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!

 

 

 

 

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

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!).

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.

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.

 

 

 

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.

 

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!!!

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.  ;)

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!).

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

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.

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).

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!

Dr. Ludlam Video

A video presentation by Dr. Ludlam (then head of the Swedish Pituitary Center) in 2009 full of lots of great information:  http://videomedia2.swedish.org/mediasite/Viewer/?peid=39242af8fe64424085947b98913661f1  It is DEFINITELY worth watching, especially if you don't yet know much about Cushing's, symptoms, testing, types, surgery and recurrence.

This is not my endocrinologist, but they are similar in their diagnosis strategies and philosophies from what I can tell.  No wonder so many were saddened by his loss when Big Pharma stole him away!  Luckily, I will be able to hear him speak in-person while at the Magic Foundation's Annual Cushing's/AGHD/hypo-pit Convention in just a few more days.

Fat bias and being the bigger person

I woke up this morning, earlier than I'd like to, and couldn't stop thinking while I lay there trying to get back to sleep.  What was on my mind first thing in the morning?  Something I've been avoiding thinking about for a while...Traveling alone on my upcoming trip, and doing so with the help of wheelchairs at airports.

You see, there is a fat bias in our society, even fat shaming.  If you are sick, it is because you are fat.  If you are fat, it is because you are lazy and overeat (uneducated, ignorant, gluttonous, eat unhealthy foods, won't exercise, "let yourself go", have no respect for yourself, are selfish --take your pick).  It couldn't be that your illness, which you couldn't have avoided, directly CAUSED your weight gain and energy loss!  Oh, no!  Granted, few know about this disease, but still the judgment hurts and really, should we be making these kinds of judgments about anyone?  How sad that it's the norm for us to think of and treat others that way, regardless of the cause.  I have a friend in a community that treats being overweight like it would leprosy --it's contagious, so openly shame and avoid those people!  Some of the most awesome and amazing people I know are fat.  Some of the most vile and despicable people I've met are skinny (and oft-times despite horrible diet and exercise habits).  Have we completely forgotten that it's what's on the inside that count's?

So, like I said, I'm travelling alone.  I have zero energy.  I also happen to be quite obese for my short size.  I cannot change either at the present, and I know because I've had the brain surgery to try to fix the cause, along with years of trying diets, lifestyle changes, exercise...everything that "they" say would work.  It doesn't.  I can't override a tumor sending hormones to EVERY cell in my body.  I can only hope to remove it and it's insidious signals that are destroying my life. 

As I ride through the airport in a wheelchair because I cannot walk for long distances or stand in a line for very long (like at the TSA check point), I am overly aware of the looks I get, some quite nasty, that even have come from the person driving the wheelchair.  They don't know that I have a tumor, or that I had brain surgery 2 months ago.  They don't know that my body has literally been eating it's own muscles and bones.  What's worse is that even if they knew, experience tells me they won't care. 

What if I want to get something from the food court before my flight leaves?  Will they wheel me there?  What if I want a pedicure at the airport while I wait?  Is that too petty to warrant a stop?  Will I have to walk the rest of the way then?  Honestly, I feel awful that I'd even have to ask.  Will people glare at me even more because I was wheeled to the gate entrance, but later walked to the restroom and back on my own without any outward sign of disability?  Yes, they do that too.  Heck, even the DAY I was released from the hospital after having my surgery, I rode in a little handicap cart because we needed to buy food (and Gatorade!) and I didn't want to sit in a hot car alone.  People gave me nasty looks then too (not all, obviously, but it's hard to ignore the ones that do).  If I'd had the energy, I'd have happily told them to shove it, I'd had brain surgery 3 days before, but again, they wouldn't have cared and I'd have just wasted more of my precious energy (that's not figurative!). 

And worse yet, I know that by saying all this it will still get put back on me...because that is what always happens.  I get told that *I* need to be the bigger person.  (Ha ha!  Is that a pun?  Ugh.)  That I need to stop caring what others think of me.  That I need to be happy and friendly despite people that don't even know me treating me so awfully, everywhere I go.  And that I need to expend the extra energy to prove them wrong.  Somehow fixing/changing it is all on me.  Well, you know what, I do NOT have the energy for any of that.  That is not an understatement or me being lazy.  I.DO.NOT.  So, for once judgmental people, YOU be the bigger person. 

Letters to family and friends explaining Cushing's

In this link there are examples of two letters that people have written to explain Cushing's to their families and friends, along with a news interview of someone with Cushing's.  We all know Cushing's is hard to understand, but hopefully spreading the word and educating can start to help with that:  http://www.cushings-help.com/family-letter.htm

What not to say to a Cushie:

What not to say to a Cushie:...Or anyone else, really, cause it's just rude!  Imagine saying this to someone with MS?  Or Cancer?  Just because our illness involves a loss of energy and massive weight gain, does not make it okay.
You think you're tired? OMG I have travelled so much during the last month, I can barely see straight!

You are lucky! I wish I could get that much time off work!

You could stand to gain a few pounds anyway...

I bet you'll feel a lot better if you just go for a little run. Just force yourself to get back out there.

My Dentist: "So what are the symptoms of Cushings anyway?" I explain weakness, tiredness, etc. The Dentist and hygienist laugh and both say maybe they have it too, then.

So do you think you got this because you are a workaholic?

Coworkers ask if I want to do Zumba. (I guess they really don't understand how I feel...I can barely walk upstairs).

"When are you due?" My answer is always three months.

Why don't you try Weight Watchers? That always works for me when I need to lose those extra 5 pounds after Christmas...

In a Christmas card this year: "If you have your health, you have everything!" Gee, thanks for letting me know I have nothing!

Just decide to be happy/healthy/stress-free.

You'd gain muscle strength if you'd do more.

Maybe you should start taking more Vitamin C, Zinc, etc....

If you exercised, you'd have MORE energy.

Have you tried a juice fast?

How often do you eat at McDonalds? It could be fatty liver disease.

Well, if I stopped exercising, I'd gain weight too!

How much of you doing nothing is necessary, and how much is you just trying to conserve energy?

You'd be able to sleep at night if you just learned to shut off your brain.

Well, that's a relief! The endocrinologist said there's nothing wrong with you!

If there was something wrong, it would have showed up on a test or MRI by now....

It's not good for you to stay stuck in the house all the time. You need to try and keep up your regular routine. (This from an endocrinologist)

Well, at least it's not cancer! (Or ebola/ flesh eating disease/ what I had 10 years ago)

When you hear hoof beats...think horses, not zebras.

It's just low Vitamin D.

You must be depressed.
"Don't you get bored sitting at home all day, not working or going anywhere?"  As if I'm actually sitting at home all day doing nothing...

Oh trust me, I know all about hormone problems. Last week I had THE WORST PMS!!!

After I explained to a coworker about Cushings, she said to me: "My niece has weight problems so she went to an endocrinologist and after she did some tests to see if it was a hormone problem, which it wasn't, she did some diet and exercise and it worked! You should do the same." I just turned my back to her.

Have you tried walking?

When explaining why surgery didn't bring a cure: "Doctor's just tell you the worst-case scenario.  Stop being so negative!"

(These posts are shared with permission of the original poster's, from a Cushing's support group, edited by me for typos and clarity.)

How many spoons do YOU have?

I know I've linked to the spoon theory before, but it is such a good analogy and so fitting, I think it is worth another look.  This time I was brave enough to go through my day's routine and come up with a basic idea of how many spoons certain tasks require, and come up with an idea of how many spoons I actually have right now.  Every time my number of spoons drops significantly as my disease progresses, it is shocking and unbelievable.  Sometimes it is gradual (because I do have more spoons in a high than I do in a low) and less noticeable, but there have been a handful of times when my spoons just disappeared and I hit a new "baseline" and had to re-adjust my days to what I am capable of.  I'm leery to post this because of the attacks and lack of help we have received in the past, but I think people who are learning and suffering need to know they are not alone, and also, be spurred on to action now.  You need to fight now for that diagnosis and treatment that you need, or you will start to lose your ability to do so.


I'd say this week I've had roughly 20-30 spoons per day. Hard to know exactly since every day is different both in the number of spoons I have and the number of spoons I need.  I generally don't know upon waking that I have more or less, I just find out when I run out.  It doesn't hit you gradually, either you have the spoons required or you don't, and when you don't, you're done.  Resting doesn't bring more, and using more today does not equate to building up stamina giving me more tomorrow.  That is something few understand.  That system in my body is broken right now.  Every system is, really.  Overdoing it can lead to the need for strong medications, hospitalization, or injury and illness.  Actually, it will.

I can count spoons really easily now that I have so few!  So here goes:
1 spoon to get out of bed
1 spoon to pick out my clothes
1 spoon to use the restroom and put on deodorant in the morning.
1 spoon to brush my teeth and comb my hair.
1 spoon to weigh myself and note my symptoms (I track them) for the last day/this morning.
2 spoons to actually dress
1 spoon for each time I go to the bathroom (and then back to my chair) throughout the day
2 spoons for each time I have to correct, reprimand (yell at! lol) a kid
5 spoons to add wood to the wood stove
2 spoons for every time someone complains to me about their life and stressors
10 spoons if someone is confrontational or rude to me
5 spoons extra if it is a hot day, drawn right off the bat, and probably 5 more for every 2 hours I sweat after that
10-15 spoons to shower or bathe, and another 10-15 spoons to dry off/get dressed afterwards
5 spoons to get myself a snack (without cooking/prep involved)
15 spoons to make food that involves cooking at all or prep time (ie, preheating the oven, boiling water, etc. not "cooking from scratch") 
5 spoons to help a kid get dressed, with shoes and coat (I have 6, but not all need help)
5 spoons to walk to the car
5 spoons to pick up some extra garbage or dirty clothes as I walk to the bathroom (added to the 5 spoons for going to the loo)
5 spoons to undress and get ready for bed
5 spoons to socialize with others (it takes energy!)
10 spoons to play a video game that is timed (So I just can't/don't)
10 spoons for looking at bills (for the stress/anxiety it brings, along with the confusion of hospital billing and insurance) 
2 spoons for answering the phone each time it rings (I don't know who is calling and I am not a phone talker. Britton and mom have their own ring tones on my cell, which helps)
5-20 spoons for someone coming over to visit my house
10 spoons if I let myself think about how we will manage another surgery recovery (if surgery actually works this time)

So, now start to count up your day (and healthy people do FAR more than this because I did far more than this) and when you hit 25 spoons, stop.  See how far you've made it today.  What happens for the rest of the day?  I am not able to nap or just "do nothing" for the rest of the day.  Did you notice I don't have any "fun" activities on there?  I don't usually get to do those either, despite having many interests and abilities, because the choice has already been made that family come first and I do not have the energy for extra.  I wish I did, but you do what you have to do to survive.  Right now my creative outlet is in the form of online posts and energy conservation.  lol  And my method of serving and interacting with others is also on the internet.  I have a continual stream of new "friends" on facebook, Cushies, who are looking for support and information.  And I LOVE to help and teach others.  In real life, I only see two adults: my husband and my mother, with the exception of when I leave the home (almost exclusively for church, about 2-3/4 Sundays a month --but getting to church and being there costs me about 50 spoons, so you do THAT math).  I don't really get visitors, and that is just as well, because I don't have the energy for that either even though I miss having friends and doing fun things.  Heck, I miss cooking and cleaning! 

I know it is hard to understand, and people really cannot fathom it because it is so foreign to what they know and their norm, but I hope this has helped explain some of what it is like on a daily basis. 

What it feels like to have Cushing's

I figure this is a good "object lesson" idea for helping people understand more than just the loss of energy we are dealing with (ie, spoon theory). This barely scratches the surface, too:

High blood pressure, diabetes, premature aging, anxiety, chronic pain, depression, loss of circadian rhythm (ie, awake all night, asleep all day), bipolar disorder, central obesity, osteoporosis, hypothyroidism, anemia, electrolyte imbalance, easy bruising, chronic fatigue syndrome, loss of sex drive, arthritis, sleep apnea, acne, insulin resistance, acanthosis nigricans, speech problems (poor word recall, stutter, etc), memory loss, PCOS, eczema, OCD, thinning skin, vitamin D deficiency, immune suppression, edema, hair loss, hirsutism, severe proximal myopathy, adrenal insufficiency (in a low), hypokalemia, eye myopathy, sweating profusely, poor wound healing, moon facies, exercise intolerance, infertility, hypopituitarism, etc...

If you know how hard any of these are individually on a person, perhaps this list of common illnesses associated with Cushing's Disease will help give a glimpse into the struggles "Cushies" live with on a daily basis. We don't want sympathy but we would like those around us to be more understanding of our struggles and limitations.

Cushing's Onset Pictures -side by side

These are pictures showing before onset, and after onset of Cushing's Disease (roughly 17 years ago) along with a picture taken the day of my diagnosis appointment on the LA piers.  Hopefully I can give you an "After Cushing's" picture in another year or two as well.

Quack, quack, quack! Pretendo's and Endiot's

It is the norm among Cushing's Disease sufferers to have gone to multiple doctors and received little to no help, often even being put down by them.  It can be so discouraging to feel like you are up against a wall, especially when you barely have enough emotional and physical energy to get through the day.  You know you're ill, and you know it isn't as simple as diet and exercise because you were getting continually (and drastically) worse despite keeping up on your routine, or despite making drastic improvements.  You don't care what your diagnosis is as long as it is accurate, but you CERTAINLY are seeking one.  You're sick and you want to get better!  Who wouldn't?  (See my previous post "Diagnosis Seeking" for more on that topic, and lack of logic condemning it.)

Some doctors will run many tests trying to help, but don't really know how to figure out the vast mess of symptoms and signs that come with Cushing's, or really don't know enough about how and when to test or how to interpret the results.  Some refuse to help at all, blaming everything on a lack of exercise and poor diet.  Even endocrinologists, doctors of internal medicine who have then gone for further training in the endocrine system, are rarely up to the task.  We even have nick-names for the worst of them, calling them "Pretend-o's" and "Endi-ots."

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. 

For example, according to the April 2005 edition of Endocrine News (in "Unlocking the Mysteries of Cushing's Syndrome"), 2-4 % of all patients with type II Diabetes have Cushing's.  There is a statistically significant number of patients with uncontrollable high blood pressure that have Cushing's Disease as well.  That is a fairly large number of people who are suffering needlessly, being treated only for specific symptoms without the cause of their illness(es) being addressed.  The longer the wait, the more damage is done to their body, often permanent damage.  I pray they get a diagnosis before it is too late.  Please help us spread the word and help us save lives!

Quality of Life and Cushing's Disease

In a retrospective study of the Quality of Life for Cushing's Patients there is a graph showing the quality of life for those suffering from Cushing's Disease compared to the general population, to people suffering from MS, or to people suffering from various kinds of cancer: http://eje-online.org/content/167/3/311.long  The whole article is worth reading (not if you're depressed about your disease at this time, though --wait till you're feeling better) as it talks about what things do and don't improve with remission, mortality rates, etc.

BUT, this is what I really wanted to point out:

I find it interesting that Cushing's Disease is as bad or worse than cancer in quality of life.  A friend says that comparing diseases is like comparing poop --it's all nasty.  And that is very much true, but cancer is a good reference point because so many are familiar with what cancer is, has lost someone to cancer, and overall people understand the suffering cancer patients go through.  So when we say our quality of life is like that of a cancer patient, it is easier to imagine and understand.  Many don't realize how devastating this disease is.  It effects over 70% of our body's functions (including our ability to think, remember, and our emotional reactions).  Sadly, there is just not enough awareness out there...and many treat us as if we are just fat and lazy, need to stop being so negative and whiney, and that we're just deciding to feel bad instead of actually being VERY ill.  We often have to endure rude comments from friends and family, even doctors who should know better, and are frequently left without support in times of great need.  I hope in the future there will be better recognition, testing, and treatment...and most especially, that there will be greater awareness among the general population, so that others won't have to suffer as much due to this disease's affects as we still do at this time.

Distinguishing cortisol highs and lows in Cyclic/Episodic Cushing's Disease

Cyclic Symptoms: (These are ones that I have experienced, and everyone's "cluster" of symptoms for highs and lows are a bit different, but many of these are typical.)

Weight gain
High - Gain ½-2 lbs per day generally
Low – Lose roughly ¼-1 lb per day

Bowel movements
High - Constipation
Low – Normal to loose bowels

Urination
High – Frequent urination , higher volume, waking at night to go
Low – Less frequent urination, lower volume

Sleep patterns
High – Wide awake late at night (1am+), difficulty staying asleep/falling asleep
Low – Easily fall asleep by 10pm, less waking at night

Emotional state
High – Generally better mood overall
Low – Inability to cope with normal circumstances –easy to anger or become tearful

Acne
High – Lots of deep, painful acne, generally appearing on back, arms, legs, face, buttocks, chest, etc. Low – A few shallow pimples if any, generally on the face  Bruising
High – Easy, deep bruising without known cause (can take weeks to fade)
Low – Usually no new bruises, or small, known cause and quick healing (days)

Aches/Pains
High – Very few aches/pains, and only with a known cause
Low – Frequent, generalized aches and pains, headaches

Temperature
High – Profuse sweating, always hot (regardless of temperature), flushed face
Low – Far less extreme, though still generally on the warm side

Muscle weakness
High – Generally lessening strength, but not noticeable in a high cycle
Low – Weak, shaky muscles with difficulty walking or lifting very light objects

Dyshidrotic eczema
High – remission in a high cycle, but usually have residual peeling skin for roughly a week where the eczema had been located
Low – Patches of eczema appear on my fingers, palms and feet, often itchy and sometimes bordering on painful (if large in size)  





 

Symptoms of Cushing's

Rather than reinventing what would be a long symptom's list, I'm going to post a link to a very good list of symptoms that has already been made:

http://cushieblog.com/2014/04/03/cushings-awareness-challenge-day-3-symptoms/

Treatment for Cushing's

There are various methods of treatment for endogenous ("in the body") Cushing's Syndrome/Disease, but the primary method is always surgery to remove the cause: the tumor tissue.

Ectopic disease can originate anywhere in the body, but it is generally found in the lung tissue.  The method of treatment and the surgery performed varies by location.  This type is frequently cancerous, but is the rarest of the rare, and very few (VERY FEW) have this form of Cushing's. 

With adrenal disease, the surgery will consist of an experienced surgeon removing one or both adrenal glands (unilateral- or bilateral- adrenalectomy, respectively).  This is generally done laparoscopically through the abdomen, as the adrenals are located atop each kidney.  Adrenal Cushing's Syndrome is the second most common form of endogenous Cushing's, but it is a very late second (which will become clear below).

In Cushing's Disease, which is pituitary in origin, surgery to remove the tumor is preformed by endo-nasal or transphenoidal surgery (meaning, they use small instruments and go through your nose and sinuses to access the pituitary at the base of the brain).  This is the most common form of Cushing's, with something like 80-90% of endogenous disease being pituitary in origin. 

Surgery recovery and remission/cure will be addressed in another post.

When surgery isn't effective in bringing lasting remission (or fails), or when surgery isn't an option for whatever reason, other methods of treatment can be explored.

One such option is medical therapy, wherein the patient takes medications to lessen or, hopefully, stop the effects of Cushing's Disease/Syndrome.  There are a few medications on the market, but they all have some pretty nasty side-effects, some can cause permanent damage to organs, are generally costly (anywhere from $200/month up to $15,000/month), and are not a permanent option, though they can buy you some time and help reverse some effects of this illness to make you a better candidate for surgery.

A second non-surgical option is radiation.  There are different types used, but the basic gist is that certain parameters must be met to be a candidate, and while this form of treatment can bring a permanent cure, it too comes with risks.  Frequently the radiation damages the organ (such as pituitary) along with the tumor cells, which may lessen or stop production of certain essential hormones, requiring replacement through medication.  Also, there is generally a long wait for the treatment to take full effect, anywhere from 1-5 years, during which time the patient usually is on medical therapy.

I would also like to address failed surgery or recurrence of Cushing's Disease (pituitary).  When Cushing's Disease is back after a first surgery (or second, etc), generally the endocrinologist and neurosurgeon look for a "target" on MRI.  If a target (suspected tumor) is found, the most likely treatment is generally repeat pituitary surgery, though in some cases medication is used for a while or radiation is tried.  There are increased risks to second (and later) pituitary surgeries, and the statistical cure rate also drops, so sometimes medication or radiation therapy are preferred instead. 

If a target is not found, the patient can agree to "exploratory" surgery hoping one is found, partial pituitary removal, or complete pituitary removal.  These can be reasonable options, and each person and medical team must weigh the benefits and risks for each individual case and circumstance.  But frequently when a surgical target is not found, the patient is either put on medication, sent for radiation therapy, or (if it is a slow-growing tumor, hyperplasia, etc) the patient may undergo Bilateral Adrenalectomy. 

Bilateral Adrenalectomy (BLA) as a course of action following failed surgery or recurrent disease is becoming more common.  Cushing's is a very drastic disease that has an average mortality of 5 years untreated...the patients will continue to get sicker and sicker, and eventually die from it's effects.  Many are already quite ill at the time of their first diagnosis.  Sometimes drastic disease requires drastic actions.  Removing the adrenal glands will not stop the signal from the tumor tissue calling for the production of more cortisol, but it will stop the reception of that signal (ie, there are no longer adrenal glands to receive the ACTH signal and produce excess cortisol on-demand).  This will leave the patient dependent on replacement hormones for life (a state closely related to Addison's Disease) as cortisol is a necessary hormone, but it is in essence trading an untreatable disease for a treatable one.  Many BLA-ers as we call them, after adjusting to their new routines and allowing their body time to heal, actually begin to see marked improvement in their quality of life and symptomology.  It is still a delicate balance, as their body cannot produce two necessary hormones, and comes with many risks, but with careful planning, education of the patient (family, friends and medical personnel too), and quality medical care, a long, active life is definitely possible.

Personally, I am still awaiting further testing to confirm that I am not in remission, and a pituitary MRI and follow-up with my neurosurgeon in about 3 weeks will tell us whether a second pituitary surgery or BLA will be the next step for me (if I am indeed not in remission). While I would prefer another pituitary surgery that led to remission/cure without pituitary damage, the risks of pituitary damage from repeat surgery (along with CSF leaks, etc) are very likely.  So it IS likely that hormone replacement post-op will be necessary, and the pituitary makes a large number of necessary hormones.  I would prefer to replace two hormones from adrenalectomy over 6+ hormones from pituitary damage.  The balancing required can be quite confusing, medications costly, etc.  While the threat of adrenal crisis is real, if a BLA is in my future, we have decided as a couple that even if I were to die in 5 or 10 years from such an event, we would prefer I have 5-10 good years where I could be actively involved and enjoying family life (with BLA) than to spend 5-10 years becoming bed-bound, increasingly ill, miserable, and dying from this disease.  It is a tough decision, and even though I haven't hit that stage yet, I am sometimes hit with a very real fear of what such a decision would mean for the long-term.  Would it be worth it to be "healthy?"  Absolutely.