Having told the kids our bad news, we then had to tell everybody else. I emailed a summary to everybody we could think of that we had an email address for, and of course talked to people that asked. We got pretty good at talking about it. We gave people the impression that we were "strong", but mostly we were just becoming practiced, whereas the poor folks we were talking to were struggling through unfamiliar territory.
I seemed to be recovering from the hospital stay. I was walking several miles a day, and working with some light weights. Didn't have much intellectual ambition though. I was able to do some computer and paper work.
Come the weekend, I sort of crashed. Mom & Dad were here for Easter. Saturday morning we deocorated Easter eggs (something of a production at our house). I walked four miles. But in the afternoon I lay on the couch and barely moved. I moved to the air mattress in the basement that evening (a little more comfortable with a distended stomach). Patti came down and asked if I wanted to help hide Easter eggs. I told her I was sorry, but could she just do it without me? She choked up a bit, and said that it was OK, she was going to have to get used to doing things without me.
On Easter, after the egg hunt, we went to Church. I managed to sing up front, but I was pretty wobbly. The service seemed to last forever. I went back to eating wheat (yumm!), in preparation for my upcoming celiac disease biopsy. I had been wheat free for well over a month, and I would have to damage my intestinal lining all over again in order for the test to show conclusively that I had the reaction.
Whether it was the wheat, the quantity of food, or some obscure cycle in my body's rhythms, I was pretty much a wreck that day. I mostly lay around in glassy eyed pain. Patti was sure I was in a downward spiral that I wasn't coming out of. She cried on my shoulder that night that it wasn't fair I was being taken so soon.
Fortunately, my demise was not yet imminent. I was rather better in the days that followed. We had an appointment on Tuesday in Cleveland with the hepatologist, that cheered Patti up immensely. He didn't really give us any better news, but at least he did not give us the impression that he had written us off as history. He said I was strong, these tumors often (but not always) grow very slowly. My liver functions are nearly normal, aside from my high bilirubin count which he was still willing to dismiss as Gilbert's syndrome. We had a list of questions for him.
How long have I got? Still didn't get a straight answer on this one. Not a really fair question for him I suppose. He's a liver guy, not a cancer specialist. But he did say he is involved with one case where the guy is still working full time ten years after diagnosis. Where I fit on that fellow's time line he could not say, nor could he say whether that was typical.
What percent of my liver is now tumor? Again, he couldn't say. There were a half dozen or so tumors visible on cat scan, one being quite large, but there were also an unknown quantity of micro-tumors in the "normal" liver tissue. He could not bring the CAT scan pictures up to view on the terminal in his office, and the radiologist had not recorded a written estimate of that figure.
What will happen to me if my portal hypertension gets worse? Nothing. Once the hospital has "banded" all the varicose veins in my esophagus to prevent them from bursting, there should be no additional worrisome symptoms from the hypertension.
What will be the manner of my death? Assuming death comes from liver failure, and not a metastasis in some other organ, I will go into a coma and slip away.
Is it OK to drink wine? Now THERE's a question to give a liver guy vapor lock. He seized up pretty good and said he would not add any stress to my liver. Then he grudgingly conceded that perhaps half a glass every couple weeks might be OK. Rather like saying you may drive your Porsche forty fathoms per fortnight.
Is Viagra OK? This made Patti turn red, but hey - ask the guy who knows while the asking is good. I could well imagine THAT could burst a vessel or two. But he said Viagra was fine, though he didn't expect the portal hypertension or liver stress to be a direct cause of any sexual difficulties.
Can the portal hypertension cause vision problems? No, though fluid imbalance might. Whatever that means.
What analgesics should I use for headaches or fever? Any of the usual, but only within the recommended dosages. Limit Tylenol to 3 grams per day.
What about stronger pain killers? Again, the usual prescription narcotics. Percoset, morphine, demerol.
What is causing my belly pain after eating? Unknown! Apparently neither the portal hypertension nor the liver stress would be expected to cause it. The hormones put out by the tumor might, but the usual suspect there would be gastrin. To cause pain, gastrin would have to first cause ulcers. I had no stomach or duodenal ulcers visible during my endoscopy.
Is there any point in having the blood flow study CAT scan that was scheduled in two days? Yes. I apparently misunderstood its purpose. It would tell me if I was elgible for chemo-embolization of the large tumor, which would definitely be a good thing. In chemo-embolization, they pop some chemo drugs up into the branch of the hepatic artery that feeds the tumor, to clot the artery and starve the tumor of blood. This should only be done if there is enough additional blood flow to the surrounding liver tissue from the portal vein to keep it alive while the tumor is dying.
If we eliminated the original source tumor, could I be eligible for a liver transplant? No. A transplant would shorten my life, not lengthen it. The immuno-suppression drugs administered after the transplant would allow the micro-tumors scattered throughout my body to bloom like wild flowers on a spring day.
As I said, for some reason this visit cheered Patti up. Two days later I had more procedures. First was the endoscopy. They went in to check the banding of the varices. More had popped up. This time they banded four. Last time it was three. I don't care for this trend. They also took a biopsy from the duodenum to test for celiac disease.
I should have tried harder to divine the purpose of the doctor's pre-op questions. "Do you have difficulty with post operative pain?". I considered for a moment, then said "I hurt worse than most people, but complain less." In retrospect I should have said, "Oh my yes! It's awful! Can you by any chance prescribe me some percoset for this evening?". The last time I had this procedure done, I was not yet eating. Also, the hospital did give me pain killers to get me through the night. For the day following banding, swallowing feels like dropping a hedgehog down a ladder. It bounces painfully off each rung on the way down.
I also had a special CAT scan to image blood flow through my liver. I could not eat or drink before either test. I had not been drinking much anyway, so my last urine early that morning was the color of dark tea. I was awfully uncomfortable after the endoscopy. In the radiology waiting room, they gave me a quart of Crystal Lite lemonade containing God knows what. Then I waited. And waited. I fully expected my bladder to be screaming at me, but in fact I absorbed the fluid like a sponge. I did however, have a splitting headache. All that sugar (or does Crystal Lite use artificial sweetener?) had come into my system and there was not enough water to move it anywhere. When I was finally ushered to the actual CAT scan area, they asked me repeatedly whether I was diabetic. Seems like something they should have asked before having you chug all that lemonade.
The scan itself was quick and painless, and we finally got out of the hospital. Patti repeatedly tells me that I need to drink twelve glasses of water a day. I have always dismissed this as an old wives tale. The rumor is that it originated as military guidelines for troops doing heavy labor in high altitude deserts. But there must be a reason some of these wives live to be old. It took me an afternoon and evening of steady water drinking to get my pee back to a nice pale yellow color. I felt much better in many ways, though I still yearned for a percoset pill or two. I am now a firm believer in hydration, if not in the magic number of twelve glasses a day.
Even hydrated, I was pretty much a wreck. We waited til the next day to face the journey home. Patti was upbeat. She had a plan and could see light at the end of the tunnel. The kids were pretty much adjusted. We had filled in friends and family on what details we had. So Saturday, there was nothing for me to do but finally emotionally crash. There are good days, bad days, and "middling" days. Good days are easy - you sail through. Bad days are easy - you shrug your shoulders and lay down. Middling days are hard. You have to work at them. Saturday was a middling day. I tried to walk and stay active and positive. But mostly I just sat around outside in the weak sunshine in a melancholy funk. It was my turn to rest from being "strong".
The bills have begun to arrive. A few of the smaller ones we have talked down. But the Southwest General emergency room bill was $10,000. Cleveland Clinic tells us over the phone that so far we have run up a bill of $35,000 there. Neither place admits to there being any way they will reduce these bills, though everyone including the doctors tell us that they can be negotiated down. Neither of these bills include the doctor's charges (radiologists, pathologists, gastroenterologists, left nostril specialists, etc.) that interacted with me, my bodily fluids, or otherwise left their fingerprints on my chart.
The bill that really feels like a betrayal though, is the one from the Sturgis emergency room. All we needed was a stool sample to be tested for presence of blood. A test we later found we could do at home for eight dollars. There was no blood, so there was no emergency. But we were charged $500, plus $235 for the doctor. I almost cried.
We have since been to the Michigan Department of Family Services. We found them to be very frustrated and busy, but also very professional, understanding, flexible, prompt in giving service, and not too bureaucratic. To our surprise, there is a very good chance we will qualify retroactively for at least some kind medicaid assistance with the bills. Actually, we shouldn't be surprised. We haven't made enough money in any of the past three years to pay income tax. Our taxes this year were all self employment tax that was not offset by child tax credits. But our house is nearly paid for, we have no other debts, and we have some 401k's and (did) have a few thousand in savings. Apparently the rumor that you have to be completely bereft of assets before they can help you is not true. They provide some kind of service to 6,000 of the 48,000 residents of the county, though only 300 are on cash assistance (What most of us call "welfare"). It is hard to tell what we'll get though, because there are over 30 different government programs with conflicting criteria managed over a eight or ten computer systems going all the way back to 1970's DOS platforms. We will have to finish filling out the forms and see what cranks out of the black box.
The gym held a "sleep over" fund raiser for us. It was wonderfully successful. About eighty kids came, and several people that didn't send kids gave us cash donations. We were very touched by the outpouring of support. Until you experience the need, you always feel kind of invisible within a community. This will get us through this past month and the one to come, when my ability to earn money is so compromised. We've also had a lot of support in the form of prayer and meals, by which we are also touched.
I talked to Terry, my brother the Pathologist. He got copies of my slides and agreed with the diagnosis. He also clarified the question of how rare my condition is.
In statistics, you have to ask your questions carefully to get an answer that means what you think it means. Carcinoid tumors are pretty common. They find them frequently in removed appendixes. They are usually small, inactive, and classified as benign. They don't even bother recording them in the tumor registry. Carcinoid tumors that become large, active and cause enough problems to be classified as cancer, are much more rare. Terry's group sees about 20 of these per year in the 20,000 or so Lexington area tumors they do pathology studies on. Of these twenty, only one or two are aggressive enough to have metastasized to the liver.
Doing a wild extrapolation, I'd guess the country as a whole sees 30 to 50 of these. Since carcinoid tumors grow so slowly, most of the diagnoses are made in older patients. So if the question is, "What is the annual occurrence of liver metastases of carcinoid tumors diagnosed in patients under 50 years of age in the United States?", I'm guessing the answer is between five and ten.
[Note: Since writing this, I've found firmer internet statistics of 6,000 "clinically significant" carcinoid tumors diagnosed per year in the US, of which about 600 have visible signs of "carcinoid syndrome" (like me) such as facial flushing and diahrea. The stats were not broken down by ages.]
The oncologist called me with test results. They are mostly not good. I do not have clinical evidence of coeliac disease. So I can eat the occasional jelly donut, but I don't have that to explain my digestive problems. On the other hand a wheat free diet seems to help if not cure it, so she says stick with it by all means.
The CAT scan shows a clot in the posterior branch of the right portal vein. This is the cause of my portal hypertension. It means they can not kill the large tumor with chemo-embolisation without also killing a significant amount of liver. The only remaining treatment options she knows of are some experimental protocols involving interferon at Sloan-Kettering and MD something-or-other.
The CAT scan also found another tumor. This one is in the pancreas, and may be the primary. It appears to be pressing on the common duct, preventing pancreatic enzymes from entering the bile duct. This WOULD explain some of my digestive pain. She thinks it explains my jaundice. Have I had any jaundice lately?
I explained that yesterday I was "yellow man". The whites of my eyes were a dull yellow, my skin was bronze, and where my face was reddened by the sun, I was a mottled orange.
This news seemed to alarm her a bit, and she wanted to schedule the placement of a common duct stint (a stiff tube to hold the duct open where it is near the tumor) as soon as practical. Patti and I talked it over, and decided to have that done there at Cleveland Clinic, rather than at U of M. It is scheduled for this coming Tuesday, May 6.
As I talked to the doctors in my family, I got a better understanding of what is going on, and am getting excited about this procedure. I have repainted my mental picture of what has been going on with me.
I think I have had the neuro-endocrine or carcinoid tumor in my pancreas for a very long time. It is perhaps what causes my Gilbert's syndrome, though a doctor will no doubt tell me I am wrong as I have been about so many things.
I think it slowly grew until it started squishing my common duct. It was too small to show up on a normal CAT scan, and anyway wasn't being looked for. It caused my digestive pain and discomfort. When my gall bladder was removed, there was suddenly free space in the vicinity, and things rearranged so that the duct could open back up, giving the impression that the gall bladder may indeed have been the culprit.
Whether from the nearby surgical trauma, or just cuz, the tumor metastasized to my liver. There the tumors grew over the next several years until they replaced the space opened up by the missing gall bladder, and began pushing back against the common duct. They also crushed capillaries near the portal vein, causing portal hypertension and the varices in my esophagus. The rest of the story you know.
I'm glad I had that extra scan.
Because of pain and frequent trips to the bathroom, it is very hard to sleep at night as it is. Understanding that this procedure may relieve the misery I've been through over the past year in a matter of hours, I am becoming incredibly excited and sleep is impossible. I am counting down the days. I have for the moment abandoned my quest to put weight back on and am eating lightly to avoid pain and perhaps be able to sleep (and see Stephanie's state gymnastics meet). Wish me luck! Like any surgical procedure it is possible for it to go horribly wrong, or at least not yield the hoped for result.
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