Canadian CF Research Achievements Funded by Cystic Fibrosis Canada
- 1970s – Scientists introduce revolutionary diets which are unrestricted in fats, and make changes in pancreatic enzyme therapy, to help children with cystic fibrosis grow and gain weight.
- 1985 – Researchers map the gene responsible for cystic fibrosis to chromosome 7, helping to close in on the precise location of the gene.
- 1988 – Surgeons perform the world’s first successful double-lung transplant on a person with cystic fibrosis.
- 1989 – Researchers discover the gene responsible for cystic fibrosis.
- 1990 – Scientists reverse the CF defect under laboratory conditions by constructing a normal version of the gene responsible for cystic fibrosis and transferring it into CF-affected cells, demonstrating the potential for treating cystic fibrosis through gene therapy.
- 1990 – Researchers identify the precise cells in the body affected by cystic fibrosis and obtain the first direct evidence that the dysfunctional CFTR (cystic fibrosis transmembrane conductance regulator) protein is a chloride channel.
- 1992 – Researchers establish a new therapeutic approach to treat Pseudomonas aeruginosa bacterial infections by using alternating courses of different antibiotics.
- 1998 – Scientists replace the dysfunctional protein in cystic fibrosis, CFTR, with functional CFTR protein in a mouse model, enhancing the possibility of CF protein replacement therapy.
- 1998 – Researchers discover how to ensure the CF mouse model survives beyond weaning, through dietary changes, and prepare normal CFTR protein for possible therapeutic use.
- 2001 – Investigators complete Phase I clinical trials for dextran, a compound that seems to prevent bacteria from sticking to CF-affected lungs and promote mucus clearance; and test Prolastin®, a compound that reduces lung damage.
- 2005 – Researchers demonstrate that cationic peptides - naturally-occurring antibiotics – have the potential to reduce infection and inflammation in CF patients.
- 2007– In a mouse model, researchers develop and test an effective vaccine against the bacterium Burkholderia cenocepacia – a harmful, even deadly, bacteria in CF lungs.
- 2008 – Researchers identify two modifier genes that affect lung disease severity and the age of the first onset of Pseudomonas aeruginosa bacterial infection.
- 2008 – Investigators identify a new approach to treating “lung flare-ups” by targeting a bacterium that aggravates Pseudomonas aeruginosa infections, instead of targeting Pseudomonas aeruginosa directly.
- 2008 – Scientists screen tens of thousands of compounds and identify several that seem to restore (or compensate for) defective CFTR protein activity.
- 2009 – Researchers identify a modifier gene that may increase the likelihood of liver disease in people with cystic fibrosis.
- 2009 – Researchers discover a gene therapy technique that could double the number and improve the condition of donor lungs, dramatically enhancing health for cystic fibrosis patients who have had transplants.
- 2009 – Scientists discover that the mutated gene that causes cystic fibrosis is also responsible for muscle loss in CF patients, paving the way for novel therapies to improve the function of the diaphragm and other muscles in CF patients.
- 2010 – Scientists show under laboratory conditions that the drug glafenine, used for pain relief, can ‘rescue’ the CFTR protein allowing it to reach the cell membrane.
- 2010 – Researchers find that transient administration of an immunosuppressant drug reduces the harmful immune response in mice to viral vectors. This finding may help make gene therapy a possible treatment for CF in the near future.
- 2010 – Scientists link a decline in lung health leading to hospitalization in people with CF to the bacteria Stenotrophomonas maltophilia.
- 2010 – Researchers show that 25% of adults with CF attending clinics in Ontario are infected with transmissible strains of Pseudomonas aeruginosa. Infection with the “Liverpool” Pseudomonas strain is associated with increased risk of death or lung transplantation.
- 2010 – Researchers demonstrate that the female hormone 17beta-estradiol (E2) increases lung inflammation and suppresses immune system defences in male mice infected with Pseudomonas aeruginosa. This finding may help explain the “gender gap” in CF.
- 2010 – Scientists find an association between a mutation in the TLR5 gene and improved health indicators, such as higher body mass index (BMI), in adults with CF. This finding could lead to new treatments specifically targeting TLR5 to reduce inflammation and lung tissue damage.