M and I sat down with the surgeon for a follow-up yesterday morning. He started by asking how things had been going over the last three weeks and I explained that my digestive symptoms had been getting much worse and that I had been doing some “controlled experiments” to see if my troubles were related to eating various foods – and that certain foods did seem to trigger attacks. He informed me that the findings of my experiment actually didn’t seem to fit carcinoid syndrome and as he started to explain why, all I could do is tell myself over and over, don’t cry don’t cry don’t cry.
On his end, he told me he had been busy looking into my case. Part of his investigations involved speaking with the endocrinologist I had been seeing at his hospital between 2006 and 2012 (one who did not diagnose my Cushing’s). Don’t cry don’t cry. He told me he sat down with his radiologist and they looked over everything together and they did not see anything obvious on the CT scan from the NIH (in January). Don’t you dare cry, you just hold it together until you get out of his office. It was happening again, just like with the oncologist and all the other doctors I had seen so far. He looked at my scans, decided there wasn’t enough evidence, talked to my old doctor, decided I was a nut case, and was going to tell me to go home. I never should have come here, I fucking knew it. Just don’t cry.
But he didn’t call me a nut case and tell me to go home and stop chasing imaginary problems. He said, “we need to go forward based on your flushing and the Octreoscan.” And he further said that what my former endocrinologist told him was that although my test results never squared up with Cushing’s while I was his patient (which is not true at all, but I was not going to argue this point now), it was always very clear something was really wrong and that my symptoms were real. He and my endocrinologist concluded that that this was the reason I had racked up so many frequent flier miles trying to get things sorted out. And he didn’t want me to rack up any more. He said it over and over again – your symptoms are real – and commented on the fact that I was flushing like crazy as I was sitting in front of him again. He asked M to look too: “Is this how she flushes at home? I have been sitting here watching you, and you have not scratched your neck or touched that area of your chest and you are very flushed. You are not red like that anywhere else. Your symptoms (I was coughing and wheezing too) are very real. You have ruled out everything else. And you have a positive Octreoscan.” He said he had consulted with thoracic surgery and they reviewed all of my information and determined that if I wanted to go ahead with it, they would do it.
At this point, I was very confused and so when he asked me if I had any questions I said: “wait a minute, just to be clear, is this really going to happen?” And when he said yes, I started flat out bawling. So much for don’t cry. I was so damn relieved. I did my usual Canadian “sorry, sorry, sorry!!!” as I searched around for a tissue and pulled myself together and then we got back to business. He told me about the surgeon who is going to do the procedure and that they wanted to get an updated CT scan and for me to meet with her in person in the immediate future. I had the scan this morning and meet the thoracic surgeon tomorrow. We talked briefly about the surgery and I am busy compiling questions and reading up on it today so I am prepared when I meet her tomorrow.
From this point onward, I am going to refer to this wonderful surgeon as Gandalf, as I suspect he will be sticking around long enough that he needs a name. [As an aside, my naming conventions are the following. If you are a doctor or a dear friend and you do a great job or are especially wonderful to me you will get a nickname like the Wizard or Gandalf (or the Coven). If you do an incredibly shitty job or do something else to really piss me off, I will name a tumor after you.]
Gandalf appears to be a doctor that is extremely careful with his words and he cautioned repeatedly that he can’t guarantee this surgery will fix everything or anything for that matter. That I should know going in that there was a chance that they could remove my thymus and I would wake up and still be symptomatic and all we will have done is rule out my thymus as the source of my current problems. He told me repeatedly that no matter what the findings from the surgery are it does not mean I will be diagnosed with MEN-1. And I get all of that. I realize there is a chance that the Octreoscan finding could be incidental. And that the flushing is caused by a tumor in an entirely different location. But I am much more willing to take the chance we are wrong about my thymus and we remove it for nothing than I am willing to take the chance we are ignoring a potentially malignant tumor because we can’t establish with certainty it is there. Trust me, I don’t want to have MEN-1 either. This is the kind of disease where if you tell a doctor you are being evaluated for MEN-1 the most common response is “I really hope you don’t have MEN-1.” I hope I don’t have it too! However, the only thing worse than having MEN-1 (or something like it) is having MEN-1 and not knowing it.
So my big question for him was “on the off chance I do have a neuroendocrine tumor in my thymus and this is confirmed post-op, if this isn’t MEN-1, is the fact that I had pituitary tumors causing Cushing’s last year and now this thymic tumor this year just exceptionally bad luck?” His response? First, there is more than an “off chance” I have a NET in my thymus (I think so too). Second, it would unlikely be a coincidence. It is likely that I either have what would be a very unusual presentation of MEN-1 or I have another MEN-1 like syndrome that the medical establishment does not yet understand very well.
And that is part of the problem I have been having. Although MEN-1 can manifest in a very wide variety of ways, typically people my age have other more common tumors by now – parathyroid adenomas (which I still think I might have), prolactinomas, other kinds of tumors. ACTH producing pituitary tumors (i.e., the kind that produce Cushing’s) are possible but relatively uncommon for MEN-1. Of the group of patients with MEN-1 (a very rare disorder) and pituitary tumors, only 4% had tumors that caused Cushing’s. In the general population, approximately 10 patients in a million get Cushing’s every year. We are talking very low odds. Thymic NETs are much more rare than even Cushing’s: approximately 1-2 people per 100,000 are diagnosed with a “carcinoid” every year. Out of this population only 0.3% are located in the thymus. This translates into 1-2 people of out 33,333,333 people a year getting a thymic “carcinoid” (the tumor we suspect I have). Taking the statistics one step further, the likelihood I would get both ACTH-producing pituitary tumors and a thymic NET by pure coincidence? Roughly 1 in 1,667,000,000,000 (all that stats training comes in handy sometimes!). Unfuckinglikely. Much more likely the same underlying disease process caused both. That’s not being a drama queen or a pessimist. That’s just looking at the numbers and being realistic.
So, no matter what happens with the surgery, I do understand (and have understood for quite some time now as I have been running around without a diagnosis) that this surgery is not likely going to be a cure-all. There is still a subset of symptoms that are plaguing me that are likely due to yet another currently undiagnosed problem. And I will still likely not come out of this surgery with a full diagnosis and fully articulated plan going forward either. But getting cleared for the surgery and hopefully getting one more tumor out of the road it is a big step in the right direction. And having Gandalf (and the wonderful people and organization that led me to him in the first place) in my corner is the biggest step forward of all.
a tale of two tumors 