Bronchiectasis and Inflammation
CF is described as a classic example of chronic inflammatory disease. Bronchiectasis, the main constituent of CF lung disease, is the result of out-of-control inflammation. It is described as the over-dilation of our airways to the point where they lose their elasticity and scarify. This causes the airways to become fragile and increases the risk of them collapsing or breaking, which can lead to hemoptysis (bleeding of the bronchial arteries into the airways leading to coughing up of blood). Our airways over-dilate in part because they are filled with mucus, and in order to move any air through a clogged up airway, they must widen themselves. But bronchiectasis is also caused by neutrophilic inflammation and the airway scarification and restructuring that results.
The inflammatory processes that cause bronchiectasis are complicated and not fully understood, but much of the problem has to do with over-recruitment of neutrophils into the airways. Neutrophils are white blood cells that kill pathogens and help clean up infection. Under normal circumstances, these guys are wonderful and target infection quickly, clean it up, then move on. But in situations of chronic infection, neutrophils (NTs) never really move on. In CF the situation is even more complex. It turns out that CF lungs have higher than normal levels of NT elastase (an enzyme that NTs use to kill pathogens and break down tissue) even without pathogenic colonization [23,24]. We don't know why this is for sure, but I postulate that one possibility could be autoimmunity. In fact, cases of non-CF bronchiectasis in older Americans are increasing at a rate of about 8.7% per year, and it is often diagnosed in patients with autoimmune diseases, especially rheumatoid arthritis . Regardless, neutrophils and the inflammatory mediators that they secrete (e.g. NT elastase, IL-8) cause further neutrophil recruitment, tissue damage, and airway remodeling (i.e. bronchiectasis) . It has been found that the greater the level of NT elastase in CF sputum, the lower a person's FEV1 will be . Not only does NT elastase break down the elastin, collagen, and protoeoglycans in the tissue of our airways, but it also makes our mucus thicker and harder to expel. This is a problem because thick mucus provides an even better home for pathogens to breed, plus it makes it harder for the NTs to move around in the airways, so they get stuck, die, and cause mucus pooling, which causes the airways to widen even more to get around the mucus pooling, causing further bronchiectasis. Pulmozyme (DNAase) works by breaking apart neutrophil elastase in the airways, making the mucus thinner and easier to cough out.
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 . 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.
Given all this, I have decided to focus on improving my lung symptoms by targeting inflammation as its main cause. I have struggled with bouts of significant hemoptysis in the last several years, and had my first pulmonary embolism in May. I have noticed that I get hemoptysis when I am particularly inflamed, caused either by a bad lung infection, inflammatory foods, and/or environmental irritants (e.g. campfire smoke or heavy pollen). Thus, in addition to adhering to the GAPS diet, I got my doc to give me a prescription for NAC, and we'll see if this improves things. In addition, to treat systemic inflammation one must consider the role of gut inflammation, the cause of most inflammatory disease. Targeting the root cause of my systemic inflammation by focusing on the gut through improving my diet and cultivating a more balanced intestinal bacteria community has yielded very positive results so far, and I'm only in the very beginning of this treatment regime, so more progress is sure to come.
Your Gut is Your Immune System
One way that your gut acts as part of your immune system is that the latter part of your small intestine, called the ileum, is lined with tissue called lymph nodes which kill and remove bacteria, viruses, fungi, and dead cells from the lymph (the fluid that circulates through the lymphatic system, exists in the spaces between organs, and interacts with the blood). In this way your ileum is a direct part of the immune system, and its ability to remove and kill pathogens in the interstitial space and the blood is critical to our health. When an infection is detected, the lymph nodes create lymphocytes which kill the pathogens, causing pain and inflammation in the lymph nodes. This means that if the lymph nodes in the gut are chronically exposed to toxins and pathogens, or if they have trapped pathogens within the nodes but can't clear them out, the ileum can become inflamed and painful causing serious digestive upset, reduced absorption capacity, and compromised immunity .