Friday, July 11, 2014

What it's like to see a doctor about Cushing's:

I posted this a year or two ago, but that link disappeared, so I'm reposting now that a friend has re-added it! 



So, not all doctors are like this, but MANY are...and most endocrinologists (that should be more educated about this disease) are as well, sad as that is.  There ARE some good ones out there, and we flock to them for help.  They save our lives and we love them eternally for it.  My endocrinologist (one of the ones we all flock to) says that the majority of his patients end up knowing more about this disease than their local endocrinologists do. 



Still, this is too true of our collective, ongoing experiences to not share:






https://www.youtube.com/watch?v=GRaDr3cPAmE










For some reason, it isn't letting me insert the video so you'll have to follow the link.


Even now, after a diagnosis, surgery, etc I am leery of seeing doctors and discussing it, despite LOVING to share the information and help others.  I have ample proof I was and still am ill.  But the vastness of how this disease affects one's system is astounding, so no matter how small the reason for seeing a doctor, everything is impacted by this disease and they end up asking a LOT of questions because they don't know much about it.  When you're sick (on top of your debilitating disease), it's tiring to have to educate your doctors.









Friday, June 13, 2014

Youtube Video

Brief patient interviews were recorded at the Magic Foundation conference in April to be posted on Youtube, as a way to help raise awareness for our diseases.  Here's a link to mine: https://www.youtube.com/watch?v=JcNnNVXd9UY&list=UUYTG0ASue_DHuQOC3ZNtiZw

And a link to my friend Alicia's: https://www.youtube.com/watch?v=-a0BwcAyWbo&index=3&list=UUYTG0ASue_DHuQOC3ZNtiZw


She's been cleared for her BLA and will be having surgery and hopefully on her way to wellness soon!  I hope to follow shortly behind in her footsteps!



Wednesday, June 11, 2014

Update: Post-op pit, not in remission

It's been a while since I've updated with my own status/progress, so here goes:

I had pituitary surgery on 2-5-2014 and am now 4 months post-op.  We knew early on that it didn't look like remission, I was off my replacement hormones within a month, had to wait for my cycles to normalize a bit (I guess all that pituitary fileting was noticed! lol) and about a month ago now I started testing in earnest for my re-diagnosis.  In April I had a post-op MRI and follow-up with my neurosurgeon, who said I did not have a visible target on MRI, so I wasn't a candidate for repeat surgery or radiation therapy.  So, we now know that a bilateral adrenalectomy (BLA, the surgical removal of both adrenal glands) is in my near future...but I need a multitude of lab tests to prove I need it, and give a surgeon enough reasoning to permanently remove two very vital little organs and put me on life-sustaining medication instead. 


After intensive testing one week, and hit/miss testing the next (I was cycling lower and thus stopped testing), I now have 5 diagnostic-level high lab results.  Because of the severity and permanency of this next surgery, my endocrinologist has asked me to keep testing.  If my pattern holds true (it's never perfect), then in about a week I will start testing again in the hopes of doubling the number of diagnostic-level highs that I have and move on to the surgeon referral process.  It'll take a couple of weeks (2-4) to get my lab results back, and another couple of weeks to get my endocrinologist appointment after that if I do get sufficient highs.  I'm *really* hoping he won't make me go on medication prior to surgery as they all have their (rather large and often permanent) side effects, and I'd like to move forward towards a permanent cure and health!  Not to mention, my deductible is met for the year, so this year would REALLY be nice. 


The surgeon I'm leaning towards is in Wisconsin and has a boatload of experience with this surgery.  The runner up is in Seattle's Virginia Mason (closer to home), but he has a lot less experience with BLA's and rest tissue testing/surgery.  We'll just have to see how things play out and when.  We're hopeful, and I'm starting to allow myself to plan for a near-future in which I am somewhat functional and active again.  I can't wait!  Once again, it sounds silly to be excited and wishful about having surgery to give me Addison's disease, just as it was to be thrilled to be told I had a tumor, dreaded disease, and needed brain surgery.  But, I've been sick for so long and becoming more and more debilitated and sick the longer this has gone on, I am excited at the prospect of any semblance of improvement, health and normalcy!  (Okay, within reason...I am well educated and using logic, etc on this, but...YAY!)  I can feel it is within my reach again.  I'm on the path and moving forward.

Monday, May 26, 2014

So you think you have Cushing's? Here's what to do...

A link with suggestions from people who've been there, done that...and who really do know their biznatch:  Cushiwiki  This will link you to page two of a three page game plan.  Buckle down; it's going to be a bumpy ride but you CAN make it to the other side!

Tuesday, May 6, 2014

Brain Atrophy in Cushing's Disease (Cognitive and Emotional changes on the brain)

"Brain Atrophy" is a scary term, right?  But I do use it.  When I say my memory is mush, or I get confused, can't remember things, can't focus, etc...I actually mean it!  It doesn't make me feel any better to know you forget names too.  ;)  I forget things like my own age, social security number, the age of my children (all of them at the same time), can't look from a phone book to the phone and remember a number, etc.  And if I get interrupted mid-sentence/story/thought, it's just gone...It won't come back for a good 5-50 minutes, so don't bother asking what I was saying!  I can't concentrate well enough to play simple songs on the piano or to even read a "for fun" book sometimes (when I used to play complicated pieces and read medical studies), can't concentrate well enough to feel safe driving (even in "Nowhere, Alaska"), and when I try to look at my options and think things through logically, I miss big, obvious things that someone else has to point out to me.  I say the wrong word ALL the time (Like "kitchen" instead of "bathroom" --my poor kids and husband!) and cannot be trusted to order food or make travel reservations alone because I mess things up royally.  It is a constant, ongoing problem...and I'm 33, not 66.


So when someone, presumably in an effort to make me feel better, won't allow that I do indeed have a real problem, it is actually irritating and I'll throw that term out there.  My body metabolizes bones and muscle, AND it shrinks my brain.  I WIN!  HA HA HA!  Oh....wait.  I am glad to be corrected when I make an obvious mistake, but PLEASE do it nicely.  It is hard enough living with the staggering effects of this disease all on it's own, I don't need it thrown in my face and laughed at too (Yes, this applies to you too, grammar Nazi's!  You know who you are!).  Forgive me if I cannot find the humor in it at times; I bet you'd find it hard too.  I do my best, but public humiliation shouldn't be expected to be funny to the person being humiliated.


Moving on to the medical side, here are some good excerpts from the article/study linked below:



"Cushing's disease is associated with brain atrophy and cognitive deficits. Excess glucocorticoids cause retraction and simplification of dendrites in the hippocampus, and this morphological change probably accounts for the hippocampal volume loss. Mechanisms by which glucocorticoids affect the brain include decreased neurogenesis and synthesis of neurotrophic factors, impaired glucose utilization, and increased actions of excitatory amino acids. In this review, the timing, pathology, and pathophysiology of the brain atrophy in Cushing's disease are discussed. The correlation of atrophy with cognitive deficits and its reversibility is also reviewed."


It makes real, visible changes in our brains.  Isn't THAT a comforting thought!  (Can you read my sarcasm?)  A friend and I were just discussing whether there was a possibility for this to help in faster diagnosis times.  Heaven knows we need that!


"Cognitive impairments are common in patients with Cushing's disease and are correlated with elevated cortisol levels. Starkman and coworkers[22] found cognitive deficits in several domains in patients with Cushing's disease, including verbal intellectual skills, learning, and memory. The largest decline in cognitive function in this study was found in measures of the verbal intelligence quotient and verbal learning and recall. These impairments are consistent with the clinical cognitive complaints reported by patients with Cushing's disease.[24] In contrast to dementia, delirium, and aging, which show increased vulnerability across visuospatial measures, verbal functions are most prominently affected in Cushing's disease. The deficits in verbal intellectual skills suggest involvement of the neocortex, whereas the impairments in verbal learning and recall are consistent with the increasingly accepted view that the hippocampus is especially vulnerable to the effects of glucocorticoids.[17,22,27,29]"


It's not just me!  And it is interesting to find that it is different in its presentation for Cushing's than for other causes of mental deficits.


"These data highlight the fact that the effects of glucocorticoids are not limited to the hippocampus-atrophy has been documented in the prefrontal cortex and other cortical areas.[1,12] It is also entirely possible that glucocorticoids affect specific areas of the brain differently. For example, in contrast to the dendritic atrophy observed in the hippocampus and prefrontal cortex, glucocorticoids increase dendritic growth in the amygdala.[12] "


Did you just read that?!?  So all those emotional changes that we blame on hormones alone, REALLY also are due to changes in the brain caused by the initial excess of cortisol (and the whole cascade of issues that stems from that --because really, all the hormones get thrown out of balance).  Can you think or fee" differently than your brain is wired to think and feel?  Recognizing the source could help dampen the effects thoughts and emotions can have on our lives, but we cannot simply chose to not think or feel that way, nor can we be expected to gain mastery over it.


"Primate studies using exogenous glucocorticoids show that hippocampal changes are present within 1 year of glucocorticoid exposure. Because of the often insidious onset of symptoms in Cushing's disease, no specific data on exposure duration and brain atrophy are available. Clinical investigations of exogenous glucocorticoid therapy show an exposure duration of as short as 2 to 6 months before changes in the brain and hippocampus are detected.[5,21] Cerebral cortical atrophy has been reported within 6 months of glucocorticoid exposure, even in children.[21] ...For example, Newcomer and colleagues[15] found deficits in verbal declarative memory after 4 days of cortisol exposure..."


Wow.  Seriously?  4 days to 6 months is all that is needed to make obvious changes to the brain.  No wonder it has gotten progressively worse over the past ?17? years...but most drastically in the past 3-4.  But don't worry, there IS some good news:


"Cerebral atrophy has been shown to be reversible in patients with Cushing's disease.[8] Following resection of adrenocorticotropin hormone-secreting pituitary adenomas, hippocampal formation volume has been shown to increase by as much as 10%.[23] The increase in hippocampal formation volume correlates with the magnitude of decrease in urinary free cortisol. In addition, improvements in memory correlate with decreases in cortisol levels as well as with increases in hippocampal formation volume.[9] Age has been identified as a significant factor that influences the speed of recovery. Younger patients regain and sustain their improvement in cognitive functioning more quickly than older subjects. These findings suggest that at least some of the deleterious effects of prolonged hyper-cortisolemia on cognitive functioning and hippocampal volume are reversible."

So, their findings suggest it can in fact be reversed, at least in part...and that it happens faster with younger patients.  I think we knew that anecdotally (that the young recovery from this disease faster, in general, than the old).  I'm hoping I hit cure/remission while young enough to recover on the quicker side...of course, I've also read articles stating that length of illness plays a large role in it as well.  But honestly, I'll take any improvement I can get and be happy for it! 


http://www.medscape.com/viewarticle/566312

Friday, May 2, 2014

How Cushing's has broken my body

I was planning to take a decent break after the whole "post every day in April for Cushing's Awareness Month" thing, but I somehow woke up knowing I needed to get this thought (and information) out there...


When my exhaustion comes up or people are around me a while, I continue to be told/asked things like:
"Have you tried walking?"
"If you did a little extra each day, you'd build muscle."
"Stress hormones are the problem?  Well, stop stressing!"
"I know you're tired, but if you don't exercise, you'll lose muscle and gain weight."
"Are you counting calories?  Not doing much, no wonder you're gaining weight."


I know these questions stem from ignorance about the disease and some people really are wanting to be helpful.  But it really is counter-intuitive, I'm not making that up. And when people feel the need to say these things to me it still hurts my feelings a bit even when I remember that.  It just reminds me that they haven't learned the basics about my disease, and it still feels as though they are blaming my symptoms on me again...like I'm a fat, lazy, idiot. 


Who doesn't know you exercise to maintain and gain muscle?  That you lose it if you don't use it?  That eating too much (or incorrectly) causes weight gain?  That in healthy people, expending energy in the form of exercise can actually increase your overall energy in the long-run?


Seriously, my body is broken.  These "normal" systems just don't work.

Cushing's causes the demineralization of bones.  Cushies break bones very easily.  Ever heard of someone waking up to a broken ankle with no trauma?  Yep.  Breaking ribs just rolling over in bed?  That too.  Imagine that isn't an 80 year old, but a 35 or 45 year old.  We develop osteopenia and osteoporosis as part of the disease process, and sometimes that is the first time doctors take notice that something abnormal is going on in our bodies.  I have a friend who is paralyzed because of broken vertebrae thanks to this disease.  It's horrible.  And it isn't his fault.


Now, imagine that your body also metabolizes your own muscles.  Really.  No amount of exercise can stop that, only curing the disease can...and then you have the long, daunting task of trying to rebuild amidst pain, exhaustion, and weakness.  I have a supplement that *might* help SLOW it but cannot stop it.  This is why people with my disease tend to have skinny arms and legs.  The first muscles to go (dramatically, anyway) are the quads and biceps.  It becomes difficult/impossible to walk up stairs, to stand unassisted from a squat or to get up from a seated position without using your arms.  And as far as the biceps go, one of the first things people notice is the inability to open lids...that requires your upper arm muscles more than you'd think.  As an example, I can't open about every 3rd Gatorade lid these days.  It is absurd!  Something so simple, and I can't do it.  And trust me, I open those bottles multiple times a day, every day, yet I am still losing that ability.  (I NEED the salt to raise my BP due to the lack of another hormone, that's a diagnosed need and I will die of dehydration without it, so don't even go there; I've already had that lecture from strangers in Wal-Mart or friends at a dinner table in public.) 


At the same time, imagine your disease is CAUSING extreme, uncontrollable weight gain.  That's one of the most obvious symptoms --it's not the disease, it's a SYMPTOM!  It doesn't matter how many calories you cut or how perfect your diet is, the weight just keeps piling on.  At very best you can maintain (not lose), but even that isn't possible for most.  It centers around your face (moon facies), your neck (sub-clavicular fat pads, buffalo hump, big chin), and your abdomen.  It is both firm on top (almost hard like a pregnancy belly) and soft on the bottom and hanging down (pendulous abdomen).  You CAN eat low-carb, low-starch, low-sugar to try to slow the onset of diabetes and weight gain, but you cannot stop what the chemicals in your body are doing.  Cortisol is directly involved in blood sugar within the body, and it can be catastrophic when either too high or too low.  For example, I eat roughly the same diet.  It isn't perfect by ANY means, and I have variations, but in a high I gain between .5-2lbs a day and on that same diet in a low, I lose .25-.5 pounds a day.  It isn't my diet, it is my cortisol levels...and the highs are outstripping the lows.  I'm in a pattern of slow gain, but it is very disheartening to see.


I mean, we need to use reason here.  If you are able to continue to exercise (strenuous is NOT recommended, I was flat-out told not to do that anymore), obviously it is a good idea to maintain as much muscle mass and strength as you can.  That said, I do NOT gain more energy if I use what I have.  Backwards perhaps, but true.  I don't get rejuvenation from sleep either...often waking up feeling as tired as when I went to bed (Cushing's messes up your sleep cycles, if you can get sleep).  Obviously it is wise to eat well, but remember most of us are dead broke with all these medical bills (and healthy food DOES cost more) and in my case, I am unable to cook any more.  I used to cook from scratch and eat a very clean diet, now even the energy to put a frozen meal in the oven, then remove it and dish it up later is too much most days.  We know that eating better is good for our bodies.  But, the reality is that being perfect in these things will not stop the disease nor it's effects on our bodies.  Even if you mean well, please don't say things that suggest we could stop it if we just tried harder or made a little change ourselves --it is hurtful and even if you don't mean to, pushes blame onto us.


Now, I really need to explain the "broken" thing.  Our hormone systems (at least in the pituitary) generally work in feedback loops.  Parts in the brain take in information about our body's condition, external environment, emotions, etc.  It interprets that information and makes judgments about what our body needs more or less of and sends the appropriate signals to other parts of the body (like the pituitary).  The pituitary then sends out stimulating hormones (in the case of Cushing's, ACTH - Adrenocorticotropic Hormone) that tell other glands to produce their various hormones for use.  Again, in the case of Cushing's, ACTH stimulates Cortisol specifically.  When there is sufficient cortisol in the body, it "feeds back" to the brain and pituitary that there is sufficient and the production of ACTH stops/drops. 


With Cushing's, that loop is broken.  Really broken.  There is a tumor getting in the way and throwing everything off.  The tumor produces ACTH on it's own, stimulating cortisol production by the adrenals.  That in turn shuts down the pituitary from producing it, because there is already enough or too much.  It means that our bodies don't react normally to internal nor external stressors.  We have FAR too much cortisol at night, when it should be almost non-existent in the body to allow for sleep.  We can have low cortisol during the day when we need it to be active.  We can be "revved up" like we're running a marathon while sitting on a chair, resting.  But it also means that when we are stressed, ill (just a cold, even), in pain, or exerting extra energy, our body does not have the feedback to produce more cortisol to allow us to function appropriately. 

This is why even in a high cortisol day, if I overdo it, I can crash so hard I can't keep my eyes open.  Why I can become so cold that blankets, warm clothes and a wood stove (on an already warm day) cannot warm me up.  Why I can become nauseated (another low symptom) and that can lead to serious danger (Look up adrenal crisis).  You see, if I use up whatever my tumor is producing, generally speaking, my pituitary doesn't fill in the gap because it is suppressed (asleep).  It doesn't say "She's doing too much, let's produce more hormones so she can get through it."  I've noticed this happen in cortisol highs and lows.  Generally I'm FAR more careful in a low because I already know my body cannot handle much of anything, but there were times when I would push myself in a high to get a job done, and then I would crash and burn.  It was bad news and I didn't understand how dangerous it was at the time (okay, I still do it sometimes, but I REALLY try not to).  And sadly, unlike the normal progression of tiring while exerting yourself, it generally didn't come with any warning signs.  I was capable, then I wasn't.  It was sudden.  I cannot tell when I am coming up on that wall, but once I hit it, it is too late.  I am ill, not just a little tired.  And because sleep only does a little in my Cushing's situation, I don't start fresh tomorrow.  Now I'm in the whole...one I might not recover from.  This new level of exhaustion at times has become my new "normal."  It's bad news, but it's my life.  So if I say something as silly as driving in "Nowhere, Alaska" is too stressful, I mean it.  It can make me physically ill, and it doesn't mean I mentally stress over it..it is my body recognizing the energy drain.  I cannot lose any more of what I currently have...there just isn't enough left.


I know this sounds so negative and depressing.  It is.  It wasn't my intent to end that way, but the reality is, this disease hurts us so much already, we don't need misunderstanding (even misguided desires to help) causing us more.  And seriously, just ask!  We would love nothing more than to educate others about what we're going through.  We know it is so convoluted and overwhelming and that you can only handle little tidbits at a time, but any understanding is better than none.  It can be so hard for us not to do those things we used to, especially things we consider necessary, simple, or things we love and enjoy, and it helps to have someone understand that and not pressure us into doing more than we should or judging us for not reacting like we used to.  We still want to be friends and would love nothing more than to help you or go do those fun things with you...we just know our limits.  Stick by us and please don't take it personally.

Wednesday, April 30, 2014

The Emotional Side of the Magic Convention

I decided that I'd separate off the emotional information and experiences from the more technical ones of yesterday's post.  I'll start by sharing some notes I took as Catherine Jonas, LMFT, CMC from The Pituitary Center at Cedars-Sinai Medical Center spoke on "Tools for Coping with my Pituitary Disorder."


  • Coping Mechanisms:
    Fighting Spirit 
    Avoidance/Denial 
    Fatalism 
    Helplessness/Hopelessness 
    Anxious Preoccupation
  • Most of us fluctuate between these various coping mechanisms, and they can all be useful and worthwhile to get us through a hard situation as long as we don't get stuck in one long-term.
  • Other good methods of coping are: problem solving, decision making, seeking info and setting goals.
  • Remember to engage in pleasant activities --things that make you happy or bring you joy.  Not everything should be about your disease and limitations.
  • We often deny ourselves positive experiences and happiness by almost-instantaneous irrational thought processes.  It is good to recognize them for what they are so that we do not isolate ourselves and limit our chances for support and enjoyment.
  • How to differentiate between rational and irrational thinking:
    1.  Is it based in fact?
    2.  Is it/does it bring an emotion I want?
    3.  Does it help me reach my goals?
An example of the latter was:  I'm invited to attend a wedding.  Immediate, irrational thought: I can't go because I won't ever find a dress that will fit me/that I will look good in.  Is it fact?  Does it bring positive emotions?  Does it help reach goals?  We do this to ourselves so often.  There are many health-related reasons that we are in isolation much of the time, but she suggested that we get out of our comfort zones just a little, and don't limit ourselves more than we have to.  There is a difference between knowing our boundaries and healthy limitations (physical and emotional energy, etc), and shutting ourselves off from what could be positive experiences because of irrational, unhelpful thoughts.


I have to say, despite all the stress and exertion it took for me to get to the convention (I had a lot of travelling on my own, plus an MRI and doctor's appointment) and the heat and humidity that made me feel ill while there, I am BEYOND happy that I went.  I plan to NEVER travel alone again, maybe for life (ha ha) because of how sick I felt the first few days, BUT it was worth it all.  I cannot truly explain how it felt to be there.  I had a decent understanding of my disease and the endocrine system (not perfect, just decent) before I went.  I did learn a lot of valuable information, and it was so validating to hear it spoken by national experts from large, recognized hospitals and institutions.  Still, what I most cherish from my time at the Magic Convention is the time I had with others like me.  They are "my people" as my friend JennyZ would say.  I cannot express the immediate bond that I felt with those fighting this disease just like me; we really are like family.  I felt safe and comfortable with them and that is a HUGE deal.  I didn't have to put on pretenses, deal with dirty looks or judgment, didn't have to pretend I had more energy or was happier than I really was, didn't have to stand up for myself and explain my illness...They just got it.  I've met my best friends on the internet via Cushing's support groups --and they are a very, very real part of my life.  Now I've been able to meet some of them in person, and I pray they will forever stay part of my family.  I could talk about this for hours and still not get across the immense feeling of acceptance, friendship, companionship, even relief that I felt there this weekend.  I am not alone.


Some funny tid-bits from the convention to shed a little light on what I mean:

  • The convention was changed from 2 full days of presentations to 3 roughly half days of presentations because it was too exhausting for us patients in attendance...That tells you something right there!
  • It was COMPLETELY normal and acceptable to go take a nap between lunch and dinner.  It was NOT considered being antisocial or lazy, but "necessary" to be able to get up for dinner and function the following day.
  • Every day (often multiple times a day), we would have to re-ask others' names.  It wasn't considered rude --EVERYONE did it.  And yes, we wore name tags.  LOL!