cushing's-syndrome

My new hero: Harvey William Cushing.

Father of modern neurosurgery- did brain surgery long before the advent of the MRI. He studied under Sir William Osler who founded the first residency programs in the United States as well as a little place called Johns Hopkins Hospital…maybe you’ve heard of it?  

Almost everything important is named after him: Cushing’s Syndrome, Cushing’s Disease, Cushing’s Triad… 

Cushings Triad by the way is: Hypertension, Bradycardia, and Cheyenne Stokes breathing due to increased inter-cranial pressure. 

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So I think I may finally FINALLY have an answer.  Typing this out is very hard for me because I tend to question the hell out of myself.  I have also lost a lot of faith in the medical industry because of how many times I’ve been turned away with no answers.

However, I think I may finally have a damn good idea what is going on, and I’m building up my courage to ask my doc to be tested.  I think I may have Cushing’s Syndrome.  The photos above are presented as evidence of some of the most common symptoms: central body weight gain, moon face, and acne.  The first three pictures are from before I got sick.  I ran every other day and loved it, I liked to be active, and I didn’t have too much trouble with my skin.  The fourth image is of just after I started to get sick.  I developed horrid acne that I STILL have trouble with at 26 years old and began to develop the “moon face” that is common to Cushing’s.  The fifth image is just one year later, with an extra 60 pounds.  As the last three images show, over the last seven years the weight gain and moon face have just gotten worse.  I have tried exercising, dieting, going vegetarian,  abusing laxatives, and limiting myself to 1000 calories a day with little to NO results.  I just tipped the scales at 200 pounds.  No matter what I do I can’t seem to STOP this weight gain.

There are other symptoms I have too.  Anxiety and depression, muscle weakness, purple stretch marks on my belly and thighs, fatigue, and headache.  Furthermore, there are symptoms that only crop up in long term untreated Cushing’s.  Namely hypertension, tachycardia, and insomnia.  ALL of which I’ve developed in the last month or so.

Lastly, one of the medications used to treat Cushing’s is Metoprolol.  My doc started me on this drug two days ago, and I feel better than I’ve felt in a LONG time.  My husband immediately noticed and improvement in my physical strength AND my attitude.

I’m GOING to ask to be tested.  The question is, will the doc take me seriously?

I’m always shy, I’m always afraid to express my concerns, I’m always afraid of being swept under the rug… I’m NOT giving up this time.  I’m not walking away unanswered…

Drugs used in the treatment of hyperadrenal disorders

As a test, I’m going to try writing up my revision notes on here. Bit of a strange place to start, but it’s as good a place as any, I suppose. So here we go, a quick rundown on the drugs used to treat hyperadrenal disorders.

Specifically this will be about hypersecretion of hormones of the adrenal cortex. A quick reminder of the anatomy of the adrenal glands (or suprarenal, if you prefer):

So you have the inner medulla and the outer cortex, the latter consisting of the zona reticularis, zona fasciculata and zona glomerulosa. I remember these names by thinking of the letters GFR (because this also means Glomerular Filtration Rate which, as a side note, is  around120ml/min). 

Basically, the adrenal cortex pumps out steroids. A quick rundown of what the different sections of the adrenal cortex produce:

1.) Zona glomerulosa - mineralocorticoids (aldosterone etc.)

2.) Zona fasciculata - glucocorticoids (e.g. cortisol)

3.) Zona reticularis - sex steroids (e.g. dehydroepiandrosterone, DHEA)

So, there are three drugs I’ll talk about:

1.) Metryrapone

2.) Ketoconazole

3.) Spironolactone

The first two work by inhibiting steroid biosynthesis.

Spironolactone is a mineralocorticoid receptor (MR) antagonist i.e. it binds to the receptor, but does not produce a physiological response, thereby blocking the effect of agonists (things that bind and DO produce a response).

Firstly - Metyrapone

Like we said before, Metyrapone works by inhibiting steroidogenesis - specifically it inhibits a rather important enzyme by the name of 11-beta hydroxylase. I’ll show in a diagram why this is important:

As you can see, 11-beta hydroxylase converts 11-deoxcortisol into cortisol, and 11-beta corticosterone into corticosterone. Therefore, if it is blocked by Metyrapone, neither cortisol nor corticosterone will be synthesised - thereby treating the hypersecretion of cortisol, for example in Cushing’s Syndrome.

Cortisol works on a feedback loop - if it stops being produced, this will send negative feedback back to the hypothalamus and pituitary, which translates to increased production of ACTH (which stimulates cortisol production).

As I mentioned before, Metyrapone can be used to control Cushing’s Syndrome prior to surgery or for long-term amelioration of hypercortisolism if it persists/if surgery isn’t possible.

It does, however, come with some unwanted actions, like many drugs:

1.) Nausea, vomiting, dizzines

2.) Sedation, hypoadrenalism

3.) Hypertension on long term administration (11-deoxycorticosterone accumulates in the z. glomerulosa and has aldosterone-like (mineralocorticoid) effects => salt retention and hypertension.)

4.) Hirsuitism (excessive hairiness)

Okay, so that’s Metryrapone! Now for the second drug…

Ketoconazole

This is mainly used as an antifungal agent, but at higher concentrations can inhibit steroidogenesis (glucocorticoids, mineralocorticoids and sex steroids). As with Metyrapone, it can also be used to treat Cushing’s 

Unwanted actions include nausea, vomiting and abdominal pain. Also unwanted are:

1.) Alopecia (baldness)

2.) Gynaecomastia (enlargement of breasts in males, oligospermia (deficiency of sperm in the semen), impotence and decreased libido

3.) Ventricular tachycardia 

4.) Liver damage - possibly fatal. Liver function must be monitored on a weekly basis 

And finally…

Spironolactone

This drug is used to treat primary hyperaldosteronism, also known as Conn’s Syndrome.

It must first be converted into its active metabolite, Canrenone. As I mentioned before, Spironolactone works not by inhibiting steroidogenesis, but by working as a competitive antagonist to the mineralocorticoid receptor. This blocks Na+ resorption and K+ secretion in the kidney tubules, so is classed as a potassium sparing diuretic.

Unwanted actions include:

1.) Menstrual irregularities 

2.) Gynaecomastia (due to androgen receptor binding)

3.) GI tract irritation

N.B. Spironolactone must not be used in patients with renal/hepatic disease; it is contraindicated.

And that’s about it. Forgive the rather disorganised/potentially unclear nature of this post, but it was my first one like this. I might do more. I’m not really sure - there’s a hell of a lot to get through and writing this out, while a good reminder, does take an extra bit of time. We shall see!

Watson

So the ultrasound revealed….normal! I guess I just have a random lump on the back of my neck that appeared six months ago. I am happy about that. kel-rhea, I did have alarmist theories about tumors, cancer, etc, and at one point we thought it was Cushing’s Syndrome, but my cortisol levels were normal so it was not that! I believe that the Seroquel is what caused my weight gain, insulin resistance, and uncontrollable high blood sugars, so I am happy to be almost completely off it and experiencing a lot of improvement in all those areas. I’ve been on it for years and I thought I might be taking it forever but I feel so blessed that I don’t need it at this point anymore! I am still on a lot of psych meds but that’s a very strong one so to be able to come off it feels really incredible. Tomorrow at my psych appointment we’ll see how the psychiatrist feels and where we want to proceed from here. Seroquel made me able to sleep so insomnia has been increasingly more of an issue- we’ll see if we can change some things around that will help with that! 

I’m going to post in a bit about the theme of the day but I just wanted to share the good news about my neck :)

Horribly Unflattering Picture Time


I still have not heard back from Mayo. I’m really fucking frustrated.  The waiting is driving me crazy, and I’m way past the point of being patient.  In the meantime, I’ve been thinking for a while now it was time to get updated pictures to track where everything was, which of course always helps with the self-esteem.  But nonetheless, I will share my shittiest pictures with everybody because I need to track it, and it’s helped me seeing other people’s images. 

So, updated body picture: 


Is it just me, or do my legs look smaller?  I think they do, but I really can’t tell for sure.  This is the larger/full body version:

And of course, the awesome buffalo hump:

That has definitely gotten worse.  Funny how when I started keeping track of this I was so unsure of if I had that damn hump or not.  I’d say it’s pretty obvious now.

Definite moon face I was unsure of:

The shitty dark spots on my neck that never go away (this plus the bad breaking out are what I’m most self-conscious about):

And even though they’re hard to see, the stretch marks:

I’ve tried several times to get better pictures of these but with no luck.  But there you have it.  Crappy pictures of the freaky things going on with my body. 

Today was pretty rough.  I woke up this morning with a horrible headache and spent most of the day sleeping off and on as I tried to get rid of it.  I also felt so fucking drained (and still do), like I cannot get enough sleep no matter what I do.  I haven’t felt this shitty in a while.  As I was struggling, I wondered how it is I’ve been able to feel so much better lately in general, and I think it’s because I broke up with my boyfriend before Thanksgiving.  In particular, I’m not running back and forth between here and Oklahoma.  I’m taking care of just me instead of me and him, and for the most part I no longer have the stress of dealing with him.  I didn’t even realize just how huge of a role stress played until he was gone for a few weeks and came here for Christmas (long story, but he still came home with me for Christmas).  It was definitely an eye-opener.  I don’t know what I did for today to hurt, though. I did nothing different than I usually do, although it has rained all day. 

I’ve also been trying to pay attention to things my body has been doing lately since my cortisol has been high twice in a row.  One thing that’s strange is I typically barely eat.  I can barely stomach things, and I usually only want healthy shit.  But since before Christmas, I feel like I am starving constantly.  My stomach will be growling within 2 hours of eating a normal-sized meal.  It hasn’t been every day, but it has been frequent.  I have also been craving everything that’s horribly bad for you, and I have no idea why.  I still don’t eat that shit except on rare occasions.  I have not given into any of that, but I would be lying if I said I wasn’t struggling with it.  I think that’s part of why I want Mayo to call me so fucking badly.  I also remembered when I first started gaining weight that this happened (back when I was working out an hour a day 6 days a week and still eating healthy).  I always assumed it was because of my birth control because it said one of the side effects was “change in appetite,” but I’ve been wondering if that’s actually what caused it now.  I remember it happening after I moved to Dallas, too, and I gained significant weight at that time as well, again, while dieting and exercising.  So I really wonder if there’s something to that.

The other thing is, the dark spots on my body are reeeeally bad right now, and I’ve been consistently breaking out very badly for about a month or so now.  I’m even breaking out on my shoulders again, and that has not happened in a very long time.  I don’t know when the previous timeframes were that the skin issues were this bad, but I remember them happening on more than one occasion.  I just don’t have a specific timeframe aside from I remember it being really bad once day when I worked at the fabric store, which would have been in 2006.  It’s all gotten better and worse again over the years, which seems to support the whole hormones cycling thing.  I just don’t want to say for certain that it does since I don’t have specific timeframes on it. 

I’m so fucking tired.  I’m going to sleep again.  I’ll update the overview page some other time.

Cushing's disease

So it’s official this is what I have. Some of you guys have asked what is really going on with me. I haven’t been upfront about it, mainly because I was waiting for the official confirmation.

I have a 7mm tumor on my pituitary gland. I get to sit down with a neurologist on the 21st. Hopefully we can schedule a surgery soon. I start school on the 25th and I hope I don’t miss too much of it.

Am I scared yes! I’m having BRAIN SURGERY!!! Without your brain your not even you and can’t even function. Fuck this rare disease! And fuck this tumor that I named Joel.

If all goes well, all my health problems should disappear.

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The top picture and the bottom picture show my shredded pre-Cushing’s abs. Call me conceited, but I worked hard for those! xD The elevated levels of cortisol my body has been exposed to for so long has induced insulin resistance as well as fat gain along my midsection…  my muscles waste away very easily as well; cortisol is highly catabolic (the middle picture). And no, I did not stop exercising nor did I start eating donuts (I actually have been on a stricter diet believe it or not). 

A New Outlook

   Before I begin, I’d like to ask a simple question. What is it that defines you? Better yet, what is it that dictates your happiness? Do you wake up every morning believing wholeheartedly that you are living life the way you envisioned? If you are one of those people who sits back from time to time and questions whether you are truly on the correct path, then I encourage you to hear my story and follow me throughout my journey.

    My entire life I have been driven by the need to succeed. I have literally invested my whole being in one goal; to be rich. I suppose this stems from my upbringing. My childhood was difficult as my parents were divorced, young, and struggling to support themselves, let alone a child. Even today I see how hard my parents have to struggle just to live comfortable lives. And so, from a very young age, I made a promise to myself that I would be so successful that money would never even cross my mind.

   Growing up, things came easily to me. I never had to try hard to get good grades, I was handsome, I always had friends…I was loved. People have always told me that I am going to do big things with my life, that I just have that “it” factor. These things have both come as a blessing and a curse. When I was about 12 years old I made a promise.  I would not subject my children to the hardships I was faced to overcome as an adolescent. Although this goal of monetary stability is one shared amongst many of us, my perception of wealth and success became noticeably unrealistic as I matured. Dreams of owning a nice home and a happy family turned into the necessity of living a lavish lifestyle complete with multiple extravagant homes, nice vehicles, and yearly vacations.  The pressure to succeed had blinded me from the realities of life.

   Throughout middle school until the end of my freshman year of high school I was on top of the world. I was a sports star, had decent grades, amazing friends, and a beautiful girlfriend. I felt untouchable. These goals I set for myself, they were all easily attainable in my mind. Nothing was going to stop me and nothing could. Then came my sophomore year.

   I began to notice a change in my body 5 years ago during the summer before my sophomore year of high school. Severe acne started to cultivate on my once spotless face and my face was noticeably rounder and shiny. My family and I attributed my acne to being a typical teenager and the round face was surely a result of me putting on weight to play varsity football (so we thought). After a bout with Acutane the majority of my acne went away but my face shape remained the same and I was also starting to develop a significant amount of body hair. This troubled me because none of my family members are very hairy, but I figured that maybe it was my Italian genes kicking in. After about a year I really began to fear that something was wrong with me. My face had literally tripled in size and I could never lose weight. At times I would spend up to 3 hours in the gym and eat perfectly with no results to show for it. Around this time I was also beginning to experience chronic anxiety that coincided with sleep deprivation and mental instability. For some reason I could always find something to attribute all of these things to. “I’m probably not sleeping because I’m so stressed out, which is also causing my acne and mental instability."For four more years I would wake up every morning and wonder who the person starting at me in the mirror was. It certainly wasn’t Josh Blevins. My symptoms were getting worse; I was losing my identity. Why, how had my body and mind transformed so much in such little time? It’s hard to explain the feeling of knowing that something is wrong with you and not having a suitable answer to your problems. People called me crazy for the longest time because they were convinced nothing was out of the ordinary.

    Fast forward to August 4th 2012. By this time my face had become so swelled that I was going in to have facial liposuction and buccal fat removal surgery performed. My face had gotten so big that this was the only solution. I remember feeling so happy that morning, finally my face would look the way it should! After having the procedure done (by far the most physically painful experience of my life, and I have my entire left ribcage and hip tattooed) the doctor sat me up and put me into a wheelchair. As I was rolling out of his office he said, "Hey Josh one more thing. I want you to go get some blood work done and get tested for an extremely rare disease called Cushing’s syndrome. It could be nothing but you seem to have some of the typical symptoms. Go get that done and let me know what happens.” After weeks of extensive testing, I was indeed diagnosed with Cushing’s syndrome.

   For those of you who don’t know what Cushing’s syndrome is I will attempt to explain. Cushing’s syndrome is a disease that affects one’s endocrine system by producing an overabundance of a hormone known as cortisol. Cortisol is released into the body in order to regulate stress levels, however, if too much is being produced it can cause a variety of health problems. These include: a condition known as “moonface”, insomnia, severe depression, mental instability, excess body hair, acne, excess upper body weight, high blood pressure, and yes, even death. Cushing’s is often undiagnosed as it’s symptoms are typically treated individually and quite honestly, testing for Cushing’s is a long process doctors tend to avoid. The overproduction of cortisol is usually found in one of two places. Either a tumor forms on the pituitary gland of the brain’s frontal lobe or a tumor forms on the adrenal glands. For me, a tumor has formed on the pituitary gland and has been the cause of all of these previously unexplainable changes in my body. Learning about this has come both as a shock and a relief. I would be lying if I said that I am not terrified that I have a potentially life-threatening tumor in my brain. I am relieved, however, that this condition is treatable and if I can be treated properly, I will look, think, and feel the way I knew I was supposed to for the last five years of my life.

  We have all heard the stories of people experiencing “spiritual awakenings” or having sudden, life-changing, epiphanies. I can remember sitting in church as a kid, surrounded by people that were being brought to tears and wondering what they were feeling and why I wasn’t feeling the same thing. I have always been a firm believer in God but, to be honest, the supernatural aspect of my faith has never been something I truly gave much thought. Yes, people would proclaim miracles, and I certainly have been blessed throughout the years, but I never really believed in the miracles we read about in the Bible or the countless “convict turned Christian” testimonies that are reiterated in churches throughout America. For the majority of my life I proclaimed to be a Christian, however, led a lifestyle that continuously placed my wishes, my desires, before my faith. Throughout high school I engaged myself in activities most Christians would consider to be disgraceful. I will be the first to admit that I have done some things that most followers of God would be ashamed of. But I did not come here to tell you about my faith, rather what caused me to take on a new perspective of my faith and my life in general.

   After learning that I have Cushing’s disease I have truly been taken back. A new perspective on life has been introduced to me. Although the drive to succeed and make money is still present, I have discovered a more important aspect of life…happiness. I have come to realize that we must enjoy life and embrace each day as a blessing. Surrounding myself with positivity, friends, and genuinely happy people has become my new focus. I used to laugh at people who would say “find what makes you happy”, but honestly I see the validity behind that statement now. I seek out God more than ever before and truly believe that my relationship with him has strengthened my will. I also have vowed to eat healthily and do everything I can to fight this disease. For so long I was caught up in what the world told me I should look like or how I should act or even what I should be interested in. Now I realize that life is too short to be focused on what others perceive as correct. We must enjoy life. Never sacrifice your happiness, health, or well being for a dollar bill or an approval.

   This brings me to why I have started this blog. I plan on documenting every step of my battle with Cushing’s syndrome. From my current state, to surgery, to recovery and beyond, I plan on sharing my story with all of you as I embark on this journey. My hope is to inform and inspire. Too few people know about this disease but, more importantly, too few people are living life for what it should be. Please join me on my journey. I hope to share all of the emotions and memories of this transition to a new lifestyle with all who care to listen. 

Test #1

Had my first cortisol test today. Unless it is perfectly normal I will demand further testing, even if they say it’s not elevated “enough”. My general physician was very supportive, so I’m sure he’d condone going to an endocrinologist no matter what the results are. The beta blockers finally have my blood pressure in check, kind of. Now instead of being too high it’s too low! So I think we will compromise with a dose and a half instead of a double dose. I’ve got high hopes, crossing fingers and toes.

A light is on the horizon.

Finally home new specialist was the nicest Doctor I have ever seen he took his time listened and jumped straight into action. Still no answers straight away but that was never going to happen but he thinks it’s more then likely a combination of several conditions making me feel so weak and all round ill. We have 158 conditions to go through and cross off as he feels like it is related to my coeliac’s which as nearly most know is an autoimmune disorder in itself. For now blood work today and more in early in the week. Cushing syndrome is one thing he thinks I may be suffering from. My adrenals will be tested further as we go.
He is coming to my hometown in less then a fortnight to see me and start going through all my records and start a pathway to full diagnosis but he thinks with a combined effort I might get rid of my walking stick but it’s going to be a long process but it’s better then no hope at all!!!
Possibility in near future for hospitalisation for a few days to have controlled tests done but I won’t really know anymore till he studies up on all my records the poor man.
He is hands down the most intelligent determined but honest Dr I have ever met, he is just publishing a paper on a whole new disorder he has discovered but he is hopeful mine will be one very well known.
I know I am going to be a pin cushion for a long time and still no exact diagnosis and no treatment but it’s a start.
He spoke about helping me once diagnosed and treated correctly to create a team to rehabilitate me physically which gave me a lot of hope and made me smile even if I suffer several illnesses even if they can not be cured but controlled I will be so much happier just to feel a little strength would be amazing.

Final Countdown

With just twelve short hours before surgery takes place and I am on my way to living a new life, I have gone into a deep reflection period. This process has been by far the most physically, emotionally, and mentally exhausting experience I have ever been through. I have realized that life is much too short to concern ourselves with the little annoyances of the every day grind. For the past few days I have been doing a lot of reminiscing with my family and friends. I can remember back when I was younger, being extremely arrogant and vain. This process had truly taken me back and made me appreciate everything that I have. I firmly believe that God has spoken to me through this and humbled me, made me appreciate life and the countless blessing He has bestowed upon me.

Yesterday I went to get my haircut from my man AJ at Capitol Barbershop and we got into a great conversation. He reminded me that there are two ways to go about attaining things we want in life: we can bulldoze our way through things, become a juggernaut of sorts and just destroy everything in our path. Or we can strive to do things to the best of our ability with positivity, a strong work ethic, but most importantly, a cool head. This rang so true for me. I have really taken on a new philosophy about life. We can not be in a rush to get rich. Of course, we should always strive for greatness and success however, always remember that what is most important is if you can look yourself in the mirror at the end of the day and honestly say that you tried your best and you did everything with your morals intact.

Life is a journey, so stop focusing on the destination. To all my family and friends that have supported me throughout this process, I would like to say thank you for the unconditional love and continued support. The positivity you have surrounded me with has truly changed my life. Positive energy and surrounding yourself with positive influences DOES affect every aspect of your life. I encourage everyone to reflect and really hone in on what you value most in life, because it should not be superficial or material. Friendship and love lasts beyond what any dollar amount can bring you. I thank God for everything he has and continues to bless my life with. I am at the tail end now and I look forward to sharing my recovery with all of you. Much Love.