Saturday, May 2, 2015

The Magic Foundation Adult Convention

The adult program and registration details are now available here!  

I'm so excited!  I'm planning to attend.  My endocrinologist is speaking Sunday, and some of the topics I look forward to most right now are things like dealing with insurance, oxytocin replacement, how hormones interact, hormone testing and replacement in hypopituitarism, and solu-cortef pumps!  YAY! 

Thursday, April 30, 2015

Why I Think I'm in Remission, and why this isn't the end.

A friend of mine requested I write this post. This is a question everyone asks, and often repeatedly: "Am I in remission?" or "Is this symptom I'm experiencing the start of Cushing's coming back?"  I mean, lets face it: this disease is a mess from start to finish.  Nothing about it is easy.  Nothing is straight forward.  Nothing is black and white and nothing is clear and obvious.  Heck, I sat in a conference room full of Cushing's Patients a year ago and listened to a top endocrinologist in the Cushing's world tell us that one of his doctor-friends had recently said to him "If I had to deal with Cushing's all day every day, I'd kill myself."  Now, this was said somewhat jokingly and we all laughed, so don't take that the wrong way.  He was discussing the ins- and outs- of testing and how complex it is, with high recurrence rates to boot, and that it is complicated for the doctors and confuses them too.

I am pretty sure I'm in remission.  I see more and more signs of that for sure and very few signs of Cushing's now, even though I struggle every minute with the damage it has done.  My life is still a mess.  My health is still a mess.  My symptoms and labs STILL confuse my doctors constantly.  I'm still confused daily by what my body is doing.  No two days are alike, and yet the symptoms caused by various hormone imbalances are...and trying to tease out which hormone is causing which symptom, and whether it is high or low, is just a blast.  A blast, I tell ya.  I still have to watch for whether Cushing's will come back and I am still walking my "tight rope" but it does look like I am in recovery from Cushing's itself.

There is not one diagnostic test nor one symptom by which to judge.  It is not that simple.  Again, it's a weight scale like that of diagnosis.  Even the specialists are informed by the tests, but rely heavily on what *we* report for signs and symptoms, almost more than the testing. So, I'll go through some of what has informed my opinion on the matter for my own remission:

Sleep/Insomnia  I am able to sleep now.  I think I could even nap during the day if I didn't have 6 kids running around.  Before I started my growth hormone replacement, I still had issues with sleep, woke up frequently, didn't feel rested when I woke in the morning, and I had difficulty falling asleep, but it just had a different flavor to it.  I was tired but couldn't sleep instead of wired and couldn't sleep.  It wasn't like I was "bouncing off the walls" with racing thoughts, etc and signs of high cortisol, I simply couldn't sleep well.  Luckily, once we diagnosed my AGHD and replaced that hormone, I started sleeping better from day 1.  Now I have no insomnia or sleep issues.  I can fall asleep at a reasonable time (that adjusted gradually), and I wake up at a reasonable time...kind of.  lol  I'm still tired a lot, but that's recovery.

Appetite/Craving  I can skip multiple meals a day.  I shouldn't, because it messes with my blood sugar which messes with my liver, growth hormone/IGF-1, and can cause some adrenal insufficiency if I'm on the edge, but I have to actively remind myself to eat most days.  I also can't eat even half as much as I used to.  I didn't think I ate too much quantity before, but now some days I have difficulty finishing a small home-made burger (and I do mean small) or I can only eat half of a sandwich.  I'm full.  It is like my stomach has shrunk, except it didn't have a chance to do that...it's literally that my cortisol is lower so my appetite is lower.  It's that direct of a connection.  I also crave carbs and sweets a lot less.  I wasn't a huge carb eater before because my body always seemed to have a greater need for protein and vegetables, but I did crave them sometimes.  Now I can't even eat potatoes...in any form.  I have a hard time eating bread, even freshly homemade bread straight from the oven.  That too was a gradual change, but it's a pretty obvious one.  I can skip the icecream even when others around me are eating it, and I don't feel like I'm missing out.  It just doesn't interest me.  I'm still a chocolate fiend, that hasn't changed.  But you know what I crave these days?  The only thing?  Melted, salty, fatty cheese.  I mean, I liked it before, and loved Pizza, etc...but this is constant.  Weird, eh?  I know my body needs the salt, but perhaps it's my body's way of saying I need the fat too.  Time will tell.

Acne  I still have acne issues.  It's still obnoxious.  I have scars everywhere and I still get big, cystic acne, but I think it is slowly, very slowly fading.  Maybe that just takes time (it's been about 6 months since I switched from symptoms of failed surgery to symptoms of remission), or maybe the growth hormone replacement is helping with that too (when I became hypopit at 6 months post-op, I lost 100% of my GH production).  I can say that the cystic acne doesn't show up at specific times in my cycles...so maybe it is just working it's way out of my system.  The acne I get that is related to my cycle is MUCH more normal.  I've not experienced it in so long it is weird, but I simply get more blackheads or little whiteheads around PMS time and they disappear quickly without becoming massive and cystic.  It's crazy.  Hopefully the trend of the cystic acne dissipating will continue.

Weight Gain/Loss  I've mentioned before that in my high cortisol cycles I could gain around .5-2 pounds in a day, and in a low cycle I could lose .25-1 pound in a day (something like that?) without any changes to my diet or activity, it was pure hormones.  So, I would go up and down over the course of a month, but I gained faster than I lost and it was an obviously upward trend.  Now I still have some ups and downs in the weight department...like when I increase my GH dose and that causes swelling, or when we went on our trip to Seattle/OR a few weeks ago and ate lots of yummy food, or my fondu-birthday gift we indulged in for a few days, or when my period starts...but I'm on a definite losing trend.  I'm down 19+ pounds from my highest weight, again without any changes to my diet or activity level.  I'm still pretty much chair-bound (one step up from bedridden), and my appetite has changed as stated above, but I still eat the same basic foods and still indulge in my chocolate cravings.  I'm at the point where I desperately wish I could physically cook and exercise (or simply be active) like I once did.  I bet the muscle would pile on and the weight would fall off.  I think I've gained just enough energy in my 3 months on GH to *want* to be active and do things even though I still can't, whereas just 2-3 months ago I was so physically exhausted I didn't even consider it an option, so it is actually a bit more irritating and depressing right now!  lol  Talk about an odd turn of events.

Tests  My post-op cortisol testing was too high, but wasn't horribly convincing.  The neurosurgeon had obviously removed the bulk of the problem, but there was still some residual something going on.  I went on cortisol lowering medication, which I couldn't seem to get stable on (despite having taken it before surgery and doing relatively well on it).  All in one month, we found out that medication was causing me liver damage (it's a known problem, thus the reason I was being screened...but this was the least problematic of the possible medication options), an MRI showed that I had developed empty sella (my pituitary was being smushed by CSF coming from the space surrounding my brain above --this can cause a loss of pituitary hormone production), and my IGF-1 (a marker for growth hormone deficiency/excess) had suddenly dropped by about half, to below range.  I left that appointment with a lot of lab work to do.  I was still testing for Cushing's Disease re-diagnosis, and also adding in more pituitary hormone labs to see if and how the empty sella was affecting my hormone levels.  As it turned out, from a GH stim a few months later, I had severe adult growth hormone deficiency (my body failed to stim at all), and we then began watching the other pituitary hormones to see if they would follow suit.  The jury is still out on that one.  I have "low-normal" results on various hormones, with accompanying symptoms, so only more time and tests will tell.  But, I dutifully did a few more of my Cushing's tests, but then stopped about half way through those (again, it was a good 8-12 total serial tests, so it takes a while).  I just didn't feel "high" when I should have, so stopped testing.  My endocrinologist didn't require me to keep testing to confirm it was gone, he trusted that I knew what I was talking about.  (I *may* need to retest when the AGHD is fully treated, but I doubt it at this point.)

Vitamin D and Ferritin  These were surprising and unexpected.  Some of it may be due to the liver damage (that can affect these results), but after over 3 years of high-dose replacement with 50K iu's of D3/week and 6 ironsorbs/day not getting me into the "normal range", those results suddenly were fine.  I backed off on my D3 and iron per my endocrinologist's request, and the D did fall somewhat, so I'll stay on that (I mean, I DO live in a rainforest in Alaska, and sit indoors in my chair all day!), but...now the replacements work!  My iron has been stable since, despite my ferritin having been at a 4.8 when I started - ie, I needed infusions...and badly.  I think this is another sign that the excess cortisol has left my body.  I may not be suddenly "cured" from these deficits, but the replacements for them actually do what they're supposed to.

Buffalo Hump  This one I didn't notice until my husband pointed it out.  He was rubbing my neck one night, about 5 months ago, and said that my hump was suddenly soft and squishy instead of hard and solid.  My hump had always been a smaller one, but it was there.  Now, a few months later, I just have regular fat skin there.  It may always have a slight hump-like appearance, or it may fade as I lose weight, I don't know...but I'm happy.

High/Low Cycling  This is the biggy.  I still feel like total crap during my period...I still have lower cortisol then, but I don't get highs any more.  There were two days total at some point about 2 months ago where I was able to do more, but it still didn't feel "high", I just felt kinda human again.  And it never came back.  Instead I feel pretty low most of the time, to the point that we are watching me for adrenal insufficiency.  I stray into the "may need a cortisol stim test" range some times, and will continue testing that every so often.  I don't seem to have any adrenal reserve at this point, and when I do much of anything (and I mean anything), I start developing symptoms of adrenal insufficiency.  When I started my GH replacement shots, I had a nasty reaction that my endocrinologist feels was due to the GH catabolizing cortisol, making my cortisol levels drop too low.  Luckily I didn't go into crisis, but it did mean it took me 3 months to get onto the dose my endo wanted me to start at with GH.  We don't know how much I'll need in the end, but I have to be very careful increasing my dosage now because it will lower my cortisol further each time.  I have the feeling I will be panhypopit eventually (lose more pituitary hormone production), thanks to the empty sella, but you never know...so I'm trying to be very careful and not take hc unless I absolutely need it (when I go AI) in case there is a chance my pituitary and adrenals will recover.  I appear to have no adrenal reserve (just enough to get by if I don't do anything, but not enough if I exert myself, am ill, go through a period of stress, etc) and have been trying not to surpass my limits which really is a bit of a crap shoot, to be honest.  (Again, the tight rope analogy from earlier this month.)  But, back to the cycling.  I'm not cycling.  I'm not high.  This is THE most clear reason I don't think I'm dealing with active cushing's any longer.

All that said, being in remission from Cushing's is obviously not the end.  Do I sound like I'm healthy?  I still can't be active.  I still feel ill in one way or another most days.  I've traded Cushing's for hypopituitarism.  And, frankly, I'm good with that.  It is still obnoxious and complicated, but it is far more treatable (if/when my hormones stabilize and we are able to figure out precisely which I need to replace and how much).

Who would have thought that some measly CS fluid would have killed off what was left of my Cushing's?  It's a major blessing; a totally unexpected and unusual one.  The rest of the damage that has caused (or is still causing) will still be a lifelong struggle, but hopefully I'll get some stability and some semblance of a life back, becoming active and relatively healthy again.  I will always have to keep an eye out for the signs and symptoms of Cushing's to return, and many live in fear of that with each symptom that pops up.  I will worry about that when I have reason to worry, but right now I am enjoying that I no longer have to fight that battle. 

I've not experienced a return to what and where I was before, that rarely happens, what I am experiencing is a new body, with different needs, limitations, etc.  It is a new day and I will be a new me. 
Now my job is to learn about this new body, to help it recover and be healthy in it's unique challenges, and to make the best of it.  It'll take some time.  It's intimidating, daunting, and tiring, but I think I've moved through the grieving process to an acceptance that that previous life and previous me is gone.  I've buried her and mourned her loss.  Like any other loss, grief can return unexpectedly, but it is generally momentary.  I know she's gone and won't be coming back, and I can be grateful for what she taught me and enjoy the memories.  What I have now is discovery.  Who is this Cushing's-Free Catherine?  What are her limitations?  Her strengths?  Her abilities?  What brings her joy?  I look forward to meeting this new Catherine, and learning all about her.  It makes me nervous because it's all new, but it is also exciting.

Wednesday, April 29, 2015

Testing for Cushing's, MN Saliva

Oh my goodness!  I just realized I forgot salivary cortisol levels in my previous posts about cortisol testing for Cushing's Disease!!! 

Midnight Salivary cortisol levels are checked at...you guessed it...Midnight, and use a sample of (You guessed this one too, didn't you?) saliva.  Yep, spit.  This is another "Gold Standard" test used today by endocrinologists to diagnose Cushing's.  It's a simple test you can do at home, freeze, and take with you to the lab when it's convenient to you.  It is especially helpful for cyclic patients.  You can wait till midnight (really, it is often done at 11pm) and if you don't feel high, you don't have to test and can save it for another night.

Because cortisol should be at its lowest at night, at nearly zero, and because Cushing's frequently causes high cortisol at night (flipped diurnal rhythm), it is a great time to test for abnormal highs  Anything above 0.09 on a midnight salivary cortisol is diagnostic for Cushing's, assuming you didn't accidentally contaminate the sample (via bleeding gums, cosmetics, etc).  Generally contaminated samples are sky high, though, or are marked by the lab as contaminated. 

The collection containers may be little plastic tubes you spit into, but more often now they are little cotton swabs that fit inside those tubes.  (Think of a mini-tampon, and you've got the right idea.)  You don't eat or drink (or brush your teeth) for X amount of minutes before the test, and then you put it in your mouth and let it absorb as much saliva as you can.  Then you place it in the tube, seal it, label it, place it in it's bag, and then in the freezer till the next day you go to the lab.  I don't know how long these hold, so I wouldn't wait forever, but you can definitely go a few days.  LabCorp seems to be using a newer collector model that to me looks like a mini lol.ipop with a plastic handle.  Whatever the method, it's all quite similar. 

*Note that there are a number of saliva testing labs online that do it via the mail...these results are generally not accepted by doctors, better to get a doctor to order the test and do it through a standardized lab if you want to use it for diagnostic purposes*

Need-to-Know Cushing's Basics

Here are some posts that I made last year that I think would be helpful to review for basic info (and some in-depth info), if you don't already know your way around a Cushing's Diagnosis:

Symptoms of Cushing's 

Types of Cushing's Syndrome

Cushing's Tumors

Distinguishing Cortisol Highs and Lows

Treatment for Cushing's

And there is a series of posts I made this month that goes through commonly-used tests starting with: "Testing for Cushing's, Introduction"

And, if you want to get a feel for what the struggle is like?  Here's a monologue I wrote that was preformed readers-theater style locally: A Big, Fat Target

Tuesday, April 28, 2015

Testing for Cushing's, Finale

DIAGNOSIS!  Or so we hope.  I know it sounds weird to hope for a diagnosis, tumor, and surgery (often neuro-surgery!), but that's reality.  When you feel horribly sick, you're losing your mind, your body is morphing right before your eyes into something foreign and gross, and it feels like there is nothing you can do about it?  Trust me, you are GLAD you find a possible reason for it, some proactive way to fight back (test, see doctors, learn) and a chance to get rid of the source of the problem and recover from it.

But, this isn't a simple diagnosis by ANY stretch of the imagination.  Many illnesses have fairly cut-and-dry diagnostic testing.  The test either says you have it or you don't, bam.  But that isn't the case with this disease.

Think of it like a scale: You have to accumulate evidence proving you have Cushing's, and doctors pretty much have the scales tipping the other direction from the moment they see you.  It's sad, but true.  "It's rare."  You might just be depressed, lazy, and eat too much and poor quality foods, etc.  The scales are generally tipped to assume that is the case, and you have to provide overwhelming evidence to the contrary.  And yes, it is often the patient that has to meet the burden of proof.  It's left in our hands, that is why it is SO important to become a well-educated, informed, proactive patient.


Like I said, you basically have to accumulate enough evidence to tip the scale far enough in the direction of a diagnosis to prove their preconceived opinion wrong.  What that takes is so completely different from doctor to doctor that it is unreal.   
UN.  REAL.
Remember that there is a HUGE list of signs and symptoms, and how each patient presents with a different cluster from among that list?  Remember that there is no one definitive diagnostic test that alone can prove and disprove Cushing's in all patients?  It is apparently the norm for doctors to play favorites with testing, giving more weight to certain tests and significantly less to others (or they simply don't even order the other types of tests).  Some have favorites among signs and symptoms (signs being things that can be viewed and measured like blood pressure and stretch marks, and symptoms being what you report feeling or noticing like fatigue and weakness).  If your individual presentation doesn't match up with their preconceived ideas of how a Cushing's Patient should look, you are SOL. 

You *might* have a chance to persuade them to let you keep testing and prove them wrong, but that's not often the case and most of us are too sick to fight that losing battle.  You usually have to start over with another doctor, which is often a reason why many of us end up having to travel to see a specialist who deals with Cushing's all the time.  I've heard of yet worse things happening, like doctors calling patients crazy, putting such things in their medical files, and even going out of their way to contact other health care providers of their patient to let them know.  I had a friend whose "well-respected" doctor did just that, contacting her employer's insurance company telling them she had nothing physically wrong with her and maybe was just depressed.  She was a smart cookie, did her research, and found a doctor willing to treat a person and not a text book.  She received the denial notification for her disability literally while out-of-state for her brain surgery.  (Which, she later won on appeal, obviously.)

There is yet another obstacle in our way related to doctors' opinion/training...that of the lab tests themselves.  We've discussed briefly in the previous posts about individual tests for Cushing's and that they have some inherent flaws (and episodic or cyclical illness especially), but we haven't yet discussed how doctors apply their reference ranges.  I'm not sure how they are calculated (there's got to be a bell curve in there somewhere?) so we'll just stick to how these ranges may apply...

But first lets talk about thyroid hormone testing as an example.  Most people are at least a little more familiar with the blood draws for TSH (pituitary hormone that tells the thyroid to produce thyroid hormone), Free T3 and Free T4.  Much of the medical world has come to realize that having thyroid hormone levels within the normal range doesn't mean everything is hunky-dory.  You could have a TSH at the top of the range and Free T's at the bottom of theirs, and need thyroid replacement.  You could be like me and have a doctor say "Your TSH is in-range so you're good!" even when my ACTUAL thyroid hormones were below the range.  More and more doctors are starting to understand how these play together, and that the range itself is really is too wide.  It's just a ballpark idea and not a set and fast rule.

So, Cushing's testing is kind of like that, except it's kind of the opposite.  Being outside the range, in our case, isn't considered proof you are sick.  "It's normal!" is often told to patients whose levels are DOUBLE the range.  In my opinion this pretty much defies the purpose of a "normal range" to begin with (in both cases, cortisol and thyroid testing), suggests we are using FAR outdated and inaccurate diagnostic tools, and certainly at the very least suggests a need for re-evaluation and recalculation so that they actually HAVE some meaning that is useful...but I'll get to my point.

Many doctors have been taught that cortisol levels 2-3 times the normal range is "pathologic."  That means that with tests results that high, you are pretty much guaranteed to have a disease causing the overproduction (as opposed to anorexia, alcoholism, or depression, stress, etc).  Just to be clear, what that means for us lay people is that if the cutoff on the top end of the normal range is 50 (so everything over that is flagged as high by the lab), levels around 100-150 or higher are proof of disease.  

*There is a chance it is caused by excess steroids such as prednisone pills, inhalers, creams, etc, but this can be rather easily ruled out*

Having levels between 50-150 doesn't mean you don't have high cortisol, but means that your doctor needs to rule out other causes as the source of those high tests, often through repeat testing and most doctors can rule much of it out at your very first appointment by taking a standard, detailed, patient history.  So for most cases, having a test above the range should just mean doing a few more tests than someone whose tests are at or above that 2-3X the norm level.

Well, here's the difficult part of this one: More and more I am hearing of doctors, and endocrinologists specifically, who are interpreting that 2-3X normal cutoff as the line for diagnosis itself.  This means they are saying if your high results aren't at least THAT high, then you are sent home with no diagnosis or treatment.  High cortisol, even when "mildly" elevated, can do very severe damage over time.  

It's absurd and flat-out-lazy, in my insufficiently-humble opinion, that doctors drop the ball.  SO much of these downfalls could be avoided if they just tried.  I don't know what else to say about it!  These doctors are costing patients health, years, and possibly their lives and it makes me both angry and so, so sad. 

If you find yourself in any of these precarious situations with a doctor, and it doesn't look like your concerns are going to be resolved, please ask other patients (with similar testing, symptoms, etc would be best) which doctors helped them and get your butt there.  It may be completely obvious to you that you are sick.  It may be that other cushing's patients can see from your results that you are too.  And it may be that other doctors would see "disease" if they saw that same data.  


Become an informed patient, keep copies of all your results, imaging, etc yourself, ask questions, get yourself to the right doctor(s), and truly be a responsible partner in your care.   
This is your life.  

 *I did have to prove my disease like everyone else. It was a very long process.  I did have experiences similar to others' of being dismissed and blamed for decades beforehand, but mostly by pediatricians, general practitioners, and doctors of internal medicine (well, and friends and family).  I was lucky that by the time I was sick enough to start taking sole charge of my situation, regardless of those experiences, I had a patient support group to help me get to an endocrinologist who believed me when I explained my signs and symptoms, my efforts to diet and exercise, etc. and was compassionate and caring from the beginning.  It could have been much, much worse.  This battle has been hard enough as it is.  I am so very grateful to those family, friends, and health care providers who have made and continue to make this process easier, who believe(d) me, and who treated me with kindness, respect, caring, compassion and support.  It is noticed, it is appreciated, it is and always will be remembered.*

Monday, April 27, 2015

Testing for Cushing's, Imaging

Imaging such as MRI's, CT's, Octreotide Scans, and Gadolinium Scans (newer) will and may be used in the search for the source of your Cushing's Syndrome, though don't worry, it's not usually all of these, nor all at once!

Because a pituitary source is the most likely location of an ACTH-producing tumor by far, the pituitary is usually the first place they look unless laboratory testing strongly suggests an adrenal or ectopic source.  (And, I'll probably give the most info here since this is where the bulk of my experience has been)

The imaging of choice for pituitary tumors is a 3T dynamic pituitary MRI. 

3T denotes the strength of the magnet used (3 Tesla's).  There are still a lot of 1.5 T machines in use, but they do NOT give as clear an image as a 3T machine for pituitary tumors, which is why 3T is now the standard when looking for pituitary microadenomas (more common than macro's, which are larger and could be more easily seen on a lower-strength machine).  Microadenomas are under 1 cm in size, and are often as small as 3mm (or even less!).  Look at a pencil eraser, then cut it in half...now picture this chunk inside a bean and sitting at the base of your skull, almost in the center of your head.  Think it'd be hard to see?  Yep.  Many, many radiologists and doctor's miss them, which is why it is important to get copies of your imaging and not just the report, AND to be sure a Cushing's-experienced radiologist, endocrinologist and/or neurosurgeon looks at the actual images.  (Ask me how I learned this one the hard way?  Yep.  Wasted a year of my life sick with no known course of action because I didn't know this...okay, I also didn't know when to test and testing at the wrong time.  So it was a double whammy, but either of those having been different would have suggested I needed to keep testing.) 

The word "dynamic" refers to how the dye is injected.  They first run a number of scans without contrast dye.  Then they either have two tech's work together in unison, one to inject the dye as the other starts the machine with an audible count-down to be sure they inject and start at the same time OR the tech will hook you up to an IV-infusion type machine that he can set to inject the medication automatically as the tech starts the MRI.  It is VERY time-specific.  The reason it must be done this way is that these tiny tumors generally look like normal tissue on a pre-contrasted MRI.  And, they uptake dye like normal tissue and thus will often not show up on a post-contrast MRI.  So why do the MRI?  Because they absorb the dye at a slightly slower rate.  A healthy pituitary should absorb the dye in a uniform way.  These ACTH-producing microadenoma's will tend to look like a dark spot when that happens, but as you keep looking through the timed scans, eventually it will lighten up like hte rest of the pituitary.  Dynamic is important.  You want this done right.  Most surgeons won't go looking for a tumor no one can see.  It isn't exactly low-risk.  But staying sick isn't low-risk either.  You want that proof so you can get well.

Sometimes the paperwork will say with/without contrast, but even so, it has to be done a VERY specific way as explained above.  You will know they aren't doing a dynamic if it is just one person administering the MRI, and they walk in with an injection needle and then expect to walk out and start the scan afterwards on their own.  I had somehow been scheduled wrong once, and the tech came in in just this manner.  I KNEW it wasn't right, so refused to let him inject me (once it is injected, there is no way of doing a dynamic for some time, and I was on a trip with no way of coming back later).  I knew better and it wasn't going to happen!  I told him no, it was supposed to be a 3T dynamic pit MRI.  He argued with me, I didn't relent, and finally he listened when I told him to call the neurosurgeon's office to ask them (it was a post-surgery MRI ordered by the neurosurgeon this time, and I was at one of their hospital's imaging facilities).  Sure enough, I was right.  They had me on the wrong machine, even!  A 1.5 T.  Something had gotten lost in the shuffle of scheduling.  The tech's attitude then switched from irritation and frustration with me, to being impressed I knew it wasn't the right protocol.  He asked if I was a tech myself.  lol  It was a mess of a day as they tried to get me fit into the right type of machine (which were fully scheduled for the day already), but they were kind and it worked out.  I got my 3T MRI.  When you're making decisions about whether to have repeat brain surgery, the right testing (and imaging) is obviously important; it was worth a day of work.

"Pituitary"
should be obvious, right?  I mean, that's what we're trying to see!  But no, sometimes doctors will order a brain MRI.  Yes, both take pictures of your brain AND your pituitary, but the pituitary is tiny and the brain is big, and a brain MRI's "slices" (pictures) are spread farther apart.  We need the focus on the pituitary, and we need those super-fine, close-together slices.  Brain MRI's aren't likely to catch microadenoma's, and as many of us know from experience, it's hard enough to find them with the right protocol.


The next most common cause of endogenous Cushing's Syndrome (meaning outside the pituitary), is an adrenal source...meaning a tumor on an adrenal gland.  Laboratory testing can suggest this is likely, because often ACTH is low or near zero with these patients.  Adrenal glands can be hard to visualize adequately, as they're fairly small and sit like little pyramid hats atop each kidney.  Sometimes an MRI is used to image them, but I believe more often, an abdominal CT is the imaging of choice. 


Suspected Ectopic Cushing's Syndrome (meaning a source not in the pituitary or adrenals) usually produces very HIGH amounts of ACTH, so lab testing may suggest this is the type of source to look for.  It also often requires an MRI or CT, but these may not be sufficient to identify a tumor.  In this instance, as well as sometimes after a failed Bilateral adrenalectomy, more specialized, whole-body testing is required.  In many ways it is the same sort of scan, but they use a contrast dye that can help pinpoint a tumor specifically (ie, the dye will be attracted to that type of tissue, I believe tissues with somatostatin receptors) and will "light up" on the scan.  The most common type is an Octreotide scan, but there is a new type of scan, that may be more sensitive, that is starting to be used for testing also called a Gadolinium scan.  Neither of these types of tests are widely available, again it is specialized testing and generally patients have to travel to find a facility with the ability to preform them.

Sunday, April 26, 2015

Testing for Cushing's, IPSS

Inferior Petrosal Sinus Sampling (IPSS) is another interesting test, sometimes ordered by endocrinologists, sometimes ordered by neurosurgeon's.  It is preformed by experienced interventional radiologists, generally at a large hospital. 

If you've ever heard of a cardiac catheter, where they feed a tube or wire up from your femoral artery into your heart...that's similar to what this test is.  Except they thread two up towards either side of your pituitary.  "Inferior Petrosal Sinus" is the location of the sampling area.  When everything is in place, IV's, catheters, etc...they inject you with medication that is meant to force an ACTH producing tumor to produce high levels of ACTH.  At timed intervals, they then draw blood samples from your left and right sides at various points (including just below the pituitary).  All this data is put into a table, which then can show whether your levels were highest from near the pituitary (which would point to a pituitary tumor) or another area (which would point to an ectopic tumor somewhere else in the body).  It *can* also help identify which side of the body or pituitary it is coming from, but at least with pituitary sources, it is only about as accurate as a coin toss in pinpointing sides.   

The test can be useful, but really, you want a surgeon that will explore your entire gland to look for tumor tissue anyway, not only rely on a test with poor statistical accuracy.  When they explore the gland they make little slices through the entire surface of the pituitary, which generally heals back together well, looking for pockets of tumor tissue (which we jokingly call "fileting").  Many times tumors of florid Cushing's patients are nicely encapsulated circles, but often, cyclic or eptopic patients' tumors are gooey (getting destroyed by suction with no tumor tissue for pathology testing), or oddly shaped (think of dumbbells, octopus tentacles, a thin flat tumor spreading the entire bottom of the gland, multiple tumors, etc).  If doctor's aren't experienced in these kinds of tumors and are only looking for a perfect tumor and only looking in one spot, they may miss the tumor entirely, spread gooey tissue around (YIKES!), or miss parts of the tumor...leading to a failed surgery or a brief remission followed by a recurrence of Cushing's Disease, which is already hard to cure.  A recurrence may be unavoidable, but you certainly want the best chance possible. 

I also strongly encourage you not to settle for the nearest neurosurgeon --what happens in surgery can make all the difference in your quality of life for years to come.  Study it out, ask informed questions, talk to their past patients, and make sure you are comfortable with their experience (of removing Cushing's-specific tumors) and their methodology before letting them into your head.  It is your head.  You have to live with the results.