Cystic Fibrosis Awareness Month: Increasing Life Expectancy, Promising Advances and Challenges

Cystic Fibrosis Awareness Month: Increasing Life Expectancy, Promising Advances and Challenges

The month of May is dedicated to raising awareness of a disease that once claimed most sufferers at a young age, but for which science and care have made very significant gains - cystic fibrosis.

Cystic fibrosis primarily affects the lungs, but can also damage multiple organs including the pancreas, resulting in a high risk of diabetes. In collaboration with the CHUM, the IRCM, which follows many patients with cystic fibrosis and diabetes in its clinic, would like to emphasize that research holds great hope for these patients, who have recently benefited from a new drug, Trikafta, which constitutes a major advance, in addition to the innovative care approaches implemented at the Institute. 

"This new drug allows us to move from treating the consequences of the disease to treating the cause. The rapid improvements observed are impressive, I would even say magical," says Dr. Rémi Rabasa-Lhoret, Director of the Metabolic Diseases Unit at the IRCM.  

Cystic fibrosis is the most common fatal genetic disease in Canadian children and young adults.

Increasing life expectancy brings challenges

As life expectancy for people with cystic fibrosis has increased from 20 to more than 50 years in the 1980s to 2016, new challenges have emerged that clinicians and researchers are working to overcome. Important new complications, such as diabetes (excess blood sugar) secondary to cystic fibrosis, have emerged.

The exact causes of diabetes secondary to cystic fibrosis are not well established. A research group at the IRCM is attempting to elucidate the mechanisms involved in the development of diabetes secondary to cystic fibrosis, as well as the relationship between elevated blood sugar levels and changes in weight and lung function. The team is also investigating various strategies to simplify screening for CF-related diabetes. 

The IRCM applauds the resilience of people living with cystic fibrosis, and is committed to continuing research and refinement of care to ensure that the next generation of health and life expectancy indicators for these patients will be ever better.

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