Taking Stock: October Edition

Here’s what is currently taken care of and still outstanding.  The to-do list is slowly getting shorter!

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.  My coughing and wheezing stopped completely post-op and have stayed away over the past 3 1/2 months, despite the fact that the pathologist didn’t find a tumor AT ALL.  My pathology has been sent away for a second opinion.  I am still flushing and the flushing is triggered by the same set of things, but the flushing is much more mild than what it used to be.

3.   I had a repeat Octreoscan in August.  The Octreoscan was read as negative, but I saw some spots lighting up near my sacrum and my liver looked “mottled” during the 24 hour scan (the latter according to the radiologist).   I went to see some out-of-town neuroendocrine experts about my situation.  They changed the read on the Octreoscan from “negative” to “positive” according to their nurse (I haven’t spoken with the oncologist myself since seeing them).  They also read the 2014 MRI of my sacrum as “abnormal” and ordered a new MRI.   The bone doctor agreed that the Octreoscan and MRI didn’t look normal to him either.  But so far, I don’t think anyone yet knows what the significance of the findings are.

4.  I had parathyroid surgery and a hyperplastic gland removed September 2015.  To everyone’s surprise, I also had a thymic mass removed (along with 14 lymph nodes)!  Pathology came back as normal thymic tissue (of course) and I don’t feel any different, but it is interesting to me nonetheless.   New labs to see where my calcium and PTH are now that surgery is over are pending.

5.  I let the NIH know what has been going on, as I was part of their MEN1/hyperparathyroidism study.  The genetic counsellor at the NIH confirmed that I meet the clinical criteria for MEN1.  They want to see me back there if my calcium starts to creep up again.

6.  My IGF-1 was dropping steadily over the course of the last six months, so the day after I had parathyroid surgery, I had a glucagon stim test for GH deficiency at the request of the Wizard.   The pituitary surgery I had last year can cause permanent damage to the pituitary and lead to a decline in the hormones secreted by the pituitary as a result.  Since GH is one of the first pituitary hormones to be affected if the pituitary is damaged, and since IGF-1 is stimulated by GH, doing the stim test made a lot of sense.   It was pretty unpleasant getting this test the day after surgery (the doctor administering the test told me I was very brave before we started and now I know why – it made me pretty nauseous and required repeated blood draws over the course of four hours on a day I really would have preferred staying in bed).  But it is over and I am glad I did it.  The good news is that I passed the test – it doesn’t appear that I have GH deficiency, which hopefully means my pituitary is functioning just fine.  So why is my IGF-1 so low?  This is far from confirmed, but it does increase my suspicion that I have too much somatostatin (which inhibits IGF-1, TSH, glucagon, gastrin – all of which are too low based on my labs).  Somatostatin can be produced by NETs (and we suspect I still have one) and too much somatostatin can also cause malabsorption (which I have as well).  Hopefully a biopsy or some other future testing will shed some light on this issue soon.

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

7.  I am waiting for either the bone doctor or the NET experts to tell me what the abnormalities on the MRI and Octreoscan mean (if anything).  The new MRI was read locally as clear of masses, but the new MRI looks worse than the one I had last year (the bone doctor has seen the images and agrees what we see certainly look like lesions) so I will be curious to hear what the experts have to say about this.  I was told the most likely next step was going to be a bone biopsy to see what these things are.   For now, I wait for everyone to get back to me with their official thoughts on the scans.  I have more pain and pain in new spots that also look suspect (to me) on the MRI so I am anxious to get this sorted out.  Stay tuned.

8.  I am still having some malabsorption/digestion issues.  This is going on over a year now.  The good news is that Gandalf is back (yay!!!) and has referred me to a GI doctor that he describes as a “very careful and thorough diagnostician” which sounds like exactly what I need.  This GI doctor is in high demand and so I have a bit of a wait until I will get to see him, but I very happy the ball is rolling.

9.  I found what looks/feels like a little growth behind my ear.  Getting a dermatology evaluation for this soon.  MEN-1 patients tend to get lipomas, which are benign growths under the skin.  Based on my reading, it looks and feels like this is what it could be.  Let’s see what the dermatologist says.

In terms of symptoms, I am left with some relatively minor flushing (as long as I avoid stress), my GI issues, quite a bit of fatigue, and some growing bone pain.   All could easily be explained by my ongoing problems, I think.  Except for this thing behind my ear, NO NEW PROBLEMS!!!  For so many months, going on years now, I feel like my body turned on me and waged a multi-pronged attack on itself.  I finally feel like I am gaining on it now – removing problems at a faster rate than they are piling on.  I feel somewhat optimistic there will be a day in the foreseeable future where I might not have new problems to fix for awhile.

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Little Richard

A little over two weeks ago, M and I flew to Los Angeles from Boston for my parathyroid surgery.  The day before surgery, we got to meet the surgeon (hereafter Merlin) that I had been corresponding with and doing tests for since February.   Merlin (and his staff) is just as personable in real life as he has been over the phone and via email and it is really nice to finally meet him.  We talk in detail about my labs, my symptoms and my other endocrine problems.  He discusses with me all of the potential outcomes of the surgery, good and bad – he could go in and find none of my parathyroid glands are bad and we need to continue to search for an explanation for my labs and symptoms, he could find all of my parathyroid glands are bad and need to go, he could find anything in between.

Merlin decides he would like to do an ultrasound of my neck to see whether he can locate any offending parathyroid glands prior to surgery.  As is no surprise, having a clear target before you slice someone open is helpful!  It takes Merlin all of 30 seconds to locate Little Richard, my big fat rogue parathyroid gland.   He showed M and I the images from the ultrasound – my thyroid, this oblong black area attached to the thyroid that is Little Richard – and he explains why he thinks the black area is a parathyroid adenoma.  Interestingly, Merlin only sees one area that could be a parathyroid adenoma as he continues the ultrasound on other regions of my neck.  The reason this is interesting is that I had an ultrasound in Boston many months earlier, as well as a specialized parathyroid scan called a Sestamibi scan, both to look for parathyroid adenomas.

Sestamibi scans (and ultrasounds for that matter) are notoriously inaccurate methods of locating parathyroid tumors.  A well-known parathyroid surgeon, Dr. James Norman, claims:  Scans cannot find 50% of parathyroid tumors and when they do, they are wrong 50% of the time.  His writing on this topic really is worth a read:

http://www.parathyroid.com/finding-parathyroid.htm

What Dr. Norman describes on his website is remarkably consistent with my experience.  I was one of the 50% that had a negative Sestamibi scan.  The other interesting point that Dr. Norman makes, is that the results of an ultrasound are only as good as the person conducting the ultrasound!  This is kind of a scary truth to hear, but an important one.  And it is totally consistent with my experiences as well.  I had an ultrasound in Boston at roughly the same time I had the Sestamibi scan at a well regarded teaching hospital.  Here is an excerpt from the ultrasound report:

Screen Shot 2015-09-23 at 8.32.31 AMThat’s right.  There were EIGHT different spots that the doctor conducting the ultrasound thought could either be parathyroid glands (and parathyroid glands are typically only observed on ultrasound if they are hyperplastic or have adenomas because a healthy parathyroid is about the size of a grain of rice and therefore too tiny to see on an ultrasound).  And despite the fact that there were EIGHT areas that could be an enlarged parathyroid glands, the ultrasound was read as normal BECAUSE THE SESTAMIBI SCAN WAS NEGATIVE.  I thought this was a weird conclusion at the time.  If the Sestamibi scan was the only scan that mattered, why bother doing the ultrasound?  Wasn’t the point of the ultrasound to catch problems that the Sestamibi scan might have missed?  I have never heard of one scan being declared negative because an entirely different scan was negative.  That is exactly what this doctor does, however, many months ago.   Now do you see why it is so incredibly hard to have this stuff figured out?  What happened to me happens to patients all the time – even the good surgeons are aware of it.   This is why patients struggle for years to get diagnosed.  How are patients supposed to know, without doing a pile of reading, that the results of the scan are purely in the eye of the beholder?  Yet again, if I would have believed the radiology reports were the truth, I would have concluded there was nothing wrong with me and I would have stopped trying to chase this down.  And that would have been a mistake.  ARGH!

But I digress.  Merlin found ONE abnormality and therefore planned to start the surgery the next day by starting with this abnormality.

Parathyroid surgery is interesting and unusual in the sense that there are typically four parathyroid glands and some may be healthy while others are diseased (hyperplasia or have an adenoma).  Some people have more than four glands; some have less.  And the parathyroid glands are typically located behind the thyroid but can also appear in other locations as well.  Because of this variation in the number and location of the glands, there is also much more uncertainty as to whether finding and removing one bad parathyroid gland will cure a patient with hyperparathyroidism.  If a surgeon only looks for and removes one gland, how does he/she know if this is the only problem gland?  One way to help resolve this uncertainty is through intra-operative hormone testing.  If removing a hyperplastic gland or an adenoma doesn’t cause PTH to drop significantly, the surgeon knows that there is still a problem parathyroid gland somewhere.  If PTH does drop significantly (i.e., a drop in PTH of at least 50%), then the surgeon has some reassurance that all of the problem glands have been removed.  This is not a completely foolproof system – no system is.  But it has been shown to result in a 98 percent cure rate.  You don’t have to take my word for this – you can read more about it here:

http://endocrinediseases.org/parathyroid/surgery_pth_monitoring.shtml

To this end, at the beginning of the surgery, Merlin obtained a baseline measurement of my parathyroid hormone  (PTH) – the hormone that I had been producing in excess and had been inflating my calcium levels.   He then headed straight for the region of my neck on the right side where he found an abnormality on my ultrasound the day before.  And lo and behold, he was right – there was Little Richard, my hyperplastic parathyroid gland.  He removed Little Richard and retested my PTH.   It dropped a little, but nowhere close to 50%.  So he kept looking.  He could only find one parathyroid gland on my right side, so he went over to the left side of my neck.  Both parathyroids were located and looked normal.  They were both biopsied to confirm that in fact they were normal and preliminary pathology conducted while I was in the OR indicated that they were normal.  These glands were subsequently fitted with little clips so that if they go bad in the future, they are easy to spot, regardless of the skill level of the person that goes looking for them.

At this point, Merlin checked my PTH again and it was still high.  So he headed south, into my chest on the right hand side (as this is the side where only one gland had been located so far).  Sometimes parathyroid glands can be located deep in the chest, sometimes also in the thymus.   I had my thymus removed a few months earlier due to a suspected carcinoid, so that wasn’t an issue for me, but Merlin went down there to take a look anyway.  He found a few leftover pieces of thymus that he removed along with 14 lymph nodes.  After cleaning out this upper chest cavity, he checked my PTH again.  It now dropped by well over 50%.  And so, three and one half hours later, Merlin stopped.

I don’t remember talking to Merlin after surgery, as I was still pretty out of it from the anesthesia.  But this much was communicated to M, who did get a chance to speak with him.  I did get to see all of the details myself about a week after surgery when I was sent a copy of the operative report.  The operative report was quite interesting.  Here is the summary:

Screen Shot 2015-09-23 at 9.30.06 AM

Wait.  What?  THYMIC MASS?  I had my thymus removed in June and no tumor was identified in pathology.  Granted, I got better, so I didn’t believe that.  But a piece of thymus was left in my chest and that leftover piece had a mass?  I read further into the report because I can’t quite believe this and see it very clearly says Merlin saw a “nodular mass in the superior tip of my thymus” which was still sitting there in my chest.

I want to be clear – the reason I am showing you pieces of my reports is because this whole thing seems so outlandish to me that I wouldn’t believe it if someone just told me this story.  I would want proof.  So, I am showing you the reports so you can see I am not just making this shit up or exaggerating or adding my own twist to the story for dramatic effect.  This is really, truly happening.  I barely believe it myself most days – and I am living it.

Of course, the pathology on this thymic mass came back as normal thymus tissue.  But given what happened after thoracic surgery in June, I am of course suspicious of the pathology.  But who knows?  Perhaps this is all incidental?  I may never know the answer to that question.  I am just going to be glad that Merlin found it and took it out, along with my bad parathyroid gland, and I will leave it at that for now.

On The Road Again

A few weeks back, I hopped on a plane to go see a neuroendocrine expert.  I had initially contacted this team of doctors in April or May and after reviewing my file, they agreed to see me.   This was when I had my positive Octreoscan for tumor activity in the thymus, a negative CT scan of the same area, and could barely breathe or move.  I was planning to go and see them in late June, but then I was encouraged to go and see Gandalf and he got me cleared for surgery locally and so I cancelled my out of town trip.

As way of background, here is where things stood at this point.  I got dramatically better after thoracic surgery in June.  I went from looking like this most days:

Pre-op Flushing

to looking like I had a mild, even sunburn in the neck and chest area.  I stopped coughing altogether.  I went from wheezing all of the time to the odd wheeze here and there.  If I was exposed to a lot of stress, I would have pretty severe carcinoid syndrome symptoms – flushing, wheezing, diarrhea (and some hives too on a couple of occasions) – but if I stayed away from stress to the best of my abilities I was feeling pretty good.  I also was able to stop taking daily anti-histamines.  I couldn’t complete a full sentence without coughing or gasping for air unless I was on anti-histamines before.  So, based on symptoms alone, it appeared that the surgery must have removed something bad – otherwise, I wouldn’t have improved!  However, the surgeons never saw a tumor during the surgery and no tumor was identified by the pathologist either.  This discrepancy between the improvement in my symptoms and the pathology report left me feeling that I should get a second opinion on the pathology.  And although my symptoms had diminished, I was still flushing and I was still having GI issues.  So I was very glad I had had the surgery, regardless of what the pathology report said, but I still needed to figure out where the rest of this NET was hiding.

I also had been in the middle of a work-up to figure out what was in my sacrum.  I had been experiencing nocturnal pain in my sacrum for over a year and I found (what now has been confirmed by four doctors) masses in this area on an old MRI from last year.  After thoracic surgery in June, I had a follow-up Octreoscan to locate any remaining neuroendocrine tumor activity and although the report was officially negative, there was some question marks by the radiologist about whether there was uptake in my liver in some of the scans.  Equally concerning to me was that there appeared to be uptake low in my sacral/pelvic area, the same rough location that I already knew I had masses.  This of course is not mentioned in the radiology report, but I can see it as clear as day.

So, with all of half-positive, half-negative information, I decide it is a good idea to go and get a second opinion on whether I have a serious problem on my hands or whether this is nothing to worry about.  I contact this group of specialists and ask them if they are still willing to see me, after I send them an update on my situation post thymectomy.  They tell me they will see me (they don’t see everyone) and we set up an appointment.

As I am staring out the window of the plane as I am about to touch down in another strange city, it hits me that this whole thing is so crazy.  What has happened to my life?  This whole world I am living in seems so bizarre.  How do you go from being a normal healthy person to someone who – on more than one occasion and for more than one kind of tumor – has had to pack up and fly across the country in hopes some doctor can help you.   Having to do this to see the Wizard when I discovered the first set of tumors, in my pituitary, was a surreal experience.  But how can it be the case I am still doing this, two years later?  I have turned into some sort of professional patient, with weird problems and doctors in a bunch of different cities.   I still don’t understand how I got here.  But I am here and I am trying very hard to make the best of it.

As usual, I go into this appointment ready to be turned away.  The oncologist the team decides I should see starts out the same way everyone does these days: “I just reviewed your chart.  You have had a terrible couple of years” followed by “your pathology was negative and your scans were read as normal.”  So I calmly and patiently explain how my symptoms have changed and I show him pictures of my flushing before and after surgery.  This of course catches his attention – he agrees that it appears something bad had been going on and something had improved after surgery.  He asked me to sign a bunch of forms so his team could get my pathology shipped to them and have their own pathologist look at it.  Excellent.

I also showed him pictures of the Octreoscan I just had and how it appeared the scan didn’t look normal to me (with my usual little jokes and caveats that yes, I realize it is annoying a patient is in your office trying to tell you that they think they know how to read an Octreoscan better than a radiologist).  But he agreed, it didn’t look normal.  He wasn’t an expert at looking at these things and wanted his radiologist to look at it.  But he acknowledged that this happened regularly here – people came in with negative scans, according to the radiology report from their home hospital, that were in fact positive.  So, they would look at the scans.

I also showed him the pictures of the MRI of my sacrum.  This, he agreed immediately, was not normal.  He asked me what the report said, and so we pulled it up so he could read it.  He told me the report was so bizarre and so inconsistent with the actual MRI that the only logical explanation was that the wrong report was attached to the MRI.  He told me he wanted an updated MRI of this area, so he ordered that, and some histamine labs.  He also told me they would discuss my case at “tumor board” (their weekly meeting of the entire team of surgeons and oncologists to discuss new and/or complicated cases) and he told me the team would make a decision as to whether they could help me and what the next steps would be.  That said, he anticipated a bone biopsy of one of these masses was the most likely next step.

After the meeting of the tumor board, I received a phone call from the oncologist’s nurse.  They have decided they want me to complete a few more histamine tests and it is clear they are trying to figure out whether my flushing and other symptoms are coming from a tumor or mast cell disorders (which also cause flushing).  My labs all come back normal – no sign of a mast cell disorder, but no biochemical evidence of a tumor either.  This is not so surprising as these tumors don’t typically show up in labs unless they have spread to the liver. And all my labs for carcinoids have been negative to date – but lots of patients with known carcinoids have negative labs too. However, it certainly doesn’t help point any of us in a clear direction either.  I ask the nurse if their radiologist has looked at my 2014 scan and my new Octreoscan.  She skims the notes from the tumor board and tells me that they have.  The MRI of my sacrum, she says, is abnormal.  The Octreoscan, which was read as normal at home, is now also read as abnormal by their radiologist (I knew that scan wasn’t normal!!) but they are still collecting evidence (the new MRI) to try and determine the significance of the Octreoscan findings.

I came home and had the MRI locally.  Usually getting the scans doesn’t stress me out so much, but this one stresses me out a lot.  I have to fight not to cry in the MRI machine.  I am worried.  The MRI from 2014 shows some serious abnormalities, no one is questioning this now.  I have had a pile of pain over the past year and pain in new spots down there now as well.  And zero treatment for whatever these things are.  What if the existing ones got bigger?  What if they spread?  I feel gripped with fear and I just want to know what they are NOW and for someone to tell me this can be fixed or managed NOW.  But that is not going to happen.  This whole business is going to continue to proceed at its slow, painful pace.

I wait 2-3 days after the MRI to drive over to the hospital and pick up a copy of the scan and the report.  The report pisses me off.  I am fully expecting this report to actually document the problem areas, as the whole reason it is ordered is to get an update on my sacral masses.  And in fact, this information is in the history of the radiologist’s report.

Screen Shot 2015-09-18 at 8.57.51 AM

And then I keep reading through to the conclusion of the report.

Screen Shot 2015-09-18 at 8.58.04 AM

What???  I pop the CD into my laptop to take a look at the images myself.  Is it possible that my old report was correct but in fact it had been attached to the wrong set of images as the oncologist pondered?  No.  In fact, that is not what has happened.  Here was the concerning area we all saw on my 2014 MRI.

Screen Shot 2015-06-04 at 2.38.43 PM

And here, is the updated MRI (click on the images to enlarge them):

Screen Shot 2015-09-24 at 12.20.26 PM

The picture on the right is exactly the same masses I had last year!  Exactly the same! I become filled with a black rage.  How can this happen?  I have pain. I have four doctors that have looked at the images with me saying, without a doubt, there are masses in my sacrum. I have a history spelled out by the radiologist saying the sacrum had masses last year.  I can find this image in about 30 seconds.  And yet my MRI is read as normal AGAIN.  And I see new spots, including the spot on the lower left corner of the picture on the left.  The reason I knew to look in this area on the MRI is BECAUSE I HAVE PAIN HERE.  And you see the same thing that you see in my sacrum:  something that looks like a black circle in the pelvic bone.   I locate several other areas that look like this, black circles in the bone, in my vertebrae, in my pelvis.  And then I stop looking at the MRI.  I am angry and I am scared.

And you should be scared too.  If I had not been a patient that went so far as to study up on MRI imaging of bones, look at my MRIs and take pictures of the images in to more than one doctor, what would be happening to me?  Imagine you had an MRI last year that was normal and because you still had pain this year, your doctor ordered another one and it was also negative?  A few years ago, I would have said “Phew, there is nothing seriously wrong.  It must be arthritis and a bulging disc.  I can take meds and physio for that.”  Now, I know better.  I know if the conclusion doesn’t make sense when compared to the symptoms, I can’t just trust what I am being told.  If I hadn’t looked at the images myself, I would have NO idea something was terribly wrong. I would NOT have gone across the country to get a second opinion. I STILL have no confirmation of what these things are.

It is frightening to think about the possibility that medicine is not black and white.  That doctors don’t always get it right. That mistakes aren’t just made in rare cases but they are made all the time. You could think that what is happening to me is exceptionally bad luck.  But I have heard enough patient stories at this point to know that this just isn’t true.  It frightens me and it should frighten you too.  I want to frighten you so that when you end up in this situation you remember my story and you don’t stick your head in the sand.  If this happened to me, it can happen to you.  It happens a lot.

This is why when a radiology  or pathology report is negative these days, and it doesn’t match my symptoms, I don’t believe it anymore.  I am put in a situation where I have to question EVERYTHING that doesn’t make sense.  I hate living like this.  There is nothing more I would love to do right now than hand all of this off to one doctor who is going to figure everything out and take this pressure off me.  But that is not reality.  No doctor can or will do this for you.  I don’t feel I am qualified to be in charge of this complicated, horrific mess that is my body.  I am not a doctor.  This shit is rare and subtle and scary.  And there is no one manning the ship yet.

Roadblocks

After what felt like a couple of months of good progress, I feel like I have hit some roadblocks again.

First, there is disagreement among the doctors I am currently seeing as to whether I have hyperparathyroidism or not.   The surgeon, who I trust and comes highly recommended, thinks I do.  The Wizard, who I also trust very much, does not.   In particular, he thinks my labs aren’t high enough to warrant surgery and that there might be other explanations for my labs and symptoms.  While the decision is up to me at the end of the day, I honestly would be feeling a lot better about proceeding with surgery with him on board.  I don’t want to go against his advice.  I don’t want him to get upset with me and I don’t want to jeopardize a great doctor-patient relationship.  I trust him very much and I know when he offers advice it is because he is concerned about my wellbeing.   But at this point in the game, I trust myself too.  And there is evidence in favor of surgery too – the same evidence the surgeon relied upon when deciding to clear me for surgery.    I am part of a patient group where I have, literally, seen spreadsheets of patient labs that were diagnosed with hyperparathyroidism, had surgery and got better.  My spreadsheet of labs looks worse than some of the other sets of labs I have seen.  My labs certainly don’t look as bad as some of the labs I have seen either – they aren’t off-the-charts high.   But they do look abnormal and there is a clear upward trend in both my PTH and my calcium.  I am not a doctor.  However, I do have a PhD in economics and if there is one thing I can confidently do with a high level of expertise, it is recognize a trend in a set of numbers over time.   The diagnosis for hyperparathyroidism is based on the labs – and my labs have been trending up over the last 18 months.

I also know, from my experiences getting diagnosed with Cushing’s and from reading about the experiences of other patients with hyperparathyroidism, that typically the patients with insanely high labs have been sick with tumors for years.  I don’t want to be sick for years – I want to catch as many problems as I can EARLY.   The downside to catching things early, as I think basically summarizes my experiences through the  medical nightmare of the last few years, is that your labs and scans look mildly abnormal.  Would diagnosis be more straightforward if I stayed sick and waited a few years for things to look worse?  Probably.  Would the damage to my body be irreversible and in all likelihood shorten my lifespan?  Probably.   I feel like I am being forced to choose between this unending battle to get diagnosed and treated or staying sick until there is a tumor the size of a pumpkin in me that will be impossible to miss on a scan.   I don’t want to struggle and fight at all, but if these are the options on the table, I am going to choose to struggle to get diagnosed, folks.   In addition to a desire to prevent irreversible damage, I also am stuck living with very real, disabling symptoms right now.  I don’t want to sleep half the day and be in pain every night any longer than I have to.   Hyperparathryoidism isn’t going to kill me if I wait a few more years to get diagnosed (although some of the other problems might), but it will leave me feeling terrible.

And if the surgeon and I are wrong?  If I don’t have hyperparathyroidism and he opens me up and finds everything is fine? I will have flown across the country and had unnecessary surgery against my doctor’s advice.  This is not an outcome I would be happy about at all, but I am willing to take the chance at this stage.  It is a relatively minor surgery and, worst case scenario, we have ruled out the parathyroids as part of my problem.  It was the same thing with my thymus.  We all agreed the evidence wasn’t 100% clear the thymus was a problem, but the suspicion was sufficiently high (based, legitimately, on my symptoms and scans) that it was worth taking it out and seeing what happened. And even though the pathology hasn’t (at least not yet) confirmed I had a carcinoid there, I GOT BETTER.  I stopped coughing and wheezing.  I stopped walking around with a purple spot in the middle of my chest.  Something happened.  Maybe if I had waited a couple of years, gasping for air in the meantime, the tumor would have grown and we would have clearly seen it on a CT and it would have easily been detected in the pathology.  I would have a diagnosis in hand and no doubt in the world about it.   And there is also a good chance it would have spread and been much more difficult to treat as a consequence.   I think I caught it, whatever “it” was, early and there is still a very real  chance it has already spread.  But at this point in time, I have no pathology report to confirm it and therefore have no treatment plan going forward.  Do you see my dilemma?

Second, the doctor that really seemed to move me forward several steps, Gandalf, has disappeared.  I saw him in July and I thought we made tentative plans to take care of a bunch of items – I was getting an Octreoscan that he was going to look at to see why I was still flushing, he was going to help me get a second opinion on my pathology, he was going to help me get a GI consult if my digestive problems didn’t clear up (and they haven’t).  Our plan was to stay in touch via email.  I saw him five weeks ago and have emailed him twice, but I haven’t received any response.  Not so much as an acknowledgment that he saw my messages.  I had hoped maybe my pathology got sent out anyway, but I have called the pathology lab at the hospital and it is still there.   Am I being impatient?  Has something happened to him?  Are my messages going into his junk folder?  Is he just incredibly busy (which I would completely understand).  Or has he, for reasons I don’t quite understand, become fed up with me and decided he is just going to freeze me out?  I don’t think I have been pushy or difficult, and M was with me at our last appointment and agrees everything seemed to be on track, but now I am questioning everything. I just have no idea.  None at all.  But this is very concerning to me and I don’t feel like I have much choice other than to keep forging ahead without him for now.   And I worry that forging ahead without him might also make him mad if it turns out he was just very busy and lining things up in the background while he hasn’t been in contact.  But how am I supposed to know and how long am I supposed to wait?

Finally, I am really frustrated with the bone doctor.  I had high hopes for him when I saw him in July.  He ordered tests, sounded like he was going to quickly figure out what was wrong with my sacrum.  But I have been back to see him twice now and it feels like am getting nowhere.  I showed him an MRI of my sacrum from June of last year that shows masses in my sacrum.  At that time, I asked him if he would order an updated MRI – I know if there is going to be any sort of intervention we would need to see an updated MRI no matter what.  Instead, he ordered an X-ray and an Octreoscan, which was fine.  At the follow-up appointment, he couldn’t find my X-ray in the system at all.  Couldn’t find it! And told me to come back in a month, when the Octreoscan was back.   I asked him again if he would order the MRI while we waited and he said “let’s wait and see what the Octreoscan says.”  So we wait.  I see him yesterday for the second follow-up.  He hasn’t looked at the Octreoscan or the X-ray and he claims he can’t pull them up and look at them right then and there.  So, why the hell am I coming back to see him, for the third time, if he hasn’t looked at any of the scans that were done two weeks ago?  I am prepared this time, though, and have the actual CDs of the X-ray and the Octreoscan in my bag.  I also have my laptop, which has the scans and the software needed to read them loaded on it, as well as screenshots from the scans.  So, I smile and ask him if I can be annoying and ask him to take a look at what I have to show him.  What is he going to say, no?

For the record, the report from the Octreoscan is annoying too.  It says my liver looked “mottled” at 24 hours (meaning there was some suspicious areas lighting up) and so the scan was repeated and now looked normal at 48 hours.   So, the conclusion is that my liver is fine.  But does this mean my liver is fine or that it isn’t?  Carcinoids, if that is what was removed from my thymus in June, often are not found until they have spread to the liver, so it seems pretty important to get a definitive conclusion on this.  I know the protocol for the scan is to scan at 4 hours and at 24 hours.  I also know that sometimes a 48 hour scan is done if there is significant bowel activity at the 24 hour mark.  But the liver is nowhere close to the bowel, so is it kosher to rescan at 48 hours because of suspicious activity in the liver?  And if the scan is clear at 48 hours, does it mean the liver is fine or does it just mean that the radioactive material is no longer bound to a tumor?  So many questions that I just don’t have the answer to.   The other problem with the report is that it doesn’t mention anywhere the fact that there looks to be an abnormality down by my sacrum.  That’s right, in the region of my body that prompted the Octreoscan to be ordered in the first place.

I showed the bone doctor the following screen shots from the Octreoscan.  See that black spot?  The picture on the left is my pelvic area as viewed from the side.  The picture on the right is my pelvic area from the back.  The black spots appear to correspond to something near my sacrum:

Screen Shot 2015-08-21 at 12.06.24 PM

I have other pictures too, pictures taken as part of the Octreoscan and at different points in time (4 hours and 24 hours).  The full body scan, for example, shows the same thing:

Screen Shot 2015-08-21 at 12.31.57 PM

This looks like it could be neuroendocrine activity to me.  The bone doctor agrees – this doesn’t look normal to him either and he doesn’t understand why the report doesn’t mention it.  I also show him this picture from the Gallium 68 scan (which is a much more sensitive scan than the Octreoscan for neuroendocrine activity) I had back in April, that seems to highlight an even bigger spot in the same location:

Screen Shot 2015-08-21 at 11.56.10 AM

See that circle and a diagonal dark area at the bottom of the picture (both marked with green arrows)?  That looks to be my sacral area as well and also seems pretty dark (the black stuff above is my liver and kidneys and, as they are working to clear the radioactive material from my body, they are supposed to be dark).   Is this something significant or is it just noise or incidental uptake?  I would be more inclined to believe that if I only saw this happen on one picture, in one scan.  However, given the same area keeps lighting up on different scans,  done at different institutions months apart, I think the possibility this is nothing is pretty low.  But of course every single radiology report says the scans have no abnormal uptake.

I ask the bone doctor if he will please circle back with the radiologist and ask him to look at this again.  I also offer to send him an email with exactly these pictures so he can pass them along to the radiologist so he can see exactly what I am talking about.  And I do this – I send the email.  But I honestly don’t think he will do it.  He seems very disengaged during the appointment, like he doesn’t want to deal with any of this.  He doesn’t even ask me how I am feeling.  All he does is tell me he agrees this doesn’t look normal.  And since the Octreoscan is read as normal, he finally orders the MRI of my sacrum. And tells me to come back in mid-October.

I keep thinking that the next doctor appointment is going to be the one where everything is going to fall into place.  That someone is going to take care of everything, take this out of my hands.  That this is going to require less work on my end at some point.  The reality is that has not happened yet and maybe it will never happen.   But that doesn’t change the fact that I need to keep moving forward until I get all of this figured out, one way or the other.

Stress will make you sick

I remember well a conversation I had about ten years ago with one of my brothers.  At the time, he was completing his medical residency in psychiatry.  He was also a smoker.   We were having a drink together and as his older (and overbearing) sister, I couldn’t stop myself from asking him: “how is it that you have gone through medical school and you continue to smoke, given you know how bad it is for you?”   After hearing my question, my brother paused, took a long drag on his cigarette and replied:  “Do you know what is really bad for you?  Stress.”  We both had a good laugh at this point, but more and more I realize he had a point:  stress IS really bad for you.

I am acutely aware of the effect of stress on my health at present for two reasons.  First, I am under a lot of stress.  Being sick, whether it is acute or chronic illness, is stressful.   Pain, troubling symptoms, uncertainty, tests, doctors appointments are stressful.   Second, given I still have carcinoid syndrome, stress manifests itself physically in unpleasant and distinctive ways these days.    When I have encountered acutely stressful situations in recent months, whether it be an uncomfortable encounter with someone, a letter from the IRS (handy tip:  doing your own taxes a month after brain surgery may not be a great idea), or finding out I need another surgery, I end up running to the bathroom with what feels like dysentery and I start wheezing and break out in hives.   Sometimes, it is only when I start breaking out in hives that I actually realize how stressful a particular encounter is!  My body has developed a finely tuned stress-o-meter that is very hard to ignore.  As weird as this sounds, this is actually quite common for patients with neuroendocrine tumors and it has a medical explanation:  neuroendocrine tumors produce hormones (such as serotonin and histamine) and stress can cause large amounts of the hormones secreted by the tumors to be dumped into your system, causing exactly the kinds of symptoms I have been experiencing.  These days, I often have a stress-o-meter reading right about here:

Stress

This basically means it doesn’t take much additional stress for me to get physically ill.  I don’t want to live in the red zone on my stress-o-meter and am working very hard to try and get as far into the green zone as I can.   As a result, stress management has become an important part of my plan to stay sane and get my health back.  It is just as important as being careful about what I eat and continuing to chase down and treat the remaining medical problems in the queue.   Part of my stress management plan involves reducing stress through things like gentle exercise, petting the dogs, reading a book.  The other big part of my plan that has made a major difference is avoiding stress: limiting the amount of stress I allow myself to be exposed to.

One of the most important lessons I have learned from being sick is the importance of saying no and creating healthy boundaries.  I am by no means great at doing this, but as time goes on I am getting better and better.   There is a certain amount of stress in my life I cannot avoid at present, but this stress really is limited to my body, my finances, and anything that happens to my inner circle of loved ones – that’s a tiny set of people and issues, at the end of the day.   And when I do face stress that cannot be avoided, I force myself to set limits on it.  Upset about a bad doctor appointment or a negative test result?  I give myself exactly 24 hours to mope about it and then I let it go.  Period.  Ruminating over something that I have no control over and that happened last week, much less six months ago, is a really dumb way to use up my limited resources for handling stress today.

The rest of the stress I face in life is stress I can choose to avoid.  And I am making every effort at present to do just that.  I cannot – and will not – cope with drama when I am already in the red zone.  I will let passive aggressive (or aggressive) comments or questions slide off my back.  I will not worry endlessly if someone is upset at me over an unintended slight.  I will not feel obligated to help other people fix their problems.  I will not take the bait when I am poked.  I will quickly and quietly escort troublemakers to the door.  And I won’t beat myself up for that.  This doesn’t make me a selfish or callous person – I am simply putting my own oxygen mask on first.   If I allow myself to get sucked into avoidable stress right now, I have no one to blame but myself.

Saying no and laying down healthy boundaries stressed me out at first.  It is hard to do this and not feel guilty.  But with practice, this is getting easier and easier.  I am still far from being a Zen master, but I am getting better every day.  Not everyone has reacted well to the new boundaries, but I am okay with that too.  People that get mad when you put your own oxygen mask on first probably also failed to notice you were struggling to breathe in the first place.   Those aren’t the kinds of people you want in your life anyway.

You are the only one that can limit avoidable stress to protect your health, just like you are the one that is responsible if you get sick because you decided to eat fried chicken instead of kale (and sometimes it is worth it!).  If you want to have an oasis of calm, in spite of the stress you cannot avoid, you have to create it yourself.  No one can or will do it for you.

I have MEN1

I have been trying to sort out whether I have hyperparathyroidism (HPTH) for almost a year now.   Hyperparathyroidism is tumors in the parathyroid glands.  The tumors cause the parathyroid glands to produce too much parathyroid hormone (PTH).  The sole function of the parathyroid glands is to regulate calcium, so patients with HPTH end up with levels of calcium that are too high and high calcium, in turn, makes them sick.

I have had my calcium and PTH checked 14 times since November 2013.  And there has been a clear trend.  First, my calcium increased by almost a full integer (calcium typically remains in a very narrow range, fluctuating up and down by 0.2 or so).   Then, my PTH slowly inched up as this new calcium level remained roughly the same.

Despite being told repeatedly not to worry about this (including quite recently), I was always suspicious.  As was the case when I had Cushing’s,  I did my homework and read extensively – the experiences of other patients, the very informative website put together by a well-known parathyroid surgeon, Dr. James Norman (www.parathyroid.com), and medical research.  I discovered lots of patients had HPTH with labs that looked pretty similar to mine.  Mine were definitely on the lower end in the beginning, but the trend over time was pointing more and more towards HPTH.

Based on my labs in 2014, I got into a study at the NIH on hyperparathyroidism and MEN1.  But I was told I didn’t have it while I was there.  The worst experience I ever had with a doctor in history, and I have seen A LOT of doctors, was with a local parathyroid surgeon that I consulted with after my trip to the NIH.  He not only refused to look at my PTH labs from outside his hospital but also said, to my face, he didn’t think I had Cushing’s last year and that I should “go home, live my life and stop chasing problems that don’t exist.”  This was seven months ago and I was very sick at the time (I am still pissed about this.  Not helping a patient is one thing – attacking them is another).

Because I was not convinced I didn’t have HPTH, I decided to go ahead and get another opinion anyway.  Just to be clear, and as I have said many times before, I didn’t WANT HPTH and I wasn’t convinced I had it either.  But I did know I was very sick, some of my symptoms were consistent with HPTH, and these labs (along with my bone turnover markers and my ALP) were the only ones that were coming back abnormal.  So the only logical thing to do was to continue to chase this down.  What else was I going to do?

The parathyroid surgeons I consulted with this winter agreed that my case was not a clear-cut case but was certainly suspicious and they ordered more tests.  Right around this time, I started having severe problems with what I would later learn was carcinoid syndrome.  Since parathyroid tumors are typically benign and carcinoids are neuroendocrine cancer, I decided to put the investigations into HPTH on hold while I focused on the carcinoid.  So I didn’t complete the PTH tests.

Fast forward to July.  Thoracic surgery to remove what we suspect is a thymic carcinoid is behind me, I am investigating the masses in my sacrum and the bone doctor orders another round of PTH and calcium tests.  I see that my PTH has tripled since it was last tested this winter.  I am of course excited by this news.  This confirms my suspicions.  When I see the bone doctor on follow-up he tells me he doesn’t think I have HPTH.  I politely tell him I don’t agree, but don’t push the issue as I really want his opinion on the masses in my sacrum.  And I send my new labs back to the surgeons I consulted this winter and I finish their tests.

Shortly thereafter, I get a call from the parathyroid surgeon:  I have been diagnosed with HPTH based on my new labs and the trend in the labs.  This time, the surgeon is definitive about the conclusion:  there is no doubt in his mind and there is no doubt in mine either.   The surgeon tells me that my labs are most consistent with parathyroid hyperplasia, which is a situation in which all of the parathyroid glands tend to become enlarged (as opposed to having a solitary adenoma on one parathyroid gland).  This is a relatively common form of HPTH in MEN1 patients in particular.  Because of this, all four of my parathyroid glands will be thoroughly examined during the surgery and all of the enlarged ones will be removed.  Because I am a tricky case, my surgery will occur in the hospital rather than at the surgeon’s private surgical suite.  The surgery itself is very straightforward and non-invasive.  I am not worried about this part at all.  I will post more about this later.

Given my history of pituitary tumors, a diagnosis of hyperparathyroidism confirms the clinical diagnosis of MEN1.  Add to that the carcinoid and the nails are firmly hammered into the coffin.   I feel a mix of relief, shock, fear, anger and sadness that I had to fight this hard to figure it out and that this is indeed what is wrong with me.   This is a scary diagnosis, and although it is one I have been worried about for a year, it is going to take time to accept it.  But I have had to accept lots of other things over the past few years and I tell myself that in time I will accept this too.

Life is what happens to you while you’re busy making other plans

I am pretty OCD by nature and so it has been easy to turn this quest for medical answers into an obsession.  For the past couple of years, I have tried to put everything else in life behind getting diagnosed and treated.  This was not completely irrational, as I was/am sick and very limited in what else I could do besides try and figure this out.  But the other big motivation was simply the belief that the harder I worked at fixing this problem, the faster I would get better so I could go back to work and my regular life.   With running and work and most every other challenge I had faced in life so far, hard work lead to the desired outcome:  a degree, a marathon, a publication, a promotion, you name it.  I figured that the same strategy could be applied here and with the same effect.  The problem is brute force is only getting me so far: this is just not a problem that I can completely solve and fix on my own.

Stage one of the five stages of grief is denial and, despite dealing with the reality of my situation daily, I do think I have been in denial about the fact that this “temporary” health issue is probably going to be permanent in one form or another.  Whenever people try to ask questions such as “if you can’t run again, what will you do instead?” or “if you can’t go back to your job (with it’s crazy hours), what will you do instead?” my response has always been “I am not even going to go there.”   The only thing that has kept me from completely melting down many days is the belief, however misguided it may be at this point, that I was going to return to my former life – marathons, crazy work hours and all.   But as the days and weeks of being sick have now turned into years, I am forced to face the fact that I need to figure out a way to make the most of today too.  I need to start feeling and acting like the days and weeks that are passing by now are valuable moments.  This is not the life I want, perhaps.  But it is still a life.  And it is not just my life that is being put on hold – M’s life is being put on hold  too.  So, I have started to use some of the time I used to spend becoming an amateur medical researcher/radiologist/endocrinologist (and a professional harasser of doctors) for other purposes.  I am still sick and I still can’t do most of the things I want to do, but there are things I can do to try and improve my health NOW and to enjoy life more NOW while I wait for the other medical problems I have to get fixed.

I have been reading a lot about nutrition and am very focused now on maximizing the amount of nutrients that go on my plate.  I was always a reasonably healthy eater, but now I really want to give my body the best possible chance to recover and get better while I am still sick. I also started exercising again.  I can’t do much, but I am trying to do something every day I feel well enough to try.  I started with walking.  The walks used to be short and painful, but I have been able to gradually increase the length and speed of my walks and reduce some of the discomfort.  Part of this has been made possible because I recently caved in and started taking the  pain meds I had been prescribed, which for stupid macho reasons I was refusing to do before (and this only after I got a lecture on the consequences of chronic pain from my psychiatrist who told me to stop being a “Calvinist” with the pain meds).  Now, I am almost up to five miles at a time.  I have been walking with a friend of mine at times, and also with M, which has been a great way to sneak in some quality time with those I care about too.  We have an erg at home, and the other day I was able to erg a little.  I used to be able to easily erg 10,000 meters at a time and was super happy that I was able to crank out 2,500 meters on my first try.  Not fast.  Not pretty.  Not painless.  But I did it!

I still need to sleep many hours during the day.  I still have a lot of pain.  I am still very limited in terms of how much stress, mental and physical, I can handle without getting sick.  I still don’t know if I will be able to run a marathon or go back to being a workaholic.  But these little steps have enabled me to lose about 15 pounds in six weeks, to improve what I am eating, to spend quality time with people that make me happy, to enjoy a few activities that make me feel good about myself.

It’s not the life I want, but it is a start.