"Another problem with neutrophilic inflammation in CF is that although the NTs are called in to kill pathogens, they don't actually do a very good job because they are lacking in the antioxidant glutathione - the number one most important tool that NTs use to kill pathogens. It is thought that CF cells have a hard time secreting glutathione into intercellular space because of the faulty CFTR channel, so NTs have less access to this important antioxidant. Several studies have looked into reducing systemic inflammation by boosting NT glutathione levels with NAC supplementation. N-acetylcystine (NAC) is a molecule necessary for the synthesis of glutathione in the body, otherwise called a glutathione precursor. Direct supplementation with glutathione is largely ineffective because it is easily oxidized by environmental factors. For years NAC has been used to boost glutathione in the lungs of CF patients by inhaling it in a prescription called "Mucomyst". This was effective, but went out of fashion as pulmozyme came along, plus I've heard that mucomyst wasn't so popular because it had a rotten egg smell when aerosolized. Now there is more research suggesting that oral supplementation with NAC may also be effective in boosting NT glutathione in the body as a whole and in the lungs in particular, making our immune system more effective at clearing infection. In a preliminary study, oral NAC supplementationsignificantly boosted NT glutathione and blood glutathione in the whole body, significantly reduced the number of NTs in the airways, significantly reduced IL-8 (an inflammatory marker) in the airways, and significantly reduced NT elastase in the sputum [25]. This is a big deal! This means that instead of dealing with systemic inflammation through palliative treatments like steroids, high-dose ibuprofen, and azithromycin, NAC supplementation targets the root cause of the problem with a completely harmless treatment that has virtually no side-effects and no known level of toxicity! A larger clinical study has just been concluded on the effect of NAC on CF lungs, and I look forward to exploring the results when they're published. The standard dose for oral NAC is 600 mg three times a day, and you can ask your doctor for a prescription for it. It is used in modern medicine to treat tylenol poisoning and interstitial lung disease, so your doc should be able to get your insurance company to cover it no problem. If not, you can also get it over the counter. There's a lot of interest in the CF community in a new over the counter form of it called PharmaNAC. "
source: http://cfnaturalhealth.weebly.com/blog/previous/3
Inhaled NAC:
http://www.ncbi.nlm.nih.gov/pubmed/10090545
