The story of a treasure map in the hands of a blindfolded and handcuffed man

After arriving in the ER Monday night, I was finally transferred to a regular hospital room Tuesday evening where I stayed until Saturday afternoon.

While in the hospital, I was treated with Pulmicort, the third steroid asthma medication I had tried this month.  As with the others, it didn’t help improve my breathing function.  And just as before I went to the ER, I had exactly the same breathing problems as before:  if I stayed very still and quiet I could rest comfortably.  If I tried to get out of bed or talk, I would gasp for air and cough.

I met the attending that would be responsible for my care Wednesday morning.  I got a very good first impression of him.  We talked about my symptoms and he looked at my chest where there were still clear localized signs of flushing.  I talked to him about some of my other odd labs (e.g., my very low TSH despite lowish T3 and T4 and the connection to somatostatin) and he seemed to take all of this information in and was receptive to the fact that this could all point to some sort of endocrine problem.  He wanted to get an endocrine consult and told me he would be lining that up.  I passed along the Wizard’s pager number and asked him to contact him as he knew my case better than anyone. I had been letting the Wizard know what was happening via email updates as the situation unfolded so he knew what was going on as well.

But the endocrinology consult never happened.  The next day, I was told the Wizard couldn’t be reached.  A message had been left for him, but the name and phone number on the message wasn’t clear and the Wizard’s admin person couldn’t make out anything except the main hospital number in the message.  She also eventually left a message for the attending with the Wizard’s private email address to help the attending connect with the Wizard, but the attending never emailed the Wizard.  The attending wanted the Wizard to fax over all my records (which suggested someone wanted to look at them, although this seemed so much less efficient than just talking to the Wizard) and to speed that up, M kindly brought all my records to the hospital.  The attending refused to take them from me.

Partway through this process, I asked the attending for his contact information so I could help facilitate a direct contact with the Wizard.  After he left the room, I picked up the card to look at it and realized I couldn’t read it.  My reading vision within about 14 inches of my eyes was all messed up.  I couldn’t focus and everything was blurry.  My longer range vision was perfect, which is why I never noticed the problem until this point.  Right away we alerted the nurse who paged the doctor.  He ordered a CT scan of my head right away and we were told the CT scan came back normal.

At this point I am going on three days in the hospital with no additional tests, no endocrinology consult, no plan. I started to get more pushy and asked more and more questions when the attending would come in to see me.  Given the asthma meds weren’t working why weren’t we doing more tests to rule out problems other than asthma?  The Octreoscan pointed to a potential thymic NET and the NIH said I was supposed to have a follow CT scan right about now, so could you please order one?   Thymic tumors could cause vision problems and there are blood tests to determine whether this is the problem. Can you order those?  Given the flushing, can’t we please run some carcinoid tests?  Do you  have any explanation for why my blood pressure spiked on Monday night?  Can you please email the Wizard?  And so on.

And the result was that the attending stopped coming by as often.  And had random and stupid answers for my questions.  He doesn’t see any breathing problems because I am breathing fine when he comes to see me (because I am in bed) and my oxygenation levels are fine.  The nurses are upset by this and start leaving detailed notes in my electronic record about the fact that THEY CAN HEAR a wheeze in my chest (in the same spot as the Octreoscan of course) and that when they see me move around I AM struggling for air and coughing.  They also tell the attending repeatedly they think I need a CT and a bronchoscope, but they don’t get anywhere either.  When I point blank asked him for a CT scan of my thymus, he said maybe later but that he was reluctant to expose me to further radiation for my own safety.  He thinks maybe I am flushing because I have hyperthyroidism because my TSH is so low, but the thyroid tests he orders indicate I have hypothryoidism, not hyperthyroidism (as I have been saying all along).  The blood pressure spike?  He wonders where the ER personnel simply put the cuff on wrong and it was an error.  As he wasn’t there and didn’t see it himself, he didn’t want to comment on that.   My new vision impairment?  He told me that maybe I was diabetic because I had Cushing’s, but I had never had blood sugar issues and he never actually ran any tests to see if in fact it was a blood sugar problem.  He never ordered any sort of follow-up tests for my eyes in the days in the hospital that followed.  He refused to order the blood tests I asked about to rule out thymic causes of vision problems because he feels an endocrinologist should order those, and of course he hasn’t been in contact with my endocrinologist.  The only thing that did get ordered was a 5-HIAA 24 hour urine test for carcinoids, which I was happy to get started on (although this particular test is rarely useful in diagnosing thymic carcinoids it was at least something).

When he walked into my room Friday morning, I burst into tears.  I was trying to be a reasonable patient, but the weekend was coming and I knew nothing was going to happen over the weekend and I was just asking for reasonable tests and for contact to be made with my endocrinologist and nothing was happening.  I was beyond frustrated at this point.  And so finally he agreed to order some tests and consults.  But the ones he ordered were pretty useless.  He ordered a second pulmonary function test, which indicated I had evidence of obstructive lung disease that didn’t improve after taking asthma meds (i.e., the test results were NOT consistent with asthma).  He also, for reasons that completely escape me, ordered a consult with an ENT who put a scope through my nose to look at my voicebox – a good 4-5 inches above the problem area – to make sure the problem was not coming from my throat.   Which of course it wasn’t.  Why he would order a scope of my voicebox but not my bronchial tubes is a complete mystery to me.  All the ENT saw was an area that was a little beat up, likely from GERD.  And they put me back on Prilosec.   Needless to say, this has not helped my breathing improve.

The attending promised me he would come back Friday afternoon so I could talk to him about the plan before the weekend.  But he never did.  I had the nurse page him and he never responded.  So I waited in hopes I would actually see someone Saturday morning.  After four days of sitting and waiting in a hospital bed, I wanted to get out at this point so I could at least go somewhere I could get some help.

In part, I am angry at the attending at this point.  It seems so incredibly obvious to me what needs to be ordered and his refusal to do anything is beyond frustrating.  How can he be so f’ing blind?  How can he not just run some of these things to rule out a carcinoid, if for no other reason than he has no other (intelligent) ideas for what is going on?  But if I try and put myself in his shoes, I do feel bad for him too.  If I were him, I wouldn’t want a patient like me coming in.  No way.  No local endocrinologist (and bad blood with the local endos after they completely bungled my Cushing’s diagnosis and I complained about them last year).  And an array of weird and confusing symptoms and nothing but a suspicious Octreoscan.  And what is he going to do with that?  He has never looked at an Octreoscan in his life and never will – that is not what he is trained to do.  He is a hospitalist whose job is to stabilize people, get consults with the right doctors and get the patients out of the hospital.  The blindfold I blame on him.  The handcuffs I blame on the system.

And part of that “system” is me.  I know from my discharge notes that the hospital is frustrated with me because my care is fragmented – I have specialists and doctors all over the place.  But this did not happen because of poor planning or carelessness on my part.  When I started getting sick back in 2012, I had all my care through one hospital.  I had two doctors –  one endocrinologist and one PCP – and I had been seeing both of them for many years and they communicated easily with each other.   But then I got sick and repeatedly saw other doctors in the system that failed to diagnose me.  And my PCP was unable to get me to a specialist that could help me locally and made it clear she thought it was laughable that I was considering going to LA to see some doctor she didn’t know to get help.  But after exhausting options at three local hospitals, it was necessary.  This is not an ideal situation for me either.  But it was necessary to get rid of Cushing’s.  And so now that I am sick again, trying to sort out another rare endocrine problem again, I am doing so with fragmented care.   Its frustrating for the doctors and it is frustrating for me.  But that is my reality and the reality, I have learned, of many patients with rare diseases.  And there is no way I am going to stop seeing the Wizard now – he is far away and outside the system but at the moment he is the only doctor I trust can help me sort this out.  Again.

Saturday morning, there was a very welcome breath of fresh air.  A new attending came in and after I showed her the picture of my chest and explained what was going on she said to me “Well! We should order a CT of your chest!”  And it was done within the hour.  Thoracic surgery also came to see me and collected my history all over again.  And everyone looked at the CT images and came back and told me…they hadn’t changed since the ones done at the NIH.  The report doesn’t sound like they are clear on what exactly they are looking at though, which is consistent with the debate that occurred at the NIH. The report describes what they see as soft quadrangular tissue in the mediastinum “most in keeping with residual thymic tissue or thymic hyperplasia.”   The radiologist thought that I needed to have more detailed scans (i.e., an MRI) to try and get a better look at what this “thing” is….in 3 to 6 months.  If we need to get a better look at this “thing” why wait?  Why not get a better look now, given my symptoms NOW and the fact that you aren’t sure what you are seeing NOW? That is a question I don’t have the answer to.  Thoracics subsequently told me they agree something is clearly wrong but they don’t have the expertise as surgeons to sort it out, so I needed to see someone who did.  And while this is a perfectly reasonable answer, I am not given a referral to see anyone that might have this expertise when I am discharged that day.

Sunday night, I had a chance to talk to the Wizard.  His conclusion:  “you need to get that thing biopsied.”  It is his opinion (and mine too) that we need to rule a thymic carcinoid out.  Carcinoids in general can be pretty slow growing, but thymic carcinoids in MEN-1 patients are typically very aggressive – they are a leading cause of death in MEN-1 patients (who already have markedly lower life expectancies) and the thymus is often removed as a preventative measure in MEN-1 patients to avoid ending up with a thymic carcinoid.  Needless to say I am anxious to get this sorted out in the event this is what we are dealing with.  So, I return home and wait for Monday morning to try and figure out how to make this happen.

In case you are wondering about the one relevant test that was ordered…  The 5-HIAA test for carcinoids was rejected by the lab because the wrong collection container was used.  And the test was not reordered.