The Butterfly Girl
23-year-old Cristina Perez has a rare, incurable disorder
By: BRENDA DURAN - North County Times | Tuesday, May 27, 2008 2:26 PM PDT ∞
Holding back tears, Cristina Perez gently clings to a towel as her mother, Cindi Hofer, applies a special ointment to the sores on her back, preparing it for bandages.
Jamie Scott Lytle
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Colors: A poem by Cristina Perez
Like a butterfly
I soar through the sky
My fragile wings
Pumping hard to fly
Over the mountains
And over the hills
Flashing my colors
My hope and my will
Feeling so small
So lonely and weak
Yet proud of my colors
My faith and defeats
The blues and the reds
Oranges and greens
Purples and yellows
No color unseen
Not even the gray
The dark and the dreary
They represent strength
To get through the fury
You can't judge this butterfly
By how it appears
Inside is the strength
To soar through the fears
Cristina Perez' personal website -
www.ebpatient.com
Cristina Perez' Online EB store -
www.ebhealthandhome.com
Rays of late-morning sunshine filter through the white miniblinds of Cristina Perez's window and illuminate the vivid patchwork of purple and red sores that cover her pale, 4-foot, 10-inch body.
Bending slowly forward on a padded table, Cristina, 23, flinches in pain as she unwinds the rolls of white gauze that keep her fragile skin from falling off.
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Cristina's mother, Cindi Hofer, sits beside her, meticulously cutting out squares of padded bandages. They are her daughter's "second skin," the one that keeps her alive for another day.
Donning rubber gloves, Cindi carefully applies a special ointment to her daughter's open sores and wipes it across her blistered skin before putting the new bandages in place.
"Just hold still for a second, honey," Cindi says, as she drains the blood-filled blisters on Cristina's back. "I'm almost done."
Cristina takes a deep breath, clenches a towel in her thin arms and shuts her eyes.
She knows this hour of agony soon will be over, this constant reminder of the deadly disease that has haunted her since birth -- Epidermolysis Bullosa.
One day at a time
It was in elementary school that Cristina first recognized she was different.
She recalls developing a crush on a boy. One day, when they were on the playground, Cristina accidentally fell.
While the boy and her classmates looked on in confusion, Cristina was rushed to the hospital.
From that day on, she said, she became self-conscious about her bandaged body and frail skin.
In the years that followed, she made many attempts to be "normal."
She began to dye her bandages to match her skin color so she wouldn't attract attention. She fixed up her chestnut brown hair and bought the latest fashions.
"I was hoping people would focus on my clothes, not my skin," Cristina said, recalling her teen years.
Eventually, she decided she needed to teach her classmates about her disease.
The rare, incurable genetic skin disorder known as EB affects 12,000 people in the United States and causes severe blistering inside and outside the body, making the epidermis fragile enough to fall off with a simple rub. The disease is not specific to race and afflicts both boys and girls.
Each year in junior high, Cristina gave a presentation on EB for her classmates, something she said alienated many of them.
"A lot of people just chose to ignore me," said Cristina, while watching television in the apartment that she shares with her mother in the southwest San Marcos community of San Elijo Hills. "Instead of staring, they would just act like I wasn't there."
Weighing less than 70 pounds, with her pale complexion, the red and purple sores, and seated in a wheelchair, it was difficult to fit in, she said.
As well, the lack of nutrients in Cristina's body kept her from going through puberty -- her body remains that of a young girl.
Those afflicted with recessive dystrophic EB, such as Cristina, have a difficult time absorbing nutrients and gaining weight. The nutrients that her body does absorb are used to heal the wounds.
Cristina said people thought her high-pitched, soft voice was that of a child -- no one knew it was the result of severely scarred vocal chords.
As she has aged, Cristina said many of her physical disabilities have gone beyond being a frustration.
They are unbearable.
At restaurants, waiters automatically give her a child's menu. At the mall, she goes straight to a young girl's apparel store, Limited Too. When telemarketers call, they refuse to believe she is a 23-year-old adult able to make her own decisions about their products.
And her deformities have made it all but impossible to make friends and build a social life.
Over the years, her hands have taken the shape of a fist -- her fingers fused together from years of tissue scarring. Only two protruding thumbs remain.
At 16, she had all but three of her teeth pulled to keep them from decaying because of the lack of nutrition.
These days, her smiles are limited.
"It's hard to talk to people," said the soft-spoken Cristina. "So I just don't try to most of the time."
Instead, she has focused on the bigger picture: teaching people about her disease to rid them of ignorance.
This is her life's mission.
"I'm just thankful I am still alive, I don't want to waste any moment," said Cristina. "Mentally, I've learned to just deal with it. I don't want anyone to feel sorry for me, I just want them to know about EB."
Cristina's crusade
The more that people know about EB, the more they can help push for a cure and learn not to judge others based on looks, said Cristina.
In the corner of her purple bedroom decorated with butterflies, stuffed animals, fantasy books and stars, Cristina spends all of the time and energy she has on "good days" to build an EB awareness community online.
At her desktop computer, she uses her thumbs to type 44 words per minute on the Web site she created when she was 13. She updates the latest news on EB, finds the most recent medical research on the disease and types personal essays on her personal space, www.ebpatient.com.
It is there that she tells the story about beating the odds stacked against her at birth, and reaches out to others in the section called "Through the Eyes of A Patient."
"It feels really lonely when others don't know what you are going through," Cristina said. "This just makes me feel like I am not alone."
She provides tips on bathing, bandages and nutrition for others with EB. She also lists resources, including research and support groups for more than 200 of her international friends living with EB.
And she lets everyone know what it's like to live in the quiet world that she shares with her mother; Simba, her white Shih Tzu; and Jasmin, her big, fluffy gray cat.
When it comes to expressing her feelings, Cristina prefers to reveal her thoughts on paper or online.
For years, she has found solace in poetry and short stories. It is there that she acknowledges death and pain along with hope, fear and anger.
Cristina said that writing lets her become the hip-hop dancer, the veterinarian, the blushing bride or the mother of three that she dreams of being.
The rest of the time, she said, she makes the best of what she has.
Next month, she will open an online store to accompany her Web site, where EB patients will be able to buy wound care products and other things she's found that "make life easier."
The site was Cindi's idea. It will help Cristina become an entrepreneur at home, as she was never able to go to college to earn a degree. And, it will make her a businesswoman and an inspiration to others with EB, Cindi said.
"I have hope that there will be a cure, but I don't think much about it," Cristina said. "I am just going to stay focused on living with EB as best as I possibly can."
The wounds that never heal
Cristina knows her disease can turn deadly at any moment. The last 23 years, she said, have been about staying alive.
Cristina has lost five of her closest friends to EB this year alone.
She refuses to become number six.
Most with the disease do not live to see their 30s because of the malnutrition, the development of skin cancer and severe infections, said Dr. Lawrence Eichenfield, chief of pediatric and adolescent dermatology at Children's Hospital, San Diego.
"The disease takes a toll over time," said Eichenfield, who treats the three people in San Diego County, including Cristina, who suffer from the disease. "It will continually break down the body's defenses in order to heal all the open wounds."
Cristina said that making sure the wounds are clean and free from infection is only half the battle.
Because of the vast open surface areas that are covered in sores, Cristina secretes large amounts of body fluid that cause her hemoglobin, the oxygen-carrying protein in red blood cells, to dip.
Every year, she has to get two blood transfusions to stabilize her hemoglobin levels.
There also is the constant worry that the lining of her esophagus will become so thin that she will be unable to eat.
At the age of 8, she threw up the lining and developed a staph infection that sent her to the hospital for a month.
Since then, she has had two surgeries to make sure she will be able to swallow pureed foods. Her favorites are pastas and blended fruit and vegetable smoothies.
Every day, she must be careful with the solid foods she does eat. Steaks, chips and large chunks of food are out of the question.
After years of trying all kinds of antibiotic creams, her back, the most affected part of her body, has become the latest guinea pig for healing potions, the one place she spends the most time trying to heal, she said.
She uses such natural remedies as "Egyptian Magic," a cream of olive oil and bee pollen, and Manuka honey, a speciality from Switzerland that resembles caramel.
The two are slathered on Cristina's back when she changes her bandages every other day. It is the first time in her life she said she has seen positive results.
To prove it, she has featured a "wound photo diary" on her Web site.
After every bandage change, Cindi photographs Cristina's back to document how the mass of flesh that remains raw has dramatically improved.
She said she hopes it will inspire other EB patients to try it.
"She has not healed in over a decade," Cindi said. "She has endured unbelievable pain for so long. Her strength amazes me."
Although mother and daughter have turned to natural remedies these days, they must stay on top of the annual medical appointments.
On an early May morning, Cindi wraps her arm around a weak Cristina as they head into the lobby of Children's Hospital, San Diego, where lab assistant John Hunyady will draw her blood.
The sight of a needle has caused her many sleepless nights, Cindi said, but today it must be done.
Hunyady will check to see if Cristina's hemoglobin levels have dipped too low and relay the results to her doctor, who will tell her whether she will make it through the year. It's possible her kidneys and liver could be damaged from the lack of nutrients.
"Don't look, you're doing great," Cindi tells an anxious Cristina as her blood is drawn. "It's the first time you didn't have a panic attack -- this is a good sign!"
Three weeks later, Hunyady calls Cindi and tells her Cristina must receive her second blood transfusion of the year. Her hemoglobin level, he said, has dipped too low. The call does not scare Cristina.
Dr. Eichenfield credits Cristina's mental outlook on life, and strength, with sustaining her.
"Cristina is a very special person," Eichenfield said. "She is an inspiration to other EB sufferers around the world. I believe her positive attitude and willingness to live a full life is what keeps her going. She never lets anything get in her way."
Camp Wonder.
Weeks after her second blood transfusion, Cristina is eager to take advantage of her borrowed time by traveling to the one place she is the most comfortable in her own skin. Camp Wonder.
Among vineyards and the dry mountains of Contra Costa County in Northern California, Cristina will be reunited with her EB friends -- known as butterfly children because of their fragile skin -- at the annual one-week camp sponsored by the Children's Skin Disease Foundation.
"It's all I think about all year," Cristina said.
She bought new shirts with colorful butterflies and a new pair of jeans.
Those who attend are living with a range of skin diseases from mild psoriasis to EB. All of them know what it feels like to be an outsider.
At camp, no one looks twice at the bandaged children riding in their wheelchairs, or asks questions of those whose skin is peeling off. There are no stares.
"They come here and exhale," said Diane Lewis, the camp's co-director of art. "This is an environment of love and acceptance. They don't have to worry about society's magnifying glass."
From afar, the camp seems a regular summer camp filled with outdoor activities, arts and crafts, games and food.
But a closer look reveals the camp's subtle features, such as "Club Med," also known as the "med shed."
Inside the shed are dermatology students who for the first time are seeing the campers get their bandages changed and soaked in bleach baths. It is their first chance to learn to treat severe skin disorders like EB. For many of them, it is the first time they have seen the ravaging scars that EB leaves.
"These are amazing kids," said Stephanie Kappel, a UC Irvine dermatology student. "Just so amazing and inspiring."
Both the dermatology students and camp organizers make sure every camper knows there are nurses at the shed who are on call 24 hours to treat open wounds. They are the ones who make sure the fragile campers have fun.
This year, Cristina asked her mom to share her experience with other young mothers at the camp and give them advice on how to raise a child with EB.
"This is the only place where people understand what you are talking about," Cristina reminds Cindi on the second day of camp.
Throughout the day, Cindi finds herself heading to the "med shed" between cigarette breaks to brag about Cristina's progress and to tell the nurses about the natural remedies she has found.
She tells them how well Cristina's back has been doing since she started using the honey and "Egyptian Magic." She gives them advice on how to change bandages and proudly tells them about her daughter's independence.
"She pays her own rent, I'm the one who is renting a room from her," Cindi says with a smile.
Meanwhile, Cristina rolls around the camp in her wheelchair with friends Jamie, 28; Emmie Lou, 20; Jessica, 26; and Nouvelette, 23, who have decided to call themselves the "EB Girlz."
Like Cristina, all are ecstatic to see each other and be able to talk.
"This is good. I spend all my time talking to social services, insurers and doctors back home," said Cristina's friend, Jessica Simpson, from Virginia.
This year, the girls will be in charge of putting together a camp newsletter.
But first they must catch up on their lives while spending the day in their cabin.
While most young adults would spend camp time talking about makeup, clothes and college, the "EB Girlz" focus on surgeries, cancer scares and bandage changes.
They talk about the medicine that has worked for them, the amputation scares, the loss of one of their close friends last year -- but in between, they also find time to giggle among themselves like a pack of teenage girls at a slumber party.
The last time they all saw each other was at the December wedding of Cristina's best friend, Jamie.
Jamie, 28, who has been fighting cancer that developed on her arms last year, said that like Cristina, she considers herself someone who has been able to beat the odds, and was also able to find the "perfect man."
She jokes about getting married last year to a man she initially thought was trying to do his good deed by "dating the handicapped girl."
"I never trusted anyone that looked at me and said, 'Oh baby!' "Jamie jokingly told the other girls.
For Cristina, the simple girl talk is the highlight of the camp, something she has never taken for granted.
"Even if they didn't have activities, I still want to come just to be able to talk to my friends," said Cristina.
As the day turns to late afternoon, the "EB Girlz" head to the arts and crafts room to get ready for "Carnival Night."
The dining hall has been decorated and the night will be spent playing booth games and watching entertainment.
Cristina and her friends decide they will make up a game to poke fun at the social realities that many EB patients face by challenging the campers to "Guess Our Age."
"I get a lot of 8s and 10s," Cristina tells her friends, while putting butterfly stickers on a poster.
Everyone will be a star for the night, the camp counselors announce.
The "EB Girlz" get to choose from a selection of colorful boas and headbands, and Cristina picks a yellow-beaded headband adorned with a bright yellow feather, a green boa and a pair of sparkly glitter glasses.
In that moment, her face lights up and she cracks a smile.
She quietly lets out a chuckle when she glances over at her friends, who are pointing at one another and giggling.
It's the moment she's been waiting for all year:
A time to laugh with friends and forget about EB, bandages and pain.
"This is what keeps me going," she said.
Contact staff writer Brenda Duran at (760) 761-4408 or bduran@nctimes.com
