Cystic Fibrosis Survival Rates: Even Better Than Reported?

A new report suggests that when cystic fibrosis (CF) survival estimates are based on living to a specified age, the CF survival rate is higher than the reported overall median age of survival.

The authors of the review, published online ahead of print in Current Opinion in Pulmonary Medicine, set out to describe the different measures of CF survival and review the recently published literature about CF survival in order to understand the changing epidemiology of the condition.

When they tracked patient outcomes using national data registries for CF, the Ontario, Canada, based authors observed a dramatic improvement in CF patients’ survival. Because survival can be measured in numerous different ways, the authors note that it is important for clinicians to “understand these different metrics in order to clearly translate this information to patients and their families.”

They write that a number of individual factors have an impact on survival, including patients’ sex, age at diagnosis, ethnicity, and degree of lung function. They note that survival estimates that are conditional on surviving to a specified age are more relevant to CF patients and are higher than the reported overall median age of survival. The median age of survival continues to increase in many countries, while CF mortality rates are decreasing.

The prolonged survival in CF could be a result of newborn screening for the condition, the authors suggest.

“When comparing survival between countries, one needs to ensure that similar data collection and processing techniques are used to ensure valid and robust comparisons,” they conclude.

—Michael Gerchufsky

Reference:

Corriveau S, Sykes J, Stephenson AL. Cystic fibrosis survival: the changing epidemiology [published online August 22, 2018]. Curr Opin Pulm Med. doi:10.1097/MCP.0000000000000520.