4 months post-op BLA (bilateral adrenalectomy) upate

It should be considered a good sign that I went so long without posting!  I am 4 months post-BLA this past weekend.  Adjusting to life with Addisons while weaning down to my therapuetic steroid dose continue to be a roller coaster ride.  I can say that I most certainly feel different, and that I believe my Cushing's remission is closer with every steroid wean I succesfully endure.  I say 'succesfully' as recent prior attempt did land me into AI (adrenal insufficiency) and resulted in three days down recovering from just that.  I took the wean much slower this time, two weeks to be exact.  And though I still feel every ache and pain that a wean brings, I do think I made it through this time.  And hopefully just 1-2 more weans to go. 

With that said, last week I discovered the misshapen, lopsided belly I have been noting for several weeks and then finally bulged...is indeed an incisional hernia.  Sigh.  I see a surgeon this week to find out how this will be dealt with.  And I find myself learning, much sooner than I had hoped, the protocol for surgery for steroid dependent patients.  And what questions to ask a potential surgeon to make sure he/she is competent to care for a steroid dependent paitent.  Sigh

I wanted to share with you all some videos of members of my Cushings community.  These brave individuals met with a drug company last fall and allowed their stories/interviews to be filmed.  If you have time to watch any or all of the, you will have a better understanding of the difficulties living with Cushings brings.  Thanks for watching.  Please comment your thoughts on them...I'd love to hear from you!

Short video interviews with a Cushie

Bilateral adrenalectomy

For anyone reading my blog as research in consideration of having a BLA -- I have created a Word document of my observations and tips for Dr Fraker, UPenn hospital, and some general BLA.  If you are interested in reading this, please comment to me and I'll email it to you. 

This was my first laproscopic abdominal procedure, and so as much as I tried to prepare myself, there is not a way to fully be prepared for how it will feel.  This surgery was as hard as my first pituitary surgery, but in a different way.  I was thankful for not having the intense headaches, but getting in and out of bed was extremely difficult.  Heck, shifting myself in bed was difficult!  For surgery I was given very high dose of IV steroids, which then i would be weaned down over the next 3 days in the hospital.  I needed this as now my body is not able to make cortisol, the stress hormone, which our bodies make more in times of stress, like surgery.  These high dose brings out Cushing's symptoms, so in my first post-op days I still felt very Cushie...puffy, bright red face, able to sleep much, high anxiety.  So swollen that when my IV blew, after several attempts I ended up with an IV in my left index finger!  My steroid dose was cut in half each day, and by my last night in the hospital I was feeling withdrawal symptoms, the worst of it as pain in my spine.  After 4 sleepless nights in the hospital, I was ready to leave and rest in a comfy hotel bed for a few nights.  I was fearful of riding in a vehicle, the roads are really bumpy in Philly!  Our shuttle driver was very empathic and did an awesome job delivering my back to the hotel without much jostling about.

I got settled into our room, where we stayed another 2 nights.  I would not recommend traveling any sooner.  I was on the cusp of not knowing if two days would be enough time.  Then I thought I could have stayed another two days in the hotel to feel more at ease about traveling. But with his help, assistance at the airport, and staying on top of pain meds, I felt I could do it. 

We got home about 1:30 a.m., it was a long day, but getting home was a huge worry lifted! 

“Our study shows that BLA (bilateral adrenalectomry) for persistent Cushing's disease provides patients with considerable improvement in their Cushing-related symptoms with concordant increase in their quality of life. After BLA, patients may attain the same (or better) quality of life as patients initially cured by transsphenoidal pituitary tumor resection. We think that BLA is a safe and effective treatment of the 10% to 30% of patients who fail initial therapy for Cushing's disease, and should be considered preferentially over other available therapies”  http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1877068/

 

I like that statement!   

Today I am 25 days post-op my BLA surgery and my mantra is ‘slow and steady’.  I thought I would outline this step of my journey for those who may be considering a BLA as treatment for their persistent Cushings.  Making the decision to have this surgery is one not made lightly.  There are sacrifices that will be made living without adrenal glands, and making the decision to have a chronic illness for the rest of your life is made with a heavy heart.  When I first began researching Cushing’s two years ago, and would read accounts fellow Cushies having a BLA I can remember thinking “wow…so extreme…I’ll never get to that point”  and yet so quickly I did.  The rapid transformation of a healthy body to one daily struggling with fatigue, weakness, pain, nausea, mood swings, brain fog, insomnia, anxiety, swelling…..well it changed my perspective.  I am tired of my days being consumed with my disease and I want to move forward with life, define my new ‘normal’ and go about living!

At my two-week post-op surgery #2 phone appointment with me endo, he told me that BLA was my next step and it was devastating.  I had at least 4 more weeks of surgery recovery, had to wean off the replacement steroids so that I could begin the grueling process of testing (AGAIN!) to prove active disease for surgery referral.  And if I haven’t mentioned yet, weaning of steroids is “not for wimps” as a wise Cushie friend often reminds me.   

As a Cushie my body has got accustomed to high levels of natural steroid, and for anyone who has taken a round of prednisone will tell you that it does a body wonders.  The anti-inflammatory effect masking pain from physical symptoms such as losing all my muscle strength (steroid induced myopathy) and carrying an extra 50 lbs, most of which is in my abdomen throwing off my center of gravity, not to mention my back! So lowering replacement steroids reveals prior masked aches and pains.   Dropping the levels of cortisol also bring on symptoms of Adrenal Insufficiency (AI) (nausea, GI issues, headache, low blood pressure, heart palpitations, dizziness) Oddly enough even if my cortisol levels could be higher than normal, or within normal range, the change from higher levels to lower levels can bring on AI.  AI can quickly worsen and lead to full blown Adrenal Crisis, which is life-threatening; so weaning off steroids has to be done slowly and carefully.

 

But nonetheless, has to be done in order to move forward in the treatment process! 

 I was able to wean off my replacement steroids in 6 weeks, another indicator that surgery was not successful.  I geared myself of for marathon testing and hit it hard…like my full time job.  The more I tested, the more likely I was to show the highs and the be done testing!  In just over a month of collecting saliva, urine, and midnight blood draws…I had enough results for the surgery referral. 

 During this time I researched surgeons, my biggest resource being my online Cushings support group friends.  I have grown to trust these people for their knowledge and experience in the disease.  Many of them had spent countless hours researching surgeons, and were willing to share with me the facts.  There were 4 surgeons that work with my endo, and I knew I was going to choose one of them.  Two were in California so that alone crossed them off the list.  The remaining two were equal distance in travel time for me, so I pursued them.  I wanted a surgeon who had lots of experience in removal of the adrenal glands, as there is always a small chance that adrenal cells/tissue (microscopic) that mistakenly get left behind can regenerate and begin to produce cortisol, causing Cushings to reoccur.  I choose Dr. Douglas Fraker at University of Pennsylvania Hospital