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.