Sunday, November 30, 2014

LA trip: MRI, LFT's and Keto, Dr. F, AGHD, friends and a documentary

Yeah, so that is a really long title, but I have a lot of updating to do on many subjects!

If you remember, Dr. F (my endocrinologist in LA) put me on Ketoconazole at my last appointment, gave me lab slips to check my liver enzymes after about 2 months on the medication, and gave me an order for a repeat MRI after about 3 months of treatment with keto.  You see, it appears that when keto messes with the feedback loop of ACTH and cortisol, it can sometimes spur the tumor to grow or produce more, making it more obvious.  I just say "makes it angry", so now you'll know what I mean by that.  But Keto also commonly causes liver damage, making the liver "angry" too.  (And this is the safest and least damaging of the few medications available for Cushing's; to my knowledge of the others, they involve multiple organs and can be really harsh).  I was on keto after my initial diagnosis in 2013 while I waited for surgery, and had no real problems.

Anyway, there are no 3T MRI machines in "Nowhere, Alaska", and no roads out.  The closest is Seattle and that place charges boatloads for their "magical" MRI's, making it cheaper by far for me to pay the added expense to fly to the Los Angeles area and have my MRI done there.  Plus, I like to.see Dr. F while I'm in town as in-person appointments just work so much better for me than phone ones or email ones.

So the second week of November, Britton and I flew down for my MRI and appointment.  We were lucky and were able to meet with a number of "Cushie's" while there (Friends I've made online, most I'd not met in person yet, all who have or had Cushing's Disease) which made the trip just amazing.  I'll talk more about that later and start with the medical stuff.

So, my MRI went well.  The machine in Torrance is SO much more comfortable than the GE one I was in at MDA, and this one didn't try to burn my arms at the end, though their headphone music system wasn't working so I had to spend an hour with my thoughts and some really loud noises with nothing to look at (no, falling asleep isn't an option for me; I wish!). 

When we met with friends at the hotel that afternoon, we put my disc in their computer and sent a drop-box copy to another "cushie" friend who loves to read MRI's as a hobby (not an MD, but she's pretty dang good).  There were new signs of empty sella, which means my pituitary is flattening, and there is (Cerebro-Spinal Fluid) CSF in my sella.  Empty sella syndrome tends to lead to hypopituitarism.  So far I'd been okay and my pituitary was still functioning normally...though that isn't to say it won't lose function over time.  There is no way to know, we just have to watch symptoms and test routinely as time goes on to see if things worsen.

There were two other things noticed...a possible new tumor on my MRI, when compared to my old pre-op MRI (different spot but same side of pituitary), and some crazy stuff going on in my sinus that looks like it is full of bubbles (on CT in August it looked like a solid mass about 2cm by 2.5cm, that the ENT's thought was scar tissue).  None of us had seen that before, and my friend called it a "train wreck."

At my appointment, we discussed my liver function tests (LFT's), which I had drawn the week before and had come back significantly elevated (like 5 times the high end of the normal range), and I was taken off of ketoconazole.  Luckily I'd swung into a low cortisol cycle the week before and had stopped taking it because it was making me swing too low and I hadn't restarted yet. 

Not surprisingly, Dr. F viewed my MRI and made the same statements my friend had (aside from the sinus one).  He suggested I send my MRI on to Dr. McC (neurosurgeon in Houston) to get his opinion on whether that was a target (ie, tumor).  The radiologist later called it "changes from surgery", but he also admitted to missing the original tumor on my very first MRI, so I'm waiting for an expert to weigh in on that.  Dr. F also wanted to retest my thyroid and growth hormone (GH), because those are usually the first two hormones to be affected by pituitary damage (the empty sella).

My thyroid is still happy, but my IGF-1 (the test they run to check GH) had dropped by half and was now well below the normal range for my age and gender.  I'm not sure if my low IGF-1 was affected by my liver damage, so we are retesting my liver weekly and then will go from there.  If that IGF-1 stays low, I will need a GH stim test to prove my pituitary isn't capable of producing enough (for the insurance company who HATES to pay for this expensive medication --and it IS expensive) and then I will go on daily injections for life to replace the hormone my body can no longer make.  (FYI, a stim test is where they force your body, through medication, to make as much growth hormone as it can and test at exact time intervals to see your body's response.)

Here is where you say, "Growth hormone?!  You're 33!  Surely you've stopped growing by now?  Why would you need daily GH?"  Well, here's why: Adult Growth Hormone Deficiency (AGHD) causes all kinds of problems.  It's like cortisol in that it does a specific job, and that's all we tend to think about, but when it is out of whack, all KINDS of body systems suffer.  Just to name a few, it leads to abnormally high cholesterol and thus cardiac/artery issues.  It leads to thinning bones and muscle loss.  It leads to accumulation of visceral fat.  The list goes on, and it is not pretty.  Combine that with the big list of what Cushing's does, and some of those equate to a double whammy.  I'm 33, I certainly don't want a heart attack or osteoporosis already!  The side effects of taking too much are swelling or pain in the hands...when that hits, you can simply lower your dose.  Not hard to balance those risks/benefits, eh?  And just in case you're wondering, it is taken for life because pituitary's aren't known to recover.

Livers, though, can.  I've since tested on the 20th and 26th, and from the 7th to the 26th, my LFT's dropped by half!  Half of that in those last 6 days!  It looks like the momentum of my liver's recovery with the medication out of my system is improving and I'll be within normal range in no time.  I will test those again this next week and see how I'm doing (Dr. F's suggestion), and then we can progress with testing for GH and be certain the results will be accurate. 

If I need a stim, it cannot be done locally (I've called everyone including the hospital), so I've already contacted Swedish and would have it done by Dr. Yuen there.  He's an endocrinologist on Dr. F's recommended endo's list who used to work out of OHSU in Oregon and recently switched to working at Swedish's pituitary center.  YAY!  His scheduler said all I would need is the order from Dr. F and/or a referral sent to them and I could schedule my GH stim with about a week wait between making the appointment and having the test (Minus the winter holiday weeks).  Bonus: He's done MANY studies on growth hormone. 

And something else that is awesome?  Both a Cushie friend and an AGHD/hypo-pit friend (that I met at the Magic Foundation's adult conference this April) live in the general area and have offered me a place to stay!  Obviously this calls for two trips, right?  (Oh, I wish!!!)

Hmm...what else?  Oh, friends and the documentary.  Well, that part was just awesome, but this post is already long and wordy so I probably should save it for a future post.  CLIFF HANGER!

ETA: I forgot to mention that I am also retesting for Cushing's, to see if my testing is more definitive for a repeat diagnosis (and moving on to repeat surgery).  It'll take me some time, though...it's a great many tests that must be done while I'm in a high cycle.  Add to that that neither lab in town can do all of the tests (each can do some of my list, meaning all get hit but I have to juggle between them) and they have different drop off restrictions for time of day and days of the week.  Thanksgiving didn't help that, either.  ;)

Friday, September 26, 2014

Persistent Cushing's Disease -Update 9/26

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

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


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


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


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

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

Friday, August 8, 2014

Update August 2014

Well, I guess I should update everyone who might be reading.  My local friend, Amber, is traveling to see Dr. F for a new patient appointment, and Britton decided I should go with her!  I'll be getting my own follow-up appointment as well, in about 3 weeks.  We're both "invalids" but in different ways, have similar schedules and a very similar love of good food.  I can't wait! 

I now have 8 diagnostic high tests.  I have 6 diagnostic 17 hydroxycorticosteroids averaging double the range (but ranging from just decimal points over to three times the high end of normal).  At least these are consistently coming back high, showing there is indeed an issue, but the testing might be a little one-sided for BLA surgery clearance.  I have one diagnostic midnight serum of 8 (in a range to 7.5) and one high 10 hr UFC.  I have a feeling with these he will want me to go on medication for a while, especially since I'm going downhill so quickly with my mobility, etc., but I am STILL really hoping for surgery this fall.

I've had to stop testing to treat my sinus infection.  It is a little overly-attached if you ask me.  I'm on antibiotic #4 now, probably day 50 of antibiotic treatment so far.  It looks to be sphenoid-based, and after this last 30-day stint on med 4 is completed, I'll be getting a CT scan and ENT appointment to see whether it is gone or if I need a procedure to help drain the sphenoid sinus. 

We fly out the week after to see Dr. F.  I am REALLY looking forward to seeing him in person and at the very least, getting a good game plan together, even if it isn't what I am hoping for.  And, I am looking forward to a bit of a break from my day-to-day to spend some time with a friend relaxing, eating good food, and trying not to get too hot in Beverly Hills in August!

Wednesday, August 6, 2014

Pituitary conditions, PNA letter to families and friends

Dear Pituitary Families and Friends,

For your sake and the sake of your loved one, we ask that you take a few minutes to read this article. You may think pituitary patients are difficult and tend to complain a lot, but please let us explain. One of the hardest things for pituitary patients is the fact that there are a lot of unknowns. There are multiple symptoms that may (or may not) occur. The s...ymptoms may subside for a while, only to come back unexpectedly, much worse than before.

Exhaustion, fatigue, weakness in limbs, loss of libido, infertility, headaches, excessive sweating, apathy, anger, rage, depression, mood swings, uncontrollable weight gain, diabetes, and visual disturbances. These are just some of the symptoms of a pituitary disorder. Taken individually, these symptoms can be debilitating, but together they can be devastating. Can you imagine the burden on the patient if they also lose support from the people they depend on the most?

Symptoms such as the ones listed above can adversely affect the family unit. The apathy for example, can make family members feel like the person is disconnected or doesn't care what is happening with everyone else. Because of the disease, the patient may not even recognize that he or she is behaving this way. In addition, anger, depression and uncontrollable rage can also make the family feel like they are under siege: abused, unloved, unappreciated and unneeded. The fact is, this is when pituitary patients need their family the most. It's understandable to want to pull away and retreat emotionally, but that just ends up isolating the patient even more. These are the times that your loved one needs you to stand by him or her and try to understand that the disease is controlling the bad behavior. If your loved one had cancer would you accuse that person of being lazy or making up the symptoms? Of course not, but this happens regularly to pituitary patients. We all know that cancer is a frightening disease that does horrible things to patients. Many people do not know about pituitary/ hormonal disorders and the negative effects it has on a person's everyday life.

In times of crisis, the family dynamics become magnified. Patients dealing with a diagnosis of an unknown intruder in their body need the support and understanding of their family more than ever.

Now imagine that the intruder is unseen (have you ever tried to make sense of those MRIs?) and not clearly definable. When people hear that their loved one has a pituitary tumor, but that it is not cancerous or terminal, they are relieved and think that everything is going to be okay. Unfortunately, what they don't know is that the pituitary issue will affect every aspect of the patient's life, possibly indefinitely.

When people hear about a tumor, they don't think about how it diminishes the patients' mental health, their quality of their life, or their ability to accomplish everyday tasks. Pituitary tumors are usually benign, but they do cause some severe symptoms and they can require a lifetime of treatment and monitoring. Cancer is something that you can fight and BEAT, but for most pituitary patients, the battle goes on and on and on.

Understanding and acceptance truly comes from knowledge. The more you know about the disorder, the better prepared you are to support your loved one through his or her daily struggles. It can be very frustrating to have to care for someone who is ill, to have to pick up the slack with the daily chores they used to be able to do. When a patient is diagnosed with a pituitary or hormonal disorder, it is important that the family comes together to develop a plan of attack. Someone faced with a chronic illness will be able to handle it much better if they have the necessary support. The first and most important thing that any loved one can do is get educated about their loved one's disorder. Read the information contained on our website www.pituitary.org, purchase our Pituitary Patient Resource Guide, and learn as much as you can from trusted sources. Our information is compiled from the world's renowned experts in pituitary medicine.

The more you are aware of up front, the better equipped you will be to make decisions. Your family will have to make adjustments. This is not going to be easy, but it can be made less stressful. Within the immediate family/household, all roles need to be assessed and redefined if necessary. For example, the person with pituitary disease may not be strong enough to carry out their regular household chores or do the same amount of driving. They may be too scattered to effectively manage the family's finances or schedules. Their emotions may get out of control at home or at work.

If you know going in what changes to expect, they are much easier to accept and adapt to. One thing that is very important to note; if family and friends are not supportive, if they do not believe the patient when he or she reports struggles caused by this disorder, it can exacerbate your loved one's symptoms. The fatigue and strain that they go through will be amplified if they have to justify their symptoms to people. Sometimes people are accusatory and assume that the patient is just lazy. Patients are told that they'd probably feel better if they just ate a little less and exercised a little more. This kind of attitude undermines the patient's treatment. There is a medical explanation for their symptoms and for their inability to live a normal life. It is not just another excuse. They did not choose this illness. They do not want to miss out on family functions; they don't like that they can't do their part. They cannot control their symptoms, so they need your understanding. Thank you for everything that you do to be supportive. The PNA is here to support you in your quest for information. Please contact us with your questions and we will be happy to point you in the right direction.

Message to Pituitary Families
By Tammy Mazzella, Pituitary Network Association

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!


 
 


 


 

Tuesday, April 29, 2014

Trip and Information/Quotes from Magic --READ THIS!

Wow, where do I start?  I'm home again, and I SO need to rest for a few days (what's new, right? lol).  The trip went well, even though I was over-exerting myself in hot weather with little sleep.  OUCH.  But, it was so worth it!  I'm just not going to travel alone again...maybe ever!  ;)

So, the big updates are that I had my post-op MRI and follow up appointment (though it did NOT go as planned).  My MRI looks better post-op than it did before surgery (good news, right?!)  I went from having what looked like a post-op MRI (chunk missing here, blob added there) to having what looks like a normal MRI (perfect little "u" shape).  There is no surgical target on my MRI, which means unless something changes (ie, grows) between now and the time I'm finished with my re-diagnosis testing, I am not a candidate for further pit surgery nor either form of radiation therapy.  With those MRI findings plus the thrice-repeated "hyperplasia" (in different forms/ways) on my pathology report, Dr. McC says bilateral adrenalectomy (BLA, removal of both adrenal glands) is the next course of treatment for me.  I still need a great deal more testing before I have some life-giving glands permanently removed from my body, but now we know where we're headed, how many tests I'll need, and which surgeons to look into.  So far I'm considering a surgeon close to home at Virginia Mason in Seattle or a more experienced (in BLA's specifically) surgeon in Wisconsin.  It'll be a big change, with my life literally being dependent on the intake of medication throughout the day for survival and the constant risk of adrenal crisis, basically Addison's disease without ANY cushion of poorly-functioning adrenals (cause I won't have any!), but it should bring a lasting cure from Cushing's disease and even with the illness I'll be trading it for, my quality of life should improve dramatically. 

On to MAGIC:  How on earth do I sum up 3 days of learning, emotions, and fun!?  I already had a basic understanding of the pituitary gland's feedback systems, hormones and functions, along with a decent understanding of my disease and it's treatments, but I did learn some new things along with some nuances and interesting points, for sure.  I'll go through my notes and share what I learned and found most interesting.


  • I learned that 15-30 minutes was NOT enough time for even 3 people to share their Cushing's stories.
  • In endogenous Cushing's Syndrome (meaning, coming from within the body, not from taking medications), 70% is Cushing's Disease (pituitary tumor), 10% is ectopic, 15% is adrenal, and 5% are "other" --don't ask me?
  • 50% of pathology-proven Cushing's Disease cases (ie, they have surgery and the tumor is found and tests positive as an ACTH producing adenoma) were not seen on MRI prior to surgery.
  • Adrenals can become hypertrophied from long-term ACTH-driven Cushing's Disease...meaning, they've been forced to produce so much for so long that they kind of just kick into overdrive regardless of the current stimulating hormone production (ie, even after a tumor is removed from the pituitary, your adrenals might not slow down), though it isn't known how common/rare this is.
  • Cardiovascular damage is the biggest concern, with 4 times the mortality rate in Cushing's Syndrome compared to the national average.
  • Cushing's is really more like a state of Adrenal Insufficiency with Hypercortisolism superimposed upon it.  IE, the pituitary's (and post-op, your adrenals') own ACTH/cortisol production is suppressed because of the tumor = adrenal insufficiency/Addison's disease. The tumor is causing excess production of ACTH/cortisol (ie, Cushing's) = hypercortisolism.  So when the source of hypercortisolism is successfully removed/treated, your body is left in a state of adrenal insufficiency, requiring steroid replacement therapy and a slow weaning process to "wake" your pituitary and adrenals in the hopes of returning full function.
  • It can take up to 5 years for your adrenals to fully wake up.
  • Incidence (new cases) .7-2.4/million cases per year in Europe, looks to be higher in the US.
  • Prevalence (number of cases, old or new, at any given time) is roughly 60-100/100,000.
  • Some co-morbidities (couldn't take notes fast enough to get the whole list): carotid atherosclerosis, coronary artery disease, dyslipidemia, depression, hypertension, diabetes, obesity, etc.
  • The pituitary is made from neuro-endocrine tissue with similar receptors.
  • Diagnosis is a 3-step process.  The first step is ALL-IMPORTANT, because getting the first step wrong can lead to falsely-concordant results in steps 2 and 3.  
Step 1: Suspect Cushing's Syndrome without exogenous glucocorticoid use (steroid-based medication).  Test for high cortisol, exclude other physiological causes = Cushing's Syndrome diagnosis.
Step 2: Confirm high ACTH (helps differentiate causes of Cushing's Syndrome --ie, ectopic, adrenal, pituitary)
Step 3: Establish pituitary source of ACTH via MRI or IPSS = "Cushing's Disease" diagnosis. 
  • Cushing's diagnosis is like a justice scale -- you add weights for and against the diagnosis based on severity and likelihood.  Some tests and symptoms carry more weight than others. 
  • UFC's that are 3+ times the normal limit are considered "pathogenic", ie, nothing else can really cause levels that high.  Having results below that limit does not exclude Cushing's Syndrome, it just means further testing/investigation to be sure there are no other causes.
  • Few people have truly cyclic disease, which is easily predictable in it's pattern of high and low/normal cortisol production, and a more preferable term might be "variable" disease or hypercortisolism, because it varies from person to person and day/week/month/year within the same person.
  • ACTH has a 15 minute half-life, so samples can become degraded easily.
  • Pituitary MRI contrast shows differences in blood flow and tissue density.  ACTH-producing adenomas are very similar in tissue density and blood flow to that of healthy pituitary tissue, though not identical, so on a pre- and post-contrast MRI, pituitary adenomas are very, VERY easily missed.  The biggest difference (often the only difference) is visible with "Dynamic" MRI protocols, as the contrast uptake (absorption of the dye) in adenoma's is just slightly delayed in comparison to that of normal pituitary tissues.  So as the pituitary absorbs the dye and whitens, there might remain a slightly darker grey area (tumor tissue) for a few moments.  If the MRI scan is not performed during that "uptake" time, it will eventually also absorb the dye so as to often look indistinguishable from the gland itself.
  • There is a 10-35% chance of surgery failure (meaning surgery doesn't lead to remission).
  • 5 year recurrence rates in those who did receive surgical remission are as high as 25%.
  • 10 year recurrence rates are as high as 44%
  • It is said the longer you follow a patient in remission, the higher the rates of recurrence will go.
  • Repeat pituitary surgeries (after the first whether successful or not) have a less than 50% remission rate, along with increased incidence of CSF leaks (cerebrospinal fluid) and loss of pituitary hormone production (hypo-pituitarism).  3rd + surgeries decrease/increase those odds, respectively.
  • Radiotherapy (Radiation) has a mean reported remission time of 3 years.
  • Radiotherapy leads to hypo-pituitarism in 50-60% of cases, with a risk of damage to surrounding structures.
  • Bilateral Adrenalectomy (BLA, removal of both adrenal glands laparoscopically) has a risk of Nelson's Syndrome, corticoid deficiencies (Adrenal insufficiency/crisis), higher rate of ER trips. 
  • BLA-ers have a Quality Of Life (QOL) level that is VERY closely matched to that of Diabetes patients in terms of lifelong follow-up, testing, medications, etc.  (As compared to Cushing's Disease which has a QOL lower than that of Cancer patients, with an average 5 year mortality rate untreated.)
  • On Korlym, 60% of diabetic patients had a greater than or equal to 25% reduction in their glucose curve, and a 38% reduction in hypertension.  Side affects of nausea, fatigue, low potassium, endometrial issues, etc...(Couldn't type fast enough again!)
  • On Ketoconazole, roughly 40-50% had normalization of Urinary Free Cortisol labs (UFC's), though 19% of those still had no change in symptoms.  Keto shuts off the production of cortisol (synthesis), though hopefully only partly!  Side affects: nausea, diarrhea, low cortisol, increased liver enzymes, etc.
  • On Cabergoline (used more often for prolactinomas than ACTH-producing adenomas), in studies with 12-30 patients there was UFC normalization in 25-36%, with a small increase in usefulness seen over time.  (Works with Dopamine receptors)  Side affects of GI issues and dizziness, etc.
  • Signifor works on somatostatin receptors specific to pituitary ACTH tumors and not healthy pituitary tissue, so it allows your pituitary to "wake up" and function, though there are similar receptors found in the GI tract (diarrhea, etc as side effect) and pancreas.  It causes diabetes because of it's affects on the pancreas, but that is easily treated with insulin and quickly stabilizes (doesn't continue to worsen).  29% initially mildly elevated LFT's that usually resolves itself.  Side affects are basically those of steroid withdrawal. In one patient it did cause QT prolongation (heart rhythm changes), so monitoring is necessary to be sure you aren't that "one."
  • Increased IGF-1 is seen in Acromegaly.
  • Only 15 pituitary surgeries are required to complete a residency and become a Neurosurgeon.  It takes MANY more than that to become proficient, and statistics are poor below 200 total. 
  • Cushing's tumors are often VERY different than other types of pituitary tumors (gooey, milky, etc) and surgeons inexperienced in Cushing's-Specific pituitary surgeries may miss the tumorous tissue completely, or even "scramble it like an egg" thus spreading the tumor tissue unknowingly.  It is VERY important to see a Cushing's-experienced surgeon.
  • GPS mapping can be used to locate tumors in the posterior-pituitary region during surgery (an instance in which the tumor isn't visible upon opening for surgery).
  • Endoscopic dopplers (ultrasound on the end of what looks like a bent, stainless steel chopstick) can be used to locate the carotid arteries before and during removal of the sella turcica bones, dura, etc. 
  • The carotids sit as "goal posts" on either side of the pituitary gland with the optic nerves above.
  • Cushing's patients CAN get pregnant.
  • BLA/Addison's is not a reason to deny yourself a family (ie, pregnancy can be quite manageable) 

 


Some great quotes from this weekend:


  • "This is about the messiest disease out there."  -Dr. Ludlam, Director of "all things pituitary" at Novartis Pharmaceuticals, and previous Chief of Endocrinology at Swedish in Seattle, known to many as the director of "Camp Cushie".
  • "I've worked as a patient advocate for many different diseases before...Cancer, etc.  You guys have it so much worse.  I didn't hate doctors till I saw what you go through; their mindset and biases make it so much harder for you!" -Cushing's Patient Advocate
  • "The Alamo has come to symbolize fighting a battle with impossible odds...which some of you do on a daily basis."  -Dr. Heaney, Professor of Endocrinology, UCLA 
  • In speaking about post-op weaning from replacement medications and how awful it makes you feel, "You have to pay that piper." -Dr. Ludlam
  • "My mother died of Cancer...and when she was diagnosed she said to me, 'At least I don't have Cushing's.'  I'd talked to her about my work...She got it."  Paraphrased from Kate, A Cushing's Support Advocate (Nurse) for Corcept Pharmaceuticals.
  • "I was not ready to do pituitary surgery when I became a neurosurgeon." -Dr. Kelley, Neurosurgeon at UCLA, paraphrased from him speaking of the requirements to graduate from residency and the amount really required to become proficient.  Kudos to him for stating that the requirements (cases seen) are far too few, and that much more experience is required to become skilled...which he since has, to many patient's appreciation! **UPDATE - Dr. Daniel F Kelly is now the Director of the Brain Tumor Center at John Wayne Cancer Institute.  drdanielfkelly@gmail.com **
  • "I've had other doctor's tell me that if they had to diagnose and treat Cushing's all day, they'd quit [or shoot themselves]!" -Dr. Ludlam, said jovially about the confusion and long process that is the diagnosis and treatment of Cushing's disease.
  • "I haven't seen people suffer so much and be so misunderstood."  Kate, Cushing's Patient Advocate

Monday, April 28, 2014

Home, here I come!

Well, it is sure to have been an amazing, exhilarating, educating and enlightening trip...but I will also undoubtedly be exhausted and glad to be home again.  Today, I should be flying from Seattle home.  PHEW!  I'll be checking in tomorrow with updates as to what I've learned and how everything went for the last two days' posts of the Cushing's Awareness Blogger's Challenge.  I'm so glad I did it this year even though I haven't always had the energy to go as in-depth and get as down and dirty as I'd have liked (yes, even writing takes a toll on my energy, even though it is very slight --it is more the emotions it drags up).  On those days when I just couldn't, I relied on the strength of others to get me through (ie, linking to their posts and previous posts of mine!).

Sunday, April 27, 2014

Magic Convention 2014, Day 3

Day 3's speakers:

Hormone Replacement for the Addison’s Patient: Anthony P. Heaney, M.D., Ph.D.Monitoring your medications requires a delicate balance of hormonal treatments.  The segment will discuss the importance of lifelong management of your medications and healthcare.
Life after Bilateral Adrenalectomy - A Patient’s Perspective:  Philip ButlerA patient’s journey from onset of symptoms, diagnosis of Cushing's disease and treatments leading to bilateral adrenalectomy.  Why it is important to research and fight for your life.
 
Cushings: How to stay connected and informed, Facilitated by Stacey Seitz: The last segment of the program will be an open discussion on how, as a Cushing's community, we can impact the lives of others dealing with Cushing's.
 
AGHD/Hypopit: How to stay connected and informed, Facilitated by Ann Rumrill:
The last segment of the program will be an open discussion on how to find the support and information we may need to manage our pituitary disorders.

Saturday, April 26, 2014

Magic Convention 2014, Day 2

Day 2's speakers:

Tools for Coping with My Pituitary Condition:  Catherine Jonas, LMFT, CMCFrom diagnosis to life-long maintenance, this presentation will address the psychological stressors of having a Pituitary condition.  Identifying your coping strategies and incorporating them into your life will help you better manage living with a chronic illness.
 
Surgical Options for Treating Pituitary Tumors:  Daniel F. Kelley, M.D.The surgical options for treating pituitary tumors will be reviewed and examined in the presentation.  The latest techniques, neurosurgical technology and innovation will be discussed.
 
Caregiver Support:  Catherine Jonas, LMFT, CMCAn open dialogue tailored to the family member/caregiver who is dealing with his/her own thoughts and emotions about a loved one with a pituitary condition.  Techniques for balancing self-care amidst caring for another.
 
 
 


Friday, April 25, 2014

Magic Convention 2014, Day 1

And it begins!!!  Okay, well, technically it started last night, but today starts the education, etc.

Today's speakers:
The Anatomy of the Pituitary Gland:  Daphne T. Adelman, BSN, MBA
The pituitary gland is often called the master gland of the body.  It is no surprise that diseases that affect the pituitary gland have a large impact on the lives of those suffering from pituitary disorders.  This segment will discuss the functions of a healthy pituitary and the impact of disease on the gland.

Cushings: Meet and Greet, Facilitated by Stacey Seitz: This segment of the program will be a meet and greet for the Cushings participants. Connecting with others by the sharing of stories, pictures and experiences with their own Cushing’s journey.

AGHD/Hypopit: Meet and Greet, Facilitated by Ann Rumrill: This segment of the program will be a meet and greet for the adult growth hormone deficient and hypopit participants. Connecting with others by the sharing of stories, pictures and experiences with their own journey.

Cushings Disease:  William H. Ludlam, M.D., Ph.D.Proper diagnosis is important in understanding the scope of Cushing’s Disease and the direction for treatment. This segment will address testing procedures, diagnosis, and treatment options and what to expect in remission.

Growth Hormone Deficiencies:  David L. Kleinberg, M.D.This segment will go over the different types of tests for Growth Hormone Deficiencies and how to manage life with replacement hormones.
 
Lunch with Guest Speaker on the “Highlights Project”: A representative from the Novartis/Highlight Project will be introducing and detailing the benefits of the project. The Highlights Project is a partnership between Novartis Pharmaceuticals and Kevyn Aucoin Beauty. Kevin Aucoin was a world-renowned makeup artist and photographer who also had Acromegaly. Highlights is designed to provide makeup tips and artistry for patients living with pituitary disorders, including Cushing’s disease, that may impact their appearance. They will be available during the convention to offer assistance in dealing with cosmetic challenges
Enhancing Your Doctor’s Appointment:  Daphne T. Adelman, BSN, MBA
Techniques will be discussed on how the patient can become their own advocate in their healthcare. The tests for endocrine disorders will be reviewed and discussed to help the patient understand their significance.
 

Thursday, April 24, 2014

BUSY DAY!!!

Today I have my blood draw first thing in the morning to check my kidney function (prior to having the dye contrast for my MRI)...it's protocol here.

Shortly after that, I'll be getting my MRI.  I sure hope MD Anderson's 3T Machines are set up with music like they are at MRI-Centers in LA where I've had my previous ones.  The second time I had one, their system wasn't working for some reason and it sure made the hour feel longer and the anxiety of being stuck in a tube, unable to move, with a "cage" on my head a bit more unbearable.  The good news is that the results will be visible on their computer system almost as quickly as they are taken, so when I get to my appointment, we'll be ready to go! 

I'll have a bit of a break for lunch (REALLY hoping the MRI is on-time) and my friend, Katherine Benner, will be in recovery in the neurosurgery unit from her Pituitary surgery, so I will be going to pop in and say hello.  She tells me Attorneys get extra-special treatment when they are in the hospital, so I hope she gets care that is beyond reproach and her surgery and recovery go well.

After visiting Katherine, I will be headed to the neurosurgery clinic for my appointment.  It doesn't look as though surgery was a success in curing my Cushing's, though tumor tissue was found and removed and surgery itself went quite well with a too-easy recovery.  My testing to prove recurrence/failed surgery will not be complete at that point, but this appointment will tell me whether, after the testing is sufficient, I will be looking at having a repeat pituitary surgery or if we will move to the next option, which is a bilateral adrenalectomy.  Big news either way.  I was really hoping to have sufficient lab work in by this appointment so that if pit surgery was the preferred option, I could possibly even extend my trip and have it the following week (I know, right?  They're awesome like that, though.), but my high/low cycles haven't been cooperative thus far.  I have a too-high ACTH for a midnight blood draw (when it should all be near zero) and what appears to be a too-high serum free cortisol, but I had to stop testing because I started getting migraines and then I swung low.  In theory my next high will start while I'm in Texas, if I follow my pre-surgery patterns, but who wants to do 24 hour urines while on a trip?  Apparently, this gal just might.  *Oy.*

ANYWAY, after my appointment, I will then be headed back to San Antonio for the "Meet and Greet" that starts off the Convention.  There will be hors d'oeuvres and displays, and it is also when we check in and get our final registration/convention paperwork, etc.  YAY!!!

Wednesday, April 23, 2014

HELLO TEXAS!

Today I arrive in Texas, on my way to the MD Anderson Cancer Center for my appointments starting in the morning.  With a 3 hour time difference, let's pray that I don't sleep through any alarms tomorrow morning!  Mean thing to do to a Cushie, making them have morning appointments! 
 


This is the main campus of MD Anderson.  When we said it was a maze, we weren't joking!  Sky bridges from one building to the next, little carts to ferry people like they have in airports (Beep beep!), multiple cafeterias, office locations given coordinates (7th floor, G elevator), it's own beauty shop, and even it's own hotel attached via sky bridge.  What is more impressive in comparison to all of that, is how just plain NICE everyone is.  I mean, people are nice in KTN, but for the most part, MD Anderson was amazing.  Everyone smiles genuinely, everyone likes helping you.  I even watched a gentleman in a lab coat walking through the hall stop and pick up a patient's tablet before it hit the floor (he'd fallen asleep waiting outside of the surgical area and it was sliding slowly off his lap).  Something small, sure, but it is just an example of the full-blown hospitality that is common-place there.  I've not been to a hotel that treated me any better, though I am glad none of them tried to drill into my skull.  ;)

Tuesday, April 22, 2014

Trip Time!

Today I leave on a trip to Texas!  I will first have a post-op MRI of my pituitary gland and a follow-up appointment with my neurosurgeon at MD Anderson in Houston, and then I will be in San Antonio for the Magic Foundation's Cushing's Convention.  There will be much news this week/weekend, but since I cannot guarantee I will have the time, nor the energy, to post while travelling, I will schedule a post for each day of my trip just to be sure I don't slack off from completing the Cushing's Blogger Challenge for the full 30 days.  I'll post the programs for the days of the convention, and hopefully I can use what I learn while there for new posts after I return home.  I may not be posting these blog posts to facebook, but if you feel so inclined, feel free to do it yourself (but someone please tag me so I can share it too if I get on!).

Monday, April 21, 2014

BlogRoll!!! And some oldies but goodies.

Well, you learn something new every day!  I just recently found out that a Cushing's-Survivor friend, MaryO, keeps an ongoing "blogroll" on the right hand side of her blog.  It shows various posts others writing about Cushing's have added, and links you to their blogs as well.  Talk about a fabulous network of information at your finger-tips!  I'll be leaving tomorrow on my trip, and while there will still be posts, they won't be quite the same...So to satiate your Cushing's blogger's appetite, here's a list of others you can enjoy!

Here is a link to her blog: http://cushie-blogger.blogspot.com/  Check out the blog roll to the right and see what speaks to you today.

Also, I will take this opportunity to link you to a few of my older blog posts (from before this blogger's challenge) that I think just might hit home for you:

Positive and Negative...Lets talk about illness

The Hormone Storms of Cushing's

"Diagnosis seeking" and other comments

My Cushing's Story

Sunday, April 20, 2014

A thief named Cushing's


I was trying to think of another topic to blog today, and coming up blank.  So I decided to look at a list that was made a while back of suggested topics from last year: http://cushie-blogger.blogspot.com/2013/03/cushings-awareness-blogging-challenge.html

One of them DID jump out to me this time:  "What three things has Cushing's stolen from you? What do you miss the most? What can you do in your Cushing's life to still achieve any of those goals? What new goals did Cushing's bring to you?"

Here comes the introspection...

1.  My ability to think clearly and remember well.  I consider myself a "jack of all trades, master of none."  I've loved my ability to do whatever I set my mind to.  To be able to easily learn whatever interests me and quickly become proficient and knowledgeable in that area.  It used to be that I'd watch a tv show, see a performance, or learn of a profession and think to myself, "Huh...that's kind of fun.  I could do that!"  I still say that in my head, but I have to remind myself now that I no longer have those options.  I can barely read a book.  I can no longer sift through complicated medical studies and come out well informed.  I can't remember names or phone numbers long enough to switch from one screen to the next.  I can at one minute use a big, unusual word like crenellations and yet not be able to come up with the word "kitchen" the next.  I used to be able to just sit in a college lecture and get a good grade without doing homework or every opening the text book.  I used to be really good at remembering information.  I was even really good at imitation --able to learn skills with ease by closely watching someone else do it well.  In every new thing I tried (karate, violin, tap dance, acting, art, philosophy, counseling/clinical psychology, emergency medicine, new languages, home construction/design, interval running, teaching, religious studies, sewing, goat midwifery, cooking, writing, etc, etc) I would always be told "Wow, you've got natural ability!"  Or "Are you sure you've never done this before?"  And everyone always told me I was "meant" to do whatever it was I was trying out at that time.  It made it (makes it?) almost impossible to know what my true passions or true talents might be, but I could rest assured that I would be able to master whatever skill I found necessary.  I still wish I could do many things and try many things, but it is almost hurtful to read all the inspirational posters and memes that say things like "Follow your dreams," "Make 'someday' today," or "Tomorrow starts today."  No, my tomorrow has to start tomorrow...when, someday, I get a cure and will hopefully mostly-recover from this disease that enslaves my mind and body.  Today, all I can do is survive and try to do so in the best way I can.

2.  My ability to just push through and make things happen.  I was so used to this.  I'd just push myself if/when needed and it wasn't a big deal.  That's normal, right?  Need to stay up till 3am to finish those dresses I sewed for my daughters' baptisms?  No problem.  Need to lift a 90lb bale of hay?  I've got this!  Need to chase a 2 year old through the house bent on making a mess of anything she touches?  That's just motherhood!  Dip 10 pounds of strawberries in chocolate for a luncheon?  YES!  Homeschool children during the day, sing and tap dance in a musical at night all while 20 weeks pregnant?  Sounds like a blast!  No more.  I lost this ability gradually over many, many years of worsening illness, but I still miss it a lot.  It irks me to no end when people treat me like I'm just lazy.  They obviously haven't taken the time to get to know me now (or never knew me before I was ill).  I made myself horribly sick when my illness started to really, noticeably progress because I was used to being able to push myself.  And when I did, I could rest up and in a day or two be back to normal.  Suddenly that was no longer the case (It did gradually worsen, but I think my awareness of the drastic change was sudden).  I would often run the primary alone while one counselor was out of town and the president was away with her husband for months treating his cancer.  2-3 hours of steady work in charge of 60+ kids (and their teachers).  I'd just push through it, but that became literally all I could do for the entire week.  Cooking, cleaning, nope.  Everyone assumed that because I looked like the normal me (albeit increasingly heavier and balder) that I must be fine...even when I explained I wasn't and that Sunday's effort stole from the rest of the week.  I started to hit a point where if I overdid it when I was tired (in a cortisol low cycle, especially), I would hit a new baseline "low" energy level and never recover.  NEVER recover.  Doing more, pushing myself DOES NOT increased my energy or stamina.  I miss "doing" everything and anything.  Really, I do.  I am glad I have an outlet for my creativity and for connection to others with the internet, but I miss "real life" more than I can express.

3.  Cushing's has completely stolen my sense of self.  Honestly, I don't know that I had this to begin with, so maybe I haven't lost it...So perhaps it has kept me from knowing my "true" self.  This is something I've spoken of before.  I even asked on facebook for what people think constitutes them.  (Er, however you'd say it.)  If your emotions are mostly dictated by hormone fluctuations, it can't be that.  If your thoughts are greatly effected by them...your choices, your physical body and physical as well as mental ability...your relationships, etc.  What part of "you" is "you?"  Many people with Cushing's remember a time before their Cushing's hit.  I hear some form of "I miss my old self" or "I want to get back to being me" at least once a week, maybe once a day.  I do technically remember a time pre-Cushing's, but it was in my pre-teen and early teen years...my formative years.  I have no idea what I would be like as an adult without Cushing's.  None.  Will my preferences change?  My reactions to others?  My interests and abilities?  Will I have friends again?  It sounds weird, but it's true.  I don't know who I am.  I know I have worth, and I have good values, but what will change when I am in remission?  I have no idea.  Still, I have faith it'll be better than where I am now, and I yearn for it.  Heck, I'm willing to have multiple brain surgeries or remove some exceedingly vital organs and live life fully dependent on medications for the chance to find out.

Saturday, April 19, 2014

So, how do you respond to someone's suffering?

I think sometimes we just don't know how to respond when we see someone in pain, having a difficult time, or suffering.  Sometimes we say things that drive a wedge between us when we were trying to be supportive or understanding.  Sometimes we increase someone's load instead of lightening it.  I believe there are people who just avoid it (and others in this situation) completely because while they may care, they don't want to do the above.  Well, I've been introduced to some really neat videos that will help you know how to respond and how not to respond.  There is no set of phrases to follow that will always work, each person and situation is different, but it is certainly a lesson worth learning to all who wish to be a support to those around them.

So, first I'll share a short video about the differences between empathy and sympathy, the first forming closer bonds, the second driving people apart:
 
Makes sense, right?
 
Now a longer video (that includes the above) that discusses our need to place blame...and that it stems from a place of desire to control.  It is sometimes easier to blame ourselves or others than to allow that there is no one to blame --this suffering just is what it is:
 
 
And this one is actually my favorite.  If you cannot bring yourself to watch them all, pick this one!  Here she shares her story of how she as a researcher had a breakdown, and learned the power of vulnerability...and with a fair dose of humor, to boot.  This might be a bit off the topic of the post, but it is SO worth the time to listen:
 
 
 
Lastly, another article that will help give a clear boundary guideline for when it is okay to share our own hurts, struggles and hardships, and when it is better for us to simply be a support to help lift someone up.  It's actually an article called "How not to say the wrong thing" from the LA times, and it teaches a very simple principle.  It is also certainly worth a few minutes (below picture from the linked article).



Friday, April 18, 2014

Community support systems and a lack of endo's

An article from May 2011's Endocrine Today explains that there is an endocrinologist shortage that only looks to be worsening.  It explains that this shortage is causing higher patient numbers per doctor, longer waits, shorter appointment times, a drop in the number of endocrine researchers, and an overall drop in the quality of patient care due to those and other related factors. 

In my previous post, Quack, quack, quack!  Pretendo's and Endiot's , I wrote:

"According to Corcept Pharmaceuticals (a company that produces one of the few medications used as a treatment for Cushing's Syndrome, and has set up informational resources for Cushing's patients): there are approximately 5,000 endocrinologists in the Unites States, and of that number, only roughly 600 of them have seen a patient with Cushing's.  And let me tell you, one patient (or even 10 or 20) does not an expert make!  Chances are good that FAR more endocrinologists have seen a patient suffering form Cushing's, but they likely didn't recognize the disease and thus the patient went undiagnosed or misdiagnosed.  Studies are coming out suggesting that this disease isn't as rare as it is reported to be, but is more likely rarely-diagnosed."

When the statistics from the above article from Endocrine Today, are applied to Corcept Pharmaceutical's number break down for Cushing's, things actually are even worse because the majority of those 5,000-6,000 endocrinologists work for the FDA, pharmaceutical companies, in research, and roughly 1/3rd are in academics.  Those may be worthwhile placements, but it leaves very few that see patients in a clinical setting.  By Andrew F. Stewart, M.D.’s estimates, there really are only about 1,000 board-certified endocrinologists serving roughly 6,000 US hospitals.  That is one endo for every 6 hospitals!  And considering that larger hospitals generally have multiple endocrinologists in their department, I would imagine that leaves a great many hospitals without any endocrinologists at all.  If we assume the 600 that have seen a Cushing's patient are spread evenly over the full 6,000 endocrinologists that also work for the FDA, pharmaceutical companies, in research and in academics (I personally know of some in each of those setting, so while it isn't a perfect estimate, it could be close enough), that only leaves roughly 60 endocrinologists in the clinical setting that have seen (and recognized) a Cushing's patient.  60!?  That's one per state!  Anyone else shocked that most of us travel cross-country to see a tiny handful of experts?  If we were to specify cyclical cushing's, the numbers would be abysmal, and the current "handful of experts" would be limited to one.

He goes on to explain some reasons why the shortage exists and some suggested ways to make improvements.  In among the suggestions was the statement that "Community-based support systems must play a larger role."  I would like to publicly thank my online Cushing's community, along with the Addison's community.  I would not have seen my endocrinologist without the help of the Addison's community.  I had eventually recognized my lows for what they were, an adrenal imbalance, and they sent me to the best doctor they could have...an expert in pituitary disorders and the hard-to-diagnose.  The ONE cyclic expert (No, he isn't a quack, he's crazy-qualified with a CV of Gold!) who figured out my "lows" were actually a by-product of my "highs."  They helped me in a time of need and got me where I needed to be.   

At that point I went all-in with the Cushing's community, and they've helped me in ways that words just cannot do justice to, but I'll give it a try anyway...They've helped me learn the ins and outs of this disease, the myriad of confusing and changing symptoms, the long and repetitive road of testing, diagnosis, long-term recovery (and life after Cushing's), post-op, surgery, helped me find the best doctors, etc., etc. And just as importantly, they've helped me to know I'm not alone and supported me every step of the way.  They've cried with me on my bad days, helped me trouble shoot when I've run into road blocks, and they've rejoiced with me on good days.  They've even helped me to laugh at my awful situation and symptoms (If it weren't so funny, it would be sad!).  I swear they often are more upset than I am when I have hardships and are also more excited than I am when things go well!  Britton was amazed at the number of Cushing's friends texting, calling, emailing, posting and PMing from all over the world when I had my pituitary surgery.  But what he was most touched by was the genuine concern, interest and love he felt coming from them.  In many ways I owe my life to this online community as much or more than I do the doctors who are diagnosing and treating me.  I hope that in my efforts to reciprocate, I can pay it forward and bring this same level of help, education and support to others who are suffering and those who follow in my footsteps. 

Thank you!