Thanks to advances in pulmonary and nutritional therapies for cystic fibrosis, children who were born and diagnosed with this disease in the United States in 2010 are expected to live longer than those born earlier, a study recently published in the journal Annals of Internal Medicine showed.
The registry-based study, entitled “Longevity of Patients With Cystic Fibrosis in 2000 to 2010 and Beyond: Survival Analysis of the Cystic Fibrosis Foundation Patient Registry,” aimed to determine the cystic fibrosis survival trends between 2000 and 2010, as well as to project the survival expectancy for children born in 2010.
Researchers analyzed the data of 110 centers accredited by the Cystic Fibrosis Foundation, including all patients represented in the Foundation’s Patient Registry between 2000 and 2010.
Results showed that the number of patients with cystic fibrosis increased from 21,000 in 2000 to 26,000 in 2010. However, the median age rose from 14.3 to 16.7 years, while adjusted mortality decreased by 1.8% each year. Projections leave researchers optimistic: if mortality remains at the levels now observed, median survival among children born in 2010 with the disease should be 37 years for women and 40 years for men. Furthermore, if the mortality rate continues to fall at the rate observed in the studied period, median survival should eventually climb to 50 years — encouraging prospects for a disease that at one time rarely saw patients live past early childhood.
These findings are important for future prognostic discussions. In addition, researchers emphasize that the health care system should expect a higher number of adults with cystic fibrosis in the near future. The team warns, however, that the Cystic Fibrosis Foundation Patient Registry does not include all patients with this disease in the United States, which somewhat limits the findings of this study.