Researchers have found that an emerging population of middle-aged cystic fibrosis (CF) patients contains significantly more females and includes a large proportion of patients who lived for decades without a diagnosis or specialized care. The findings published in the American Journal of Respiratory and Critical Care Medicine may help guide future treatment of CF as survival past 40 becomes increasingly common.
In 1962, the median predicted survival for children with CF was 10 years. Today, it is 37, and children diagnosed today can expect to live into their 50s. In addition, many more patients with non-traditional symptoms are being diagnosed for the first time as adults.
The researchers analyzed epidemiological and health data on 156 CF patients over 40 years of age who receive care at National Jewish Health. In addition, data were analyzed on nearly 3,000 patients from around the country who were included in the Cystic Fibrosis Foundation Patient Registry from 1992-2007.
The researchers found that fewer females diagnosed as children survived to age 40. However, among those diagnosed as adults, females represented a significant majority, accounting for 54% nationally. Among the adult diagnosed patients, females survived on average 9 to 14 years longer than males.
The findings also indicate that patients diagnosed as adults do not really have milder diseases—as is commonly believed—just delayed onset of an equally severe form of the disease. Although patients diagnosed as adults live longer than those diagnosed as children, the adult-diagnosed patients lose lung function as rapidly as those diagnosed in childhood, and approximately 85% die of respiratory failure or post-transplant complications.
Researchers believe CF remains undiagnosed in a significant number of adults. The findings indicate that once those patients are accurately diagnosed, proper care at a CF center can significantly improve their health, including reversing progressive lung function decline and improving their lung function for at least four years.
Older patients commonly do not get specialized CF care. Less than half of long-term CF survivors continued to be seen at CF centers as they pass 40 years, with the fewest among the adult-diagnosed patients.
“In the coming years, more and more cystic fibrosis patients will be living into their 40s, 50s and beyond,” said Jerry Nick, MD, a pulmonologist at National Jewish Health and lead author of the study. “Our findings concerning the role of gender, in survival, progressing of disease, and type of care in current long-term survivors provides important insights that will help us prepare for better treatment of the steadily aging CF population.”