it's a very long story Stroppy ...
but ended up in hospital, middle of the night, in Aug '10 ...
acute pancreatitis (I haven't drunk in 35 years) and vital organs shutting down, with no immediate cause (had been fine all day, 'til I started chucking up at tea time and continued 'til 2.30a.m. when the medics arrived)...
spent a week on an I.V. morphine drip and lost 3.5 stone in 10 days. Haematologist consultant said I had summat wrong but they didn't know what - my blood results were like Sigourney Weavers in Alien Resurrection

...
three bone marrow and bone biopsies, multiple tests and 6 months later they told me I had Myelofibrosis - terminal (google it - horrendous end)

...
a few weeks after that a pathology Prof. in Cardiff who'd been eyeballing my biopsies came up with a.n.other diagnosis which he'd only ever seen once in his career ...
more tests at DNA level this time and told I had Gaucher's disease.
Rare as hen's teeth - 1:1,250,000 chance of inheriting it ...
#15 in Wales, 200 in the uk, 5000 in the world.
Was told from the outset, that whatever I did for a living, I wouldn't be doing again

...
TBH I wasn't fit enough to walk 20 yds for months ..
I'd shut shop on a Friday night and never got to go back
Gauchers has 3 types .. two fatal, t'other fatal if untreated
took 13 months of fighting to get my treatment funded ('cos it's so rare - and expensive)
here's the techy bit - I am missing a gene on a chromosome that is responsible for creating an enzyme in my body called Chitotriosidase - this breaks down the blood cell walls as/when your bloods regenerate - we all have a full 8+ pints of fresh blood weekly - constant oil change - your spleen is responsible, with the enzyme's help, for excreting the detritus from this from your body (amongst other things) - normal spleen = 200gm .. mine was estimated at 3200gms - was full of crud, along with a few other organs - hence the shut down.
they still don't understand how I managed 50 odd years, before 'crashing' ??
been on a weekly treatment since Feb. '12 - I.V. infusion of an artificial enzyme -- took nigh on 2 years to get my spleen back to a normal level, but the treatment keeps it that way.
Pam has been trained to canulate me to administer the treatment once a week.
basically keeps me going - as said all Gaucher's types are fatal, untreated
the artificial enzyme is 'grown' on the back of hamster ovary stem cells ...
so when you see me talk about me 'hamster juice', that's why ...
and no I don't store nuts/food in cheek pouches, or build newspaper nests in corners ...
heard 'em all


...
my treatment is for the rest of my
natural ..
keeps me alive, but is a hell of a tie ...
we can do it anywhere really, but...
the enzyme has to be stored at between 2 and 6 degs C and is burgered, above or below that, so although the kit is easily portable - that isn't

travel is not easy - more than a week presents extreme difficulties
TBH I consider myself lucky .. I don't get a huge number of the possible issues with the disease .. there are four A4 pages of possible issues / symptoms / effects which I don't seem to suffer with.
do get periodic random pain episodes - organs give me hell some nights - Gaucher's gives you gall stones, so have to watch what I eat and I need my gall bladder, so have to treat it carefully .. and my spleen can still play up .. usually about 2 a.m. pain'll wake me up and needs a tramadol/paracetamol cocktail to get on top of it ... had one last night for no reason I can fathom - but can't lie down when it hits - sleep upright in a chair, once the pain killers kick in and have morphine 'in stock', if the cocktail doesn't work ... can be
'rolling 'round the floor' pain, some nights, but rarely in the day


... been on 'screen saver' most of the day
So - I was forced into early retirement - well before I was ready - but had no choice ... dealing with the sale and clearance of my yard damned near killed me - again ...
but could've done with a couple more working years - had just bought this place and had a shed to make and several other projects to create .. but at least I'm here.

quick worker
