Megan Barron, 22, has epidermolysis bullosa, a genetic skin condition that makes her skin extremely fragile and is often nicknamed "the butterfly disease," though it's not a disease. (Courtesy Megan Barron)
Life isn't easy for children and adults whose skin is so fragile that the
tiniest friction can tear it away. They're called "butterfly children," and they
have a range of genetic skin conditions called epidermolysis bullosa, which means they can't properly make
the protein that anchors skin in place.
But they aren't all children and they're tougher than your average butterfly.
Rabble of Butterflies
Lizzy Hendrickson is 3 1/2 years old and has epidermolysis bullosa simplex, a
mild form of the condition that is still painful. Yet she dresses her own wounds
and sometimes lets her 6-year-old big sister help even though it hurts.
Megan Barron, 22, has dystrophic epidermolysis bullosa, a severe form of the
condition characterized by scar tissue. Her hands have scarred into fists except
for her thumbs, which have been surgically freed for typing and other tasks, and
she has a few blisters on the pale skin of her face, framed by strawberry blond
Yet she was determined to go to college in North Carolina instead of her home
state of Florida. She's set to graduate in May with a major in history and a
minor in political science. She's even been a White House intern, beating out
hundreds -- if not thousands -- of applicants from around the country.
Tripp Roth had the most fatal form of epidermolysis bullosa, yet he survived
longer than doctors ever expected and lived to be 2 years and 8 months old. Even
if his face was filled with scabs and blisters, his mother was always astounded
at how Tripp could always muster up a smile.
Rafi Kopelan is 5 years old and has a more severe form of the condition,
warranting two surgeries to prevent scar tissue from blocking her throat and a
bone marrow transplant to help her skin make the protein it needs to stay
anchored in place. She's shy, but she has no problem explaining to middle school
students why her skin is full of wounds that don't always heal. She even opens
up with a joke to put her audience at ease.
"It's literally the worst disease you've never heard of," said Rafi's father,
Brett Kopelan, who now runs the Distrophic Epidermolysis Bullosa Research Institution for
America. "The simplest of things, like dental cleaning or brushing her
teeth, is enough to rip the inside of her mouth open, yet this kid gets up and
goes to school every day with a smile on her face. I don't know how she does it.
I don't know how any of these kids do it."
Epidermolysis bullosa occurs in one of every 50,000 live births, according to
data from the National Epidermolysis Bullosa Registry. Although it's divided
into three main types, depending on which dominant and recessive genes the child
inherits from his or her parents, there are really closer to 27 different
varieties, and every patient is different.
An estimated 25,000 to 50,000 people have epidermolysis bullosa in the United
States. But with a total population in the country of almost 314 million people,
the disease is rare enough that raising awareness is a key issue the community.
Just this week, a family with children who had epidermolysis bullosa filed a
lawsuit against a Golden Corral restaurant in Michigan for violating the
Americans with Disabilities Act. According to the complaint, the restaurant owners approached the family and
asked what was "wrong" with the baby who was covered in scabs. Although the
child's mother explained the condition, the owner said the condition was
obviously a "contagious" disease and that the family should "go find somewhere
else to eat."
For 22-year-old Barron, the experience is all too familiar. She said she's
been asked if she was contagious, and said she's very tactful about how she
dresses to avoid making people uncomfortable about the open wounds on her skin.
"My heart sinks for these kids," Barron said in an email that she typed with
her thumbs -- a skill she taught herself in the third grade. "So many times I
fear revealing too much of my skin and bandages. ... I fear this very reaction
if someone sees too much."
In a phone interview a few minutes later, she explained that people get
"freaked out" by the open sores and it sometimes makes her feel like a leper.
Although Barron, like many other children with epidermolysis bullosa, was
initially worried about making friends in school, by the time she got to college
she learned to ignore the stares, relax and let her real friends in.
She said her friends accepted her feeding tube (for extra nutrition) and her
full-time nurse (for help with daily bandaging to avoid infection) without
making her feel weird about it.
"Hanging out in my room, going out on weekends, it's just Megan -- and that's
what I love about them, that they treat me that way," Barron said.
No Known Cure
Parenting children with epidermolysis bullosa is often a full-time job, and
sometimes that job is expected to come to an abrupt end because the condition
comes with many internal complications that often lead to early deaths.
When Courtney Roth gave birth in 2009, her son, Tripp, had mostly
unblemished skin aside from a blister on his head. She would eventually learn
that he had junctional epidermolysis bullosa, the most fatal type of the
condition because it leads to loss of fluid and infection from so many open
sores. Blistering of internal organs, malnutrition and other complications are
So Roth started blogging to vent her feelings and chronicle the short time
doctors told her she would have with her newborn, whose skin would blister no
matter how well she bandaged his fragile body. No one expected Tripp to live
until he was 2 years and 8 months old, or that the blog, "'EB'ing a Mommy" would win her recognition as an
ABCNews.com Reader's Choice Hero in 2011.
Roth, now 27, said mothering Tripp was trial and error the whole way because
every wound would be different. Some would heal and others wouldn't. She found a
community of other parents to help her through it, and she now offers her
contact information to other parents of children with epidermolysis bullosa.
"These moms and these parents, they're yearning for somebody that's going
through the same situation," she said. "It's probably one of the cruelest,
hardest things to have to go through, to watch your child suffer like that
knowing you can't do anything about it."
She said she rarely slept more than 20 minutes at a time, but she was
fortunate that she didn't have other children and that she did not have to work
during that time.
"I was always in his face," she said. "We just loved him. We loved him so
much. I don't know if that's what kept him going. I know for a fact that he kept
Tripp died in January 2012, but Roth isn't ready to read over her blog
entries just yet.
"It made you think: If he could survive and smile and laugh and play, then
there's nothing in the world to ever complain about or be upset about," she
said. "I just miss him a lot."
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