6 ms·

I wouldn’t dismiss your claims, but I’d have some questions. First, have you had genetic testing to confirm that you have CF? If so, then that completely rules

I wouldn’t dismiss your claims, but I’d have some questions. First, have you had genetic testing to confirm that you have CF? If so, then that completely rules out a misdiagnoses of something presenting with a similar set of symptoms. Second I’d be curious what form of the disease you have, since that make make your experience different from someone elses’. Concerns I’d have with accepting your theory about CF as it applies to people other than yourself (I’m not doubting your own experience) is that mucus and pus are fundamentally different, and this has been studied. For example one area of study is using various drugs to break down a key component of mucous called mucins, and this is based on assays of CF mucus. You would expect pus to be composed primarily of dead tissue and immune cells, especially neutrophils. No one is going to mistake a slide of pus and a slide of mucus, and no one will mistake a spectrograph of the two either. In support of your idea is that the genetic basis for CF is known and can be replicated in tests, and the pathological mechanism involving over absorbing calcium and sodium ions is also known and reproduced. BUT... it’s also known that overproduction of mucus is not related directly to CFTR! What that means is that the observed overproduction is a secondary result of the underlying condition. That leaves room for the literature to be correct (probably) without accounting for all expressions of the disease. In other words you could be right, and so could the researchers and doctors. One thing that occurs to me is that a thicker mucus as expected with CF might still create the perception of dryness. Accumulations or blockages resulting from it would be the result of overproduction, but still lead to poor “delivery on target” so to speak. It doesn’t help if one’s vagina is effectively dry because instead of producting a slippery material, an overly viscous substance is produced. Maybe in some people the net result is a lack of mucus where it needs to be, spread around, and resulting infections. It could be that the prognosis for people with a form less associated with overproduction also leads to a better prognosis, because it’s fundamentally an easier problem to solve than other forms or expressions. It’s an interesting idea, although I’m sorry that you have to live with it.

DoreenMichele 8y ago
Thank you. First, have you have genetic testing to confirm that you have CF? Unfortunately, no. I have had two or three blood tests that indicate I do not have any of the 100 most common alleles. Last I checked, there were 1600 known alleles. My insurance denied my CF specialist's request for a more comprehensive (and expensive) test from Stanford. I was diagnosed with atypical cystic fibrosis at age 35 based on sweat chloride results and medical history. My sweat chloride results were 41, as were my oldest son's. My understanding is that historically, below 40 was normal and above 80 was CF. The numbers in between were an undefined grey zone. At some point, they decided that this grey zone was a milder form of CF. At the time that I was diagnosed, the life expectancy for CF was 36. I was diagnosed just before I turned 36. So logically I shouldn't have any of the more common alleles. Those alleles really should result in a case of classical CF and have a more severe presentation. My best understanding is that I have the same pattern of issues as classical CF, just less severe. The lessened severity combined with a late diagnosis meant I had my own mental models for what was going on with my health and allowed me to question what doctors were telling me. Because CF is so deadly, most people with a diagnosis are terrified of questioning what they are told. I stopped participating on CF lists because they aren't places you can have a meaningful discussion of the science. I've talked to a guy with a PhD in chemistry and a guy with a PhD in biology to help me understand a few things about how CF functions at the cellular level without having to deal with histrionics and hatred from people terrified of questioning the current mental models. One thing that occurs to me is that a thicker mucus as expected with CF might still create the perception of dryness. Mucus is supposed to be slippery. You can have thicker mucus from being dehydrated. Or it firms up to trap invaders. If it is thick, it is either trapping something or it's incorrectly formulated. It can't do it's job. People with CF are chronically infected. Logically, thickened mucus is a reflection of a high load of infection, at least in part. it’s also known that overproduction of mucus is not related directly to CFTR! What that means is that the observed overproduction is a secondary result of the underlying condition. I think most of what we think of as CF is really secondary, tertiary or further downstream effects. If we had a condition called Pale Skin Disorder where you were chronically sunburned to the point of being at high risk of dying from skin cancer, you would have a lot of symptoms that aren't per se inherent to having pale skin. If you are chronically infected your entire life and chronically malnourished your entire life because people don't know how to address issues with the CFTR, this will have consequences that aren't per se due to the defect in the CFTR. No one is going to mistake a slide of pus and a slide of mucus, and no one will mistake a spectrograph of the two either. No, of course not. But at what point does mucus become phlegm? How chemically deranged does mucus need to be before it gets recognized as not normal, healthy mucus? How many microbes does it need to contain to be considered some kind of drainage due to infection rather than mucus? Phlegm is not pus. But they are both a form of infected drainage. I don't have the language I want for the ideas I have, in part because I generally don't get meaningful engagement. I'm trying to make a distinction that's clear in my mind. When I'm adequately hydrated and have enough salt, etc, my mucus membranes are moist and I'm less infected. I no longer have vaginal dryness either. I spent some time waking up worried that my period had started because dry was the norm. If it was wet, I was bleeding. That fact did not prevent me from having vaginal discharge, including from frequent yeast infections. I no longer get yeast infections either.

DoreenMichele 8y ago

Thank you. First, have you have genetic testing to confirm that you have CF? Unfortunately, no. I have had two or three blood tests that indicate I do not have any of the 100 most common alleles. Last I checked, there were 1600 known alleles. My insurance denied my CF specialist's request for a more comprehensive (and expensive) test from Stanford. I was diagnosed with atypical cystic fibrosis at age 35 based on sweat chloride results and medical history. My sweat chloride results were 41, as were my oldest son's. My understanding is that historically, below 40 was normal and above 80 was CF. The numbers in between were an undefined grey zone. At some point, they decided that this grey zone was a milder form of CF. At the time that I was diagnosed, the life expectancy for CF was 36. I was diagnosed just before I turned 36. So logically I shouldn't have any of the more common alleles. Those alleles really should result in a case of classical CF and have a more severe presentation. My best understanding is that I have the same pattern of issues as classical CF, just less severe. The lessened severity combined with a late diagnosis meant I had my own mental models for what was going on with my health and allowed me to question what doctors were telling me. Because CF is so deadly, most people with a diagnosis are terrified of questioning what they are told. I stopped participating on CF lists because they aren't places you can have a meaningful discussion of the science. I've talked to a guy with a PhD in chemistry and a guy with a PhD in biology to help me understand a few things about how CF functions at the cellular level without having to deal with histrionics and hatred from people terrified of questioning the current mental models. One thing that occurs to me is that a thicker mucus as expected with CF might still create the perception of dryness. Mucus is supposed to be slippery. You can have thicker mucus from being dehydrated. Or it firms up to trap invaders. If it is thick, it is either trapping something or it's incorrectly formulated. It can't do it's job. People with CF are chronically infected. Logically, thickened mucus is a reflection of a high load of infection, at least in part. it’s also known that overproduction of mucus is not related directly to CFTR! What that means is that the observed overproduction is a secondary result of the underlying condition. I think most of what we think of as CF is really secondary, tertiary or further downstream effects. If we had a condition called Pale Skin Disorder where you were chronically sunburned to the point of being at high risk of dying from skin cancer, you would have a lot of symptoms that aren't per se inherent to having pale skin. If you are chronically infected your entire life and chronically malnourished your entire life because people don't know how to address issues with the CFTR, this will have consequences that aren't per se due to the defect in the CFTR. No one is going to mistake a slide of pus and a slide of mucus, and no one will mistake a spectrograph of the two either. No, of course not. But at what point does mucus become phlegm? How chemically deranged does mucus need to be before it gets recognized as not normal, healthy mucus? How many microbes does it need to contain to be considered some kind of drainage due to infection rather than mucus? Phlegm is not pus. But they are both a form of infected drainage. I don't have the language I want for the ideas I have, in part because I generally don't get meaningful engagement. I'm trying to make a distinction that's clear in my mind. When I'm adequately hydrated and have enough salt, etc, my mucus membranes are moist and I'm less infected. I no longer have vaginal dryness either. I spent some time waking up worried that my period had started because dry was the norm. If it was wet, I was bleeding. That fact did not prevent me from having vaginal discharge, including from frequent yeast infections. I no longer get yeast infections either.