Amara Sherko was only 2 years old when she underwent her third open heart surgery in June 2019.
Born with only the right half of her heart fully developed and functioning, a condition known as hypoplastic left heart syndrome, Amara’s survival depended on a series of three unique surgeries to reconstruct her heart and reroute blood flow.
Although grateful for the care that extended their daughter’s life, Adrian and Jason Sherko worried that the procedures helping to keep Amara alive could prove traumatic for their toddler who was too young to understand.
“Toddlers with her condition frequently have posttraumatic stress disorder and fear of doctors and hospitals,” Adrian said. “It’s understandable given all they experience. We would never want Amara to fear the people who literally saved her life and are going to be a part of her life forever.”
That’s why a pediatric clinical trial, which offered a study treatment option that would significantly reduce blood draws for an entire year after surgery, held special meaning for the Sherko family.
An alternative option through research
During the Fontan procedure – the final planned operation in the three-surgery series – surgeons reroute oxygen-poor blood from the lower part of the body directly to the lungs so oxygen is replenished before entering the heart. Although this ensures oxygen-rich blood reaches the body, the modified flow increases the risk for potentially life-threatening blood clots.
To reduce risk post-surgery, Amara was to receive standard treatment for one year with warfarin, an anticoagulant drug that prevents blood clots from forming. Unfortunately, warfarin requires frequent blood draws to monitor levels and adjust the amount given.
Around the time of Amara’s Fontan procedure, Advocate Children’s Hospital in Oak Lawn, Illinois, served as a site for the UNIVERSE clinical trial, which was evaluating the anticoagulant drug XARELTO® for its effectiveness in helping to prevent blood clots in children following Fontan surgery. XARELTO® works differently compared to warfarin so frequent blood draws to monitor drug levels aren’t needed.
Pediatric cardiologist Andrew Van Bergen, MD, who led the Advocate Aurora Research Institute team, and Bonnie Hughes, BSN, who served as the lead senior research coordinator, presented the research and standard-of-care options to Amara’s parents.
“I had numerous discussions with Dr. Van Bergen and Bonnie about the risks on both sides,” Adrian said. “XARELTO® and warfarin are both blood thinners, and all blood thinners come with risks for bleeding, which can be scary when given to a 2-year-old. But with XARELTO®, Amara didn’t have to endure the physical and mental side effects of those frequent blood draws.”
Amara, one of 112 pediatric research participants who contributed to the UNIVERSE clinical trial, completed her participation in 2020.
Study leads to FDA approval
UNIVERSE trial results led to the December 2021 U.S. Food and Drug Administration (FDA) approval of XARELTO® for two pediatric indications, one being treatment to help prevent blood clots in children aged 2 years and older with congenital heart disease who have undergone the Fontan procedure. This was an important approval as, previously, it had only been approved for use in adults.
“FDA approval means children now have access to valuable treatment options they didn’t have before,” Dr. Van Bergen said.
Young heart warriors and their families face intense and often harrowing health challenges. For Amara’s parents, access to a less-invasive treatment option through research spared their toddler from additional pain and fear after surgery.
“I had this 2-year-old who endured far more than any child should ever have to do, and she’d just had her third extensive open heart surgery,” Adrian said. “Amara had post-op complications requiring readmission to the hospital, had to be spoon-fed thickened liquids every day and was on a multitude of medications post-surgery for weeks. It was all unimaginable. If we had the addition of those frequent blood draws directly postop, it would have been horrendous.”
Today, Amara is an active 5-year-old who loves riding her bike, going on boat rides and playing on the beach.
“Amara is amazing and full of life,” Adrian said. “She is also full of spunk, which I kind of laugh at because I think she needed that spunk to get through all she has faced. She’s incredible.”
How you can help kids like Amara
You can make a difference for children like Amara by making a gift to support critical research and clinical trials in Illinois and Wisconsin.