updates

So the BMB was sort of a disaster. To make a long story short, somehow, somewhere, communication lines got twisted and i was not tested for the c-kit mutation. It doesn't matter how or why it happened. It just did. What we do know is that I have a 10% infiltration and that my mast cells are spindle shaped. So now we know without a shadow of a doubt that I have Indolent Systemic Mastocytosis. We also now have a baseline for mast cell infiltration, should it get worse. They are trying to see if they can test the remaining aspirate for the mutation, but it is unclear if that is possible. So the waiting game continues. Rad.

In other news, last Friday (the 13th!) I was supposed to have lithotripsy as I've got a kidney stone blocking my left kidney. Well, I went through the whole pre screening process at MGH for anesthesia and it still went all sorts of wrong.

this is what i wrote to a mailing list:

Soooo, tomorrow I am scheduled to have my lithotripsy. Tonight I find out that my anesthesiologist (at MGH, as only MGH and Faulker seem to have lithotripsy machines and all my doctors stressed I needed to go to MGH even though that meant scheduling 2 months out since the doctors at MGH would be far more familiar with mastocytosis than at a smaller hospital) has never seen a masto patient. She then tried to reassure me by telling me she'd read some case studies. I then asked her what treatment plan her and my mast cell specialist had come up with since when I went in for my pre-op testing the intake doctor stressed how important communication with my team of physicians would be, specifically my immunologist and my GI specialist since both of them are heavily involved in mast cell patients and surgical protocol. It is imperitive since I know of a few masto patients who ended up having their masto discovered after they flat-lined during simple surgery due to reactions to anesthesia.


Well, she didn't actually call my mast cell specialist (instead for some reason i cannot understand she contacted my endocrinologist, who while awesome, i've seen maybe 4 times), but she did read some papers (even though they've had my immunologist's contact info for like, 2 weeks). She then said if I was uncomfortable with her lack of firsthand knowledge of the disease she'd page my immunologist tomorrow (being as I have to be at MGH at 7am I have no idea what time they were planning on paging her) only my immunologist is going down to DC tomorrow for some important immunology conference. which may be an even bigger deal now that stem cells are back on the table or being looked to be put back on the table.

So I had to spend nearly an hour on the phone with the doctor explaining, again, that no I don't have rast-tested allergies but that I instead have indolent systemic mastocytosis which is not the same as having allergies save for the risk of death due to anaphylaxis. It was awesome that they called me the day before surgery to ask these questions even though I went in for a 3 hour pre-op consult nearly two weeks ago where none of this came up.

In addition to this excitement, I was told by the pre-op anesthesiologist that I would need to meet with the Chief of Anesthesia before the procedure since my case would be tricky. Only
that guy is out of state too now.

So I am so freaking out right now. Since I am about to get my period my mast cells are more active than usual and I've been flushing like mad, I got hit with the not-testing for the c-kit mutation drama fresh in my mind, and now it's like, every possible thing could go wrong for my surgery scheduled for Friday the 13th, something I was never superstitious about until right about now.

Between the pms/masto instability, the fact that my immunologist is not reachable, the fact that my anesthesiologist has never had a masto patient before, the fact I was told to have the surgery at MGH instead of a smaller hospital specifically to avoid having an anesthesiologist
unfamiliar with the disease which meant delaying surgery for almost nearly 2 months, the fact that the Cheif who I was apparently "supposed" to speak with due to the complicated nature of my case is also unreachable, has left me feeling very very very uncomfortable with the idea of having surgery tomorrow. I am about 10k times more anxious than I was about the BMB, and I honestly am wondering if, if delaying the surgery to fix the above messed up variables won't cause renal damage, if it wouldn't be worth rescheduling. Of course, I would've liked to have known all this before the night before the surgery too, so as not to have to make a snap decision first thing in the morning after i get to the hospital.....

It is so frustrating. I mean, MGH and Dana Farber are considered to be some of the best hospitals in the world and after the past two weeks they make me want to put my head through the wall.
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After this, I saw my surgeon who was also unhappy with how things were handled and then decided to postpone the surgery. It honestly seemed like the anesthesiologist was more excited to have a House-like patient than actually listening to what the patient was saying. So now it looks like I am having the procedure done on the 27th now, with a dedicated anesthesiologist and all of that.

And then after that I have my disability hearing. yeehaw.