The Huffington Post – The Duchenne Clock

The Huffington Post, 24 June 2015 – I wake suddenly and breathlessly, eyes springing open, heart thudding like a drum, as if I am a soldier on perpetual watch, and my first thoughts are for my sons. Theo fell three times yesterday, I think. His legs simply gave way. He could not keep up with Max, his best friend, who grows stronger and faster by the day, where Theo becomes weaker and slower. The disease takes its sinister hold.

How long do we have before he can no longer walk? I wonder. Time takes its course, and will not stop for him. Tick tock. Tick tock. The Duchenne clock. How I hate this ever present sound in my ear. How I wish time would stop. Klara wished for this yesterday, while lying in bed with Theo and Oskar, that time stand still for our precious boys. We do not want to witness their inevitable decline. But it will not.

Enough of this, I say, and rally myself for the day. But on my feet and in the shower, water running down my face, it is not long before my mind returns to Theo and the events of yesterday. He stood there, I remember, as he so often does, with a quiet dignity, watching Max run away from him and into the arms of another child. He knew his legs would not carry him, and so he watched them, Max and the other child, with an awkward mix of curiosity, joy and sadness. He was happy his best friend was happy, but also sad he could not keep up with him, which meant he was now alone. Theo then turned and looked at me with those blue grey eyes, which possess a striking glint of yellow, the very same as Klara’s, his mother’s – these eyes I can lose myself in – and asked, “Is Max still my friend?”

This question hurt, and I felt my throat contract and stomach tense. It hurt because it captured what his condition, Duchenne muscular dystrophy, is doing to him, slowly robbing him of his mobility, independence, freedom. Tick, tock. Tick tock. That awful clock again.

Not quite four, Theo is unaware of the severity of his condition. He senses it, that something is not quite right, that he and his brother Oskar are somehow different, but no more than this. He does not know the extent of his decline, the stages he will go through, which every Duchenne parent dreads, the legs going first, then the arms, until he can no longer even put his arms around me, and finally the heart… beating its last premature breath.

I walk back into the bedroom and sit on the end of the bed. Theo is still asleep. I love looking at him as he sleeps. He seems to embody such perfect peace. I lean over and stroke his skin with the tips of my fingers, then kiss his forehead a number of times and hold my head next to his. Then I lie down next to him and simply stare. I could lie beside him like this for hours, if I had the time. Tick tock. I am entirely his, I realise, will do whatever he asks of me. I will fight for him to the very end.

Klara and I inhabit a new reality now, almost a year on from Theo and Oskar’s diagnosis. We accept he cannot run; cannot walk upstairs unassisted, and can only go down them on his bottom; cannot walk for long before his legs hurt and he must stop. But in all these actions that his peers do without a second thought, which they, and we, all take for granted, Theo persists, always smiling. Caroline, the pre-school manager, refers to his resilience, this quality he needs to resist the disease, to ensure it is life-affirming not life-limiting. How he will need to fight to nurture this quality as he grows and the disease limits him more, I think, as I lift myself from the bed and make my way to the front door.

I do not want Theo and Oskar to die before me. No, I want them to live far beyond me, to experience all of life, its many facets and stages. What sustains us, Klara and I, is trying to realise this, to do all we can to ensure they, and other Duchenne boys, lead long and full lives, not restricted by their condition but rather empowered by it, enabling them to nurture their spirits and fully embrace life in all its joy, pain and wonder.

But we cannot do this without the care and support of others, I realise, closing the door behind me and stepping out into the street. There are so many who have helped, who give us the will and belief to carry on. Our families, our friends, but also fellow parents of Theo and Oskar’s peers, who are working so hard to help us and them. There is something wonderful in what they’ve come up with – Maria, Hannah, Rachel and others – a day of blue wigs and fun, Blue Hair Day, a day dedicated to Theo, Oskar and all the other boys afflicted by this cruel disease. It encourages us to laugh in the face of Duchenne, to face it with joy not despair, and herein lies the victory – the disease will not beat us, the clock will not govern my every waking hour, my sons and others will be afforded the time they deserve.

The Huffington Post – What will my sons do now?

The Huffington Post, 20 March 2015 –I last wrote about the diagnosis of my two young sons with Duchenne muscular dystrophy, a fatal genetic muscle wasting disease that will likely kill them before they become men, over six months ago. I was in crisis then, reeling from the shock of the diagnosis, and what this meant for me, Klara, and most importantly, Theo and Oskar.

The prevailing emotion was fear, my mind and heart plagued by an endless carousel of worry and dread. How quickly will they decline? When will they be in wheelchairs? How will Klara cope? How will I cope? Might a significant treatment or cure come? How should we bring them up now? What should our hopes and aspirations be for them, in light of their terminal condition?

Then came the realisation that many of these questions were the unanswerable cries of a desperate parent whose children were not going to be what he had expected, indeed, what he had wished them to be. Parents have the unfortunate disposition either to mould children in their own image, or to force them to be what they are not and never will be. What did I want Theo and Oskar to be? One a great rugby player, the other a great writer?

Such wishes, however, have been rendered meaningless by their Duchenne – the single defective gene they carry, which now seeks to define their whole being. To be a great rugby player requires significant physical prowess and strength, which my sons will never possess, as they cannot produce the one protein vital for all muscles – dystrophin. Without this, muscles can do little more than waste away. And to be a great writer requires time, many decades in fact, which my sons do not have either. Duchenne is ruthless in its course, giving its sufferers little more than a glimmer of adulthood before it takes the most precious organs of all – the heart and lungs.

But then, perhaps Theo and Oskar, irrespective of their illness, will aspire to be neither rugby players nor writers. In Theo, I see a charming, imaginative, thoughtful, resilient little boy who cares little for rugby. And in Oskar, though he is still so very young, I do not see a mind which aspires for literary greatness, but rather one which is ever present and playful, both of which will preclude him from being a literary great, which requires introspection and melancholy.

Much of my suffering, and the suffering of all parents with Duchenne children, in the early years of the disease at least, is attributable to the above – the shattering of expectations. And yet they are just this, merely hopes and wishes, and as I slowly let go of them, my suffering diminishes. Why hanker after what is no longer possible, indeed, what my sons might never have wanted any way.

Theo and Oskar’s disease will shape who they are, but need it limit them, their experience of life. We live in a society dominated by insatiable aspiration and achievement, and push our children to live by these cultural standards without question. Unless they become successful lawyers, doctors, accountants or engineers, they have somehow failed, not made the most of life. But are not such aspirations, in truth, life-limiting rather than life-enhancing. Success, as our culture sees it, gives us wealth and power. But what else does it give us? Very little, in fact.

The parent of a disabled child, you are suddenly forced to look with a hard and discerning eye at the society your children live in. Will it nurture them, in spite of their condition, or will it neglect them? How will they live in a society that requires them to compete first and foremost, to be faster, stronger, wealthier, more powerful than the next man?

What drives me now, more than anything, is to nurture my sons’ spirits, to give them the strength to challenge, and perhaps even change, the culture they live in – in whatever small way. Their spirits will prove far more important than what they might earn and own, what they might do for a living.

It requires considerable strength to live another way, to live a life that your parents might not want, indeed that society might not want. Klara and I will do all we can to ensure Theo and Oskar go their own way, find their own meaning in life. We will do this for them, and for other disabled children.

If you would like to help fund the world’s best researchers and scientists find a cure, please donate to Harrison’s Fund: harrisonsfund.com

huffingtonpost.co.uk/nick-taussig

The Guardian – Our beautiful sons could die before us

The Guardian, 16 August 2014 – Our beautiful sons could die before us: Nick Taussig thought his son Theo was a bit of a late developer. If only that were true. Doctors diagnosed Duchenne, a devastating genetic disorder – and everything changed. He will not rest until he finds a cure.

We judged it to be little more than a routine appointment with the pediatrician, and so only I had gone, not my wife Klara. Both of us did not need to be there. Would she not confirm what we suspected? That our soon-to-be three-year-old son, Theo, was just like me, no more than a late starter. My mother had reassured me – as only mothers can – that I had been the very same. I did not crawl, just walked, and this I did about a year too late. And my speech, well, that was late also. I had said nothing until I was two and a half.

Theo was his father’s son, therefore, developing in his own inimitable way, and the pre-school manager was simply being neurotic and overzealous in her concern for Theo’s development – his failure to hit certain key milestones. His nursery was not worried in the slightest. An occupational therapist, a physiotherapist and a speech therapist had assessed him, and they had raised no major concerns. And so was this not just final confirmation that Theo was fine, albeit a little slow in getting his act together?

The pediatrician took to him immediately, Theo charming her with his big blue eyes and shy smile, behind which lurks a great confidence, as if he already knows he’s in possession of good looks, which will serve him well as a teenager and young man. She took out some toys. He did as he always did and flopped clumsily on the floor, almost in the manner of a rag doll, legs splayed either side, and began to play. She asked me about him, the usual questions. When did he sit up on his own, crawl, walk, talk, blah-blah-blah? I answered as best I could, unsure of exact dates, though my answers were not important because, well, he was his father’s son.

He sat for quite a while and played. “I think he’s an intellectual,” the pediatrician said warmly, then asked him to get up, and he did as he always did and heaved himself to his feet, using his hands to help him stand, looking as if he was climbing up his legs. She then said she wanted to see him walk, and we left her consulting room. Theo, excited by the change of scene, did as he always did and waddled fast down the corridor, his idiosyncratic gait – hips swinging camply from side-to-side – a perfect metaphor for his development: he did things his way. And lastly, she wanted to see him walk up some stairs, which he likewise struggled with, taking one tentative step at a time whilst clutching at the wall in the absence of a rail, until after a few steps he gave up, crawled up a few more, then insisted that daddy carry him the rest of the way.

We returned to her consulting room, where she explained she would like to take a blood test, which I judged to be no more than precautionary. We arranged it for the next morning. And that was that.

She called the following afternoon, the results already through, asking to see us tomorrow, and this time could we both attend please, mum and dad. “Is there something wrong, do you think?” Klara asked. “No, not all. I think she’s just being very efficient,” I replied.

We walked into the hospital again on the morning of Thursday 26 June. This is just six weeks ago, I realise as I write this, and yet it feels like an eternity. Why does time have the habit of slowing down quite so much when we are in pain? I still cannot remember exactly what she, the pediatrician, said, though she spoke with great tenderness, her voice quivering, on the verge of tears. “He has very high levels of creatine kinase…this points to muscular dystrophy…and by virtue of his age and sex, it’s statistically most likely he has Duchenne.” I could not bear to hear this last word, which possessed an appalling finality, but even worse, I could not bear to see my precious wife floundering with the diagnosis, because she did not yet know what I knew, what this word meant.

I knew because a year before I had approached Alex Smith of Harrison’s Fund further to reading a newspaper article entitled, “I Wish My Son Had Cancer,” Alex alluding to the fact that cancer research is far better funded and resourced than muscular dystrophy. I was interested in making a documentary film about his extraordinary attempt to complete an Ironman Triathlon – a 3.8km swim, a 180km bike ride and a 42.2km marathon – carrying his 35kg disabled son, Harrison, the whole way. To this end, I’d even written a pitch for broadcast, describing how Alex had first learned of his son’s fate, the doctor explaining that, “Harrison cannot produce dystrophin, a protein we all need to build up and protect our muscles. As a result, every muscle in his body is deteriorating. He will most likely be in a wheelchair by twelve, will suffer from respiratory failure, heart failure and other debilitating orthopaedic complications, and will die in his late teens or early twenties.” Was there any hope? Alex had asked. “No, the disease is 100% fatal,” the doctor had replied. This was now my son’s fate as well.

I began to cry, then found I could not stop. I was barely able to speak. The appointment ended with the recommendation that Oskar, our eight-month-old son, be tested also. Klara thanked the pediatrician, and the four of us left. I held Oskar, while Theo took mummy’s hand. We walked aimlessly through the maze of corridors, until Klara eventually suggested we go to the canteen and eat something: the boys needed their lunch.

I ordered some food, for Klara and I too, though well aware that neither of us would eat. How could we? Klara uttered the word, at which point I told her what I knew of Duchenne. She started to weep, in this dour canteen, as a group of student doctors looked over at us, likely wondering what diagnosis we had just received. Theo responded by smiling at his mother in between a large mouthful of mozzarella and tomato panini, as if reassuring her that, in spite of the fatal genetic illness he had just been diagnosed with that would kill him before he was a man, all would be okay. And I could not bear this smile, because at that moment all could never be okay, as our son, our beautiful son, was going to die before us.

We have to call family and friends, Klara and I realised when we got home, and this was exhausting, as they all responded so differently, some crying while others held it together, doing their best to be strong and supportive. We got to the point where we were tired of having the same conversation over and over. “What’s the treatment?” There is no treatment. “But what about steroids?” They do little more than postpone the inevitable by a few years. “Might there be a scientific breakthrough?” Possibly, though we cannot count on this. “How are you?” We’re ok, when what we really wanted to say was we’re drowning. “You’re so brave.” Thank you, when what we really wanted to say was what fucking choice do we have, should we just give up now?!

The next few days and weeks, Klara and I entered our own private hell, the shock of the news quickly replaced by a desperate grief – it was as if our son had already died – and in my case, a malignant sadness, which gnawed at my heart like a cancer. I was unable to sleep, to think, and struggled to even look at Theo, as whenever I did, all I saw was his cruel and painful decline, his muscles wasting away before my eyes. He would never play rugby, never make love, never make it to university, never realise his full potential. One morning, after just a few hours sleep, I went for a run. I pushed myself until I could not take another stride, then sat down on a bench and wept, praying that Theo live a full life and I die.

Oskar was diagnosed with Duchenne as well. Klara was distraught when I called from work and told her. Her parting words to me were, “We must be incompatible.” I did not tell her that she is the carrier of the defective gene, X-linked inheritance a defining characteristic of Duchenne. I hurried home as fast as I could, desperately worried for her. I felt like I inhabited a nightmare as I stood there, on a packed platform, listening to the loud and continuous laughter of a group of businessmen beside me. Lost in a haze of grief, I got on the wrong train. When I did finally get home, I found Klara with my parents. She looked fragile, unlike her, as she is always so very strong. “It’s me, isn’t it, I’ve given it to them…” she uttered, and all I could do was hold her and kiss her and tell her I loved her.

The following week, the love and support of family, friends and colleagues proved vital, sustaining Klara and I when all felt lost. We found ourselves fluctuating wildly between hope and despair, one minute sure that a cure would be found in time, further to reading yet another article online, the next convinced that one would not, with us doing little more than deluding ourselves. It took my sister to remind me that, “Right now, the boys are very happy, and will continue to be, as long as you give them all your love and care.” And she was right.

I will cherish them to the very end, but also will not rest until a treatment and/or cure is found, of which there is the real prospect of one now. There are 2,500 British children with Duchenne and 300,000 worldwide. Ninety nine per cent of sufferers are male. It is a cruel and horrible disease, and the most common fatal genetic disorder to affect children around the world.

Last weekend, we were in a children’s playground. Klara was helping Theo navigate a few of the different climbing frames. A father behind them escorted his two daughters, Theo’s age or thereabouts, and became increasingly impatient. Why is this boy so slow? Why is his mother so protective of him? he wondered, then huffed and sighed. I wanted to rip his head off. How dare he dismiss my son in this way! Does he not realise that this woman, my beautiful wife, is not cosseting her child, but rather aiding him, because his hips and shoulders are too weak? My son has a disease, is disabled, and this prick doesn’t realise it. Well, I will make him realise, I thought, as I prepared to confront him. I looked at Theo again and he was smiling, enjoying the challenge of the last obstacle, and I looked at Klara, who was so present with him and always has been. Christ Nick! I rebuked myself, and walked away.

In that moment, I understood that I have cherished and loved Theo and Oskar more this past month than ever before. Their diagnosis, in this respect, has been a perverse blessing, forcing me out of my stupor and delusion, and urging me to really be with them as opposed to being too often preoccupied with work and worry. Klara knew from the moment they were born that we were blessed and privileged to have them, and has never lost sight of this. But I did lose sight, on occasion. I took them, my beautiful boys, for granted.

A wonderful man, a friend of my sister’s, said to me a few weeks ago, “Certain children choose their parents, and Theo and Oskar have chosen you and Klara, as you possess the qualities and strength they need.” Before I was told my sons were dying, I would have dismissed these words as no more than sentimental claptrap, but now, I hold them close to my heart.

If you would like to help fund the world’s best researchers and scientists find a cure, please donate to Harrison’s Fund: harrisonsfund.com

theguardian.com/lifeandstyle/2014/aug/16/our-beautiful-sons-could-die-before-us

The Big Issue – Nick Taussig, Five Crime Novels Everyone Should Read Before They Die

“A dazzling study of mental anguish and moral dilemma” Author Nick Taussig picks his essential crime fiction reads… The Big Issue, 7 August 2013

 

1. Crime and Punishment, Fyodor Dostoyevsky
Following the nihilistic student Raskolnikov, this is a dazzling study of mental anguish and moral dilemma.
2. The Godfather, Mario Puzo
We’ve all seen the trilogy of films but read the original novel, stunning in its detail of the inner workings of the Cosa Nostra.
3. Papillon, Henri Charrière
First published as a memoir – the tale of a French criminal escaping from countless penal colonies – it later transpired the author was prone to fantasy and that the book was a novel after all. But what a creation.
4. The Spy Who Came In From The Cold, John le Carré
The master espionage writer’s third novel was brilliant, not least because you feel the writer in every sinew of his hero, Leamas.
5. The Big Sleep, Raymond Chandler
Revel in the plot’s complexity and how Chandler leads you one way then the other. Yes, the double-cross extends to the reader also.
Nick Taussig’s latest novel, The Distinguished Assassin, is out now in hardback (Dissident, £12.99)

Independent – Five-minute memoir: Nick Taussig recalls a particularly trying trip across Russia

‘Mother Russia’ had long intrigued the author, but a journey across the country almost changed his mind… The Independent, 3 August 2013

A lifelong student of Russian literature – no one wrestles with the shadow self quite like a Russian novelist – it was perhaps inevitable that I would write a novel profoundly Russian in character, and that in order to write it I’d first have to travel Mother Russia’s length and breadth, from Moscow to Magadan.

This preoccupation, with the likes of Dostoevsky, Tolstoy and Solzhenitsyn, was likely inspired first, by my Slav background – my grandparents Central European Jewish émigrés – and second, by my Czech wife, who like so many had the great privilege of living under Soviet Communism, this quintessentially Russian creation.

For only the Russians were willing to submit themselves, for quite so long, to such a contemptible system, to live according to a single prescribed ideology, which claimed to offer a lifetime of perfect Communist harmony but actually provided the very opposite, a lifetime of hardship, frustration and dread. Why was this? The answer surely lay in a Russian pilgrimage, I concluded.

Moscow I could handle on my own, I thought, though for the rest of the journey, to Siberia and beyond, I’d need a fellow pilgrim, and crucially one who spoke more Russian than I did. I was very fortunate here. My father, a passionate historian, linguist and former director of foreign language services for BBC World Service, agreed to accompany me, and what proved most valuable, when the odyssey got difficult, which it did, was less his above credentials and more the man himself – his great, indefatigable, big-hearted, fiercely intelligent and slightly mad (but in the best possible way) spirit. Without him, Mother Russia would have got the better of me.

Things got tricky in Moscow after just three days, when I was suddenly, and rather unceremoniously, accosted by two men while taking pictures of the Lubyanka, the former headquarters of the KGB. Dressed in dark suits, the two men, whom it transpired were FSB (Federal Security Service) agents, had bounded towards me, and before I quite knew what was happening, took me by either arm, and without saying a word, marched me through an underpass. They held me outside a side entrance to the main building of the FSB, while a third man, having confiscated my passport, ran checks on me inside, sure that I was a British spy. The two agents, big, bolshy Slavs, said nothing, simply stood guard over me like two great Russian oaks. Only after 40 minutes, when their colleague returned, did they release me, having deleted every photo I’d taken, their parting words, “Fuck off!”, spoken in perfect English.

What I immediately gleaned from this experience is that the country remains insular, suspicious and authoritarian, having placed far too much power in the hands of its security services. Perhaps the Russian people are predisposed to live under strong authority, I wondered, and thus accept the terrible abuses of Putin’s authoritarianism, just as they did Soviet Communism.

The flight from Moscow to Magadan, some 5,500km east, was nine hours, yet this did not include the stopover in Bratsk, where we were herded like cattle into a dingy brown Soviet-era waiting room and instructed to wait, for how long it was unclear. After three hours, my father had the gall to ask an airline attendant, “When might we be on our way again, do you suppose?”. His question met with the curt, officious answer, “Soon!”. No more was offered. In Russia, the customer is not always right. We finally boarded again, and when we arrived in Magadan were exhausted, this compounded by the fact that we had flown across eight time zones but were still in the same country.

We had not booked a place to stay in advance – my failing – and so trawled Magadan’s few hotels in search of a spare room. None could accommodate us, but for the last. We were taken to our rooms – designated for non-Russians. My father smiled wryly, as both of us shuffled wearily into our respective abodes. He clearly foresaw what was in store, and well, we were not to be disappointed. The wallpaper was peeling, the plaster cracked, the furniture dilapidated, the bathtub Stalin-era – little more than a urinal – the room fit for a fugitive and no more. It was, like the airline attendant, Bratsk Airport, the two FSB agents and Moscow itself, grim, colourless and indifferent. I sat down on the poorly sprung bed, then heard my father howl with laughter. “The pilgrimage is complete. What better view of the troubled Russian soul than this, comrade. How they’ve made us suffer!”

Nick Taussig’s latest novel, ‘The Distinguished Assassin’, is out now

independent.co.uk/arts-entertainment/books/features/fiveminute-memoir-nick-taussig

Marcel Berlins reviews “The Distinguished Assassin” in The Times

“The Distinguished Assassin is Professor Aleksei Klebnikov, banished to a Gulag labour camp in 1949 on trumped-up charges. Set free in 1952, he becomes a hitman for a gangster, assigned to murder six brutal, highly placed Communist officials. Klebnikov’s ultimate aim is to kill the man responsible for his captivity and who, he believes, seduced his wife in his absence. The story is told in alternate chapters covering his time as prisoner and after his release. Through Klebnikov, the plight of the Russian people under Stalinist rule is grippingly demonstrated. Taussig’s style – short on dialogue and long on descriptions and Klebnikov’s thoughts –takes a bit of getting used to, but turns out to be effective for the passionate political and emotional content of his novel.”
Marcel Berlins, The Times

The Times – 8th June 2013