The Aftermath of Surgery: Part III

My follow-up with Gandalf has finally arrived.  Gandalf is a renowned endocrine surgeon who got me in the hands of the thoracic surgeon and cleared for surgery.

I was really looking forward to this appointment, partly because I had a tonne of questions and partly because I wanted him to see how much better I was and to say thank you for pushing forward with my case in the face of some uncertainty regarding the CT scan.   After giving him a quick update, we launched into my questions.  Here were the questions and his responses.

1.  What should we make of the pathology report?  He told me the thoracic surgeon let him know I wanted to send my slides somewhere else for a second opinion, so I asked him what his thoughts were on the pathology.  He agreed with me that a second opinion was a good idea.  For the record, so does the Wizard. He agrees it would be much better if we had a “smoking gun” and that we should try and get one, especially since the surgery does appear to have done something.  Furthermore, he had thoughts on where we should try and send it and even offered to help facilitate the process.  I JUST LOVE THIS DOCTOR.  Stay tuned on this one.

2.  What does Gandalf make of the fact that some symptoms are gone, some are better, and some are unchanged?  The most likely outcome is the thymus had to go but we didn’t get all of the carcinoid.  The plan from here is to do another Octreoscan and hope something shows up.   He could clearly see  by observing me in his office before and after surgery that, just as I report, some of my symptoms were gone and that the flushing had changed but was still there.  He didn’t have to take my word for it, he could see it all for himself.

I mentioned to Gandalf at this point that the bone doctor did order an Octreoscan – did they coordinate on this?   It turns out it was news to Gandalf that I had seen the bone doctor, so I filled him in on what happened at my last appointment, including my parathyroid labs.  All of this came as a surprise to him and he seemed pretty intrigued as he looked at my labs.  I also showed him the same picture of my MRI and he had some questions about what the radiology report had said.  I relayed the information from the radiology report to him as well.   He paused and asked me if the MRI was conducted at his hospital. I was relieved to tell him that it was not.

3.   Now that we talked about the parathyroid labs, what did he think about them?  He didn’t say I had hyperparathyroidism, but this was clearly on his mind, as he told me we need to follow the trend in the labs, that some of my other labs were also consistent with hyperparathyroidism and furthermore that hyperparathyroidism could cause “brown tumors” in bone as well, which he speculated might be what the bone doctor is looking for.   He also told me that the bone doctor was the perfect person to be seeing for these issues and that he would be coordinating with the bone doctor going forward.  Gandalf does parathyroid surgery among other things, so at this point I can’t help but think he will be handling one for me in the not too distant future.  But we shall see.

4.  The other big issue unresolved issue on my list was my GI problems.  I have been hoping it was just the thymic NET preventing my food from getting completely digested, but this doesn’t seem to have improved since surgery.   As a result, I am getting increasingly worried about my pancreas, which is another common location for NETs.   Given the pancreas plays a big role in digestion, this wasn’t a crazy place for my mind to go.  Pancreatic tumors can be difficult to see on the sorts of scans I have already had and I knew that the “gold standard” for imaging the pancreas for MEN-1 patients was an endoscopic ultrasound.  So instead of beating around the bush, I just flat out asked if he would refer me for one.  He asked me what I had tried at home to fix my problems and I told him the truth – not much.  I didn’t really know what to try and given all the other problems I had been actively trying to address, I had put this on the back burner.   He asked me to try and add more fiber (Metamucil) for a month but then said if it didn’t work within two weeks it probably wasn’t going to work.  If adding fiber didn’t fix the problem, he would refer me for a GI evaluation.  This seemed very reasonable to me.  He told me that, in fact, he had a MEN-1 family visiting from out of state this very day and that an endoscopic ultrasound was part of the work-up these family members had today as well.  So we were on the same page yet again.  I really will feel much more at ease once we take a look at my pancreas, one way or the other, so I am glad he is open to it as well.

5.  Finally, he asked us if we had any other questions or concerns, so  M brought up the fatigue.  I still require a solid 12-14 hours of solid sleep to feel decent during the remaining 10-12 hours in the day.   When M told him that it was truly shocking to see how deeply I slept during the day, he simply said “I don’t think we got it all,” referring to the thoracic surgery I had last month.  This was our fear as well.

Gandalf gave me his email address and we agreed to stay in contact via email while the various issues get handled.  There are several irons in the fire now – the second opinion on pathology, the Octreoscan and whether there is NET remaining after my thoracic surgery, the masses in my sacrum, my parathyroid labs, and likely a GI evaluation and endoscopic ultrasound of my pancreas.  Hopefully these irons will allow us to get to the bottom of most of my remaining issues.  The only other item left on the agenda is my abnormal pituitary labs and I will be following up with the Wizard on those and will be reporting back on this soon.

It’s still a long list, but we are getting there.

The Bone Doctor

Following my recent appointment with my wise new PCP (hereafter Yoda), I was referred to a doctor that specializes in bone metabolism.  This appointment happened to fall between my follow-up with the thoracic surgeon and my next appointment with Gandalf.  Now that the surgery was over and I could breathe and talk again, my bones were moving up on the list of priorities so the timing seemed perfect.   I had enjoyed a nice break from bone pain while I was on heavy painkillers after surgery and now that I didn’t need drugs for surgery recovery anymore, the back pain had returned with a vengeance.  I was ready to check this bone shit off my medical to do list.

M and I went into the appointment armed with a list of questions, my bone labs from the past year and the pictures from my MRI (I had already given Yoda the CD).   M and I first spent a long time with the bone doctor’s fellow, who documented all of the crap that had been going on over the last couple of years – Cushing’s, the carcinoid, the bone pain and labs and the whole pile of other symptoms that were as of yet unaccounted for.   She was great.  We had seen A LOT of fellows over the past couple of years and she stands out as the one that did a lot of listening and asking questions as opposed to telling me how my symptoms didn’t fit Cushing’s or didn’t fit carcinoid or whatever other diagnosis was on the table.  I am going to bet money that she is going to be the kind of doctor that is looking to figure out what is wrong instead of just focusing on what isn’t wrong once she is done with her training as well.

We really liked the bone doctor too.  I don’t know if it is the fact that I now have a paper trail and some “street cred” at this hospital (between Yoda and Gandalf and the surgery) or if I am just on a roll now but the bone doctor seems really great too.  The Wizard, Gandalf, Yoda, now this guy.  I feel like when I see a doctor lately, I am not being treated like a crazy hypochondriac or a woman in her 40s with unrealistic expectations about what getting older feels like.   He tells me he already received a detailed note from Yoda.  He reviews my history with me and agrees there have been a lot of major endocrine problems.  He confirms with me that the NIH concluded I did not have MEN-1 and he tells me he is very glad to hear that (I am not convinced MEN-1 is off the table, obviously, but I keep my mouth shut about it).  He sees all the labs and agrees there is a problem.   He and the fellow even agree my calcium is too high.  It is not crazy high, but too high for my age.  We go to look at the MRI but it isn’t uploaded in the system, so I show him the same image I showed Yoda:

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When he sees the images he simply says “Wow. Wow. Wow.” then passes my iPhone to the fellow and says “You need to see this.”

He confirms that there are masses in my spine.  He tells us the good news is that the sacrum is a large bone and this means it may be possible to remove them surgically.   He believes this could be the cause of both my high calcium, my high bone turnover labs and my pain.  When he says to me, after looking at the pictures “No wonder you are in pain – this has to hurt” I start bawling all over again (I do a lot of this these days).   Yes! The pain was BAD.  I had been trying to tell doctors this for months and months and months and with the exception of the Wizard all I kept hearing is “well, you are getting older/it is probably arthritis/you are just getting deconditioned” and all this other dismissive shit.  I KNEW this pain wasn’t just getting older.   This pain was BAD and it was every fucking night.  I couldn’t roll over in bed or get in and out of a chair or bed without pain.  It was such a relief to have someone just validate what I had been feeling all these months that I cried.  I am not crazy.  I am not a baby.  I have been hurting.  The pain is REAL and it has a real cause too.  I felt like the bone doctor just told me he was going to let me out of prison after being trapped in a shitty cell for the last year.  If you don’t think it is a relief to have a specialist confirm you have painful tumors in your spine, then you need to try and live in my piece of shit body for a year first.  I think there is a good chance you might change your mind.

The bone doctor subsequently orders a large set of labs (parathyroid, calcium, Vitamin D and a few others I had never seen before) and new imaging (an X-ray and a bone scan).  The labs and X-ray I complete that day.  He tells me he wants to see me back soon and he gives me a note for his staff to double book me for an urgent appointment in two weeks – just enough time to get the results back.  I don’t know the results of the X-ray, but I do know that nuclear medicine called me a week ago to change the bone scan to an Octreoscan (in case you forgot, this is a special scan designed to locate neuroendocrine tumors).  I am not sure if it is because he has talked to Gandalf and is therefore coordinating with him (Gandalf wants an Octreoscan too) or if it is because I had a recent bone scan (ordered by the Wizard a couple of months ago) or if there is some other reason.  He has given me some recommendations for medications and other treatments to help manage my pain (one of which is to simply take something that knocks me out so I am unaware of the pain at night) and they are helping.

And the labs have come back now as well.   The lab results are extremely interesting along two dimensions.  First, my bone turnover is starting to get better.  I don’t know exactly why this is the case, but there are a few explanations.  One is that the Wizard has been progressively dropping my thyroid meds in our little experiment so maybe being hypothyroid has slowed the bone turnover to some extent.  Another very interesting finding from my ongoing research is that excess histamine causes high bone turnover!  Histamine!  I never would have guessed this one in a million years.  Is it possible that the excess histamine I was obviously suffering from pre-thymectomy was at least partially responsible for my bone labs as well?  Now that the thymus is out and the excess histamine is clearly gone (I haven’t touched an anti-histamine in weeks), is it possible my bone turnover has slowed down as a result?   It will never cease to amaze me how excess hormones can mess up your body in the most unexpected ways.  I am very curious to ask the bone doctor about this at our follow-up appointment.

The more interesting finding in the labs ordered by the bone doctor is that my parathyroid hormone (PTH)  TRIPLED since it was last measured in April.  My calcium stayed high and now my PTH tripled.  I just can’t get over this.  High calcium and high PTH together are the basis for a diagnosis of…..HYPERPARATHYROIDISM.  That’s parathyroid tumors, exactly what I had been let into the NIH study for way back in January.  January! It had been ruled out then and subsequently put on the back burner by me when the carcinoid syndrome became severe throughout the winter.  But in my mind, parathyroid tumors had not ever been ruled out.   My Vitamin D 1,25 was also high, which can also be a sign of hyperparathyroidism.

So what did all of this mean?  I would have to see what the bone doctor, the Wizard and Gandalf had to say about all of this.  But I did know this:  an official clinical diagnosis of MEN-1 was based on the presence of tumors in TWO of the three “P’s” (pituitary, parathyroid, and pancreas).  I had confirmed pituitary tumors.  If I had parathyroid tumors – which were diagnosed by the presence of high calcium and high PTH – then I would automatically be diagnosed with MEN-1.   Neuroendocrine tumors in the thymus and other locations (like what I believe we had already just taken out not even a month ago) are part of MEN-1, but are not diagnostic for MEN-1.   But parathyroid tumors would complete the diagnosis.

Would my new labs be enough to confirm hyperparathyroidism?  Would they at least raise the suspicion for MEN-1?  Would I finally get a diagnosis and therefore start getting followed and screened for this disease without having to fight tooth and nail for every test?  And what was the conclusion going to be about my bones?  What exactly are these masses?  Are they metastases from neuroendocrine cancer in my thymus?  From another location?  Are they “brown tumors” from hyperparathyroidism?  Are they a sign of some other problem that isn’t even on my radar yet?  And what can be done about it?

As I begin telling those close to me about all of these results, I quickly realize that this news is upsetting to people.   People are worried and upset there are signs of more tumors, new tumors, etc.  And while I can understand the news is upsetting to everyone else, and of course it is concerning to me too, I can honestly say this is the happiest I have felt in a long time and the reason is simple: I ALREADY KNEW I WAS STILL SICK.  I have been living with a pile of problems – problems from Cushing’s, problems after Cushing’s.  I am in pain and wiped out all the time.  I know some people look at me and think I don’t look sick anymore (although I certainly don’t look like myself, that much is clear) and they wonder why I don’t go back to work or speculate amongst themselves that I am just soaking this whole “being sick” thing.  Trust me.  Spending half your day in bed every day, not working, being in pain, not being able to do a fraction of the stuff I used to be able to do is no life.  I don’t want to live this way!  But I can see it in their eyes and hear it in their questions.  People think they are being very subtle, but I am not stupid, I can read between the lines and it makes me so fucking angry sometimes.  At the same time, I know/feel like I have recovered from some of my medical issues, but I never felt that I had recovered.  Not even close.  I have been sitting here dealing with the knowledge that I am still sick and had been struggling like hell to figure out what else is wrong and how to get it fixed for a long time.  Now, I can pass some of this worry and fight over to a set of doctors who I actually believe can help me.   This is all good news.  The more abnormal results come in, the faster this is going to get figured out and fixed.

I am finally starting to feel a little optimistic again.   Finally, things were starting to fall into place.  This was going to get sorted out.  I was going to get treatment.  I was going to get better.  Maybe not in the next month or six months, but there might actually be a day in the next year when all of this shit was going to get surgically removed and/or treated.  Maybe I was actually going to be healthy again.  I am a bit scared to think about that possibility too much yet.  But it was hard not to get my hopes up about it a little bit.

The Aftermath of Surgery: Part II

As usual, nothing is black and white with me and this disease.

I saw the thoracic surgeon two weeks after my surgery.   She asked me how I had been feeling and I reported to her what had clearly become my new post-op norm:  I stopped coughing and wheezing completely and I was still flushing, although the flushing was much better than before.  She then told me that the pathology report was “interesting” and made a bit of a face:  the pathologist did not find a tumor.   She was completely surprised and kept saying that she didn’t think we got it and that I might need surgery again.  I told her I was not that surprised by the pathology report.  In fact, I had told M the previous night I thought there was a 70 percent chance the pathology report would read this way.    After all, no tumor was clearly spotted on any of the CT scans and the surgeon didn’t see a clear tumor during the surgery either.  That didn’t mean I believed I had surgery for nothing!  The Octreoscan and my symptoms still meant something.  It just meant, in my mind, that the tumor (or tumors) were very small.   And, given some of my symptoms resolved completely after surgery, I knew something bad must have been removed during that surgery regardless of what the pathology report said.  I said exactly this to the surgeon and then we had a very circular conversation in which I would say “I stopped coughing and struggling to breathe – you had to have taken something bad out” and she would reply “I never touched your airway, we are going to do another scan and if we see anything I will go back in and take it out” and I would say “but I stopped coughing…” followed by “but I never touched your airway” and on and on.

Finally I just told her that I wanted a copy of the pathology report and I wanted to get a second opinion on the pathology because there was no way that it could be right and I asked her how I could go about having the specimen sent somewhere else.  And I told her that she should sleep well at night knowing whatever she did in there made life in my body much more bearable and I was very grateful for that.   She said she was sending me back to Gandalf for follow-up, they had already discussed in detail and had planned to let me heal a bit more and then do some more scanning and that she might see me back again depending on what the scans said.

A year or two ago, I would have received this news and concluded that I just had surgery and no tumor was removed.  At this stage, however, I have a whole new perspective.  I understand that a pathology report is not THE TRUTH.  A pathology report is the summary of the analysis performed by a single human being on a specimen.   That is all it is.   Until you get a rare disease (or any other disease for that matter) and thrust into this world of tests and scans and specialists, it is all too easy to stick your head in the sand and forget that medicine is not an exact science and doctors are human beings and very little about getting sick, diagnosed and (hopefully) better may be black and white.  That’s scary but that is THE TRUTH too.

I read the report as soon as I got to the car and cried all the way home as M drove.   Yes, I was expecting this sort of stupid report.  Yes, I still believed that no matter what the pathology report said, my thymus had to come out and it was now out. Yes, no matter what the report said, I felt better.  But why couldn’t things be black and white, just once?  Why couldn’t that stupid report have just supported an actual diagnosis?  I am so sick of fighting to get stuff figured out.  Sick of getting second and third and fourth opinions.  And I am left feeling simultaneously angry at the pathologist and sorry for him at the same time.    How could he not find something?  Did he look for ten minutes and then just give up?  Is it possible he didn’t recognize tumor tissue that was staring him in the face?  Why didn’t he try and stain the tissue anyway?  Or was he frustrated too?  He was sent a specimen labelled “intra-thymic carcinoid” and he can’t find anything.   He’s probably never seen a thymic carcinoid before – very few pathologists have.  And now he can’t find it.  Has this ruined his day just like it is ruining mine?

Even though this was the outcome I expected, I was still frustrated beyond belief.  I just want a damn diagnosis.  I want to be able to say “I have X” instead of having to make these qualified statements “We think I have neuroendocrine cancer because of A, B and C; however it hasn’t been confirmed by D and E.”  But at some point, I realize this is unnecessary.   I am stuck in the past, when my diagnosis WAS being questioned all the time and I need to let that go.  No one is doing that now.  The Wizard is not questioning it.  Gandalf is not questioning it.   The thoracic surgeon is not questioning it either.  “Carcinoid” and “carcinoid syndrome” are all over my chart.   The diagnosis is not being questioned.  All that is being questioned is whether we got it out.

The more troubling piece, besides ripping open some psychological wounds from bad interactions with doctors over the past few years, is that without proper classification of a tumor there might not be a clear treatment plan.  Neuroendocrine cancer, like other cancers, can vary in aggressiveness.  There are a number of analyses that need to be conducted on the tumors by the pathologists to classify exactly what kind of cancer one is dealing with and this analysis can have important implications for treatment post-op, just like it does for other cancer patients.  So, “no tumor identified” is not just annoying.  It makes the path forward for treatment very unclear.   All the more reason to figure out a way to get a second opinion on the pathology.

One interesting item on the pathology report is that the specimen contained a “fibrotic area” and some “mild follicular hyperplasia” and I am not sure about the significance of either.  The former, in particular, seems quite interesting as I discovered there is a large medical literature on carcinoids and fibrosis.  For example:

In particular, the literature indicates that the hormones secreted by the tumors can cause fibrosis locally (i.e., in the location of the tumor) or distally (i.e., in a location other than that of the tumor).

So, could this “fibrotic area” mentioned in the pathology report be indirect evidence of a carcinoid?

As usual, I have so many questions and so few answers as I wait for the next doctor appointment.

The Aftermath of Surgery, Part I

I had thoracic surgery to remove my thymus almost a month ago and a lot has happened since then.

I was very nervous going into surgery, not because of the surgery itself but because of two anesthesia complications introduced by the tumors.  First, since having pituitary surgery last year, I am not able to make large amounts of cortisol on short notice for stressful situations such as a major surgery.  Therefore, it is necessary to receive steroid “stress doses” to avoid adrenal crisis during surgery.  Second, a carcinoid crisis, whereby neuroendocrine tumors can secrete toxic levels of hormones when the body is exposed to stress, can also be triggered by surgery.  Adrenal crises and carcinoid crises can each be fatal if not treated appropriately and swiftly. And neither are very common, so given both were issues of concern for me, I was very nervous about whether anesthesia was going to be prepared.  I printed off materials and took them to the pre-op appointment that documented the drug protocols I hoped would be followed to help things go smoothly.  The anesthesiology resident I saw at the appointment was beyond excited.  So many interesting and unusual problems all in one person (I am also missing the enzyme required to process a family of paralytics used in surgery in addition to the endocrine problems).  She informed me I covered several of those rare cases that show up on medical exams but doctors never actually get to see in practice.  As a result, she also understood why I wanted to make sure all the right protocols were followed.

I knew I wasn’t going to be able to relax completely until I talked to the doctor that was going to be overseeing all of this the day of surgery.  So as M and I packed up to go to the hospital the morning of surgery, I triple checked we had all the protocols with us just in case I was going to have to argue with someone about it that morning.  I was put at ease as soon as I met the team that morning.  The nurses in the pre-op area told me I was the “patient of the day” and that my case had been the major topic of discussion all morning and that all sorts of special preparation had been taking place. Teams of doctors and medical students were walking by and commenting “that’s the carcinoid patient” and pointing at my bay.  The anesthesiologist on my case specialized in complex cases as well and he spent a lot of time with me. We discussed the protocols in detail and he ensured me that he not only had devoted his entire morning (my surgery was at 11) to my case, but that he spent the prior evening studying up on it as well.  Infusions of octreotide and solucortef (the drugs used to prevent carcinoid and adrenal crises, respectively) were going to be started before surgery and continue through to the day after surgery.  Extra IV lines – arterial and central lines – were being put in place in case I did go into crisis and needed large amounts of fluids fast.  After hearing all this, I could relax.  I felt like I was in good hands.

Arterial lines are not a big deal, but because (as the name suggests) they go into the artery, there is a bit more of a production getting them in (and they are definitely more painful and messy!).  The anesthesiology resident first attempted to place the line but didn’t succeed.  So an ultrasound machine was brought out so she could try and guide the IV in to the artery with a better view. This didn’t work either, so the attending anesthesiologist was brought back.  He tried a third time, also using the ultrasound to try and guide it in.  At this point, I am uncomfortable and ready to have this over with, but calm – it is certainly not the end of the world.  But the anesthesiologist looks over at me and stops and starts asking me if I am ok.  I told him yes, totally.  But I have apparently become very red – of course the stress has made me start flushing, which I am used to by now but he isn’t and it has freaked him out. He keeps asking me, over and over, “do you feel ok?” and I keep saying yes.  But he immediately stops trying to place the line, announces “we are giving her a break” and cleans up and leaves the room. He is pacing around about 30 feet away and looks stressed.  Everything gets put on hold for about an hour.  I haven’t seen M for a few hours now and now I am calm but afraid.  The fact that the tumor is acting up before we even get going is worrying me.  It’s hard having so little control over what your body is going to do.  I just want to see M for a few minutes before I go in, but given the flurry of activity, they want him out of there until they are ready.

I finally get to see M and return to a normal color and off I go.

Surgery went very well. There were no complications – which I credit completely to a very well prepared team of doctors.   I have to say, having dealt with my share of incompetent and/or uncaring doctors over the past few years, I can recognize and appreciate a competent and caring team when I see one.   The thymus and all surrounding tissue was removed.  The surgeons looked at the nodule in my lung and determined it looked like a lymph node and decided to leave it in there.  They did not see a clear tumor in the process of removing my thymus, but the entire specimen was sent to pathology.

So the big question post-op was whether the surgery caused the carcinoid syndrome to go away.  The answer, as usual, was not black and white.  My coughing and wheezing went away completely, even when I stopped taking anti-histamines.  My flushing and diarrhea did not go away.  The pattern of flushing did change – it is more pink than purple and more diffuse than before.  But it is still there and still triggered by the same things.

At this point, my best guess is that this means my thymus had to come out and removing it fixed some but not all of my problems.  For now, I decide to focus on recovering from surgery and to just wait and see how my symptoms evolve, what the pathology report says, and what the surgeons have to say when I see them in a few weeks’ time.