Wednesday is Robot Day

Since I never really believed I was going to get cleared for surgery, I really didn’t think too much about the surgery itself or what was going to follow.  But this shit is real now!  So the day after my appointment with Gandalf, I had my final pre-op CT scan and then started reading in earnest to try and make sure I asked all the right questions at my consult with thoracic surgery the following day.  I read up on all the different ways a thymectomy can be done and the pros and cons.  I read up on surgical management of thymic neuroendocrine tumors and what to expect during and after the surgery.  In case you are interested in reading more yourself, this article was a particularly informative one for me:

http://journals.lww.com/pancreasjournal/Fulltext/2010/08000/NANETS_Consensus_Guideline_for_the_Diagnosis_and.8.aspx

Reading this stuff in black and white both scares and reassures me.  It really underscores the need to have this surgery.  There is no doubt in my mind, but it helps to see it in writing, from a source I trust.   I need to know this.  I need to do this.  That is all there is to it.  It is ok to be afraid, as long as fear moves you forward and doesn’t paralyze you.  Someone as a joke once told me that “Scared But Determined” should be on my tombstone.  It is certainly the motto I am living by today.

I made a list of questions and circulated it to M, The Coven (a dear group of friends that have been with me through this every step of the way) and some very informed and helpful family members so they could add their own questions to the list.  Knowledge is power, as they say, and given time was of the essence their input was very helpful.  The more people thinking about your problem the better, in my books.

My cramming on Thursday left me with a lot more questions than answers by the time my appointment with the thoracic surgeon rolled around Friday.   She was very no-nonsense and forthcoming with information.  My CT scan was consistent with the others – no obvious surgical target but a few lumps and bumps that could be a tumor.  As we looked at the images together, she was of much the same mind as Gandalf:  *maybe” this is something?  *Maybe” that is something?  For certainty, my symptoms and the Octreoscan ARE something, but as the surgeon said, none of us really know what we will find until they get in there.

The surgery itself is quite interesting.  The surgery will be conducted by two thoracic surgeons who work as a team.  The thymus is in a tough to access spot, nestled between the heart, the breastbone, lungs and major veins, arteries and nerves running through the chest.  Cozy!

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To get my thymus out, the surgeons have decided to use a robot assisted surgery.  Four robotic arms are placed into one side of the chest.  One of the arms has a camera and the others have surgical implements. Together, the surgeons control the arms of the robot from a console in the OR, using images from the camera as a guide.

The plan is to remove the entire thymus as well as the bed of fat between the thymus and the heart.  If anything else looks suspicious while the surgeons are in there, it will get removed as well.  I also have a small nodule in my lung that I have known about for about a year now.  It is difficult to characterize the nodule from the CT scan – it could be a lymph node, it could be a tumor, it could be benign or not.  It is anyone’s guess.  The plan is to remove the thing in my lung as well if it is easy for the robot to access.   Since the surgery is minimally invasive, if all goes according to plan, my recovery should be relatively easy, with only a day or two in the hospital after the operation.  Not so bad!

The second half of the thoracic surgery team was out of town and will be gone all of next week as well.  He still needs to review my case and sign off before we can move ahead.  I am hoping this is just a formality, since Gandalf and the first thoracic surgeon have already signed off.  We have a few pre-op details to take care of after that and there will be several details to work out with anesthesiology due to complications introduced by my pituitary surgery last year and complications introduced by this current tumor.  But knowing what problems to prepare for is 95 percent of the battle.

I won’t have a firm surgery date until thoracic surgeon #2 is back, but I was told to plan for either the third or fourth Wednesday in June as Wednesday is “robot day” for the surgeons.  In the meantime, we will all continue preparing for the surgery and for what will follow.

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Gandalf

M and I sat down with the surgeon for a follow-up yesterday morning.  He started by asking how things had been going over the last three weeks and I explained that my digestive symptoms had been getting much worse and that I had been doing some “controlled experiments” to see if my troubles were related to eating various foods – and that certain foods did seem to trigger attacks.   He informed me that the findings of my experiment actually didn’t seem to fit carcinoid syndrome and as he started to explain why, all I could do is tell myself over and over, don’t cry don’t cry don’t cry.

On his end, he told me he had been busy looking into my case.  Part of his investigations involved speaking with the endocrinologist I had been seeing at his hospital between 2006 and 2012 (one who did not diagnose my Cushing’s).   Don’t cry don’t cry.    He told me he sat down with his radiologist and they looked over everything together and they did not see anything obvious on the CT scan from the NIH (in January).  Don’t you dare cry, you just hold it together until you get out of his office.  It was happening again, just like with the oncologist and all the other doctors I had seen so far.  He looked at my scans, decided there wasn’t enough evidence, talked to my old doctor, decided I was a nut case, and was going to tell me to go home.   I never should have come here, I fucking knew it.  Just don’t cry.

But he didn’t call me a nut case and tell me to go home and stop chasing imaginary problems.  He said, “we need to go forward based on your flushing and the Octreoscan.”  And he further said that what my former endocrinologist told him was that although my test results never squared up with Cushing’s while I was his patient (which is not true at all, but I was not going to argue this point now), it was always very clear something was really wrong and that my symptoms were real.  He and my endocrinologist concluded that that this was the reason I had racked up so many frequent flier miles trying to get things sorted out.  And he didn’t want me to rack up any more.   He said it over and over again – your symptoms are real – and commented on the fact that I was flushing like crazy as I was sitting in front of him again.  He asked M to look too:  “Is this how she flushes at home?  I have been sitting here watching you, and you have not scratched your neck or touched that area of your chest and you are very flushed.  You are not red like that anywhere else.  Your symptoms (I was coughing and wheezing too) are very real. You have ruled out everything else.  And you have a positive Octreoscan.”  He said he had consulted with thoracic surgery and they reviewed all of my information and determined that if I wanted to go ahead with it, they would do it.

At this point, I was very confused and so when he asked me if I had any questions I said:  “wait a minute, just to be clear, is this really going to happen?”  And when he said yes, I started flat out bawling.  So much for don’t cry.  I was so damn relieved.  I did my usual Canadian “sorry, sorry, sorry!!!” as I searched around for a tissue and pulled myself together and then we got back to business.   He told me about the surgeon who is going to do the procedure and that they wanted to get an updated CT scan and for me to meet with her in person in the immediate future.  I had the scan this morning and meet the thoracic surgeon tomorrow.  We talked briefly about the surgery and I am busy compiling questions and reading up on it today so I am prepared when I meet her tomorrow.

From this point onward, I am going to refer to this wonderful surgeon as Gandalf, as I suspect he will be sticking around long enough that he needs a name.  [As an aside, my naming conventions are the following.  If you are a doctor or a dear friend and you do a great job or are especially wonderful to me you will get a nickname like the Wizard or Gandalf (or the Coven).  If you do an incredibly shitty job or do something else to really piss me off, I will name a tumor after you.]

Gandalf appears to be a doctor that is extremely careful with his words and he cautioned repeatedly that he can’t guarantee this surgery will fix everything or anything for that matter.   That I should know going in that there was a chance that they could remove my thymus and I would wake up and still be symptomatic and all we will have done is rule out my thymus as the source of my current problems.   He told me repeatedly that no matter what the findings from the surgery are it does not mean I will be diagnosed with MEN-1.  And I get all of that.  I realize there is a chance that the Octreoscan finding could be incidental.  And that the flushing is caused by a tumor in an entirely different location.  But I am much more willing to take the chance we are wrong about my thymus and we remove it for nothing than I am willing to take the chance we are ignoring a potentially malignant tumor because we can’t establish with certainty it is there.  Trust me, I don’t want to have MEN-1 either.  This is the kind of disease where if you tell a doctor you are being evaluated for MEN-1 the most common response is “I really hope you don’t have MEN-1.”  I hope I don’t have it too!  However, the only thing worse than having MEN-1 (or something like it) is having MEN-1 and not knowing it.

So my big question for him was “on the off chance I do have a neuroendocrine tumor in my thymus and this is confirmed post-op, if this isn’t MEN-1, is the fact that I had pituitary tumors causing Cushing’s last year and now this thymic tumor this year just exceptionally bad luck?”  His response?  First, there is more than an “off chance” I have a NET in my thymus (I think so too).  Second, it would unlikely be a coincidence.  It is likely that I either have what would be a very unusual presentation of MEN-1 or I have another MEN-1 like syndrome that the medical establishment does not yet understand very well.

And that is part of the problem I have been having.  Although MEN-1 can manifest in a very wide variety of ways, typically people my age have other more common tumors by now – parathyroid adenomas (which I still think I might have), prolactinomas, other kinds of tumors.  ACTH producing pituitary tumors (i.e., the kind that produce Cushing’s) are possible but relatively uncommon for MEN-1.  Of the group of patients with MEN-1 (a very rare disorder) and pituitary tumors, only 4% had tumors that caused Cushing’s.   In the general population, approximately 10 patients in a million get Cushing’s every year.  We are talking very low odds.  Thymic NETs are much more rare than even Cushing’s:  approximately 1-2 people per 100,000 are diagnosed with a “carcinoid” every year.  Out of this population only 0.3% are located in the thymus.   This translates into 1-2 people of out 33,333,333 people a year getting a thymic “carcinoid” (the tumor we suspect I have).  Taking the statistics one step further, the likelihood I would get both ACTH-producing pituitary tumors and a thymic NET by pure coincidence?  Roughly 1 in 1,667,000,000,000 (all that stats training comes in handy sometimes!).  Unfuckinglikely.  Much more likely the same underlying disease process caused both.  That’s not being a drama queen or a pessimist.  That’s just looking at the numbers and being realistic.

So, no matter what happens with the surgery, I do understand (and have understood for quite some time now as I have been running around without a diagnosis) that this surgery is not likely going to be a cure-all.  There is still a subset of symptoms that are plaguing me that are likely due to yet another currently undiagnosed problem.  And I will still likely not come out of this surgery with a full diagnosis and fully articulated plan going forward either.  But getting cleared for the surgery and hopefully getting one more tumor out of the road it is a big step in the right direction.  And having Gandalf (and the wonderful people and organization that led me to him in the first place) in my corner is the biggest step forward of all.

Plan E

It has been a month since my last post.  I have been struggling physically and mentally and I have not felt like writing at all, but I have by no means stopped trying to sort things out.  Over the past month, my symptoms have continued to progress and there is now zero doubt in my mind that I have a “carcinoid” (i.e., neuroendocrine cancer).

The main reason I am completely convinced this is my main issue is that my symptoms are progressing.  I started “flushing” in December, started coughing and having breathing trouble in February.   In April, I completed the unholy carcinoid syndrome trifecta with profuse, watery diarrhea.  The first incident appears to have been provoked by a couple of servings of smoked salmon, which resulted in my spending the better half of the day in the bathroom.  Since then, I have added several other triggers to the list – yogurt made from coconut milk, eating certain vegetables raw (e.g., asparagus, beets), and beer.  All of these items are on the carcinoid banned food lists.  Sometimes I get a horrible stomachache to warn me of impeding trouble.  This is actually preferable to the times I get no warning at all before trouble strikes.  I will just leave it at that.  Other things – stress, taking a shower – seem to provoke an attack now as well.  As I have learned from other NET patients, both are common triggers.

I decided to try one last local doctor before hopping on a plane for help.  The doctor is a very well-known endocrine surgeon who specializes in MEN-1 and was recommended to me by other patients.  I am very reluctant to see another surgeon after the horrible experience I had in January.  But this time, I vow to do two things to ensure a better outcome.  First, I adjust my expectations and my goal for this appointment.  Instead of expecting to come out with an appointment for surgery, I plan to go in with the goal of just getting some guidance.  What would this doctor do if he were me?  Who would he go and see to try and get this thing diagnosed?  While I definitely asked these questions, I have already failed miserably on this goal – I want and need surgery and it is hard to go into any appointment now and not be really upset if that is not the outcome.  Hence the second goal: I was not going to make it easy to get dismissed.  I spent several days gathering and organizing all of my labs and reports and CDs of my imaging.  I took 50 pages of documents, 6 CDs, and supporting research backing up the fact that my symptoms and results (positive and negative) pointed to a NET.  I was really prepared to forcefully make my case.  I knew going in that going to see another surgeon without a diagnosis in hand was unlikely going to go anywhere and that if this strategy failed, I would go and see the Wizard.  So, I kept telling myself the worst thing that could happen was that I would get laughed at by another surgeon.   That smarts for a couple of days, but if that how things were going to go, I would just hightail it out of there and move on.  Easier said than done, but I was going to try.

The surgeon ended up being in the OR due to an unexpected surgery the morning of my appointment, so I had 2.5 hours to pore over my notes and my documentation as I waited.  M had come with me for moral support, but ended up having to leave as the appointment time got pushed back later and later.   Finally, I was taken into an examination room.

The surgeon was very pleasant and polite.  I informed him that he was recommended to me by someone he knew well and explained how I knew her.  I told him right away that I did not have a diagnosis and that I was aware that it was annoying to show up in a busy surgeon’s office without one.  But that my endocrinologist was not local and he wanted me to get a biopsy and I was struggling to get anyone local to take this seriously.  So, I wanted to get his thoughts on what I should do next to figure this out.

Over the course of the next hour, I laid out my history.  How I had surgery for Cushing’s last year and when my calcium started to increase I got into a hyperparathyroidism/MEN-1 study at the NIH.  How an Octreoscan at the NIH uncovered the very unexpected finding of an area in my chest suspicious for a neuroendocrine tumor.   How the constellation of symptoms I had been experiencing progressively over the past few months were all consistent with a NET.

He systematically went through every possible other explanation for my symptoms under the sun:  allergies, maybe I am flushing simply because I am quite fair, asthma, how do I know I don’t still have a pituitary tumor, you name it.  I had responses and supporting labs for each argument.  I showed him the pictures of my chest from the night I went to the ER along with the ER report documenting the seriousness of the hypertensive crisis.  I showed him how the Octreoscan compared with the spot that lit up on my chest.   He appeared to place a fair amount of weight on the Octreoscan, saying that he would not be thinking about my thymus if it hadn’t been for the Octreoscan.  He agreed with me that a thymic neuroendocrine would likely not show up in labs, so my negative carcinoid labs could not be used to rule out a carcinoid in this location either.  He looked at the CT scan and didn’t see an obvious tumor but thought he *maybe* saw something there as well.  He certainly never gave me the green light for surgery but did tell me he agreed that it appears I have ruled out everything except my thymus.

He told me that there were two ways to go at this point – he could recommend a bunch of additional labs or take a closer look at the imaging.  He thought the latter was the right way to proceed.  He took all of my images and told me they would be uploaded in their system and he would sit down with the thoracic surgeons and take a closer look (he would not be doing this surgery himself).  He said that they wouldn’t biopsy the thymus, they would just remove it if they decided to go down this road.  We talked briefly about the surgery.  The thymus is right above the heart in the front of the chest – a bit tricky to access.   We talked about the possibility of robotic surgery to remove the thymus and what that would entail.    He concluded by asking me to come back in three weeks – tomorrow – so he had time to review all of the imaging with the other surgeons in his group.  He did say that he understood I was very symptomatic and that he didn’t want to leave me hanging, so the plan could change before then.  He also said there was a possibility a thoracic surgeon might want to meet with me on the 27th as well.

So, I left the appointment not really sure how things were going to go from here.  He understands my predicament clearly and so do I:  I am very symptomatic and I have a positive Octreoscan on one hand.  On the other hand, the fact that there is no clear surgical target on the CT scan and the lack of positive labs (while consistent with NETs in that region of the body) don’t help confirm the diagnosis.  The negative Ga-68 scan also doesn’t help.  But it is not uncommon for some scans to be negative and the labs to be normal either.  From my perspective, the downside of removing my thymus is small – this is an organ I am not using anymore and the level of suspicion, for me, is high enough that I am confident it is there.  And I feel terrible and have been for months!  Thymic NETs can be particularly aggressive as well, so I would rather take the chance we take out the wrong organ than risk leaving it in and having it spread while we wait to confirm its presence on another scan.   It is common for MEN-1 patients with a healthy thymus to have it removed as a precaution!  I understand the downsides to surgery in the face of uncertainly though too.  I understand why a surgeon doesn’t want to crack my chest open on the off chance there is a tumor in there that he can’t see clearly.  I could have unnecessary surgery and wake up to find out that I am missing my thymus but still have carcinoid syndrome and that we were wrong about where the tumor was hiding.  I understand that a conscientious surgeon doesn’t want to put a patient in that position either (even a scalpel happy patient like me).

Neuroendocrine tumors, cancerous or benign, are incredibly frustrating on all sides.  The tumors can be very small and they are harder to see than other kinds of cancer as they don’t “light up” as clearly as other tumors do on scans.  But despite their size, they can make patients very sick because of the hormones that are secreted.  This is why patients can be very sick for years (the average delay in diagnosis is 5-7 years, as is true for Cushing’s as well) until they are finally diagnosed.  And often, by the time a patient is diagnosed with a NET, it has already metastasized to the bones, or lungs or liver.  Even tiny tumors can metastasize.  Sometimes, the primary tumor is never found, despite the fact that it has infiltrated other tissues.  If I can avoid it, if there is any possible way, I don’t want to catch this late.  I am working as hard as I can to make sure this isn’t the case, but I also have a whole new appreciation for patients that struggled for many years to try and figure out what was wrong with them before finally getting a diagnosis.

Today, I am feeling like I usually do before a doctor appointment these days – I am a bundle of nerves.  I am trying very hard not to get my hopes up but am already expecting to feel crushed when the doctor comes back and tells me he can’t help me.  I just wish he could send me an email saying “yes” or “no” so I could cry in the privacy of my home instead of blubbering in a doctor office yet again.   But I have to just suck it up and go.  Who knows?  The answer could be yes this time.  And if it isn’t, I will continue to get sicker and this thing will continue to grow and eventually I will be able to see the right doctor or gather the extra information I need to push this diagnosis over the edge.  That’s how the story played out with Cushing’s – it was a combination of seeing the right doctor and getting sick enough.  That’s how it will play out now as well.