By Jessica Wallace, Kamloops this Week
Adrienne Brown sleeps with her infant daughter, Izzy, in the living room of their North Kamloops apartment.
It’s the only way she can hear Izzy crying through the night, a faint sound coming from a tender throat coated in blisters and sores.
“She’s susceptible to getting them anywhere on her body,” Adrienne said. “It’s just constant.
“There’s never a time when she doesn’t have blisters.”
Four-month-old Isabelle “Izzy” Porter, has a rare and incurable disease that causes her skin to break out in blisters from the slightest of touches to the usually unnoticeable frictions of everyday life — such as the subtle brush of a bed sheet or blanket.
The genetic disease, epidermolysis bullosa, is more commonly known as EB. Younger patients are often referred to as “butterfly children,” as their skin is as fragile as butterfly wings.
The disease has various severities and Adrienne counts her family lucky, since Izzy has epidermolysis bullosa simplex.
“She has the worst of the best kind, if that makes sense,” Adrienne said.
The family’s apartment is what you might expect of a young family — toys scattered throughout, impromptu diaper-changing stations here and there and blankets on the floor.
Adrienne, 24, and her 22-year-old boyfriend, Garry Porter, had no choice but to put their daughter’s crib in the family’s living room. They couldn’t have predicted the bedroom they had ready for their first child would instead be used for storing medical supplies.
The couple met and lived in Merritt for about eight years before each moved to Kamloops for work about two years ago.
Merritt is still a place where they have many friends and family, including Izzy’s grandparents.
For Izzy, EB largely affects her hands and feet but, when KTW visited their home, she also had small scabs on her face and head.
It all has Adrienne questioning Izzy’s future — how will she ever walk? — and remaining vigilant in preventing potentially deadly infections.
Izzy’s affliction, EB simplex, affects about one in 50,000 people and doctors at Royal Inland Hospital were initially stumped when she was born on April 22 without skin on her feet and ankles.
Within 24 hours, she started developing blisters in her mouth and on her face and, after spending about four days in RIH’s neonatal intensive-care unit, Adrienne and Izzy were transported via air ambulance to B.C. Children’s Hospital in Vancouver.
“Her condition just kept getting worse and worse,” Adrienne said. “Our biggest fear was that we were never going to take her home. We thought she was going to die.”
Adrienne stayed at Ronald McDonald House and Garry visited on weekends between working shifts at an absorbent product plant in Kamloops.
When Izzy was diagnosed with EB, Adrienne and Garry were connected to others with the disease and began learning how to care for their daughter.
On June 6, Izzy was discharged and, now, back in Kamloops, Adrienne remains on maternity leave from her job as a care-aide while dad goes to work.
There are harsh realities for Izzy as she lives with EB and for her parents as they care for her.
High-level prescription drugs are regularly administered to Izzy, who would otherwise feel the pain of raw flesh and scabbing beneath her little white protective booties.
Items such as bleach are required for the seemingly simplest of parental duties, like bathing Izzy with help from specialized paediatric nurses during regular, thrice-weekly visits.
“One of the hardest parts is that I have to cause her pain every day for her own good,” Adrienne said, referring to the need to pop blisters “all day long.”
Izzy also loses her hair and nails from blisters and has irritated her own wounds on several occasions by scratching them. She requires medical diapers and a high-calorie formula because she’s always healing.
Adrienne said her daughter hasn’t spent much time outside, apart from regular trips to B.C. Children’s Hospital, because the Kamloops heat prompts breakouts. Mom said she will try again in the fall, when the weather cools down.
While she knows Izzy is really just her little girl, Adrienne has thought about the day she will send her to school.
“Kids can be mean,” Adrienne said.
She hopes to raise awareness and one day take Izzy to a patient-care conference in Toronto or the United States, where her daughter can meet kids who are 1 in 50,000 like her — and others with rarer forms of the disease.
“When you have something that makes you different, it can feel really isolating,” Adrienne said.
“As she grows up, her friends won’t have it. No one has it, no one really understands it.”
Adrienne is not shy about sharing Izzy’s story with the world and she’s not worried about curious onlookers when she finally brings her daughter outside in cooler weather.
“I’m sure some people will have opinions about it,” she said. “She has to deal with it every day.”
Not your typical shopping trips
For Adrienne Brown, dropping $1,000 on blankets isn’t all that surprising.
To protect her daughter’s highly delicate skin, Adrienne buys sheets, clothing, blankets and more made from specialty products like sheep skin or bamboo — and it’s not cheap.
“I can’t just go to Walmart,” she said.
It’s why her cousin started an online campaign to help cover some of the costs.
From day-to-day items to saving for a patient-care conference, the Go Fund Me account has raised $5,575 raised by 87 donators as of Wednesday, Aug. 26.
“Some wound-care supplies we can’t get in Canada,” Adrienne said, noting the family is always looking for ways to improve. “It’s nice for us to be able to order them.”
The fundraising campaign will also help pay for future trips to Vancouver, where Izzy has ongoing appointments at B.C. Children’s Hospital.
To help Izzy, go to gofundme.com/isabellesky.
