Pediatric palliative care enhances quality of life for patients, families

When Brett and Rhonda Yarrington learned that the baby she was carrying had Trisomy 18, a chromosomal abnormality, they weren’t given much hope. The condition causes birth defects that often lead to death in the womb or shortly after birth.

“Typically in the general medical world, they’ll call that diagnosis ‘incompatible with life,’” the father says. “Depending on who you talk to, it can be very much a doom-and-gloom scenario: You shouldn’t expect to carry this baby full term, and if you’re lucky enough to carry this baby full term, you’re only going to have a very short period of time with this baby.”

But Bella Grace Yarrington was born on Feb. 20 and, following two major surgeries, is at home in Waynesville with her family.

Brett Yarrington credits Dr. Paul Furigay and the rest of Mission Hospital’s Pediatric Palliative Care team with providing invaluable advice and support that helped the couple through the arduous process.

“When we met with Paul, we formed a very close connection with him that continues to this day,” he says. “He offered us a lot of insight. He gave us hope. I think we had a misconception about palliative care that this meant hospice; this meant terminal illness.” Dr. Furigay, says Yarrington, “let us know that we’re on Bella Grace’s schedule, and [he and his team] were there to support us with whatever type of comfort measures or even drastic measures to ensure our time with her was quality time.”

Furigay, a Mission Children’s Hospital physician who is certified in palliative care, says the roots of the specialty have been around a long time.

“Going back to the 15th century, ‘palliate’ in Latin means ‘cloak,’ to sort of protect in a way,” he says. “And I like to use that as an image for trying to protect these kids and families from any unnecessary pain or harm that might come to them throughout their experience.

“In Greek and Roman times, there was a recognition that people couldn’t cure everything, and that people suffered, and that a lot of attention to the alleviation of pain and suffering was just as important as trying to cure things. That’s been going on for a long time, but it’s usually been done as sort of a comprehensive service provided by the main doctor or the main medical team.”

Training in palliative care focuses on pain relief and management of symptoms, he explains. The underlying goal is to enhance quality of care for those facing chronic illness or a lethal diagnosis, Furigay says.

“It’s addressing the medical needs of the family but also their larger psychosocial, spiritual and existential needs as well,” he says. Furigay says palliative care complements other therapies.

Yarrington, who works as an IT consultant for an Asheville company, says that at about 20 weeks into his wife’s pregnancy with their fourth child, an ultrasound revealed a problem. She was referred for further testing, and amniocentesis led to the diagnosis of Trisomy 18, commonly called Edward’s Syndrome.

Caused by an error in cell division, Trisomy 18 results in extra chromosomes in the developing baby and disrupts the normal pattern of development in significant, often life-threatening ways, even before birth. In the United States, a Trisomy 18 error occurs in approximately one out of every 2,500 pregnancies and one in 6,000 live births.

Termination of the pregnancy wasn’t an option, says Yarrington.

“My wife and I are very strong practicing Catholics, and we are very much pro-life,” he says. “We firmly believe that a conceived child is a human being and should be treated with dignity and respect.”

The couple learned more about the malady through the Trisomy 18 Foundation, he says.

“The scenario wasn’t as doom and gloom as the information that we were given by the specialists,” Yarrington says. “There are some success stories out there that are somewhat rare, but these babies can live to be in their 20s an 30s and beyond.”

Furigay was with the couple in the delivery room when Bella Grace was born at full term.

“Dr. Furigay called me over to the side in that room and said, ‘Get your hands on this baby. This is a miracle,’” Yarrington says. The doctor “was a very calming presence in helping us through that and throughout the hours afterward.”

A week after Bella Grace’s birth, she had surgery to connect her esophagus to her stomach. Yarrington said he learned that some health systems won’t perform that procedure.

“A lot of [them] view Trisomy 18 babies as a lost cause,” he says. “They don’t want to put time and resources into a baby who might otherwise just not make it. What the group at Mission did is they looked beyond her syndrome, her diagnosis, and they treated her as a patient, as a human. And the surgery was successful.”

Before going home, Bella Grace spent a month in the Neonatal Intensive Care Unit and had more surgery to correct a heart defect. Through it all, the Pediatric Palliative Care team was there for the family, says Yarrington.

“They were our most trusted advocates and our most trusted advisors in everything,” he says. “I don’t know if I even have the words to describe what [Furigay] means to our family.” The care team was “at our beck and call whenever we needed them,” says Yarrington.

Palliative care has “that association with dying or with end of life,” says Furigay. “Unlike palliative care in the adult world, where it’s reserved for end of life or transition to hospice-type scenarios, pediatric palliative care tries to address a child’s experience with the health care system throughout [his or her life], whether it’s going to be for months or years or into young adulthood.”

A child with cancer, for example, “might have a great prognosis three or four years down the line. But for those [first] three or four years, that child is going to be in and out of the hospital, being subjected to incredible amounts of what might otherwise be uncomfortable, painful, certainly dehumanizing experiences.”

Yarrington says he realizes his daughter’s future is very uncertain. Trisomy 18 patients often succumb to heart failure or respiratory ailments like pneumonia.

“Our daughter’s heart is repaired. She’s gaining weight and doing well,” he says. “But with this diagnosis, every day is a gift, every hour is a gift. One of the things Paul told us is we need to enjoy every moment that we have with her. Because much like with anybody’s life, there’s no telling how long you’re going to have with somebody.”