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

Publish date: August 18, 2014
By
Amy Karon

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Continuing care a challenge

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]).


 

FROM ANNALS OF INTERNAL MEDICINE

References

Mortality in patients with cystic fibrosis fell by almost 2% per year in the United States between 2000 and 2010, authors of a registry-based study reported online August 18 in Annals of Internal Medicine.

Based on the analyses, patients born and diagnosed in 2010 can expect to live almost 40 years if the state of CF care remains static, and about 56 years if treatments progress at the current rate, said Todd MacKenzie, Ph.D., of Dartmouth School of Medicine in Hanover, N.H., and his associates.

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With increased standards of care, mortality in patients with cystic fibrosis has fallen by 1.8% per year between 2000 and 2010.

The study included 110 Cystic Fibrosis Foundation–accredited care centers in the United States. Mortality fell by 1.8% per year between 2000 and 2010 (95% confidence interval, 0.5%-2.7%), and males had a 19% lower risk of death than females (95% CI, 13%-24%), the investigators found. The estimated median survival for children born and diagnosed in 2010 was 39 years overall (95% CI, 38-40 years), 37 years for females (95% CI, 35-39 years), and 40 years for males (95% CI, 39-42 years), they reported (Ann. Int. Med. 2014 Aug. 18 [doi:10.7326/M13-0636]).

The results apply only to patients diagnosed in the first year of life, as patients diagnosed later are likely pancreatic-sufficient and therefore have a better prognosis, Dr. MacKenzie and associates noted. Improved survival could partially reflect the increasing diagnosis of patients with less severe phenotypes, they said.

The Cystic Fibrosis Foundation funded the study. Dr. MacKenzie reported no conflicts of interest. Three coauthors reported grant support from the Cystic Fibrosis Foundation.

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