Hope for the future

Help for those born with cystic fibrosis

Although only just over 4,300 Canadians have cystic fibrosis (CF), you may hear a lot more about the disease this May, which is CF Awareness Month. Just 70 years ago, those diagnosed with this rare genetic disorder—which affects the lungs, pancreas and other organs—rarely survived past elementary school age. Today, with new but very costly therapies, more than half of those with CF are over 18, with the median survival age at just over 50 years old.

Two Canadians are diagnosed with CF every week, and the vast majority are diagnosed by the age of 2. More than 2,000 mutations are known to affect the CF gene. The result is that a defective cystic fibrosis transmembrane conductance regulator (CFTR) gene produces malfunctioning or no CFTR proteins; these proteins are responsible for ensuring that mucus in the body is the right consistency, and not thick and sticky.

This thick mucus in the pancreas, liver and intestines causes blockages that stop enzymes from digesting food properly, which may lead to malnutrition, weight loss, a specific type of diabetes, liver problems and colorectal cancer. The poor quality of the mucus in the lungs causes bacteria not to be cleared out, and the result is many respiratory infections, inflammation and lung problems, potentially requiring a transplant.

While there is no cure for CF, some therapies have focused on clearing the mucus, reducing the inflammation in the lungs with inhaled medications or clearing the infection with antibiotics. Preventing infections through proper hygiene and immunizations is also important, and digestive enzymes can be helpful too.

Beyond all the medications and therapies, what many with CF need most are emotional support, a listening ear, a kind word, patience and understanding during their off days. More information can be found at Cysticfibrosis.ca.

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Pharmacies in Costco’s Quebec locations are independently owned and operated by pharmacists.

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