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Improved Survival of Patients With Cystic Fibrosis FREE

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The full report is titled “Lifetime of Patients With Cystic Fibrosis in 2000 to 2010 and Beyond: Survival Analysis of the Cystic Fibrosis Foundation Patient Registry.” It is in the 19 August 2014 issue of Annals of Internal Medicine (volume 161, pages 233-241). The authors are T. MacKenzie, A.H. Gifford, K.A. Sabadosa, H.B. Quinton, E.A. Knapp, C.H. Goss, and B.C. Marshall.

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Ann Intern Med. 2014;161(4):I-15. doi:10.7326/P14-9026
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What is the problem and what is known about it so far?

Cystic fibrosis (CF) is a life-shortening disease caused by an inherited gene abnormality, which can impair the function of many organs and cause symptoms that include breathing and digestive problems. Our understanding of how best to prevent complications and treat them when they occur has improved. Understanding what changes have occurred in the survival of individuals with CF is important to patients and their families and can help in the planning of health care services as people with the disease live longer.

Why did the researchers do this particular study?

To find out whether the survival of patients with CF has improved over time and to estimate the lifespan of an infant diagnosed with the disease.

How was the study done?

A database was established in 1966 to collect information about patients with CF. The database has grown to include information about patients treated at 110 specialized care centers in the United States. The information collected includes the age of patients when they were diagnosed, their symptoms, which gene abnormality they have, and the cause and date of their death. The researchers used these data to assess whether patients born and diagnosed in 2010 can be expected to live longer than those born and diagnosed in 2000.

What did the researchers find?

The average age of patients in the database increased between 2000 and 2010, and the survival of patients with CF has improved. Overall, male patients have better survival than females, as do patients with no symptoms at diagnosis. Patients with certain gene abnormalities seem to have worse survival than others. If no further improvements in survival occur, males born and diagnosed with CF in 2010 would be expected to live an average of about 40 years and females would be expected to live about 37 years. If survival continues to improve at the rate observed between 2000 and 2010, a person born and diagnosed in 2010 would be expected to live more than 50 years.

What were the limitations of the study?

It is not possible to know whether current estimates of survival will continue to be valid. In addition, the results reported are estimates for a large group of patients, some of whom can be expected to do better or worse.

What are the implications of the study?

The expected survival of patients with CF has improved. Parents of newly diagnosed infants with CF should discuss expected survival with their physicians because it may influence important life choices. The health care system should anticipate a need to care for more adults with CF as patients live longer.





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