By: ANDRÉ PICARD
The Globe and Mail
Published Thursday, Nov. 13 2014, 7:30 AM EST
Canadians with cystic fibrosis are living dramatically longer than a generation ago, to the point where CF is now considered a chronic condition, not strictly a pediatric illness.
New research published in Thursday’s edition of the European Respiratory Journal based on numbers from Canada’s CF registry shows the median age of survival for CF patients has surpassed 50, up from just 32 in 1990. (That means half live into their 50s.)
In that same period, the median age of death rose to 32 from less than 22 years. (Meaning half the people with CF die before their 32nd birthday and half die after that date.)
It is one of the world’s highest survival rates.
“Although these numbers help tell a real success story, we have to remember that people with cystic fibrosis still die prematurely,” said Anne Stephenson, a respirologist at St. Michael’s Hospital in Toronto.
She said several factors contributed to the better outcomes, including an emphasis on nutrition, better treatment of infections and the advent of lung transplants.
“It’s not one thing: Care has improved across the board, thanks to what we’ve learned from research,” Dr. Stephenson said.
CF is a genetic disease that principally affects the lungs and the digestive system. It occurs in children who inherit two defective copies of the gene responsible for cystic fibrosis; it affects about one in every 3,600 children.
While there are treatments for the symptoms – enzymes to help with digestion, antibiotics for infections, exercises to clear the lungs, and transplants – there is no cure.