Hazel McHaffie


Cursed inheritance

As I promised last week, good news this time!

Eight or nine years ago I chose Motor Neurone Disease for the degenerative condition journalist Adam O’Neill is battling with in my novel, Right to Die; Right to Diea disease that will strike right at the heart of his being and his aspirations. So I was fascinated to find Lisa Genova chose Huntington’s Disease for her tough Boston cop Joe O’Brien in Inside the O’Briens, a disease that stops his career dead in its tracks and forces him to face the horrors of genetic inheritance.

Both MND and HD are frightening, crippling, fatal conditions that rob the person of control and dignity. Getting inside the mind and body of either an Adam or a Joe is very scary stuff. So, having been there myself, I was extra curious to see how someone else tackled the ethical minefields and personal challenges associated with such a scenario; especially someone with Genova’s credentials.

This time she has inserted occasional tracts of medical explanation about the condition into her novel to inform the reader, positioning herself as a scientist; but for me her real strength lies in her ability to describe the illness from the inside. She puts the humanity into the science, compassion into the clinical facts. The insidious onset before policeman Joe even suspects the truth. The sudden weird and inexplicable bursts of anger. An inability to process instructions. Finding it impossible to keep legs and feet still on parade … in police exercises … in a restaurant. And then, once he’s diagnosed, the inexorable progression. The involuntary indiscriminate throws – punches, food, cutting words – that label him as drunk, deranged or dangerous to passers by. The red rages that cause his wife Rosie’s black eyes, terrible destruction in the walls of the family home. The fear that won’t let him ever hold his grandson. The depression that makes him constantly check his gun is still loaded and primed.

We peer into his past when Joe recalls his mother’s antsy wild black eyes as she lay in a mental institution for years; condemned to be known as an incurable drunk. The questions hitting him now nearly forty years later. How could she have remained an alcoholic in the hospital all those years? Why had his father stopped taking Joe and his sister to visit her? Why had his strong dad wept like a baby? What lessons did she actually teach him?

The author powerfully captures the brutal reality through the eyes of the rest of the family too:

Inside the O'Briens‘Huntington’s isn’t the absence of moving, thinking, and feeling. This disease is not a transcendental state of bliss. It’s a complete freak show – ugly, constant, unproductive movements, uncontrollable rage, unpredictable paranoia, obsessive thinking.’

We see the dawning terror in his wife’s eyes. Her silences. Her withdrawals. Her desperate stroking of the crucifix round her neck, the beads of her rosary. Her binning of the symbols of her ingrained Catholic faith.

Then there’s the terrible implications for their four beloved offspring. Vegan yoga teacher, Katie, living life in ‘peace, health and harmony’. Ballerina Meghan, limbs and body and mind all supple, beautiful, desirable. Firefighter JJ, taking his health and fitness for granted, using it to save others; preparing for imminent parenthood with his wife Colleen. Rebel Patrick, sewing his wild oats liberally, experimenting with life. Each one of them carrying a fifty percent chance of harbouring this cursed disease. Nothing can change that fact. Nothing can halt, slow or reverse this terrible thing. Joe, their father, is powerless to protect them. Indeed it was he, their supposed protector, who handed on the poisoned chalice in the first place. And now he must stand on the sidelines and watch them all battling with the impact of their cruel inheritance. Only they individually can decide whether to take the test, if they want to know the truth lurking unseen in their own DNA. JJ and Colleen may not even choose on behalf of their baby son.

How should Joe deal with his burdens? Is there a way out? Should he take it? How can he best support his children? Should the youngsters go for testing? What are the implications if they do/don’t? Would I want to know?

I love books that are at once a gripping read and challenge me to think deeply – especially in the field of medical ethics. And even though I’ve been into these questions already myself as an author, I thought this book was brilliant and awarded it five stars. Beautifully written, compassionate, perceptive, engrossing, provocative. Genova at the top of her game again. Seems I prefer her as neuroscientist-turned-novelist rather than simply novelist. That could well be something to do with my own position on the spectrum; nevertheless the experience of reading Love Anthony and Inside the O’Briens one after the other, has taught me something of value for my writing too.

So, Genova has already tackled Alzheimer’s, Left Neglect, autism and Huntington’s. What next? MND, she says – or as she calls it ALS. Ahah! I await that novel with bated breath!

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Mitochondrial donation – a lifeline or an historic mistake?

Imagine you’re in your late thirties. You have one child of eighteen months, Mia. Beautiful, much loved, and long awaited. But Mia is suffering from a devastating disease which is damaging her brain and other organs, she can’t eat or sit up or smile, she has frequent seizures. More than that, you know that you, her Mum, carry this disease; you are the one who passed it on to her. She has at most a couple of years to live. Before she even reaches school age you will be attending her funeral. And any future children would be very likely to inherit the same condition and go the same way.

Now imagine a doctor tells you you could have a child who is healthy and free from this terrible disease. Indeed that he could wipe it from your family entirely. For ever.Newborn baby

Now you’re talking! It’s a no-brainer surely. Who in their right mind would say no?

Ahh … well, there are some people – a lot of people actually – who would say to you: ‘No, I don’t want you to be allowed to have this healthy baby. And I’m going to campaign with all my might to stop it being legal for you to do so. I have my healthy normal kids, but you are condemned to heartache and loss because of a quirk in your genetic makeup. And my moral indignation trumps your biological rights.’

Yep, you’ve twigged. We’re talking about mitochondrial diseases. The mitochondria’s the bit outside the nucleus which doesn’t pass on any personal attributes but which helps to produce energy; the so-called power house of the cell. And faults in this area can result in terrible life-limiting diseases. Journalists talk about ‘three-parent babies’, but that’s an emotive and misleading and singularly unhelpful term. Does anyone refer to infants born via artificial insemination by donor in this way? or surrogate pregnancies using donor eggs? and yet they are more three-parent than the mitochondrial variety. Because only a miniscule amount of mitochondrial DNA (less than 0.001%) is from the donor, none of it responsible for the characteristics of the resultant child; almost all of what the infant inherits is in the nucleus, and 100% of that comes from the parental couple themselves.

On Tuesday this week parliament debated whether or not the UK should become the first country to permit mitochondrial donation to eradicate severe debilitating diseases of the mitochondria. Permission was approved. It’s worth noting that the UK has one of the most rigorous regulatory systems in the world when it comes to reproductive medicine and these various stages of consultation and approval are one aspect of that rigour.

Once again, though, up jump the zealots and pontificators and detractors – they’re attracted to developments in the field of fertility like iron filings to a magnet. But this time it’s not just the usual extreme suspects who are objecting, even the Church of England spokesmen on medical ethics are using terms like ‘irresponsible’, ‘an ethical watershed’. Why? QuestioningWell, at its heart is this factor: the procedure will fundamentally change the genetic makeup of an embryo in such a way that it will alter the inherited characteristics not just of one single individual but of future generations: modification of the germ line. The changes will be passed on; there’s no going back. And who really knows what might result from that? Nobody, that’s who. Nobody can guarantee the long term effects, because we have no data (human at least). None exist. It’s a new procedure (although in reality work has been going on for 15 years behind the scenes in preparation for this moment), but potential increased risks such as premature aging and cancer have been mooted. OK, three scientific reviews have concluded that it is unlikely to be unsafe, but it’s also a fact that scientists do not fully understand the interaction between the nuclear DNA and the mitochondria, which leaves some important questions unanswered.

However, for me this balance of risks and benefits falls into the same category as Lord Saatchi’s Medical Innovation Bill. When the chips are down, when there is nothing but bleakness, tragedy and death ahead, isn’t there a case to be made for well-informed patients/parents to take a calculated risk? We aren’t talking about offering this procedure to the rank and file of childbearing couples after all; only a minority for whom the alternative is too terrible, too heartbreaking, to contemplate. Don’t all scientific discoveries take a leap of faith at some point? Of course the researchers and scientists need to keep working to explore optimal conditions and refine techniques and enhance safety, but that’s true of even the now-common procedures like IVF. If mitochondrial donation is allowed, the children born this way will need to be monitored closely for the rest of their lives, and their children after them. Every step will be scrutinised minutely.

The HFEA Act requires that the embryo be treated with special respect. Quite right too. Few would argue. Misunderstandings are much more to do with the perceptions of what is proposed. So let’s clarify. Scientists are NOT trying to enhance humans in any way, they are not even trying to alter a child’s characteristics. They are aiming to prevent crippling and often fatal diseases and avoid couples now and in the future suffering the incomparable heartache of losing their children in these tragic potentially-avoidable circumstances.

Religious adherents may protest loudly, and indeed in our democratic society, they have a right to their opinions, but what of the Biblical injunctions: Do to others as you would they should do to you, and judge not that you be not judged? Should a Jew or a Muslim or a Roman Catholic or an evangelical Christian or whoever impose their specific viewpoint on Mia’s Mum? On society in general? These are terribly difficult and painful moral and personal issues which affect couples at a very fundamental level. What gives others the right to dictate, I should like to know?

Religious objections aside, a valid sticking point for a lot of people is one of safety and timing. Pre-clinical safety tests have not yet been concluded, and until the results of those investigations are known, it does seem premature to rush through new legislation.

So, we aren’t there yet. Now that the new legislation has passed this week’s hurdle, it comes before the House of Lords on 23 February. If it’s successful then there follows a careful and exact and rigorous process of trials. Caution is the name of the game just as it was with IVF; scientists agonised waiting to see if those babies were healthy. It’s possible the first human trials could begin in October this year, with the first babies born in autumn next year.

The idea that this is some form of malign genetic manipulation is nonsensical, but I did smile at the terse letter to the Telegraph from a man in Aylesbury:

GM crops, bad; GM children, good. How very strange.

He could go far in the literary world but perhaps not the scientific.

What do you think?

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Truth, fact and fiction

Sometimes fiction allows one to tell a truth that couldn’t be so readily conveyed factually because of all the complexities of real life: so said best-selling crime writer, Val McDermid, at a Blackwell’s Bookshop event on Thursday evening last week. How true.

Blackwell's eventShe was appearing with her great friend Professor Sue Black, a renowned forensic anthropologist. I’ve heard them doing a double act before at the Edinburgh International Book Festival so I knew we were in for a real treat.

The flip side of the first aphorism is: fact can sometimes be so much stranger than fiction that a novelist can’t use it because no one would believe it. How far would your credulity stretch, I wonder? Would you believe that a pile of pupae on a kitchen table in Liverpool could be found to have cocaine in its DNA, which in turn would lead to the discovery of the murder of a drug dealer no one knew had been killed? It really happened! Were you aware that only certain species of insects can lay eggs through the zip of a suitcase? Val has met the scientists who’ve investigated this phenomenon in person and seen the actual suitcases they utilised. Did you know that the ink used in tattoos migrates to the lymph nodes, so that if a corpse has had the limbs severed, the lymph nodes in each quadrant can indicate where and what colour their tattoos were? Or if indeed they were added after death. Fascinating true facts.

The evening was full of such wisdom and knowledge. All delivered with great verve and wit. Both women are excellent orators with a lively sense of humour, and a tremendous breadth and depth of knowledge. They also have massive respect for each other and for their readers’ discernment, illustrating perfectly the enormous value for a writer in working alongside experts. Their own relationship goes back twenty years and one can readily understand that in their time they’ve emptied more than one hotel foyer and coffee shop with their ghoulish conversation and macabre sense of the ridiculous! Even at this event, in full view of a large audience, they chortled gleefully about subjects not normally broadcast before the watershed! But hey, did you know that a pubic scalp looks like a tuna steak with hair? (Val said it took her a good year before she could face a tuna steak after Sue shared this fact with her.) Or that PhDs are gained on the study of the backs of men’s hands and the configurations of the male genitalia in various stages of arousal? Such essential statistics are vital for forensic scientists tracking down paedophiles and murderers apparently; and equally valuable to a crime writer weaving an authentic and challenging plot.

Sue and Val in conversationPerhaps the clearest message that came across though, was that the best scientists are the ones who can make their subject readily understandable to other people. Sue Black does exactly that. And if anyone could sell a book on forensics, it’s these two. When Val was recently approached by the Wellcome Trust to write a non-fiction book on the history of crime for their forthcoming exhibition, she initially declined. All she did was ‘make it all up’, she protested. But of course, in truth she’s a mine of information, and has an impressive contact list of experts to boot. With the inquiring mind of a journalist, and the lateral and devious thinking of a crime writer, she tracked the changes in techniques and detection over the years and produced Forensics: The Anatomy of Crime, which was published last month. In her lively description she allowed us to share her fascination with the topic that grabbed her interest most: scavenging bugs and beetles, flies and maggots, and what they can teach about a crime.

All in all a most entertaining evening that reinforced for me the importance of meticulous research and true engagement with one’s readers/listeners.

(Footnote for those who are anxious about my current health problems: I was closely guarded by my son and daughter from door to door. I’m happy to report that in spite of the excitement and laughter my heart rose nobly to the occasion and I lived to write this report.)





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Authentic fiction

I do love hearing from readers, and I’m always impressed when they make contact. It’s something I rarely do, but should do more often – no, not with myself, with authors whose work I’ve enjoyed.

‘Star letter’ this week goes to ‘Trish’ who wrote asking for the reference for a medical thriller mentioned in my novel Right to Die. The main protagonist, Adam O’Neill, a journalist who has developed Motor Neurone Disease, lends the thriller to his GP, Dr Curtis, and writes in his diary:

Right to DieHe’d brought back a book I’d lent him, a brilliant medical thriller about a serial murderer who developed aplastic anaemia. I’d been impressed by the twist in the tale, and thought Curtis might appreciate its medical cleverness. The killer went on to have a bone marrow transplant from his sister. Result: when he was a suspect years later the forensic people said he couldn’t be the killer because his DNA didn’t match the samples from the crime scene. Naturally, the hotshot detective got hold of some distant relative and winkled out the story of the transplant and hey ho, the villain’s clapped up in jail and they’ve thrown away the key.

The crime story was entirely fictitious, but of course I was chuffed to find someone wanted to read it. And more delighted still that my correspondent wrote to me subsequently to say she was a nurse who works with people with MND, and how true Right to Die rang with her experience. That kind of endorsement from experts is special. Very confirming.

In a couple of weeks time I’m due to attend a bookclub where the members are going to be discussing Right to Die, so I’m hoping they’ll be as enthusiastic as Trish. I know there’s at least one doctor in the group, so I’m not expecting an easy ride. But the challenge is stimulating, and there’s a particular thrill in hearing other people talk about my characters as if they’re real people. To me they are; I know them intimately.

Trish’s endorsement made me feel so chirpy I abandoned research into auto-immune hepatitis (for my current novel) and went off to try out a new recipe for a raspberry and amaretti gateau. Nicely used up the last of the raspberries from the garden.

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Eyes closed but wide awake

Life chez nous is becoming very scrambled this spring.  Three weddings and a couple of conferences all involving long car journeys (of the several-hundred-miles-in-one-hop variety) … looming literary appointments … elderly folk needing tlc … family commitments … amongst the usual hmdrum responsibilities. Which is a long-winded way of saying, not much time to sit writing novels.

As far as the current story (about a family whose lives are devastated by a car crash) goes, I know I need to lift the mood somewhat. And I’ve identified the way forward: another secondary narrative thread. But it requires a lot of concentration to hang on to which of my characters is doing what, where and in what time frame; to place enough cues strategically without losing pace or flow. So, with everything else going on, it’s left to the deep recesses of the night-time brain to develop this new storyline.

Which reminds me of the narrator in the fictional Diary of an Ex-Detective (1959): ‘When I am deeply perplexed it is my practice to go to bed, and lie there till I have solved my doubts and perplexities. With my eyes closed, but wide awake, and nothing to disturb me, I can work out my problems.’ quoted in the The Suspicions of Mr Whicher. I’m doing a fair bit of problem-solving with my eyes closed these days, not just during the long watches of the night, but also on those aforementioned long journeys – not, I hasten to add, when I’m in the driving seat.

But for those of you who haven’t made the acquaintance of late great Mr Whicher, here’s a summary. One summer night in 1860 the well-to-do Kent family went to sleep in an elegant Georgian house in Wiltshire. Mr and the second Mrs Samuel Kent, their children, their domestic staff. Next morning their world is blown apart by the discovery of the gruesome murder of one of the children. What’s more it seems that the perpetrator of the crime must be someone within the household.

The celebrated detective Jack Whicher from Scotland Yard is brought in to investigate but his conclusions fly in the face of the verdicts of the local police and others. He believes the daughter of Samuel Kent’s first marriage is to blame, but almost everyone else comes into the frame at some stage. And so powerful are the voices raised in opposition that Mr Whicher’s mighty reputation crumbles and he fades into obscurity. The true story only evolves gradually over many years.

The Suspicions of Mr WhicherThe book is a reconstruction of a real case but begins like a novel. Whatever its later shortcomings, hats off to Kate Summerscale for her meticulously detailed research. She weaves in the work of authors of the time, historical landmarks, other notorious cases, alongside her account of who said what, who did what, and when. Rather too many deviations, in my view, detracting from the pace of the main storyline. Probably why she needs to repeat points so often to remind the reader of the salient facts of this case. But in the process she brings into stark relief the harsh and capricious nature of the legal system of the day, with all its limitations. There’s no DNA evidence, no CCTV documentation, no sophisticated pathology result, to substantiate the circumstantial suspicions. The death penalty is meted out after short brutal trials. Public hangings are still a spectator sport.

And the class structure still divides. Whicher himself is seen by some as a greedy and inept working class fellow, meddling in middle class affairs. But by others as a fearless pursuer of the truth irrespective of class distinctions. Damned if he does, damned if he doesn’t.

His investigation certainly shines a light into a closed-up house, into the secretive world beneath the veneer of well-to-do respectability, into the divided loyalties above and below stairs, into the complex emotions of step-relations. Police procedure involves measuring breasts, examining night attire for bodily fluids, asking indelicate questions of nice young ladies – all in a context of Victorian prudery and secrecy. Sensational stuff for its age. It attracts huge media attention. And the echoes and repercussions go on for decades.

I enjoyed the clever piecing together of the fragments of a story from many sources. And the unravelling of a family’s life during that era. A clever idea adroitly executed. I wasn’t so keen on the time it took to tell the story and the repetitive elements. But it made me appreciate the fact that at least any false trails I might lay won’t lead to the gallows.

Hmmm, clutching at straws comes to mind!

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