Carcinoid tumors are odd birds. They are of a group of cells in your body that are known as the neuroendocrine system. Neuroendocrine cells receive some sort of signal from your nervous signal and, in response, they release some sort of hormone into your blood system. Those hormones then stimulate another part of your body to perform some task.
For example, if someone sneaks up behind you in the parking lot and screams loudly, your adrenaline gland releases a huge amount of adrenaline into your blood stream, enabling the fight or flight mechanism. Insulin is another example. If you consume a large amount of sugar, your pancreas releases insulin to bring help bring the level of sugar down in your blood to a manageable level.
The neuroendocrine cells from which carcinoid tumors grow are cells that generate serotonin. Serotonin serves several purposes in body but one of the primary jobs is to regulate intestinal movements. The tumors don't forget their previous life as neuroendocrine cells but now there are a much greater number of these cells. What that means is that they still attempt to respond to the signals to regulate intestinal movements.
This results in things getting out of balance. The tumors will get the signal that food has entered the digestive tract and then decide to start telling the rest of the digestive tract to begin processing the food. Normally, this is just fine but with all the extra cells, way too much serotonin gets released and the digestive system goes into overdrive, leading to, often, explosive diarrhea.
In the same vein, kallikrene (yet another hormone but an odd one) is released at times which lead to the blood vessels increasing in size. This affect people notice is that the face and chest will often turn quite red and there is often a feeling of light-headedness that accompanies this along with a drop in blood pressure. I can usually cause this to happen by walking up 5 flights of stairs and then watch my face turn red 45 seconds after reaching the 5th floor.
Carcinoid crisis is when this happens to the extreme and it usually happens at one of the single most scary times possible. General anesthesia is the most complicated portion of a surgery and it is one of the single biggest causes of carcinoid crisis. Under general anesthesia, my entire upper body will turn almost purple, my heart rate drops and my blood pressure hits the floor.
I typically have a fairly low blood pressure (~110/75) but the last time crisis occurred during surgery, I dropped to 75/40 and lower. Heart rate (usually around 65 for me) dropped into the 40s and I was standing at the precipice of death. For this reason, I always try to avoid general anesthesia for any procedures and it appears that low levels of propofol are usually sufficient for the procedures that I have been receiving lately.
Sometime, I will need general anesthesia and we have a plan for that as well. Sandostatin is a drug that seems to inhibit carcinoid syndrome and is generally given in small doses. Whenever I do need a major surgery, I will be admitted the night before and then put on a continual high-dose drip of sandostatin. Just prior to anesthesia being given, I will receive another bolus shot of sandostatin and then the drip will continue throughout the procedure. This worked for me in my second major surgery of 2012 and, if I have to go under again, we hope it will work again.
If I still go into crisis, the key is to then just start giving massive doses of sandostatin and hang with me because I have always come out of it. It takes as long as an hour but as long as my heart continues to pump, I will survive the crisis because the body will simply run out of kallikrene. Whenever I do go in for procedures, even with propofol, I still try my best to scare the anesthesiologist as much as possible and ensure that they have sufficient sandostatin ON HAND in the operating room.
Sunday, May 25, 2014
Sunday, May 18, 2014
B is for Bowels
Carcinoid Cancer is frequently all about the bowels. Most carcinoids are detected in the bowels with about 40% being found (like mine), in the terminal illium (end of the small intestine). Many are found in the rectum, some are in the pancreas and a surprising number of appendectomies are actually caused by the seeds of a carcinoid tumor.
Once the cancer starts, it grows very slowly, sometimes for ten or twenty years before it actually begins to cause any side affects and I have no idea how long mine had been hidden. What I do know is that it was found in 2001 due to a complete small bowel obstruction and had grown to 1.7cm at that time which about twice the size at which they generally begin to metastasize. My best guess is that the cancer started growing about the same time I started grad school in 1990 and has been growing very slowly since that point.
Once the cancer metastasizes, it usually stays in the abdomen, preferring to affect any nearby tissues. It frequently goes to the liver, and often ends up clustering itself around the mesenteric lymph nodes which is exactly the course my cancer took. When it metastasizes, that is when it actually begins to grow much faster and bigger. No one really knows why (yet) but it may be that the soil where the cancer first sprouts is not very fertile and the places it goes to are much more accommodating. This is why I have had 3-4cm tumors throughout my abdomen, even though the primary was quite small and likely would have stayed that way.
Another reason B is for Bowels is the diarrhea that carcinoid syndrome brings with it. The tumors act like the neuro-endocrine cells from which they were formed and generate all the signals that those types of cells generate. However, there are now so many more of them and they often act in concert, flooding your body with signals such as "Open the blood vessels" or "Empty the Bowels NOW" and in such volume that your body behaves oddly. These actions can be somewhat controlled by some drugs such as Sandostatin but there is only so much you can do.
Finally, B is for Bowels because the tumors regularly cause obstruction and ischemia in the bowels. For me, this has happened four times so far. The tumors have bound up the small and large intestines to the point that nothing can pass and this has lead to dramatic weight loss. The tumors have further impinged on the superior mesenteric vein, preventing blood from getting to the intestines so that they can do their work of digestion.
Of course these last two reasons mean I am continually worried about my bowels. If I am having too much diarrhea, I am frustrated at the bathroom time required and the interruptions to normal life. If I am not having much diarrhea, I begin to worry that I am starting to obstruct again and that I will be back on the OR table for another extended laparotomy. Right now, I am in the later camp and am stressed that things are starting to obstruct and don't know how I will deal with yet another 12hrs of surgery and 8 weeks of recovery.
Bowel obstruction is difficult to diagnose in some respects. There are some standard symptoms but those symptoms could be any of a number of other things as well.
Of course, once it does obstruct, what do you do? Usually it requires some fairly urgent surgery but there aren't many surgeons willing to go into an abdomen with as many adhesions as I have. If I wait too long, I get weak and surviving/recovery from surgery will be all the more strenuous. And, of course, there is the voice in the back of your head saying that maybe if you ignore it, it will just go away.
October is 13 years since my first laprarotomy and I have had them again in 10/2003, 1/2010, 6/2012, and 11/2012. I would really like to get another year or more before having a surgery that takes me out for months.
Once the cancer starts, it grows very slowly, sometimes for ten or twenty years before it actually begins to cause any side affects and I have no idea how long mine had been hidden. What I do know is that it was found in 2001 due to a complete small bowel obstruction and had grown to 1.7cm at that time which about twice the size at which they generally begin to metastasize. My best guess is that the cancer started growing about the same time I started grad school in 1990 and has been growing very slowly since that point.
Once the cancer metastasizes, it usually stays in the abdomen, preferring to affect any nearby tissues. It frequently goes to the liver, and often ends up clustering itself around the mesenteric lymph nodes which is exactly the course my cancer took. When it metastasizes, that is when it actually begins to grow much faster and bigger. No one really knows why (yet) but it may be that the soil where the cancer first sprouts is not very fertile and the places it goes to are much more accommodating. This is why I have had 3-4cm tumors throughout my abdomen, even though the primary was quite small and likely would have stayed that way.
Another reason B is for Bowels is the diarrhea that carcinoid syndrome brings with it. The tumors act like the neuro-endocrine cells from which they were formed and generate all the signals that those types of cells generate. However, there are now so many more of them and they often act in concert, flooding your body with signals such as "Open the blood vessels" or "Empty the Bowels NOW" and in such volume that your body behaves oddly. These actions can be somewhat controlled by some drugs such as Sandostatin but there is only so much you can do.
Finally, B is for Bowels because the tumors regularly cause obstruction and ischemia in the bowels. For me, this has happened four times so far. The tumors have bound up the small and large intestines to the point that nothing can pass and this has lead to dramatic weight loss. The tumors have further impinged on the superior mesenteric vein, preventing blood from getting to the intestines so that they can do their work of digestion.
Of course these last two reasons mean I am continually worried about my bowels. If I am having too much diarrhea, I am frustrated at the bathroom time required and the interruptions to normal life. If I am not having much diarrhea, I begin to worry that I am starting to obstruct again and that I will be back on the OR table for another extended laparotomy. Right now, I am in the later camp and am stressed that things are starting to obstruct and don't know how I will deal with yet another 12hrs of surgery and 8 weeks of recovery.
Bowel obstruction is difficult to diagnose in some respects. There are some standard symptoms but those symptoms could be any of a number of other things as well.
- You will generally have a loss of appetite (Hmmm, that could be my kidney failure).
- You will have abdominal pain (have had that for 13 years from the diarrhea and other).
- You will sometimes throw up indigested food (not having that currently but it sounds like just being sick).
- CT scans work well as long as you can use contrast (can't due to my kidney failure).
Of course, once it does obstruct, what do you do? Usually it requires some fairly urgent surgery but there aren't many surgeons willing to go into an abdomen with as many adhesions as I have. If I wait too long, I get weak and surviving/recovery from surgery will be all the more strenuous. And, of course, there is the voice in the back of your head saying that maybe if you ignore it, it will just go away.
October is 13 years since my first laprarotomy and I have had them again in 10/2003, 1/2010, 6/2012, and 11/2012. I would really like to get another year or more before having a surgery that takes me out for months.
Sunday, May 11, 2014
My ABCs of Cancer: A is for anxiety
Madhulika Sikka wrote a book on the ABCs of breast cancer. Stephanie has encouraged me to write my own ABCs of cancer. If nothing else, it gives me topics for quite a while if I am able to keep up the writing.
So, let's start off with 'A is for Anxiety'. Having or having had cancer puts one in a continual state of anxiety and your life is never the same again. You are continually ruled by the latest lab reports, scans, or blood tests. Your life comes to a complete halt in those days before the test and in the days afterwards as you wait for the results.
I have several friends who have been diagnosed with prostate cancer and their big test is the PSA test. Men begin getting this test later in life and it almost always comes back normal (less than 4) but for all too many people, it crosses that magic line and their life with cancer begins. The funny thing about these tests is that they are not precise. Someone can have a PSA of 3.5 and be in full blown metestatic disease while someone else has a PSA of 6 with only the early stages of cancer. You just never know.
Of course, that is where the major anxiety kicks in. You have the cancer removed by some means and then you watch the PSA level and it hopefully begins to drop. You get more and more confidence as that number continues to drop but your stress level goes through the roof when the time for your next blood test comes around.
My cancer has an extra annoyance that comes with it. One of the standard markers is the Chromogranin-a and there is not a consensus amongst labs about how to perform it. Some labs have a 'good' range of 0-35 with their marker while other labs have 0-5. Furthermore, this blood test is thrown off by commonly used protein pump inhibitors (Nexium, Protonix, Prevacid) that are prescribed for acid reflux and similar disorders.
Other markers are becoming available for me that are less variable and reduce the anxiety some but it is never gone. Even for those friends who have dealt with their cancers and reached the magic 5 year mark, you never know for sure. Another friend passed her 4 year mark in the clear but then the 5 year test showed that she is not through the storm. That is Anxiety with a capitol A.
I know everyone worries about mortal illnesses from time to time but when you have or have had cancer, this worry coms with extra intensity. You almost become a hypochondriac. Every twinge, headache, cramp, upset stomach, or cold makes you worry that the cancer is attacking with a vengeance. It takes days to get a couple of days off of the cancer rollercoaster just to get back in line for the next ride.
I have been on that ride for 12.5 years now and it seems to never stop. The good thing about it is that as long as I am on the ride, I am alive and able to enjoy the view some. There is a thrill to the minor successes when you get off the ride for a while and get to wander around the amusement park, taking in the sunshine and the smells. Something about surviving that last ride can make everything else just a little bit brighter.
So, let's start off with 'A is for Anxiety'. Having or having had cancer puts one in a continual state of anxiety and your life is never the same again. You are continually ruled by the latest lab reports, scans, or blood tests. Your life comes to a complete halt in those days before the test and in the days afterwards as you wait for the results.
I have several friends who have been diagnosed with prostate cancer and their big test is the PSA test. Men begin getting this test later in life and it almost always comes back normal (less than 4) but for all too many people, it crosses that magic line and their life with cancer begins. The funny thing about these tests is that they are not precise. Someone can have a PSA of 3.5 and be in full blown metestatic disease while someone else has a PSA of 6 with only the early stages of cancer. You just never know.
Of course, that is where the major anxiety kicks in. You have the cancer removed by some means and then you watch the PSA level and it hopefully begins to drop. You get more and more confidence as that number continues to drop but your stress level goes through the roof when the time for your next blood test comes around.
My cancer has an extra annoyance that comes with it. One of the standard markers is the Chromogranin-a and there is not a consensus amongst labs about how to perform it. Some labs have a 'good' range of 0-35 with their marker while other labs have 0-5. Furthermore, this blood test is thrown off by commonly used protein pump inhibitors (Nexium, Protonix, Prevacid) that are prescribed for acid reflux and similar disorders.
Other markers are becoming available for me that are less variable and reduce the anxiety some but it is never gone. Even for those friends who have dealt with their cancers and reached the magic 5 year mark, you never know for sure. Another friend passed her 4 year mark in the clear but then the 5 year test showed that she is not through the storm. That is Anxiety with a capitol A.
I know everyone worries about mortal illnesses from time to time but when you have or have had cancer, this worry coms with extra intensity. You almost become a hypochondriac. Every twinge, headache, cramp, upset stomach, or cold makes you worry that the cancer is attacking with a vengeance. It takes days to get a couple of days off of the cancer rollercoaster just to get back in line for the next ride.
I have been on that ride for 12.5 years now and it seems to never stop. The good thing about it is that as long as I am on the ride, I am alive and able to enjoy the view some. There is a thrill to the minor successes when you get off the ride for a while and get to wander around the amusement park, taking in the sunshine and the smells. Something about surviving that last ride can make everything else just a little bit brighter.
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