Although people diagnosed with cystic fibrosis are surviving longer than ever before, their lifespan is nearly 10 years longer in Canada than the United States, reports new research.

Cystic fibrosis is a genetic disease that affects the lungs, pancreas, liver, intestines, sinuses, and sex organs. Cystic fibrosis causes your mucus to be thick and sticky.

The mucus clogs the lungs, causing breathing problems and making it easy for bacteria to grow. This can lead to repeated lung infections and lung damage.

Differences in survival between U.S. and Canadian patients depended U.S. patients' insurance status - Canadians have universal, publicly funded health care. U.S. patients with private health insurance had similar survival rates to their Canadian counterparts.

However, Canadians had a 44 percent lower risk for death than U.S. patients receiving continuous Medicaid or Medicare, a 36 percent lower risk than those receiving intermittent Medicaid or Medicare coverage, and a 77 percent lower risk than those with unknown or no health insurance.

Funding for the research was provided by the U.S. Cystic Fibrosis Foundation.

High Fat Diet A Factor

There is as yet no cure for CF, but treatments have improved greatly in recent years. In the past, most deaths from CF were in children and teenagers. Today, with improved treatments, some people who have CF are living into their forties, fifties, or older.

Canada began to see greater improvements than the U.S. starting in 1995, with a more dramatic increase in the survival rate in Canada noted in 2005. Better nutritional status in cystic fibrosis has been linked to improved survival.

Canadians with cystic fibrosis were exposed to a high fat diet in the 1970s, which was not implemented in the U.S. until the 1980s, which may explain the improved survival rates starting in 1995. Lung transplantation is one of the few treatments for cystic fibrosis that can positively impact survival almost immediately and a higher proportion of cystic fibrosis patients receive a transplant in Canada than in the U.S..

The survival difference increased dramatically in 2005, the year that the U.S. began using a lung allocation score to prioritize people on the lung transplant waiting list. This score is not used in Canada.

The findings highlight the central goal of the U.S. Cystic Fibrosis Foundation’s lung transplant initiative, which is to improve transplant outcomes for people with cystic fibrosis in the United States.

Researchers at St. Michael’s Hospital in Toronto, Ontario, Canada studied national cystic fibrosis registry data for 5,941 patients in Canada and 45,456 patients in the U.S. from 1990 to 2013. They found that the median age of survival was 50.9 years in Canada compared to 40.6 years in the U.S., based on most recent registry data (2009 to 2013).

Even after adjusting for patient characteristics, such as age and severity of disease, the risk for death among people with cystic fibrosis was 34 percent lower in Canada than in the United States.

Cystic Fibrosis is also known as Mucoviscidosis, Pancreas fibrocystic disease, Mucoviscidosis of the pancreas, and Fibrocystic disease of the pancreas.

Anne L. Stephenson, MD, PhD; Jenna Sykes, MMath; Sanja Stanojevic, PhD; Bradley S. Quon, MD, MSc; Bruce C. Marshall, MD; Kristofer Petren, BA, BSc; Josh Ostrenga, MSc; Aliza K. Fink, DSc; Alexander Elbert, PhD; Christopher H. Goss, MD, MSc Survival Comparison of Patients With Cystic Fibrosis in Canada and the United States: A Population-Based Cohort Study Ann Intern Med. 2017. DOI: 10.7326/M16-0858

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