A baby born with cystic fibrosis in the early ’70s would almost surely have died by age 2. Even 15 years ago, individuals with cystic fibrosis had a life expectancy of only 20 years. In 2009, though, medical advancements and a whole-life approach to treatment have upped that life expectancy to 38 years. And in six to nine months, East Tennessee should have a care center dedicated to serving those who are now living to adulthood, geared towards both prolonging their lives and helping them enjoy a higher standard of living.
“We already have a local children’s care center at East Tennessee Children’s Hospital, but 40 percent of CF patients in our area are now over 18, and Children’s can’t serve them,” says Brian Jenkins, associate executive director for the East Tennessee chapter of the Cystic Fibrosis Foundation. “Local pulmonologists who know how to treat CF are doing the best they can to serve the adults, but we’re working to establish an adult center so the adults can have more appropriate care.”
Funding and location details are being ironed out as quickly as possible, says Jenkins. “We’ll have an initial startup from local money, and $250,000 is our estimate to secure the center. We do not have any funds secured, but we have ‘asks’ out, applications being considered by foundations in the East Tennessee area.”
At the same time, the foundation is trying to pull together the necessary medical community resources. “We’re currently working with UT Hospital—that’s about as detailed as I can be at this point,” says Jenkins. “Our care centers are all within hospitals, because all the needed pieces can be there; pulmonology, respiratory, dietary. It’s a very extensive disease.”
Cystic fibrosis is an inherited disease that affects the body’s ability to move salt and water in and out of cells, causing the lungs and pancreas to secrete thick mucus, blocking passageways and preventing proper function. It primarily affects people of white northern-European descent, and the disease occurs pretty evenly across the nation, says John Rogers, medical director of the Cystic Fibrosis Center in pediatric pulmonology at ETCH.
A new local adult care center in Knoxville would be only the second in Tennessee; Vanderbilt in Nashville pioneered the first more than 10 years ago. “Studies have shown that people who are taken care of at centers, who receive a multi-disciplinary therapeutic approach, live longer and are healthier for a longer period of time,” says Rogers. “But back when so few patients were living to adulthood, doctors were not initially trained to treat adult CF patients. More and more adult doctors in this area now have familiarity with adult CF, and we’re blessed and pleased that we have a pulmonology group willing to help develop an adult center here.”
Even though the eventual downfall of an individual with cystic fibrosis tends to be some type of chronic obstructive pulmonary disease (COPD), Rogers stresses that CF affects many other organs and parts of the body, not just the lungs. “There have been new medicines, many new therapies developed in the past 15 years that can prolong lives, but a big part of the success is making sure people are educated about them. It’s also important that children and adults stay well-nourished and are physically fit, have good strength and endurance. That requires the input of nutritionists, respiratory therapists, physical therapists.”
According to foundation records, there are currently 296 registered CF patients in East Tennessee, 105 age 18 or older, 61 ages 12-17, the rest under 12. Because medical strides have been so recent and rapid, some older patients were, to put it bluntly, not expecting to live as long as they now can, which can create additional uncertainties and social issues, says Jenkins. That’s why social workers and mental health professionals will be part of the adult care center. “Everyone is reassuring the individual that care is there now, they can live as adults, get married, have a family,” says Jenkins.
The existing CF adult care centers closest to East Tennessee are at Vandy, at Duke in Raleigh-Durham, N.C., and in Atlanta. “By having one here at Knoxville, we’ll be able to extend the circle to Bristol and Chattanooga, and outlying areas such as Northern Georgia and Southeast Kentucky—it would become a regional center,” says Jenkins.
Rogers hopes the adult center will promote and enhance CF therapies on the national horizon, too. “People with CF have to do a lot to maintain their health. Most are on more than a dozen meds. The treatments to optimize their health can take two-three hours a day. It’s an incredible burden. But there are therapies being studied that involve a dramatic reversal of problems—not totally curative, but closer to curative. We expect them to be available in the next five years.
“I hope in coming years people with CF can live longer and not have to devote so much of their lives to medical care. And we can be a part of that.”