Tuesday, December 08, 2009

4 paragraphs to explain my disease plus: Love a Puffin, Masturbate and living.

I have just finished a blog post on the Postcard Project,in fact there was ANOTHER postcard project blog before that which I kind of forgot to mention, though I think is it a good post.

“To engage, to start a dialogue and get to know someone is a great joy, a great opportunity. I have been given this chance to get to know so many people…

..But I can make you feel a little less lonely, because I care, and I hope it shows, in a clumsy way, in the postcards I send.”

So please, if you want more Elizabeth, head over there and take a read (and a comment). Also, scantily clad Hawaiian girl if that is an incentive, plus lovely goth postcards! Linda has also written part II of the Flowers of Hawaii over at Girl’s Gotta Fly and I know she would LOVE a comment.

Also, I am sorry but the postcards this week were, um, scanty because after posting 94, I kind of used up most of the postage stamps. Oops.

The BIG news is that I have decided not to die. Sorry, you can get your entrance fee at the door. I didn’t say my body is agreeing with me on that one but I am tired of writing about pain. Geez, I am ALWAYS in pain, so unless I have something new to share, we will assume that, and that probably parts of me are falling off or looking odd or blood is coming from somewhere or another.

But, hey, still have decided not to die (screw medicine!). So I will be doing other blog topics. I will still update on health issues and want to talk about what it is like having a ‘bad’ dementia day: that Alzheimer’s, or Parkinson’s experience which frustrates caregivers. I will try to explain from my notes and experience why we cry over your not giving up toast, or cake, when you actually gave it five minutes ago, or why we call you the name of someone who is gone, dead, and how that hurts you but what does it mean for us?

Right now, with Xmas on the way, I am masturbating, oh yes I am. I woke up this morning and thought, “Elizabeth, what gets you horny?” Good thing I have all these art books. Is getting aroused at an anime drawing a good or a bad thing? I think, since I can’t really move around a lot, a good thing. But that will take a bit more time (please continue to masturbate anyway!). I am studying (rub-a-dub!), I am collecting pictures, I am trying to find resources. Yes, we are too sexy for this wheelchair, too sexy for this bath bench, help me undress, oh yes, oh my!

See, even chronically disabled, or those with degenerating conditions can be horny. Stay tuned for blog post.

I realized that some people may not know what I have medically. BEFORE I was ill, I had Marfan’s and P.A.T.’s due to an extra node in my heart. Marfan’s is a genetic bone and muscle condition which makes your muscles stretch as the genetic switch to stop your bones growing doesn’t always work. So I have different sized bones on my body from side to side, and due to my knee bone growing into my kneecap, I had surgery on both knees. I hyperextend, as the muscles are pulled longer than they are supposed to be, this causes muscle pain, and my knees to go backward, and my shoulder to dislocate more often than others.

Then I developed a disease of Central Autonomic Failure. Autonomic Failure is when the things we don’t think about, from pupils dilating to blood pressure to heart beats, to converting oxygen from lungs into the red blood cells doesn’t work, because that part is damaged. I have both Central and Peripheral Autonomic Failure, plus Secondary Raynaud’s (which means my extremities turn funny colors, are cold and for me, not for those with Raynaud’s naturally, I can have veins collapse and start to rot, and require amputation – like Diabetes – this is another reason to exercise rigorously).

I also have progressing Neuropathy, which means there are 5 different types of nerves, and my nerves are being sort of eaten by the body, so I have limited to no feeling from my HEAD to toe. But think of nerves like a giant road system. Just because thousands are destroyed doesn’t mean that a person driving back roads might not find a way to bring a message all the way forward, particularly if hundreds to thousands of cars start out to find a route. This is what happens to me and to some SCI’s who have a partial or a break with some spinal nerves. So sometimes I close a door on my hand and feel…nothing. But other times, my feet, from the cold, or when I break a toe, I get, a second or two later, a flash of pain, and then it sort of dies out, as other messages crowd out the pain message trying to get through such a odd route. I type 5 times slower because the messages to my major muscles are both slow and erratic (taking the scenic trip on the back roads).

Because I have Hypothyroidism from a secondary Autoimmune disease, it is almost certain from my symptoms and visits to three different hospitals that I have AAN, Autoimmune Autonomic Neuropathy of the vascular centered variant. Only four disease have Autonomic Failure as a primary symptom – one is POTS, Postural Orthostatic Hypotention – I have the Hypotention but I don’t have POTS, or MSA (Multiple System Atrophy), the most common of this uncommon disease, and seen mostly in seniors, with three types, one related to Parkinson’s’ (used to be called Shy-Dragers or Parkinson’s Plus). The Hospital in Vancouver confirmed Primary Autonomic Failure, the Specialist Hospital in Washington State confirmed it is not POTS or MSA. The Hospital here confirmed it is vascular in focus (meaning the veins randomly close, and I get a micro stroke called a TIA, or I get a larger one, or my ear drum blows out, or blood doesn’t get to my intestines, the food rots and I have a gastronasty!). Two down, two remain, which leaves Pure Autonomic Failure, and AAN. But Pure Autonomic Failure doesn’t have Neuropathy or Autoimmune Disease: I have both, so does AAN. These are all diseases of exclusion, determined finally in autopsy (meaning there is no, take my blood and see if the water turns blue test - you match the symptoms until nothing else fits). 90% of PAF people get MSA, the average lifespan of MSA is 4 years from onset of first symptom (not diagnosis), the average lifespan of AAN is about half that (no study of AAN has been done because no one can get enough people WITH AAN together and have enough of them live to the end of the study).

There is no way to prevent this disease. It is really rare. It is like winning the lottery, you don't win the lottery by eating green food, or doing pilates, and you don't get this or have this cured by doing any of that other. Medicine has known about this dying of brain disease for 109 years, and so far, the only treatment is a pill for those with MSA-P, that helps 10%, and now IVIG for those with AAN (plus symptom treatments, like beta blockers, oxygen, pain management, synthoid, and other symptoms treatments) IVIG is a possible good thing because IVIG passes the blood brain barrier and kills the auto-immune system of the brain which is attacking the autonomic section. It is not a cure all, it doesn't work for everyone, but it is hope of stability. And the only hope I have right now (medically).

That is what I have, that is why I stop breathing (because the switch that says, ‘breathing is good’ is too weak to work, or the nerves to my diaphragm stop working, or similar issues, like my heart is too erratic to pump blood around), or get very ill, and can’t sweat and have the air conditioner on when it is –5 outside (I do, seriously!) – turns out that masturbating makes blood flow, increases heat. I have no way of dispelling that heat. I am, this morning going, ‘oh my, oh my, my hands are green and oh, oh, oh, I am sooo close, why am I super heating, don’t think of that, think of the sexy, oh yes, so close’ – followed then by, “Oh shit! I am way, way too hot, I need to do something because death by masturbation is not as wondrous as it sounds.”

Yeah, sometimes it is GOOD when workers aren’t here, as what do I say when I have all my tops off, the air conditioner full blast and a care worker walks in, with me saying with a wild eyed expression, “Quick, get a cold cloth, I am masturbating, and it goes well!” So to recap, postcard project, Hawaii Flowers, not going to die, researching desire, masturbation, more masturbation, and what disease I have. That’s it. Have a great day (oh, computer thing on hold for a couple weeks, and I go in for blood work at 7-8 a.m. tomorrow). Oh yeah, and I'm going to live.


yanub said...

Overheating due to masturbation? I believe the Twilight crowd has the answer: the Childo! Just pop it in your icebox, then into your box for that "undead" feeling. (Seriously, such a thing exists. Seriously, I can't imagine anyone actually using one.)

I haven't heard Marfan syndrome called a bone and muscle disorder before. But now that I think about it, why is limb length longer due to a fibrillin malfunction? It must be some sort of brake usually, in addition to playing a role in connective tissue strength.

Very glad to hear you won't be dying. That is to say, that you will focus on living. Good. Death is an escapable ghoul. No point in setting a table place for it.

Veralidaine said...

I'm very glad that you are going to live (and hopefully your body cooperates with that decision). I like Dax from the last post--cute little fellow!

Thank you for this post. In all the time we have known each other I never really knew about the Marfans. You'd mentioned that you had surgeries as a teen, but I didn't know what for.

Short comment today, just wanted to leave a comment to let you know I am here and reading and my thoughts are with you ever day.

JaneB said...

Glad to hear you are NDY, and that you have decided to stay that way. Once you decide something, well, I can't imagine it not happening! Many hugs

FridaWrites said...

Glad to hear you will still be with us for a while. Somehow I had MSA in my head instead of AAN in spite of what you wrote after the Seattle visit--thanks for re-explaining; memory issues of my own from pain. Exercise (in multiple senses) is good for you. If anyone can defy the odds, or defy them for longer, it is and will be you. I am proud of you, always.

Neil said...

Um, about the question "What gets Elizabeth horny?" Shouldn't the answer be "Linda!"? (Please pardon the punctuation; sometimes English punctuation rules suck.)

My Beloved says that if we lived on Terry Pratchett's Discworld (English comedy/fantasy series), Death would be having some very scary near-Beth experiences.

Oh, I HOPE it's not as fugly in victoria as it is on the prairies today. It's -32C at the moment, so give Linda a hug and tell her from me to rejoice that she's not here.

Love and zen hugs,

Stephanie said...

YAY it is such a relief to hear you say you are going to live. I was worried about you for a while (and dealing with my own issues that made me not want to crawl out of my hole long enough to stop by, read a blog and write a comment. I'm sorry. :()

Thanks for the post on your many diseases. Now I can actually look up what they are and try to understand them better. It's been at the back of my mind for a while now.

Air conditioner at -5 REALLY? With snow outside? That would be hilarious.

Okay, go postcards, go masturbating, go living. Go!

wendryn said...

I'm very glad to hear you have decided not to die. I'm glad you have decided to screw medicine as well as bronze! :)

Thank you for all of the medical information - I knew all of it, I think, but it's laid out very understandably here.

The Postcard Project is pretty amazing!

Aviatrix said...

I think that's an excellent decision, and I fully support it. I hope you spend as few of your future years as possible trying to make telephone arrangements for things to be delivered/repaired/stopped or started.

So that's why they call it "getting hot and bothered."

Julia said...

Hi Elizabeth! My name is Julia, I'm from Brazil. Lately me and some friends have been reading your posts and we learned how aewsome you are. You're so smart and talented, and you've been to Japan, Gosh, my dream is to go to Japan!
We just wanted to say that we really enjoy checking out your blog every now and then and we wanted you to know that you have some fans here in Brazil!

BIG BIG hug!!!

Elizabeth McClung said...

Julia: Thanks, I would love to send you or any of the other readers in Brazil a postcard (honestly, it is fun!) - I love Japan and would love to go back, but Hawaii was great too in a different way and I got to visit a Japanese temple there that few know about. Brazil would be great but I think a few too many hours in flight.

If I can help in any way a trip to Japan, or send some postcards down to Brazil (on Japanese Washi paper postcard - new year cards are a tradition), please let me know. Thanks so much.

Yanub: Oh, I think if I had the nerve connections something would kegal in contraction with what the Childo is - yeah, you go ahead and try it first with your rabbit.

I think people tend to focus on the heart wall problems but the height issue is listed as a symptom except it can be quite painful to have your muscles stretched longer than they are supposed to be.

Yes, when in small room tend to think about what is at hand: death, cookies, pain, pocky, suffering - you know, the things around me. I am going to try to get out of that mindset and back to the goth perverted academic I am.

Veralidaine: thanks so much for making the comment and letting me know you are reading. That means a great deal to me. I like Dax too, I am trying to find more like him as what better Xmas gift than a skelanimal with an impairment?

Ah, sorry, yes, couldn't walk, so they had to cut up my knees, then I did the marathons under the 'use it or lose it' clause of how I live.

Jane B: Oh, if that were only true, world domination would be mine. But I am, like some people I know, kind of organized into lists, break down what needs to be done, where the hard parts are and then figure out how to over come that.

Fridawrites: I tried to make the shortest but also most accessible summary so that people have some idea of the diseases I am talking about - AAN is sort of a ghost disease, pops up now and then in literature but never enough for a full article it seems. I will try to continue my home exercise program of thinking naughty thoughts to circulate blood - and do boxing as well! Masturbation is recommended in women for migranes and insomnia - though how with a blinding migrane a woman could think the fantasies seems quite the challenge.

Neil: Well, I like Linda and I like Linda to try out different outfits but Linda has her romance books and I have mine - we all have fantasies, and even in a marriage, acknowleging that has worked for us.

I very much like your Beloved's Terry Pratchett humor - yes, I am hopefully scaring Death by my weekly appearances, "Oh, I'm dead, no, can you wait here, I'll be back later."

It was minus 5 or more and started snowing after I got back from the blood draw (6 vials my ASS! Those were the huge vials that need lots of blood, I think I gave at least 12 oz if not 16oz (Does that make me the 'big Gulp' yet in 7-11 blood tech speak?).

Stephanie, it is snowing outside, I have two blankets on my lower body and a tank top and the air conditioner on my torso - it IS humorous.

Thanks for the comment, I am glad you came out of the hole and hope your issues are okay, or if not, look a little smaller and easier to deal with.

Glad the summary helped.

Wendryn: Yes, I will continue to search for the most effective ways of maintaining health and quality of life but onward! Don't die in the winter, so why die in the summer when things get interesting? And too hot out!

cheryl g said...

I am glad you have decided not to die. You have a formidable will so when you say that you only have x number of days to live it scares me doubly since I think your mind is capable of making that statement true whether your systems are truly at that point or not. Having your will and determination focused on living makes me feel less scared witless.

Masturbating seems an excellent past time. I will ponder ways to keep you cool without ruining the mood – I think your cooling vest would be a mood killer with that cold clammy sensation. Perhaps you could try going topless with the AC running full bore?

Thank you for the synopsis of your illness. I think it will help others understand what you have better and understand how your physical condition can change so dramatically from moment to moment.

Lene Andersen said...

Yay on the deciding not to die (and if anyone can make it by sheer willpower, it's you) and yay on the wanking - it's such a life affirming thing to do. Until the overheating. Damn. Hope you find a way around that.

OK< maybe stupid question here... isn't masturbation sort of like exercise? Might it make you sweat? Could it be medicinal??

Victor Kellar said...

Glad you decided to live. Because I'm no expert, but I'm pretty sure you can't masturbate when you're dead ...

Raccoon said...

yanub, I had an ice mold once. Just had to be aware of the ice melting. And the frostbite, if it was left in one spot too long...

Beth, thank you for the run down, the "overview," of what's going on. I like the comparison to a lottery. I showed it to one of my staff; we both learned some stuff.

NDY. Good.

Tom P. said...

I haven't been commenting but I wanted to let you know that I am out here and reading every post.

Michel has had a tough week as her hands are swollen because of the Syringomyelia. Apparently this is common and leads to something called "hook hands".

Baba Yaga said...

Hi, Beth.

Racing against time on the library computer, so may not have time to comment much on posts. (Warranty information - such details as *who did I buy the damn' monitor from* - is stored on my computer. I have discovered the flaw in that plan.)

But I wanted to tell you, I found size 10, woollen, stripey socks today. (Still faster than I knit. Which, looked at sideways an with a determined squint, retrospectively justifies my not having even bought the stripey yarn yet. Or maybe it doesn't.)

They will be on their way as soon as I can haul myself to the post. (Probably beginning of next week. I'd like it to be sooner, but some of that rest nonsense I keep suggesting *you try seems to be in order, after the last week of running around, aping a 'normal' person.)

3 minutes' warning - tyranny!

SharonMV said...

so glad you decided to Live! You have such a strong will, intellect & spirit that your decisions have power.
Thanks for the explanation of your illnesses & the terminology. I can follow pretty well from you've said previously - but I have the medical knowledge derived from the fun experience of being undiagnosed for years, followed by dxs of several rare diseases. though not as rare as AAN.
I like seeing pictures of the postcard backs to see all the stamping & stickers.

Did you get my postcard? A package of stickers is on its way now. Sorry that I did not answer your latest e-mail. I can't find my e-mail! Probably me & not the computer - somehow can't access it.


Anonymous said...

It is rather interesting for me to read this blog. Thanx for it. I like such topics and everything connected to this matter. I would like to read more soon.