Hey there,
From my understanding, these drugs are targeted at people with specific mutations whereas the cftr protein is at the cell surface, but not folded correctly. These drugs correct the protein, thus restoring proper function. It won`t reverse damage, but i suspect that with reduced mucus and inflammation, people should get some lung function back even if they are pretty low (as long as there isn`t tooo much scarring).
For people that have culture bugs their whole life, i don`t think you`ll get rid of them, but it should be easier to treat. Look at people who have had transplants and still have problems with the bugs they cultured before. Hopefully though with the mucus in check, the bugs won`t be much of a problem and the cycle of infection, inflammation, mucus production will lesson dramatically.
One anti infective medicine though that i have high hopes for is a nano emulsion already proven to kill most bugs that kill us, and is in phase 3 studies for other infectious diseases. Looks really promising!
<a target=_blank class=ftalternatingbarlinklarge href="http://nanotechnologytoday.blogspot.com/2009/03/nanoemulsion-potent-against-superbugs.html
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from the website:
In cell cultures in the lab, the U-M scientists tested a nanoemulsion against 150 bacterial strains that attack cystic fibrosis patients. The emulsion proved effective at killing all of them, including one-third that are resistant to many antibiotics and 13 percent that resist all antibiotics.
They then tested the nanoemulsion against several bacterial strains grown in biofilms and sputum, to more closely simulate conditions in a patient's body. Antibiotics often can't penetrate biofilms and sputum unless given at high doses with unacceptable side effects.
"We saw, not surprisingly, that greater concentrations of nanoemulsion were required to kill the bacteria, but we saw no strains that were resistant," LiPuma says. Whether humans can tolerate those concentrations well remains to be seen.
LiPuma's lab, funded by the Cystic Fibrosis Foundation as a national reference lab, has collected more than 30,000 strains of bacteria from the lungs of cystic fibrosis patients. The lab receives samples from around the world for analysis.
What's next
The University of Michigan has filed for patent protection on the CF nanoemulsion, and licensed this technology to Ann Arbor-based NanoBio Corporation. Baker is a founder and equity holder of NanoBio. NanoBio and LiPuma's lab will cooperate in the next steps toward bringing the treatment to market. LiPuma is optimistic that if animal and human trials go well, a nanoemulsion treatment for cystic fibrosis infections could be available in as little as five years. ##
Hope that helps a bit
Kiel