Ever the one to be pleased to hear from independent publishers with an interesting list, I was delighted to get my hands on some titles from PortobelloBooks.
The list looks wide-ranging and already I'm two books into the three that arrived and have a few more on request.
The one that I really have to tell you about first to get it off my mind is The Family That Couldn't Sleep Unravelling a Venetian Medical Mystery by D.T.Max.
I have to get it done and dusted because to be honest it's terrifying.
I was glued to this on the morning it arrived and though I haven't read every single word, I've read most of it and what I have read quite unnerved me.
You get immune when you work in a doctor's surgery.Not blase but you need some defence mechanisms in place.Terrible things happen daily and you quickly learn not to think that the unusual patient's symptoms, resulting in a rare and unpleasant diagnosis and discussed incredulously over coffee, actually seem to mirror a whole host of your own that you've just been ignoring or keeping quiet about.
It would be quite easy to have self-diagnosed something rare/fatal by lunchtime every day, imagine it will likely fulminate through the afternoon and you'll doubtless be dead by teatime and with a whole load of records not written up.Trust me, they'd call you back from the dead to write up records.
So I'm quite good at coping with it all in a book, but the unthinkable tragedy of this one just chilled me to the bone,
"The first symptoms begin after just a few nights without sleep, with shaking, sweating and overwhelming anxiety. Several weeks on the insomniac is unsteady on his feet, wracked by fevers, and drifting in an out of delirium as the body's vital organs begin to fail. Finally, after months of agonising open-eyed deterioration, the victim falls into a state of exhaustion resembling a coma and mercifully dies."
Fatal Familial Insomnia and no, I'd never heard of it either.
Now don't panic, it's an incredibly rare, inherited disease which has afflicted one noble Venetian family since the eighteenth century and only a handful of other families worldwide.The cruelty is the fact that it strikes at random once child-bearing years are over, and as an autosomal dominant mutation there is a 50% chance of a child getting it too.
The family agreed to undergo testing to aid the research and though the names of family members who will succumb is known in the laboratories the family agreed they didn't want to be told.
So this all feels quite far and distant from the rest of us until Max reveals that the research has also identified that this is all caused by a rogue protein known as a prion and then suddenly you think it's right on the doorstep because so was CJD or Mad Cow Disease a few years ago.
Having read in tragic detail the implications for the Venetian family, it's therefore tricky to hold your nerve when you read Max's investigation into that whole sorry saga here in the UK. We all remember it well, the incredulity as more and more was revealed and Max can barely disguise his scorn for what went on.
It does not make for comfortable reading and I'll own up I had to skip bits and when I finally reached the end and the hope of some comfort there wasn't a great deal to be had. Max himself suffers from an undiagnosed neurological disease and recounts his own story.
All in all there is not a lot of joy in a book like this, but the truth is there to be told and someone has to tell it so that someone else can read it. The research looks impressive enough and you have to applaud D.T.Max for taking on a subject so close to his personal situation, it can't have been easy.
Thankfully another book from that pile, The Visible World by Mark Slouka was waiting to effect a rescue and I was quickly and gratefully esconced in wartime Prague, but many thanks to Portobello Books for a highly challenging read.
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