Good Patients Are Good Auditors

In my former healthy life (and hopefully in my future healthy life), I was a litigation consultant.  I worked for a  firm full of PhD Economists and MBAs that provides economic analysis in the form of expert reports and testimony at trial in high profile lawsuits.  If you take a look at the Wall Street Journal on any given day, there is a good chance we are involved in the one of the cases on the front page.   Look around your house and I can almost guarantee I have worked on cases involving some of the products you use or the companies that make, market or sell them.

The work our firm does requires a great deal of attention to detail – not only because we want to make sure the job is done right on principle but also because there is a set of lawyers and consultants on the other side of the lawsuit that are going to dissect our work in great detail to try and find any sort of potential flaw in our analysis to bolster their case.  And so, for this reason, every important piece of analysis goes through a complete internal audit where a brand new team comes in and reviews every sentence, every source, every line of computer code to double and triple check the entire analysis.   The audit team and the team working on the original report, together, make sure the final work product is flawless.  This process is not in place because the original team is incompetent or untrustworthy – it is in place out of recognition of the fact that the team is made up of humans that completed a very complicated analysis under demanding time constraints.  Some angles or analyses may have been overlooked; some mistakes may have been made.  The audit process is time consuming and expensive but necessary to find those mistakes before the other side does.

As a patient, especially a patient with a rare and complicated disease like Cushing’s, it is important for the doctor and patient to work together in the same way.   Your doctor is the original team – planning and conducting the original analysis.  You are the auditor – going through each step of the completed analysis and double checking that the logic of the analysis passes the “sniff” test, that the numbers are correct and interpreted properly and that no stone was left unturned during the original analysis.  If you don’t serve as an auditor, you and your team could miss some very important mistakes.

I relearned this lesson over the course of the past two days as I reviewed some of my old medical records back from 2007.  Before the last few years, I didn’t audit anything.  I never even bothered to look at my lab results or even ask what labs my doctor ran.  I trusted my former endocrinologist completely.  Until very recently, it also never occurred to me that I had Cushing’s before the beginning of 2012.  So what I saw in my record shocked me.

As I look back through my records, I now see that as far back as May 2007, I was complaining of being very tired.  I had a known pituitary tumor since 2003 and my endocrinologist ordered follow-up MRIs every year to make sure it was stable.  I had some discharge from my breasts over the course of several years and my endocrinologist examined me, saw it for himself, sent me for a mammogram, and checked my prolactin.   As part of my initial work-up after joining his practice, he checked my growth hormone and found it was elevated.  We talked about the symptoms of excess growth hormone and he mentioned that your hands and feet can grow – and my feet HAD increased by a full size in the last few years.   My running shoe size (I have worn the same exact make and model of shoe for almost a decade) went up a size and a half.   I remember asking him whether this meant I might get a few inches taller too (that didn’t sound so bad!) and he laughed and told me just my feet would grow and that I would look like Ronald McDonald.

Given my odd labs, my breast discharge, my big feet, and my old pituitary MRIs, he ordered a few extra tests just to make sure everything was fine:  an overnight dexamethasone suppression test and a growth hormone suppression test.  The dexamethasone suppression test is a screening test for Cushing’s disease.  The growth hormone suppression test is a screening test for acromegaly.  Both disorders are caused by pituitary tumors that are secreting hormones- ACTH in the former case and growth hormone in the latter.

The suppression tests take advantage of the fact that tumors don’t respect the normal feedback loop of the pituitary gland.  A substance is administered (dexamethasone for the former test and glucose for the latter test) that in a healthy person is supposed to suppress the relevant hormone produced by the pituitary gland.   For example, after administering dexamethasone, a healthy pituitary gland will realize it should stop pumping out ACTH and cortisol levels will fall correspondingly.  If a tumor is producing ACTH, however, administering dexamethasone won’t result in a marked drop in cortisol because the tumor doesn’t care about the dexamethasone or what the rest of the pituitary gland wants to do.  It just keeps spitting out ACTH.   To make a long story short, the dexamethasone suppression test should indicate that cortisol levels are suppressed below a particular cut-off and the growth hormone suppression test should indicate that growth hormone levels are suppressed below a particular cut-off in healthy individuals.  If you don’t suppress on these tests?  You need to start checking to make sure you don’t have a pituitary tumor that is actively producing hormones.

Once the tests came back, he assured me during my follow-up that both tests came back normal and there was nothing to worry about.  I had been complaining of being tired on my current dose of thyroid meds and he was reluctant to increase the dose and told me it was probably the marathon training wearing me down and that I should try drinking more coffee (the last part is literally written into my chart).  As for my feet, we both chalked it up to possible design changes that Asics must have made to my favorite shoe.

This endocrinologist seemed super thorough to me – he ran all these tests, he ordered follow-up MRIs, he sent me for a mammogram.  So, it never even occurred to me that I should actually look at the test results or ask further questions.  I was tired, but I was working and running a lot and it’s probably normal to be wiped out from that.  So I drank more coffee and kept bringing it up with him in my appointments but we both concluded it probably was my lifestyle and not my thyroid making me tired, as my thyroid levels were pretty normal.

So imagine my surprise when I dusted off my old labs yesterday and today and discovered I failed to suppress on BOTH the dexamethasone test and the GH suppression test.  BOTH.  In 2007.  I am still trying to process this information.

I knew I failed to suppress on a dexamethasone test in 2013 and I had a heated argument with my endocrinologist at that time because he told me I did suppress on that test.  But in 2013, when it was completely obvious something was wrong, I looked up the cut-off and my lab values and found out he was using the wrong cut-off.  I sent him an email with the Endocrinology Guidelines laying out the proper cut-offs attached and pointed out that I had in fact FAILED to suppress on the 2013 test.  But by then, we were mad at each other and all he was willing to do was see me in his office in six weeks after refusing to order a follow-up MRI.  That was our last conversation.

When I had my final set of labs drawn at MD Anderson the day before pituitary surgery for Cushing’s in 2014, my IGF-1 was beyond the top of the reference range.  And I had really severe carpal tunnel syndrome at that point and noticed my tongue felt too big for my mouth as well.  So at this point, I already suspected my growth hormone might have been too high in addition to my ACTH.  But I am now much more suspicious that my two little pituitary tumors were probably producing both hormones – and that I wasn’t someone that just started getting rapidly sick in 2012 but had actually started getting sick as far back as at least 2007.