Harper Foy, 9, ran around the soccer field at Edmonds Civic Field while her father, Kevin Foy, chased her with a small, orange football in his hand. He threw the ball at her and missed. Harper laughed, picked the ball off the ground and prepared to throw it back at her father. Her mother Angie watched them from a distance.

“Doctors had said she would be limited physically, but Harper had dreams of her own,” Angie said. 

Harper has harlequin ichthyosis, a rare, congenital skin condition that causes the skin to thicken and form hard, scaly patches in diamond or triangular patterns. The condition gets its name from how an infant’s face can be pulled tight in a way that looks like a clown’s smile. Harper was born at Seattle Children’s Hospital in 2015 and has been under the care of a medical team led by dermatologist Dr. Deepti Gupta. 

Angie and Kevin, who have lived in Edmonds since 2002, said that there is no road map for raising a child with harlequin ichthyosis because it is a rare condition. Worldwide, about 200 cases have been reported since 1750. However, the couple has developed a daily routine over seven years to take care of Harper.

Some features of harlequin ichthyosis include eyelids and lips that turn outward, ears and nose that are either missing or not fully developed, and stiff joints that are hard to move. Infants with harlequin ichthyosis have a weakened skin barrier that easily loses water, electrolyte imbalances, body temperature dysregulation and an increased risk of infections. These factors make harlequin ichthyosis life-threatening even with proper treatment.

“We went through a lot of trial and error, trying different things to make her comfortable,” he said. “Some things worked, some things didn’t. Between my wife and I, we have a lingo of how we take care of [Harper].”

Harper takes three one-hour showers or baths a day to keep her skin moisturized and alleviate symptoms, such as cracking and itching. Angie said that the cracks beneath Harper’s feet can be painful when she walks.

“She mainly gets [cracks] in her feet and hands,” Angie said. “And we would lather her with a jar of Aquaphor every two to three days. We plan our whole days around her schedule. It is a lot but it’s our norm.”

Kevin said that Harper sits in front of a heater after she gets her Aquaphor treatment. 

“The process can’t be skipped, so we just wake up earlier,” he said. “To get to an 8 a.m. soccer game, we’d wake up at 6. Most kids don’t do that on Saturdays but Harper’s a great trooper. She’s used to it, and she understands.”

“And she’s tired a lot,” Angie said, adding that her daughter’s body does not thermoregulate well because of her skin condition. “It’s a lot for a 9-year-old to do all the things that she does. But she can’t just roll out of bed, be late and just put her hair up and go. We can’t do that. She can’t go anywhere without taking a shower first thing in the morning.”

Angie said she had to put her real estate career on hold to take care of Harper full time. “She’s my world and I had to take a step back [in my career], and I’ve been in real estate for 24 years,” she said. “Kevin works from home, which is great, but it’s mainly me who is taking her back and forth from school.”

Angie also takes Harper home in the middle of the day to shower and moisturize before sending her back to school.

According to Angie, no one in Edmonds or other nearby cities has harlequin ichthyosis. She is part of the nonprofit Foundation for Ichthyosis & Related Skin Types (FIRST) that helps families and individuals living with all types of ichthyosis, including sharing caregiving resources and personal experiences.

“There is a family that lives in New York that Harper had got to meet a couple of years ago that has harlequin ichthyosis,” Angie said. “Her diagnosis is a little bit different than Harper’s. She’s in a wheelchair and has arthritis.”

Angie and Kevin also run Harper’s Playhouse, an online store that sells a variety of clothes where a portion of sales goes to Seattle Children’s Foundation.

What causes harlequin ichthyosis?

Harlequin ichthyosis is primarily caused by a mutation in the gene ABCA12, which is responsible for regulating specialized proteins in the skin to keep it sturdy and skin formation during fetal development. While inflammation is a common symptom of the condition, not much is known about how it contributes to harlequin ichthyosis.

While prenatal screening for harlequin ichthyosis is difficult, two researchers from Dailan Medical University in China reported in 2023 that having a 2D-ultrasound combined with a 3D-ultrasound in the third trimester of pregnancy “can sharply reduce the rate of missing diagnosis or misdiagnosis” of harlequin ichthyosis. 

They cited a case of a 40-year-old pregnant woman who had both scans, and doctors detected evidence of harlequin ichthyosis in the fetus, such as clenched fingers, clubbed feet, underdeveloped nose and ears and peeling skin. 

About 1 in 300,000 infants get harlequin ichthyosis, and there is no significant association between gender and race and the prevalence of the disorder, according to a 2020 study published in The Journal of Clinical Investigation.

Harlequin ichthyosis has the highest death rates within the first three months of life, mainly due to respiratory failure or severe infections such as sepsis, affecting 75% of cases. About one-third of patients experience repeated skin infections, and 44% struggle to maintain a healthy weight.

Angie said Harper has dreams of her own, defying what people had expected of her capabilities with her skin condition. “She even took up sports, surprising everyone with her passion and determination,” Angie said.

Harper was also chosen to be in a fashion show for the nonprofit Runway of Dreams in New York City last summer. She walked the runway sporting a teal and white polka dot dress, a rainbow unicorn bag and a pair of rainbow glitter sneakers.

However, Angie said that people who have never met Harper before think she has a sunburn because of her skin’s deep red color. “I just want people to not treat [Harper] differently because of her skin color,” she said. “She is just like you and I. We’re all different and unique.”

Harper said she wanted to be a physical education teacher someday and likes all her friends in school. “I want [kids] to experience different sports, and I like sports,” she said.

– Story and photos by Nick Ng