Shoreham family celebrates children's successful heart transplant surgeries each Christmas season

At Christmastime, two glowing hearts — one lit in red lights, the other blue — adorn the rooftop of Ashley and Brian Cotter's Shoreham home, where the couple cherishes the donated gifts that beat inside both their young children.

Three days before Christmas Day 2019, 6-month-old Ruby Cotter underwent an eight-hour, lifesaving heart transplant operation. Now 5 years old, she had been diagnosed with dilated cardiomyopathy, meaning her heart lacked the strength to adequately pump blood throughout her body. Ruby's younger brother, Everett, was born five days after Christmas 2020. The following December, he was diagnosed with the same condition. Now 3, Everett took his first steps in the hospital during his six-month wait for a new heart.

About once a month, the Cotters embark on the more than 70-mile drive from their Shoreham home to NewYork-Presbyterian Morgan Stanley Children's Hospital in Manhattan for Ruby and Everett's routine echocardiograms, biopsies and blood work. Occasionally, an otherwise common illness can mean a 104-degree fever for the sister and brother, or congestion that makes breathing difficult for them. Both take immunosuppressants and are more susceptible to catching common illnesses and those turning "more severe," especially in the winter, Ashley Cotter said.

"She got a staph infection so she got admitted for a week," she added about Ruby. "Then [Everett] had pneumonia and he was in for a week."

Feeling blessed

Despite the occasional setbacks, and considering that they spent Ruby’s first Christmas at NewYork-Presbyterian, the Cotters said they feel blessed, courtesy of two still-anonymous donor families. Every Dec. 22 — and then again on each June 11, the date of Everett’s transplant — the couple throws each child a "heartiversary," during which they fill their home with about 100 balloons, many heart-shaped — red for Ruby, blue for Everett — and sing "Happy Heart Day" before slicing up cake. All as a result of the selfless acts of people they will likely never meet.

"It was a gift for us but a tragic day for them," Brian Cotter said at the family home on a recent afternoon while Ruby and Everett laughed and used colored markers to doodle on sheets of paper in the next room. "Their holidays are probably still a rough time for them."

About 500 pediatric heart transplants are conducted every year in the United States, usually because of a form of cardiomyopathy, which weakens heart muscles, according to NewYork-Presbyterian's website. The condition's various forms can develop in people of any age.

Nearly 40% of all children diagnosed with dilated cardiomyopathy, when the left ventricle swells and fails to adequately pump blood to the aorta, either undergo a heart transplant or die within two years of diagnosis, according to a study in the Journal of the American College of Cardiology and available on the website for the National Library of Medicine, part of the National Institutes of Health.

Ruby and Everett will likely need second heart transplant surgeries when they are “young adults,” said Dr. Irene Lytrivi, a pediatric cardiologist at NewYork-Presbyterian Morgan Stanley Children’s Hospital who has treated both since meeting Ruby in 2019.

Ruby was 3 months old when her parents rushed her to the hospital after she wouldn’t eat and "her limbs were very cold, which we now know means she had very poor circulation," Brian Cotter said.

"She was very blue," Ashley Cotter recalled. "She was almost see-through."

The infant was hooked up to an external pump device called a Berlin Heart, the tubes from which went up through her stomach and into her heart.

"We would visibly see the blood coming out of her body and getting pumped back in," Brian Cotter recalled. "It was a very crazy thing to see every day."

Help with the essentials

During this three-month wait for a new heart, family, friends and community members donated to an online funding effort to help the family. The Cotters took leaves from their jobs to care for Ruby and Everett.

"We knew we were going to be able to pay our bills," Brian Cotter said of the fundraising effort's effect on the family.

Ashley Cotter is a quality assurance and risk management specialist for American Regent Inc., a Shirley-based pharmaceutical manufacturing company.

"We knew that we would be able to come home to our house," added Brian Cotter, who works as an aviation communications technician at the 106th Rescue Wing of the New York Air National Guard in Westhampton Beach.

All four of Ruby’s grandparents visited Ruby while she was hooked up to machines, and brought food and other essentials to her mom and dad.

"It was very hard for us," recalled Jo Ann Buhler, one of Ruby’s grandmothers. "We didn't know anybody else who had a transplant."

Lifesaving gifts

Three days before Christmas Day 2019, surgeons at the hospital put a new heart inside Ruby during an eight-hour operation. Ashley Cotter said she was "thrilled," but "at the same time, heartbroken."

It meant another family "is losing their child and they’ve decided to save someone else in the way that they are not able to save their own child," she said.

Nearly a year after Ruby was discharged in January 2020, Everett arrived on Dec. 30, born seemingly healthy. It wasn't until Everett neared his first birthday that his parents noticed he had symptoms similar to Ruby's and took him to a hospital.

The entire family was in "complete shock," when physicians diagnosed Everett with dilated cardiomyopathy, Buhler said, but at least, unlike Ruby's ordeal, she knew what was in store for her grandson.

"With her, we were petrified, and with him, we saw how healthy she became, how she came out of it," Buhler said. "She was doing so well that we were definitely more hopeful and less scared."

For about half the 750,000 Americans estimated to have dilated cardiomyopathy, the condition is passed genetically from parents to children, according to MedlinePlus, an online health resource of the National Library of Medicine. Soon after Ruby’s transplant, Ashley and Brian Cotter learned neither of them carried the gene that could lead to the condition. Brian said the couple later learned that a gene in Ruby and Everett with instructions for making a protein found in cardiac muscle contained a mutation associated with dilated cardiomyopathy.

During his six-month wait for a new heart, Everett was only the eighth child in the nation to receive a surgically implanted pediatric Jarvik heart, an experimental descendant of a decades-old artificial heart. With an ear to his son’s chest, Brian Cotter said he could hear what he described as "a jet engine the size of a AA battery" circulating Everett’s blood internally without the need for the same sort of tubes that once pumped life through his daughter.

Challenges to come

Like his sister, Everett was sent home about two weeks after his operation. Since then, he has returned to the hospital for several related health issues, including an "episode of rejection," Lytrivi said.

Neither child has a notion of the challenges they face or what others may come.

Whatever awaits them, their parents said they are raising them to live life without limitations.

"I don’t want them to grow up thinking that they’re different or weaker or sick," Brian Cotter said.

While the couple reflected on the last five years of hospital stays, recoveries, community support and the sacrifices that gave both of their children second chances, Everett lined up his action figures. Ruby plopped face down on the living room couch and pretended she was a sloth. She made a deal with Everett: her green gummy bears for his red ones.

"Who knows where we would be as a family if our children did not have this gift?" Ashley Cotter asked.

This Christmas, just three days after she blows out the candles on her fifth "heartiversary" cake, Ruby will open an American Girl doll veterinary set, while Everett will unwrap a Hot Wheels racetrack. "Normalcy," in the form of baking cookies and hiding an Elf on the Shelf, is the gift Ashley Cotter said she and her husband want most this Christmas season.

"When you spend a Christmas in the hospital and you’re with a sick child," Brian Cotter said, "you really appreciate the Christmases after that."