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Cystic fibrosis patients losing a connection

With new guidelines urging them to stay apart, patients are forced to choose between community and isolation

Katie Lockwood (with her husband, Arden) has cystic fibrosis and always valued being part of the CF patient community which provided answers, guidance, and support.

JULIA CUMES FOR THE BOSTON GLOBE

Katie Lockwood (with her husband, Arden) has cystic fibrosis and always valued being part of the CF patient community which provided answers, guidance, and support.

Cystic fibrosis has not been easy for Katie Lockwood.

But, along with a regimen of chest physical therapy to keep the sticky mucus from building up in her lungs, frequent doctor visits, and the emotional burden of an uncertain future, the disease also brought with it a community. When she was 4, Lockwood spent a week at a summer camp just for CF patients. While growing up, she attended at least five CF fund-raisers each year, enjoyed annual ice-skating and dance parties to raise money for research, learned public speaking at a young age, even met actress Rosie O’Donnell.

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Lockwood, now 25, remembers these connections as “the only good part of having a disability.”

In many ways, life will be different for those now growing up with the genetic disease. Children born with cystic fibrosis three decades ago weren’t expected to live much beyond college graduation; now, average life expectancy for the 30,000 Americans with CF approaches 40. The first treatment to target genetic anomalies that cause CF is now on the market.

“I used to go to a lot of funerals of 6- and 7-year-olds — now we’re going to weddings of 25-year-olds,” says Dr. Henry Dorkin, the director of Boston Children’s Hospital Cystic Fibrosis Center. “I like this a lot better.”

But along with the medical advances comes increasing isolation: Doctors now know that contact between one person with CF and another can be deadly.

Most recently, the Cystic Fibrosis Foundation, the national nonprofit that funds research and accredits hospital centers that treat CF patients, announced its recommendations for stricter infection control guidelines. At outdoor CF Foundation events, patients shouldn’t come closer than six feet from each other. At indoor events, only one CF patient can attend at a time. Gone are the summer camps and dance parties of Lockwood’s youth.

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“It’s another loss in a lifetime of being compromised by the disease. It’s just one more thing that makes them feel different,” says Elaine Low, Katie’s mother. “But as a parent? I understand.”

In the 1980s, when Lockwood was born, physicians knew that certain bacteria, generally harmless to normal lungs, can devastate the lungs of people with cystic fibrosis. But they didn’t know how easily the bugs could be spread.

But in the early 1990s, using new techniques of molecular testing, researchers found that the exact same strain of a particularly dangerous bacteria, Burkholderia cepacia, had been passed from child to child at CF summer camps. By the mid-1990s, the summer camps were closed. Lockwood never returned for her second summer.

Infection control efforts ramped up, focused primarily on Burkholderia. The bacteria is commonly found in soil and water, but the strains in the natural environment are usually different from the strains found in CF patients. For people with CF, many infections with Burkholderia can be deadly. As a result, those found to have Burkholderia in mucus from their lungs were scheduled to have clinic visits at different times so as not to spread the bug in the CF clinic. They were asked not to attend the CF Foundation’s indoor events, due to the risk of infecting others.

They were, essentially, “microbiological pariahs,” said Dr. Lisa Saiman, a professor of pediatrics at Columbia University Medical Center who heads the CF Foundation’s committee to update infection prevention and control recommendations.

Caitlin O’Hara, a graphic designer living in Boston, was terrified of Burkholderia. Even before the current guidelines, the 30-year-old remembers, her mother was intensely preoccupied with infection control, and went above and beyond the regulations that were in place at the time. When transient flares of her lung disease would force O’Hara into the hospital for brief stints, her mother would arrive with cleaning supplies. O’Hara was careful to ask doctors and nurses to fully don their gowns and gloves before examining her.

While the medical team would do what they could to “appease” her mother, O’Hara remembers how people would roll their eyes, exasperated. “They acted like we were paranoid, and that’s a terrible feeling,” she said.

“I didn’t like being in the hospital because I felt like I was being exposed,” remembers O’Hara. “We were so vigilant in the face of everyone, back then, not being quite so vigilant.”

Despite her vigilance, O’Hara contracted Burkholderia cepacia after a hospital stay at age 16. Since then, she says, her lung function has declined – she’s now undergoing evaluation for lung transplant.

Around that time, Saiman was asked by the CF Foundation to review evidence of CF person-to-CF person transmission of bugs other than Burkholderia. Although patients with Burkholderia were segregated from others with CF at that time, everyone else still freely mingled. The 2003 guidelines Saiman and her team published were a significant shift: Cystic fibrosis centers were asked to minimize contact between all patients — not just those with Burkholderia — at clinic visits and in the hospital. CF patients were encouraged to stay three feet away from each other, they were discouraged from socializing outside of the hospital, and were even cautioned to avoid attending the same school as another CF patient.

But perhaps even these recommendations weren’t enough. An event in the early 2000s led Boston Children’s Hospital to adopt even more stringent guidelines. Sure, CF patients in the hospital were under strict instructions to stay away from each other, but it wasn’t unheard of for one patient — bored and alone — to sneak into someone else’s room at night.

This changed when doctors started to identify a new bug, called Burkholderia dolosa, in the lungs of their CF patients. The bug was nearly impossible to treat, even with the strongest intravenous antibiotics. Lung function plummeted. Patients died. While some remained stable, others alive today are waiting for lung transplants. An epidemiologic hunt began. Doctors ultimately drew a chain of connection that linked patient to patient through indirect encounters at various clinics, in the hospital, at a Super Bowl game.

As a result, “We took Draconian measures,” said Dr. Ahmet Uluer, the director of Brigham and Women’s Hospital and Boston Children’s Hospital Adult Cystic Fibrosis Center. The cystic fibrosis team did away with communal waiting rooms. Patients would wait in washed and disinfected exam rooms instead. Physicians wore gowns and gloves to examine each patient. When patients were admitted to the hospital, they could leave their rooms only with an escort — again, to limit the chance for contact with others. Webcasts and online streaming of live events became the new way to disseminate information, rather than in-person gatherings. There hasn’t been another case of Burkholderia dolosa at Children’s Hospital since.

Children’s wasn’t alone. Epidemiologic studies have described other bugs that have moved from patient to patient in CF centers throughout the globe. Researchers have also learned that less virulent bugs can cause more long-term lung damage than previously thought. And experiments have proven that germs can travel farther through the air than doctors once thought — not just three feet, maybe twice as far.

Which is why, Saiman explains, it was time to update the infection prevention and control guidelines. The CF Foundation got together another committee of doctors, microbiologists, social workers, parents of children with CF, and an adult with CF. Two years, countless conference calls and meetings later, they voted on an extensive set of recommendations. The new knowledge about how droplets can travel means that CF patients at outdoor events shouldn’t come closer than six feet from each other. As many different bugs — not just Burkholderia — can cause lung damage, and pass from patient to patient more frequently than previously thought, only one person with CF should attend an indoor event sponsored by the CF Foundation or CF centers at a time.

“We can’t quantify the risks, but we don’t want to take any risk with somebody’s life,” Saiman explains. She acknowledges, of course, that adults are still free to take whatever risks they choose in their everyday lives. These aren’t laws; the guidelines can’t keep two CF patients from going out to the movies, or dinner, or falling in love, but in the interest of safety, they’re certainly discouraged from doing so.

For CF patients, the reception has been mixed; some, like O’Hara, are relieved, others view the recommendations as unnecessarily paternalistic. “It’s their job to cure me, not to protect me,” said Lockwood.

When Kayla Small received a letter from her doctors about the new recommendations some months back, she felt “defeated.” Small, now 29, didn’t grow up with friends who had cystic fibrosis. Her family wasn’t particularly active in the CF Foundation. But in her late adolescence, she started to wonder what it might be like to find a community of others who shared her experience with the disease.

One afternoon back in 2006, Small met a boy with cystic fibrosis in the clinic waiting room. They went out for drinks. Their hands touched. She was breaking the rules.

But when he told her that Burkholderia cepacia was growing in his sputum, Small was not willing to risk her health. They never saw each other again.

These days, years later, Small will occasionally seek out online cystic fibrosis message boards. She felt a spark of jealousy recently when she read about five cystic fibrosis patients who are all close friends after receiving lung transplants.

But to find support, Small — like those growing up with CF today, without summer camps or communal waiting rooms — finds that she must look outside the world of cystic fibrosis. “To have people know about you having CF is of immense value, and to have it in the open is of immense value,” she said, “But that doesn’t mean they have to have the same experience.”

Dr. Daniela J. Lamas can be reached at danielalamasmd@gmail.com.

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