'Butterfly child' finds strength to live with skin disorder
Her blue eyes twinkle as if they are powered by the smile spreading across the face of Caroline Provost as the 8-year-old Palatine girl ponders the list of things she'd love to do — ride a two-wheeler, go swimming, own a dog, wear her blonde hair in pigtails, play soccer, visit the American Girl store with her doll also named Caroline, go sledding, have sleepovers, ride a roller coaster and do all those fun things second-graders do.
Her smile turns into a grimace, and her eyes squeeze tight as the pain brings her back to her reality as a child with epidermolysis bullosa.
"Ouch!" she cries as her bandages are gently pulled from one of the dozens and dozens of blisters on her right leg. The new blisters are lanced with a needle. Changing the protective wrap covering all but her face and hands is a two-hour ordeal that Caroline endures every night, along with a shorter touch-up in the morning.
"They call them butterfly kids because their skin is as delicate as a butterfly wing," says her mother, Adrienne Provost.
Caroline is a social butterfly at Kimball Hill School in Rolling Meadows, where she gabs with a gaggle of BFFs (Best Friends Forever), eats lunch with her buddies and gets along with her classmates.
"She brings a smile on her face when she walks in the door every day," says teacher Kristine Plazyk.
"The kids have learned to gently high-five her," says Michelle Whiteside, a Palatine Township Elementary District 15 aide dedicated to protecting Caroline from the mayhem of grade-school lunchrooms, hallways and playgrounds.
"By the grace of God, I just try to make her life as normal as possible," says Whiteside, 53, who is in her third year as Caroline's aide and knows how to stay in the background as much as possible. "She's one of the greatest blessings of my life. She's such a joyful child. And she's a force to be reckoned with."
A very good reader who devours books, Caroline sits on a special pad and uses a soft grip on her pencil as she neatly writes, "My favorite part of Halloween was being in the parade." Caroline also says she likes the Three Musketeers bar she packs in her lunch, the witch costume she wore because the rabbit costume would have ripped the skin off her arms, the visit from her mom to watch the parade, and the night spent trick-or-treating with her pal Libby Honerbaum.
"Remember how we laughed so long?" Libby says at the next day's recess.
"I'm her BFF, too," notes Cece Gray, 7, another longtime pal who spends part of the recess on the swings next to Caroline, whose hands are protected by thick, soft mittens.
Dad Pete Provost, the youngest of the 13 kids in a family who grew up to produce 33 healthy grandkids, says he never imagined Caroline's birth would be anything out of the ordinary. The Provosts' first child, Elizabeth, was born on Easter Sunday and weighed in at 9½ pounds, but the birth by C-section went smoothly and she's now a healthy 12-year-old seventh-grader. The birth of Patrick, now a 10-year-old fifth-grader, followed the same routine. When Caroline was born at 10:29 a.m. on Aug. 22, 2005, at Northwest Community Hospital in Arlington Heights, the proud dad didn't even get a chance to snip the umbilical cord.
"They whisked the baby off. They knew something was wrong," he says.
Caroline's tiny body was blistered and so much tissue and skin was missing that doctors could see the white bones exposed in her left leg.
"The first time I saw her she was strapped down in the ambulance transport," says Adrienne Provost, who wouldn't get to hold her baby for another two weeks.
"It was just surreal," remembers Pete Provost, who drove with one of his sisters to what is now Lurie Children's Hospital in Chicago, which was better equipped to handle a baby with epidermolysis bullosa. Because EB also affects mucus membranes and can cause blistering in the mouth, throat, esophagus, upper airway, stomach, intestines, urinary tract and other internal organs, many EB babies die from complications with breathing, nutrition and hydration, as well as infections.
"They need tender, loving care," says Dr. Amy S. Paller, who treats Caroline, is chair of the dermatology department at Northwestern University Feinberg School of Medicine, and is a leader in the EB research field. "We have to be incredibly careful to avoid anything that is traumatic to the skin or mucus membrane. You can't take blood pressure because the trauma of squeezing the skin can lead to more blistering."
When Caroline was a month old, doctors inserted a feeding tube, which remained for years.
After 133 days in the hospital, the baby girl was healthy enough to come home with a medical supply chest and 14 prescriptions. On a January day that was literally blistering cold for Caroline, her parents covered her head with a soft baby cap. "By the time she got home, she had a ring of blisters around her head," says Pete Provost, who works as a network administrator for a landscaping firm based in Lake Bluff.
Affecting about one in 50,000 people, the epidermolysis bullosa disorder results in the lack of a protein that binds the outer layer of skin called epidermis to the dermis layer beneath. Just as grass without roots can be torn away from the ground, skin without that binding protein tears easily.
There are four basic types of EB and nearly two dozen subtypes. Caroline has a form known as junctional non-Herlitz, which might affect one in a million people, according to the National Institutes of Health. Her EB is less severe than some forms and generally doesn't shorten a patient's life span, but it does result in alopecia (hair loss) and malformed or missing fingernails, toenails and tooth enamel. Caroline has stainless-steel caps on her molars so they don't break when she eats, and she still must be careful when she chews.
The constant care her parents and home nurses provided Caroline as an infant has gotten easier in some ways, such as the discovery that organic coconut oil made the bandaging process less painful and enabled them to complete that four-hour ritual in half the time. But age also has added stress. As a 4-year-old, Caroline fell and bumped her nose on the ground, shearing all the skin from the center of her face. Caroline longs to wear her hair in pigtails, but that would expose the large bald spot on the back of her head.
She takes several medications, but they can't stop the constant itching of her skin. Scratching causes the skin to blister and bleed, but Caroline can't resist the urge. Home nurse Pat Anderson, who is considered a member of the family, gently applies cream and tries to distract Caroline, who often goes by the nickname Cara. Playing Minecraft on a cellphone while her bandages are changed helps get her mind off the itching, but preventing her from scratching is a constant battle.
Even in her sleep, the girl sometimes scratches away patches of skin on her face. The pain of the blisters has been likened to a second-degree burn. Caroline can't go outside if it's too hot, too cold or too windy. Having a bowel movement is a painful ordeal. "If she rubs her eye because she's tired, she could tear her eyelid," her mom says.
Forced to be constantly obsessed with Caroline's safety, her parents recently agreed to let her ride her three-wheeler without a helmet. "She doesn't wear the helmet anymore because it hurts her head more than it helps it," her mom says.
The emotional pain is another story. At the start of the school year, Caroline went to every classroom, explained her condition and told kids she had to be treated a little differently.
"She's such a trouper. She is a strong little girl," says Tracey Wrobel, the school principal.
Insurance pays for home nursing care, medicine and her bandages, which cost about $100,000 last year, says her mom, who, as a consultant with a benefits administration firm has the expertise needed to stay on top of the ever-changing insurance coverage issues for Caroline.
The creams and bandages are better than they were a decade ago, but treatment still consists of merely treating the symptoms. Doctors haven't found a cure. Researchers, including Dr. Jakub Tolar at the University of Minnesota's Stem Cell Institute, are working with stem cells and the effort to fix or change the genes EB patients need to made the skin adhere normally. "But we don't know how to do that yet," says Paller, who adds that dermatology is much more than acne and eczema.
In the meantime, the doctor says she remains impressed by how much time, effort and love dedicated parents give to their children with EB.
"Cara is lucky to have these two wonderful people," Anderson says of Caroline's parents. "They are amazing."
The parents took Caroline bowling on her birthday, where she rolled the ball off a platform to avoid hurting herself. But one of the saddest parts of the disorder is that it makes it difficult to find an event the entire family can enjoy together.
Families dealing with EB learn about research and fundraising efforts through the debra.org website of the Dystrophic Epidermolysis Bullosa Research Association of America, which calls EB "the worst disease you've never heard of," and at the childrenscancer.org/PUCK/ website of Pioneering Unique Cures for Kids. The families also share information on their Facebook EB Lounge.
The Provosts say it's heartbreaking to hear Caroline whimper from the pain of the bandage change, "but it's part of the process," Pete Provost says. They focus on making her as comfortable and active as they can today, and say they will deal with tomorrow tomorrow. Even in the sadness created by this cruel disorder, there is love and laughter and an appreciation for all the support they receive and the joy Caroline adds to their lives.
"One of the best things about being parents of a child with special needs is the kindness and goodness we've experienced," Adrienne Provost says. "It makes you think what are the things you can do to help others."
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