Kim Scharf got the idea for her book when her granddaughter went to the emergency room. It was not a rare occurrence, but this stay would be a long one, lasting two months as complications arose from the debilitating health condition he had been born with. Waiting at home while the rest of the family — including Kim’s daughter and Evan’s mother, Lindzi — went to the hospital, Kim didn’t know what to do with herself. “So I sat down,” she tells SheKnows, “and honestly, it just kind of poured out.”
“It” would eventually become the story-slash-poem-slash-children’s book called Piper Joy and the Grand Old Tree. Released last November, it is Kim’s ode to Evan, who died two months before her fourth birthday. It’s also a display of Kim’s unfulfilled dreams for her granddaughter and a celebration of her unique spirit, which was “unlike anything I’ve ever felt,” Lindzi tells SheKnows.
Battling a rare, devastating disease
Diagnosed with mitochondrial disease at four months old, Evan Frances Buckner’s life expectancy was somewhere between 16 months and four years. Mitochondrial diseases are considered rare — affecting only 1 in 5,000 people; studies say — and shocking. Defined by Mayo Clinic As a group of genetic conditions that affect how the mitochondria work in the body, mitochondrial diseases can affect almost any part of the body, including cells in the brain, nerves, muscles, heart, and kidneys. And, unlike other diseases, early diagnoses mean worse outcomes.
For Evan, it started as a series of small, seemingly unrelated symptoms. “He had extreme acid reflux,” Lindzi recalls. “He started having these choking attacks that they called laryngomalacia, but that was expected to clear up on its own. He had mild hydronephrosis [kidney swelling from built-up urine]is also expected to correct itself.” Evan was also born deaf, “which was shocking in itself,” says Lindzi.
Any of the symptoms, taken individually, were not necessarily considered serious. Overall, though, “these little things were adding up to something,” says Lindzi.
Eventually, Evan came down with a cold that got so bad the family had to take her to the hospital, a pattern that would repeat itself over and over again. “Every time she got a cold,” Lindsey recalls, “we had to go back to the hospital because of how it affected her.” It was two years before Evan was finally diagnosed with mitochondrial disease.
“Being in her presence was literally magic”
There is no treatment or cure for the mitochondrial disorder, and Evan faced dozens of physical challenges every day, including not being able to sit up on her own. He received about nine medications each morning via syringes. Still, Evan was filled with an infectious joy, Lindzi says.
Part of it was her unique style. Evan rocked her signature sunglasses and a sweet smile and had an aura that made strangers stop her family in the street, saying things like, “You just made my day. Just seeing your child made my day,” Kim recalled. “Evan had a presence that people would flip through and have their hearts clenched when they saw her,” agrees Lindsay.
This quirky style was a reflection of her spirit. “We dressed her in a way that let people know, the moment they saw her, what we saw without all of that,” explains Lindzi. “Her presence was literally magic.”
Evan also got cochlear implants, electronic devices that improve hearing by transmitting sound signals directly to the auditory nerve, per Mayo Clinic. (Hearing aids, by contrast, just amplify the sound.) The implants “changed her life,” Lindzi recalls, and Evan quickly became a music lover (Ludacris’ “Llama Llama Red Pajama” was a favorite) and a bookworm who loved when family read to her.
“She was cheerful and happy, and she was cheerful and happy despitesays Lindzi. “I think it’s a lesson for us and for anyone that he had every reason not to smile and he still smiled in the midst of those difficult times. It was really something.”
Courtesy of Kim Scharf
Honoring Evan
Evan has inspired everything from Kim’s tattoo (llama, for the aforementioned song), ringsand articles (written by Lindzi, journalist). Even Evan’s brother Reid’s name is linked to Evan, as a tribute to Lindzi’s brother and Evan’s love of books. Reid was born just three months before Evan died and Kim says: “Evan made sure he stayed long enough to meet him.”
Now, Evan even has her own children’s book Piper Joy, which Lindsay and Kim agree she would love. “Evan liked anything Evan,” Lindsey laughs.
Piper Joy herself does not have a mitochondrial disorder. She’s also older than Evan and physically able to do things Evan couldn’t, like walk and stand on her own—though eagle-eyed readers will notice that Piper Joy’s bright red headband is, actually, a set of cochlear implants like Evan’s.
But when Kim explains it, all the connections make sense. Piper Joy, she explains, is a projection of who she thinks Evan could have been without mitochondrial disease, while being inspired by Evan’s curiosity and joy in the world around her.
“We spent a lot of time in her backyard,” Kim explains. Since Evan couldn’t sit unsupported, they lay together on their backs, looking up at the sky, the trees, and the things around them. “I used to watch her, and her eyes would shift and look everywhere,” Kim says. “So many of the things that are in the yard [in Piper Joy] they are in Lindzi’s yard. I gave that child the opportunity to speak up for Evan and say what made her feel like this was her safe place.” The book will also help other people facing similar health problems, Kim says, as a portion of its proceeds will go to nonprofits researching mitochondrial diseases.
And the response to the book, overall, has been supportive and moving. “I’m still blown away by the notes I get about Evan,” says Lindzi.
“Every parent wants to know that their child has had every opportunity to make their mark,” he continues. “Evan didn’t have a chance [do that] alone, yet her spirit left such a mark on us all. That’s why we feel it’s our privilege to be able to carry on her legacy.”
Evan’s story, Kim and Lindzi say, shows that we should never take anything for granted. “She had to try so hard for every part of her being. Just breathing was an act of resistance,” says Lindzi. “Just being was an effort. A smile was an effort’ — and yet Evan smiled all the time. “I have such a deep appreciation for that.”
Piper Joy it’s a way to get that message across, honor Evan, and share her spirit, all in one. “This lights up the sky with her name like a flame,” says Kim. “This is my contribution to it, and it’s been a pleasure.”
