How Good is Your Safety Net: Join your Local Cushie Group

Having Cushing's often feels like we are free-falling.
Join your local Cushing's group and grab an arm or leg. Hang on forever.

How good is your safety net? If you have an adrenal crisis, do you have friends who can support you? The best way to tighten our collective safety nets as Cushies is to get to know other Cushies. Nearby Cushies are even better. I whole-heartedly encourage each of you to connect with Cushing's patients in your area. 

I realized lately that many newer Cushies don't know that we have Cushie groups set up for every US state. I'm sharing my post announcing this back in February 2018.

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US STATE GROUPS: Tonight I completed a dream project to create a state cushie group for 49 of 50 US states. 

So I took my own advice and I created a state cushie group for 49 of 50 states. Please join your group. Go to search box in Facebook and search for your home state plus the word Cushies, like this: Texas Cushies. Montana Cushies. Florida Cushies. Each of us Cushies has the unique ability to help other Cushies, no matter where in this big world you are and they are. I am asking for your help in connecting Cushies.

As we see new people come into the groups or someone is frustrated over an uncooperative doctor who is unwilling to test more, prescribe more HC post op, or consider reoccurrence, please look at their profile to see if they list a location where they live. Then reply to them and post: Please join @ sign then group name, like @Colorado Cushies, to activate a link to that group. You can be the person who makes the difference for someone fighting to find answers.

We also have regional groups called Midwest Cushies, New England Cushies, Southern Cushies, Pacific NW Cushies, and Central Texas Cushies. We even have enough patients to create city-specific groups like DFW Cushies (Dallas-Fort Worth Cushies) and Houston Cushing's Support Group. If you are willing to travel for good health care, be sure to join.

I am active in the DFW Cushies group, and these ladies are a life-line for me. We have gotten together a half dozen times, and each has brought me closer to people who understand what Life is like as a Cushie. We understand the lingo, the complications, the horrible symptoms, the weaning, the surgeries, the doctors!, and the struggles. Meet regularly. Help locally. Then those groups can organize events, prep people for surgery, meet with families, go to doctor appointments... you name it. We can help push someone to their next step to wellness, and we can be there when someone really needs someone to listen. This type of unwavering support should not be underestimated. We all need it. We deserve it. We shall have it. Bring the Cushing's experience from the Facebook groups into real life and make real friends and lasting friendships. We are all out there, and all you have to do is find us. Go to a gathering even though your heart is beating out of your chest and the doubt and fear of judgment about your looks makes you want to flake out and stay at home. Go. I wish for you the lasting friendships I have made in my local area.

We must create a safety net ourselves. Even though the medical community continues to fail us and this disease's aftermath plagues us, we can cling tight to each other and keep each other safe.

Please take a pledge and commit to helping me funnel Cushies to their state groups.

Now, I move on to the other 100 Cushing's projects I have my heart set on to complete.

Be well.

Moxie

P. S. Please be sure to like my Fight Cushing's with Moxie page on Facebook, where I will post every time I update this blog.

P. S. S. If I have helped you move forward on your path to better health by answering questions or you have benefit from the material I have written, please consider leaving a review on my Fight Cushing's with Moxie page on Facebook. Those reviews are the best thanks for the almost 11 years of maintaining this blog and being a Cushing's advocate. It will keep me going!!

My Very Own "CSI: Cyclical Cushing's"

HAS AN ENDOCRINOLOGIST EVER TOLD YOU THAT CYCLICAL CUSHING'S WAS NEW OR RARE?

For the past six years or 2,190 days, my battle with Cushing's has been a lopsided fight. As a new patient, I went in totally unarmed, with only my patient history, medical knowledge, lab protocals, medical literature, etc. We all know, facts, stories, labs, and witnesses weren't enough to corroborate this bizarre tale of strange physical deformities and an emotional roller coaster ride.  We were completely unarmed against the MDs who stood before us.  

I have spent my time in the chat room trenches with our Cushie platoon, breaking down all the information, developing hypotheses, discussing theories, and offering help. It's very difficult to move beyond that when we, the patients, have critical information about our peers yet it goes unpublished by our doctor friends in the endocrinology.
For example, many endocrinologists I know tell me that cyclical Cushing's is very rare, fairly new, and poorly defined. Cyclical Cushing's is not well understood in the medical literature. Although I respond with a "Well, we have a lot of friends on the message boards with Cyclical Cushing's, so that doesn't do much good for me," it is undoubtedly a conversation killer. My having no come back to that questionable remark about the RARITY of Cyclical Cushing's has always bothered me to the core.

Well, I've complained about that before, but TODAY what is chapping my hide is this article I found after a simple Google search for the keywords Cyclical Cushing's.
First, you will read in the abstract that more than five cases were cyclical. By the end, there are more.


Second, I was really beside myself in a FIT when I reviewed the dates for these articles in the Selected References.  I will highlight some below. You will see some medical journal articles cited from 1956 and later.  Cyclical Cushing's has been studied and written on 50 years before I sought diagnosis and treatment in 2007. Why did these endocrinologist bozos act like they didn't know anything about cyclical Cushing's? What are these teachers teaching these endocrinologists in medical school? Why did they all treat me like there was just NO POSSIBLE WAY I had cyclical Cushing's and thereby must just be depressed, fat, extra body-haired, bruised, forgetful but rageful person. I am so furious that I always pressed for more information about cyclical Cushing's, and few had a comprehensive answer explaining what we DO know about the matter.

Please take the time to read this article below, as my flabbergasted mind and I sit here wondering how these countless endiots could not remember even ONE of the 13 articles referenced in the article alone that studies fluctuating or cyclical cortisol when I questioned them about cyclical Cushing's.


Another choice bit: the referenced articles below were published in 1956, 1958, 1961, 1973, 1975, 1976 (2), 1979 (3), 1980, 1982, and 1985. Any one of these show researchers had already discovered the distinct subset of cyclical Cushing's patients and tried to determine the best way to diagnose and treat patients.  From what I have seen 30 to 55 years later to the present, it seems that garden-variety endocrinologists and even well renowned pituitary centers have been riding on the diabetes bus (obesity! heart attacks! exercise! insulin!) all the while totally neglecting data that others clearly have shown us so long before. 


Does anyone coming out of medical school know how to research and prepare a proper literature review? Do they just ignore the clankiness of old articles and focus on single-hospital data studies?  I am mean, come on, people!  Do I have to do everything?  


When I cool down, I'll begin to read the articles post below (with links). I am truly interested to figure out who knew what and when.  This is my own CSI: Cyclical Cushing's.   

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PMCID: PMC1418075

Five cases of cyclical Cushing's syndrome.
A B Atkinson, A L Kennedy, D J Carson, D R Hadden, J A Weaver, and B Sheridan


British Medical Journal (Clinical Research Ed) . 1985 November 23; 291(6507): 1453–1457.

Abstract

Reported cases of cyclical Cushing's syndrome are rare. Of 14 successive patients with Cushing's syndrome nine collected sequential urine samples for the estimation of cortisol:creatinine ratio. Five had cyclical Cushing's syndrome while two had considerable variation in urinary cortisol excretion without a cyclical pattern being established. Two of the five patients with a cyclical syndrome had paradoxical responses to dexamethasone. In only one patient with a cyclical pattern did the cortisol:creatinine ratio fall after treatment with bromocriptine or cyproheptadine, or both. The high incidence of the cyclical form of Cushing's syndrome has important clinical implications. A high index of suspicion of the syndrome is required in patients with symptoms or signs of Cushing's syndrome but with normal cortisol values, in patients with fluctuating cortisol values, and in patients with anomalous responses to dexamethasone. Because of possible variations in steroidogenesis the results of drug studies in Cushing's syndrome must be interpreted cautiously.

Full text

Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article(989K), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References.

Selected References
These references are in PubMed. This may not be the complete list of references from this article.

• Bailey RE. Periodic hormonogenesis--a new phenomenon. Periodicity in function of a hormone-producing tumor in man. J Clin Endocrinol Metab. 1971 Mar;32(3):317–327.[PubMed]

• Brown RD, Van Loon GR, Orth DN, Liddle GW. Cushing's disease with periodic hormonogenesis: one explanation for paradoxical response to dexamethasone. J Clin Endocrinol Metab. 1973 Mar;36(3):445–451. [PubMed]

• BIRKE G, DICZFALUSY E. Fluctuation in the excretion of adrenocortical steroids in a case of Cushing's syndrome. J Clin Endocrinol Metab. 1956 Feb;16(2):286–290. [PubMed]

• Chajek T, Romanoff H. Cushing syndrome with cyclical edema and periodic secretion of corticosteroids. Arch Intern Med. 1976 Apr;136(4):441–443. [PubMed]

• Liberman B, Wajchenberg BL, Tambascia MA, Mesquita CH. Periodic remission in Cushing's disease with paradoxical dexamethasone response: an expression of periodic hormonogenesis.J Clin Endocrinol Metab. 1976 Oct;43(4):913–918. [PubMed]

• Oates TW, McCourt JP, Friedman WA, Agee OF, Rhoton AL, Thomas WC., Jr Cushing's disease with cyclic hormonogenesis and diabetes insipidus. Neurosurgery. 1979 Nov;5(5):598–603. [PubMed]

• Cook DM, Kendall JW, Jordan R. Cushing syndrome: current concepts of diagnosis and therapy. West J Med. 1980 Feb;132(2):111–122. [PMC free article] [PubMed]

• Schteingart DE, McKenzie AK. Twelve-hour cycles of adrenocorticotropin and cortisol secretion in Cushing's disease. J Clin Endocrinol Metab. 1980 Nov;51(5):1195–1198.[PubMed]

• Jordan RM, Ramos-Gabatin A, Kendall JW, Gaudette D, Walls RC. Dynamics of adrenocorticotropin (ACTH) secretion in cyclic Cushing's syndrome: evidence for more than one abnormal ACTH biorhythm. J Clin Endocrinol Metab. 1982 Sep;55(3):531–537. [PubMed]

• Crapo L. Cushing's syndrome: a review of diagnostic tests. Metabolism. 1979 Sep;28(9):955–977. [PubMed]

• Riad-Fahmy D, Read GF, Gaskell SJ, Dyas J, Hindawi R. A simple, direct radioimmunoassay for plasma cortisol, featuring a 125I radioligand and a solid-phase separation technique. Clin Chem. 1979 May;25(5):665–668. [PubMed]

• Cook JG. Factors influencing the assay of creatinine. Ann Clin Biochem. 1975 Nov;12(6):219–232. [PubMed]

• Atkinson AB, Chestnutt A, Crothers E, Woods R, Weaver JA, Kennedy L, Sheridan B. Cyclical Cushing's disease: two distinct rhythms in a patient with a basophil adenoma. J Clin Endocrinol Metab. 1985 Feb;60(2):328–332. [PubMed]

• Scott RS, Espiner EA, Donald RA. Intermittent Cushing's disease with spontaneous remission.Clin Endocrinol (Oxf) 1979 Nov;11(5):561–566. [PubMed]

• PASQUALINI RQ, GUREVICH N. Spontaneous remission in a case of Cushing's syndrome. J Clin Endocrinol Metab. 1956 Mar;16(3):406–411. [PubMed]

• BASSOE HH, EMBERLAND R, STOA KF. Fluctuating steroid excretion in Cushing's syndrome. Acta Endocrinol (Copenh) 1958 Jun;28(2):163–168. [PubMed]

• Aber CP, Cheetham HD. Cyclical Cushing's Syndrome. Br Med J. 1961 Feb 4;1(5222):336–338.[PMC free article] [PubMed]

• Blau N, Miller WE, Miller ER, Jr, Cervi-Skinner SJ. Spontaneous remission of Cushing's syndrome in a patient with an adrenal adenoma. J Clin Endocrinol Metab. 1975 Apr;40(4):659–663. [PubMed]

• Green JR, van't Hoff W. Cushing's syndrome with fluctuation due to adrenal adenoma. J Clin Endocrinol Metab. 1975 Aug;41(2):235–240. [PubMed]

• Bochner F, Burke CJ, Lloyd HM, Nurnberg BI. Intermittent Cushing's disease. Am J Med.1979 Sep;67(3):507–510. [PubMed]

Gambling on a Cushing's Diagnosis: What are the Odds?

A Cushing’s friend of mine recently asked what we can say to our doctors to explain why we think we have Cushing’s. The Cushing’s community knows that the burden of proof falls on our shoulders to prove we have Cushing’s to a doctor, before tests are run and even after positive tests confirm it.  These doctors play judge and we Cushing’s patients must prove beyond a reasonable doubt that we have Cushing’s, often in the hostile setting of doctor’s office after doctor’s office. Seems harsh? Seems unfair?

Doctors are taught in medical school that they are unlikely to see a case of Cushing’s in their entire medical careers.  In fact, doctors admit that Cushing's may be the most difficult diagnosis to make in all of medicine. Sigh. So we walk in, prepared as our Cushie friends have recommended and armed with symptoms list, photo summary, and past lab results. Doctors are still incredibly dismissive and cruel. They blame the patient for their weight gain, regardless of exercise or dietary habits. They routinely dismiss all points the patient makes.  Many doctors eagerly counter every point and spout facts that are incorrect according to published medical literature.

So what are we supposed to do? Give up? No. We keep fighting no matter what.

To that end, I wanted to share the argument I have used for a long time on several occasions. In fact, I just used this during my appointment with a new neurosurgeon this past Friday.

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In statistics, we learn the concept of probability. Probability is the chance that something will happen - how likely it is that some event will happen.  With each variable added, we decrease the probability that each one of those things can occur together at the same time. 

Think of the elusive, rare Cushing's diagnosis as a slot machine with 7 wheels. Each wheel is labeled with nice fruit images and contains one of the following: 

·       all Cushing's symptoms

·       high urine cortisol

·       high midnight cortisol serum

·       high saliva cortisol

·       high ACTH

·       positive IPSS

·       MRI showing pituitary tumor

Now spin the wheel.  For a Cushing’s diagnosis, each wheel must land on the diagnostic criteria for Cushing’s listed above.

What is the likelihood that all of those things are going to "hit" and line up for the jackpot?

It is not probable. In fact, the odds are stacked against you to have all those things line up like they do. For us Cushing's patients, THEY DO LINE UP. WE DO HAVE ALL THOSE THINGS CONFIRMING CUSHING'S. It is a RARE occurrence, and it doesn't happen often, but people do spin the wheels on a slot machine and line up all the wheels to win. For us, of course, Cushing's is a HUGE loss -- no win at all.

Now, let’s take a gander at what the diagnosing slot machine would have to look like for me to get my Cushing’s diagnosis, since my doctors require multiple high test results in each of the testing categories.

To confirm my Cushing's diagnosis, it would take
28 slot machines with 5 wheels and all 140 wheels
landing on Cushing's. Guess what? They did. Lucky me.

·       many Cushing's symptoms (75 wheels for each of my Cushing's symptoms)

·       high urine cortisol (25 wheels for each test)

·       high midnight cortisol serum (25 wheels for each test)

·       high saliva cortisol (7 wheels for each test)

·       high ACTH (4 wheels for each test)

·       positive IPSS (1 wheel)

·       multiple MRIs showing pituitary tumor (3 wheels)

Now then, based on my lab values in each category, for my diagnosis, we have built a slot machine with 140 wheels. Think of how much space this would take up in a Vegas casino!). 

So, let’s imagine me going up to have a seat. 

I pull the arm.

I spin the wheel.

One by one, each wheel lands on the picture showing CUSHING’S.

My Cushing’s diagnosis is confirmed. It is rare, but it is not impossible. Yet, doctors still argue and berate the patient.  You can see why I have very little tolerance for someone who calls my diagnosis, or any Cushie’s diagnosis, into question. It is ludicrous, unfathomable, and unconscionable for any medical professional to doubt us, due to their ignorance, pre-conceived notions, lack of medical curiosity, or their tight schedule that prevents them from listening to us and really understanding our whole history. The way doctors treat Cushing’s patients should be illegal.

My first neurosurgeon told me that in medical school, doctors are taught to find the one disease that explains everything and to not accept many "little" diagnoses strung together. It was imperative to find the diagnosis that captures as many of the symptoms as possible.  I implore the medical community to remember that lesson and do more to help their patients.

There is no doubt why we Cushing’s patients are the determined lot we are. We know our own bodies. It is our unrelenting pursuit for the truth, our unwillingness to give up on ourselves, and our ability to learn about our disease from those who face its challenges every day that enable us to fit all the puzzle-piece-diagnoses together in search for our cure. 

That's why I look at *all* of my friends as Cushie warriors.

A Cushie's Job: Trudging through the Medical Literature

If you have spent any amount of time on this blog, you will know that I am patient advocate who encourages you to be the most informed and self-aware patient you can be.  For us Cushies, especially us cyclical Cushing's patients, it is imperative to be up-to-date on the medical literature.

You also know how difficult that task can be as we fight to keep our energy up for basic daily tasks and chores.  You also remember how Cushing's causes cognitive impairments that just don't give us the right state of mind -- clarity or positivity -- to take on a project this big.

So, I decided to just post stuff here, as I find it.  It may be a repeat (sorry, I probably forgot), or it may be new. Regardless, it will be stuff I am stumbling through as I relearn everything I have to know about Cushing's in order to make my decision.

I am reading through the medical literature for articles like Long-term remission rates after pituitary surgery for Cushing’s disease: the need for long-term surveillance posted by our dear Cushie friend Robin at Survive The Journey.  I am also trying to wade a list of articles my friend Susan recommended about cyclical Cushing's posted on PubMed, the government's database.  These free articles include research done with your tax dollars at the National Institute of Health (NIH). The NIH is considered an authority on Cushing's and even cyclical Cushing's. Based on what I have seen in my five years, the NIH seem to specialize in pediatric Cushing's cases more than adults, i.e. I see more pediatric patients than adult patients accepted for testing and treatment at the NIH.

So, I have to make my way through the medical literature about:

• quality of life for patients after several pituitary surgeries vs. quality of life for those who chose BLA
• remission rates for each type of treatment (3rd pituitary surgery vs BLA)
• enzymes and other hormones produced in the pituitary that may vanish after repeated pituitary surgery
• patient success stories for each

I mean, all this and a bag of chips. EVERYTHING!  I read a lot of this stuff in the beginning. It was "this could happen to me" reading, so the shock resonated longer than the information. Meaning, the scariness stuck when the facts didn't. 

Now, I find my mind so hazy that I just can't read through these medically-rigorous articles anymore. I really struggle.
I can't remember what I read already.
I can't find something I already found before.
I can't even think of good keywords to search.
I've already made a million laps around the internet.

It's a mess. I AM A MESS!

So, being that it is my blog, this is what I'm gonna do.  I plan to "store" articles here that I am reviewing for my own benefit. If you see something that you have never seen before, well, you benefit, too.

HEY!  THEN MAYBE YOU CAN MAKE MY DECISION FOR ME!

I Hate You, Cushing's Disease

Hi everyone. Sorry for such a long absence. In this past, I take time off from writing when I myself am too sick to talk about Cushing's.  When I am exhausted all the days, riddled with headaches, body aches, dizziness, and muscle spasms, I don't feel like the best public speaker for Cushing's. Nor do I have the energy to queue up posts to show up in my absence. Nope. I was too sick for all that.

This absence, like other times, is caused by my own testing, diagnosis, and clearance for third pituitary surgery. That's right. You read that right.

I still have Cushing's disease. 

I still have high cortisol at diagnostic levels on four different diagnostic tests. 

I have a third tumor showing on my pituitary that did not show itself last year at surgery.

I am still fighting Cushing's.  

I hate Cushing's.

When will it ever end?!

High cortisol and high ACTH.
GO AWAY.

Things get really complicated when you are this far into a Cushing's diagnosis. The likelihood of a positive outcome decreases with each stage of treatment. There aren't a lot of people in this world "like you," and even fewer that you may have access to. Nope, for sure, these tiny details make the next decision even more difficult on the patient.


And frankly, when can I cry UNCLE?
When can I say that I've had enough of this beast called Cushing's?
When will this be over?!


I am considering next steps for treatment:  third pituitary surgery vs. bilateral adrenalectomy with risk of developing Nelson's syndrome from an untreated pituitary tumor vs. medical therapy such as Korlym.   Each has its risks and benefits.  Which risk is greatest? Which benefit is greatest? Well, it seems everyone has a different opinion on that.

I fear that the effort to make this decision will exceed the energy I have in a day.  I spent the first 72 hours after learning of the presence of a third pituitary tumor in a tailspin. First, notify the family. Second, notify my Cushies, my besties. Third, reach out to neurosurgeons for second, third, and fourth opinions.  Fourth, reach out to Cushies who have been *here,* who have made this decision, and see if they can tell me something I don't know, something I've forgotten, something I just can't know until I've lived it. Fifth, look around the rest of my life, and do some things that need completing. Things that are super important to me, like completing the redecorating of my preschooler's room. Watch out world, a Cushie's got things to do!

My main concern now is wondering how I will make the right decision. How will I know what to do? Somehow the right path for me will present itself. Right? 


I am keeping my eyes open for all the signs, asking for help from everyone I can, including friends I rely on and can trust. 


I participate in my own healthcare. I am an advocate because this is my life, my body. No one knows it better than me, and I know me best. I've never left me behind.  I've never failed to show up.  I haven't missed any important events.  I have, however, been too busy to listen to myself.  I have placed other opinions before my own.  I have muted my instincts to appease someone else.  


In this situation, after all that has happened to me, after all I have faced, I just can't let that happen. I will know the right answer when it feels right *to me.*   So, isn't that my answer?   If I can keep my aim right there, right in that small spot of people to please, I hope to hit the bulls eye. As long as I can live with my decision, I'll know I made the right one.  When that happens or what it will be, I do not know. Stay tuned as I share information I'm sorting through as I make my decision.

Day 23: File It Away...Solu-Cortef has a new National Drug Code

SCENARIO:  You are a Cushie headed for pituitary or adrenal surgery and you are taking care of business! You drop off your prescription for Solu-Cortef and hydrocortisone at the pharmacy. When you return in an hour, the staff informs you they can't find Solu-cortef in the system.  They no longer offer it, so the pharmacy can't fill it.

WHAT? You panic. You have to have this medication. A post op Cushie and her Solu-Cortef injectible is like a diabetic and his glucagon injection.  In a crisis, you could die without it.

What are you gonna do?  Call your doctor?  Doctors don't mess with this minutia. Nurses/staff don't know even know what Cushing's is, how it is treated. They do not know how to navigate the system at this level. After many exasperated phone calls and voice mails back and forth... nothing. No medicine for you.

What are you gonna do?  Put on your super hero cape.
It's up to you, the patient, again.
So what are you gonna do?

SOLUTION:  You fall back on the details you learned from a patient with Cushing's... me or any other who has shared any bit of information with you.  You have voraciously read this blog and many others over the years.  You read and you learn.  You file them away in that brain of yours, never quite sure you could recall anything up there when needed because the Cushing's dulls your brain so much. Either you remember, or a Cushie friend who read and remembers does.

PATIENTS KNOW BEST.

These are the kinds of details... the minutia... that Cushing's patients must wade through to stay alive. Make sure you know all that you can know about your health and disease. I promise that you will save your own life on many, many occasions.

~ * ~  * ~  * ~

Not sure why Solu-Cortef is important? You can read about Cushie troubles.  

~ * ~  * ~  * ~

 Solu-Cortef has a new National Drug Code (NDC)

We have recently been informed that Pfizer, the manufacturer of Solu-Cortef®, in the convenient "acto-vials," has removed the preservatives from their product, so have been issued a new NDC number by the FDA. The number is 0009001103.

This NDC number change has apparently made it difficult for pharmacies to find the product in their computer systems, resulting in erroneously informing people that it is no longer available for prescription.

The product is available, but with a new code. The number is 0009001103.

If your pharmacy is having trouble obtaining these products, please instruct them to call Pfizer's Customer Service at 1(800) 533-4535.

Other possible contact numbers for Pfizer are 1(800) 821-7000 or patient services at 1(888) 691-6813.  Toll-free: 866-227-3737.  Phone: 908-364-0272