Living Dangerously With Cystic Fibrosis

March 20, 2008 — -- For Kayla Small and Maggie Marcus, being roommates is truly living dangerously.

The two college-age friends have a rare disease that means sitting side by side in a car, sharing a glass or indulging in a bar-side Generation Y-style kiss could put their lives at risk.

Small and Marcus suffer from cystic fibrosis, a genetic disease that affects about 1 in 2,500 white Americans. Other races can be affected, but the majority of cases are found among those of European descent.

CF is characterized by extremely thick mucus that clogs the lungs and digestive tract, causing respiratory and digestive problems.

The viscous sputum can make breathing difficult, but it's the ideal environment for bacteria of all kinds to thrive and form large antibiotic-resistant colonies.

Healthy people don't get sick when exposed to the bacteria of a person with CF. Trouble starts when bacteria is passed from one CF sufferer to another, and the consequences can be deadly.

CF patients generally start off with comparatively mild infections like staph, but as the disease progresses, the strains that take root become progressively more virulent.

Even with regular checkups, one can never be absolutely sure what kind of bacteria is growing at a given moment, and if a person with a particularly vicious bacteria passes it to someone who doesn't have it yet, he or she can inadvertently quicken the other's decline.

The bacteria of concern to CF patients are easily contracted, so doctors advise patients to keep a minimum safe distance of three feet between each other at all times.

"It's a struggle," said Clement Ren, director of the Cystic Fibrosis Center at the University of Rochester. "You want to provide guidelines that work but are not unreasonable. You can't put everyone in spacesuits."

Yet Small, a published writer and a Columbia University graduate, subscribes to a personal philosophy that values intimacy with others who share her experience above all else -- even if it could mean she will get sicker faster.

Bill Taub, a clinical social worker at Duke University Cystic Fibrosis Center, estimates that about 10 percent of CF patients he has worked with have expressed similar feelings.

"We in the field need to accept that some will make that decision," he said. "A patient once told me he didn't want to feel like a leper. Some people feel they need to ignore the restrictions in service to the quality of their lives. I am very sympathetic with her experience. That doesn't mean I agree with it."

Most of the outlaws are young, somewhere between 13 and 25, by Taub's estimate.

The mean life expectancy for CF patients is 37, but doctors say it is becoming increasingly common for patients to live into their 40s and 50s -- an enormous leap in 50-odd years. When the disease was first identified, children who contracted it rarely made it past the age of 2.

But averages don't mean much when you see your friends die young. A girl Small knew as a teenager died at 24, and a boy she met on the Internet died at 22 waiting for a lung transplant.

She says she broke off contact with him out of fear of witnessing the death of one of her best friends.

She's trying to work through her fear. She's even thinking about going to visit an ex-boyfriend with CF who told her to stay away because he'd contracted cepacia -- the category of bacteria that contains the strain that signals the end of the line for many.

Small and her BFF -- best friend forever, in text parlance -- are determined to spend as much time together as possible.

They play Marco-Polo in the supermarket -- "we go to different aisles and find each other by the sound of coughing" -- have slightly ironic slumber parties at their parents' homes and aren't above making out at the bar after a few drinks.

Her mom doesn't get it, and Small says it upsets her seeing them sharing beds, clothes and coffee cups.

"My mother said, 'I thought you wanted a long, healthy life. I thought you had goals,'" Small said. "This was my goal. She doesn't understand why we can't maintain our friendship and sleep in different rooms, or sit in the car with one in front and one in back. This is what I've always wanted -- intimacy."

Isolation wasn't always an issue for people with cystic fibrosis. Before the medical community realized the danger, patients shared rooms in hospitals, went on special overnight field trips, attended summer camps especially for them.

Thomas Murphy, director of Duke University's Cystic Fibrosis Center, says things changed in the early 1990s when data started trickling in from treatment clinics around the world.

Relatively healthy people with CF were getting inexplicably sicker and dying.

It appeared to have something to do with close proximity. Because people with CF have to go to clinics regularly to get checked out, they got to know each other. They made friends, made dates, sometimes married.

Researchers figured out that among the adult populations, patients were sharing more than understanding and love, they were sharing bacteria -- sometimes trading up.

Then there were the camps for children.

"We were encouraging them to get together, then came this info and we said, you know, these camps have got to go," Murphy said.

It was an unpopular decision at the time, but a necessary one.

Murphy says he was instrumental in closing North Carolina's Camp Kaleidoscope -- a beloved place for kids with serious illness -- to children with CF in 1993, but there were hundreds of such camps.

Ann McMullen, a nurse practitioner at the University of Rochester Medical Center, used to take groups of kids with CF to a 4H fair. She says they loved it.

Yet 20 years later she councils young people to keep each other at arm's length.

"Our approach to counseling young people is from a perspective of hope," she said. "It's a difficult message to get across in a positive way, but we say 'you don't need his bacteria and he doesn't need yours. You are each fighting your own fight.'"

The dictums are for their protection, and McMullen says the benefits of guidelines outweigh the negatives. But the negatives can't be overlooked. It's harder for people to "get support from each other. It has had an impact."

Meeting the social needs of sufferers wasn't a problem when cystic fibrosis was first brought to the public's attention: Children weren't expected to live past their second birthdays.

"This problem of loneliness has come out of the success in treatment," said Laurie Fink of the not-for-profit Cystic Fibrosis Foundation.

People like Joan Finnegan Brooks, 47, wasn't expected to make it to school age. Her brother died of CF when she was 9, but she has been very fortunate.

She meets her need for connection with others like herself through her work -- she does consulting for companies that work in CF treatment -- and through working with people who have loved ones with CF, such as parents and caregivers in the Boston area.

She connects with others like herself mostly by phone and e-mail. And when they meet in person, they follow the guidelines very carefully.

"It kind of sucks," she said.

But, like most people with CF, Brooks protects her physical health and the health of others with CF.

"It must be harder for young people, though," she said quietly. "That sense of isolation."

Lauren Beyenhof, 27, has a strain of cepacia growing in her lungs so she takes extra precautions -- she even wears a face mask when she travels on a plane.

True, there is the slight chance she'll be seated next to someone else with CF, but mostly the mask is in place to protect her own deeply compromised lungs. A healthy person's cold germ could put her in bed for a month.

Beyenhof, who lives in San Diego with her husband, understands Small's need to connect. Before discovering an online community and creating a forum of her own, she had an "absolute craving" for the simplest interaction. The need was deep-seated.

At 17, while being hospitalized for an infection, she befriended a boy of the same age. He and his brother both had CF.

One day they piled into the same room to watch a movie. When the staff discovered what they were up to they "hit the roof" before separating the three. The 17-year-old boy died soon after that.

Through the Internet Beyenhof has made friends with CF sufferers in her area. She visits them, but, because of the deadly cepacia, wears a mask, gown and gloves.

She leaves little gifts for her acquaintances at the hospital, but they have to air out at the nurses' station for 24 hours to make sure there are no "live bugs" on them before being delivered.

Beyenhof follows the rules of engagement, but has friends who choose like Small not to. She does not judge them.

"For them it is about quality of life, how they choose to spend the time they have left," she said.