Tracking Down A Surgeon

This is supposed to be the easy part. Have tumor, have diagnosis, have referral, get surgeon - right?

Nothing comes easy in medicine anymore, or at least not for me.

Ten years ago I made a friend in Los Angeles; it was like meeting a long lost brother. The weirdest experience in meeting someone and the most comforting one also. When I first moved to LA I roomed with him for about a year. His cousin Ginnie is a very interesting woman who does spiritual travel tours. She is into everything and anything paranormal, is that the right word? From arranging group tours of Machu Picchu to Stonehenge, she's your girl when you want to seek out something different. She also knows the best ghost trackers and clairvoyants. We went to lunch one day and I asked her if I had some sort of hex on me, as I can get bad service anywhere. lol She leaned back and took me in, then leaned back in and confided that indeed there was a problem. I have an 'inverted aura', she said. According to her it is common in people with severe ailments. I think she was finding a nice was to say I am transparent. :)

It must be my aura that is keeping two surgeon assistants from returning my calls, despite my daily inquiries. Either that or I am getting the hint that no one is allowed to contemplate scheduling surgery if they are planning their holiday festivities. *sigh*

When my heart gives out, I'm sure it'll be at shift change. On a Friday. Before Christmas. Or in June, with fresh interns. Good Lord, the medical community is hell-bent on killing me.

Surgical Consult

I saw the surgeon for my consult today. We sat down in the conference room and pulled up my last MRI on the huge monitor attached to the wall.




He flips through my MRI images, points to this tumor and tells me that this is the absolute worst location for a pituitary tumor; midline. If I have it operated on I have a 50% chance of losing all pituitary function, including ADH (antidiuretic hormone).

That'd mean my body would be unable to concentrate my urine and I'd be dependent on medication for the rest of my life, along with other medications to replace the rest of the hormones I'd lack for (I already am missing most of them). I happen to know a few people with DI and it truly is a horror-story disease. Insurance companies don't want to pay for the medications because it is so expensive, medications don't work all that well and it just haunts your day, all day - every day. I've read quite a bit about it, but none of it describes the daily battles my friends go through or the games insurance companies play to not pay for the medication. Ugh.

He tried to blame my 2-1/2 year headache on something neurological or optical, the typical shuffling off of the problem to another specialty or doctor to deal with. But I've been to the top of the food chain for specialists in Southern California and I called him on it. He supposed that I may have a small bleed off of the tumor area that is irritating my optic nerve (left eye) and research shows that even the smallest of pituitary tumors can cause incredible headaches. Size doesn't matter, for some reason the excess hormones they produce triggers intracranial pressure. The left eye problem leaves me unable to drive after dark and the constant headache is debilitating, fentanyl & morphine or not.

He recommended that I wait until things got worse, bad enough that 50% was a better choice.

I'm not sure if I want to know what is worse than wracked with pain, chronically bedridden, unable to keep up with just the dishes, properly care for my kids, or - you know - work in my profession & support myself.

Can this be any more of a nightmare????

He's supposed to call my specialist and discuss our meeting with him. And for what it is worth, he has been following my case for over a year through my specialist - so my MRIs and condition aren't new news to him and he's had plenty of time to review the films.

Maybe I am at a point where I am so invested and depressed that all I hear in regards to my case are the negatives, and perhaps my specialist will email me that I am on my way to surgery? Who knows. I don't know, but when I find out - I'll post about it.

In my email today:

The Star card suggests that your alter ego is the Goddess, whose superpower for rising to the occasion lies in your innate ability for inspiration. Pursue your dreams and what makes you happy -- life's too short. Allow time for you today. You may even get your fifteen minutes of fame by seeking recognition from others and striving to sparkle in the limelight. You are immortal! Sometimes it's better to burn out than just fade away.

Surgical consultation next week

I hope. I'll keep my fingers crossed. I have completed the cardiology appointment and subsequent scan at Cedars-Sinai. Also went to the ophthalmologist and followed up with the visual field test. Those were the final tests, folks. (Folks, as if anyone is listening - rofl!)

Cedars-Sinai tried to kill me. My standing pulse was running 111, I thought for sure it would go down if I just had a few minutes of rest on the cot. To my surprise it only lowered my pulse to 96. I guess that is what I gave up when I stopped taking Atenolol after the Stress Echos showed that my blood pressure was bottoming out and bottoming out worse while on Atenolol. Anyhow, the on-call cardiologist at C-S tried to give me 100mg right off the bat, but my bp was 100/60 and I drove myself there. Yikes. I talked them into trying 50mg but they weren't happy that it 'only' drove my heart down to 73bpm. WTH? After 100mg they couldn't give me nitro on the CT table because my bp was too low. Uh, duh!? 68/40 is quite low, you say? Then I had to get up and drive myself home to watch my two children. Good Lord.

I made my husband hire a babysitter. Mind you, this is a man that laughs in my face when I tell him I am sick and would not lift a finger to help me even though our children suffer for my inability to do what I used to do. Little things like, get out of bed every day - or - clean the house - or - do laundry to completion - or - make homemade meals and bake from scratch like I used to. :( I didn't give him a choice this time and it wasn't up for discussion. My chest felt imploded the whole drive home.

The visual field test showed a splatter of blind spots off on the edges of both of my visual fields. I expected it, as I bump into people that stand beside me in blind spots all the time. I work extra hard in traffic to check, re-check and look directly when I am changing lanes because I know I have a deficit. I didn't need anyone to tell me there is a problem, but at least the proof is there this time.

I wish I felt up to mixing in family posts with my updates. Here is a picture of my baby girl, she was Tinker Bell for Halloween and loved every moment. Every compliment made her walk even more dainty; she has the cutest 'dainty' walk and loves to play ballerina. I asked her to model her Tinker Bell outfit again today and she found it in a flash. lol

Angina or Ulcer?

I went to see the Cardiologist yesterday. I hadn't planned on taking my two year old with me, as I had asked my husband to watch her for this appointment so that I could try to answer questions correctly and without interruption. I don't care to get into specifics; suffice it to say he left me high and dry at the last minute. Ava's usual response to a doctor entering an exam room is screaming non-stop until they leave. I did my best to entertain her and talked to her about why we were there before Dr. Cardiologist came in. It helped some; she's very reasonable for two years old and I was proud of how well she did this time. :)

The end result of the visit: scheduled an EBCT, mostly because I have odd symptoms, odd diseases with odd presentations, and my specialist insists on it. He ran cholesterol bloodwork and routine bloodwork. I'll see him again next month. The EBCT will be done at Cedars-Sinai next Monday morning. I still haven't looked up what an EBCT is, I've done enough research on medical tests to last me a lifetime and for this I'm just going blind. I gathered enough to know it is about calcium deposits and that is enough. ;) If it were a two-day low-dose dex suppression with CRH stim, I'd be more into it.

He thinks that the angina-like discomfort I have could be an old ulcer acting up. Back in the mid-90's I had a bleeding ulcer that just wouldn't quit until I started taking Prilosec. At that time Prilosec was a new drug on the market and without insurance, ouch, it cost me a small fortune. Without it I couldn't eat, so I paid for it and it cured me. Could it be causing the pain that crushes my left sternum? goes to my jaw, collarbone, left arm, mid-chest? I don't know, but I'll go on the Protonix and hope it helps. Near-constant angina pain is annoying. Sometimes more than annoying, but as compared to constant headache and adrenal pain? Everything else only rates as a nuisance. lol

Steroids, It's Not Just For Breakfast Anymore

Ouch. Try going off steroids cold-turkey. Better off, don't - just say you did. Good Lord, help me. Yes, now I'm off steroids but I am also useless. With two under five and no Calvary on the horizon, these little things matter. ;)

NIH is OUT, Los Angeles-recommended surgeon is IN.

Cardiologist appointment is today. Visual field test is tomorrow.

If get through the cardiologist appointment today, on a wing and a prayer, I'll schedule a consultation with the surgeon.

My specialist says I need an EBCT scan. Do I look it up what that E stands for in conjunction with CT? I do not have it in me today. Oh, let us have it be a surprise.

Carney Complex

Over a year ago I predicted that I had a pituitary tumor. No amount of heckling, belittling or guffaws could get me to back down from my theory. I work in an industry where I engineer automatic building systems that work on negative feedback. On a superficial level, endocrine systems work the same way. If I had an entire building system fail, lights - air handlers - boiler - evaporative cooler - et al, I certainly wouldn't go around looking at each individual system endlessly; scratching my head like an idiot. The main controller is the first place to start, not the last place to check. That is precisely how it only made sense that either my pituitary or hypothalamus were at fault.

As sure as I was about that, is as sure as I am that this goes further than my pituitary tumors. My main suspect is Carney Complex.

57% female and 57% of the deaths are due to heart related causes. Nice little 'rhyme' there.

Quick read:

Spotty skin pigmentation lesions, such as lentigines (small, brown to black, non or slightly elevated, round or irregular) and blue nevi (large, blue to black, domed lesion) observed primarily in the face, eyelids, ears, and borders of the lips are the most common clinical manifestation of CNC (77%). Lentigines tend to fade with the age, usually after the fourth decade of life.

Myxomas are frequent lesions in CNC patients; heart myxomas (53%) occur multicentrically, and in any, or all, cardiac chambers; skin myxomas (33%) are detected in the eyelid, the external ear canal, the nipple, the oropharynx, the female genital tract and the female pelvis. Breast myxomas are often bilateral and present in more than 70% of adult women with CNC.

Psammomatous melanotic shwannomas , very rare tumors (10%), may occur anywhere in the peripheral nervous system, but most frequently in the gastrointestinal tract and paraspinal symphatetic chain.

Breast ductal adenomas, unusual mammary tumors akin to intraductal papillomas have been detected in 3% of CNC cases.

Endocrine lesions in CNC include testicular neoplasms (33%), primary pigmented nodule adrenocortical disease (PPNAD) (26%), growth hormone (GH) and prolactin-producing pituitary tumors (14%) and thyroid cancer (5%).

Long Read:

Pituitary involvement with Carney's syndrome was found in 11% of patients.

Adrenal glands:
PPNAD pigmented adrenocortical nodules ranging in size from submicroscopic to 10 mm in diameter, The adrenal glands feature multiple black and brown cortical nodules that contain large cells with pigment-laden, eosinophilic cytoplasm in the presence of internodular cortical atrophy. Furthermore, PPNAD is characterized by undetectable or low levels of ACTH.

The most common presenting signs or symptoms of Cushing's syndrome with PPNAD were approximately the same as those associated with Cushing's syndrome and included central obesity, weight gain, hirsutism, and hypertension in addition to other manifestations.

Computed tomography (CT) scanning of the adrenals was performed in 33 cases in the above study. The adrenals appeared normal in 45% and bilaterally enlarged in 27%. These data show that adrenal gland imaging of Carney's complex can be indistinguishable from that of other adrenal condition characterized by adrenal nodularity, which is frequently present in other primary forms of the Cushing syndrome and in normal elderly persons.

Heart:
Familial atrial myxomas, in contrast to non-familial myxomas, occur earlier in life, typically presenting in the second and third decades (average age 24 years).

Breasts:
In one study, breast myxoid fibroadenomas or other myxoid lesions were reported in 20% (19 patients) of females with Carney's complex, usually presenting as one or more discrete, asymptomatic mammary masses. Tumors were bilateral in slightly less than 10% of subjects (8 female patients). The breast lesions associated with Carney's Complex ranged in size from 2 mm to 2 cm in diameter. Mammary ductal adenoma was also found to occur in four patients.

Schwannomas:
Carney's complex involves tumors that affect peripheral nerves as well. In 1934 Bjorneboe first described these tumors which later became know as psammomatous melanotic schwannomas (Bjorneboe, 1934). The lesion was found to occur equally between sexes (Killeen et al., 1988). This particular tumor has malignant potential and four patients died as a result of metastasis.

I have tons lentigines in all the wrong places, cutaneous myxomas, undiagnosed adrenal-something on the MRI but normal CT reading, 90% of the time my ATCH is undetectable, pituitary tumor(s), large ovarian cyst, a breast lump-something that was diagnosed when I was 15 that hasn't changed in size [I believe they said fibrous? I cannot remember], and I really do need to see a cardiologist. I don't buy the two abnormal stress ECHOs and a Cardiac X diagnosis with all of these other problems staring at me. I think I need a 2nd opinion, ummn ok I have to have one before surgery anyhow or my specialist will kick my a**. I'll call tomorrow, I promise.

I AM the Evil Mother

First off, no one with a brain tumor on Fentanyl should be left home to deal with a two year old let alone a two year old and a four year old. Four days alone with them can drive you insane.

I'm trying to wean down off of hydrocortisone. Steroids are seriously hard-core drugs, you can take that from someone that knows hard-core drugs. I really have to reign myself in when people act nonchalant about steroid inhalers, topical steroid creams and, of course, oral steroid prescriptions. I know they are sometimes necessary, but I don't think people are given adequate warnings as to what they are doing to their very delicate HPA-axis. A month ago I was up to 70mg of hydrocortisone, equivalent thereof when I convert the bump of prednisone I added, and I've been coming down since. I immediately went to 20mg, once out of need, but had to stay at 20mg longer than I wanted. Last week I hit 15mg and, woe is me, today being Sunday and a fresh week - I tried out 10mg. "Looks like I picked the wrong week to quit amphetamines."

It has been a long day to say the least. Have you ever seen a Crayola Wonder "Mess Free" Activity Book? There is nothing more appealing to a parent than the words Activity mixed with Mess Free. I bought both of the girls a Crayola Color Explosion tablet. When you use the 'Color Reveal' markers, rainbow colors show through the black paper. As my 2 year old demonstrated today, pour Orange Juice on it and pat well: your child turns into the Teal Green Goblin! And so does your carpet. ;) Almost all of it came off in the tub, so it all turned out well. That isn't to say that the two beautiful troublemakers stopped there, that was breakfast time. Good heavens we had a fighting, screaming, yelling, biting, pulling hair, sassing Mother day.

Bedtime for the beautiful children? 5:45pm without one complaint which I suspect is based in pure guilt. But it still makes me The Evil Mother. :)

three lefts make a right

It is the funniest thing, if you aren't me. My left side is cursed. My left knee, left shoulder, left elbow, left hip, left eye all have something in common; pain.

I've had knee surgery and shoulder surgery, besides the meniscus and the bone spur osteoarthritis has staged an attack. Why only my left side? Who knows, I guess. At some point I'll probably feel blessed that it isn't bilateral. However, I am wondering how much longer my hip will hold out between the pain and the lower bone density it has vs. the right.

I'm in the middle of one of my "left-side" attacks. Wednesday night was the bi-monthly UCLA pituitary support group meeting and I try to make it to all of the meetings. This meeting's speaker was Dr. Heaney who has recently been stolen from Cedars-Sinai by Dr. Kelly of UCLA to join the UCLA team. I could listen to Dr. Heaney talk all day about pituitary tumors and the research he does on their origin. It was a good meeting. Ah, off-track again. I digress... for whatever reason, my neurologist says it is 'atypical migraine'. Whatever it is, driving after dark sets off a whole attack on the left side of my head. The oncoming headlights catch my left eye & trigger stabbing pains. It doesn't matter if I am a driver or passenger, btw, but it sure is easier to ride with your eyes covered than drive, lol. By the next day my left eye gets smaller, the upper lid droops, the lower lid migrates upwards. My face gets numb around my eye and the entire left hemisphere of my head feels thoroughly battered.

It really makes deciding to go to meetings difficult, for Lord knows I'm sure to be battling this for a week at least. Just like last time. And the time before that. Arg.

Imitrex. Morphine. Repeat.

...a picture is worth a thousand words...

The test underneath writes:

"Characteristic growth retardation and obesity in a 15-yr-old patient with Cushing's disease in comparison to her healthy INDENTICAL twin sister."

Isn't that amazing? Wow.

Problems and Options

As it stands, at this moment, I know the following things have to be dealt with:

1. hypopituitary - TSH, FSH, LH, GH (dropping), ACTH

Most likely because of the two tumors on my pituitary that are up against the stalk, and the congenital cyst inside.

2. Constant Headache, two and one half years and counting.

3. Double Vision

#2 & 3, I hope those resolve after pit surgery. Please, please, please please.

4. Chronic Flank Pain

I need to wait for Dr. Expert to return from vacation and tell me what the adrenal images mean. I've sent them to plenty of people but no one has responded, not even my doctor friends. Eek.

5. Unstable Angina

I missed my appointment on the 25th last month. Social Security (disability) called me in for an appointment without much notice and it ended up taking so long I was worn out and late - missed my cardiology appointment. However, I have no excuse but fear for not calling to make another one. Bad, bad, bad. My Los Angeles specialist says it doesn't matter if I have Cushing's if I drop dead of a heart attack. Very subtle. LOL

-----------

Pituitary surgery options:

I have been referred to surgery from my LA specialist to an LA surgeon, however I am waiting until next Monday to hear back from the NIH. I'd rather go to their facility and contribute to research on pituitary disorders, plus I need a work up on Carney Complex and that is another study I am qualified for enrollment.

Carney Complex is an autosomal dominant disorder, so it takes top priority as my girls might have it also. They both have the same sort of lentigines that I have. My oldest daughter is popping them up everywhere but hasn't even one solar freckle. It freaks me out.

Adrenal Images

A few weeks ago it occurred to me I didn't need to beg someone for an adrenal MRI, I already have one! D'oh. Never mind me while I make this into a long story. ;)

I had one done earlier this year: I went to the ER for severe flank pain. Ok, wait. Actually, I went to my regular doctor for severe flank pain. He said it might be appendicitis or it might be a kidney stone and he ordered a scan; an ultrasound if memory serves me right. That night I couldn't get out of bed and my husband was being less than understanding. He was solo on child duty and told me if I was in that much pain I should go to the ER. So I did. Mistake!

I ended up with an appendectomy. I have lots of scar tissue and a hernia repair done at my belly button, so the surgeon and I talked beforehand in the OR and agreed that he should avoid the mesh. I don't respond well to most pain medication, specifically demerol and vicodin plus most others don't affect me as they should. I don't know why and I've been like this all my life. I've been given the 'druggie' eyeball by doctors before, but it isn't like that. Heck, I took 45mg of morphine before I went to the ER and I was still hurting so bad that I went in. All that did was paralyze my intestines and make me look like a junky to the surgeon. *sigh* I came out of surgery in my usual style, which includes waking up screaming in pain. I swear all of the surgeries I have and come out of in such severe pain, because they don't know and don't listen to me at all about what pain meds work and what don't, these surgeries are seriously messing with my psyche. :( My ever-so-helpful [NOT] husband kept telling them to give me more morphine. Umm, the drug that wasn't helping me so that I went to the ER to begin with? Until they gave me so much I almost stopped breathing. Nice.

The internal meds doctor on call caught ahold of my labwork and freaked out. My 8am cortisol level while I was in the ER was 1.1 which is quite low, but normal for me. A normal range is 8.0 to 23.0. He immediately put me on 60mg of Prednisone and 60mg of hydrocortisone. I haven't been off steroids since. Since I can't get off steroids I am done with any cortisol testing that really means anything. Nice.

Since I came out of surgery still in the same pain I came to the ER with, it seems I didn't really need the appendectomy after all. I left the hospital after a 7 day stay with a card for a pain management doctor's office and a date for nerve block surgery.

In the meantime, during my stay, the pain mgt doctor ordered an MRI. It was this thoracic MRI I realized, just a few weeks ago, might hold some pictures of my adrenal glands that could shed some light on my constant pain (now on a fentanyl patch) and final diagnosis.

Here they are:

But you look so good Part II

I had to laugh when I caught a glimpse of my last post. Just yesterday I took my youngest daughter to her pediatrician for an emergency visit, not that I am laughing about that mind you. She is on the BRAT diet for a few days and seems better already, thankfully. The nurse I love asked me how I was doing with my pituitary problems then ended the conversation with the inevitable, "but you look so good! At least you feel good through this!". I just smiled and said it was the steroids I am taking. I didn't mention that I had to stress dose twice just to get the energy to get there to their office for the visit. What's the use? *just smile, Lisa, just smile*

"But you look so good!"

What kind of planet are these people from? They know I have tumors, they know my adrenals have shut down, they know I am in constant pain w/ Fentanyl to get me through the day without chanting "please let me die", and even with it the breakthrough pain is pretty bad. The hydrocortisone, fat chipmunk cheeks and constant sweat dripping down my forehead keeps me looking 'robust' I imagine. Nonetheless it is enough to make me want to scream.

"Oh, you had to literally crawl up and down the stairs last week because you were so sick. And no one cared so they left you home with the 4-1/2 year old and 2 year old. *tsk tsk* "But you look so well!" Excuse me while I use my very last bit of energy to wrap my hands around their neck. Punch them in the chest so they can experience some of my unstable angina. *Good Lord give me strength!*

Hijacked

Another Cushing's patient says it better than I can. I'm fully going to blame the tumors for this, as I used to be an honor student with one horribly disgraceful B+ in elementary school. My vocabulary has eroded over the years, my grammar slipping and sentence structure crumbling with poor punctuation, and intervals spaced with periods that would make my public relation's writer mother-in-law have a grand mal seizure.

I digress. A post on being hijacked by this disease, doctor name abbreviations changed to protect the innocent ;):

"....I sorted through old photos to take with me to my initial apt with Dr. X. There are not many pictures of me during the last 6-8 years, but a few. It was sad, yet confirming to see the pictures of what I looked like before Cushings hijacked my body. I had one of those pictures on my desk because I wanted to keep it in front of me to remind me of what I will someday look like again....or at least reasonably resemble! My best friend for the past 8 years, said "who is this"? I thought she was joking, but then quickly realized she wasn't....that's when I knew how drastic the change had been on my appearance and how easily & naturally I still thought about myself the way I was BC.....(before cushings).

Little did I know that there would be another opportunity to be "gutted" when I took all the pictures I collected to my first apt with Dr. X a couple of weeks ago and heard him say repeatedly, "is this one you"? AHHHHHHHHH...that hurt!! I wanted to scream, "...OF COURSE THAT'S ME....ARE YOU BLIND? HOW CAN YOU NOT TELL IT'S ME?" Then I heard the comment, "yes, I can see there's been a dramatic change."

I have found not being recognized is more hurtful than being glared at by other women in public. It felt like not being acknowledged.........not being recognized felt like the person I loved had died and others had forgotten. How could my self-image be so tied to my appearance? In some ways I think I've learned that there's a part of me that refuses to let cushings move in and become a permanent part of my life. It's not me. It's a temporary intruder that has cleverly snuck in to my life and made it's presence known so slowly that others do not realize I've been taken hostage.....that I need help.

I used to trust doctors to do what was in my best interest. When I lost that trust, I began to trust in the medical tests despite the competence or disposition of a doctor. Now I've even come to realize the variables that impact and can skew tests.....didn't put the purple tubes for ACTH on ice fast enough, didn't spin it soon enough, forgot to send the right paperwork for me with the samples....then I learn that the test results may not reflect my condition because my body is producing the hormones on a cyclical basis. So, I have learned to trust myself above all others and above the science behind the tests.

I've listened to 5 endos at highly ranked facility tell me nothing was wrong, I should see a psychiatrist, stop eating so much, exercise vigorously,....yet knowing in my heart they were wrong even if I was too exhausted and weak to speak up for myself.

Then I learned to go into a doc's apt with a plan, just like a business meeting....."what do I want to walk out of here with?" is the question I asked myself. I learned what kind of information to share, what to leave out as I grew confident of what information was relevant and how to "tell my story" in a concise, factual manner. I developed my own "dog & pony show" as we call it in my company, complete with charts, pictures and a rehearsed speech. I learned how to "fail fast" in my relationships with doctors as my ability to sense their level of comfort and competence.

I have grown in ways I never expected or intended. I often wonder if I'll ever need to fight like this again. I question what the "life lessons" are that I'm supposed to be learning.....as I don't enjoy repeating painful lessons. smile.gif I feel like I've had to endure "Olympic style" training in order to physically and mentally survive, yet what is the purpose of these skills once I'm on the other side of this fight?

Will I ever be "nice" again? Will I be able to trust others with my well being? It's brought out an ugliness in me that I don't like. I don't like having to use my energy to pound on others to send me copies of my test results, make sure the insurance company pays for my benefits, assert myself with office staff to make my next doctors apt soon because I'm an emergency!

This disease has not only stolen my appearance, but held me at gunpoint to be fighting for myself on all fronts. I don't like fighting, pushing, raising my voice so I can be heard by doctors that are lending a deaf ear to my cry for help. It's hard enough being sick and it's not in my nature to be pushing all the time. I want to be the peace-maker, nurturer, the one with the infectious laugh that brightens other peoples day....that leaves them feeling better than they did when they came in. It seems like a luxury now.....and one I will not take for granted again."

Oh, all of that hits home with me. I am living it. One day I hope to write a manual, or at least a pamphlet, to help other people with rare or hard to diagnose diseases navigate the medical system.

Here are my tumors, aren't they delicious? Rathke's Cleft Cyst at the bottom.

Here I am. Here is me. On the left, that was me in 1998. On the right, that is me just a few weeks ago.


Yikes, what kind of nasty disease does such a horrible thing to you? Can you imagine thinking you still look like the left image and catching yourself in the mirror once or twice a day, SURPRISE!?!

Ok, so what is Cushing's Disease?

Cushing's syndrome occurs when the body's tissues are exposed to excessive levels of cortisol for long periods of time. Many people suffer the symptoms of Cushing's syndrome because they take glucocorticoid hormones such as prednisone for asthma, rheumatoid arthritis, lupus and other inflammatory diseases, or for immunosuppression after transplantation.

Others develop Cushing's syndrome because of overproduction of cortisol by the body. Normally, the production of cortisol follows a precise chain of events. First, the hypothalamus, a part of the brain which is about the size of a small sugar cube, sends corticotropin releasing hormone (CRH) to the pituitary gland. CRH causes the pituitary to secrete ACTH (adrenocorticotropin), a hormone that stimulates the adrenal glands. When the adrenals, which are located just above the kidneys, receive the ACTH, they respond by releasing cortisol into the bloodstream.

What are the symptoms of Cushing's Disease?

Symptoms vary, but most people have upper body obesity, rounded face, increased fat around the neck, and thinning arms and legs. Children tend to be obese with slowed growth rates.

Other symptoms appear in the skin, which becomes fragile and thin. It bruises easily and heals poorly. Purplish pink stretch marks may appear on the abdomen, thighs, buttocks, arms and breasts. The bones are weakened, and routine activities such as bending, lifting or rising from a chair may lead to backaches, rib and spinal column fractures.

Most people have severe fatigue, weak muscles, high blood pressure and high blood sugar. Irritability, anxiety and depression are common.

Women usually have excess hair growth on their faces, necks, chests, abdomens, and thighs. Their menstrual periods may become irregular or stop. Men have decreased fertility with diminished or absent desire for sex.

My first symptoms started in childhood. My weight fluctuated for no reason, to the point that I did not know what I looked like. From day to day my face bloated and thinned out. It was enough to drive a young girl crazy. I used to have dreams where my body would expand and contract; like a balloon being blown up with the air let out, repeatedly. I had tons of symptoms, but our family doctor blew them off and convinced my parents that I was a chronic complainer. lol.

In junior high every other girl had long since started their period. I hadn't. By tenth grade it started to bother me. In the summer before 10th grade I started getting deep purple stretchmarks. They ran down my hips to my thighs, across the sides of my breasts that had just barely grown. I didn't dare say anything to anyone about it. I used to sneak into my mother's room and use her precious bottle of pure cocoa butter on them, praying that they would go away.

That is how my Cushing's Disease started, twenty years ago. It wasn't until last year that a pituitary tumor was found during a Dynamic MRI at Cedars-Sinai. On July 31st this year, Portland's OHSU found a pituitary tumor and and congential cyst in the center of my pituitary. A second reading of my films showed two tumors and the cyst. I fear a third opinion. ;)

Last night my Los Angeles endocrinologist/specialist offered surgery for the second time. Last year he offered up surgery and I missed the boat. I've been kicking myself ever since then. I'm crazy excited today and I am seeing the light at the end of this long, long tunnel. I need to keep this journal. I need to keep my head on straight to guide me through surgery and recovery. Someday I just might be well for the first time in my life and I have to know where I came from to appreciate it.