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."

Last Christmas, I gave you my heart

...and I won't ever get it back, but that's OK. We were brother and sister for five decades, nothing can change that, and your memory will always be with me.

Memory is a funny thing, though – yesterday I was inconsolable because I’d just idly wondered what we were doing this time last year, then realised that I’d got rid of my 2018 appointment diary and also yours. The reminder of all those medical and social service appointments; ears, eyes, skin, teeth, hands and feet and bumpsadaisy, there wasn’t a bit of you that didn’t have a problem and someone trying their best to fix it; the waiting in for wheelchair repair and every other repair under the sun; meetings with management of the awful care agency, always promising but never delivering; the council officer and police calls about the upstairs neighbour; the shopping and the schlepping and the fun things too, your lunches with Helen and Sophie and me and the cinema and your boys’ night out with Simon at the pub. 

I couldn’t bear the reminders of all the bad stuff; we’d already lived it for too long. But I’m sad to forget some of those little things, though maybe they never really leave but are always there in the close weave of our souls.

We went to two carol concerts, that I remember, about this time last year. Both of which we had to jump ship halfway through because you were so uncomfortable. Being in a confined, unfamiliar space was very hard for you to manage, even in the wheelchair that you said was your most comfortable place anywhere. We’d both stopped caring if people stared as you jerked and rocked and shuffled about, but now the Christmas spirit had arrived and people were kind. You loved the craic, the decorations and festive air, and you certainly enjoyed the mulled wine and snacks.

All this stays with me in my bank of treasured memories. We never imagined that you wouldn’t be doing the same thing with us this year, and I’m still getting used to you not being here. I wouldn't want to put the clock back and see you getting worse and more uncomfortable all the time, maybe not even wanting to or able to go out much. We have a lifetime of memories and good times to look back on, and you definitely quit while you were ahead. It's just a funny time for the ones you left behind. 

But don't worry. It’s Ok – just strange. 

Baring my soul in public

I’m a very private person yet happily write all sorts of personal things here in my blog. When I was asked to write a diary about caring for my brother, it didn’t seem to be any different and actually I was really delighted to think of reaching many, (many!) more people. Nevertheless, now this piece has been published in the online “slow news” journal Tortoise with 7,000 + subscribers*, I feel quite strange about it all.

Nick and I both wanted to do it because we both think the world needs to be more inclusive and more forgiving. Huntington’s is a bastard, no two ways about it, but we want it to be a condition people know about and can recognise, not something hidden and shameful.

So many families live under that shadow; I can understand why you might not want people to know that you have an incurable hereditary illness in the family but in the end, if it’s in your gene pool it will get you and the silence helps no-one.

And carers – we’re so invisible to so much of the world that even a popular "Discounts for Carers"  scheme only recognises paid care workers, not unpaid carers. How nuts is that?

So I wrote this piece for all carers and for anyone living with a life limiting condition that takes them out of plain sight of the world.

It’s really not intended to be grim reading – there’s frustration and anger and heartbreak there, but humour too I hope, powered by the belief in a better society that looks after its weak and helpless, and strength in solidarity, and always, always love. 

*It's a fantastic platform for informed long and short reads - the news behind the news. Proud to be associated with such intelligent and interesting journalism. 

Choice

Choice – it’s one of the biggest words you’ll hear bandied about when you’re caring for someone.

The big rule of person-centred care is that you respect the rights of each individual and as far as you can, support them to live as they would wish. As long as they have mental capacity, you must always give them a choice about how they want to do things, what they want to wear and what they’d like to eat.

I was thinking the other day how nice Nick looks lately, almost back to his old self if you didn’t look too closely. We bought him a lot of new clothes for Christmas and his birthday, none of them chosen by him but all by us thinking what he might like, and he looks fantastic. 

He’d always taken a lot of pride in his dress but in the last year of living on his own he’d been buying things that made him look ten years older. Shapeless jackets and baggy old-feller's trousers and slip on shoes.

By the time he came to Sheffield and was still more or less dressing himself in the mornings, he had really started looking like a funny old bloke, wearing an odd assortment of garments that didn’t go together at all. That was his choice, though. Now we’ve overridden it by buying his clothes ourselves, even though it’s him who decides in the morning which of them he wants to wear. Though the carers override his choice too when he wants the same socks four days running, because HD dramatically affects your sense of personal hygiene.  

Then there’s the wine, the chocolate and now the CBD. Nick became obsessed with the latter and wants it all the time. I’ve had to keep explaining that he can’t just binge on it as there was only a limited amount, when it’s gone it’s gone, and six lots at once really won’t make him feel better than one.

I’m rationing everything, including Mars Bars (which he’s not really meant to have in the first place because they’re a dietician’s nightmare. But he longs for them so), because otherwise he doesn’t know when to stop and will neck it all at once. I’ve tried giving him the benefit of the doubt, but rationing is the result of bitter experience and seems to be the only way to go. I might not exactly know best, but in his best interests I've got a pretty good idea. 

So if the person has capacity but limited understanding of the consequences and wants to do something that is going to be bad for them, then giving them a choice is tricky.

It still doesn’t sit quite right with me, but I’ve become the publican who’s seen it all, saying, “ That's all for tonight. You’ve had enough, sir”

Drugs?

“You’ve been in the wars!”

An acquaintance in the changing room points to my bruises. They’re yellowing now but still very visible against my wet skin as we step out of the showers.

I just laugh it off – you should have seen the other guy! – but the truth is, this is normal. Pushing Nick’s tank of a wheelchair, getting it in and out of the car, or even just folding it up to live in the hall of his small flat, is very hard to do without some injury to self. 

Helping him get his shirt off, or put his shoes on, or move his chair closer to the table all have their risks; even when I’m anticipating it and try to duck, the force of his involuntary whacks can knock me sideways. Or the unexpected head butt as he suddenly lurches backwards, or the shin splintering kick as I stand by him in the cramped crowded lift at the hospital, me as the buffer taking one for the team instead of some poor stranger.

It’s hard to explain this to someone who doesn’t know Nick, or Huntington’s. And not everyone with Huntington’s has quite this degree of violent movement. But it is one of those things you get used to, and that probably seem quite weird to anyone outside this caring lark. Other carers have bad backs from constant lifting, washing, changing of sheets, I know. Not to mention all the invisible and psychological stresses of the role – things even the brightest changing room light would not reveal.

But as I look down at my arms and legs, I realise that the bruises are old, they’re not fresh. I think it might be some time since Nick has really lashed out, and we haven’t been out much because he’s been so ga-ga since taking these new tablets and wants to sleep a lot more than usual.

So they’re working. Well, we knew that, but perhaps they’re working in a good way. They’re making him very sleepy, even with the half dose, but if they are calming his movements then that’s surely a good thing. And to my amazement, when I came to do the online shop this week I realised that there was still a cupboard full of wine and no need to order any more, so he is drinking a great deal less.

I’m still slightly uneasy though. Nick is now taking quite a potent cocktail of medication.

The drugs used to treat chorea are typically anti-psychotics. Sometimes at high doses these can mimic just the effects you want to avoid – severe agitation, risk of falling, choking - and as HD progresses it’s not always easy to tell what is the side effect and what is the actual illness. I can’t tell exactly what effect the new tablet and the newly reduced dosage are having, all I know is that things are subtly different and his sleeping and activity pattern has changed. Maybe all we can hope for is damage limitation? It’s hard to know. *

This afternoon we are going to see the specialist again for an emergency review, and we’ll see what he says. 

Meanwhile I was wondering what to title this post and all I could think of was the Charlie Sheen character at the police station in "Ferris Bueller's Day Off". 

"Drugs?"  

And isn't that as good a way to start the day as any?  

*Thanks to the wonderful online HD community for this link about medications:

What's broken cannot be unbroken, but I like to think a heart can mend

Today I was walking past a local cafe feeling happy and light of heart. I saw someone I knew sitting by the window; we nodded to each other and smiled, and automatically I smiled too at the person he was with, a proper, open-hearted glad to be alive and good to know you smile, and she did the same - except that it was someone who'd fallen out with me some time ago and we have not spoken to each other since. We both smiled at each other before we realised who it was. In that instant, though, it felt like things were healed. We might not ever be friends, but something just mended in a glance, like some invisible clicking back into place.

Nick's fifth remote control has broken and we are waiting for a replacement to be sent out. It's been a week now. The TV is now out of warranty but we're getting the remote free as a goodwill gesture so I can't make too much of a fuss, but until it arrives he can only watch one channel on his television with an annoying error signal flashing on and off which would do my head in after two seconds but he seems to be able to ignore. I curse the day we bought the bloody smart TV, it's been nothing but trouble, but it's also a lifeline for him.
I did buy a universal remote when we first realised that the TV remote was so vulnerable to being chucked around and bashed, but we've never been able to match it to the television. The OT has been trying to install a bionic arm that attaches to Nick's table to grip the remote more securely, and she brought along her technical team to help, and they couldn't get it to match so I know it's not just me - it's a combination of annoying so-called smart TV and the Nick factor.

Meanwhile, the big comfortable black chair that Nick sits in all day has broken. I got a text from him yesterday to say that a wheel had fallen off - and it surely has. The chair is capsized in the corner like some poor broken creature. The company who fitted it say they can't do any repairs until they get a referral from the NHS, as although they supplied the chair, it was the NHS physio who made the purchase and therefore it's up to her to instigate a repair order. What, even in dire emergency? But they're adamant that they need the physio's sign off but she won't be at work again until the end of next week and emergency or no, there seems to be no Plan B.
Thankfully we still have the old orthopaedic chair I got for peanuts on the Abbeydale Road, and Nick can sit in it reasonably comfortably, but not for too long before his body starts rebelling. He is playing musical chairs with that and the armchair supplied by the council, which has always been too low for him to easily get out of.
Two of his absolute basics have disintegrated, and I feel so helpless for him. It's like some kind of horrible party game where all his favourite things are taken away, one by one. Still, we keep cheerful. He's really looking forward to Christmas and I am planning some treats for him. Tonight we're going to hear a friend's choir, and we'll have some Christmas lunches. If you're reading this and you're within shouting distance, get in touch, we'll be here and we're up for some festive jollies.

But thinking back to seeing the woman I had fallen out with; even though Nick's special things are broken and it's frustrating and sad for him, he's in good spirits. The breakages are constant; it's one of the features of Huntington's that you might not hear so much about but for us it's non stop, and it's hard to anticipate what's going to go next, or the difficulty level of getting it replaced or repaired.
If you or I broke our glasses or the car wouldn't start, it would be annoying and inconvenient but we'd manage; for Nick, he has so few resources that if his TV and hearing aids and radio and chair stop working and we're not there, then he's effectively in solitary confinement.  It's amazing then that he is so phlegmatic about it all.
In his pyramid of human needs, physical comfort and company from the TV are so important, but the really essential things are his human relationships and the cuddles he gets with his cat. These keep him going. And having such a short term memory that he can't remember enough to bear a grudge, so he stays open hearted like a child. I think as the silly season races up ahead, that it's probably a really good way to be.

Food for Thought

I have been pressing for ages for Nick’s care provider to have Huntington’s awareness training for their staff. They can have free specialist training, the council have said they’ll foot the bill for the staff time, it’s professional development, surely a total no brainer?

It’s been recommended all along by the speech and swallowing specialist and the NHS neuro team, and they said they’d willingly do a free session for the carers any time they like. Diana, our regional advisor from HDA, has also offered to do a session for a minimal cost, but the care manager has been dragging his feet.

Meanwhile, I’ve continued to wring my hands over their constant misdemeanours: kitchen surfaces awash with spilled wine and the bathroom floor unspeakable after the carers have just left; microwaved meals heated up on “Defrost”; food I’d put out for Nick and labeled going ignored: forgetting / not bothering to give Nick a pudding or gravy as requested, to increase his calorie intake and make it easier to swallow; presenting him with an entire chocolate cheesecake for dinner; feeding him while standing over him looking bored, spoon in one hand and checking their phone with the other.

Good nutrition is one thing but basic common sense is surely the most crucial thing of all. I’ve been a carer myself and you do need to be on the alert all the time, checking your client's signs of well-being from minute to minute like a cut rate Sherlock Holmes, with eyes in the back of your head for an ongoing risk assessment. But they simply don’t seem to notice the glaringly obvious.

Anyway, to my amazement they finally had the training this week and all the carers who Nick currently sees were there.

Afterwards I got a memo telling me that I am not buying the right kind of food for Nick as he shouldn’t be eating pasta at all (his favourite) but needs soft, fork-mashable foods that are easy to swallow. And a high calorie diet with lots of added butter and cheese, so please make sure that they are available. 

Oh, right! I was quite indignant that they should have two hours of HD awareness and suddenly they’re the experts. But at the same time I was really pleased that at last perhaps they get it, and that they want to be involved more directly in Nick’s care. We’ll see how things go from now on, but I do have some hopes that these niggles will start to improve. I’d like to think we can work together rather than me always having to tell them what to do, and them feeling wrong footed.

It also reminded me that I don’t actually know it all, and in my quest to protect Nick and look after his health and nutrition, there will be things that I’ve missed too. Sometimes it takes a fresh eye, or someone fresh from training, to see these things and point them out. They’re right about the pasta – I’ve been noticing for a while that it is getting much harder for him to eat it, and it’s me who’s been resisting giving him a diet of mashed potato and pureed food because I can’t bear its implications. There’s no looking back from the puree, is there, not unless you’re a baby.

With this in mind, I had to laugh today. Nick went for lunch with one of the friends who occasionally takes him out, and they went to a pub. She is sensible and knows all about the importance of nutrition and what not to feed him in case of choking. She has seen his struggles to eat and doesn't flinch at having to help him. 

“What did you have to eat?” I asked him later, when he was telling me what a good time he’d had.

“I had ham, egg and chips” he said.

“Goodness, darling!” I said. “Did you manage that Ok?”

My friend later confirmed that he had eaten the whole lot, and all on his own, every bite. It took him half an hour but it's just what he wanted. Where there’s a will, there’s a way, I guess, and it looks as if it’s not all just mashed potato quite yet.

Firefighting

The mornings are fresh and light now and if the weather is not exactly warm, trees are budding and flowers blossoming. Nature’s sap is rising and it’s heartening to see Spring on its way but I’m feeling so tired.

My heart is yearning for travel and new places. The thought of a holiday brings tears to my eyes. I want to organise some kind of break – for me and my boys but also for Nick, just to breathe some new air, but we are so busy fire-fighting to stay in one place that it is hard to plan ahead.

I need to renew Nick’s Blue Badge this week and drive thirty miles to pick up a temporary loaned wheelchair from the Red Cross. He urgently needs a new one as the wheelchair I got him when he first arrived in Sheffield is literally falling apart. He used to go out on a Saturday with a carer from my old agency but the wheelchair is so heavy and unwieldy, with a footplate that can suddenly swing out of place, and Nick is so prone to tipping backwards, that she slipped a disc using it and was off work for a month. 

We discovered that it was not strictly legal for a carer to use equipment that hadn’t been serviced, so even if she felt able to return, she is not insured and simply couldn’t do it any more.

We could just buy another one but we’ve been advised not to do this by the neuro service as Nick’s needs are so particular that they say he needs to be assessed by a multi-agency team. He’s on the waiting list for an assessment but that’s a long, long list. So for now we’re struggling on with the old warhorse, it hasn’t fallen apart just yet and it is a lifeline for him to be able to go out.

Meanwhile, I'm pleading for another service review with Social Services and the care company because a new support plan was drawn up at the end of February to reflect Nick’s changing condition and his increased difficulty with eating.

Carers are supposed to come at a **Time Critical** 8pm every night to administer meds, get him changed into night wear and prepare a hot meal, then stay with Nick while he eats it. 

I see him every day and Simon or I will go in every other evening and often do the dinner ourselves so we can all have a meal together, but we can't be there every single night so we try to work around the carers. 

They’re not coming at 8pm though: since the time critical plan was issued, they have been coming without exception between 6.30 and 7pm. We’ve almost made a game of it, popping in at my old time of “just after the Archers” and more often than not the carers will have been and gone and Nick will already be in his pyjamas. 

It’s too early for him to eat, so of course they are not staying with him – and even if they do stay in the flat while he eats, we have usually found them in the kitchen next door looking at their phones.

When I challenged one of them about this, she apologised for being late! 

It is still very hard to communicate with most of the carers and I have still not figured out how much they do understand. One of them comes from the Ivory Coast so I've been attempting to speak with him in French but my French is probably as iffy as his English so it's kind of desperate measures. One day, I think, I will write a sitcom about all this and it will be ten times darker than anything Jo Brand has yet to come up with. 

She also said that they hadn't even known anything about staying with Nick while he ate, and this was news to her.

We had a review last week for the social worker, two members of the neuro team and the care company manager to see how the new regime was going. I was so relieved to think we could discuss Nick's care needs and iron out these problems together, ensuring a smoothly running joined up service that kept him looked after and safe. Just one little fly in the ointment - the care manager didn’t turn up. Even though the meeting had been booked in since February. 

Now we have to arrange another one, trying to find a time that everyone can do, and apart from the pain in the arseness of that, it is extremely stressful for Nick.

And me. As a battle-weary sister exhausted from constant chasing and complaining for every little thing, it was a wonderful thing to hear the social worker tear strips off the care company for the manager’s rudeness in not turning up to the meeting or even letting anyone know he wasn’t coming. She was really furious at him for wasting everyone else's time, and rightly so. 

Welcome to my world, I thought. But also - thank you so much for taking this on. I am so unused to someone else doing that, and it strikes me that this is what family carers do - we take on so much of the fighting to be heard or taken seriously as a service user, so much of the legwork, the chasing up and all the in-between stuff that somehow doesn't happen otherwise. 

She and the neuro team physio both grimaced when I said, Don't Social Services and the NHS teams automatically communicate to each other about service users? Especially the ones with complex needs? 

I had naively thought that they would. But both their services are totally overstretched and their case loads ridiculous, so apparently not. We are lucky to have this support but it is in no way joined up, and the pulling it all together and being in touch with everybody is, it seems, my job. 

The overall co-ordination and the fire-fighting is down to the person's family, and they all say Nick is lucky to have me. 

No wonder I'm tired. You have to be fit to do this job. Pass me my imaginary length of rope and my fire-fighter's helmet, I'm going in. 

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.  

An Unexpected Kindness

My heart sank when the upstairs neighbour came round to complain about the noise. It’s happening all over again, I thought.

I invited him in to meet Nick. At least that way, he could see for himself that Nick is not well, and Nick could understand the impact that his loud radio has on other people. The neighbour turned out to have tinnitus like me and gets very affected by external noise. He didn’t want to make a fuss, he said, but it would really help if the volume wasn’t turned up so high that vibrations were buzzing through the ceiling.

I stuck one of my dayglo post-it notes to the wall reminding him to keep it down, and “I will” said Nick, which is his constant saying now – totally meaningless, he says it about the wine (“Please pace yourself, bro”), the carers (“Nick, you must tell them what you want”), the radio, the reminders to check his phone for texts, you name it.

“I will,” he says, like some bridal ceremony on a loop, but even if he thinks he will I know perfectly well that most of the time he won’t. (“I forgot” is the other most popular saying.)

So I have been fretting a bit about the radio as well as all the other stuff I’m worrying about, since both Nick’s physical condition and his mental capacity seem to be deteriorating almost in front of my eyes. The carers are a still a huge worry; some of them have been really keen, reading the daily notes and making sure Nick has his non-slip mats and good grip cutlery to help him eat his beautifully chopped up meals. They have made his bed every time and someone has even been making a stab at the recycling!

Others are just not listening, or not looking. Why did they give him dry toast again this morning when the clear instructions were for two of those sticky malt loaf things that he likes, that the dietician recommended and that were in full view on the kitchen counter? Why was Nick wearing socks with his toes sticking out, bits of last night’s pasta still curling up on the seat of his chair, and a shit smear on his bathroom basin that had been there for three days (I deliberately didn’t do anything about it as I knew someone was coming in to clean today, and I wanted to see if they would notice. Sure enough, they said in their notes that they had thoroughly cleaned bathroom and kitchen, but the shit smear was still there. I felt like putting one of my post-it notes beside it with a big arrow, but in the end I didn’t.

It’s not good enough. It makes it hard to relax. So I feel anxious all the time with that awful infectious dread that has no exact source but just grabs you in the chest and sits there. I know that I can’t sustain this and do the real work of caring for Nick but it’s a real effort to lighten up and switch off, or move into another gear.

But things do give me hope. In the space of a few days, which is often the way, we have had the extremes of understanding and kindness.

One, the business exemption checking service, who wrote a stern letter billing Nick for willful mis-use of a form at the dentist’s. Sorry??

Long story. But to cut it short, Nick’s dear friend Dave from school had been alarmed by the tale of the wobbly teeth. He arranged for Nick to have an appointment with another old school-friend who is now a dentist.

This happened while I was away for a couple of days, so Simon took Nick. He said it was very moving to see the two old friends meeting again after all these years. Nick needed a small filling and had a scale and polish, with a reminder to brush more regularly and stay off the Snickers bars last thing at night. We didn’t think any more of it until this letter arrived.

Apparently Nick had signed the exemption form saying that he received ESA (and therefore did not pay for his treatment) when actually since coming to Sheffield he only gets contribution based ESA as his finances were so tangled that the income related part of it has never been sorted out. Another thing I’m still chasing. So anyway, he should have paid the fee upfront and shouldn't have signed the form. 

Simon didn’t realise – he just assumed that Nick didn’t pay for his treatment – and apart from a few flashes of lucidity, Nick will sign anything put in front of him these days. So that was that, but now, for his perfidious attempt to dodge the system, Nick is being charged a sizeable penalty on top of the actual fee.

I wrote at once to explain that Nick has a serious impairment that affects his ability to process information and of course this was a mistake, our sincere apologies, we will pay the bearer forthwith. But that wasn’t good enough. They replied that we would need to send a letter from a medical professional confirming that the illness in question would have that effect, and one form Nick giving his permission for me to discuss the case on his behalf. As well as prompt payment of the penalty along with weekly interest that was now accruing. If they decided that there was justification enough to waive the penalty then they would perhaps refund it.

Give me strength!

I have sent the proof they asked for and paid the original fee – stuff their penalty! –

and fumed to myself about the extra time it takes up to contact the GP and ask her to write the letter (an email won’t do), and also compose and print out a letter for Nick to sign. Not for the first time, I wish people- organisations, I mean – had a clue about the enormity of work involved for the average carer to just keep the wheels on the road. They genuinely seem to think that we have nothing better to do than gather six different forms of evidence and proof of I.D in hard copy, then send them all first class with recorded delivery.

If only there were some shortcuts for all this admin so that I don’t have to waste another afternoon jumping through other people’s hoops.

And then, just as I was feeling really low and that the milk of human kindness had definitely gone sour, I bumped into Nick’s upstairs neighbour at the paper shop.

“I’m so sorry” he said, “I did some research on your brother’s illness and it’s awful isn’t it? And he’s not going to get any better. Poor chap.”

He asked if there was anything he could do to help. You’ve just done it, I said. You’ve taken an interest – you’ve cared. That means so much.

It means so much that another person gets it, understands a bit about the situation. I realize that maybe there’s something I can do to ease my frustration with all these time-munching organisations. Who knows, it might make a difference and it will make me feel better.

So, every time I have to deal with the council, the benefits agencies, anything official like the dental exemption checking service, I am going to include a leaflet about Huntington’s in the envelope. I’ve started doing this with all my official correspondence. Just so you know, guys!