Life expectancy approached 40 years for children born with CF in 2010

The findings are "worth celebrating," said Dr. Joseph Pilewski and Dr. Darren Taichman. "However, continuing to ensure the kind of care that has resulted in a growing number of patients with CF living far into adulthood will not be simple or inexpensive."

The patients in the study all were treated at CF Foundation–accredited centers, which provide coordinated, multidisciplinary care, Dr. Pilewski and Dr. Taichman said. And patients with CF often need care from additional specialists for comorbidities such as diabetes, osteoporosis, cancer, depression, and anxiety, they added. "Caring for adults with CF requires a village."

As for other complex primary care scenarios, coordinating and managing the medical and social issues of CF patients can be time-intensive, stressful, and poorly reimbursed, they said. Furthermore, coordinated nursing, social work, nutrition, and respiratory therapy services often are not available outside accredited CF care centers.

"Will the remarkable community support that has enabled progress in CF care and survival continue in an era when growing proportions of patients are no longer children, for whom it may be easier to solicit donations?" Dr. Pilewski and Dr. Taichman asked. "If not, we will need to think hard about other solutions because, although survival of patients with CF has improved, many still suffer and die prematurely."

Dr. Pilewski is codirector of the Adult Cystic Fibrosis program at the University of Pittsburgh Medical Center. Dr. Taichman is executive deputy editor for Annals of Internal Medicine. These remarks were taken from their editorial accompanying Dr. MacKenzie’s report (Ann. Int. Med. 2014 Aug. 18 [doi:10.7326/M14-1534]).