SALT LAKE CITY — McKinley Lael Nilson picks up a musical bell toy and runs around the room with it.
The 2 1/2 year old also tries out a xylophone and a boomwhacker, as well as a drum — by jumping on it, of course. As she moseys around the room, a pair of certified musical therapists sing soothing tones, making games out of the music. McKinley, busy being herself, is visibly happy. So are her caregivers, as well as her parents, whom she is giggling and playing with.
The site of the toddler's music play date is a special therapy room, called Sophie's Place, at Primary Children's Hospital, a home away from home she knows all too well. McKinley, from Santaquin, has lived at the hospital for five months, beginning when she fell suddenly and seriously ill when her heart began to fail.
But if all goes according to plan, by Monday the young girl will be walking out of Primary Children's, doing so as the first patient in Utah to have a lifesaving but intrusive device called a Berlin Heart removed without needing a heart transplant.
Already, her life has been returning to normal, largely unhindered by either her heart's general recovery or the removal surgery performed Aug. 16.
"She wants to run, jump, and play, do all the things that make parents nervous," McKinley's father, Brian Nilson, said affectionately. "She's kind of making up for lost time."
The toddler's recovery, in which she has regained the basic functions of the left side of her heart without the continued assistance of the Berlin Heart device, is astounding, said her physician, Dr. Ashwin Lal.
"With how sick her heart appeared, we expected she would end up with a heart transplant," said Lal, who oversees pediatric heart failure and heart transplantation cases at Primary Children's Hospital.
For a patient, forgoing a transplant can mean taking significantly fewer medications, a lessened chance for serious long-term complications, and not needing multiple other heart transplants later in life when their donated organ is finally rejected by their body.
If his daughter were to get a transplant, Brian Nilson said, her new heart would "get her into her teens, maybe early 20s, on average," before she would need another one, making her long-term cardiovascular health "a game of trying to keep up."
Lal, also a professor of pediatric cardiology for the University of Utah, estimated fewer than 20 people nationwide have been able to transition off the Berlin Heart permanently without needing a new heart.
"Most of the major centers across the country ... have taken out one, or at most two," Lal said. "Nobody's been taking these out as a common occurrence."
In March, McKinley became severely ill when her heart began to fail. Doctors believe a Parvo B19 virus infection ultimately affected her heart, leading to a condition called lymphocytic myocarditis.
The serious illness, estimated to affect 200 people each year in the United States, significantly interferes with the ability of a person's heart to pump blood properly, and increases the risk of both heart attack and stroke.
When McKinley first checked into Primary Children's, said her mother Ruth Nilson, "she was in a really bad spot."
The boisterous, energetic toddler was suddenly spending a lot of time essentially on bed rest, her parents said, under the careful watch of doctors closely monitoring her heart's performance.
As the talk of needing a transplant grew, the gravity of McKinley's situation weighed more heavily than ever on her mom and dad.
"That whole prospect was very scary, especially the more we learned about it," Brian Nilson said. "There's a lot more to it than I initially knew of."
By the end of March, thanks to the "severely decreased function of her heart," doctors had no choice but to equip McKinley with a Berlin Heart, Lal said.
"Within that two weeks, we didn't see that improvement we were hoping to see," he said.
McKinley was then placed on a transplant list, and for the next few months doctors were prepared to go that route if the opportunity arose.
"Had we received a call from a good organ donor, we would have taken that and given her a new heart," Lal said.
The device is not meant as a permanent solution, but rather as a "bridge to transplant," because "every day on a Berlin Heart has its risks, with one of the major risks being a stroke," he said. Plus, according to Lal, the device is "very intrusive," particularly for an active toddler.
"(Large tubes are) coming out from the bottom of the chest," outside of the patient's body, he said. "If those (tubes) move too much it can really impair healing."
Brian Nilson was surprised his daughter ever adjusted to the cumbersome device as well as she did.
"I can't believe how well she's handled it," he said. "She kind of took it in stride. ... She never messed with her Berlin (Heart) at all."
For sick children who can successfully use a less intensive device, recovery without needing a heart transplant is more common, Lal said. That equation changes radically once the Berlin Heart is inserted. But for McKinley, doctors continued to see month-by-month improvement encouraging enough to consider removing it without requiring she get a heart transplant.
Even after finding out doctors thought McKinley might avoid a transplant altogether, her parents were cautious about getting ahead of themselves.
"It wasn't something unheard of, just not a very probable chance for us," Brian Nilson said. "We didn't want to get our hopes up too high."
As a parent dealing with a seriously ill child, he said, he had started to train his mind to "go to the worst places and think of the worst possibilities."
Lal noted that McKinley's journey from serious transplant candidate to someone who wouldn't need one "wasn't an exactly smooth road."
"We had some hiccups on the way. She did have a stroke that was noted," Lal said. "Then we started some additional therapies and we ended up seeing some additional improvement, which led us to say 'OK, we should really consider this.'"
Some of those new therapies included using a fairly "novel" heart medication which McKinley's medical team used after consulting data sets available for its use on adults, weighing options and deciding to make a carefully calculated risk, Lal said.
When it came to taking out the Berlin Heart, the surgery itself was not without its own risks, but more than a week after her surgery, McKinley has continued to get stronger and stronger, and has been as active as ever.
At McKinley's lowest point, when she had the least energy and was the least like herself, Lal and others at the hospital "kept assuring us, you're going to get your daughter back," Brian Nilson said — and they were right.
"We saw her attitude coming out ... in glimpses at first, and more as time went on," he said. "It's a huge difference."
Adds Ruth Nilson, through some emotion, "I feel like the doctors gave us the ultimate gift."
McKinley's older siblings, two brothers and a sister, seem to agree. On hearing she was well enough to come home, they were "ecstatic," their dad said. "They were just jumping and dancing and chanting."3 comments on this story
Lal said McKinley's success has him and his colleagues looking closely at how her treatment could be applied to others. Still, he said, most of what would factor into a patient's chances are the details of their own illness, and "we're still guarded" about using the same course of action again without substantial reasons to do so.
Despite how unlikely McKinley's recovery story seems to be, Ruth Nilson said her dream is to see that her daughter "doesn't stay the only one" in Utah to avoid a transplant after depending on a Berlin Heart.
"I want her to be the hope for other families," she said. "Never give up hope, because you never know what could happen."