The aftermath of Cushing’s…what the hell is wrong with me now?

Just to give some context for what is to come, I am going to try and summarize where things stand for me right now.

During the process of recovering from pituitary surgery for Cushing’s last February, I started to develop increasingly severe back pain in May.  I had radiofrequency ablation (a doctor sticks a probe into the spinal facets and burns the nerve endings off) twice to help reduce my pain between June and November.   I had an incident in mid-September (that I thought was an adrenal crisis) which ended with a seizure in a local ER.  And after the trip to the ER, things started to spiral downwards again.  I was initially worried Cushing’s had come back, but this just didn’t feel like Cushing’s…

So it was time to take stock and start investigating again.  Throughout the spring and summer, the only abnormal labs I had were consistently high Alkaline Phosphatase (ALP) results.  This can indicate bone or liver problems, but related testing indicated mine was pointing towards bone.  I also had a few high N-telopeptide labs – combined with my high ALP, this pointed to high bone turnover which is not normal for someone in their 40s.   Since I was having bone pain and since these labs were abnormal, I started doing some digging around to try and figure out what this could be.   The list of things I found included bone diseases like Paget’s and osteomalacia (caused by a Vitamin D deficiency), but neither seemed to fit.  Other things on the list were various cancers (of which I had no other sign) and hyperparathyroidism.  Primary hyperparathyroidism (HPTH) is caused by a tumor in the parathyroid gland that produces excess parathyroid hormone (PTH) and the excess PTH stimulates your body to find calcium and dump it into your bloodstream.   If you substitute pituitary for parathyroid, ACTH for PTH and cortisol for calcium, you basically have Cushing’s – so I knew this drill pretty well already.  I needed to start getting repeated calcium and PTH measurements to try and rule this in or out.

I did notice that my calcium fluctuated a lot while I had Cushing’s – and calcium (in a healthy person) is supposed to stay in a very narrow range.  So this looked suspicious to me.  I also noticed that while I was getting testing for Cushing’s my PTH was at the top of the normal reference range on a few occasions.  My research also indicated that cortisol could suppress calcium – so maybe this is why when I tapered off hydrocortisone (essentially a synthetic version of cortisol that is taken after surgery until your pituitary can regulate cortisol properly again) my bone pain started?  Could it be that I also had a parathyroid tumor and that the excess cortisol I had during Cushing’s was hiding it?

The only way to test my theory was to start testing my PTH and calcium. And so this is what I did in September 2014.  The Wizard ordered a round of tests and lo and behold, between June and September my calcium had jumped from 9.3 to 10.3.  Calcium doesn’t do that in a normal person and according the website of the parathyroid surgeon  that does more of these surgeries than anyone in the US (, people in their 40s should NOT have calcium in the 10s.   Typically, PTH is also high for patients that have HPTH.  Mine was not “high” (it was in the 20s), but it was still a little “too high” given my high calcium readings – when calcium is too high, PTH should be really low so your body stops dumping calcium in your blood.   Even though I didn’t have a smoking gun, the Wizard agreed to humor me and we did several sets of calcium and PTH tests over the next few months.  They all indicated I had calcium levels in the 10s or very high 9s.  And my PTH kept steadily increasing too, from low 20s up through the 30s.

I was pretty convinced I had a parathyroid tumor by now.  In addition to my labs, my new and progressing symptoms fit HPTH as well.  I had a lot of bone pain.  Despite starting to run and go back to work between May and September, I was getting extremely fatigued again.  Despite growing back a lot of the hair I lost during Cushing’s, I started losing hair in chunks the size of a quarter.  My blood pressure had almost come completely back to normal after Cushing’s, but now was fluctuating between normal and 150/100.  All signs of HPTH.  Unfortunately, all signs of 100 different diseases too.  But just like when I had Cushing’s, the symptoms and the labs really did point in the direction of a parathyroid problem.

And if I did have a pituitary tumor and a parathyroid tumor?  That pointed to much more serious problem:  Multiple Endocrine Neoplasia, Type 1 (MEN-1).  MEN-1 is a genetic mutation that predisposes patients to developing neuroendocrine tumors (NETs) in the pituitary gland, the parathyroid glands, and the pancreas.  Having tumors in 2 out of the 3 locations means you will be diagnosed with MEN-1.  There are also genetic tests for MEN-1, but the tests only identify a known mutation in 60 percent of cases.  MEN-1 sounded like pretty bad news and the more I read about it the more I really hoped I didn’t have it.  But if I did have it, I wanted to make sure I found out about it as soon as possible so I at least could find tumors and remove them as fast as possible.

And so by November, with my growing collection of calcium and PTH labs in hand, I was on a mission to rule HPTH (and MEN-1) in or out.  If I had MEN-1, I would have to begin figuring out if there were other tumors lurking around that needed to come out and begin a lifelong process of checking for tumors and removing them.  If I didn’t have HPTH?  I was back to Square 1 and would have to start down another path to try and figure out what was wrong.