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.

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