EDMONDS — Harper Foy arrived a month early. Her mom was out gardening when labor began. What Angie and Kevin Foy didn’t know — could never have suspected — was that their baby girl would come into this world with an incredibly rare condition and daunting challenges for life.
“The more we share our story, the less people will be scared,” Angie Foy said Wednesday as Harper, an energetic 4-year-old, jumped around and dribbled a basketball through the family’s kitchen.
Harper doesn’t look like most other people. Today, her skin appears somewhat like a burn victim’s might.
At birth her parents were stunned by her condition. She suffers from harlequin ichthyosis, a severe genetic disorder affecting the skin. It’s so rare, the Foys said, that doctors at EvergreenHealth, the Kirkland hospital where she was born Sept. 20, 2015, had never seen it.
Infants with harlequin ichthyosis are born with very hard, thick skin covering most of their bodies. It forms large plates separated by deep cracks. The condition alters the shape of the eyelids, nose, mouth and ears. And that hard skin severely restricts movement, breathing especially.
“It builds up over pregnancy. With time it becomes armor-like,” said Dr. Deepti Gupta, a dermatologist at Seattle Children’s Hospital and an assistant professor in the University of Washington School of Medicine’s pediatrics department.
After birth, she said, the plaques break up and slowly lift off.
“There is no cure for Harper’s condition,” Gupta said Thursday. Since those anguishing early days, she has treated the little girl and gotten to know her unbeatable spirit. She has seen the Foys’ devotion to Harper’s care.
“She’s spunky,” said Angie Foy, 43, who shares with her husband a nearly round-the-clock routine. They bathe Harper around four times each day, for about an hour, and cover her little body with Aquaphor. The ointment keeps her skin from turning scaly.
“She’s definitely got an awesome personality,” said Kevin Foy, 41. “She’s got a great spirit. If she didn’t, I don’t know if she’d be here.”
In the past, “it used to be very rare for affected infants to survive the newborn period,” according to the U.S. Library of Medicine, part of the National Institutes of Health. Risks include respiratory failure, dehydration and life-threatening infection.
“It’s actually very, very rare,” one in about 500,000 births, said Gupta, who saw such a case during her medical training in San Francisco. With improved care in today’s neonatal intensive care units, and the use of retinoid medication, “more are living longer and longer,” Gupta said. “We’re seeing the earliest of those patients in their 20s and 30s.”
Harlequin ichthyosis is caused by mutations in a gene, ABCA12, that normally provides instructions for making a protein needed for normal skin development. Both parents of an affected child have the mutated gene, but typically not the condition.
The Foys both carry the mutated gene. But with a son, 9, and daughter, 20, not affected by harlequin ichthyosis, they had no idea. Angie Foy had an ultrasound exam during pregnancy. The sonogram pictured nothing of concern, they said.
Gupta said skin isn’t fully formed until the third trimester of a pregnancy.
“Never in a million years,” Angie Foy said, could they have foreseen their youngest child’s condition.
Vital care at home
Every four hours or so, Harper’s parents give her a bath or shower. They have a special tub, with micro-bubble technology that’s helpful for people with skin conditions.
Soaks and ointment keep Harper’s skin from thickening, turning hard and cracking.
“Her body is always making new skin,” Angie Foy said. “We have to do it or it would build up again.”
“It’s a 24-hour job,” said Kevin Foy, who also coaches son Jaxson’s fourth-grade basketball, soccer and football teams. Jaxson attends Holy Rosary School in Edmonds, where the Foys said the community has shown great support.
To care for Harper, both parents work from home. Her reddish skin is now paperthin and prone to bleeding even from the little bumps of normal childhood activities. Angie Foy sells real estate. Her husband works in the telecom industry, and is involved in cell tower leases and construction.
On Wednesday, as Harper dashed into her room, she ran into something that resulted in a small cut. There were a few tears as the 4-year-old endured placement of a Band-Aid on the side of her head.
“If she falls, she bleeds,” her mom said as Harper retreated under a blanket next to her dad on the couch.
As a baby, during a couple months at Seattle Children’s, Harper had surgery to release pressure caused by the plaques. The condition includes the risk of losing extremities due to a lack of blood circulation. Early on, Harper lost the ends of some of her fingers.
“She’s a fighter,” Kevin Foy said.
Harper accompanies her dad when he coaches her brother’s teams, and even has her own whistle.
The prospect of school, though, presents hurdles. Right now, Harper can’t go down stairs because the skin on her legs cracks. During a school day, her dad said, she’d need to come home after four hours to shower.
“We need to get her stronger and bigger,” Angie Foy said.
There are other concerns. People with harlequin ichthyosis are sensitive to sun. They’ll always have red itchy skin. And because sweating is impaired, overheating is an issue. Harper also needs more fluids and calories than normal.
“They do have higher calorie requirements,” Gupta said. “The skin is doing a lot more work.”
The doctor sees the possibility of independent lives for Harper and others with her condition. There’s no effect on cognitive abilities, said Gupta, who described Harper as a bright child who understands her care routine.
Someday, “I think she should be able to live independently,” Gupta said. “They’ve been doing a lot of skin care from day one. As she gets older, I bet she’ll take some of it over herself. It’s a constant in her life.”
Harper in the spotlight
Harper said Wednesday that her favorite toys are a monster truck and a basketball. She’s also part of something unexpected. Late last year, she did a photo shoot with a modeling agency.
That chapter in her life was featured in a Dec. 19 blog, “Harper models to inspire the world: You’re Beautiful in Your Own Skin,” on the Seattle Children’s Hospital website.
Angie Foy said people often stare at Harper, or avoid getting close because they fear her condition is contagious.
“We want her to know she is beautiful, both inside and out,” Harper’s mom said in the blog, written by Kathryn Mueller.
The Foys have signed a contract for Harper with TCM Models & Talent, a Seattle-based agency, and hope others will get a chance to see the little girl they adore.
“The modeling industry is moving toward more inclusion,” said Brittni Thoreson, who books kids through the children’s division of the agency. “We represent Harper now,” Thoreson said. She is seeing more opportunities for those with special needs, people of color, larger-size models and whole families.
“We’re broadening our horizons. The whole industry is moving toward reflecting real humans,” Thoreson said. “And Harper has quite the little personality.”
Harper was involved in an initial shoot with photographer Elke Van de Velde. Some pictures show her wearing Seahawks gear. In others, she’s with doctors and nurses from Seattle Children’s.
“It’s a fun opportunity for kids to kind of come out of their shells,” Thoreson said.
“She’s an amazing girl. Part of her energy is just Harper,” Gupta said. “The message of beauty kind of lies in all of us. It’s more than skin deep.”
Julie Muhlstein: 425-339-3460; firstname.lastname@example.org.
More about Harper Foy, an Edmonds girl born with harlequin ichthyosis, at: pulse.seattlechildrens.org/harper-models-to-inspire-the-world-youre-beautiful-in-your-own-skin
Learn about the genetic skin disorder harlequin ichthyosis at: rarediseases.info.nih.gov/diseases/6568/harlequin-ichthyosis