19 Nov Cystic Fibrosis Survival Improved By Decades
MedicalResearch.com Interview with:
Anne Stephenson MD, PhD
Division of Respirology
The Toronto Adult Cystic Fibrosis Centre
St. Michael’s Hospital Toronto, ON
Medical Research: What is the background for this study? What are the main findings?
Dr. Stephenson: Cystic Fibrosis is progressive genetic disease that results in very thick secretions in various organs such as the lungs, pancreas, and digestive tract. Over time, these thick secretions damage organs in particular, the lungs, which results in respiratory failure due to recurrent chest infections. Cystic Fibrosis patients are also at high risk for malnutrition due to the inability to absorb food which is associated with poor survival. In the 1960s, people with Cystic Fibrosis died at a very young age and in fact, parents who had a child born with Cystic Fibrosis at that time were told that there was a 50% chance their child would not live to attend kindergarten. Over the last several decades, we have seen a significant increase in the survival of individuals with CF. Individuals born with Cystic Fibrosis today can expect to not only attend kindergarten, but complete high school, perhaps attend college or university, have a career, get married or have children as people are living well into adulthood with this disease. The median age of survival in Cystic Fibrosis today is approximately 50 years of age in Canada which is quite remarkable.
Medical Research: What should clinicians and patients take away from your report?
Dr. Stephenson: The demographics of the CF population are changing. Individuals with Cystic Fibrosis are living well into adulthood and Cystic Fibrosis is no longer a paediatric disease. In addition, fewer people with CF are malnourished compared to 20 years ago and currently almost 60% of the Cystic Fibrosis population is over the age of 18 years. CF patients are dealing with more complications such as diabetes, anxiety, depression etc than previously seen because they are living longer.
There are many reasons for improved survival such as preventing malnutrition, aggressive treatment of infections, implementing infection control measure to prevent infection and in the case of end-stage lung disease, the availability of lung transplantation.
Despite these improvements, every year people with Cystic Fibrosis die and in 2012 the median age of death was 32 years. This means that half of the patients who died in 2012, died before their 32nd birthday.
Medical Research: What recommendations do you have for future research as a result of this study?
Dr. Stephenson: We have made significant advances in Cystic Fibrosis care that have improved survival but we need to do more research to increase our understanding about the factors that are associated with mortality, morbidity and quality of life. Recent medications have been developed to correct the underlying cause of Cystic Fibrosis and we need to continue our goal of finding a cure for the this devastating disease.
Citation:
Anne L. Stephenson, Melissa Tom, Yves Berthiaume, Lianne G. Singer, Shawn D. Aaron, G.a. Whitmore, and Sanja Stanojevic. A contemporary survival analysis of individuals with cystic fibrosis: a cohort study. European Respiratory Journal, November 2014 DOI: 10.1183/09031936.00119714
Last Updated on November 19, 2014 by Marie Benz MD FAAD