Another 27 Milligrams
08/05/10 18:38
As promised, I am going to tell you the significance of the title of my last post. Twenty-seven milligrams—what does it mean?
First of all, let me digress a little bit and tell you about our experiences with oral benzyl isothiocyanate—
To start out, let me tell you that, as far as I know, everyone who took this dietary supplement took it under the care and supervision of their personal physicians. It is not the purpose of Sharktank, or myself, to undertake the medical care of CF patients. If a patient wants to change their treatment regimen, that’s their and their physician’s business. But, the fact is that they do it, and they often fill us in on the details.
When Keith was in the hospital, I often found myself out on a back step of some door of the hospital that no one ever went into, and that was always locked, smoking cigarettes, and thinking about the science of CF. I knew that my son was so damaged by the disease that his only hope of surviving was going to be as if some angel came down from the sky and passed a wand over him (mixing my metaphors a bit here, I admit) and said, “Poof! You no longer have cystic fibrosis.” That’s what I was searching for, you see; something to stop the ravages of the disease and let him heal.
Well, of course, we all know, now, that that didn’t happen, but we must ask ourselves what would the outcome be, if it had happened? Would he, all of a sudden, have been a normal kid, rising up out of his sickbed, and running through the nights, partying with his friends?
Of course not. Even if the “Poof!” magical wand had passed over him, he would have still been damaged. His lungs, especially, and of course, his pancreas, to a degree.
Let us talk about the pancreas, first, and then we’ll get to the lungs. The pancreas has two types of functions: endocrine and exocrine. Both are affected in CF, to varying degrees, but it is the exocrine function that we will discuss first. Anyone who takes pancreatic enzymes knows that the purpose of these is to replace the fat metabolizing enzymes that the CF pancreas cannot excrete. The longer you have the disease, the more enzymes you have to take to digest the fat in your diet, because the pancreatic cells that perform the exocrine function are slowly being destroyed. So, if you have cystic fibrosis and you get the “Poof!” wand passed over you, you’re still going to need to take pancreatic enzymes, depending on how many of those cells have been destroyed. It largely depends on how old you are, as to how much of the exocrine function has been destroyed. You will get some back, because the body has a way of healing itself. But never all of it. And, certainly, no matter how much you get back, it is dependent on the time you spend healing. And, of course, healing can only begin to take effect once the conditions causing the damage are stopped. You gotta have the “Poof!” first.
Now, the endocrine function of the pancreas—if you have CF and CF-related diabetes, you’re familiar with this function. You can’t damage one kind of cell in the pancreas, without also damaging others. When enough of the cells responsible for the endocrine function of the pancreas are damaged, they stop producing insulin (and glucagon, too, but that’s a subject for another day). I suspect that the lack of insulin production by the pancreatic B cells has more to do with the nerve cells than it does with the B cells, themselves, because certain drug treatments have been known to restore insulin expression in these tissues, and bariatric surgery has been shown to be curative, in many cases of non-insulin dependent diabetes, but again, that’s the subject for another day. The reason that I mention this, though, is because I suspect, as well, that at least some insulin expression might be restored, if the “Poof!” can take place in CF patients. I have concrete reasons to suspect this, but they are the subject of another paper, that I will write in the future, so I won’t talk about them here.
And, now, to the lungs. Obviously, as we’ve all been told, time and time again, once you do damage to the lungs, it’s done. There’s no going back. And cystic fibrosis is a disease of the lower airways—the alveoli, which you can picture as little machines for oxygen exchange—are damaged beyond repair.
But, if you were to take the alveoli out and stretch them on a flat surface, you’ve have enough to cover at least one tennis court. Lots to work with there. And where there’s life, there’s hope. The human body has enormous restorative and compensatory abilities. If you’re lucky enough to experience the “Poof!” and you’re still alive and functioning, you’re going to undergo a healing process and get at least some of the function back. What you don’t get back, you’ll compensate for. Depending on how long you’ve been sick and the damage done, though, you’ll never get it all back. I didn’t see my son running marathon races, if that angel with the wand had come down, but what I did envision was him coughing up all that gunk that was in his lungs.
And that’s exactly what happened when CF patients took oral benzyl isothiocyanate. Their enzyme intake had to be radically lowered. The ones that had CF-related diabetes began experiencing normal blood glucose levels. And they coughed up some really old crap from their lungs (they described it, variously, as a “flushing out” and as a “flushing of the toilet.” Heh.). They got their “Poof!” It lasted a couple of weeks, and then it stopped. The reason that it stopped is the same reason that azithromycin and colchicine, administered orally, don’t do the complete job of inducing a functionally redundant protein in CF patients—the liver proteins, and particularly, MRP2, filters these compounds out of the bloodstream, before they can induce these same proteins in the lungs.
So, what does “27 milligrams” have to do with anything here? Twenty seven milligrams is the amount of benzyl isothiocyanate that caused the “Poof!” in an 80kg CF patient, when administered transdermally. And it continues to do so, even after more than three months. Why doesn’t it stop, like the oral benzyl isothiocyanate stopped? Because it bypasses first pass hepatic metabolism and goes directly into the bloodstream.
Poof!
First of all, let me digress a little bit and tell you about our experiences with oral benzyl isothiocyanate—
To start out, let me tell you that, as far as I know, everyone who took this dietary supplement took it under the care and supervision of their personal physicians. It is not the purpose of Sharktank, or myself, to undertake the medical care of CF patients. If a patient wants to change their treatment regimen, that’s their and their physician’s business. But, the fact is that they do it, and they often fill us in on the details.
When Keith was in the hospital, I often found myself out on a back step of some door of the hospital that no one ever went into, and that was always locked, smoking cigarettes, and thinking about the science of CF. I knew that my son was so damaged by the disease that his only hope of surviving was going to be as if some angel came down from the sky and passed a wand over him (mixing my metaphors a bit here, I admit) and said, “Poof! You no longer have cystic fibrosis.” That’s what I was searching for, you see; something to stop the ravages of the disease and let him heal.
Well, of course, we all know, now, that that didn’t happen, but we must ask ourselves what would the outcome be, if it had happened? Would he, all of a sudden, have been a normal kid, rising up out of his sickbed, and running through the nights, partying with his friends?
Of course not. Even if the “Poof!” magical wand had passed over him, he would have still been damaged. His lungs, especially, and of course, his pancreas, to a degree.
Let us talk about the pancreas, first, and then we’ll get to the lungs. The pancreas has two types of functions: endocrine and exocrine. Both are affected in CF, to varying degrees, but it is the exocrine function that we will discuss first. Anyone who takes pancreatic enzymes knows that the purpose of these is to replace the fat metabolizing enzymes that the CF pancreas cannot excrete. The longer you have the disease, the more enzymes you have to take to digest the fat in your diet, because the pancreatic cells that perform the exocrine function are slowly being destroyed. So, if you have cystic fibrosis and you get the “Poof!” wand passed over you, you’re still going to need to take pancreatic enzymes, depending on how many of those cells have been destroyed. It largely depends on how old you are, as to how much of the exocrine function has been destroyed. You will get some back, because the body has a way of healing itself. But never all of it. And, certainly, no matter how much you get back, it is dependent on the time you spend healing. And, of course, healing can only begin to take effect once the conditions causing the damage are stopped. You gotta have the “Poof!” first.
Now, the endocrine function of the pancreas—if you have CF and CF-related diabetes, you’re familiar with this function. You can’t damage one kind of cell in the pancreas, without also damaging others. When enough of the cells responsible for the endocrine function of the pancreas are damaged, they stop producing insulin (and glucagon, too, but that’s a subject for another day). I suspect that the lack of insulin production by the pancreatic B cells has more to do with the nerve cells than it does with the B cells, themselves, because certain drug treatments have been known to restore insulin expression in these tissues, and bariatric surgery has been shown to be curative, in many cases of non-insulin dependent diabetes, but again, that’s the subject for another day. The reason that I mention this, though, is because I suspect, as well, that at least some insulin expression might be restored, if the “Poof!” can take place in CF patients. I have concrete reasons to suspect this, but they are the subject of another paper, that I will write in the future, so I won’t talk about them here.
And, now, to the lungs. Obviously, as we’ve all been told, time and time again, once you do damage to the lungs, it’s done. There’s no going back. And cystic fibrosis is a disease of the lower airways—the alveoli, which you can picture as little machines for oxygen exchange—are damaged beyond repair.
But, if you were to take the alveoli out and stretch them on a flat surface, you’ve have enough to cover at least one tennis court. Lots to work with there. And where there’s life, there’s hope. The human body has enormous restorative and compensatory abilities. If you’re lucky enough to experience the “Poof!” and you’re still alive and functioning, you’re going to undergo a healing process and get at least some of the function back. What you don’t get back, you’ll compensate for. Depending on how long you’ve been sick and the damage done, though, you’ll never get it all back. I didn’t see my son running marathon races, if that angel with the wand had come down, but what I did envision was him coughing up all that gunk that was in his lungs.
And that’s exactly what happened when CF patients took oral benzyl isothiocyanate. Their enzyme intake had to be radically lowered. The ones that had CF-related diabetes began experiencing normal blood glucose levels. And they coughed up some really old crap from their lungs (they described it, variously, as a “flushing out” and as a “flushing of the toilet.” Heh.). They got their “Poof!” It lasted a couple of weeks, and then it stopped. The reason that it stopped is the same reason that azithromycin and colchicine, administered orally, don’t do the complete job of inducing a functionally redundant protein in CF patients—the liver proteins, and particularly, MRP2, filters these compounds out of the bloodstream, before they can induce these same proteins in the lungs.
So, what does “27 milligrams” have to do with anything here? Twenty seven milligrams is the amount of benzyl isothiocyanate that caused the “Poof!” in an 80kg CF patient, when administered transdermally. And it continues to do so, even after more than three months. Why doesn’t it stop, like the oral benzyl isothiocyanate stopped? Because it bypasses first pass hepatic metabolism and goes directly into the bloodstream.
Poof!