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!!!