The Monster in the Room

Friday, 5/26, I woke at 2AM feeling very full.  Thursday evening Dowie & Bob (Barbie’s sister and her husband) had stayed with us on their way to New Hampshire, and Dowie had cooked a very nice chicken and mushroom dinner, so I thought I had overeaten.  I went to the bathroom, and I expected things would settle down.  But they didn’t.  I still felt full, and I was cold.  We have a down comforter on our bed that normally makes me sweat in minutes, so I pulled that over me.  But it didn’t work.  In fact, I got colder.  Shivering all over.  Eventually I shoved over onto Barbie’s side of the bed, as she has an electric blanket on her side of the bed.  Actually, the same blanket is on both sides of the bed, but my side is never plugged in. 

By 5:30 I was shivering so bad that my teeth were chattering.  Barbie woke up and said I was an oven, I was so hot.  She sprang into action, getting the thermometer to take my temperature (103.3), getting a couple of Tylenol to get the temperature under control, and insisting that I call Beth Israel Hospital to see what I should do.  They said, “Go to the emergency room.”

Tuesday of that week I had taken the first of a new medicine for Multiple Myeloma, Ninlaro.  As a result, we assumed this was a delayed reaction to the new medicine.  My Free Lambda numbers had been going up all spring, and the Ninlaro was a replacement for Velcade.  Velcade is given by injection, and I had been going into Beth Israel (BI) every Tuesday to get my shots.  Ninlaro is the same class of drug as Velcade, a proteasome inhibitor, just in pill form.  Proteasomes break down proteins in cancer cells, and inhibiting their action results in a buildup of cellular wastes, which in turn kills the cancer cells.  Ninlaro is the same class of drug, but enough different that it could well turn the growth of my Free Lambda numbers around.  But I took the first Ninlaro pill Tuesday evening, more than 2 days before I started to have problems Friday morning.

We got to the emergency room at Emerson Hospital by 7:30.  Everything was very quiet, so I got a bed and attention right away.  Immediately they took blood samples and vitals.  They took extra blood samples to culture the blood to see if there was systemic infection of the blood.  The emergency room doctor took my symptoms and did a general inspection.  They immediately did a chest X-ray and did not see any pneumonia present…at that time.  Palpitating my abdomen, the doctor suspected something with the gall bladder.  An ultra-sound of my torso revealed that my bile duct was enlarged or distended.  They suspected that a gall stone might have passed recently.  So they took a CT Scan of that area.  By this time the blood tests were back, and my liver function tests were off the chart.  Because I take coumadin on a regular basis to thin my blood to prevent pulmonary embolisms, they were nervous about injecting iodine into my blood to provide contrast for the CT Scan.  The CT Scan revealed that there were no stones in my gall bladder (which would have been the case if I had passed a big one recently), but there was a growth on the pancreas that was probably causing the problem with the bile duct.

At this point it was decided that my case was beyond the capabilities of Emerson, and I should go into Beth Israel in Boston, where I have been receiving regular treatment since 2012 for Multiple Myeloma.  All the Emerson imaging was put on a disc, I was put into an ambulance, and we were all off to BI.  It was about 4:30 when we got to the BI emergency room, and it was abuzz with activity.  They retook all the blood samples and placed their own IV lines for the administration of medicines.  Bridget, our doctor daughter-in-law who used to work at BI, joined us in the emergency room, and helped us interpret much of the information we were receiving.  The real emergency concerned the blood infection raging through my system, presumably caused by the blocked duct from the liver.  If the bile is not able to drain, the infection goes from the blood, to the organs, to full body sepsis.  I was well on the way, but the antibiotics Emerson commenced held the infection in check while immediate steps were taken to relieve the back up in the duct.  By Friday evening I was admitted, ending up in a room on Feldberg 7, the same floor where I got my stem cell transplant back in 2012!

Early Saturday morning they performed an ERCP (Endoscopic retrograde cholangiopancreatography) in which they send an endoscope down your throat through your stomach and into your intestine to look at and open the bile duct.  In my case the goal was to free the duct so that the normal excretions coming from the pancreas and the liver could flow through it.  Presumably the reason I was feeling full on Friday morning was that the blockage of the duct had caused the upstream organs (liver, gall bladder, pancreas) to become engorged with fluids.  By this time the blood that they had drawn at Emerson to culture to see if my blood had any infection had shown that I did have bacteria in my blood, that I was in a septic condition.  All of which means I was very sick. 

A Dr. Sawhney performed the ERCP, and spoke with us before and after the operation; in what has become a pattern, what they actually ended up doing differed from what was originally planned, as they reacted to the situation they found, rather than the one they expected to find.  He said he probably would not take any biopsies, as the emergency nature of my condition made clearing the duct paramount.  What they found was that they could put a stent into the pancreatic duct, but the duct from the gall bladder and liver was so obstructed that they could not get through from their end.  They were able to take biopsies of the growth that was causing the blockage.  That all happened Saturday morning.

Early Sunday morning I went down for another procedure to free the duct, this time coming in from an incision in my side, performed by a team of Interventional Radiologists.  Who knew there was even a branch of medicine called Interventional Radiology?  From this direction, they were able to get a stent into the bile duct and relieve the blockage.  Here is a diagram that one of the doctors drew for us to understand what was going on:

Unfortunately, I managed to cut off the bottom of the diagram.  The trapezoidal shape in the middle left is the liver, which is in the background.  The eggplant shaped thing in front of the liver is the gall bladder.  The shape with wavy lines to the right is the pancreas.  The round thing with diagonal lines is the growth that is causing all the trouble.  The tubes coming down from the gall bladder and the pancreas meet and then empty into the lower intestine, are the ducts that have been blocked by the monster in the room.

They placed a tube in the liver/gall bladder area that had 3 different sections.  In the middle were several perforations that allow the fluids to flow into the tube.  They call these fenestrations.  On the end going through the bile duct into the intestine are things to make sure it doesn’t get crushed by the same pressure the closed the duct in the first place.  The third part is the tube going out through the skin to an external collection bag. 

This provided us with an education in what your liver / gall bladder does on a regular basis.  First there were very thick, dark fluids that came out, presumably infected by the accumulation caused by the blockage.  Then they got lighter in color, and there were odd things floating in the bag.  Again, presumably the result of things being out of whack for a while.  Finally, the fluid was a greenish-brown color, a color that you would never choose for any wallpaper.  An interesting thing is how the flow was very uneven.  When it was flowing, it would fill up the bag (about a liter in size) in no time.  Other times, nothing would appear. One time as we were watching, the color of the fluid turned black.  Barbie accused me of being like a squid, and sending out ink.

I had an odd reaction from the anesthesia they gave me for the Sunday morning operation—a strong urge to pee every 15 minutes.  It didn’t matter that I had almost no urine in my bladder, I still had a very urgent need to go.  This kept up for most of Sunday, and I got up several times Sunday night.  By the middle of Sunday night, the frequency was greatly reduced and by Monday it was gone, but it was a lesson.  Never again will I complain when Barbie says we need to stop at a rest area.  [I have been known to voice such complaints in the past.]

By Monday I was feeling much better.  Yes, it was difficult to breathe deeply, as there was a wound in my lower right rib cage.  But compared to the time when the bile and liver were backed up, all was sweetness and light.  But there was still a monster in the room.  What was the growth that caused the blockage?  We could speculate, but the answer would not come until pathology had done their job analyzing the biopsies.  The medical team that deals with cross-department cases was scheduled to meet Tuesday afternoon to discuss my case amongst several others, so the pathology department was notified that their results were needed and expected.  Someone in that department worked over the weekend preparing the specimens for analysis and doing the required staining.  But there was no actual news.

All other things were going well.  My liver numbers were coming down precipitously once the blockage was gone.  The antibiotic was having the desired effect on the bacteria in my bloodstream.  They measured the amount of fluid I was collecting in the bile collection bag, and determined that all was well.  They capped the external end of the tube, and assumed that all the fluid generated would flow out the other end into my intestine.  I even had a chance to take a shower.  But all this while we were on pins and needles as to what the answer to the big question would be:  what was the monster in the room?

Well Tuesday at 2:30 the answer came: a new form of cancer.  This was not the preferred answer.  It would have been much better if it had been a tumorous form of Multiple Myeloma.  Instead it is an adenocarcinoma, a kind of cancer that grows out of epithelial cells that have mutated and turned cancerous.  The strategy for how to treat the cancer depends on a few things we don’t know yet.  Exactly what is the source and flavor of the cancer?  It can be from the bile duct, the pancreas, or the ampule, the valve into the intestine of the bile duct.  Is it confined to the area close to the blockage, or has it been lurking there for a long while and has it spread to other areas?  Obviously, if it has spread, things are much worse.  Finally, can it be surgically removed?  Inevitably it will have developed blood supplies and in effect grown into the tissues it is attached to.  The question is will the surgeons be able to reconstruct the underlying plumbing after they have removed the cancer?  The preference is for it to have only grown into a limited set of tissues so that the repairs required are minor.

They did an MRI scan Tuesday evening to get better images of that area to address some of the questions.  The MRI process requires you to hold your breath quite frequently.  Remarkable how motivated one is to make it so that the process produces the best possible images.

We met Wednesday morning with Dr. Ben Schlecter, an upper-GI cancer specialist.  He said he is one doctor nobody ever wants to see.  Upper-GI cancers are located in an area that is difficult to treat, and some of them are fairly aggressive.  They think the cancer I have is only moderately aggressive.  So, it is more important to find the right strategy for fighting it than to start right away.  The results from the MRI raise the possibility that there may be metastases on my liver.  These are very small, and they may be “old age spots”, not cancer.  He ordered an ultrasound to further define what the spots are.  This was done Wednesday afternoon, and the results were inconclusive.  I could see some of the images the ultrasound was creating, and they were not very well defined.  Ideally, they will be able to take biopsies of the spots in the liver to determine just what they are.  But that will happen sometime next week.

I was discharged from BI late Wednesday afternoon.  Barbie had a rehearsal for a joint choral event in Worcester at 5PM, so there was major pressure for us to get out of there.  We didn’t get out until well after 4:30, so 5PM in Worcester was hopeless.  She eventually did make it there, and she did meet the conductor of the combined choral event (Benjamin Britten’s War Requiem this coming November in Mechanic’s Hall).

But the drama was not over.  Early Thursday morning, again at 2AM, I felt very uncomfortable.  It felt like extreme pressure in my liver area.  I took my temperature, but it was nothing (97.7).  I wondered if it would make sense to uncap the tube coming out my right side to relieve the pressure on my liver.  Eventually I called BI, and the doctor on call said to leave the cap on the tube in place and go to the nearest emergency room.  So we did another early morning trip to the Emerson ER.  Again, standard blood draws, chest x-ray, local exam.  But there was no smoking gun, no preferred diagnosis.  And by 6:30 I realized that I was actually feeling better.  Whatever was causing the sense of discomfort and pressure was gone.  Mysteriously so, but then we don’t need to have all mysteries solved.  So I agreed to a final ultrasound of my legs because my feet were swollen in exchange for a speedy discharge after that.

The domestic politics of all this merits a discussion.  Barbie’s 50^th college reunion (Wells College, Aurora, NY) was scheduled for this coming weekend and 4 Wells alumnae were going to drive out together leaving our house at 10:30 Thursday morning.  Barbie was heavily involved in a reunion of Henry’s Eight, an acapella choral group she led during her time at Wells.  My getting up on Thursday morning and saying I needed to go to the ER probably had ruled out her going to the reunion.  Barbie said that she would not blame me for ruining her weekend because it was not something stupid I did that caused it.  But still it made the decision to go to the ER more difficult.  Well, if we could get out of the ER by 8AM, she could make it back to Stow in time to join the gaggle going out to Wells.  Which is what happened.  Part of the deal was that I needed to stay overnight at a friend’s house so if another early morning event happened, someone else could drive me to the ER.  As I write this post, Barbie is out in Aurora, and having a grand time at the reunion.

The big news is the monster in the room – another form of cancer, a version that is difficult to treat.  We will know more in the upcoming days, but the big picture will not change.  This is a second cancer, requiring a second course of treatment.  Right now, it is preempting the Multiple Myeloma treatments, but eventually both cancers will have to be treated simultaneously.  Dr. Levine has long said that they can now turn Multiple Myeloma into a chronic condition instead of a fatal disease.  But that does not give you a perpetual ticket.  Something else is going to get you.  Is this new monster that something else?  We don’t know.  Stay tuned.