First Surgery after Bilateral Adrenalectomy

Surgery is a big deal with no adrenal glands. All that cutting into skin and stuff is stressful to the body. With no adrenals to increase cortisol and offset the body's biological need, patients with adrenal insufficiency (primary or secondary) must take extra cortisol for that surgery. It is a whole big thing that we patients have to do to make sure we don't die on the table -- even before we can think about the success or complications of the surgery we are undergoing that day!!

I realized that I posted this to my Fight Cushing's with Moxie page on Facebook but not here on my blog. So, today we go back in time a bit to ensure this important message is here with all the rest of them!

============

July 21, 2020 

AI’s always on my mind, sung to the tune of Elvis' You are always on my mind (btw Elvis had Cushing's, you know!)

Today I checked into the hospital for gall bladder surgery. It’s my first surgery since becoming permanently adrenal insufficient in 12/31/2013 when I had both adrenal glands removed. I’ve done everything I’m supposed to do to prepare, and I’ve increased the basal rate on my infusion pump to dispense 6 units of cortisol per hour (3 mg HC). I’ve spoken to surgeon, endocrinologist, and anesthesiologist about my steroid plan for surgery. I will remind everyone again this am what I require. This is a day surgery, and with proper steroid replacement, I hope to be home return home today. p.s I tested negative for covid Sunday.

https://cortisolmusings.wordpress.com/.../extreme.../

UPDATE 1: All went well. Staying overnight to give steroids via IV push, take IV fluids, and monitor blood pressure.

Looking back at the path you chose

In case you haven't noticed, helping other Cushing's patients wade through the cortisol quagmire is kinda a big deal for me. I choose to spend my free time doing it. Why?

I don't like seeing others struggle. 

I don't like that Cushing's is ruining Cushies' lives. 

I don't like how poorly doctors treat us. 

I detest inefficiency.

So I do my best to take time and answer questions from blog readers and facebook friends.  Tonight, the post was about ketoconazole. The Cushie had gone to the emergency room twice for adrenal insufficiency and adrenal crisis while taking ketoconazole. 

I remembered that I too took ketoconazole. My memory isn't what it used to be, and as such, I set out to search for the word keto on this blog, for so much time has elapsed that my Cushie brain can't hold the details any longer. Fair enough. 

I found the link I wanted (Keto: It Was Fun While It Lasted), but I found the post that proceeded that one by just three days called New Chapter of my Life dated November 3, 2013.  I shared the link for you to review if you are so inclined.

I read through this lost post wondering what was the new chapter of my life. I don't remember any new chapter of my life?! I was floored with emotion as I read my own words recounting the story of my decision to proceed with a bilateral adrenalectomy, which doctors often dissuade us from considering because BLA is the last resort treatment for Cushing's. 

The phrase, "I did everything the medical community asked of me" really hit home. Because don't we all? Aren't Cushies pushed beyond the point we can handle emotionally, mentally, and physically every single day and somehow manage to fight on until the day we reach remission? Does anyone remember my explanation for how statistically rare it is to have Cushing's and yet, we do? Cushies have hit the unlucky jackpot? for Cushing's.

BLA is not easy. It has taken many, many days to get to know my body and what it needs. I do stand tall in the explanation that I made to myself at that time, as it is still one that stands up to reason and emotion. It stands the test of time. 

~*~*~*~*~*~*~

I will post one day soon about the improvements I've experienced since BLA. I will foreshadow that it is difficult to parse out what long term effects are from Cushing's and high cortisol and what I can blame on rheumatoid arthritis. That's for another day.

See you soon, Cushies.

Day 2: Good News + How Many Doctors does a Cushie require?!

Hello all.  I'm on Day 2 of the Cushing's Awareness Challenge for Bloggers.  As you can see, it is now April 11th. I believe I stated before that I will make 30 posts in April, not that they would be done daily. :)   I hope you will forgive me.

If you are a Cushing's patient or "Cushie," you will not be surprised that my blogging is off scheduled from ... well, multiple causes:

• Cushing's
• Addison's
• high cortisol
• low cortisol
• brain fog
• anxiety
• procrastination
• facebook
• email
• text
• performance pressure
• the words "deadline" 
• "done today" 
• "everyone is waiting on me"
• "but I made a promise" 
• "I will disappoint everyone" 
• "I have so much to do"
• + any other symptom(s) that decide(s) to STAND UP and BE HEARD that day.

and other delays from being a patient with chronic illness:

• calling the insurance for preauthorization on procedures, surgeries, imaging, growth hormone injections
• calling the insurance for review of benefits
• calling the insurance for error in claim(s)
• calling the insurance for prescription pricing and coverage
• calling the insurance for specialty injection for adult growth hormone deficiency
• MyCharting/calling the doctor(s) for appointments, questions, requests, refills
• checking MyChart for appointments, lab results, and imaging reports
• studying every word in the lab results and imaging reports

So, it is this last one that I decided to focus on today.

Regular readers (some o' y'all tell me it's true!) know that I advocate for patients to self-advocate as well as to promote cortisol imbalance awareness (Cushing's, Addison's, Adrenal Insufficiency).

I want to share one way I do decipher imaging reports with online medical dictionaries, such as merriam-webster.com/medical, online-medical-dictionary, drugs.com/medical_dictionary. Anyone who has searched the internet knows how to enter keywords.  Then what?  

This is happening to me today, and I had to stop and share it.

I am reading the report from x-rays I had of my hips from mid March, just about a month ago. These images were taken 20 minutes before my appointment with a orthopedic specialist/ surgeon. I met the doctor, and he said he sees some inflammation in my pelvis but the medicine I take for rheumatoid arthritis will also help the inflammation in the hips and low back. WHOA!  That's good news. I don't often get good news. I mean, actual "that pain will go away," "that tumor is history," or "these lab results explain your complete symptomology!"  Uh uh.  

I hear more of "I'll write a referral/ send your case to the ... 

• sleep medicine for sleep apnea due to weight
• rheumatology
• physical therapy
• dermatology
• mineral endocrinology (I know, right?! that's another post)
• allergy and asthma
• ophthalmology for cornea (contacts for keratoconus) and retina annual exams for RA med (more later) 
• sleep apnea
• ob/gyn
• advanced ob/gyn ("for female hormone stuff)
• nutritionist (August 2017)
• orthopedic hip speciality
• pain specialist 
• pain clinic with TWO doctors (physical therapist, psychiatrist = summer 2017)

That's 12 doctors I've seen since I started as a patient as UTSW in Dallas and three I'll add by the end of summer. 15 doctors!

Did you notice anyone missing?!

I also rely on my PCP and endocrinologist as a team for my medical health.

I see a chiropractor regularly to work out back pain.

I cling to my psychiatrist and psychologist for my mental and "behavioral" health. All are wonderful and *actually remember* what I told them last time. Novel concept for a doc these days and a total plus, right?! 

So add 4 more doctors. That's 16 doctors now plus 3 by the end of summer.  That's 19 doctors! I CAN'T WRAP MY HEAD AROUND THAT. Yet, this is the reality that many Cushies find themselves in.

Let's remember, folks. I am in remission.

My first pituitary surgery was June 18, 2009.
My second pituitary surgery was April 20, 2011.
My BLA was December 31, 2013.

I am more than three years post op BLA, and I still need all those doctors to find treatment and comfort for all that lingers after cortisol excess damaged my body.

Cushing's is not joke. It doesn't mess around.

Will this all happen to you? Will you need this much help this long after treatment?  I don't know. No one knows, not even the doctors who tell you that "you are cured, stop worrying." Cushies know better than to use the word "cure." We say remission, and still fear the day that high cortisol symptoms decide to say "Hello. I'm back."

Describe Life after BLA

Nah. I don't feel like it.  I don't want to write down all my happenings. I want to clearly explain everything but that takes time.  I don't feel like explaining things today.

It has been 28 days since my BLA on 12/31/2014.  The day of my surgery, I was given 300 mg of hydrocortisone intravenously.  I was given 150 mg the next day.  I have been tapering down since then. I left the hospital on a dose of 45 mg hydrocortisone at 7:30 am and 15 mg hydrocortisone at 3:00 pm. I added 0.05 mg fludrocortisone, a replacement for the hormone aldosterone, twice a day since I was post-op day 6.  Every week or so, I decrease my hydrocortisone dose to something near 15/5.  I am getting pretty close so that is good. My doctor will like that, because if I take more than my body needs, the extra hydrocortisone gives me Cushing's again. EEK!  NO!

Last Sunday, I dropped my dose again from 20/5 to 17.5/5. I took the former dose for 7 or 8 days. Even that small of a drop takes the wind out of my sails.  On Sunday, I woke at 7:30 am and was back asleep for a nap by 11:00 am.  This, too, will pass.

I discovered that my dear friend Karen shared her post op BLA experience online and she provides clear explanation.

So today, I'll let Karen explain everything. I hope you feel well again soon.

http://m.caringbridge.org/visit/karenthames1/journal/entry/id/594801436/page/1/num/1

Good news 7 days post op BLA

I wanted a quantitative way to track my progress post BLA. My mother, a fantastic seamstress, took my measurements in 12 different places on my body the night before my surgery.  I will use this to monitor the impact of bilateral adrenalectomy and even assess whether I made the right decision for this irreversible treatment. 

I also check in mentally and emotionally each day. I dug out and will restart my gratitude journal that Cushing's emotional imbalance kept me from filling.  I feel happy and content. I'm not filled with rage and frustration, classic symptoms of high cortisol. I am grateful and filled with love. My mind is clear, and my heart is open.  My body and my mind are no longer fighting. They are in harmony. I feel happy.

Cushing's never let me feel that way. 

My body is no longer hanging on to fat because my cortisol is no longer high. Cortisol is the hormone that controls stress response.  High cortisol tells your body to pack on fat to protect vital organs in event of bear mauling or being chased by a lion. 

My abnormal adrenal glands (weighing 10 and 13 grams when normal is 4-6) were spewing cortisol out and flooding my body. My basement and whole house was under water, but even some doctors said no and blocked surgery.  

On New Years Eve, I shut the valve of overflowing cortisol when I was brave enough to trust my body and agree to remove my adrenals. By taking steroid cortisol medication each day, I now take less than my body was making due to the tumors. I take 35 mg hydrocortisone at 7:30 am and 7.5 mg hydrocortisone at 3 pm to replace cortisol made in adrenals. I take 0.05 mg Florinef twice a day to replace the aldosterone hormone also made in the adrenals. I will take these pills, in varying doses, every day for the rest of my life. 

Bilateral adrenalectomy is not the latest weight loss surgery. I am seeing results because tumors took over cortisol production and my body was flooded. Now my body is reacting--as it is built to do--by dropping weight it no longer needs. 

Thank you for your love and support. It's an incredible ride.

-- Melissa 

***Total inches lost***

7 days post op BLA

* measured by seamstress mama
** double checked many times for accuracy

Goodbye Cushing's: Finally, I get my BLA

I am finally approved for my BLA in Los Angeles, CA for December 31, 2013.  It's our wedding anniversary, so my husband said he would take me out to Beverly Hills for dinner and the place should be cool because we hear celebrities go there.  

I just got the final word from the surgeon on Saturday at noon that he will do my BLA on Tuesday.

There has been a lot of activity in the past two months.  In November, I tested again and got the highest and most conclusive tests results confirming Cushing's once again.  My Cushing's specialist cleared me for surgery.  Surgeon set a surgery date of 12/17/13 and sent me to staff endocrinologists to confirm.  First endocrinologist, a diabetes specialist, reviewed my case, said no, and blocked my surgery.  After imploring the surgeon to help me, he scheduled an appointment with another endocrinologist.  She was fascinated but perplexed by my case.  I'm a medical curiosity!  She invited another doctor in.  I debated two endocrinologists at the same time for two hours. We spread all my photos, charts, lab results, MRI studies, and clinic notes across the table.  They were misinformed on many aspects of the medical literature for Cyclical Cushing's but wouldn't listen to me.  "Bilateral adrenalectomy is not reversible!  You will be on medication the rest of your life!  You will be miserable!"  They blocked my surgery even though I had a doctor -- a NIH-trained physician who specializes in Cushing's and sees 1000 Cushing's patients in his practice -- who referred me to surgery. My surgery on December 17 was cancelled.

I left the hospital, dejected, uncertain, and suicidal. I know I am dying inside this broken body. My family circled the wagons and protected me. My mom flew in from out of state to help care for me, my husband, and daughter. She spent Christmas away from my father and family for the first time ever just to be with us.  I continued to test per the deal I struck with the endocrinologists but I quickly tapped out. Three 24-UFCs with 17-ohcs, five midnight saliva cortisol tests, plus a low dose dexamethasone test really did me in.  Test results showed even more high tests that point to hypercortisolemia:  two high urine cortisols in 17-ohcs and two high MN cortisol salivas (2x and 3x upper limit of normal).  I suppressed on dex test, as many cyclical Cushing'a patients do. My surgeon agreed to do the surgery again.

I am comfortable in my decision and ready to move forward.  Today, we will rally and get the house ready for my absence, and I'll started up a "feel good box" of things to keep me occupied during my time in the hospital.

When I see the photos, I feel both sadness and hope.  Sadness because of all the time I have lost to be my best self. Hopeful now that I can say goodbye to her and start a new life.  Those two Melissas are gone. I will never be those people again. Hopeful because the new Melissa will be the MELISSA REMIX - the best combination of us all.

I wanted to share this with all y'all here. I found blogging to be something that helps me make sense of what is happening to me, a place where I can PUT it and release it as I try to live every hour of my life.  I am so pleased to hear that people relate to my experiences and they are rooting for me. I am rooting for you, too. 

Well, Happy New Years Cushing's family!

May us all blossom in our new healthy state in 2014.

Melissa

Cushies can die after exhausting all treatment options

This makes me incredibly sad.  I have had two unsuccessful pituitary surgeries, and I am currently doing medication therapy (pm ketoconazole with am Cortef) until the tumor culprit comes out of hiding and presents itself on the pituitary MRI for a third pituitary surgery.  No one knows how long that will take.

After my first pituitary surgery, I developed ptosis, or drooping eyelid.

**************

Cushing’s: the worst case scenario

Viv Thornton-Jones

Churchill Hospital, Oxford, UK.
Endocrine Abstracts (2008) 15 S58

---

We present the case of a 40-year-old female who was referred to our Department in 1993, for further management following the diagnosis of Cushing’s disease. She proceeded to a transsphenoidal adenenomatectomy (TSA, note: pituitary surgery) which resulted in a biochemical cure.

In 1998 she presented with recurrence of Cushing’s Disease, which was managed by a 2nd TSA (pituitary surgery) followed by external beam irradiation.

Bilateral adrenalectomy followed a year later, due to the inability to control her disease.

In 2001 she presented with Nelson’s Syndrome managed by a 3rd TSA (pituitary surgery) followed this time with Gamma Knife surgery.

In 2004 she presented with manifestations consistent with recurrence of Nelson’s Syndrome and proceeded to a 4th TSA (pituitary surgery). 

Despite the risk of blindness, the patient agreed to a second course of Gamma Knife treatment for the possibility of tumour control.

Over the next 2 years her clinical picture deteriorated, resulting in a right partial ptosis and a sixth nerve palsy.

She was referred to an Oncologist who offered her Chemotherapy, but she refused treatment.

The patient was then in the care of the Palliative Care Team and she died peacefully at home in 2006.

---

Endocrine Abstracts (2008) 15 S58

Thank you to Ami and MaryO for finding this abstract.