An eight-year-old girl is one of the few people in the world with a genetic condition so rare that fewer than seven Americans are born with it each year.
Harper Foy of Edmonds, Washington, was diagnosed at birth with harlequin ichthyosis (HI), one of the rarest genetic skin disorders that causes her skin to shed every four hours.
Harper is believed to be only the 20th person in the United States and 100th in the world to live with her condition, which causes skin grows 10 times faster than normal.
Doctors thought it would kill her, but she has defied the odds and now models professionally for children’s clothing brands such as Little Stocking Company and Costco.
Harper’s mother described her daughter as “the most confident, brave, outgoing, party-loving girl.”
Babies with harlequin ichthyosis are born prematurely with very hard, thick skin that covers most of their body. In Harper’s case, her skin formed large diamond-shaped plates that were separated by deep cracks.
However, she constantly needs to keep her skin hydrated and uses a 14 oz jar of Aquaphor, a Vaseline product that heals and moisturizes chapped skin. every other day just to keep her skin hydrated, which cost her family almost $60 a week.
When Harper was born, her doctors had never seen her condition before and warned her parents that their daughter was unlikely to survive.
In children with harlequin ichthyosis, thick flakes of skin develop on the matrix, stop circulation in feet and hands at birth.
When Harper was born, she was rushed to the intensive care unit, and although surgeons were able to remove the plates, she lost the tips of four of her fingers.
After a month in the hospital fighting an infection, Harper beat the odds and was allowed to return home. Most babies born with this condition do not survive beyond the first week of life.
For two years, he had trouble feeding and eating and needed to get nutrients through a feeding tube.
Now, she must also receive three one-hour showers or baths daily to stay comfortable.
Babies with harlequin ichthyosis are born prematurely with very hard, thick skin that covers most of their body.
In Harper’s case, her skin formed large diamond-shaped plates that were separated by deep cracks.
After the neonatal period, the hard skin plaques slough off and the skin develops widespread scaling and redness.
These skin abnormalities affected the shape of his nose, eyelids, mouth and ears, and limited the movement of his arms and legs.
Restricted chest movement due to skin tightness can sometimes lead to breathing difficulties and respiratory failure in babies with this condition.
Normally, the skin forms a protective barrier between the body and the surrounding environment.
However, skin abnormalities associated with harlequin ichthyosis disrupt this barrier, making it difficult for affected babies to control water loss and regulate their body temperature.
Foy, 47, a real estate broker, said: “We get a lot of stares every time we go out in public.” Not a day goes by without people staring at her. It can be very disturbing
Harper was connected to a feeding tube for two years and now must receive three one-hour showers or baths a day to stay comfortable.
Babies with HI often experience excessive fluid loss, dehydration, and develop life-threatening infections in the first weeks of life.
Harper is always cold and constantly fighting infections.
The Foy family was shocked when they received the little girl’s diagnosis. Not only did no one in her family suffer from this condition, but they had never heard of it either.
IH is caused by mutations in a certain gene responsible for producing a protein necessary for skin cells to develop normally. When the gene mutates, the skin barrier is altered.
It is inherited in an autosomal recessive pattern, which occurs when an individual inherits an abnormal gene from each parent, meaning that both Harper’s mom and dad had the gene and passed it on to her.
Although Harper’s mother, Angie Foy, says she is a happy child, she still faces some discrimination due to her condition.
Foy, 47, a real estate broker, said: “We get a lot of stares every time we go out in public.” Not a day goes by without people staring at her. It can be very disturbing.
“A mother told her children to stay away from Harper because she thought Harper was contagious and sick.”
Foy added that people often think her daughter got sunburned and forgot to put sunscreen on her.
Despite the struggle of living with this condition, Harper never let it stop her. She is a confident child and has worked as a model since she was four years old, her mother said.
‘We are Harper’s biggest supporters and we tell her there is nothing she can’t do. We encourage positive self-talk every day with her.
“I sent a photo of Harper to a local modeling agency three and a half years ago with a little bio about Harper, and they were intrigued and asked us to come meet her,” Ms. Foy said.
‘We arrived and they fell in love with her bubbly, infectious, happy self, and they hired her that day. The rest is history.
‘She was born to be in front of the camera. She really is a natural and loves having her pictures taken.
‘You can see it in the photos in his portfolio. And he’s gained a lot of confidence too,” her mother said.
Mrs Foy described her daughter as “the most confident, brave, outgoing, party girl”.
He added: “Harper’s most unique characteristic is her personality and her compassion for others. She has the biggest heart.
‘Appearance does not determine your value as a human being. We all look different and that’s okay.’