So, let’s just get this right. You died, I fortunately was there, then the kind Palliative nurses put some weird elastic contraption on your jaw to keep your mouth from falling open and I said goodbye – again. Then, presumably, they whisked you off to reside in the hospital mortuary for a bit and the next time we “see” you, you’re in a coffin in the side entrance of the crematorium, waiting for the funeral service to begin.
In between times however, Lord only knows what they did with your body. I’m not sure we really want to know too much either, although if I’m honest, it’s another of those morbidly fascinating things that you kind of want to know but don’t want to at the same time. And you know me, I always like to KNOW about stuff, especially the stuff ‘not talked about’. I’m so not like Gran in that respect. Gran didn’t like to know about stuff you don’t normally talk about; she went all tight-lipped and stiff, preferring always to simply ignore it and pretend it didn’t exist. Out of sight, out of mind. How different I am. Maybe that’s why.
Anyway, we know they’ve conducted a brain autopsy on you because we were told they would and we didn’t have much choice in the matter anyway. As was explained prior to your death, all CJD patients in France are highly recommended to undergo this procedure post-mortem as it will determine what kind of CJD variant the patient had, plus provide valuable research information for the future.
So, where exactly is your brain currently? It didn’t go with you in the coffin, did it? Or at least it didn’t go as a whole organ. We know the autopsy was done in Paris so is it currently residing in some formaldehyde-filled jar in a hospital science lab with a sticky label on it denoting “Property of…”? Seems so rude to just remove something that belonged to you, something that you couldn’t live without and that served you so well for seventy two years, and then just plonk it in a jar somewhere for clever, white-coated men to fiddle about with. There’s a part of me that would kind of like it back, but then what would one do with it?! I know you’d be utterly horrified at that thought so perhaps we’ll forget that idea. Although you did love Kathy Reich and her forensic science books in real life, Mum. How bloody ironic.
So, we’re missing your brain, or part of it, whilst it’s still under observation in Gay Paris. At least you’d approve of that. You did love Paris with all it’s style and class and bloody rude Parisians. I have to say Mum, that I’d put money on the fact that not many Sheffielder’s brains end up in Paris to be this closely examined. And, as we would have a good snort about over a cuppatea, there could be a lot worse places where one could be left – the Northern General NHS would probably be up there for a start! How common, ‘eh Mum ;-). Now you wouldn’t have liked that, would you?
The thing that is truly horrendous to think too much about is the fact that whilst they are slicing, examining, dyeing, testing and doing whatever else with your brain, your two children are in fact playing russian roulette in the Waiting Room next door. For the last five months since you died, Robert and I have just had to sit by and wait for the hospital to eventually call us, hopefully to relieve us of the angst that you could have unknowingly passed this on genetically to both, or one, of us. We are literally on the edge of our seats, the potential outcome of the quality of the rest of our lives is held in the rubber-gloved hands of some lab scientist in Paris.
Will it be Sporadic (as predicted by your hospital, based on blood tests and MRI imagery), Variant, Iatrogenic or God forbid, the family death sentence, Genetic CJD? What’s the difference between all those forms of CJD then, you may well ask? Well, that’s the billion dollar question isn’t it? Global Governments and some of the worlds biggest money-making industries will quickly and reassuringly tell you that they are completely different and not in any way connected. I, for one, don’t believe that. Just like I don’t believe that this disease is famously quoted as affecting “one a million” either. I am an active member of various CJD groups and organisations and I can tell you that having read everything I can get my hands on about CJD all the afore-mentioned variants could well be proven to be linked by cross-contamination from animals to humans via ingesting animal products and by-products, and also passed from human to human via infected blood. And there’s waaaaaaay more than “one in a million’ who gets it too.
Frustratingly, there is currently no blood test that can detect CJD either. Another reason why this disease needs more recognition and funding.In brief, here’s what the powers-that-be put out as the ‘official word’ so as not to cause widespread panic amongst the public.CJD is diagnosed as being one of four variants:
– Sporadic CJD
This is reportedly the most common variant. Up to 80% of people diagnosed with CJD are labelled as having the “sporadic” version and it has been found in every country in the world. Basically it translates as “occurring without really knowing why”. What triggers the CJD to be activated is anyone’s guess. However, the most favoured current theory suggests that the normal prion protein in the brain undergoes a spontaneous change to the abnormal form, thereby resulting in disease. If this theory is correct (and it still has not been proven at this point, hence the very urgent need for more Government-backed research funding) then the disease arises simply as a “chance” event inside the brain and therefore not ‘caught’ in any way. I, for one, do not believe this is entirely true. The scientists and medical fields simply don’t know enough about this disease and have therefore given this variant the wide-open, loosely termed “sporadic” title. It basically means very little. As I say, they need way more research and financial backing to find out exactly the cause, diagnosis, management and hopefully cure of this dreadful disease.
– Variant CJD
This is the variant everyone’s heard of connected to CJD. It the “Mad Cow Disease” version. The one that gained rapid notoriety in the mid 1990’s, the one that filled the media’s pages and news slots, the one that put the fear of God into everyone in the UK, the one that the Government tried to brush under the carpet. The current view on variant CJD is that it has resulted from transmission of infection from BSE in cattle to humans via infectivity in food. Given that CJD can have an incubation period of many decades and that, as I said earlier, there is no blood test that can detect CJD, none of us know how many people are carriers of this disease and how many will go on to develop vCJD in the future. Now just how frightening is that?
– Iatrogenic CJD
We’re told this is very ‘rare’ too. Go onto any online CJD forum though and you’ll read a different story. This is CJD which has been accidentally transmitted during the course of medical or surgical procedures – notably brain surgery – as instruments used on a person with CJD may not be disposed of and re-used on another person. It happens more often that we like to think about sadly. Another important example of this variant however relates to CJD transmitted via Human Growth Hormone treatment in childhood.
– Genetic CJD
The dreaded version as it affects generations of families. It is seemingly rare but who really knows the truth behind this statement? In this form, CJD is caused by an inherited abnormal gene. The illness is therefore not “caught” in any way and we’re told that there is no causal relationship between this form and BSE. In most cases, the illness is known within the family because of the family history. Occasionally though, genetic cases are seen in which no previous family history is identified.
So here we sit, and continue to wait for the official diagnosis of what exactly killed you on 5th October 2013, just three months after we got married. From the biggest high to the biggest low in one fell swoop.
Meanwhile the wheels of life continue to turn, and my brother and I try to live as fully as we can knowing how very quickly life can be taken away.
Let’s hope the CJD death sentence leaves us alone.