He has toured 47 states and 23 countries to increase awareness of cystic fibrosis (CF)—a genetic disorder that causes mucus to build up and clog some organs of the body, primarily the lungs—and he gets hugs everywhere he goes. This furry advocate is Burke P. Bear, a cuddly teddy bear named in honor of Burke P. Derr, who died two days before his 19th birthday in 1997 from complications of CF.
Today Burke’s memory lives on through the work of his father, Bob Derr, for Pennsylvania Cystic Fibrosis, Inc. (PACFI), and the CF researchers it supports, including Antoinette Moran, M.D., a renowned pediatric endocrinologist in the University of Minnesota Medical School’s Department of Pediatrics.
Burke was a pretty typical kid in most regards, but his CF landed him in the hospital about three times a year for weeks at a time. Some of his friends, on top of their CF, also had cystic fibrosis-related diabetes (CFRD), which affects about 20 percent of adolescents and 40 to 50 percent of adults who have CF.
In the past, people with CF who developed diabetes faced a much higher mortality rate than those without diabetes. Gradually, as doctors have become more aggressive about screening for diabetes, the number of deaths stemming from CFRD has decreased.
Much of that progress is thanks to Moran’s work. She pioneered CFRD research during her residency at the University of Minnesota in the 1980s when she noticed that a surprisingly high number of CF patients were developing diabetes. And much of Moran’s progress was made possible by PACFI, which has supported her CFRD studies for the last decade.
Moran has used the PACFI funding to get fledgling research ideas off the ground. Currently, she’s putting the organization’s money toward a study focused on updating mortality rate trends in CFRD patients.
The progress holds special meaning for Bob Derr and for everyone who knew Burke. “To lose someone you love and to have something like this come out of it is remarkable,” Derr says.
- Grace Birnstengel