Cystic Fibrosis - Disease Information

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Cystic fibrosis:

  • Is the most common, fatal genetic disease affecting young Canadians
  • Is a multi-organ disease that affects mainly the lungs and the digestive system
  • Causes the body to produce abnormally thick, sticky mucus that: blocks airways, can lead to fatal lung infections, blocks ducts of the pancreas, prevents enzymes from reaching the intestines to digest food.
  • Is not contagious
  • Is caused by a mutation, or defect, in the Cftr gene
  • Currently there is no cure for cystic fibrosis, but research inspires real hope

Individuals with cystic fibrosis:

  • May have a range of symptoms, including: difficulty breathing, very salty-tasting skin, persistent coughing, wheezing or pneumonia, poor weight gain (despite a normal or large appetite).
  • Are affected by CF with varying degrees of severity
  • Undergo rigorous physiotherapy programs every day to relieve congestion in their lungs.
  • Consume artificial enzymes every time they eat to help them absorb adequate nutrients from their food.
  • Are living longer than ever before.
  • May experience various diseases related to cystic fibrosis as they age, such as: diabetes, osteoporosis, arthritis, chronic pancreatitis, liver disease, nasal polyps, allergic aspergillosis.

Cystic fibrosis statistics:

  • Approximately one in every 3,600 children born in Canada has CF.
  • Approximately 60% of patients in Canada are diagnosed with cystic fibrosis in the first year of life, and 90% by 10 years of age.
  • Approximately 3,500 children, adolescents and adults in Canada attend specialized cystic fibrosis clinics.
  • One in every 25 Canadians carries a defective version of the gene responsible for cystic fibrosis
  • More than 50% of Canadians who have cystic fibrosis carry two copies of the most common cystic fibrosis mutation, ?F508.
  • More than 85% of all individuals with cystic fibrosis in Canada carry at least one copy of ?F508.
  • In the 1960s, most children with cystic fibrosis did not live long enough to attend kindergarten.
  • Today, half of all Canadians with cystic fibrosis may be expected to live into their late 30s, and even beyond.
  • In 2007, for the first time in Canada, the number of adults with cystic fibrosis - i.e. persons aged 18 and older - surpassed the number of children.

Cystic fibrosis research:

  • In 1989, a Canadian-led team of researchers identified the gene responsible for cystic fibrosis.
  • More than 1,500 mutations, or changes, in the gene responsible for cystic fibrosis have been found.
  • CCFF-funded researchers are tackling all aspects of the cystic fibrosis problem, from correcting the basic defect, to developing treatments for lung infection and inflammation.
  • Because individuals with cystic fibrosis experience a wide range of symptoms, CF research may also shed light on other diseases including asthma, HIV/AIDS, and epilepsy.
  • The current pace of cystic fibrosis research suggests it is reasonable to be optimistic about finding a cure or an effective control for cystic fibrosis.