I wonder if he wonders why

I spoke to my general doctor tonight about my CSF leak test results and headaches. The test came back clear, but it is likely that I periodically leak if I am too active, blow my nose too hard, if the wind blows in the wrong direction, or if my husband pisses me off. [I could be kidding about one of those.]

We talked about his septum surgery, neti pots, saline rinses, nose-blowing, kids. I love my doctor. :) He mostly sees Veterans at his clinic and I really appreciate him. I hope he knows it.

Oops, where was I?

Oh yeah! At some point I had to confess that I had thought about the potential for a leak and decided that no matter what the results, I do not want or need any additional surgery in that area of my body ever again. So I'll live with it, try to take it easy and have it heal up on its own.

He asked me if I wanted any pain meds. I hmm'd and haww'd because, dammit, it really does hurt. My headache, that is. You know you are not an addict when: the thought of opioid-induced constipation makes you come to your senses and say, "Uh, no. That's ok."

He either thinks I am a pain loving super freaking hero, or that I am stupid. One of those. ;)

Happy National Cushing's Disease Awareness Day

Today is National Cushing's Awareness Day.

I asked a few local papers (ok, is LA considered "local"? ) to do an article about Cushing's. Long story short, only one Cushing's article in the entire USA, that I can find.

Regardless, in doing my search I found an article about a new veterinarian in-house lab machine that will do endocrinology tests while the patients wait.

MEANWHILE, I had my post-op gallon of blood drawn on Saturday. It is Tuesday and I am checking my online fax account for the results umpteen times a day.

Allow me to repeat this, because I do not have enough swear words in my vocabulary:
VETERINARIAN IN-HOUSE LAB EQUIPMENT - PRODUCING RESULTS FOR ANIMALS AND THEIR OWNERS WHILE THEY WAIT

I give up. I give up. I give up.

I'm going to schedule an appointment with a veterinarian. Canine cushing's disease: They get all the press, no one doubts them and now they can get their results in the office. WTF???

I'm a big weaner

I've managed to wean from 60mg of hydrocortisone to 5mg in less than 7 days! Yay! Yippee. Actually I think I could have skipped the 5mg today, but I took it just for the hell of it. One tiny ping in my adrenals and I dropped 5mg, you know, just in case.

Ok, honestly? That isn't good news in the Cushing's world. My body shouldn't be able to compensate for that rapid of a drop in steroids in such a short time. I suppose it means that the source of my Cushing's is finding a way to come back to life. *fingers in ears* LA LA LA LA LA

I saw my primary doctor's assistant today to see if they could order a radiological test to find the source of my leaky brain. They were pretty stumped. I don't want to bug them, but they haven't called me yet. They said they would. I'm afraid he'll drop me as a patient if I keep 'coming down' with questions and problems that they can't deal with. I really like him and his staff; I try to send them goodies for the office whenever they help me through situations like the one I am dealing with. He must really like people with strep or UTIs after my visits. ;)

I made an appointment with an ENT surgeon for Monday, just to cover my bases. Once again, I hope I don't scare the bejeezus out of him and have him send me packing. It was already suggested that I go back to Houston for the leak repair. No. Freaking. Way. is my non-verbalized response.

I know a neurosurgeon in Los Angeles, but I don't think a neurosurgeon is needed for this - more an ENT problem. I think. I don't know. Which brings me to my most recent thought: wouldn't it be nice to just have NORMAL PROBLEMS? How about that osteoarthritis I have in all of my left-side joints. Couldn't that be my only cross to bear? Wouldn't that be nice?!? I try not to talk about my medical issues out loud to normal people any more. I scare them. *ducking*

I've had it. Up to my neck, err - brain.

My brain is still leaking. Dammit.

"My brain is leaking! My brain is leaking!"

Friday will mark four weeks since surgery and I am spitting mad that they didn't believe me when I told them I had a leak at the hospital. Sorry, dear city of Houston, I don't think we have a future together. We don't seem to mesh well. ;)

I've tried laying flat on my back every night when I sleep and it isn't helping. Can I just say I deserve a medal for even sleeping like that for one night, let alone four or five? Yes, I do. I've had to resort to sleeping on the couch so that the width of sleeping area discourages my sleeping self from turning.

I'm trying to arrange for the radiological tests to determine the exact location of the drip, and have an appointment with an ENT/surgeon on Monday. There is no way I am going under without them knowing exactly where to stick in a plug. *sigh*

I started the daily growth hormone shots seven days ago. It is really starting to help. Now I can almost actually lift my feet when I walk. Yeehaw!

I'm making an effort to get off of the steroids so that I can start testing again soon. I managed to drop from 60mg of cortef to 20mg in less than five days. *blowing on knuckles, rubbing on chest* Holy wean, Batman! :)

Life Is But A Dream, Sweetheart

I've had a perfect weekend, and perfect day today. Everything is going so well that I am afraid I'm going to die in my sleep tonight. *blink* Just kidding!

Husband came home last night and cleaned the whole house! He mopped and did other stuff I can't do right now. :)

My mother-in-law came over this morning and watched the girls while I napped. Then our babysitter came over this afternoon and I went out to do my stockpiling. I know I should rest, but honestly being home listening to screeching children tends to be more stressful to me.

I cleaned up at CVS and Ralphs, with free razors and toothpaste and I found lint rollers for freeeeee (after the coupons I happened to have on me) on clearance at Ralphs. My second trip to the grocery store did me in though, as I forgot to get the rabbits their food and veggies and I started bleeding out of my nose. Oopsy. Ok, ok, ok - I'll slow down.

I'm just afraid that this "feeling good" won't last and I want to spend every minute "doing" and "going", because it has been so long since I could. I'll rest tomorrow.

My growth hormone starter kit should be here tomorrow or the next day. I can't wait to start!

Life is rolling along!

Back from MD Anderson

Surgery went well. 

I'm home now, resting in bed. Physically I feel much better than before surgery. I can walk and go upstairs with ease. I feel very well except the head hurting. 

With Cushing's Disease it is not necessarily good news to feel so wonderful post-op, but I am taking it as confirmation that I can't take my six week reprieve from Cushing's lightly. The last time I had surgery I had a wonderful 6 weeks of feeling great, so I am presuming the same this time.

Shame on me for coming here with great news and turning it towards the bad, but I am going to get off of hydrocortisone as quickly as I can and get to testing my adrenals. I still suspect Primary Pigmented Adrenal glands or abnormal hormone receptors in the adrenals .

I still needed this surgery very badly, as the surgeon (who ROCKS, btw) found a pre-existing CSF leak in the sella area. IMCC couldn't find the exact "hole" so he used a liberal amount of "brain spackle" to seal it up. There was a whiteish lesion on the left side of the pituitary and he removed a lobe because it looked funny. The leak makes sense, or lack thereof now, because my ears no longer ring 24/7 and my left ear has stopping hurting. Pathology returned as normal, like last time. He doesn't know what the lesion was, but I am glad to be rid of it.

My 3 day post op numbers were excellent: 4.5 cortisol and ACTH at the bottom of range

After they removed the nasal packing I had some sort of leak, but with the brain spackle they say it is isn't possible for it to have been from inside the pituitary area. Laying flat helped, taking Aleve helped get my pain down & wits back.

Quite a few of us Cushing's patients have had problems with the nursing staff on the Brain & Spine floor.  I want to band together and write a letter reaching out to the Pain Specialist department and see if they will work in conjunction with IMCC's office to take pain management out of the nursing staff's hands for the first two days after surgery. We have to do this so that future Cushie's don't suffer what we have been through.

If I were given the option of living through those first two days again, or death - I'd ask for stationary. Because I need to write some letters before I go. It was that bad.

For Cushing's there is a hormonal interaction aspect with pain management and I believe we may have to step up for Cushing's at MDA, since their expertise is cancer. I want any one that goes to IMCC, such an excellent surgeon, to not have to encounter difficulties in the post-op period.

Anyone that makes me cry for 8 hours, laying flat with my mouth open and tears streaming out of the corners of my eyes, and makes my father cry with me usually gets special attention in the letter department. This time, however, an intervention & new alliance between the Pain Specialist & future Cushie patients may have the best impact.

The pain issue goes beyond your average nurse and hospital stay problems, I feel. I think that having Cushing's our incredibly jacked up hormonal system creates problems that cannot be anticipated. I have researched the underlying reasons for this previous, finding that the precursor hormone to ACTH also makes peptides that are involved in beta-endorphins.

Link: http://www.vivo.colostate.edu/hbooks/pathphys/endocrine/hypopit/acth.html

Right after surgery I would have to expect that our pituitary is "shocked" at the insult of being toyed with and that ACTH is low or none; of course being low due too a lack of the precursor hormone:

"The major attributes of the hormones other than ACTH that are produced in this process are summarized as follows:

Lipotropin: Originally described as having weak lipolytic effects, its major importance is as the precursor to beta-endorphin.

Beta-endorphin and Met-enkephalin: Opioid peptides with pain-alleviation and euphoric effects.

Melanocyte-stimulating hormone (MSH): Known to control melanin pigmentation in the skin of most vertebrates."

To me, it makes sense like nuts and bolts, if you keep throwing nuts at a problem it won't fix if you do not have any bolts to hold them. Am I making sense? I'm trying to say that people enduring pituitary surgery need specialized management for their pain. This should be recognized and dealt with by removing nurses from the decision role in immediate post-op pain management. 

And the nurses. Arg. They wouldn't even let me talk to a pain specialist OR the neurosurgeon on call, I begged them. I had to call the hospital and have him paged to call ME directly. Then everything changed. It was a complete blockage by the nurses. And they just kept closing my door so no one could see me in there crying. Nice. Real nice.

When the doctor got ahold of them, after our conversation - if you call weeping silently and barely managing to speak a conversation - the nurses were reamed for a few hours. Yowza! They came to me and yelled at me for calling the doctor. "You do not call the doctor directly!" and "How did you get his number?". *"Uh, because I am smarter than you are - bitch. Ding. Ding. Ding." After interrogation, I ended up crying out like Richard Gere in "An Officer And a Gentleman", with spittle and tears and raw emotion: "That's not my problem! That's not my problem!"

I know they felt that I was extra whiny. Extra dramatic. But in reality Cushing's Disease calls out for special monitoring and treatment by qualified medical doctors that can treat us.

I got the diladid pump after I managed to see the Pain Specialist, but it made me retain my urine. They refused to put a catheter back in, but also yelled at me for being up (to attempt to go pee) because I was pouring fluid out of my nose (and I did the halo test on a paper towel - very positive). I would have to get up every hour, go and sit & sit & sit forever on the potty and could NOT go. I also had an IV drip going, which added to the urine that was accumulating. Thank heavens for a friend that called the charge nurse for me. My regular nurse refused to check my bladder with a scan, she insisted that I wait another 6 or 8 hours!!! The charge nurse made her do it, and when it showed urine, the nurse did a straight catheter. A whole liter came out. !!! Even then I wasn't empty, I could tell.


My surgeon's assistant took me off of the diluadid pump and put me on Norco. By that time the pain wasn't as SEVERE as it had been, that spike going through my left eye like it had been before, so I was ok with it. They showed up every 6 hours to give me the Norco no problem. When I found out that I was getting out the next day, and after my mandatory lay flat day, I got up and took Aleve without telling the nurses. I was too scared of them to take it without telling them previously, but by then I didn't care. Aleve took away 100% of my pain, go figure.

Anyhow, back to bed for me. My head hurts, but in a good "healing" sort of way. :)

Here we go again

I'm off to Texas this coming week for a second surgery. This surgeon is more "aggressive" than the first one and I expect that he'll remove half of my pituitary gland. I'm a bit nervous, but like most things medical I try to keep myself from thinking about it until it comes time.

My specialist prescribed Ketoconazole to halt my overworking adrenal glands, I flunked the growth hormone stimulation test, and I have cataracts on both eyes. I'll be happy to be off of the Ketoconazole and replacement steroids. Thyroid tests showed that T4 to T3 conversion isn't working, so there are two meds for thyroid replacement now. I'm a bit insulted that my thyroid has decided to gang up on me, on top of the panhypopituitarism.

Pfizer is working to get the growth hormone meds approved and I can't wait to start on it. So far the diabetes insipidus symptoms are somewhat manageable without medication, and I hopeit stays that way. I'm realllly sick of the constant pee breaks. If you have to think of where the bathrooms are before you make a trip to buy groceries or take your kids to the park, and you aren't pregnant, you have problems. Walking is difficult and I always wonder if I'll get shot looks for being lazy if I were to grab a motorized chair in the stores. I haven't done it yet. I'm too chicken. Instead I plod along and sweat through my clothes, my hair soaked. I don't know why I care more about what people think than how I feel.

Regarding surgery: If the tumor wasn't right up against the ICA and showing on my field vision tests, then I'd prefer a good ol' Bilateral Adrenalectomy. *sigh* Maybe radiation and a BLA are in my future, but not just yet.

:)

Damn you, Cushing's Disease

How to disappoint all of your friends, relatives and two young children: Spend a month recuperating from pituitary surgery in June - and not have your remission stick. In September I realized that the Cushing's had returned, if it ever left, and I'm just now ready to admit to it.

Thankfully the road is paved already and my specialist is recommending a repeat surgery. Tomorrow I go in for a pituitary MRI and with any luck we can find the source. The abdominal MRI from two weeks ago shows normal adrenal glands. In a twisted way I was hoping that my adrenals were the culprit, even though it goes against all likelihood. Removing the adrenal glands would almost certainly put an end to the Cushing's misery. I'd take Addison's back with a warm heart, since my weight has ballooned beyond all recognition. (I avoid mirrors.) They discovered a cyst on my spleen and liver during the abdominal MRI. No one has addressed those findings, so I don't know what to make of it. A quick Google tells me that it is rare, ha - who knew?, and LORD HELP ME, I now know what 'non-parasitic' means. *la la la la la la* (fingers in ears)

I have some more blood testing to do at midnight, which runs a close second to my most despised form of testing torture, and the MRI tomorrow. As far as symptoms go, weight gain - cystic 'steroid' acne - flank pain - and very recently a deterioration of the vision in my left eye (again). The double vision is slight and I only get migraines once a week or so. My bout with constant 24 hour a day headache & migraine has ruined my ability to describe pain. I cannot fathom calling the first surgery a 'failure', since it single-handedly took away the constant headache.

I'm rambling and it is late.... I'll be back with MRI results soon.

The Aftermath

It has been two and a half months since surgery and about time for an update.

I've had my post-op blood work and visit with my specialist. It was basically a review on, at 8 weeks post-op, how the (hormonal) chips had fallen so far.

My pituitary is making ACTH hormone in the mid-range and my adrenals are responding. This is terrific news, coupled with the fact that I did not sustain any damage to my posterior pituitary or ability to produce ADH. I have no secondary adrenal insufficiency or diabetes inspidus, the two life-threatening hormone deficiencies. Very cool!

However, my levels of FSH, LH, TSH, IGF-1, Testosterone and DHEA are all very low. For now we are implementing testosterone gel, estrogen gel, DHEA and maintaining my thyroid medication. My T4 values are good, but my body doesn't seem to be converting T4 to T3 like it should be doing.

I'll do more lab work in 6 weeks and we'll see if I need to start growth hormone and T3 medication.

I have to say that losing my 2-1/2 year headache after surgery was more than I could ask for. Life without a headache is still so amazing to me that sometimes I just sit still and enjoy 'being'. I used to try to meditate in an effort to control the pain. Everyday I would practice and never quite get there. I think all of that practice was good for me and I find it easier to drop into relaxed state now.

Being hypopituitary isn't something I'm thankful for, I still wish I hadn't been put off for years and the damage would have been avoided. But it is a heck of a lot better than panhypopituitary, untreated hormone deficiencies, and a 8/10 headache. :)

I'm doing well.

10 days post-op

This morning I woke up without a trace of a headache. For someone who has had a headache for 3 years and 2 months, this was a major event! I laid still for two hours, not daring to move a muscle in case it wouldn't last. Two hours of silently rejoicing over something most people take for granted, I suppose.

It didn't seem so pathetic until my headache crept back later this afternoon. *sigh* I guess progress has to start somewhere.

I'm really hoping that this intense and focused headache, somewhat different from my previous resident terror, is from The Scab. It hasn't fallen out yet. However, I am reluctant to wish for something so miraculous. There is the possibility of analgesic rebound, but very slight at this point. I go long periods between morphine dosages without problems at home. I'm waiting until I am stronger and more stable to discontinue the Fentanyl patch. There's no payoff with it, as I do not feel it when I am wearing the patch but I can only presume that stopping cold turkey would be stressful. I'll ask my specialist for his advice on that one.

I wish I was as overjoyed as the last time I posted, but hey - headaches suck! ;)

Six days post-op

morning of day 6, talking to my girls:

I think I have discovered the reason why people go downhill at 7 days post-surgery.

Today friend, and fellow pituitary Cushing's survivor, Robin forewarned me that around 10 days after surgery the big ol' scab that is lurking in my sinuses will fall off. Yeowza! She casually mentioned that it gagged her. Oh, I can't wait! Eww.

It's day 6 and I am out of Percocet. Is it any wonder that other people get worse at day 7? I don't want to go on one of my tangents about pain management, but that is just silly. By the time a Cushing's patient has made it to treatment they've endured plenty of pain; joint, muscular, headache, and unrelenting pain that people can't imagine. Excess cortisol breaks down your connecting tissues, and for some related reason the pain of anything knocking into me or even a normal bump into a wall hurts like you could not fathom. Giving someone 7 days of pain medication and telling them it'll take 2-3 weeks for the surgical site to heal is plain mean, imho. I'm a walking pharmacy, so I have fentanyl and morphine to fall back on but I didn't want to go back to those medications after my cure. I despise morphine, but had to break down and take some tonight. I have a huge scab in my sinus cavity, of course my head hurts like hell. A steamy shower only provided temporary relief.

I can't wait to choke on that scab. ;)

5 days post-op

I'm healing up very nicely from surgery.

My nose is getting better. A shower or a little time over a steamy pot is enough to loosen things up and make me comfortable. I think that if my biggest annoyance with brain surgery is a little swelling and stitches in my nose, well then things went very well indeed! I need to send the surgeon, Dr. Jho, more than just a thank you card. I'll have to find a way to ask Sid at SurgeonsBlog what would please a surgeon the most from a patient filled with gratitude. Dr. Jho specialises in minimally invasive techniques for Brain and Spine surgery. He can do surgeries that do not exist to fix problems that are taken for granted as permanent elsewhere. His innovations, dedication and attitude impressed me to the core and I have searched the continent for 'The' Surgeon. His post-op morbidity statistics were the best I have seen.

I pay strict attention to the Diabetes Insipidus statistics, both transient and permanent, for a few reasons. Foremost, because I know a wonderful woman who has a bad case of DI and lives attached to her nasal medication. The ongoing horrors she endures to ensure she has adequate medication, medical coverage w/o pre-existing condition clauses, she ends up w/ Rx's that do not last as long as they should (due to medication distribution inside of the dispenser), her insurance regularly rejects paying for the $1000+ a month med. That alone is enough to feel constantly at war. I think of her often and what she endured, not just now, but to get to her diagnosis.

People with instances like hers opened my eyes to the life that begins after the cure, and I decided to do my very best job guiding my post-cure life before I got there. My key was the very best minimally invasive surgeon I could find on the planet. I found him, with much help from selfless and thorough Cushing's advice source MaryO'Connor's Cushings-help.com. Mary is my true hero in the lifelong Cushing's story of my time. Without her dedication to Cushing's and helping other people find help, I would be somewhere today lost & still fluttering in the wind, undiagnosed or probably dead.

Mary runs her site out of pocket, so please stop over and donate to her cause, I use Paypal. :) She is truly and directly saving lives with her work, and at the very least should not have to be worried about money to keep the place going. She also has health issues of her own that I would prefer to see magically disappear and fix, for Mary deserves a long healthy happy life with Tom. :)

The Start Of My After-Cure Life:

Dr. Jho took the entire tumor without disrupting the pituitary gland. He advised to leave the Rathke's Cleft cyst inside of the pituitary alone, for the same reason: "preservation"

Today is post-op Day 5, so we arrived to do my blood work this morning @ our local hospital. The lab technician was very well-schooled on ACTH draws and it turns out that their lab computer system is well-programmed. Normally I have to have fight to get the tech to ice the drawn immediately and spin it down right away. Their tag system had it on the vial slip. That was refreshing! And here I am in rural NY, they did it right. Go to Los Angeles and see who you have to throw down the hall to get a glove baggy of ice and a centrifuge. LOL

I'm trying to sleep more, so I am off to bed. I wanted to show the progress with the facial swelling. My dimple areas are back (true dimples, not those fat folds LOL) and I can feel my cheekbones coming to the surface. My legs are still swollen and such, but I'm being patient. Just trying to take it easy and get better. I still have a slight headache and my vision hasn't recovered much more, but I think it'll just take time. Swelling and whatnot. ~ Lisa

11 days pre-op. Normal, mid-swollen.

5 days post op:

Eyes are open, cheekbone pads aren't thick. Natural dimples coming back to thinned out face.

Dark picture, but just shows more of the same. Those years I aged are falling off (thankfully!) so maybe I won't look so matronly anymore. ;)

Tumor Free

It's two days post-op and I'm doing absolutely fantastic!

They took me in for surgery at 2pm on Thursday, kept me in ICU until 10pm (my left eye wasn't working, but who can blame it? It just lost it's tumah pal), spent the night in a private room and they discharged me yesterday at 10am.

We drove up to my Aunt's house to Niagara County in NY from Pittsburgh and I'll be here until tomorrow morning, then on to my Dad's house to stay until things heal up.

The surgeon says that the tumor was bigger than the MRI showed, he clearly and firmly stated that they did get ALL of it and he left the cyst to preserve my pituitary untouched. He felt it was best that way.

I can't disagree, since I feel really well. They had to cut the thin section in the middle of my nose to get more room to maneuver, but that should heal soon. I can't blow my nose for two weeks and I have a lot of drainage going down the back of my throat, but it's getting better. I just moistened up the overnight clogging and cleaned out my nose with saline gel when I woke up. Ewww.

The most surprising and wonderful thing I have to share is my new heart.

Before surgery my heart was a mess. Bumping, thumping, doing backflips and acting up all the time. The very first thing I noticed when I woke up in the recovery room was that I couldn't feel my heart beating. All of a sudden it was just humming along smoothly, like a heart should do I suppose. Up, down I went for a glasss of water this morning on the steep stairs here, and there wasn't even the slightest complaint or bump out of my new heart. It is the most amazing thing I have ever experienced beyond childbirth.

I am happy, happy, happy!!!!!!!

I'm back, with good news

I know I've been gone a while. I won't count off the months and days.

My father-in-law passed away after my last post. Cancer is a terrible thing. It was his second bout with the disease and, sadly, he went undiagnosed until Stage IV the second time around. Chemotherapy gave him some extra time but it didn't seem to be enough. I really miss Bill, and I always will.

On the way home from his funeral ceremony, which was delayed a few weeks for the Christmas holidays, I got the news that my mother was taking a turn for the worse. I flew out and drove straight to her beside getting there just hours before she drifted off into a coma. I never did get to talk to her in any meaningful sense, but that has defined our relationship for 36 years. I think its more difficult to lose a parent you weren't close to sometimes, at least initially. I spent the rest of my trip fighting an adrenal crisis that wouldn't let go.

Meanwhile the surgeon that will be performing my pituitary procedure insisted that I undergo an Inferior Petrosal Sinus Sampling. That's fine, as it assists him when mapping out an approach for surgery. However, arranging for the IPSS was another story. It took from the beginning of January until March 28th to have it scheduled, confirmed and carried out. Then another three weeks for informal results, four weeks for the official ones with a report.

I am happy to say that I do have a date for surgery: May 24th and I do have hope that this Christmas I'll feel better.

I seem to tick off years at Christmas, thinking 'Last year I thought I'd be better by now'. Maybe this is my year.

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. :)