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Living with Huntington’s disease: ‘For our family, the end of days is always close at hand’

The day I found out how I was going to die began innocuously enough: the usual blur of nappy changing and tetchy texts to my husband. Life in our recently refurbished London home had settled into a rhythm, with a low-level background of domestic discontent. Arguments about wallpaper had run their course; our cats had made their peace with our one-year-old daughter, Anna; and I was pleased to have married a responsible hedonist who liked babies but never made me feel guilty for finding them boring.

That day, my husband, Tom, had gone to work early; a documentary director, he was filming a series about the London Underground. After a sleepless night, I was eating breakfast with Anna when the landline rang. It was my dad’s old friend Eric, who had been keeping an eye on him ever since my mum had died four years earlier. We were all worried because Murph (everyone called my dad Murph) had been making some bad decisions, then digging in defiantly.

Eric said there was something I should know: Murph had been diagnosed with Huntington’s disease (HD). I’d never heard of the condition and had no idea what it was. So of course I did the one thing you should never do: I Googled it. Wikipedia made it sound a bit like Alzheimer’s and a bit like Parkinson’s, but worse than both. It was a degenerative neurological condition and it sounded terrible; poor Murph. Though it made sense, given some of the things I’d noticed about him since my mother died. He was twitchy and fidgety, and there was a kind of hesitancy to his once-confident stride.

Then there was a bit about genetics. In dry Wiki-speak, “HD is inherited in an autosomal dominant fashion. The probability of each offspring inheriting an affected gene is 50%.” All the things that would happen to my dad might also happen to me or my brother. And if I had the gene, my children would be at risk, too. Unlike Alzheimer’s, HD typically manifests in midlife, between the ages of 35 and 45. I was 35.

The first visible sign is the chorea – jerky, uncontrollable, involuntary movements in all parts of the body. As parts of the brain degenerate, patients suffer severe cognitive problems: loss of memory, of judgment, of the capacity to organise oneself. They find walking difficult and are prone to falls. They lose the capacity to swallow and sometimes die of malnutrition. Their personality is often affected, too. Reports of aggressive, compulsive and sexually inappropriate behaviour are common. Towards the end, families often see no other option than to have their suffering relatives institutionalised. There is no cure for Huntington’s disease.

I called Tom. It was about half past eight and he was on his way to film at Edgware Road station. I was in tears as I explained what Eric had told me and what I had read. As usual, he tried to calm me down, but for once his insistence that the internet was bound to be exaggerating the scale of the disaster was sadly misplaced.

Then the phone went dead. He had arrived at the station minutes after a bomb had been detonated on a train in the tunnel beneath. It was 7 July 2005, and for the rest of the day he was out of contact, caught up in the aftermath of the worst terror attack the capital had seen.

I turned on BBC News 24 and watched events unfold. I kept trying Tom’s phone but it was going straight to voicemail. I can’t remember many of the details of the day, but I do know how it felt. There was a sense that our lives had darkened around the edges. When Tom came back into range I was weeping with relief, but still unable to process the news that had blindsided us.

A mutual friend had introduced me to Tom at a party in Notting Hill in 2002. All the men in my life up to that point had been dysfunctional to varying degrees, but it was clear from the outset that Tom was both sane and interesting. Our first night together was a drunken laugh. There were no dating apps in those days – we just busked romance. It was hit and miss but more enjoyable for it. What would Tom have put on his profile? Would I have found his passion for Fulham football club off-putting? There was also his taste in clothes: I was a style fascist with an image to maintain, and wouldn’t normally have been seen dead with someone in Berghaus trousers.

But when he was speaking, all the other people in the room faded to grey. It really was love at first chat. He even made football seem fascinating. I was pleased to see my self-limiting beliefs disappear between the sofa cushions. We had so much in common. His favourite song was my favourite, too: Another Girl, Another Planet by the Only Ones. At that point, the little areas of difference still seemed interesting rather than threatening.

We kissed on top of the coats in the spare room. We went back to my house, and in the morning I wanted a McDonald’s breakfast. We both had hangovers, but Tom set off up the high street in search of Egg McMuffins. He got lost on the way back. I was worried he wouldn’t return; there were a few anxious minutes of missing him. This little scene introduced all the major themes of our subsequent relationship. Tom was brought up to be dutiful; I was raised to be bossy and needy. Looking back, I wish I’d got dressed instead of lying there like the Queen of Sheba. We could have gone to McDonald’s together. What could have begun as a collaborative partnership was skewed in my favour from the start.

A few days later, I ran into a friend and told him I’d met the person I was going to marry. I’d never thought about marrying anyone before. Scrolling back through all my relationships, I could see that none of my exes would have made good fathers, even if they’d been the marrying kind. Once I’d decided this, I thought it would be only a matter of time before Tom was persuaded, by force of argument, that I was the right woman for him.

A year later, Tom and I were in Skye on a mini-break with a friend and her soon-to-be-ex-husband. We’d spent the whole weekend arguing. I was impatient for romantic avowals, or a full explanation of why he hadn’t proposed yet. Surely he knew everything about me now?

I had form when it came to demanding presents before they could be freely given. Murph always gave me money for my birthday, which is in September. Instead of waiting, I would ask him for it in August – or July. In Skye, I wanted Tom to advance his marriage proposal in exactly the same way. But my nagging had the opposite effect (no one likes to be chivvied) and we nearly split up. Things were looking dicey on the way home but then, in the baggage reclaim area, probably still feeling ambivalent about me, he produced a diamond he’d had on him the whole time. When we got in the car, we both felt cheated of the moment he had planned as a memory to cherish. But it was all my fault.

Now, 15 years after my diagnosis, I wonder whether this kind of unreasonable and ultimately self-defeating behaviour was a product of my upbringing, or of Huntington’s disease, or a combination of the two. It would be far easier to blame HD than myself, but I can’t help thinking that I wasn’t symptomatic when I was being horrible to Tom. I may have been prematurely unempathic as a result of the disease, but that’s no excuse for throwing your weight around.

We married in 2003, at the Old Ship hotel in Brighton. From the moment I booked the wedding planners to the last song of the party, the script was a bride-focused fantasy in which Tom was marginalised. We did agree Brighton would be the best setting and both liked the Old Ship. But that was the last decision we made together.

Photographs and glass vases on the mantelpiece in Charlotte Raven's house in London, Sept 2021
‘As my short- and long-term memory were affected, I began to lose my own past.’ Photograph: Thomas Duffield/The Guardian

In the early days of our marriage, I was hypercritical about everything and everyone, including Tom’s friends and family. Having started out on the Modern Review, with Toby Young and Julie Burchill, I had become a newspaper columnist. But being paid to be horrible about people caused collateral damage to my relationships.

My opposition to him having a social life was argued on a case-by-case basis. Someone he’d met at university who became an internet entrepreneur was cast out on political grounds. Another friend wanted us to play parlour games at her 30th birthday, so I made Tom drive me home (I hate parlour games). His family played Consequences on Christmas Day, which I scorned (the only Christmas traditions in my family were drinking too much and watching TV). So I’d sit it out, tutting.

I didn’t want him to enjoy himself unless he was with me or in my milieu. When he did manage to prise himself away for an evening, I would text him compulsively. It was airless and oppressive. If we went out with his friends, I would dress to kill everyone in range.

We had plenty of space but I wanted to upsize. I found my idea of a for ever home on a lovely corner plot in a street with cherry blossom on the trees. While we were renovating it, I found out I was pregnant with Anna.

Someone once described HD as an illness of mourning, which seems very apt. You lose your identity, and some of your humanity, while remaining aware enough to keep a tally of every loss. After I found out about Murph, I decided to take a test to find out whether I had the gene. I had assumed most in my position would choose to do the same, so I was surprised to learn 95% of them don’t. To me, wanting to take the test was the rational response, avoiding it the cowardly cop-out.

When I found out I did have the HD gene, what was there to do but cry? Even stoical Tom looked stricken.

For me, it started with small, unexplained absences: car keys, glasses, a million lighters, shoes, clothes. Then I lost the world, city by city. Familiar places became a scary tangle of streets, so I stayed in the house. Then the car itself started to go missing: when we stopped at services, I could never find my way back to it. Bigger human losses followed. I lost my sexuality. Friends stopped remembering to visit me. And then I began to lose my own past: as my short- and long-term memory were affected by HD, the story of my life receded into the distance and became increasingly inaccessible to me.

Before all those losses, I was living as fully as one could with the prospect of total identity collapse. Our son, John, was born on a lovely day in April 2009, three years after I tested positive and before the onset of my symptoms. It took us a long time to decide whether to have another child, knowing they would have a 50% chance of inheriting the gene mutation. But ultimately I didn’t want Anna to be an only child, coping with weird me and having no one to play with.

People with Huntington’s disease may sometimes seem uncaring and thoughtless. Their apparent disregard for the emotional needs of a partner can be hurtful, especially when it contrasts with a formerly loving relationship; the natural tendency is for the partner to feel slighted. In these situations, the person with HD is not being deliberately awkward, wilful or unkind – their apparent self-centredness is a consequence of the loss of mental flexibility associated with the disease. They may no longer be able to put themselves in another’s shoes or weigh up both sides of an argument. They may genuinely fail to see how their actions affect others.

My various impairments kept our household from running smoothly until we found a carer, Ade. She also helped with admin and my to-do list. But empathy can’t be outsourced; my family needed a mother who could connect with them, and I was often too self-absorbed. Tom parented for the two of us (as well as earning all the money). He remembered their PE kit, involved them in creative activities such as baking so they didn’t sit in front of the telly all day, controlled their texting and took Anna to football. More importantly, he was there for them emotionally when I was semi-detached. I couldn’t be trusted with their weekly schedule; I even forgot John’s Christmas show. (“You were the only mummy who wasn’t there. The only mummy!”)

If I felt guilty, there must be hope for me. Could I have spent so long brooding about my inhumanity if I really was inhumane? I’ve read accounts of HD sufferers who feel no shame about behaving inappropriately or splashing out on cars they can’t afford, whereas I am constantly racked with guilt about ill-advised purchases and being a shit parent. I welcome this in, as a sign of life.

A note on a whiteboard in the London home of Charlotte Raven
‘He made daily to-do lists so none of us could forget anything.’ Photograph: Thomas Duffield/The Guardian

Tom has good reason to believe I was prematurely unempathic. Our relationship wasn’t loving or collaborative in the first place, so there were few reserves of goodwill to draw on when HD came to call.

Fast-forward a couple of years and I was finishing the milk before the others could get to it and sending Tom to the shop for more – every day. I never slept and made sure no one else did. My needs blotted out everyone else’s, and when I wanted something I had to have it now. I needed to be fed, loved and listened to before the children, which was a tall order as I had never been more unlovable.

My lack of empathy delivered a mortal blow to my marriage. The day before one of Tom’s work trips was always a blur of activity, so it didn’t seem all that surprising when, one morning in 2014, he took all the rubbish from our junkyard of a garden to the dump in a hired van. He had fixed the drains, called the gas people about the leak, taken John to his trapeze lesson, mowed the lawn, batch-cooked for the week, hung up all the washing and made daily to-do lists so none of us could forget anything while he was away. After a few days it occurred to me that this may have been a dress rehearsal for when he left for good, and so it proved.

We agreed to live apart. Tom deserved a second chance to be intimate with someone still capable of intimacy. It wasn’t just that, though: as well as desexualising me, HD has blunted my emotions. Tom promised he would make sure I had enough support, and he has been true to his word, but having people around to help with the practicalities and keep me company in the evenings is not the same as having a husband.

We made a plan for how we were going to tell the children we were separating, but I ended up ignoring it – I’d always tried to be straightforward with them and felt I needed to account for the fact that I was gloomier than usual. Anna barely looked up from her phone: “It was pretty obvious, Mum.” She said she wanted to live with me and seemed pleased that Tom and I were getting on, unlike her best friend’s divorced parents who couldn’t be in the same room as each other.

John is very sensitive, so I was sad but not surprised when he told me how affected he had been by our rows: “You thought I was upstairs when you were shouting at Dad, but I was outside the door.” Then, on our way back from school one day, he said, “If you and Dad divorced, it would mean a Huntington’s lady would be on her own.” Anna and I reassured him that Tom would always look after me even if we were no longer living together, but of course he was still worried.

As for me, I was heartbroken and at times indignant. How the hell could he leave me? Deep down, though, I realised if I’d treated him better before I got ill, we’d still have been together. He may only have stuck with me for as long as he did because he’d been raised to be dutiful to the point of self-abasement – if it hadn’t been for the HD, he would probably have left me years ago.

Tom moved into a flat around the corner; confusingly, we had never got on better. He said letting him go had been an act of love that would be an inspiration for Anna and John in years to come. But it was always horrible when he went home to his flat. I couldn’t stand the idea of him being with someone else, not least because they would have had to be unusually intelligent and empathic to get their heads around our unconventional setup – a woman of substance, who felt like competition when I contemplated her. Then I started to get used to it. The only thing to do is to accept the new normal.

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I wish I’d nurtured him when I had the chance, but it’s too late for regret. I don’t want to feel burdened with guilt about the times I forgot his birthday, and can see only one way of avoiding this: remember it from now on. I want to celebrate the good things we still have.

John empathises with me, picking things up when I drop them (although, sadly, he must still be paid to embark on any household chore that takes longer than three minutes). Anna and Tom, and even Tom’s family, empathise with me, offering constant reassurance that none of this is my fault. Somehow, though, I can’t now seem to empathise with myself. My therapist kept crying when I told her about the end of my marriage and the daily frustrations of life with HD, but I didn’t. I could see that it was right to work on surmounting my guilt, but forgiveness and self-acceptance seemed elusive.

When things settled down, I started to think I hadn’t done such a bad job, given the hand I’d been dealt. I helped to look after my family and kept it on an even keel in demanding circumstances without moaning or longing to be back at the top of the bill. I found this unexpectedly rewarding and did not experience motherhood as a demotion, as I might once have done. I started writing a book in the study next to the kitchen, and discovered I didn’t really mind the noise of the children coming in and out with their friends, after all.

My HD has marched on, and my relationships are reconfigured. After four years of separation, the divorce papers were served. If I had been in a better mood, a celebration might have been in order. On my birthday Tom said what a great mother he thought I was and that Anna has blossomed into an intellectually rigorous and empathic teenager who we are all proud of. John has developed a love for performing arts and goes to a dance school, navigating public transport every day on his own. On the way home, he texts me to ask how my day was, and there are lots of small check-in moments like this where I sense his presence. I sometimes wonder if his anxiety has anything to do with the fact that he was made aware of my mortality – and his own – at such a young age. For our family, the end of days is always close at hand. Anna wants to be tested for the gene when she is old enough, but I have reassured them both that, by that time, drugs will be available that treat the illness and not just the symptoms. The children’s love has enveloped me with positivity, which I couldn’t have done without. Meanwhile, the book went to full term, and this writing project has led to a transformation of my fortune. I was an onlooker to other people’s lives for longer than I’d hoped, watching parties happen around me. Now, finally, I am the author of my own destiny.

Extracted from Patient 1: Forgetting and Finding Myself by Charlotte Raven, published by Jonathan Cape on 4 November at £14.99. To support the Guardian and Observer, order your copy at guardianbookshop.com. Delivery charges may apply.

For information and advice, contact Huntington’s Disease Association