the second baby wasn’t going to make it.”

As TRAP sequence twins grow in the womb, the increasing demand for blood puts a strain on the pump twin’s heart, which is working twice as hard to sustain life. The pump twin, who usually develops normally, is at risk for heart failure. Left untreated, this complication often results in the death of both babies.

In hopes of saving Everly, Roxanne’s MFM doctor referred her to Foong-Yen Lim, MD, surgical director of Cincinnati Children’s Fetal Care Center.

“TRAP babies can come in all forms, shapes and sizes,” said Dr. Lim. “These severely malformed fetuses (i.e. acardius) have no chance of surviving. Unfortunately, because Everly shared the same placenta with her twin, she also was at risk of dying because she was pumping blood to the acardius.”

Coordinated Care Across Many Miles

Roxanne was seen at Cincinnati Children’s two days after the TRAP sequence was diagnosed, when she was around 16 weeks pregnant. Everly’s condition was stable at the time. Dr. Lim and the fetal care team began closely monitoring Everly’s heart function, the acardius’ growth rate and amniotic fluid levels. Roxanne made the trip from Tennessee to Cincinnati about every two weeks, with her MFM team managing her care at home. 

The fetal care team would eventually need to treat the TRAP sequence. But Dr. Lim wanted to balance the risks and benefits of surgery while the pregnancy was progressing to reduce the chance of premature birth.

“At early gestation, the pump twin’s heart is usually still strong, and there's no other sign saying that we absolutely have to do surgery,” Dr. Lim said.  

When Dr. Lim first saw Roxanne, the acardius-to-pump twin weight ratio was 38%. By 19 weeks and six days, that ratio had increased to 69%, indicating that the acardius was growing fast. Everly also was showing signs of developing anemia. It was time to intervene.

Dr. Lim and the fetal care team performed radiofrequency ablation (RFA) to shut off blood supply to the umbilical cord of the acardius. During this procedure, the surgeon uses ultrasound to guide a slim needle through the mother’s abdomen and uterus to the umbilical cord of the acardius. Radiofrequency energy delivered through the instrument stops the blood flow. 

A day after surgery, Everly’s anemia symptoms had improved. The Cincinnati Children’s team sent Roxanne back home to the care of her MFM team for the duration of her pregnancy.

Multidisciplinary Team Collaborates With Doctors Across the Country

At the Fetal Care Center, our team collaborates with more providers than just specialists within the hospital. We also coordinate care for high-risk patients with MFM specialists throughout the region and beyond.

“We could not have given Roxanne and baby Everly the care they needed without our collaboration with her doctors in Knoxville,” Dr. Lim said. “We always involve our referring physicians in the care and management of these patients. Even if they're already back in their hometowns, we remain available in case any questions or issues arise. We hope to minimize the chances of the patients needing to come back to Cincinnati unless it's truly necessary.”

For Roxanne, the seamless coordination of care put her mind at ease during a difficult pregnancy.

“We had a wonderful experience at Cincinnati Children’s,” she said. “The fetal care team was excellent, and we’re grateful that they saved Everly’s life.”

Roxanne’s water broke at 31 weeks gestation, but doctors put her on bedrest to let Everly continue growing as much as possible. Everly was born at 33 weeks, in June 2020, weighing almost 5 pounds. She stayed in the neonatal intensive care unit (NICU) for 40 days before going home.

As the family now anticipates Everly’s third birthday, Roxanne describes her youngest daughter as “super independent, healthy and happy.” Though Everly was a surprise addition to the family, Roxanne said, “We didn’t know that we needed Everly, but now we can’t imagine our lives without her.”

(Published May 2023)