Why a terminally ill young woman has changed her mind about living gas bloating diarrhea


She’d traveled from Los Angeles with her mother, her best friend and her pit bull, Daisy, who flopped down on the floor atop one of Claire’s ever-present oxygen tubes. A full day of appointments at a nearby medical center awaited her, when she would begin the evaluation process to see whether she might be a candidate for a double-lung transplant.

She had long managed to push through physical discomfort to lead a life that mattered. After emerging from a 16-day medically induced coma at age 13, she envisioned the Claire’s Place Foundation, which today provides financial support to struggling families affected by cystic fibrosis. She appeared in brutally honest viral videos in which she talked about topics like death and did it with a smile. Since she was 14, she had been taking to stages and wowing audiences with beyond-her-years wisdom. Along the way, she nurtured a love of travel.

Doctors there sat her down and told Claire she had to stop flying. Period. They told her that her lungs could collapse and that she ran the risk of dropping dead on a plane, she said. They warned that it would be painful and laid out what it would feel like if an air pocket in her lungs burst.

Cystic fibrosis affects more than 30,000 people in the United States (and more than 70,000 worldwide), according to the Cystic Fibrosis Foundation. The disease causes an overabundance of mucus, which traps infections and blocks airways in the lungs, complicates digestion, affects the pancreas and other organs and, eventually, leads to respiratory failure.

Claire became uber-diligent with her care. She was on top of her dozens of medications, including her shots for cystic fibrosis-related diabetes, endured as best she could the feeding tube at night, even though it made her throw up, and spent extra time with her breathing treatments. For four hours daily, she said, she wore a vest to shake her lungs and loosen mucus. She also tried supplements like turmeric and found comfort in the nettle infusions she drank throughout the day.

But even with all of this, her lung function continued to decline. In one year, she said her working lung capacity fell 10 points — from 35% to 25%. Short walks and visits to the beach left this lover of the outdoors exhausted. Simple grocery shopping became too tough to manage. She had no energy for local speaking engagements and lost her income flow. She felt homebound, trapped and unable to do what mattered to her most.

CFers, as she refers to people with cystic fibrosis, often talk about "the Wheelchair Decision" with dread, Claire said. But when breathing and getting around became too difficult and she got hers in February, it initially felt "like freedom."

With her best friend, Larissa, taking on the role of "designated wheeler," the duo could tool around outside. The day she got the wheelchair, they went on a seven-hour adventure around Los Angeles’ Venice Beach, where Claire lives, visiting the canals, going to the park, soaking in the ocean breeze.

And while most of the time, she has no qualms about needing a wheelchair, there are accessibility issues, the sidewalks are a mess, and she can’t be pushed through sand. Inevitably, she’s had those moments when she thinks, "It sucks that I need this. It’s painful that it’s gotten this far."

Then Claire, who’s undergone more than 30 surgeries and been in the hospital a quarter of her life, received another jolt during a March hospital stay. Medical staff discovered that her portacath wasn’t working. The small dome under the skin of her chest provides a central line into a vein, allowing easy administration of IV treatments — such as antibiotics, which she must take regularly to beat back constant infections. A portacath replacement means surgery, and Claire’s pulmonary function was — and remains — at a level too dangerous for her to go under anesthesia.

She’d eaten a McDonald’s McGriddle sandwich the day before — a secret she employs to put on an extra pound or two before weigh-ins — just in case they asked her and her 95-pound frame to step on a scale. She was armed with questions and a notepad, ready to studiously record all she’d learn. Since she’d been here less than two years earlier and decided against pursuing the transplant path then, she was eager to tell them why this time was different.

They talked about the battery of tests that would determine whether she would qualify to get on the waiting list, some of which Claire knew well and described as "gnarly." They discussed the lung allocation score, the number Claire will get if she’s approved that measures how sick she is and determines where she sits on the priority list.

They discussed what life on a waitlist looks like: the necessity that she stay within a four-hour drive of the center, the importance of not ignoring phone calls, the exercise she’d need to grow stronger and the multitude of blood draws and exams she’d have on her schedule.

There were 1,436 candidates for lung transplants as of April 20, according to the United Network for Organ Sharing, which manages the nation’s organ waiting lists. Of those, 122 had a primary diagnosis of cystic fibrosis. Last year, of the total 2,449 lung transplants performed, CFers accounted for nearly 11% of the recipients.

Of those who received lung transplants (not just CFers), dating to 2000, an average of 84% survived after one year, nearly 54% survived five years, and slightly more than 30% survived 10 years or more, according to the organ sharing network’s data.

Claire first visited a transplantation center at UCLA when she was 14 but said that was too early for the idea to make sense for her. At 17, she checked out the program at Stanford University but was turned down, her mom explained, because they could tell that Claire wasn’t interested. She checked out the option again, at the very place she had returned to now, when she was 19.

Fijolek, who serves on the transplant team, remembered Claire from the last time she visited UC San Diego Health. Fijolek’s job is to think about "who are these [new] lungs going to live with," she said, get a sense of the care system recipients have in place and provide support to make the process successful for everyone involved.

Is Claire compliant in taking her medications? Is she prepared to relocate near the transplantation center, where she’ll need to be for at least three months after the transplant if she gets one? Who’d move with her and drive her to appointments?

Claire mentioned how her decline, starting last fall, threw her into a depression. They talked about how she’d lost the ability to manage her physical decline, how she’d like to find a therapist who works with patients facing chronic illness, how she turns to arts and crafts projects to get out of bed — and out of her head — on rough days.

He prescribed walks every day to build up her endurance and suggested she increase her continuous oxygen flow from her normal 2 liters per minute to 6 liters per minute when exercising. He looked down at her worn Birkenstocks and said, "Your shoes will have to change."

The doctor cranked up her oxygen and told her that if she wanted to get a transplant, she’d need to be able to do 15 sits-to-stands in a minute. He said this was non-negotiable. She won’t be able to use her arms after the surgery to get up from a chair or bed, he explained.