70,000
people with CF worldwide, a lifespan depending of the resources (in USA the
lifespan it’s about 40 years old, while in Chile the average lifespan it’s
about 18 years old)
Scene from Patient Assistance Resource Center video (Youtube). Cystic Fibrosis Foundation.
It’s a disease caused by the expression of a deficient transmembrane protein called CFTR. This affects different kind of cells, mainly the epithelial cells from the airway and pancreas, causing the production of a defective mucus.
In the lungs, this defective mucus leads that several opportunistic pathogens enter to the lower airway and initiate an infection, with a consecutively progressive lung dysfunction.
One of the
main pathogens found in an infection of the lungs are the Pseudomonas
aeruginosa. When it colonize the airway, initiates the production of biofilm, a
barrier that protect it of a lot of antimicrobial substances and gave best environment
for it develop.
The actual treatments for controlling the symptoms
consist in maintain controlled the pathogens, clear the airway of the sticky
mucus, administrate complementary enzymes and regulate the function of the CFTR
channel.
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