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'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 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 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 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!), 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 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 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.   
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 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.

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.

Saturday, April 25, 2015

Testing for Cushing's, Dex

There are actually two types of Dexamethasone Suppression Tests (Dex) used in diagnosing Cushing's, a high dose and a low dose test. There are some differences in procedure, timing, guessed it...dosage.  I'm not going to go in-depth on the procedures of either, but more give a brief overview of what the test is, what they're looking for, and it's downfalls.

These tests are kind of the opposite of a "stim" test in that it isn't meant to stimulate production, but to suppress it.  The point of these tests is to give your body sufficient steroids (Dexamethasone) so that your pituitary's own feedback will stop it's production of ACTH.  In a healthy HPA axis, your body recognizes there is already enough in your system, and thus doesn't produce more.  They give the medication a set number of hours for your pituitary to respond, and then check your cortisol levels to see how much your cortisol has suppressed (or dropped).

Many forms of steroid replacement medication will show up as "cortisol" on lab tests, but in the testing used here, Dex does not.  This makes it a good medication to use for a suppression test, as any cortisol above the expected level (meaning a failure to suppress or failure to suppress enough) can be said to be caused by a process working outside of the normal feedback loop (ie, a tumor).

This is a well-used type of test, and sadly, it is often used to rule out Cushing's when that really isn't what it is good for.  These tests have a statistically high number of false negatives (I've seen published numbers as high as 30%), meaning there is a large chunk of patients who DO suppress on this test who still do go on to be proven to have Cushing's.  And if you throw in the ectopic and cyclic patient groups rarely included in such studies, things really can get muddy.  In my opinion as an educated patient, these tests are great for ruling IN Cushing's, but not for ruling it out.  What I mean by that is that it misses a LARGE number of very ill patients, and some doctors then send them away still suffering, because of these test results (even when other testing comes back routinely diagnostic) so it is not a good test to use to rule out Cushing's as a problem.  But it is good for proof of disease (ruling it in), because if you do not suppress fully on this test, it's pretty obvious there's a problem that needs further investigation and treatment.  (Sadly, patients still get sent on their not-so-merry way...It's often the luck of the draw with doctors, even "specialists" as I explained in the "Testing introduction" post before.)

Something else Dex Suppression tests are good for:  Helping to point towards source.  Adrenal sources do not often suppress, and pituitary ones can.  I honestly don't remember what ectopic sources do!  I would assume they do not suppress either, or at least not much.  But this test alone isn't a sufficient indicator anyway, and more tests and imaging are required to confirm source.  So, really, it's kind of a shot in the dark whether this test is even useful.

*Update:  Another Cushie asked if I would address the Dex-CRH test, and since I don't know too much, I'll just add it here.  I attended the 2014 Magic Convention and heard Dr. Ludlam (Endocrinologist that used to run "Camp Cushie" at Swedish in Seattle but that now works as the endocrinology director at Novartis Pharmaceuticals) speak, and he did discuss the Dex-CRH.  Keep in mind this is year-old memory I'm speaking from, so it's accuracy is suspect.  My impression is that he found it far more accurate than the Dex alone and that he was of the opinion that by using the Dex to suppress normal pituitary function, he could then use CRH (Corticotropic Releasing Hormone) to force an ACTH-producing tumor to produce, and that this resulted in less (or no?) false negatives and far more accurate testing, especially in the episodic and cyclic patient population (he preferred the term "variable").  I have no data to back this up, and it appears I didn't include this in my post of notes last April, so take it for what it is worth.  I've not heard of it being used since (which doesn't mean no one does it, but just that it isn't likely routinely or commonly used) and have not had the test myself.

Here's some info from Dr. Friedman's website:
"Dexamethasone-CRH Test
Another recommended test to distinguish between mild Cushing's syndrome and pseudo-
Cushing's states in those patients with a mildly elevated UFC is the dexamethasone-CRH test. This
test combines two tests, the low-dose dexamethasone suppression test (LDDST) and the CRH test
which individually are good but not great at distinguishing between pseudo-Cushing's states and
Cushing's syndrome. As discussed below, the dexamethasone test takes advantage of the fact that in
patients with Cushing's syndrome, dexamethasone ineffectively suppresses the production of pituitary
ACTH. CRH stimulates the pituitary to secrete ACTH which leads to an increase in cortisol levels.
Patients with Cushing's syndrome have a larger increase in plasma ACTH and cortisol levels than in
normal individuals or those patients with pseudo-Cushing's states. Although these tests individually
are helpful to diagnose Cushing's syndrome, many patients with pseudo-Cushing's states also respond
to them in a similar manner as those with Cushing's syndrome, making them not the ideal test to use
individually. Yanovski et al., (9) elected to combine the two tests and gave 39 patients with Cushing’s
syndrome and 19 patients with pseudo-Cushing’s states dexamethasone (0.5 mg) 4 times a day for 2
days starting at 12 noon (last dose at 6 A.M.). At 8 A.M. on the day of the last dose, the patients
received intravenous ovine CRH (1 μg/kg) and cortisol and ACTH were measured at various times.
All patients with Cushing’s syndrome had mild hypercortisolemia (UFC between 250 and 1000
nmol/d; 90-362 μg/d) so that UFCs between patients with Cushing’s syndrome and pseudo-Cushing’s
states completely overlapped. A plasma cortisol greater than 1.4 μg/dl (38 nmol/L) measured 15
minutes after the CRH injection correctly identified all patients with Cushing’s syndrome, while a
value less than 1.4 μg/d identified all patients with pseudo-Cushing’s states (100% sensitivity and
specificity). In contrast, the low dose dexamethasone test, had a 74% specificity and 69% sensitivity
when 17-OHS was measured on the second day of dexamethasone administration and 100% sensitivity and 56% sensitivity when UFCs were measured. The CRH stimulation test without dexamethasone pretreatment had 100% specificity and 64% sensitivity. This study has the advantage of comparing the dexamethasone-CRH test to other popular tests (LDDST and CRH test) in the same group of patients with mild Cushing’s syndrome and pseudo-Cushing’s states and clearly showed the superiority of the dexamethasone-CRH test in this group of patients. The main drawbacks to this test is that it requires a lot of steps and the drug (CRH), while no longer investigational, is expensive. A subsequent paper (70), found, as expected, that the dexamethasone-CRH test completely distinguished patients with Cushing’s syndrome from normal volunteers." 

Full PDF of article found here.

Friday, April 24, 2015

Testing for Cushing's, CBG

Serum Cortisol Binding Globulin (CBG) is another simple blood draw.  This one you don't do often, but just so your endocrinologist can get a feel for whether your CBG is high, low or normal. 

This test measures the amount of cortisol in your blood that is in bound form.  My understanding is that free cortisol is the active form, so it is the most important for determining hypercortisolism.  Since a fair amount of the testing used gives results in "total cortisol" it is useful to rule out other causes for test elevation. 

If your CBG is high, it can make your total cortisol results also come back high, even though it may not actually be problematic.  One specific instance in which high CBG is known to be elevated and confuse testing is with oral birth control pills.  They raise your CBG level and can skew (and invalidate) any test results while on them.  For this reason it is suggested you be off oral birth control (estrogens specifically, I believe) for a minimum of 6 weeks prior to cortisol testing.  And always inform your endocrinologist of all medications and supplements you are taking, including other forms of hormonal birth control, etc. 

Thursday, April 23, 2015

Testing for Cushing's, ACTH

Serum Adrenal Corticotropin Releasing Hormone (ACTH)  is a hormone produced by your pituitary.  This hormone signals the adrenals to produce more cortisol.  It is a simple blood draw, done at specific times (8am or midnight) and is only complicated by its need to be drawn into a chilled tube and kept frozen.  It has a super-short half life (somewhere around 14-17 minutes, depending on the source), meaning the levels present in a blood sample drop quickly at room temperature.  I *always* remind the lab aid about this tests' requirements if they haven't already obviously prepared a chilled tube, etc.  Depending on the lab, this test can take a bit longer than the cortisol, but it too has a fairly quick turn around.  A serum ACTH level can be a very helpful test for diagnosis as it helps point to the source of the excess cortisol state endogenous hypercortisolism (ie, the problem stems from within the body).

In the case of Cushing's Disease, there is a tumor on the pituitary that produces it's own ACTH, effectively bypassing the feedback loop that would otherwise keep your levels just where you need them in a healthy body.  This in turn causes your adrenals to produce cortisol your body doesn't actually need, and it sets off a huge chain of events that can cause a lot of damage to your body.  In this situation it also suppresses your own pituitary's production of ACTH (there is already enough in the blood supply, so your pituitary does not call for more) with it basically going to sleep as it gets out of the habit.  In cyclic cases, it is thought that the tumor turns off production (this is poorly understood and there is no explanation as to why or how, to my knowledge), and this accounts for a cyclic person swinging into a "low cycle" or normal cycle.  For this reason, the pituitary of a cyclic patient doesn't always become fully suppressed and post-operative testing may not be as clear.  If the pituitary can wake up and kick in, your body may not crash to a "zero cortisol state" postoperatively as is expected and watched for as a sign for remission in florid cases (though it certainly still does happen).  It also may mean a lower requirement for post-op steroid replacement.  But this is all so individual, dependent upon your pre-operative levels, and how your body copes with the changes.

ACTH within the normal range or somewhat elevated in the presence of hypercortisolism is suggestive of a pituitary tumor source as described above. 

Low or absent ACTH levels are indicative of an adrenal source, meaning there is a tumor on an adrenal glad itself that instead of producing ACTH, produces cortisol more directly.  Your pituitary senses the excess cortisol and thus does not send out ACTH to produce more.  This too can lead to pituitary suppression.

An excessively high ACTH level is suggestive of what is called an ectopic source.  This can be a tumor almost anywhere else in the body, that produces high amounts of ACTH.  There are some specific types of imaging that can help locate these tumors, which I'll discuss briefly in a later post.

The most common endogenous source is pituitary, a small percent is adrenal, and an even smaller percent are ectopic.

Wednesday, April 22, 2015

Testing for Cushing's, MN Serum Cort

Midnight Serum Cortisol is a simple blood test that can be a good diagnostic tool for Cushing's Disease or Syndrome.  You see, cortisol should be at it's lowest point around midnight in a healthy person, so it is a good time to test for excess because the range for "normal" is so small.

It's a simple blood draw like any other, though at least the fist one should include a serum ACTH (will be addressed in a future post) to get a better picture of what is happening with your pituitary and your adrenals.  These results come back quickly, too (BONUS!  No 2 week wait!).

The difficulty in this test is getting the lab to draw them.  I had great luck locally contacting the hospital's lab manager, explaining the test my doctor ordered, and requesting they draw it.  I explained that I would happily come in 30-60 minutes early and stay 30-60 minutes late should they be busy, giving a decent draw window for their one tech on duty at that time of night (It's a small hospital).  I was very lucky and they agreed to do it. 

I did have to enter through the ER entrance, but the security guards and ER nurses recognized me quickly (I did a series of 4 nights in a row) and admitting staff had been told I would be coming in for testing and thus admitted me as outpatient.  The diagnostic cutoff typically is 7.5 for a midnight draw.  Anything above that is considered diagnostic.  For a sleeping midnight draw (if you were spending the night at the hospital in their bed), the diagnostic cutoff is actually only 3. 

Personally, I love this test.  I think the timing is still more convenient (and the results faster and more universally accurate) than the inconvenience and shortcomings of the 24 hour urine tests.

(Note: it is also worthwhile to have had a CBG recently, as that can affect a total serum cortisol draw --more details in a future post).

Tuesday, April 21, 2015

Testing for Cushing's, 10 hr UFC

10 hour Urinary Free Cortisol is basically the same as a 24 hour UFC (in fact, most labs run it as a 24 hour sample), but you collect your urine for 10 hours only, between 10pm and 8am. 

This test is used specifically to look for a flipped diurnal rhythm...if your body produces too much cortisol at night, most often in patients who have mixed test results. This is the time of day your cortisol production should be at it's lowest, so it is a great time of day to test for excess cortisol (as we'll also discuss later with midnight testing).

The result used for this specific test is the cortisol/creatinine ratio.  My endocrinologist uses a cut off of 16 and above (or is it just above?) for diagnosis.  Sometimes the labs don't calculate it all (Some do routinely, some don't) and you have to specifically request the ratio. 

A word to the wise about dealing with labs if they aren't used to this test: They can get confused if there is no "10 hour UFC" lab listed in their computers.  I always tell mine to order it AS a 24 hour UFC when that is the case.  My favorite local lab has no qualms asking me how to order testing correctly, which I MUCH prefer to wasting my time, pee, and money!  (Isn't it nice to be treated like you know what you're doing?!) 

The one key here is that THEY MUST ENTER THE TOTAL VOLUME AND COLLECTION HOURS IN THE COMPUTER!  If not, you can call back later to fix it assuming they run the test and don't just toss the sample, but that's annoying to have to do too.  And sometimes you run into people who act like you're the one who's confused or made the error.  Just ask my friend who takes pictures of her jugs' labels...she's had to offer to send them a few times as proof!

Monday, April 20, 2015

Testing for Cushing's, 17 ohc/17 ohs

24 hour 17-hydroxycorticosteroids (17 ohc/17ohs) is a test that used to be the gold standard back before my time, was all-but thrown out when 24 hr UFC's came along, and is now making a bit of a come-back.

A 17ohc is also a 24 hour urine collection and is collected in exactly the same manner a 24 hour UFC. long as the lab processes it correctly (and neither require preservative at your particular lab), you can also run BOTH a UFC and 17ohc off of one 24 hour urine collection sample (IE, the same 24hr period, the same jug).  This makes it a super convenient test to add into the mix.

My understanding is that 17 ohc's are a product of the production/breakdown of cortisol within the body.  So, instead of measuring the amount of cortisol your body is passing unused through your kidneys, it is measuring what is more akin to the amount of cortisol your body produced (what is passed into your urine and what was metabolized elsewhere in your body). 

Despite having been practically replaced by the 24 hr UFC to the point that labs stopped offering the test and more-recently-trained doctors didn't learn much about it, they are now finding that it is a useful test in helping to confirm cyclic or episodic disease when other test results are mixed (in a situation where some testing comes back high and others come back low/normal).

The results used in this test are the mg/day (as opposed to mg/creat).

Sunday, April 19, 2015

Testing for Cushing's, 24 hour UFCs

24 hour urinary free cortisol (UFC) is considered a gold standard by most doctors. 

Basically, you are given a urine collection jug, void at a specific time in the morning (8am is the standard given, but whatever time you first wake in the morning and go to the bathroom works, you just have to write down the time and make sure you collect the same time the following morning).  This first urine of the day you flush down the toilet, but that time is the official start of your test.  You then carefully collect every drop of urine the rest of that 24 hour period, usually with a "hat" you get from the lab, pour it into the collection jug, and unless it has an acid preservative tablet (most labs don't use those for this test these days) you have to keep the jug refrigerated during and after the test.  When you get up the following morning (at the end of your 24hr testing period) and pee in your hat for the last time at the same time you peed the following morning, you add this sample to your jug and the collection sample is now complete. 

You seal the lid, measure the volume on the side, write that volume on the jug along with whatever other information it requires --generally at least you name, often collection start and stop times and dates, maybe your ordering doctor's name, the test, etc-- and this information might also need to be added to the test requisition paper as well.  DO NOT forget to fill these out completely, and I advise you now to keep a log yourself of each test you do and include in this log the day, the test type, the total VOLUME of the sample collected, and the lab you submit it to if you are using different labs for different test types (I had to due to local restrictions).  It is VERY common for labs to order these wrong, to forget to write down total sample volume (and thus not calculate results), etc, etc.  It will always be aggravating, but being prepared in this way will at least mean that some of that effort can still be useful.  I've had friends take pictures of their labels with their cell to keep track.  I personally write the information down along with my daily symptom/sign/weight tracking that I am already doing so everything is in one place.

The biggest downfall of this test seems to be that it is totaling your cortisol output over a 24 hour period.  So, if you have a flipped (backwards) diurnal rhythm and your cortisol is high at night but low during the day, your average can still show up normal or just slightly elevated, even though you may quite ill.  It takes an astute doctor with experience to figure this out and know how to test more effectively...not to mention, a doctor who trusts his patient's symptomology. 

The second reason this 24 hour total can be a downfall is that more and more we are recognizing cyclic and episodic disease states where they alternate between high cortisol and normal or low cortisol states over periods of days, weeks, months, and possibly even years.  I was a fairly predictable cyclic case (cyclic basically means more predictable, episodic means less-so) and had roughly 2 weeks of highs and 2 weeks of lows.  If I were to collect my 24 hr UFC's in a low cycle, it would not show I was ill.  In fact, this is EXACTLY what I did with my first round of testing, because I didn't know better.  I had one or two marginal high tests (above the normal range, but not sufficient evidence) and the rest were normal.  Once I learned how to track my symptoms and could better gauge when to test, I moved on to a diagnosis.

Saturday, April 18, 2015

Testing for Cushing's, Introduction

I started this blog post during last year's Cushing's Awareness Blogger's Challenge in April, and never finished it because it felt like a Herculean task.  I do believe it is important information and it turns out I had the energy to delve into it some today, but I'm going to split it into separate posts because there is a fair amount of information to cover and I only have so much energy and brainpower each day.  Below is the intro and I'll make a separate post on each different type of test in the upcoming days. 

There are various tests used to look for high cortisol levels within the body.  None is perfect and each has it's inherent strengths and weaknesses.  Not all test types will work for everyone, and they won't show a diagnostic high every time you test either, unless you have a more florid form of the disease (ie, always high all the time).  Most of us are diagnosed off of a mixture of these tests, and they often have to be repeated many times.  Here are the most common:

24 hour Urinary Free Cortisol (UFC)
24 hour 17-hydroxycorticosteroids (17 ohc/17ohs)
10 hour Urinary Free Cortisol
Midnight Serum Cortisol
Serum Adrenal Corticotropin Releasing Hormone (ACTH)
Serum Cortisol Binding Globulin (CBG)
Dexamethasone Suppression Tests (Dex)
Inferior Petrosal Sinus Sampling (IPSS)
Imaging such as MRI's and CT's

For most patients, we have to do a series of tests, usually in about 3 different types, before we get our official diagnosis and move on to treatment.  My endocrinologist typically runs 3 or 4 each of 3 different types of tests when suspicion is high for Cushing's (saliva, 24 hr UFC, and either he tags on 17ohc's to each of those 24hr samples or he might order 10 hour UFC's if he suspects you are more cyclic with higher cortisol at night and lower cortisol in the day --it just all depends on your specific case) , along with a midnight serum cortisol and ACTH level after your appointment while in his office, testing of other hormones, and usually he orders an 8am lab to be done when you get home as well (8am serum cortisol, ACTH, and maybe other hormones, vitamin D, and often ferritin as well).

I personally did countless 24hr UFC's, 17ohc's, 10 hr UFC's, Midnight Serums, a handful of ACTH's (midnight and 8am), and a handful of midnight salivary cortisols.  I got many diagnostic highs in every test type *except* for salivary.  (Sad, too, cause they are the easiest to do!)  We don't know why, but patient experience seems to show that it is QUITE common for a patient to test high in some tests and not in others...which tests work best varies from patient to patient and from case to case.

(If I miss something important, please feel free to contact me and I'll update as necessary.)

Friday, April 17, 2015

Growth Hormone Deficiency in Adulthood and the Effects of Growth Hormone Replacement: A Review

This isn't Cushing's-specific, but MANY of us have Cushing's Disease which is caused by pituitary tumors...and tumors are apparently the #1 cause of adult-onset Growth Hormone Deficiency.  We Cushing's patients have those tumors surgically removed, which surgery also can cause a loss of growth hormone production (double whammy, I say), so this is a very appropriate subject for Cushing's Patients to be aware of.  There is a lot of overlap in the symptomology as well, so AGHD needs to be ruled out as the initial cause of symptoms when being diagnosed as well as when a recurrence might be suspected.

So, today I received the Magic Foundation's Friday email which contained an amazing article with LOTS of good information related to Adult Growth Hormone Deficiency and it's treatment (replacement injections).  This article goes through changes to different body systems (metabolism, muscle, bone, skin, body fat, etc) due to AGHD, and then how those things have been affected by GH replacement therapy.  Like I said, there is a TON of interesting information here, but what I found really interesting was how some changes were immediate (happening within days or weeks), some changes didn't really take effect until 3 or 6 months of continuous treatment (and some of those seeing continued improvement up to 3 years later as well), and then there were other more negative changes that happened up-front but returned to normal as your body adjusted over time.  Click on the title below to read for yourself:

Growth Hormone Deficiency in Adulthood and the Effects of Growth Hormone Replacement: A Review

Thursday, April 16, 2015

Nerdy Zebras

We spent the last night of our trip with one of my sisters-in-law, and she pulled out this absolute beauty:

I never was a dedicated fan of zebras, or their stripes, before Cushing's Disease.  (Though admittedly, yellow and blue --the Cushing's awareness ribbon colors-- were my wedding colors!)  But now that I've been diagnosed with rare diseases, I see them everywhere and enjoy their stripyness so much more.  The awareness ribbon for rare diseases is made of zebra stripes and we often call ourselves zebras, too.  There is an oft-heard phrase doctors are taught and then repeat to us patients...patients who couldn't possibly have THAT disease's too rare!

"When you hear hoofbeats, think of horses not zebras." 

A phrase taught to medical students in the late 1940's by Dr. Theodore Woodward, 
professor at the University of Maryland School of Medicine.

Well, many of us got our nerdy glasses on, learned what we needed to know, did what we needed to do, and got our zebra selves to the doctors we needed to see to get our diagnosis and treatment.

We earned our stripes and we wear them proudly!

Tuesday, April 14, 2015

Cushie Friends

On the first night of our trip, we were able to meet up with some fellow Cushie's for dinner ON Cushing's Awareness Day -- Dr. Harvey Cushing's birthday.  It was a blast!  For some perspective, I believe between the 5 of us we've had at least 9 surgeries in an effort to cure Cushing's Disease.  LOVE THESE LADIES!!!

Monday, April 13, 2015

Pushing yourself

I've missed a few days, SORRY!  We were out of town on a crazy-busy trip where I didn't even see a computer (even though we brought a laptop with us!), and I was very, very exhausted and sore when I got home.  I went AI one night on our trip, hard and fast, and that was scary.  I had to stress-dose before I could sleep, wait for it to "wake me up" a bit...I'd really say it was more like it made my head less sludgy and more clear.  Then I went to sleep and slept well. 

So yesterday I just stayed in bed to try to recover from the trip.  If it weren't for my doctor's appointment this morning, I would definitely have preferred to stay in bed as long as possible today.

In relating my experience to my friends, one responded: "Making yourself do stuff is a good reminder of how little your body can actually do without falling apart."  Wow.  I expected the soreness and tiredness, but was shocked at just how sore and exhausted I was.  And, the AI wasn't so pleasant, just sayin'.  All my life I was the kind of person who'd just push to get the job done, but Cushing's eventually made that impossible.  If I push myself, I crash...and it's the kind of crash that can be life-threatening, and even with the proper medication, can take many days to really fully recover from. 

Friday, April 10, 2015

Sphygmomanometer (SFIG-moh-mə-NOM-i-tər)

If you are working on getting your Cushing's diagnosis (cause all that testing IS work), if you are post-op and in recovery, or if you had your adrenal glands removed (BLA), I highly recommend a sphygmomanometer (BP cuff). 

Tracking your BP and pulse can really help you get a feel for when your adrenal hormones are too high or too low.  It isn't a perfect barometer, but it can definitely help.  Many cyclic patients have a bit of a learning curve differentiating high cortisol periods from low cortisol periods when they are first learning about Cushing's and starting to test.  Even post-op you have to learn what "too low" feels like, and it can be somewhat different than the "lows" you feel when you cycle before surgery.

In general, your BP (and sometimes pulse) is higher when your cortisol level is higher.  This is a good time to test if you are not yet diagnosed.

In general, if your BP and pulse is lower, your cortisol level is also lower.

But, as I said, this is NOT a hard and fast rule and it is best to really get a feel for your own individual symptom sets, mixed with signs such as blood pressure and pulse, and to be proactive and use prudent judgment. 

In the case of adrenal insufficiency (if you cycle hard and fast, are post op, etc), things can get really bad really fast, and I always prefer to err on the side of caution.  Though too much hydrocortisone, just like high endogenous cortisol, can cause significant damage over time, a little too much cortisol at one specific time (when AI/adrenal crisis is suspected) isn't likely to do any lasting harm.  Too little in that same situation can become life-threatening quickly, and it often takes your ability to think clearly and act in the process.    

Sometimes people with adrenal insufficiency have what is called a paradoxical response This means their blood pressure goes high even though their cortisol levels are plummeting, as their body tries hard to compensate and stave off adrenal crisis.  It will crash eventually, possibly quickly and dangerously, so it is best to treat it early if you have reason to believe this is the case with you.  In such a case, adding sufficient cortisol will actually LOWER blood pressure and stabilize the patient.  I had a friend show up to the ER with a BP in the 200's/100's, was finally given 100mg solu-cortef, her AI-related symptoms resolved and her BP dropped down to around the 130's/80's.  (If you don't need the extra cortisol, it will raise your BP...she obviously was desperately in need!  Her body put that cortisol to immediate use and started to stabilize itself.)

Regardless, try to get a feel for what "normal" is for you.  Try to pay close attention to your body so you can recognize what might signify a high, or what might signify a low.  At the same time, I've noticed that AI with differing causes for me can also start with differing symptoms.  I have a typical "slow decline" set and a typical "fast crash" one.  I also found that the low symptoms I experienced due to taking a medication that lowerd my cortisol felt different than my natural "stress/overdoing it" lows.  I got used to that too.  But then this year when I added in Growth Hormone replacement, I experienced another completely different set of low symptoms!  It didn't occur to me it wouldn't be like the previous medication's symptoms.  As miserable as I felt, I didn't recognize the symptoms as being from low cortisol till my Endocrinologist brought it up as the likely cause!  DOH!  It won't always be the same, so just listen, listen, listen to that body.

Another form of Adrenal Insufficiency:
  If you happen to also be dealing with aldosterone (another adrenal hormone) or it's synthetic replacement, fludrocortisone, the pulse/BP differences can be a very helpful indicator of sufficient replacement dosage.  Addisonians, BLAers, those with hypopituitarism, and the rare few like me who have had low aldosterone despite high cortisol levels sometimes have to balance both fludro and cortisol...and it can get tricky. 

Higher BP and lower pulse suggests high aldosterone, fludrocortisone over-replacement, or too much fludro and sodium in the diet.  (But fludrocortisone requires a goodly amount of salt intake to function well, so judge wisely.)

Lower BP with higher pulse suggests a need for more salt or too low of an aldosterone/fludricortisone level.

Symptoms of high and low aldo/fludro are actually quite similar.  And another few points worth keeping in mind is that when it is hot outside, you are sweating/active, or you are dehydrated/losing fluids for other reasons, you may need to increase your fludro or salt intake (and fluids, too) to make up for the extra loss.  Some find one works better for them than the other, and how much and which appears to be fairly individual.

*This is just informational, from patient experience.  Please speak with your doctor about how and when to treat symptoms and signs.*

Thursday, April 9, 2015

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