In August, 10-year-old Josie Paugh flew to Manhattan for surgery. It was not the first time she had boarded an airplane for Lenox Hill Hospital in New York City.
“She was born with a chromosomal mutation,” said Josie’s mother, Erin. Josie’s rare condition is called facial infused lipomatosis. “We didn’t have a diagnosis until right after she turned one. We knew it was a tumor, and it wasn’t anything life threatening—it wasn’t cancerous.
“Everybody from the EMT to the specialist we saw in Des Moines, they all had to look it up. They had never seen a case like this.”
The problem was not obvious at first. Josie’s parents thought she had an ear infection, or was simply cutting teeth.
“A normal person creates cells,” Erin said. “The cells have a life cycle and they die, slough off and go away. Hers, they wouldn’t die, they just kept accumulating.”
When Josie hit growth spurts, her condition became progressively more noticeable. The unhealthy growth began smothering nerve and muscle.
“I’m hopeful, at some point, they come up with gene therapy, but I think that might be a little ways down the road,” said Erin.
The Paughs tried one pediatric plastic surgeon in Iowa, but the results were less than satisfying. By 18 months old, Josie had already undergone her first surgery. Doctors performed liposuction on her forehead and cheek. They cut her hair, and she had to wear a mask for compression therapy. Scar tissue from this surgery would cause trouble down the road.
“We knew fairly quickly that didn’t work,” Erin said. “It just wasn’t the answer. It was a band-aid fix. The tumor was back within a year. We didn’t want to pursue anything more—you just get a gut feeling when you’re in the right spot, and we hadn’t found that yet.”
Erin Paugh is a 1996 graduate of Mormon Trail Community High School. Husband Dave, a 1995 grad, along with Josie’s twin, Brooks, and little sister Amelia, now live in Indianola.
Josie’s grandparents are Kim and Jim Brown and Carlos and Cheryl Paugh of Humeston, and the late Randy Beavers of Corydon. She has two living great-grandparents, Virginia Neely of Corydon and Carol Moore of Le Roy.
Little sister Holly Dent still lives in Humeston, with Big Brother star Jason Dent and Josie’s little cousins.
Erin works one-on-one with a special needs preschooler in Chariton. Before that, she worked for a non-profit, as well as serving as a substitute teacher.
Needless to say, Josie handles her condition better than any other member of her family.
Erin keeps a blog at www.josiebeangrace.blogspot.com. She believes her online prayers led to the discover of the doctor who could help her daughter. It also helps raise awareness and understanding for those unfamiliar with Josie’s condition, and it keeps her connected with children facing the same circumstances.
Erin is a member of a support network of families, each with a child diagnosed with Josie’s condition. One of the children is from Puerto Rico. This is Josie’s extended family.
The family has found almost 20 children across the world with Josie’s condition, connected by Facebook.
“So many don’t have the opportunity to have a doctor,” Erin said.
“Erin and I are so very lucky,” her father wrote in a blog post not long after Josie was diagnosed. “Josie is everything you could ask for in a soon-to-be 19-month-old. She loves to sing, dance, play with her brother and talk in her unknown gibberish language. Josie is surrounded by a loving extended family and is supported by so many friends.”
Before that, the Paugh family connected with one other family that happened to be living in Iowa and facing the same diagnosis.
“We kept in close contact with each other,” Erin said. “Then, in the summer of 2011, this other family we know called us from New York [City] and said they’d found a doctor for their little boy.”
That physician was Dr. Milton Waner, co-founder of the Vascular Birthmark Institute. His business partner is also his wife, Dr. Teresa O, at the New York Head & Neck Institute, which is affiliated with Lenox Hill Hospital.
Erin describes Waner’s smile as comparable to a traditional depiction of Santa Claus.
That was in July, and Josie had her first surgery in January of 2012. Within the span of one year, the family traveled to Manhattan five times, once for consultation, and four times for Josie’s surgeries.
Since then, Josie has flown out every other year for an operation. The solution has been complex.
“Some might consider his approach radical,” Erin said of Waner, who has almost become part of the Paugh family. Their nickname for him is “Doctor Super.”
“Instead of just reducing the outer layers of fat, they completely stripped all of the diseased tissue inside the left side of her face,” Erin said. “With that, they took the muscle, they took everything pretty much down to the bone to prevent that disease from continuing to come back.”
For three years, Josie saw no signs of regrowth. Therefore, the doctors began facial reanimation. It is an arduous process.
“The first step was in 2016,” Erin said. “That was to take a sensory nerve from the bottom part of her ankle—if you think of an extension cord, you have to put it into a working outlet—so they plugged her newly-harvested nerve into the right side of her face, and ran it across her right cheek and above her lip, and kind of fanned it out on her left side. It takes a year for that nerve to rejuvenate and reach to the other side.”
When Josie was able to tap on the right side of her face and experience feeling on the left, it was time for the next stage.
In May of 2017, Waner prepared Josie for an operation on Aug. 15, which would be performed by Dr. O, who specializes in the nervous system of the head and neck.
“They took a strand of her inner thigh muscle and placed that on the left side, and they connected it to the newly functioning nerve,” Erin said.
Following the surgery, after the swelling went down, Josie began feeling reactionary movement in the first part of October.
“They said it’s surprising how early they’re seeing that movement,” Erin said. “She’s making great progress. It’s exciting to watch.
“Once we saw that movement start, we needed to connect with an occupational therapist who does facial retraining.”
Josie performs her occupational therapy via Skype. The closest therapist that could handle a case like hers is located in Madison, Wisc.
“I was insistent we travel to see her for the first appointment,” Erin said. “We go back in person on Spring break.”
Though Josie dreaded these trips to the East Coast at first, she has matured and learned how to deal with the emotion.
“She has the benefit of being a kid,” Erin said. “This is everything she’s ever known, and this is just part of her situation. We come home, and life goes back to normal. I don’t think she dreads anything.
“We tackle one surgery at a time, and battle one issue at a time, and try not to dwell on what comes next. She does a better job at that than I do. You take on all their worries—that’s just part of being a parent.
“Physically, she bounces right back. People have questions, like does she get picked on at school or have any issues that way, and honestly we’ve been really blessed. I think the kids she’s gone to school with have just grown up with her, and we just don’t have those problems.
“Little kids will ask the question, ‘What happened to your face?’ And she does a good job explaining—I always tried to tell her there’s a difference between curiosity and people being mean, and she’d always be able to tell the difference.
“Honestly, we’re thankful for the opportunities she’s had. She looks at it in an optimistic way, rather than, ‘whoa is me.’”
Erin summed up the experience of this past year’s hope in one of her blog posts:
You have been assigned this mountain to show others it can be moved.
“Have you ever read something and felt like it was put there just for you? I read these words while we were sitting in the waiting room during Josie’s surgery this past August and they clung to my heart. They followed me through the streets of New York and have since followed me home; reminding me of a greater purpose, urging me to do more (even when I’m not certain what or how).
“My prayer is that Josie can be a beacon for others who need a seed of hope. While Josie’s new muscle grows stronger every day and we see her smile becoming a reality I can’t help but think back to the beginning when I felt hopeless and completely defeated and alone in her diagnosis. All it took was that one little glint of hope that our friend Sam gave us six years ago, that hope is what gave us the courage to move forward. Never lose hope.”