Delta F508 Delete sounds like the name of a supersonic jet. It isn’t. It’s the type of cystic fibrosis (CF) that Claire Wineland came into the world with in 1997 and almost departed the world with—twice.
The first time was shortly after she was born, when doctors discovered she had CF, a genetic disease that causes excessive mucus secretions, especially in the lungs.
The second time was a couple of days after her 13th birthday, when she suffered catastrophic lung failure following a routine surgery on April 11, 2010 at Miller Children’s Hospital in Long Beach.
But today, as Claire—now 15—swoops to the front door of her home in Redondo Beach, a small cottage she shares with her mom, Melissa Nordquist, and half sister, Ellie, 8, her smile is so radiant, her huge brown eyes so full of life, you see only the gorgeous young woman who longs to be a stage actress someday.
As she leads the way into the kitchen, her three little Chihuahuas at her heels, it’s almost as if the oxygen tube she wears doesn’t exist, the one that trails behind her like an endless leash hooked up to a tank. She’s had to supplement her oxygen ever since the surgery two years ago that resulted in a near fatal infection and nearly took her life.
“I didn’t actually die,” Claire said as she sat down at the kitchen table and dug into a plate of fried potatoes loaded with salt, something CF sufferers secrete too rapidly. “I didn’t, like, have the white light experience. No one let it get that far. But before anyone (else) knew what was going on, it was the strangest feeling, because I knew that something was wrong. I told Mommy that I was trying to make my point (with the nurses), that something was very wrong.”
"I had 35 operations"
That Claire is as shrewd as she is beautiful is quickly ascertained, certainly by her mother, who trusts her daughter’s judgment implicitly. When nurses tried to convince Claire she was just feeling the effects of anesthesia after the surgery, Nordquist said, or that her lungs were still clogged from the operation, “she could sense it was more serious."
“I’ve had 35 operations!” Claire added as if boasting about a collection of Tony awards. “I find it funny when people (say) like, ‘Well, it might be this or that.’ I know what’s happening in my own body.”
And she was right. Within hours, her lungs collapsed. Rushed to the ICU, she was intubated while still awake. Claire’s only hope of survival, doctors later told her frantic parents, was to put her into a drug induced coma, hook her up to an oscillator ventilator and try to repair the damage to her lungs and body.
Only one problem. No child with Cystic Fibrosis had survived an oscillator ventilator, which pumps air into the lungs with ultra-ferocity. In her book about living with CF, Every Breath I Take, co-authored with Chynna Levin, Claire compares the machine to “a high powered ventilator on steroids.”
Melissa Nordquist and John Wineland, Claire’s father, were told their daughter had only a 1 percent chance of survival. They were given the option of watching the 13-year-old die or putting her on the oscillator vent. They chose the latter.
Her family, friends, and a raft of pals who had gone through school with Claire in Santa Monica formed around-the-clock prayer vigils at the hospital, at times as many as 50 people in the cafeteria. Her parents sleeping by her side in the Pediatric Intensive Care Unit, Claire finally woke up 17 days later.
During her time asleep, she encountered many an adventure, from traveling through ice bergs to being submerged under water on a Ferris wheel.
She compares the experiences to dreams in which bizarre happenings only make sense after you wake up. “I could hear the voices of people in the room, but I would turn (what they said) into something else,” she said.
When doctors wanted to weigh her, for example, they had to lift the comatose girl from the bed with straps. In her dream state, Claire pictured herself being strapped onto a Ferris wheel: “I would go (around on) the Ferris wheel, and part of it would go underwater … And so for like 15 seconds I couldn’t breathe.”
That’s because doctors periodically asked her to hold her breath.
'In my crazy dream world'
In another instance, doctors had to pack her in ice to bring down a 105 degree fever. “In my crazy dream world, I was going through the ice caps in Alaska on a tour with all of my friends and family,” Claire writes in her book. “Everyone was there because I was hearing their voices in the room.”
Alaska came to play a vivid part in Claire’s real life after she left the hospital three months later. (More about that, and the foundation, Claire’s Place, she founded with her parents, in a minute.)
First a bit about the Austin, Texas-born teenager, and how she manages to live her extraordinarily difficult life with such optimism and courage.
In her raspy, cheerful voice, Claire calls the abstract network of scars that decorate her body “my battle wounds.” The procedures all have exotic names (bronchoscopies, portacaths, fundoplications), which she recites with the ease of a Rhodes Scholar ticking off the fundamentals of physics.
For example, she explains that a fundoplication is “for acid reflux and is done by cinching the top of the stomach around the esophagus.”
“It leaves a big scar,” said Nordquist, who is as attuned as her daughter to every procedure, every medication and every piece of equipment.
Claire lifts her shirt to show off the long swipe across her stomach created by a scalpel during one of her frequent trips to Miller Children’s Hospital.
Nordquist, who quit her job as a production coordinator to care for daughters full time, is like Claire’s other half, dispensing pills, injecting saline or Heparin into a port, adjusting her feeding tube at night, whipping up salty meals, helping Claire into a electric vest that literally shakes her upper torso to clear mucus.
But Nordquist, whose nature is as sunny as her daughter’s, has been caretaking since the beginning. She was two weeks overdue in 1997, her pregnancy seemingly normal until doctors realized something was wrong and recommended a high-powered ultrasound.
“I was very nervous, it was my first baby,” said Nordquist, then 26. The ultrasound showed the baby “had a ruptured bowl and that she had a cyst in her abdominal cavity.” It turned out both parents were CF carriers, something that can be determined through blood tests prior to marriage nowadays, she said.
Up until two years ago, Nordquist and Claire’s father, John Wineland of Los Angeles, shared custody of their daughter. But after the coma, Claire’s needs—the masses of equipment, medications and treatments—made it implausible for her to live in two different cities. Seven months ago, Nordquist and the girls moved from Calabasas to Redondo Beach to be closer to Miller Children's Hospital.
“My mother could be a nurse, and so could I,” Claire said matter of factly as she watched her mother draw a hypodermic from a cupboard jam-packed with medications. “That’s the thing with CF. You have to really be a part of your care. I know every single medication that I’m on. I know when I get it. I know the IV meds I get and so does Mommy. You have to know that, because even though you should be able to trust your doctors, they are not gods, they are humans as well.”
In the next breath, she goes on to praise her doctors (as she does in her book), Drs. Eliezer Nussbaum and Inderpal Randhawa of the Miller Cystic Fibrosis Center.
You’d think that spending 35 percent of your life in a hospital would be the cruelest part of living with CF. On the contrary.
“If you’re a kid with a chronic illness, you think of the hospital as a home away from home, especially Long Beach,” said Claire, who always decorates her hospital rooms with her own quilts, pillows, lamps and sayings like “Hakuna-Matata” from Lion King and “Don’t dream it, be it.”
“The people (at Miller Children's Hospital), I’ve know since I was 4,” she said. “Some of the nurses already have my room all ready with everything that I like when I get there. I am an interesting food person; I like to have a stash of salt and catsup and butter, my secret stash of soy sauce and all that.”
Along with eating extra salt to compensate for what she loses through her skin, Claire, whose metabolism burns faster than a marathon runner during a race, has to consume 3500 calories a day.
“This is a G-tube,” she said, lifting her shirt again to show the port that leads into her stomach. “I hook it up and night and it gives me extra calories … so I don’t feel like I have to be eating all the time during the day.”
CF causes “a kind of ripple affect throughout the body,” Claire explained. “It can affect your sinuses, your lungs, your stomach, your pancreas. Now my liver is starting to act up because we’ve been pumping it full of stuff my entire life.”
As usual, there is not the slightest hint of self-pity in her voice. If anything, Claire works overtime to keep from being “that girl,” the teenager who begrudges the disease. “I’m very afraid of becoming a teenager that’s like all moody. I want to be able to keep trying. I don’t want to get to the point where giving up looks good.”
Although a positive attitude is always important, she said, “Keeping yourself alive takes so much energy, that if you don’t have something to refill your energy tank, if you don’t have something that makes you excited, it won’t be worth it.”
Claire has developed a multitude of skills and activities to keep her mind active and to keep from becoming bored in the hospital—everything from painting to writing to sign language to sewing to piano. She also has an adventurous streak and loves to travel. “I get that from my dad,” she said.
One of her most memorable trips was to Alaska in 2010. The trip not only revitalized her spirits following her coma ordeal, it formed the basis for her foundation, Claire’s Place, which is aimed at showing others how to live with CF.
Traveling with a major illness “is not simple,” Claire said. “And even though it was a major pain in the butt, it was very eye-opening.”
“We had three boxes of equipment,” said Nordquist, the organizer-in-chief in the family. Along with medical supplies and oxygen paraphernalia, “you have to carry backup batteries" in case the plane is delayed on the tarmac.
The paperwork was endless, she said, insurance obstacles; doctors had to cooperate, pharmacies, medical records had to be sent to a hospital in Alaska in case Claire had to be treated there.
Arriving in Seattle on the first leg of their trip, they found the airport “so massive,” Claire said. “We had to get someone to meet us with a wheel chair, because I couldn’t walk the whole thing.”
'Let's do it!'
Claire had chosen Sitka, Alaska because it matched the image of a beautiful cove she had seen in her dream state. “I thank the Lord for Google,” she said, laughing. “I described what I saw when I was asleep, and it came up Sitka.”
Nordquist, who had gotten the OK from doctors for Claire to travel, said, "Let’s do it!”
Despite the difficulties involved, the vacation was literally a dream come true. Sen. Lisa Murkowski invited them to a ball; they went whale watching, took a bi-plane ride over Sitka, flew with the Coast Guard in a helicopter, and got up close and personal with some grizzly bears.
But it was the grandeur of Alaska that captured the nature-loving Claire. “It was incredible," she said. "My uncle said before we went that it’s like nature showing off.”
Toting her little portable oxygen concentrator, Claire even hiked up a mountain to see some hot springs and a waterfall. The portable machine, which she drags around like a wheeled suitcase, frees her to go anywhere and everywhere.
She’s attended sailing camp, hiked a mountain in Malibu and organized a Flash Mob dance in Santa Monica (Flash Mob for Claire) that raised $24,000, and participated in a documentary about her life that garnered an Emmy for Fox News.
The Alaska trip, financed through donations from friends and family who wanted to celebrate Claire’s recovery two years ago, triggered the idea of a foundation.
“I thought how cool it would it be if there was a website, something that helps (those with CF) travel,” Claire said. “If we hadn’t traveled ourselves we wouldn’t have understood how much work it takes.”
Claire’s Place blossomed to include tips on how to live with CF in general, a blog, videos and fund-raising. Claire’s book, Every Breath I Take, is another helpful avenue for those with CF.
Co-written by Chynna Levin, whose father, Michael Levin, is a New York Times bestselling author and Wineland's friend. The book was Chynna’s "mitzvah," Nordquist said. In Jewish terms, co-writing and publishing the book was Chynna’s way of “making a difference for Claire in a positive way.”
Claire is a regular on the speaking circuit. She has already done one Ted Talk, TEDx La Jolla, and another TEDx Malibu event is scheduled for Dec. 2. Her videos are available on YouTube and on Facebook.
For information on how to contribute to her foundation, visit the Claire’s Place Foundation, Inc. website at clairesplacefoundation.org. Anyone who contributes $50, Nordquist said, will receive an autographed copy of Claire's book, which is also available on Amazon.