Wednesday, April 1, 2015

APRIL FOOLS! YOU'RE STILL SICK! Enjoy That Tight Rope You're Walking...

Well, once again it is April, which is Cushing's Awareness month.  I have again decided to participate in the blogger's challenge, posting each day this month to raise awareness for Cushing's.  I've been mulling over what to say and how to start, with no inspiration or direction coming readily to mind.  And, as it turns out, a topic presented itself today, just in time. 

I received a phone call from someone I haven't spoken to for probably most of a year.  She obviously didn't understand that I am still very sick, and she didn't seem to understand that my husband has to take up all my slack, working full time (soon to include lots of overtime and unpredictable hours), caring for our home, cars, cleaning, cooking, doing things with our 6 kids, shopping, etc, etc. 

So here's the deal: I am very ill.  I'm STILL sick.  I'm still in recovery.  I still can pretty much only function enough to supervise kids and make sure they do their school work, chores, don't fight, etc. 

For the time-being, it appears my Cushing's Disease is gone.  I still have many years of recovering from that ahead of me.  BUT, in it's place I now have empty sella and hypopituitarism.  Basically about 9 months after my brain surgery, a follow-up MRI showed a new development that wasn't seen on my 2-3 month post-op MRI.  I now have spinal fluid pushing downward from around my brain onto my pituitary and turning what should look like a rounded bridge into a smushed U shape.  This isn't uncommon after pituitary surgery, and it sometimes doesn't cause problems, but it can kill off pituitary cells, cut off blood supply, etc.  There is a chance this is the cause for my Cushing's symptoms finally disappearing 9-12 months post-op.  After doing extensive testing that required a trip out-of-state, we know it has killed off the cells in my pituitary that produce Growth Hormone.  I am no longer able to produce any at all, even when given injections basically forcing my body to produce as much as it can.  I'll trade Adult Growth Hormone Deficiency (AGHD) for Cushing's Disease (CD) gladly, but it isn't simple.  And, GH is usually the first hormone whose production is affected following pituitary insult (from a turmor itself, surgery, or apoplexy, etc) so we have to watch and wait to see if this is the start of a cascade.  I pray my pituitary is able to keep the rest of it's function, but only time and tests and symptoms will tell.

I'm now replacing GH, with an injection every night.  It's been hard getting on the medication, and I will have to slowly try to up my dose till my IGF-1 hormone levels are where they need to be.  When one hormone level changes, it affects the rest of them, and they all have to re-stabilize.  I've had some awful symptoms because of that, having to drop my dose significantly from where I was started.  Apparently GH catabolizes cortisol, and what I thought were further symptoms of Intercranial Hypertension (another rare but known side effect of GH replacement, basically a more sudden form of what is causing my empty sella) my endocrinologist thinks were symptoms of my cortisol dropping dangerously low.  It took 4 days for the negative side effects to show up, then I cut my dose in half and things improved, but it took another week or two for it to mostly resolve.  It felt awful.  It's a month later and I'm not back to that starting dose yet, but I'm slowly inching upwards trying to get where I need to be, trying to keep the negative side-effects at bay. With so many hormones in play, all affecting each other, all with similar symptoms, and with so many factors, it is a confusing mess, but I am determined to make it work.

People ask why it is so important I replace this hormone, because the name "Growth Hormone" is a bit of a misnomer.  "You're certainly finished growing by now!"  It does far more than just allow children to grow, but it is indeed responsible for that in the young, and your body produces it throughout your life at varying levels because it needs it to function properly.  I'll perhaps go further in depth on that in a post later this month, but suffice it to say Growth Hormone directly affects your circulation, bones, muscles, metabolism, and ability to think, sleep, move, and function.  Having no growth hormone has made me SO much more physically exhausted than Cushing's ever did...and that was no picnic either!  There is no other treatment at this time, and I am not willing to simply not recover.

So, my doctors and I are still trying to figure out all the ins and outs of what is wrong with my body, trying to get it stabilized, and waiting to see how and if my body will recover or if I will continue to get sicker (due to the empty sella and hypopituitarism).  We hope I don't lose any more hormone production, but even replacing just the GH is a long process with an expected wait of 6 months prior to seeing improvement.  

I'm showing signs of insufficient adrenal reserve, but I still have some adrenal hormone production, further complicating things.  These hormones are required for us to stay alive.  It's a big deal.  And adding stressors to my day is a big deal.  If I'm already too low, any stress can send me into adrenal crisis. 

I already had to stress-dose once this week, on what appeared to be a low/normal stress day for me.  What did I do?  I stood up from watching a movie with my family, and suddenly I had a headache, nausea, my head was cold and hot, sweaty and clammy, with tinnitus, sudden sleepiness, and a generalized "swirly, I'm going to die" feeling.  I took 10 mg of hydrocortisone and within about 20-30 minutes I was out of the woods.  It was around 10pm, and yet because I needed that dose of steroids, I was still able to sleep like a baby (that's roughly half to 2/3rds of a normal replacement dose if I were making none of that hormone on my own, and if I didn't need it all, would have kept me wide awake for hours).  Had I not taken that medication, I could have gone into shock, followed by coma and death. 

I know that sounds dramatic, but it is true.  I've had friends simply not wake up from a nap because they went to sleep too low and died.  It's heartbreaking and tragic.  So, I have to take medication and stay awake (and not alone, because things can get bad quickly) till I transition from feeling like death to just feeling crappy...Till it's safe to go to sleep.  And then I have to watch closely in the morning and days that follow that I'm not still working on a deficit and in danger (though I guess that danger never fully goes away).

IF I were to get so bad that the oral dose wasn't enough or couldn't be absorbed (vomiting, diarrhea, unconscious), I would need my emergency injection of 100mg solu-cortef and to be rushed to the ER.  Luckily, I've never had to use it.  I've always caught it early enough and responded well to the oral medication.  I honestly think my inability to move and do much of anything has protected me in that sense.  It would be much harder to recognize all those symptoms early (and I'd probably crash far faster) if I were out and about and active.  Trust me, I'd rather BE active and functioning, but I can see the blessing in that, at least.

Whew, didn't mean to go there, but that's the reality of what I'm dealing with every day...still.  I didn't have AI issues post-op, in fact, my cortisol levels were still too high.  But somewhere around September-November 2014, things changed.  I'm now almost 14 months post-op, and seem to be going through some of the low cortisol recovery that most people experience as they wean down from replacements over the year following surgery.  I weaned off hydrocortisone fully within a month with no issues.

But now any stressor, whether I am emotionally/mentally able to cope with it or not, can be too much for my physical, autonomic response to handle.  It is like I have no reserve, or make just barely enough cortisol to stay safe.  I've said this before, but even something enjoyable like laughing and chatting with friends can be too much in a low, because it requires energy from my body.  And something stressful yet "fake" and enjoyable like a video game (and I'm talking 1980's nintendo here, not even HALO) elicits my body's stress response and can send me into crisis when I'm already deficient.  It's CRAZY, but it is real.  I WISH I could just take enough steroid every day to not have to worry about it, but it would cause one of two additional problems.  It could cause exogenous Cushing's, with the same symptoms of Cushing's Disease I had brain surgery to treat last year, because it would add more cortisol on top of what my body is already producing.  Or it would suppress what is left of my adrenal and pituitary function, essentially turning those processes off giving little to no hope of my body being able to produce those hormones itself in the future. 

I mean, honestly, I'd rather it either produce enough or none, so it was less confusing, but I'm stuck in no-man's-land.  I'm walking a tight rope of trying to keep myself safe, while not taking over my body's own production.  And all of this is a "play it by ear" thing.  I have to watch my own signs and symptoms and figure it out on my own.  It's a mess.  It's exhausting.  I just want to feel better.

Oh, I guess I should mention I'm also having a hard time regulating my fludrocortisone (another adrenal hormone) while on and increasing my dose of GH.  My aldosterone isn't stable, and that makes me feel icky, can cause headaches too when it's too high/low, nausea, loss of appetite, swelling, dehydration, etc.  Add to that that the medicine used to treat my post-op Cushing's damaged my liver and I'm eeking towards diabetes, and it's just...overwhelming. 

I know everyone expects a quick diagnosis, cure and recovery, but I didn't win that lottery.  I know there are many others out there dealing with this same post-op nightmare that I am, even though it isn't spoken about very much.  I am still fighting every day just to keep myself alive, all while trying *not to stress* or overdo it.  I don't know if, when or how things will get better.  I am not depressed, but it IS depressing, and there are days I don't know how I will continue on.  I still have hope that things will get better and I pray that day comes, but for now I just keep slowly pushing forward, because for me there is no other option. 

As long as I am on the tight rope, I will keep walking.  I am not scared of falling off the rope, but I won't knowingly jump off either.  I know there is an end, and though it seems impossible now, I will keep trying till I find it.


  1. Great start to this year's challenge! Sorry you're still on the tight rope, though :(

    Maybe next year you can kick that April Fool to the street and be done with him!

  2. Thanks, MaryO. I'd LOVE to kick the fool to the curb!

  3. I love your fighting spirit. I am so very sorry that you are still ill though. Keep walking and maybe very soon things will get better. I will be praying for you.

  4. I think my stress level just went up one hundred fold reading about this real-life tight-rope experience. You are a hero.