42:07 I may know how I die

No seriously.  So about three years ago I had a bee sting and I got hives, they lasted about 6 months.  Seriously, for six months I had hives ... everywhere.  About a month into the hives I developed erosions, or ulcers in the roof of my mouth, on my tongue, these extended down my throat and I couldn't eat or talk easily.  These lasted about 3 months.

During this time I saw a few doctors, I was given steroid shots, I thought I had a fungal infection called thrush, so got a prescription for fungal infections.  My doctors thought I had AIDS and tested me for HIV.  Negative.  They were so sure and baffled that they tested me 3 times, all negative.

I was taking hydrogen peroxide baths and gargling that everyday and after sometime everything disappeared.  My doctor ... still baffled said ... well its gone so no need to worry.  But ... was it?

I've commented on this change about two years ago it seemed suddenly I now needed reading glasses, kinda strong ones.  I couldnt read my phone or directions without them or without taking out a contact lense.  Ive always had bad eyes ... my perscription is -8.5.  But one day a month ago I was driving and my eyes were more blury than usual and I couldnt stand it so I pulled over and took out one of my contacts.  I drove on...and when I got home as I was parking I thought to myself...but I can see really good.  I must have a contact lense still in.  I reached in but no contact...I looked around and for the first time in my life I could read street signs without a contact or glasses.  My eye sight was now the best I'd ever had.

The next day it went away and I was back to normal...still needing reading glasses and contacts.

I recently ( Feb ) developed dark pigmentation in the crease of my inner thigh and groin.  They were widespread and on bothsides ... so semetrical without touching..., flat with the skin, didn't hurt or itch but I though it looked like jock itch or ring worm.  Over the next 2 months I had no improvement using jock itch sprays and creams., even went back to hydrogen peroxide baths.  I also developed what seemed like an anal fissure...that didn't heal for months. 

So I booked a telemed session online with a dermatologist.  She said no...not a fungal infection I think you have Hailey Hailey Disease and you need to see a local dermatologist for a biopsy.  Looking this rare condition up I skimmed over a similar diease that was everything Hailey Hailey was but also presented itself first with mouth sores that came and suddenly went.  I was like ... that is all making sense now... Pemphigus Vulgaris.  It's often mistaken for candidiasis ... a fungal infection.

The building block cells of the epidermis are called keratinocytes. These cells are cemented together at special sticky spots called desmosomes. In pemphigus there are autoantibodies to a component of the desmosomes. These cause the keratinocytes to separate from each other. The gap fills with fluid, causing a blister or the skin may peel off leaving raw areas.  Most people will die from infection.

It affects any mucus membrane ... the eyes, nose, throat, mouth, anus, groin ... if its a moist pink out.

An autoimmune diease that is very rare but most often found in 30 to 50 year olds.  Well Im 42.  Its also prevalent among ethnic Jews.  I'm an ethnic Jew according to my Y chromosome haplogroup.  Ashkenazi Jew ... which by the way... they carry ( I guess we)  a long list of genetic issues of which I am a carrier for Cystic Fibrosis too.  But thats a I have a predisposition for Pemphigus ... untreated 80% of people will die within a year...99% within 5 years.  So ... I gotta get treatment right?  Treatment that drops to only 15% death rate.  No cure...just control.

It's an autoimmune disease where your immune system is attacking your skin and causing it to literally break open.

I had my appointment with a dermatologist who, took a biopsy. looked serious enough to him to warrant an immediate "minor surgery."  That hurts like a bitch, to have a nickel size whole cut into you and removed.  I joked and laughed about how the doctor, the nurse and me were all on first name basses since we were cutting into my inner thigh/groin together.  We shook hands and traded first names just before the biopsy.  Now I'm glad the doctor had hanging on the wall his residency ... was at John thats a good background.

Back home in reading I noticed that Pemphigus Vulgaris is sometimes a secondary issue.  Cancer, sometimes tumors in the brain can manifest elsewhere.  It seemed to be an easy to rule out thing because the most common issue was a Pituitary Gland tumor.  I found that 3 things would be useful to know and test for... TSH then T3 and T4 these are hormones we all should have naturally.  

TSH is from the Pituitory gland and it makes the Thyroid gland produce TS3 and TS4 which then suppress the making of more TSH.  They regulate each other and cancel out the production of one another.  Typically hyperthyroid is when you see normal TSH and high TS3 and T4.  But hypothyroid is when its all too low...

Anyhow, I took a blood test and got my results two days ago...still waiting on the biopsy news.  I scored ... pretty far into the low category in all three.  Thats actually rather rare.  Which is telling ....  TSH and TS4 and TS3 all low.  

See..usually one is high at least...but all low indicate damage to the Pituitary Gland.  The most common form of damage is a tumor.  That lowers the bodies TSH which happens when there is a lot of TS3 remember....but when TS3 gets low then TSH is supposed to jump up and it triggers more TS3 production again....but my low TS3 isnt triggering TSH and thus I still have low TS3 and TS4.  These are pretty definitive tests...

Hypothyroidism Secondary .... is what I saw at the American Thyroid Associations site.  The leading cause for this condition is a tumor in the Pituitary gland.

Where is that?  Kinda in a bad place to access...thats at the bottom and in the middle of the brain.

A tumor in the Pituitary gland also can affect your vision.  It sits just above the optic nerve and theres no room up there for a tumor so when one exists it can affect nearby structures such as the optic nerve. my vision changes have become a little more interesting.

There is a specific name for caused by a tumor.  Which ... btw now it might make sense that my immune system is attacking my tissue...if its attacking a tumor already and in a major battle its mistaking healthy tissue as collateral damage.

Paraneoplastic Pemphigus .... thats a name to keep a post it note on for me.  "The pemphigus family are rare autoimmune blistering diseases affecting skin and/or mucous membranes. Paraneoplastic conditions occur in association with malignancies (cancer). Paraneoplastic pemphigus is characterised by painful blisters and denuded areas of the mouth, lips, oesophagus and skin. It is the least common but most serious form of pemphigus, particularly considering the presence of the underlying cancer."  Fatality with treatment is 80%...without 99%. (why 99 and not 100?  Probably because someone got diagnosed and then killed themself so it didnt kill them) :-) Anyhow...I dont think I have that, cause most Pituitary tumors are thats not good...per se but its better than cancer.  The tumor itself may not spread but its affects can be big so surgery or radiation therapy is common.  

Surgery though ... like we noted before its a difficult place to be poking around're basically cutting into the base of your brain just behind your eye balls a few inches inside your head.  Thats a serious place to be opening up.

Whats the recovery like?  What about the complications?  Something tells me a lot of consent forms are involved.

I need to schedule an appointment with an Endocrinologist I think.  Because ... while I may be playing doctor, I'm not one...but I kinda feel like I've got alot of work done on my own ... dermatologist biopsy is still out for results....but the lab tests I had done on my own add some new light. 

So ... where does all this leave me?  I dont know.  I can say that I'm good ... as someone can be who might have a tumor in the brain thats causing there skin to fall apart.  :-)  But ... what can ya do?  Three phrases come to mind now...

well three and a song...

Would it help? 

I'll have my time and I wont need more.

It is well with my soul.

and ... chessy I know ...

He said
"I was in my early forties
With a lot of life before me
And a moment came that stopped me on a dime
I spent most of the next days
Looking at the x-rays
Talkin' 'bout the options
And talkin' 'bout sweet time"
I asked him
"When it sank in
That this might really be the real end
How's it hit you
When you get that kind of news?
Man, what'd you do?"

He said
"I went skydiving
I went Rocky Mountain climbing
I went 2.7 seconds on a bull named Fumanchu
And I loved deeper
And I spoke sweeter
And I gave forgiveness I'd been denying"
And he said
"Someday I hope you get the chance
To live like you were dying"


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