wow..it's been over two years.  I guess I got my life back!

I knew that I missed the blogging challenge this year, but I didn't realize I missed it two years in a row.  I'm know I don't have much, or much at all of a following.  I think blogging was more for my own therapy and it gave me hope that maybe someone searching my earmarked terms might stumble upon my zebra blog, and my experience might help them.  So many Cushing's patients before me were priceless in my research in learning how to advocate for myself and get treatment.

But, back to today!  Today I sit at the same keyboard and I'm coming upon my 4th BLAnniversary this coming October.  Much has changed, my husband and I became empty-nesters and celebrated our 20th anniversary.  Here is a picture from our trip to Vancouver/Alaska/Seattle honoring our years together....do I look different that then picture I posted of myself just before my first pituitary surgery!?

That's right, we are still cruising Disney~! 

I've settled into a routine for my life without adrenal glands, and traveling is a challenge.  But those sound like entries for the next challenge.  For today I'll leave until October 12, 2016 when I'll my best to come post a 4 year mark post.  But as little Dory says..."I have a short-term remembory problem"

Cushing's disease diagnosis

Today is May 1, 2013....on this date in 2011 I was diagnosed with Cushing's Disease.  Today am I six-months post BLA.  If you told me then this is where I'd be today, I would not have have believed it.

Sadly, though, it is to look back on the past two years and remember the struggles and heartache of searching for diagnosis, treatment, waiting to see if the surgery was successful.  It feels like lost years. 

Thankful, I must be that though living with Cushing's for about 5 years, including the years undiganosed....I am considered one to be 'fast' tracked among the many others in the Cushing's community who live with the disease untreated for as long as 10 years, some even more.  What is wrong in this day and age, that doctors do not listen and trust their patients who tell them their health has changed.  Shameful.   And so Cushie's search out the few doctors who believe the patient's account of their health.  We travel far distances for diagnosis and surgery. 

Two years ago, I awaited the phone call from my doctor on the other side of the country, telling me that my lab data was conclusive and I could pick out my surgeon.  He had a list of 4 that he recommended.  In all the great states of America...there were 4 to choose from.

Surgery is not an immediate relief for Cushing's.  Successful surgery brings cortisol withdrawal which is a long process of weaning the body back to normal levels of steroid.  I am close to that maintenance dose now, not quite there but close.  Weaning takes about 3 weeks and is dreadful. It's hard to fit that into a family's schedule.  And the more I become a Cushing's survivor and no longer Cushing's patient, it becomes even harder mentally to gear of for 3 weeks of feeling rotten.

I'm still balancing my hormones too...ones that I would have never even known I had!  Replacements for aldosterone, and growth hormone are tricky!  Yes, adults need growth hormone too!  It's how our bodies regenerate, heal, build muscle, get good sleep, and balances the body composition of fat and muscle.  I just began replacing this hormone this past Sunday, it will be up to a year before I see the full benefits of doing so.

If you are reading this and think you have Cushing's...do not give up!  I was told by 3 doctors that Cushing's was unlikely.  And here today, two years after that same diagnosis I am healing and recovery and reclaiming my life!  Thank you Jesus!

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

 

I thought i might be done blogging....i guess i'm not

Today I sit on a 10 day countdown for my next surgery. As I suspected last April my second pituitary surgery was no successful and my endo referred me onto the next step.  A more permanent step, but one that is much more likely to put me into remission from Cushings.  I will be having a bilateral adrenalectomy, removed of the two adrenal glands sitting above the kidneys that produce cortisol.  I will forever be steroid dependent.  I have two brand new spakin' medic alert bracelets to prove it.

I am told recovering from this surgery and the crash of Cushings, once again, will take upwards of a year.  So I guess I'm not done blogging :)

this low this week is kicking my butt!  i took our new puppy on a walk at lunchtime, for 40 mins....a stroll that included a stop at the walking path where she could run some 'laps' and burn some energy and it wiped me out...good and proper.  i even took a kinda nap this afternoon...the kind where you fall asleep but wake yourself up and realize it's only been 10 minutes.  those are the kinds of naps I'm capable of these days.  Days like today I'd love to sleep and hour and wake up refreshed and energized and finish out my day strong.  I'll be honest, I'm already counting down the hours to jammie-time (said in my best MCHammer impression).  I miss the days of going non-stop and being productive....I have a list of easy things to-do and nothing got done today and it's not looking promising.  I really think unless you have a chronic illness or live with a family member with one, it's just not possible to understand the depths of the fatigue it brings.  I am so fortunate that God has blessed my life in that I can take these days as slow and non-productive as I need too.  it does take it toll mentally though...nobody wants to feel like they are drowning in 'life'!