Post Surgery Post

From Barbara:

Thank you all for your good thoughts for Greg during his Whipple procedure, which lasted for 9 hours yesterday.

The surgeon (Dr. Tara Kent) reports that all went well and that they did not find anything unexpected.  After they removed the tumor and harvested a bunch of adjacent lymph nodes, they paused while an on-the-spot pathologist examined the margins of the specimen to check that the margins were free of cancerous cells.  Apparently his passed the test, so they were able to move on to the second and more challenging aspect of the procedure--reconnecting the now diminished parts. 

Good news - the pancreas looks normal, which gives Greg a leg up in the recovery process and ultimate resumption of more normal function.  Bad news - the consistency of a healthy pancreas makes it a bit more difficult to work with (I find this aspect of things quite unimaginable), which may have contributed to the length of the operation.

Bad news - the bile duct is still very distended from the obstruction.  Good news - this actually made it easier to work with.

Today was Day 1 of what is projected to be an 8 day hospital stay.  He is still affected by the anesthesia and obviously is in pain, but for someone who has just undergone such major surgery, I think he is doing appropriately.  He is equipped with a pain button he can push as frequently as every 6 minutes, which delivers a dose of nirvana, or at least something that removes the edge somewhat.

The nurse announced mid day that he needed to get up and make it into a recliner type chair, where he was to remain sitting upright for an hour.  This helps clear his lungs and get the blood circulating.  They gave him an hour's warning so he could gear up, both in terms of loading up on the pain med, and just getting psyched.  When the time came, he accomplished the task with less pain than he expected, and was able to attend to a couple rounds of two-handed bridge before declaring exhaustion and the need to return to bed and sleep.  Not bad for day one.

Currently he has tubes draining secretions from all organs involved in the digestive process that were affected by the surgery.  He will not be able to take anything in orally for several days, and they are only providing electrolytes intravenously....no nutrition, as these organs need to heal before being asked to do any work.  He will lose a lot of weight.  Unfortunately, I will no doubt pack on those pounds, as I do my duty, eating for two.

I've requested a private room for him should one become available, as currently he is sharing a very small room.  There was barely space for me, given all the equipment he is attached to.  It will be best to postpone visits for several days....probably until at least Monday, though if he rallies sooner, I'll let you all know.

The big danger now is clots.  They are walking a fine line, as usually they do not start blood thinners immediately after surgery:  obviously the organs need to heal.  But with his history and such a long operation, they have already begun administering anticoagulants.  

The other unknown is the full pathology report on the specimen they removed.  This we will not have for a week.  Meanwhile, we very sincerely thank you all for your love and concern.  I'll try to keep you up to date, but Greg usually posts the blog from his computer, which is in Stow.  I continue to stay at Brian/Bridget's house in Jamaica Plain.  I am counting on Brian's wizardry at the computer to get this out somehow!

Barbara

Day of the surgery, recovering in the PACU

Settling in to Wait for Surgery

I / we have now met with all the major players in my treatment at Beth Israel: Drs. Schlechter (hematology, oncology), Kent (pancreatic surgery), & Levine (Multiple Myeloma).  My hope that there was a “Whipple light” or immunotherapy that could spare me the big operation turned out to be baseless, as there is no alternative to the “full” Whipple.  The internal bits in the area of the Ampulla of Vater (where my tumor is located) are too closely packed in and inter-connected to do anything less than full removal.  Perhaps if the growth had been on the surface (intestine side) of the Ampulla of Vater, I could have been spared the big surgery.  But it is on the hinge of the flap, and who knows how much further up the various ducts, so an isolated extraction is not in the cards.  I must have completely misunderstood the relevance of immunotherapy.  Apparently, that is an option for post-operative treatment, not an alternative to the operation itself. 

So, I am all prepped for surgery first thing on Wednesday, 6/28.  They were going to remove the tube that currently is keeping my liver duct open on 6/20, but the surgical team for 6/28 can do that just as well, so I continue to have a capped tube sticking out of my right side, preventing swimming and complete showers.  I have resumed the treatments for Multiple Myeloma.  I have taken Ninlaro pill #2 and will take #3 this coming Friday.  To take the full course of Revlimid would take me to Monday, 6/26.  But Revlimid interferes with healing and lowers crucial blood counts, so Dr. Levine recommends stopping those treatments the weekend before the surgery.  Yes, the free lambda reading is alarming, but Dr. Levine says Multiple Myeloma is not going to kill me in the next few weeks.  Interfering with healing from the Whipple surgery could have disastrous results. 

Dr. Kent explained exactly what is involved with a Whipple procedure.  Here is a picture of the relevant pieces before the surgery. 

My growth is on the Ampulla of Vater, which is the flap covering where the bile duct and the pancreatic duct empty into the duodenum.  The parts that are circled by the pen on the diagram are the parts that are coming out. The picture makes the pieces all look quite independent and separate.  But another picture, which shows the blood vessels, gives a better impression of how interconnected they all are.  The pen drawing to the upper left is the liver.

Cutting into any one of the blood vessels in that area could be fatal, and they don’t have a way of taking out just parts of the complex.  So, they end up taking out the head of the pancreas, the gall bladder, much of the bile duct, and the duodenum.  Major surgery.

Then they stitch it back up as shown below.  

What is left of the bile duct is attached to the lower intestine, as is the pancreatic duct.  The stomach is sewn into the jejunum, a part of the lower intestine below the duodenum.  The gall bladder acts as a storage area for the juices the liver produces, but apparently the liver can store its juices on its own well enough for normal digestion to occur.  The tail of the pancreas can generally produce the hormones and digestive juices it is responsible for in the absence of the head.  Sometimes there is insufficient insulin, in which case insulin injections are required.

There are lots of risks.  Infection is always a risk in such a large, long surgery.  The places where organs are reconnected may leak.  Someone with my history of pulmonary embolisms will certainly have the risk of a blood clot.  Generally, they monitor these risks and have specific antidotes.  They will have drains next to the 3 areas where the organs are reconnected.  I will wear special socks and have compression booties to keep the blood circulating in my lower legs to minimize the chance of blood clot.  Presumably all sorts of disinfection efforts are standard for this type of surgery.  Then we fall back upon close observation of how the recovery is going and speedy analysis of whatever is going wrong.  Beth Israel has an excellent history of performing these operations, and does more annually than any of the other major hospitals in the Boston area, and apparently I am a good candidate for it.  In the past, they had to resort to the Whipple more frequently than they do today, as not many alternative treatments existed.  Now they only do these operations on patients that are likely to survive and benefit.  Let’s hope I fall into that category.

They acknowledged that this period of pre-op waiting can be stressful, but recommended we head to Cow Island for a few relaxing days during which I am to eat as many good nutritional meals as possible, and avoid any sort of risky behavior: no broken legs, hips, etc., no major cuts….given my track record, this will be a challenge as well.

Thank you all for your good thoughts and concern.  Modern medicine is indeed amazing, but I would prefer to learn about it third hand, not first hand.

Good News, Bad News

The good news is that the results from the MRI on Wednesday, 6/14, are in, and the results are great!  They were concerned about spots on my liver that might have been metastases of the cancer on my Ampulla.  If they had stayed the same size or gotten bigger, then they would be metastases.  But in fact they got smaller, so they probably were a result of the infection I had when my bile duct was blocked.  So, this means that they can concentrate on removing the growth itself.  Either through surgery or through immunotherapy.  They were going to do genetic analysis.  If I have the genes that enable this cancer (which is statistically improbable) then immunotherapy is preferred.  The downside of that eventuality is that Brian and Andrew and their kids will have to worry about the same genes.  We meet with Dr. Schlecter first thing on Monday to determine what my treatment will be.  Meanwhile they are scheduling surgery on June 28^th.

Right now I am rooting for the immunotherapy.  Apparently it is quite targeted and effective, if the cancer is caused by those genes.  The alternative is a Whipple surgery.  This is a monster surgery, lasting up to 6 hours, and doing major reconstruction of your upper GI tract.  Google it to get a sense of how complex it is.  I have been told that the growth I have is pre-cancerous and not highly developed.  Actually, they say it is lucky that the growth blocked my bile duct so that the growth was discovered.  Growths in that area have no symptoms, so they can grow for years without being detected.  So mine was detected relatively early.  My question (which I will ask Dr. Schlecter tomorrow) is with a relatively young growth, isn’t a whipple surgery an extreme solution?  Yes, the growth was strong enough to block my duct, but the biopsy says it is pre-cancerous and if it was discovered at an early stage, mightn’t there be a less extreme solution to correct it?

The bad news is that my Multiple Myeloma is coming back strong.  Tuesday they took blood samples for a variety of tests.  The key test takes 2-3 days to come back.  On Thursday evening I checked the BIDMC Patient Site, and saw that my Free Lambda was 803.  During the analysis of my illness the Multiple Myeloma drugs were suspended.  I had just started Ninlaro and Revlimid on Tuesday, 5/23.  Friday, 5/26, those were stopped.  5/23 my Free Lambda was 345.  The prior 2 tests were 411 and 419, so the trend had been down.  But 803 reverses that trend in an extreme way.  Here is the latest graph:

That is a dramatic uptick at the end.  We thought that we were in dangerous territory when the readings were in the 400’s.  After all the maximum of the normal range is 26, so 419 is 16 times the normal max.  To double that puts us well into an area of major concern.  I sent an email to Dr. Levine Thursday night and followed up with a couple of phone calls Friday.  Finally after 4PM Dr. Levine called me back and told me to resume the Multiple Myeloma medicines.  So I took a Ninlaro pill Friday night, as well as Revlimid and Dexamethasone.  Let’s hope it has a dramatic effect on the next free lambda reading – in the opposite direction.

So the drama continues.  I hope to ease up on the drama, but there will continue to be news until the ampullary adenoma is dealt with.  I’ll keep you posted.

An Update from Barbara

I cede the platform to my better half, as I just didn't have the energy to put a rational post together after a pretty long day at Beth Israel.  So hereafter are Barbie's words:

Thank you all for your kind thoughts and help through this incredible past two weeks.

Many of you know that we looked forward to a meeting today with two surgeons (upper GI cancer folks) who are currently running with the ball on this issue.  We hoped to come away from the meeting with a clear picture of what we face, and the best path forward.  But as with every test and procedure involved in trying to solve the mystery of exactly what we are dealing with,  what actually happens is not what anyone predicted; it is like a seemingly small shrub, which, when investigated further, has a huge and complex root system. Every root has many branches going from it, any one of which may represent the actual situation. We are still determining which root system to follow.

What is known: Greg has an ampullary adenoma, a tumor located on a flap at the end of the common bile duct (called the Ampulla of Vatar), which drains the liver, the gall bladder, and the pancreas.  While the biopsies taken so far do not indicate a malignancy, there is a very high false negative with these tumors, where 25-30% of “benign” tumors are actually already cancerous (in which case, it is classified as ampullary carcinoma).  Adenomas in this area always become malignant and are aggressive, though not as fast growing as those associated with pancreatic tissue. Greg’s tumor is too large for laparoscopic removal, but it does seem to be relatively superficial, and probably will be a good candidate for resection (surgical removal), as it appears not to be highly vascular.  That being said, the recommended procedure is a 6-hour operation that removes and reconstructs parts of several organs in that area.  But a small unexplained spot appeared on the liver, visible on MRI but not large enough to see clearly on a CAT scan or ultrasound.  Were it not for this unexplained lesion, they would be scheduling surgery directly at this point.

If this liver spot is cancerous, then the cancer has metastasized, and we are dealing with a radically different situation.  If so, they will not subject Greg to a large and complicated surgery, but rather would move directly to chemo therapy.  The immediate task is to define the nature of that spot.  Since it is in such a difficult location and is very small, they will first try another MRI, scheduled for next Wednesday, to see if the spot has shrunk, or stayed the same. There is a chance that the spot was caused by the infection resulting from the blocked bile duct. In that case, it should recede and shrink — that is our greatest hope at the moment.  If it has not gotten smaller, they may try a biopsy either as a separate operation, or as the opening act to the big operation they propose, called a Whipple.  Apparently they can have a pathologist right there in the operating room who could read the biopsy on the spot — and either they would go ahead if the cells are normal, or they would just stop the procedure at that time.

If the liver issue is resolved, then there is the huge and complex Whipple procedure itself.  Google it.  It is no fun.  But, if successful, it can result in a “cure”, unlike Multiple Myeloma, where there is no cure, but where the disease is “managed” with a quiver full of new drugs. 

Another wrinkle.  Greg’s mother’s family has a lot of cancer.  There is a small correlation between Multiple Myeloma and ampullary cancers, caused by a genetic mutation.  The offending genes have been identified, much as the presence of a BRACA gene is a strong predator of breast cancer.  Currently they are running genetic tests on Greg’s blood and other material he has left behind in various labs.  If he has that mutation, then our kids and their kids will need to be screened so they can be watched appropriately throughout their lives.  If he does not have that gene, then it is just a case of bad luck/coincidence.

This was going to be a quick update, just to let you all know where we stand.  But it is difficult to keep things short, as the situation is intrinsically complex.  We do not want to be learning about GI cancers, but we must.  Perhaps Wednesday’s MRI, if it shows a shrunken liver spot, can remove that unknown and the surgery will be scheduled.  If that MRI is inconclusive, we will have to get the cellular information in another way.

Greg’s situation is incredibly rare.  Cancer of the ampulla accounts for just 0.2% of all cancers — perhaps 7,000 in the USA annually (vs. 150,000 colon cancers, 250,000 breast, etc.)   By luck, thanks to daughter-in-law Bridget’s being a doctor at Beth Israel in Boston, we happen to be in the hospital that performs many of these operations — it stands either #2 or #3 in number of pancreatic surgeries performed annually in the country.  The lead surgeon does about 200 ampullary surgeries a year — a very high number, so that is good.  Interestingly, the doctor said Boston is unique — Harvard Medical School doctors are spread throughout the city’s many fine medical institutions, so that certain hospitals become associated with different specialties — lung with one hospital, blood cancers with another, etc.  We landed in the right place for upper GI cancers, and are very impressed with the doctors.

Once again, thank you for all your good wishes.  I wish I could convey good news, a definite schedule, a clear diagnosis and good prognosis.  None of those are possible at the moment. 

If you do not receive Greg’s blog, you may sign up for it, and he will provide a pretty good background on his encounter with some of life’s nastier diseases.  On the positive side, the doctors say he is very fortunate that this tumor caused a blockage, and therefore was caught quite early.  Most cancers in this area are asymptomatic and therefore are discovered too late, or by chance (when someone has an accident and the state of their upper GI is revealed for another purpose.)  Also, Greg has always been very active and is in very good shape. But, the strong drugs he takes for Multiple Myeloma must be processed by the liver, and they have had an effect…..

We’ll update you as things progress and we discover more.

Barbara

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.

Blog post 3/11/2017 – Updated 5/24/2017

Here is what I wrote on 3/11/2017, 2 ½ months ago:

Well my last post was over a year ago – on 2/13/2016.  Originally I was not posting because there wasn’t much going on.   There has been some stuff going on recently, but I haven’t posted because I wanted to talk about the resolution of the stuff.  But things are still unresolved, so I’ll talk about what is going on anyway.

First, here is the graph of my numbers, yet again:

 

The red line is Free Lambda.  It starts at the left when my disease was diagnosed in June, 2012, at 1329.  The normal max is 26, so that was 50 times the normal max.  The treatments in summer, 2012, quickly brought things back to normal.  Then in the summer of 2014, the readings began to rise again.  Treating it with Revlimid stopped the rise, but did not send it back down.  Then in early 2015 it rose dramatically again, in spite of the Revlimid.  So Velcade was added, and that brought it down dramatically.  I’ve been on weekly Velcade ever since then.  The Revlimid continued as well until last summer.  Since it was ineffective by itself and since it costs $10,000 per month (5% of which I have to pay, but that is still expensive), I asked why continue it.  So it was dropped in June, 2016.

The above graph is just too small to understand the details.  But it gives you the overview of the 2 major bouts I’ve had with rising Free Lambda numbers.  As you can see to the right, the numbers are on the rise again.  So let’s look at the data since last June.  I’ve removed the Free Kappa values and max, as Free Kappa is not a problem right now.

  

Free Lambda bounced around, mostly going sideways last summer.  It was above its normal max, but not alarmingly so.  Then in November it started to climb.  Back in 2014 when it reached 170, Dr. Levine started to deploy medicines against it.  It reached 183 in mid-December.  The decision was made to reinstate the Revlimid, on the hope that the combination of Velcade, Revlimid, and Dexamethasone would have – combined – effects that the drugs alone were not having.  But then the drug took a while to order, as I was changing Medicare Part D insurance companies, and the old company tried to evade paying for a prescription late in the year.  Once I had the Revlimid, the decision was to delay taking it until we returned from our trip to Tanzania (January 8-22), as a nasty reaction in the middle of nowhere was not something to be desired.  Then when I returned I was still on the anti-malarial drug, which gave me a bit of a rash.  Finally, late in January I did start the Revlimid.  But then I got another rash, and they suspended the Revlimid for a week.

So the latest reading is 270.3, which is 10X the normal max.  But I am feeling quite well.  No symptoms, and I am able to run every morning – although at a crawl’s pace.  Funny thing.  Back in early 2015 when I was on Revlimid alone, I thought there were minimal side effects.  But now that Revlimid has been added to a continuing regime of Velcade, I can see that Revlimid itself is the cause of many side effects.  The soles of my feet feel as if they have double calluses, and I need to take some sennocot every day to facilitate bowel movements.  But if the combined drugs bring the Free Lambda back down, those side effects are minimal.

This is far from the end of the story.  We don’t know what the next Free Lambda reading will be.  We are on pins and needles until 3/28 when the next test will be taken.  If a dramatic turn occurs, we will either continue the current regimen or drastically change it.  The good news is that there are lots of choices for new drugs to take.  You don’t want to change to the new drugs too soon, as Multiple Myeloma is continually evolving, and you want to have as many treatments as possible still effective against the disease.  This continuing mystery is why I haven’t written this blog post sooner.  Unlike Paul Harvey, I cannot provide the “rest of the story”.

Changing the topic: the Greg’s Legs team will be running/walking in the Multiple Myeloma Research Foundation’s Boston 5K on April 30^th.  We have done this race for the last 3 years, and it is a good time.  If you are in the area and up for some exercise, please join us.  My personal website is walkrun.themmrf.org/Boston/GregsLegs.  You can see last year’s team on the team page: walkrun.themmrf.org/Boston/Team/View/32396/Gregs-Legs.  You can register or donate at either site.  The whole point is raising money for research into Multiple Myeloma.  Last year’s race raised over $500K, and this year’s goal is $540K.  The MMRF runs 11 such races across the country and last year raised $2.9 million.  All of which was put to good use, as last year 4 new MM drugs were approved by the FDA.  Since there is a good chance that I will need to change to new drugs to fight Multiple Myeloma soon, I am extremely grateful that the MMRF has led the way for drug companies to pay attention to this disease.  MM might be the second most prevalent blood cancer, but with only 20,000 new patients per year, until the MMRF came along and organized patients for clinical trials and provided incentives for directed research, the drug industry was ignoring MM.  If I had gotten MM 30 years ago, I would not still be writing this blog, as the average life expectancy then was 2 years.  So, in my humble opinion, this is a good cause and one that I urge you to support.

Continuation on 5/24:

Well the race on 4/30 is long over.  I actually did quite well, finishing in 33:30, a 10:48 mile pace.  Once again I got the first place medal for men over 70.  There were 6 of us.  Barbie also got the first place medal for women over 70, but there were only 3 of them.  Our team raised $8,110, which is far more than we’ve ever raised in the past.  A big THANK-YOU to everybody who donated! The event raised over $640,000, which makes it the highest fund raising event in the MMRF’s roster of athletic events.  I’m sure the MMRF would still accept donations, but not in our team’s name.  But don’t let that stop you!

Now I can tell you the rest of the story.  Here is the latest graph:

On 2/28 the Free Lambda was 270.3, 1028% of the max of the normal range.  On 3/28 the reading was basically the same: 268.3 or 1020% of the normal max.  Then on 4/25 it had a major rise to 411.7 or 1565% of the normal max.  The latest reading is 419.7 or 1596% of the normal max.  When I meet with Dr. Levine, my oncologist, the latest reading is not available, so any decisions are made based on data a month old.  The decision was made to switch from Velcade to Ninlaro, a successor drug to Velcade.  They are both proteasome inhibitors, which means they work by making it so the Multiple Myeloma cells cannot dispose of their wastes.  So they die from their own pollution.  I asked Dr. Levine why Ninlaro would work when Velcade did not.  He said it was in the same class, but not exactly the same.  At any rate, we will soon find out.

Ninlaro is a pill, not a shot.  So I don’t need to go into Beth Israel every Tuesday any more.  But I do have to go through a telephone survey so that the drug company can be sure I know how to take the medication and how to act while on it.  Plus I get to pay for it.  3 pills cost $10,227.44, of which I get to pay $511.37.  So is it worth that much to avoid 3 round trips into Boston?  It certainly is if it brings Free Lambda back under control.

So I am now on a new regimen.  I take Ninlaro for 3 Tuesdays, Revlimid for 2 weeks daily starting on the Tuesday I take the first Ninlaro, and then Dexamethasone on the Tuesday I take the Ninlaro and the next day.  I have a weekly pill array that lets me figure all this out a week at a time.  Actually, I take 11 pills on a normal day, and then extras on the days I am taking any of the 3 described above.

I looked up Ninlaro on the internet to further explain how it works.  But the thing that caught my eye is a study that determined the relative progression-free survival rates:

NINLARO has been proven in a study to help some patients live longer without their multiple myeloma getting worse*

In a study of NINLARO, the NINLARO regimen (NINLARO+lenalidomide+dexamethasone) was proven to work in some people whose multiple myeloma had come back or stopped responding to prior therapy.

This study evaluated progression-free survival (PFS), the length of time during and after treatment that a patient lives with multiple myeloma but it does not get worse.

Treatment with the NINLARO regimen increased the median progression-free survival by about 6 months

20.6 months with the NINLARO regimen

14.7 months with the placebo regimen

 I'm not sure how to read the results of this study.  Will I be good for 20.6 months or 6 months?  I don’t think that I would have the luxury of trying a placebo regimen, as my MM seems to be on the edge of breaking out already.  But if people using the Ninlaro combination get a year and 8 months before things start to turn worse, then that is a good result.  We’ll see.

[I will soon provide another blog post, as 2 days after starting Ninlaro I had a major medical event which resulted in the suspension of my Multiple Myeloma treatments.  More to come soon…]