Two Worlds Collide

I have been on a quest since last July to get a second opinion on my thymic pathology, but it hasn’t been totally clear who (and how!) to get someone to look at it. But for some random reason one day, I did a search of clinical trials relating to pathology and stumbled onto a trial that sounded perfect for my lump of tissue:  a tissue procurement trial at the National Cancer Institute that was collecting specimens from people with lung/thymic malignancies and then collecting natural histories of the patients (this trial is not for treatment itself, just to follow the course of the disease)!  I right away sent off an email to the oncologist running the trial, explaining my situation – I had symptoms and an Octreoscan suspicious of a thymic NET, had surgery, got quite a bit better, but nothing was found in the pathology.  I told them I realized I might not fit their criteria, as I am not a pathology confirmed NET, but boy would I love to give them my tissue if they were willing to take a second look.

A few days later, I received an email from a clinical thoracic oncologist at the NCI who specializes in thymic malignancies.  He saw my email and reached out directly, telling me that they were willing to see me in clinic and evaluate me, regardless of whether they admit me in the study.  I just had to send my scans/operative reports/pathology reports and make an appointment to come to Bethesda.  This was much better news than I had been hoping for and I was of course happy to do it. It turned out that I was already going to be in DC for a work conference for almost an entire week in the near future and so I decided to try and be efficient by combining my trips.

The timing worked out well.  I was able to set things up so that I could work for two days, do medical stuff in the middle and then go back to work at the end of the week.  I had been trying to keep work and this medical stuff completely compartmentalized as I have zero idea how to integrate the two in my life (and I am still not sure I want to).  So, aside from a couple of people at work that were close to the situation, no one had any idea I would be doing anything other than working on this trip.  And for now, I planned to keep it that way.

Lucky for me, my ever-helpful SIL was nearby and able to accompany me to the NCI when the day of my visit arrived.  She was also able to stay with me the night before and after the trip.  The night before and the morning of the trip, I was a little nervous.  It is still very hard to fight the fear that I am not going to be taken seriously – I really feel scarred by my many bad prior medical experiences, despite the fact that these experiences are few and far between at this stage in the game.  So I always go into doctor appointments, even with doctors I know and trust, fearing I am going to be turned away.  I just kept telling my SIL that my goal was to get them to take my tissue. If I manage to accomplish this?  I will have met my goal.  But I was worried I came all the way here for nothing.

I have particularly mixed feelings about the NIH.  I was evaluated at the NIH for MEN1 in January 2015 and remain extremely grateful that they admitted me to their study.  While I was there, they did a bunch of scans and the Octreoscan at the NIH was where the potential problem with my thymus was located.  And it would have been almost impossible for me to get my thymus out as quickly as I had without that scan.  That’s on the positive side. On the negative side, I had less than favorable experiences with some of the doctors that had evaluated me at that time.  And despite everything that has happened over the last few years, they don’t believe I have MEN1 and won’t follow me further, unless my hyperparathyroidism comes back.  But this is a new day and a new set of doctors.

My SIL and I go through the usual routine – meet with the fellow, go through a detailed history and then see the team.  And what happened next shocked both of us.  The two thoracic oncologists said, with zero hesitation, that I had a thymic NET.  That no other explanation made sense.  Between my symptoms (and I had brought pictures of my flushing episodes as well as office notes where Gandalf described in detail the sudden onset and appearance of an episode he witnessed), my Octreoscan and the fact that the coughing and wheezing stopped completely and abruptly after surgery, there was nothing else it could be.  There was no alternative diagnosis on the table.  This is what I always thought.  But it is a hell of a lot different hearing it inside my head and hearing from a doctor.

My diagnosis was “thymic NET unless proven otherwise.”  Someone actually said it out loud, with no other qualifiers.

This is important because without a diagnosis, it is hard to get treatment or be followed.  And this is the best part of the whole meeting. These things have relatively high recurrence rates and so they want me to come back for Octreoscans and CT scans of my chest/abdomen/pelvis every 4-6 months for the foreseeable future and then annually indefinitely.  And of course, since I still have symptoms, this has been a major concern for me as well.  I am very concerned I either have another NET somewhere, that the NET I had has spread, or that there is still a piece of tumor hanging out in my chest somewhere.  And all three possibilities were also acknowledged by these oncologists.

The NCI is going to obtain ALL of my pathology – pituitary/parathyroid/thymic and have their pathologists review all of it.  They were not overly surprised by my negative pathology, they have seen this happen before.  One of the oncologists said that this stuff is so rare, there is a chance the pathologist didn’t even know what he was looking at.  The other possibility, of course, is that the tumors were so small they would be easy to miss – which sounds so crazy, but again with this disease it happens.  In any event, they couldn’t guarantee they would find anything, but would take another look.  Goal met and exceeded.

We also discussed another strange new symptom that I have developed that I thought was likely unrelated but worth mentioning just in case.  They told me, repeatedly, I needed to go home have it evaluated.  They wanted me to get a specific set of tests and to send the results back to them.  I will come back and talk about that in another post.

As you can imagine, this was a very positive development in some respects but scary in others.  I feel a bit like I had a crazy JFK conspiracy theory and just discovered it was true.  I always believed this was my diagnosis, but not having it confirmed took the sting out of it.  I now can let go of the fear that I might have had a thymic NET and wasn’t being followed and replace it with the fear that I had a thymic NET.   It is less hypothetical and more real now, even though nothing has actually changed inside my body.

I was supposed to attend a client’s cocktail party that evening for work with a few of my colleagues, but I just couldn’t do it.  I was feeling very shell shocked.  I didn’t cry and wasn’t upset, but felt sufficiently close to that edge that a glass of wine and the wrong comment could get me into that territory and a work function was not the time for it.  I had sent an email to my colleague, letting her know that I wasn’t going to make it and why and her response was “Where are you?  I want to hug you.” and even that forced me to take a few deep breaths and blink away tears.  So my SIL and I decided to grab a bite at a restaurant close to the hotel where we wouldn’t run into anyone.  I was going back to the NCI for my scans the next day and would then close the medical compartment and open up the work one again.  But until then?  I was living in the not-suitable-for-work box.

Our plan sounded great until we walked into the restaurant and discovered the cocktail party was being held in the same place we had decided to come for dinner.  And of course, there is a group of my colleagues, looking at me with a confused expression and waving me over, while I stand there trying to smile and shake my head no.   The colleague I emailed came over and told us to come join them.  I introduced my SIL and said this was an accident, we weren’t here for the party, we were just trying to grab a quiet dinner together and unwind after a stressful day.  And so she sent us on our way.  But as she turned to walk away, she grabbed both my shoulders and gave me a hard kiss on the temple.  It makes me cry just sitting here writing  about it, I don’t know why.  I can’t quite explain in words how it felt for my two worlds to collide – but something about that encounter had me pushed me to the edge of a complete meltdown.

And the meltdown arrived the next morning, when I had a good long cry before returning to the NCI for the first round of CT scans.  I still felt pretty frazzled and, after getting caught in the rain on the walk back from the subway to the hotel, was not up for the  evening’s planned work activities.  I had received several emails from colleagues in other offices that wanted to catch up while we were all in town, and ran into two more colleagues I wanted to see in the elevator, but claimed I had an upset stomach and promised to see them tomorrow.  I had a quiet dinner in my room, watched some truly wonderful/terrible reality TV, and closed the sick box.  Friday morning, I woke up in my work box and picked up where I left off on Tuesday.

 

 

 

 

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Sherlock Holmes

After recovering from surgery #3 in September, my list of remaining problems was pretty small and relatively manageable:

  1.  It wasn’t clear if the parathyroidectomy I had in September worked, as my calcium and PTH weren’t that much lower post-op then they were pre-op.  It can take awhile for the labs to settle down, though, and it turned out that was the case with me.  If there is one thing my body really likes to do, consistently, it is generate unclear and unhelpful labs!  But, slowly and surely, both my calcium and PTH kept dropping.  Six months post-op, my calcium was 9.4 and my PTH was 38.  SUCCESS!!
  2. I had a weird looking MRI of my sacrum that had elicited a wide range of opinions that someone needed to explain to me and some back pain in the same area. The back problems and pain I will come back and talk about in another post.  But the good news is that the weird looking spots on the MRI have now been interpreted as benign.  The consensus now appears to be that my chronic pain is coming from some disc degeneration and it might be fixable with the craziest PT I have ever heard of but have nevertheless committed to completing.  Given all the crap that has gone on, it is hard to be totally comfortable with waving off any sort of unusual lump or spot on a scan these days, but as you will see in some future posts, I will be able to keep an eye on it anyway, and that makes me a little more comfortable.
  3. I continue to have “flushing lite” and GI problems that never went away after surgery.   As you might recall from earlier posts, I had a thymectomy in June of last year.  Prior to the thymectomy, I suffered from non-stop coughing and wheezing, and I frequently had very angry looking flushing, typically with a purple focal point in the middle of my chest and red blotchy flushing up my chest and neck. The flushing started around December 2014 and the coughing starting in February 2015.  I had GI problems throughout this period too.  It seems I stopped digesting my food around October 2014 and by March 2015, I started having episodes of very watery diarrhea.  I luckily was able to see Gandalf and have surgery in June 2015.  After surgery, the coughing stopped completely and the angry purple flushing midchest disappeared.  What I was left with was a lighter, diffuse flushing and the GI problems.

The rest of this post is about the quest to figure out the rest of the GI problems and flushing.  This investigation started in October of 2015.  Spoiler alert:  we still don’t have an answer, but I do think we are making progress.  Gandalf referred me to a GI doctor that he likes and, having seen this doctor now for six months, I can see why.  I am going to call him Sherlock Holmes because he strikes me as a careful thinker who really likes mysteries.  He has spent much of this time systematically ruling out everything else this could be besides a NET.  The reason he is doing so is that he doesn’t want us to get fixated on a NET and ignore other potential explanations for my problems (although in my mind I am screaming OF COURSE IT HAS TO BE A NET!).   He is also running NET tests, but NET labs have always come back negative and, frustratingly, continue to do so.

The reason we are not 100% fixated on a NET is because, as you may remember, I had a positive Octreoscan before the thymectomy, I got way better after my thymectomy, but the pathologist didn’t find a tumor in my thymus!  This was a very frustrating development, because without positive pathology there was no plan at all for follow-up or treatment for what could very likely be an aggressive type of tumor.

You might be saying to yourself at this point – big deal, you get a red neck and chest and have diarrhea once in awhile.  Just go live your life! That’s not debilitating.  Just live with it!  That’s a reasonable reaction.  And I am living my life and living with the symptoms for now. I am lucky – I am not struggling for air, exhausted, or trapped in the bathroom like a lot of patients I know that do have a NET.   But this is exactly why I can’t just forget about it.  If this is a NET (and I don’t think it can be anything else, given what’s happened so far), then it is going to continue to grow and get worse.  Ideally, if we can find it and remove it now, I have a decent shot of catching it before it spreads (or spreads too far).

So far, we have ruled out mast cell disorders, celiac disease, Crohn’s, IBS, you name it.  And we figured out something interesting.  Pre-thymectomy, I discovered (through a patient support group for NET patients on Facebook!) that antihistamines helped control carcinoid syndrome symptoms for some patients.  So I tried it and it worked! If I took histamine, I stopped coughing and wheezing could have a decent day.  After my thymectomy, I stopped taking the anti-histamines, as I figured I didn’t need them anymore.  In January 2016, Sherlock Holmes asked me to start taking H1 and H2 blockers daily to see whether they would help eliminate my remaining symptoms.  And to my surprise, over the course of the next six weeks, my GI symptoms slowed down and then disappeared! Completely!  I hadn’t seen things look this normal in 18 months and was amazed.  I was still flushing, but it wasn’t getting any worse at least.  But the GI stuff completely resolved.

Then the meds stopped working.

The Twilight Zone

It’s been about five months since my last post.  I have thought about writing many times, but I have avoided it. Sitting here today, it is hard to write.  Many days, for lack of a better expression, I still feel very disoriented.  The last several months have felt a lot like an episode of the Twilight Zone.  I feel like I left my office one day, entered a time warp, and returned to the same place two years later.  And at first glance everything looks the same as when I left, but when you take a really close look, nothing is the same.

For the first few weeks, I kept waiting for the wheels to fall off.  I worked 20 hours, then 30 hours then 40 hours a week.  With each increase in hours, I worried I wasn’t going to be able to handle it physically.  But I kept working more and more and nothing bad happened.  I moved up to 50 and 60 hours.  Still, no problem.  I didn’t start to run into problems physically until I hit a stretch of weeks where I was working 70+ hours a week.  Then I did start to feel exhausted and overwhelmed.  But that’s pretty damn good, in my books.  It took my body some time to get used to sitting/standing at a desk for prolonged periods of time.  There was a whole pile of muscles that I hadn’t been using during the period of time I was spending many hours a day in bed.  But eventually, the muscles adjusted.

Mentally, the transition back to my regular life was a lot harder.  This I foolishly didn’t anticipate at all.  I can’t really put into words what it feels like to leave your office one day and not come back for two years.  Everything was the same and everything was different.  The place had kept moving along, cases and lives moved along during those two years.  My life just stopped.  It was really disorienting.  I felt a bit like Tom Hanks in Cast Away after he returns to civilization.  Did I still really have a place here?  Would I be able to find a new place here?  Could I handle the hours?  The old me loved the high stress, crazy hours of my job.  Would the new me feel the same way?  Who the hell is the new me anyway?

I felt completely off balance and untethered for the first few months back.  Slowly but surely, I have started to regain my footing and get reintegrated.  There were lots of discouraging and overwhelming days and hours, but little by little I started to feel at home at work again.  I have a lot of support at work and at home and that helped a lot.  But it has not been easy.

When I got sick I felt like I completely lost my identity:  I didn’t look like myself, I couldn’t function like myself.  My work and running were a huge part of my identity and I lost both.  While I was sick, I developed a whole new circle of connections to people that were sick and struggling with the same disease I had, people who also got ripped out of their normal lives and were trying to come to terms with their scary new reality too.  I am functioning very well now, but still have signs of some problems and feel like I am standing with one foot in each of my former worlds – my former healthy world and my former sick one.  I don’t feel like I completely fit in either one anymore.  The big challenge for me right now is figuring out how to integrate them both into what is yet to come.

 

 

 

 

Back to Work

I have now been back to work for the “first” time (full disclosure: I had a few failed attempts) in two years.  The first few days were a strange, disorienting experience.  I felt like a burglar sneaking into the building, fully expecting my pass to set off alarms when I went through security.  I had a nagging worry my office wasn’t going to be there.  I stopped by a colleague’s office – a dear friend – before going to my own office to try and delay facing what I feared: I had no office or job and somehow no one told me before today.  My friend (who is much more senior, delightfully direct, and would obviously have known and told me if I had no job to come back to) had her back turned to me when I knocked on her open door and said “give me a minute” a couple of times before turning to face me, while I stood there with a growing knot in my stomach.  And then she turned around and saw me and yelled my name and ran over and gave me a big hug.  One hurdle down – it didn’t appear they were going to throw me out of the building.

Now for hurdle number two.  Together, we walked over to my office – I really did have myself convinced at this point my office had been moved.  And let me say, this is not a crazy belief to hold after being away from your job for two years.  How many firms would hold a person’s office for them for that long, especially when it is unclear if and when the person is coming back?  I really loved my office too.  It was in a great location, in a quiet corner with floor to ceiling windows on two sides.  Many times, I thought about my office when I was sick.  All the light coming in, the view out my window.  I know it may seem strange to think about this kind of thing, but that office was my home away from home.  I could imagine people asking why someone else didn’t get this office, especially as our firm had been growing rapidly and running out of space.  I could imagine my stuff being boxed up, my painting and photograph being taken off the wall and packed up and moved to a darker, more temporary office.  Again, I recognize this may sound like a weird thing to be thinking about when you are in the middle of a health crisis, but this thought did cross my mind many times.  I suppose the act of imagining someone taking the pictures off the wall in my office represented my fear that I was never going to get this huge piece of my  identity back.   This fear was almost tangible as we made our way through the corridor.  And then I walked in and saw everything was the same as the day I left.  My pictures were hanging on the wall.  My coffee cup was still sitting on my desk. Even the tube of Chapstick that I kept between my computer monitors was still waiting for me to pick up as I used to do as I read through my email first thing every morning.  It was all there, every last physical detail of my life in that office, exactly where I left it.  And then it all of a sudden felt like the last two years were just a bad dream, that I was just coming back to my office after the weekend.

I went back and forth between these feelings for most of the week – I either was in the building illegally and  waiting to get caught or the last two years simply didn’t happen – as I slowly started to get everything working again and get reconnected with people at work.  So many people stopped by my office those first few days.  Like me, they were also in shock I was back.  Some people ran over and hugged me.  A few people blinked back tears.  I did too.  I can’t quite put into words how it all felt.  All I can tell you is that it was one of the strangest and happiest weeks of my life.

I had to figure out a way to try and ease back into work while I figured out how much work I could physically handle.  I am so much better than I was even a month ago, but I still tire pretty easily.  At the end of a few hours in the office, I realize that the muscles I use to stand or sit for extended periods of time feel sore and exhausted.   My abs were literally sore from sitting upright.  That gives you some idea of how much time I had been spending in bed.  And although I tried to wait to come back until I was relatively stable, it had really only been six weeks since surgery #3 and I still had some outstanding problems.  Was my body going to be able to handle the regular stressors of work?  The work our firm does tends to involve some erratic hours and a fair amount of stress.  The unpredictability of the job was one of the things that (in moderation) made the job fun and exciting.  But how would my piece of junk body feel about it now?  The plan was going to be to try to find the right balance of “exciting and erratic” and “calm and predictable” hours.  I was also going to start with part-time hours and slowly ramp up to full-time (which around here is typically 50+ hours a week) gradually.

The other big problem I had to deal with after my first week was the fact that I literally had nothing to wear.  When I left work the first time two years ago, I was a size 2 and had a closet full of clothes.  The first set of tumors caused me to gain 70 pounds.  The second set of tumors made it extremely hard to breathe and I was barely able to get around.  It was impossible to lose the weight, due to ongoing hormonal problems and the fact that I could not exercise at all.  I have been able to lose over 20 pounds between July and now, but obviously still have a long way to go.  Buying new clothes while I was at this “transition” weight was something I avoided doing unless absolutely necessary because it wasn’t very fun, frankly, and I didn’t want to waste money on clothes that I didn’t plan to fit into for very long (if you are thinking “it’s been two years already, just buy some clothes and don’t worry about your appearance” you can shove it up your ass.  It’s a lot harder than you think.  Go gain 70 pounds and then just get over it.  Please let me know how that works out for you).  So when I went into my closet and realized I own one pair of pants that were work appropriate and fit, I knew I was going to have to do something about this whether I liked it or not.

Luckily for me, I have a secret weapon – an incredibly stylish and talented sister-in-law.  We went through my closet and tossed or stored everything that didn’t fit.  She then helped me to order a few things that will be the work wardrobe to get me through what will hopefully be losing the next 50 pounds or so (if all goes according to plan).  The gods are clearly smiling on me as ponchos are very much in style this year – and they come in one size!  Many of them are dressy enough for work.  With a stretchy pant and some nice shoes, I hope to continue to shrink underneath the poncho of the day.  I now have a work uniform that can get me through this transition period.  I couldn’t be happier.

The weekend after my first week of work, I attended a brunch at the home of my friend, the one who walked with me to my office for the first time.  The brunch was to celebrate my marriage to M back in April.  Two of my other colleagues were also invited to the brunch, along with their spouses and children.  All three of these colleagues are in the most senior positions in our firm and are, in one capacity or another, my bosses.  We have been trying to coordinate this brunch since April, but between their bruising schedules and the fact that I have been very sick and unreliable, it has taken awhile to find a date that worked for everyone.  This weekend turned out to be the perfect time to celebrate.

The morning of the brunch,  M and I were running around doing some errands.  As I was driving alone in the car, listening to the radio, enjoying the fall foliage and looking forward to seeing everyone at this brunch, I was struck by a wave of gratitude out of the blue and started sobbing.  I was feeling better.  Not perfect, and I didn’t know how long it was going to last, but at this present moment I felt better.  A lot better.  I had started dating a wonderful man just as I was getting sick and he stuck with me through all this and married me during the worst of it.  I worked at a firm that not only didn’t boot me out when I got sick and had to take a long leave of absence, they made me feel like a long-lost relative when I came back.  I had bosses – the people I was going to have brunch with in a few hours – that were smart, funny, kind people who not only facilitated this process at work, they KEPT COMING TO SEE ME THE ENTIRE TIME I WAS SICK.  Every single month, the entire time I was ill, I saw at least one of them at my home.  I can’t tell you how many bouquets of flowers, gift baskets, and plants my firm sent me after surgeries, hospital stays and setbacks.   I appreciate the flowers and goodies very much.  But I can’t even begin to tell you what the regular visits did for me.  Anyone who has been sick for a long time can tell you how truly extraordinary this is.   That they didn’t give up on me and just disappear helped me keep hope alive when things weren’t looking or feeling very hopeful.   Later this morning, I was going to celebrate all of these wonderful things and wonderful people.

I used to think the phrase “stop and smell the roses” was just a stupid cliche.  Forget smelling the roses.  I am going to hurl myself into the rose bush every single day for the next while.  This period of time feels so magical after the past few years.  I plan to embrace every second.

Lean In

I just got my first round of labs back after my parathyroid surgery in September and the results were very disappointing:  my calcium and PTH levels are exactly the same as they were the day before surgery.   It is not 100% certain at this early stage, but this likely means we didn’t get all the bad glands during surgery.  Merlin was only able to locate three parathyroid glands (most people have four, but people can have less than four or way more than four as well) during surgery and two of the glands looked healthy so only one was removed.  Does this mean there is still another bad gland hiding somewhere in my chest?  Time would tell but it looks like the writing is on the wall, at least in chalk.

I am not at all upset at Merlin – I think he was very thorough and careful.  He spent 3 1/2 hours exploring my neck and the top of my chest during the surgery.  He removed one hyperplastic gland. He biopsied all of the remaining glands and removed a bunch of lymph nodes and some residual thymus as well.  For all these reasons, I am very glad I had the surgery and I am glad Merlin was my surgeon.   I also knew there was a very high probability I would need another parathyroid surgery down the road, as the two tiny healthy glands that he did find and biopsy were likely going to eventually go rogue at some point too.  But I did hope for a few years between now and then with no parathyroid problems in the interim.

The really crappy thing is that I had decided today was the day I was going to try and start transitioning back to work.  Maybe just a few hours a week, but I wanted to try.  I certainly don’t feel well enough to work full-time at this stage.  I am not even sure how consistently I can work part-time.  But I feel well enough to at least try.  I loved my job and I miss work.  I miss my colleagues.  I miss feeling like a productive member of society.  I am not saying that is how people that aren’t working should feel, but that is how I feel.  Work was a huge part of my life and I liked it that way.  Getting back to work, for me, means getting back to normal.   I am not ready to let go of my career, not even close, despite the fact I have been off work for the last two years.

I had initially planned on going to work first thing in the morning.  That changed when I got the email with my labs.  I needed to process these results, which were clearly a setback.  I was doing pretty well for the first few hours this morning.   I was going to do a few things at home, go for a walk, and then try going to work in the afternoon.  I made my way onto the bike path behind our house and I wasn’t even a mile from home when I started sobbing.   The disappointment hit me hard.  Why can’t anything just go smoothly?  Just once?  I am sick of surgeries.  I am sick of problems.  And what did this mean for my ability to go back to work?  How can I go back to work, knowing it is probably temporary?  How many surgeries are still coming?  How many hours am I feasibly going to be able to work?  Will I ever be able to work like my old self again?  I just felt so overwhelmed and defeated.

I called a dear friend, my sister-in-law, to vent about this news as I walked and bawled.  She commiserated with me and then reminded me of a very important fact:  I AM getting better.  Twice now in the past two years, I have been so sick that I was bed bound because of two different sets of tumors.  Twice, surgery made me better.  I am nowhere close to 100% better.  I am still pretty weak, I tire easily, I am still carrying around an extra 50 pounds from tumor #1.  I am only at about 60% – but that is a hell of a lot better than the 5% I was at before.   A good cry and this reminder did make me feel better.

The other thing that made me feel a lot better was the words of wisdom from another very smart woman – Sheryl Sandberg.   One of the partners at my firm, another amazing woman who became partner at a crazy young age and manages to balance a young family and a demanding career with grace, recommended the book “Lean In” to me some time ago.  I decided to listen to the book on Audible while I walk as a way to pass the time.  After I finished my phone conversation, I decided to pick up where I had left off in the book at the end of my last walk.  As I sniffled my way along the bike path, listening to the book, I felt like Sheryl Sandberg was talking directly to me.  She was talking about one of the biggest mistakes she sees women make in their careers – women that are planning to have kids in the future often start scaling back the types of projects they take on and the role they play at work well before they get pregnant in anticipation of the fact that they will be taking time off to have a baby in the future.  Sandberg tells her readers that this period of time – the time when you are anticipating having to take time off in the future – is exactly the time to “lean in” and take on challenging projects.  Work while you can.  Take off time when you must.   Good advice for women planning families and good advice for me.

I walked home, washed the tear marks off my face, and started to get ready to go to work.

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.

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.