Taking Stock

There are a lot of things going on at once, so I am going to summarize what has happened so far and what is outstanding.

Here are the problems that have been solved:

1.  Pituitary tumors causing Cushing’s.  I had neurosurgery to remove these in February 2014.  So far, no signs of a recurrence.  Diagnosed based on symptoms, multiple labs indicating high cortisol and a dynamic MRI indicating the presence of pituitary tumors.

2.  Thymic carcinoid.   I had thoracic surgery to remove my thymus in June 2015.   Diagnosed based on carcinoid syndrome and Octreoscan indicating area in anterior mediastinum suspicious for a neuroendocrine tumor.  Some of the symptoms of carcinoid syndrome (i.e., coughing and wheezing) disappeared and others (i.e., flushing) didn’t go away but became less severe.   No tumor was identified in pathology.

Here are the remaining problems and where I am in terms of chasing them down.

3.  Need to get a second opinion on the pathology from #2 as it has to be wrong.  It just doesn’t make sense the Octreoscan lit up in this area and my symptoms got so much better post-op and there was no carcinoid in my thymus.  I’m waiting to hear from Gandalf on this, but will come up with a different plan in the event he isn’t able to help me.

4.  I still have some signs of carcinoid syndrome and so we need to figure out where the rest of this little bastard is hiding.  I am getting an Octreoscan and hopefully something will show up and allow us to form a plan to evict the remaining neuroendocrine tumor(s).  I know enough from experience and talking to other patients that the Octreoscan may very well be a dead end, as they don’t pick up all neuroendocrine tumor activity.  Not by a long shot.  But all I can do is try!

5.  I have masses in my sacrum (and I suspect maybe my pelvis too, based on some new pain I have been having over the past six weeks).  I have had an x-ray for this (but haven’t seen the results) and the Octreoscan was ordered in part to try and figure out whether these masses are metastasis from the NET in my thymus or something else.  Waiting for follow-up with the bone doctor on all the results.   My best guess is that a biopsy and/or surgery (or some sort of treatment) will be coming in the relatively near future.

6.  My parathyroid labs are elevated.  The bone doctor doesn’t think they are a sign of primary hyperparathyroidism (i.e., parathyroid tumors).  I disagree.  My plan is to keep testing to see whether the upward trend continues.

7.  I am still having some malabsorption/digestion issues.  This is almost going on a year now.  Is it because of the carcinoid?  Is it a sign of another tumor in my pancreas or somewhere else?  Is it something totally unrelated and benign?  At this point, I have no idea, but I am hoping a combination of an endoscopic ultrasound and the Octreoscan will help rule some of these possibilities in or out.  Hopefully both sets of tests will happen soon.

8.  My IGF-1 (a crude proxy for growth hormone, which is produced by the pituitary gland) keeps dropping.  The pituitary hormone TSH has also been suppressed for quite awhile.  This might be a sign of hypopituitarism, which is relatively common after pituitary surgery (see #1).  I need to get a GH stim test to determine whether the drop in IGF-1 is due to growth hormone deficiency.  I am going to see the Wizard this month to get the test and to discuss the state of my pituitary (and all these other issues).  It’s also possible that some other hormone is suppressing my IGF-1.  But this should be more clear once a growth hormone deficiency is ruled in or out.  If I am growth hormone deficient, this could certainly be at least part of the reason I still feel so crappy.

9.  I still have very bad bone pain, but this is likely due to #5 and hopefully whatever treatment I get will take care of at least some of this pain as well.

10.  I am very fatigued starting in the early afternoon.  If I don’t lay down and have a few hours of solid sleep most afternoons, I feel really ill.  This is not normal fatigue at all and it doesn’t feel like the same sort of fatigue I had when I had Cushing’s either. I don’t know exactly what is causing this, but some combination of #4-#8 are likely responsible.

11.  Are #1-#10 all caused by the same underlying disease process?  And does this mean more problems of the same nature are coming?  Probably.  But it is likely going to take time for these questions to be answered with any precision and for me to therefore get some sort of unifying diagnosis.

This is still a large set of problems.  But taking care of #1 and #2 made my life much better and I am optimistic the other problems will be diagnosed and fixed in time as well.

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:

Screen Shot 2015-06-13 at 5.57.53 PM

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.

My Stupid Bones

I am a bit of an addict when it comes to radiology and as with most addictions, mine is probably not a healthy one.  Let me start by saying I have zero training as a radiologist.  However, most of the time I am actually able to identify the right body part (full disclosure:  I have screwed this up before.  It is a mess in there!).  I have also been able to locate all of my tumors without someone showing me where they are.  Sometimes I locate problems that no one else has pointed out yet (including the radiologist that gave the official read on the scan in question).  And I think I found a problem on an MRI of my spine.

Over the course of the last 13 months, I have had really horrible nocturnal back pain.  The pain is very localized, way down in my sacrum/tailbone area.  I am almost 100% pain-free during the day, but nights are bad.  I can barely roll over in bed, I often need to get on my hands and knees and sort of back out of bed if I do have to get up in the middle of the night.   Once I am in position I can rest ok, but if I move, the pain is really bad.  Once I get out of bed in the morning and move around a little bit, it seems to go away for most of the day.  I do notice that if I have to lay flat on a hard surface during the day, the pain will come back too (I know this because I spend a fair amount of time laying in MRI/CT/PET scan machines).   I have probably had 20 pain-free nights since it first started.  I can’t talk about it without getting all teary because I am really sick of it.

I also have high bone turnover markers (which I have mentioned before), which we measure regularly by some combination of Alkaline Phosphatase, Bone-specific Alkaline Phosphatase, and N-telopeptides.  We have been looking into several explanations for why this is the case over past few months.  The Wizard recently ordered a bone scan to see if anything turned up, but it came back as normal.  Which, as is all too common for me these days, is reassuring and annoying at the same time.  I don’t want to be loaded with “hot spots” on a bone scan, but it would be nice for a scan to point to an actual explanation for the labs and my pain too!  We do know that the pain can’t be something like a disc, because it is too far down in my spine for that.  So, what the heck is going on down there?

Since I now have an extensive collection of images of my entire body, I decided to flip through my library and take a look myself to see if I could spot anything.  I pulled up an MRI of my sacrum from June 2014 and started digging around.  And I found something that looked weird to me.   On the image, it looks like two black round spots in my sacrum, right about where my back was hurting:

Screen Shot 2015-06-04 at 2.38.43 PM

I have become pretty handy with the medical imaging software and even added the little green arrows so you can quickly spot the stuff I am concerned about.  I don’t think those things are supposed to be there.  I am not 100% sure, as I am not a doctor, but this doesn’t look normal to me.

So today, I went in for my first real appointment with my new PCP.  I fired my last PCP recently, after we had this exchange, and I was worried that bringing this up at my first appointment with my new PCP might really give him the impression I was a lunatic.  So I decided to ease into the topic and just see how it went before springing the pictures on him.   Let me just say that my initial impression of this doctor is very good.  He seems very engaged and eager to figure out and explain things.  I chose him specifically because he was highly rated, a very experienced internal medicine doctor (not “just” a PCP), and because he chose to enter a practice where he had fewer patients and could spend more time with each of them.  For a complicated patient like me, all of these attributes seemed important.   My initial impression from our “meet and greet” was that he was also the kind of physician that did not mind patients that were pro-active and had a lot of questions and wanted to understand the details of medical problems and the treatments.  Also important to me.

We talked about the fact that I was having surgery next week.  He knows my surgeons and is going to touch base with them and stop by and see me post-op in the hospital too.  He certainly doesn’t have to do that but I REALLY appreciate that he is going to do that.  He had lots of suggestions for things I should be asking anesthesia tomorrow during my final pre-op appointment and I really appreciated that as well.  So then we got into my real agenda for the day – this bone pain.  I showed him my bone labs from the Wizard and told him I had a normal bone scan.  I explained the pain to him and the fact that it tended to only show up at night. I brought in the CD of my spine MRI as well and asked if he wanted to take a look.  Unfortunately, he couldn’t load it on his computer right there in the office.

So….I paused and told him that I had looked at it myself and I thought I saw something.  I know, doctor, I am not a radiologist, but I took a screen shot of my MRI and I have it right here on my iPhone.  Want to take a look?  I cringed inside as I asked him (OMG he is going to think I am a lunatic, OMG I am a lunatic).  But to my surprise, he said “Sure, let me have a look!” After a scramble to dig it out, I showed him the above image and…he agreed with me! That’s not normal! Then he went a step further.  He pulled out an anatomy book and gave me an education on my sacrum! I could not have been happier.

First he showed me a picture of what the sacrum looks like from the back:

Screen Shot 2015-06-11 at 11.00.36 AM

So, you can see the sacrum does have two rows of holes in it.  Those holes, the sacral foramina, are where the nerves run through the sacrum.  I had actually seen these holes before when I was educating myself on the sacrum when trying to figure out if that is what I was seeing, just from a weird angle.  So, I was glad to have him walk through this with me.

Then he pulled up a picture of my sacrum from the side.  I will put the picture of my MRI below for comparison purposes:

Screen Shot 2015-06-11 at 11.00.46 AM

Screen Shot 2015-06-04 at 2.38.43 PM

The main point, if you compare these images, is that the black circles in my MRI are not in the same location as the foramina.  The sacral canal, where the foramina are located, is above and to the right of my black circles.   Comparing the same two MRI views that match the anatomy pictures, you can the black circles are in a different location than the foramina:

Screen Shot 2015-06-13 at 5.57.53 PM

The green box shows the black circles from the back view (the left image) and the side view (the right image).  The foramina can be seen in the left hand side view.  It is the larger dark circle to the right of the green box, as indicated by the green arrow.  Those black circles in the green box aren’t supposed to be there.

The next question of course is what the hell are those things then?  The short answer is that we don’t know yet.  My current theory is that my high bone turnover markers, my pain and this MRI all mean bone tumors.   I would be lying if I said bone metastasis hadn’t crossed my mind – nocturnal bone pain like mine and the bone turnover markers would be consistent with that.  And NETs like the one we are planning to evict next week often spread to lungs, liver and bone (and I have “stuff” in all those locations).  I am not saying that to be melodramatic or cause anyone to lose sleep, me included.  But the whole point of this blog was just for me to lay out what is going on and what I am thinking.  And this is what I am thinking.  It could be nothing (that’s not likely though, let’s be honest).  It could be something.  I really don’t know.  All I know is that this shit needs to get sorted out one way or the other.

And my new favorite PCP agrees – this needs further investigation.  So, the plan is to see where we are at when the dust settles after surgery next week.  And after the dust settles, he is ordering a new MRI of the spine (as this one is already a year old) and he is sending me to a highly regarded bone doctor.  He is starting to get that referral in place now, in case there is a long wait.    As usual, I am half worried and half relieved that someone is listening to me and taking this stuff seriously.

Let me tell you the other thing that made me want to run around my new PCP’s desk and hug him.  When I described my bone pain to him, he questioned me about it then concluded:  “That is so strange that your bones only hurt at night.  It makes no sense to me.  But you aren’t crazy, and if you say it only hurts at night, it only hurts at night.  I just can’t give you an explanation for WHY that is the case.”  AHHHH THANK YOU!! Thank you for just listening to what I am experiencing and believing me. Thank you for not telling me that my experiences can’t be right or that I must be experiencing something else.  It sounds so obvious that this should be the way things actually work in doctor-patient relationships.  But so often it doesn’t.

The big question you might have on your mind right now is the following: you had this MRI a year ago because of bone pain.  What did the radiology report say?  Didn’t it mention anything?  Well, here is what happened last year.  PCP #1 (I am now on PCP #3) ordered the MRI when I told her about my bone pain in May 2014.  Here is what she emailed to me.  This is a verbatim cut and paste from the summary of the radiology report.

     No evidence of sacral fracture.
     L5-S1 degenerative disc disease as above.

OK, that’s good news.  My sacrum isn’t broken.  I knew I had disc problems too, so that wasn’t a surprise.  But since the MRI didn’t appear to provide any information that would be useful in helping us sort out my bone pain, I decided I would just go and look at the report myself.  And here is what the rest of the report actually said.

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OK, now that is just weird.  The report is claiming I have “numerous surgical clips present” but to my knowledge, I have never had back surgery.  The report seems to be saying further that there is no evidence to tell them how the clips got in there.   Is it just me or does this not make sense?  It seems kind of clear to me at this point that my PCP either never read the full report OR she didn’t find it puzzling that I was full of surgical clips and complaining about back pain and never mentioned a prior back surgery to her.  So I emailed her to say “what’s up with this report and can you please have the radiologist look at this again because this doesn’t make any sense.”  So she did.  The radiologist in turn corrected my report so that it now says:

Screen Shot 2015-06-11 at 12.05.18 PM

OK.  So my back is NOT full of surgical clips.  This is good news to me, as I could take “alien abduction and resulting back surgery” off the differential diagnosis list.   Now I’m told it is “probably mineralization.”  And I don’t really know what that means or if it is harmless or significant and no one bothers to explain it to me.  I have never managed to actually locate anything that looks like a surgical clip in my MRI, for the record, so I am still not even sure where this mineralization is.  All I have been able to locate is those black circles.  But it is really disturbing to me that the initial report was so bizarre, that this MRI was read TWICE because the patient pointed out the report didn’t make any sense, and despite all of this no one managed to see or comment on what appears to be a problem so obvious an economist can spot it on the MRI.

The moral of the story dear reader:  AUDIT EVERYTHING.  And don’t stop digging until you know what is wrong with you.  I am mad at myself for not pushing someone harder to look at the MRI for a THIRD time LAST YEAR.  I am mad at myself for not sitting down with my own CD of the MRI and making sure it was normal.  In my defense, I was sick and this was one of many problems I was trying to figure out.  And it was also clear my PCP was getting fed up with me for pestering her with this when it was clear she believed there was nothing wrong beyond old age and back pain that goes with that territory.  AND IT WAS THE RADIOLOGIST’S JOB TO READ THE SCAN PROPERLY, NOT MINE.  But what is done is done.  All I can do now is keep pestering anyone who will listen – and fire everyone who won’t.

Let the Countdown Begin

I just got cleared for surgery!   Next Wednesday, the 17th of June, my thymus is coming out.  The last six months have been really brutal and I fought every day to get this figured out and for someone to take me seriously.  But I made it.

I just want to say a thank you to those of you that just kept showing up when this got hard.  Especially M.  Every single day, you just kept showing up.  You believed in me and supported me and you didn’t give up on me when so many others did.  You always did and said the right thing.   And thank you to The Coven and the Wizard and a few others too that didn’t tell me to stop being a whiner, didn’t hint that maybe nothing was wrong with me.  You patiently went through test results, millions of pictures from my scans that I kept sending you, and listened to my litany of complaints and frustrations (including, literally, months of talking about my shit).  You just kept showing up.  In the end, that’s all people really need – just someone who keeps showing up when the going gets tough.  I won’t ever forget it.

I don’t know what will come next – if the surgery will work, if it does work how many problems it will fix, what the next phase of treatment will be (if there is one).  But right now I am just very damn happy I made it this far.