The Bravest Thing I've Ever Done.

I am a huge physical coward. I climb carefully into the swimming pool down the ladder and shudder at the idea of diving in. The very thought of bungee jumping makes my stomach turn. I don’t cycle on the road any more because I’m scared of traffic. But I did do a very brave thing, one of the bravest things that anyone can do, when I took the test to see if I would develop Huntington’s Disease.

This is in the news today and it is ten times braver to do it in public with a camera crew. A few years ago my friend Jaqui did the same on the radio.

Their courage is just astonishing. 

It seemed a good time to share my story too, as I’m writing a memoir about my mum, Nick, and how Huntington’s came into our lives. It’s still work in progress but this is an excerpt about my decision to take the genetic test. I'm eternally grateful for the huge support that was around me at the time, and that for once in my life I did something really brave.

“Nick and I were having a beer, talking about Mum. The misery of her last few years, how the illness had stolen her life.

         ‘If I thought this would ever happen to me, I’d kill myself. Seriously, I would rather top myself than end up like that.’ 

He had said this before, whenever our conversation danced around the one subject that we could never quite properly talk about. But it won’t be you who ends up like Mum, I privately thought, it’s going to be me.

‘None of us know what life is going to throw at us,’ goes the saying, but actually with a Huntington’s diagnosis you do know. And it’s never good.

When you have a 50% chance of inheriting an incurable illness, the proverbial axe is always poised over your head. Or maybe the Monty Python giant foot. I don’t know if anyone not directly affected by something like this can really understand the feeling. But I will try to explain.

You are born with this built-in time bomb ticking away inside your body. And so are we all, you may say, but unlike any other illness I can think of there’s a world of difference between ‘likelihood’ and ‘definite’.

It’s not a predisposition that might be averted with careful lifestyle choices and good luck. No surgery can remove the affected part.

If you have the HD gene you will develop the illness, the one you have already seen destroying your parent or siblings, and it’s this inescapable repetition that is so nightmarish.

         So, unless you are 100% sure that you will not inherit the HD gene, if it is in your family then you are always wondering – Will it be me? And if you have siblings, inevitably – what about them?

Well, how can you think about that for long without driving yourself mad?

If you’re brave enough, you can take a predictive test that will analyse your DNA from a blood sample. The test became available in the UK in 1993 and will reveal whether or not you have inherited the genetic mutation in chromosome 4 that causes Huntington’s Disease.

The blood analysis will measure the repeat sequence of Cynosine, Adenine and Guanine, three chemicals that form the building blocks for a person’s DNA. In a “normal” person, these chemicals display a certain number of CAG repeats, usually up to 35 times.

Someone carrying the defective gene will have a much higher number of CAG repeats – typically between 37 and 45, which is the inevitable marker for the illness.

Could a high number be a mistake, miscounted or misinterpreted by an inexperienced lab technician? No.

So that’s what the blood test will show – your number of CAG repeats. Significantly higher than 35 and the HD gene is stamped through your DNA like writing in a stick of seaside rock.

If so, however young and healthy you are now, there is no escaping the disease. As typically it doesn’t begin to manifest until mid-life, it can be hard to believe that you are carrying this invisible time bomb. You can disown your family, emigrate to Australia, call yourself a different name or have a sex change. But if it has marked your card, it will come for you. No escape.

The testing process is lengthy because this is clearly not a decision to be taken lightly and there are staging posts along the way where you can discuss your fears and back out at any point before actually taking the blood test. But do you really want to know? It’s such a gigantic decision that many people don’t.

As a counsellor had said to Nick and me after Mum died, ‘You are young – get on with your lives. Don’t let this dominate you, who knows what could happen, you could live in fear of Huntington’s and then get run over by a bus!’

Not the most cheering way of putting it, perhaps, but it did the trick for us. For a while, anyway.

Because it is hard not to live with some level of fear. You do get on with life, other things take centre stage and Huntington’s gets gradually shunted to the dark corners that you don’t investigate too often.

Living with that knowledge in the back of your mind is like living under a shadow; it soon becomes normal, disregardable, your eyes adjust so you forget it is there. But nevertheless….

And as you get older and approach the age when it might begin, an undercurrent of superstition starts to envelop everything you do. When you know it might be you next, your whole being conspires to avoid properly thinking about it, even though it’s all you do think about.

Generations of families can be affected in this way, knowing exactly what’s going on but not talking about it. Watching. Dealing with the fall-out of early symptoms, which can range from a slight tic to hyper-mania, car accidents, obsession, crazy spending and sudden violent rages – but somehow not acknowledging it as the onset of HD.

Even when you are actively looking out for the symptoms. You know they’re there and in hindsight it’s so obvious, but it’s as if your brain simply cannot acknowledge the significance. It’s a weird paradox.

Whole families become experts in denial because the reality is just too overwhelming to face.

So you can read up about the illness and genetic science. You can campaign, you can talk about HD and do fundraising ‘til the cows come home but when you or a loved one are at risk yourselves, there’s always a tender nerve inside that you avoid going too near.

         Is this it? This twitchy eye, that dropped plate – is this the onset of Huntington’s? You worry about that all the time and yet when the evidence is actually there you can’t seem to see it. It’s the permanent elephant in your living room that you just can’t look at directly, however many times you have to walk around it.

I was like that for years, and then one day I just got tired and booked myself in for the test."

Following in Dad's footsteps

It’s Carers’ Week and I’ve been thinking about our dad, who looked after our mum as her illness got worse, and never had a tenth of the support I have had with Nick.

I‘m not sure that he would have liked to think of himself as “a carer” but that’s exactly what he was, and one who set a gold standard for the role.

This is a little excerpt from my book about Huntington’s and our family, thinking of all the things Dad did for our mum. Realising how much I’m following in his footsteps – just as many generations of HD families have done before us.

"I look back and wish I could talk to Dad about all this. I wish that with the wisdom and experience I have now, I could take the load off him a little - or a lot. Now that I have become my brother’s carer, I understand so much more about the duties of love.

We would have very different experiences of the caring role: so different. Ma lived at home with Dad and never had carers the way Nick does; all the bathing and dressing and bed-making and bum-wiping was down to him. The shopping and the cooking, the careful preparation of fork-mashable foods and the patient spoon-feeding.

The washing of clothes and body and clipping torn fingernails and the constant bloody bed-making. I do all these things too, but with help. He did all that on his own, day in, day out, while still somehow going to work. There was no slack, no respite.

It seems unbelievable now that Nick and I didn’t force him to get away, go away for the weekend while we held the fort, but none of us knew how to break up the terrible dance he and Ma were locked in to together, like one of those Depression era marathons – dependent on each other and almost dead on their feet but unable to let go. We did try but Ma’s panic at the thought of losing sight of him was so pitiful and he just couldn’t bring himself to do it.

So he was on his own. While I have a partner who can take over some of the legwork on those evenings when I’m just too shattered to move, he rarely had any help. No sympathetic ear to sound off to last thing at night, no hope of a gear change, no-one to give him a hug and tell him it would all be fine. No-one really who could possibly understand, not even his own family. I can’t begin to imagine how lonely that must have been.

Then again, he had worked like a Trojan all his life and had become a senior partner at his practice with a good salary and pension ahead. He and Ma could have a comfortable life and didn’t need to worry about money too much, while I wake up fretting at 5am because Nick’s DLA has not come through and the bills are mounting up.

Dad didn’t have to wring his hands over all the daily peccadilloes of council-outsourced care workers not really invested in the role, or navigate the stormy seas of the benefits system. He didn’t have to spend hours on hold on the phone waiting to talk to someone about PIP, or try to make sense of financial chaos from a time when Ma had lived independently, or try to retrieve her lost password for paperless billing from a broadband company still taking money out of an account she’d forgotten to cancel. He didn’t have to juggle child maintenance payments and mortgage for the life she had had before, now there wasn’t enough money coming in to cover them. He didn’t have to try to co-ordinate two households (badly) like me, and he didn’t have to buy and then hide a week’s worth of wine and dole out an agreed ration each day to an alcoholic. And all that is lonely work too.

The “which tablets for which hour of the day” was perhaps not so complicated for Dad. Nick was already taking medications for other health issues so he positively rattles with all the different things he is taking, while for Mum it was just heavy-duty tranquilisers. The main treatment for Huntington’s Disease at the time seemed to be Valium and Diazepam, so by the late 1980s she was spending a lot of her time in bed.

Medical appointments? I think for Ma these were gentler times where the local GP would have known you for years and would come in with your next consignment of pills on the way home from morning surgery, and sit on the end of your bed for a chat.

She didn’t half thump around but she was a petite, 8 stone woman, she didn’t have anything like Nick’s weight and super-strength, and although she couldn’t walk very well or far, she never needed a wheelchair. They visited me in London only a few months before she died and she somehow managed to stagger up the rickety iron stairway of an East London warehouse to see my artists’ studio.

The solid, high-backed orthopaedic chair we bought her soon became stained and scuffed but lasted for years after her death; I had it re-covered and it made a great nursing and reading chair when my son was tiny. It lived happily in our bedroom for a long time, then, when Nick began to need some more comfortable seating, I took it up to him in the north east. It lasted six weeks.

I wish I could talk with Dad and tell him I understand, and how terribly sorry I am for not doing more to help at the time. We could help a bit, but he wouldn't ever let us step into his shoes; he knew that either Nick or I were likely to inherit the same condition and he wanted to protect us from seeing too far into the future. 

There’s no real comparison between our lives except both of us trying to live with the fall-out of this horrible illness, seeing someone we love gradually lose everything. And knowingly making the sacrifice to try to ease things for that person as much as we can, or could. Whatever it takes.

It used to mystify dad that people would say, “Oh you’re so good to Mary, looking after her like this”, as if there was any question of doing otherwise. What else was he going to do – put her in a care home and visit at weekends? Though admittedly it could have been an option.

I have the same thing with Nick. People tell me I’m wonderful for doing so much for my brother for having such an active role in his care, but honestly I don’t see any alternative. If you’re reading this, you probably know that social care is in crisis and frankly, there are days that I do consider residential care except that there simply isn’t any.

Like dad, I am not always doing it because I want to, and certainly not out of any sense of “duty”. Well, it depends on your definition of duty. Obligation, no - loyalty and commitment, yes.

He’s my brother, my blood, and whether or not he would do the same for me, this is how it is.

It’s not a question of being wonderful or good or brave, it’s just doing what you do for someone you love. Even when it doesn’t feel like love as we know it, when you feel that you could actually strangle the person, that loyalty and commitment carry you through to keep going. So don’t tell me that you wouldn’t do the same, because you just don’t know what you are going to do until it happens to you."

Excerpt, “Days of Wine and No Roses” 

Unleashing the genie

Sometimes I wonder why I'm writing this, and who for. If you are affected by HD yourself then this is surely all stuff you already know. And if not, what sort of ghoul are you for reading someone else's misery memoir?! No, no, I don't really mean that, honest, please do keep reading.   

But what good does it actually do to talk about HD? 

For one thing, it will I hope make people more sympathetic - and more accepting. Because although this still classifies as a "rare disease", you just never know. That malodorous man lurching towards you at the bus stop or in the supermarket queue might be slurring his words because he has HD. He might smell of booze and wee and something worse; he might look as if he's slept in his clothes and it’s easy to assume he’s an addict or a street drinker, wrinkle your nose and move well away. But his trouser buttons could be done up wrong because he has HD and can’t fasten them himself and forgets to do it anyway.

The woman making a scene in the cafe who knocks a plate of food onto the floor then scrapes back her chair with excruciating screech, her voice rising into hysteria – for all we know she might be agitated because of HD. Again, there are all sorts of things we can assume about her and the British reaction is usually to avoid eye contact and get well out of the way. 

HD is ugly, embarrassing, distasteful and frightening to watch as it gradually takes over the mind and body. 

It makes you behave in odd, sometimes unsocial ways. You will be sweaty, jittery, whack out with your arms and legs and sometimes hurt someone. You may have car accidents and go into furious rages with other drivers. You lose inhibition and often a sense of personal hygiene. You miss your mouth with the fork and send the sausage flying across the room when you try to cut it with a knife. You will lose the thread of what others are saying, won’t really care anyway, obsessing over the same issue again and again until people get fed up and even start to avoid you. Or you might suddenly become catatonic, staring at the TV for hours on end, drinking. It messes with your meds but it’s one thing you can still do and by golly it helps blur the edges a bit.

By the time HD typically develops in mid life, people have often established a career, have family, home, financial and social responsibilities so it affects not just the sufferer but a whole network. 

In some ways it’s similar to dementia, hideous and unknowable, except that we seem these days to have a lot more understanding of dementia.  
And of course with HD there is the hereditary factor. 

You've probably come across the notion of Magical Thinking. The idea that if you pretend something doesn’t exist it will go away. Well, this is one of the things that has kept HD hidden for generations.

One of my oldest friends from school married a man who went on to develop HD. His mum had it, and one of his cousins. So he must have known what it was and that they were all at risk, and yet it was simply never mentioned until much much later when he started getting ill. By then they had three children, who are now all young adults and very much at risk themselves. He died a few years ago while they were all still teenagers. 
My friend nursed him, mourned him, coped amazingly well. Yet even now, having been through that, having known each other all our adult lives and shared the same ghastly coincidence of being affected by something so horrible but so uncommon, she is very reluctant to talk about it with me.  

I’ve spoken to other people who know they are at risk but whose families refuse to discuss it, as if just bringing it out into the open will unleash the evil genie. 

It's all very well for me, you might say: I know I'm in the clear and don't have to tiptoe around the subject, always waiting for the axe to fall but shoving those thoughts to the back of my mind, not talking about it in case it tempts fate. 

But those of us in the clear are still affected to the core. We still know more about the illness than anyone really ought to or wants to, and who else can bring it to attention if not us? 
Do we really want to continue the generations of silence, stigma and shame for our loved ones? 
I think the only way to banish that genie is to show it some daylight.