In 1990, the median survival age for Canadian patients with cystic fibrosis on average was 31.9 years. That number increased to 49.7 years in 2012 and has since been updated based on the 2013 data to 50.9 years. Furthermore, the median age at which Canadian CF patients die in 1990 was 21.7. That value increased to 32 years in 2012. The new data shows that the death rate decreased from 1.4 deaths per year in 1990 to 0.99 per year in 2012.
Cystic fibrosis is a chronic disease that affects the lungs and the digestive system; it is autosomal recessive genetic disorder. A defective gene causes the body to produce an unusually thick and sticky mucus that can lead to severe lung infections and pancreatic dysfunction.
“By analyzing national data, we can confirm what we are seeing clinically, specifically that our cystic fibrosis patients are living longer, well into adulthood and middle age, which is very exciting,” said Dr. Anne Stephenson in a press release. “Although improved survival is very positive news for the CF community, we must remember that people with cystic fibrosis still die prematurely. In fact, in 2012, half of the deaths due to cystic fibrosis happened before the patients reached their 32nd birthday. This reinforces the need for continued research in this area in order to optimize health for all individuals living with this progressive disease.”
According to Dr. Stephenson, these improvements in cystic fibrosis survival rates come from multi-disciplinary care, more rigorous infection control practices, new drugs, and transplant options. Also, improved nutrition and controlling lung infections were pointed out as important factors, since malnutrition and lung infections are associated with cystic fibrosis deaths. Further, all CF patient demographics had improved survival rates, even the ones with more severe cases.
Newborn screening is now available for the disease in Canadian provinces, and it is expected to also improve survival rates — data will be available within a few years. In addition, Vertex’s Kalydeco drug for CF patients was approved by Health Canada, making it the first therapy to target the underlying cause of CF: “St. Michael’s was the only site outside of the United States involved in the Phase 2 clinical trial for the drug and had the largest number of patients enrolled in the Phase 3 trial.”