Friday, September 26, 2014

Persistent Cushing's Disease -Update 9/26

I was able to get into an ENT at Cedars-Sinai and it turns out that the sinus infection is gone, but I'm left with a LOT of scar tissue in my sinuses that is very easily inflamed.  Fun times!  This MD actually scoped me to see what is going on up there.  He didn't swab it either, but that's understandable if the infection is gone.  I'm to try nasal steroids (endo okay'd it), use a humidifier, not use any fans, A/C, etc., Sudafed when I get congested, and contact him again in a month or two if that doesn't work.  It isn't really working, but we shall see.  I'm not feeling well enough to fight that battle right now, though I change my mind every time the sinus pressure becomes severe.  :)

I saw my Endocrinologist as well; he agreed I am still sick, but my testing is too mild to move on to a BLA.  (I knew it, but, UGH!)  I am on ketoconazole now to lower my cortisol, but mostly because it disrupts the feedback loop and can "anger" the tumor.  This can lead to tumor growth (so that a target shows up on MRI) and/or the tumor can start to ramp up it's production, overpowering the keto's effects.  I had this happen last year when I was on keto.  After about a month or so, it stopped working as well to lower my high cycles and I had to up my dose.  Sometimes my endo uses this medication like a further test for proof of disease.  And when that overpowering happens, going off the keto and retesting can yield much more definitive test results. 

So anyway, I'm trying to get on the dosage of keto he wants me on.  With my cyclic disease and the bulk of my tumor gone, my high cycles are lower (though still too high) and my low cycles are lower as well.  It's been a challenge, but I'm keeping at it.  After 2 months on the medication I will do some lab testing to check my liver function to be sure I'm tolerating it well in that regard, then in November I will have a repeat MRI and follow-up appointment to reassess the plan.

If a pituitary target does show up, I will move on to repeat pituitary surgery (he might want me to go off the keto and test another cycle first, who knows?).  This isn't the best option, but it isn't the worst (medication).  2nd surgeries have only a 50% remission rate, a significantly higher risk of pituitary damage causing hypopituitarism (and requiring lifelong hormone replacement), and a much higher risk of CSF leaks (requiring repair, not being upright, etc). 

If a target does not show up by November, he may have me stay on medication (I pray to God that is not the case!), he may have me go off medicine and retest to see if my results are far more definitive allowing for BLA clearance, or he might consider that as ample proof along with my lab work and allow me to move on to my bilateral adrenalectomy surgery. 

There you have it! The keto definitely helps in some ways (I can sleep at night), but it brings me too low during the day when my cortisol levels are already lower than they should be (due to the flipped diurnal rhythm of Cushing's Disease).  I'm playing the waiting game and hoping it ends soon.