Hazel McHaffie

degenerative disorders

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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A short stay in Switzerland

Panoramic trainD’you remember the BBC film of this name, A Short Stay in Switzerland, a dramatisation of the last days of Dr Anne Turner who developed an incurable degenerative disorder (PSP)? She made the front pages of the papers with her letters to friends and relations to say, ‘By the time you read this I will be dead‘. In January 2006 she travelled to Dignitas to end her life, the day before her 67th birthday, while she was still able to move and voluntarily take the lethal medication. And a report this week says that almost a quarter of terminally ill people who avail themselves of the suicide clinic’s services are from Britain (second only to Germany).

MatterhornWell, I’m grateful to be able to report that my own short stay was of a quite different order. I had eight days to revel in the spectacular scenery, travel on the world famous panoramic trains, listen to the enchanting melody of cow bells in the mountains, and inhale the pure Swiss air, with no sinister intent. All I had to do was soak up the beauty and recharge the batteries. Wonderful.

I did my best not to let the Dignitas issue cast a shadow over my holiday, but of course, books featured. After all, this was real Heidi country, Johanna Spyri was born, lived and wrote in and around the rural area of Hirzel and Zurich, and used Graubünden for the setting of her books – all places I visited. Although Spyri struggled to find a publisher initially, the two Heidi stories went on to become by far the most popular works of Swiss literature: they’ve been translated from German into 50 languages, filmed more than a dozen times, and over 50 million copies have been sold world wide. Swiss pasturesSo evocative were they of the Swiss Alps that the real locations exactly conformed to my childhood mental images.Swiss cows

Switzerland is also the stuff of the Chalet School series by Elinor M Brent-Dyer, another big part of my growing up. Stories of schoolgirls who spoke three languages fluently, whose lives were overshadowed by the sanitorium, and who seemed to grow up to have lots of children also destined for the Chalet School.  Old hardback Chalet School booksI collected most of the hardbacks (secondhand) in my youth, and passed them on to my daughter, who recently completed the set (58 books), paying a good deal more for rare copies than I ever did! Paperback Chalet School booksThe full complement are destined for the next generation. What a lovely legacy. I might even read them again myself some time – this time in the correct order! – and fill in all the gaps.

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