This is Huntington’s.

 

How is it possible for a person who can’t walk more than a few steps or wash and dress himself, to generate so much chaos? 

I ask myself this for the 500^th time.  

I used to call it the Nick factor, the way that if anything could possibly go wrong with almost anything you care to name, it would do. 

Now I wonder if it is just the way things are for anyone with an impairment and their carers, and if, for all the various avenues of support from government and social services and healthcare, life is just not set up for us.
And with complex conditions like Huntington's, there are so many factors - not just the physical symptoms which we all know are horrible and many, but the mental and cognitive and social and financial and all the other knock-ons that simply don't fit so easily into a simple category of "illness".  

And also, with HD there is so little that’s predictable. And it all happens at once. 

And I am on the alert almost every minute of every day and yet never quite prepared.

As is often the way, I came back from a lovely weekend away to a whole deluge of new crises. 

I don't usually wash or dress Nick as it needs two people, but was helping him put on his pyjamas and saw a nasty looking pressure sore on his bottom that the carers have either missed or ignored. 
There’s no record of any concerns in their daily log, and there’s not even the standard issue body map diagram showing which areas to be aware of.

Why the hell has no-one noticed this? It looks like a stage 2 to me, where the skin is broken. This is serious.

His special Omega chair with the inbuilt pressurised seat has been knackered for months so that can’t have helped. Today it gave up the ghost. It just kind of collapsed from under him, he said, and the castor came off, leaving it capsized on the floor like a poor old dinosaur. 
Thank goodness he wasn't hurt. It's the only thing he can easily sit on for long though, and the spare armchair is creaking dangerously with every shudder and kick, only made worse by the fact that he is so uncomfortable there. 

We need to get hold of the physio so she can authorise the manufacturers to come out to do a repair as soon as possible; but when I ring, she's on holiday for the next two weeks. 

And there's a worrying message on Nick's phone from his bank about low funds, and looking at his online banking it transpires that there have been three lots of £98 debited from his account by the council. Whaaaa?!!!? Thanks to his housing benefit and various exemptions, it's supposed to be under a tenner.

I get on the phone and manage to talk to someone who is as confused as me but thinks it might be something to do with a default setting by their computers when Nick’s housing benefit was recently re-assessed. (i.e we got four identical letters saying that as Nick’s circumstances had changed and he had not informed them, they were suspending his housing benefit. I know the ropes by now and apart from a knee-jerk email that I knew no-one would ever reply to, just sucked it up and made the journey to the council offices with a big sheaf of evidence to show that Nick’s circumstances had not changed and here was the proof. A week later it was reinstated and I gave a little cheer.

“But by the way” I had asked the advisors, “this won’t affect his rent will it?”

No, I was told, because he is in credit with his rent payments and the Direct Debit is ticking along as usual. Phew. All good then. 

But apparently not, as some kind of computer / human blip has alerted a default payment and Nick’s weekly direct debit has rocketed up to nearly a hundred quid, with no notification whatsoever.  And no, they can’t refund it at their end. They will send me a form to fill in which will take up to four weeks to process – never mind that he is quite spectacularly in the red right now and all his bill payments are about to bounce.

How can this be happening? I call the social worker for advice. She sends me a link for a crisis payment, because I can’t keep funding Nick for everything, I’m struggling these days to pay my own bills (Carers’ Allowance = 3p an hour according to one of my online friends) and surely the council need to take some responsibility here. An unannounced rent rise of 10 times the agreed rate? 
A bit of researching reveals that the housing benefit department and the rent department are not even both part of the local authority. One of them is a privately contracted company and communications are generated by numerical calculations rather than people and words. 
Holy Moly. It’s not quite Gilead, but we’re definitely in Terry Gilliam Brazil territory. 

Oh, and even though he was supposed to have enough to last the week, Nick has run out of wine, and his left hearing aid isn't working.

There's more, but these are the things I need to deal with most urgently and after two fairly full-on days, they're all sorted. The District Nurse has been to dress and check the sore and supply a blow-up pressure cushion which eases the discomfort of the creaky armchair and the Red Cross emergency repairs team have come to the rescue and reassembled the broken Omega. 
And after four more phone calls I found a mole at the council who told me to call Nick's bank and request an immediate refund under the Direct Debit indemnity clause. And indeed, as the debit agreement was for a stated weekly amount and this wasn't it and there had been no authority to change it, they didn't bat an eyelid and put the money back into his account straight away. 
I've checked the hearing aid and he had somehow, heroically, put in a new battery himself but not had the dexterity to remove the little orange sticker on the back so it wasn't activated. Simple thing to fix.

And I have done an online shop and got more wine. 

So, phew. 

Nick is happy again and much more comfortable; he's got his radio and his chair back and a dressing pad on his bottom. He's over the moon. The nurse will come in again tomorrow and he has money in his account again and he doesn't seem to be struggling to eat quite as badly as he was last week, and just for the rest of today I feel I can breathe a bit easier.

But this is Huntington's. It's not only the awful jerking and spasming and losing the ability to swallow. It's not only the memory loss and the mood changes and the accidents and the super-strength. The addictions and the obsessions and the reckless spending and the dental problems and the over-heating. There are so many threads that all seem to wind and unravel together that it takes your breath away. If you're not careful it can take over your own life, too. 

I sometimes feel a bit guilty for taking time off and just getting out of town, immersing myself in other things – sea air and green spaces and old friends (most of whom have had their own life upsets), and conversations about music and art and love. There I am on the move again when other carers are stuck 24/7 with no respite and Nick can’t leave his flat or lift a spoon to his lips. But these little breaks are like vitamin shots for the soul, powering me up for the return to another onslaught of what the HELL just happened and oh God I didn’t see that coming. This is Huntington's - relentless, unforseen and unpredictable. 

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”

Big Al and his crazy go-go rhythm orchestra

We all know that the internet has its dark underbelly but today this has really given me the creeps. Big Brother is definitely watching us, even though you might think he has better things to do.

See if you can explain this, pop kids: I do Little Brother’s online shopping once a week, in his flat from his laptop, at his address with his email account and bank account and internet provider. He is a creature of habit and likes the supermarket beginning with T…

I prefer to see what I’m buying, tend to shop locally where possible, and use a different supermarket for a big shop. I have never ordered groceries online or had anything more than a local veg box delivered to our home address.

Simon shops our local T… every few days but he tends to pay cash and hates being a number not a free man so he doesn’t use a loyalty card.

However – when I went to do Nick’s online shop this afternoon, the “usual purchases” suggested alongside his habitual order were exactly what Simon had last bought at our local shop. Beer, rizlas, pizza and green apples for the lad, soya yoghurt for me, and a different newspaper than the one Nick reads. How on earth were they appearing on Nick's laptop? 

Were we sure we didn’t want to order these favourites again today, asked the prompt as I tried to check out.

I don't understand how this is possible.

Simon, by the way, had not seen Nick since buying those things, but the only thing I can think of is that it’s our phones. There is no directly shared information but when we’re in Nick’s flat with our phones, the phones must all be having little chats with each other and exchanging our secrets. To the point that Simon and his phone would not even need to be anywhere near Nick but information would somehow transmit to Nick’s laptop. No, surely not, it doesn’t make sense.

I asked our millennial son and he just shrugged – he’s just so used to Big Brother hovering, he doesn’t even question it. It’s really given me the heebie jeebies, though.

When I think of algorhythms, I like to imagine a Cab Calloway style jazz swing artist, Big Al and his go-go rhythm orchestra, all wide grins and trumpets and baggy suits on a podium. The reality is nothing like as cuddly. 

But tell me this – how is it that the internet knows all these things about our consumer habits but sharing actual useful information so that carers acting on behalf of a loved one don’t have to start from scratch and go through extensive security checks every single time they call, say, the local authority?

It would be so useful if (once a basic security clearance had been given, of course) we didn’t continually have to jump through all these hoops; the GP has me down as Nick’s primary carer and first point of contact for instance but although the district nurses have had the same information, it keeps falling off their system so I have sometimes found notes from them “To whom it may concern” with a prescription to pick up meds that the GP knows nothing about, from a pharmacy that we don’t use for any of his other medications. The different departments of the local authority, likewise. 

Of course you need some basic security checks so that people aren't taken advantage of, but when you have to do it again and again and again it becomes very wearing. 

If only they could take a leaf from Tesco’s book!

Would that give me the heebie jeebies too, though, would it be too much?

Forget it, cause it’s not going to happen any time soon. The very thought is enough to short-circuit every appliance in a five mile radius.

How Come You Don’t Call Me Anymore

The thing I find hardest to watch happening to my brother is not the awful chorea - and Nick’s is really severe - not the coughing or the choking for breath or even the falling over. It’s the passivity. He still has strong views about some things, like wanting to watch the rugby on TV, or asking me to buy a particular kind of chocolate that he likes, or being very determined to send his children some birthday money. But mainly he seems content to have life happen to him.

Enviable in a way, maybe, for those of us who struggle constantly with shoulds and oughts and want to’s and what ifs – you could say that Nick has transcended all this and found his Buddha Nature.

It is infuriating beyond telling, though, that communication has ground to a halt. He keeps his mobile with him at all times and one of his tics is that he needs to have his phone and a little black cube clock always at his fingertips so he can reach out and touch them. He probably does this twenty times in the course of an hour – he just doesn’t actually look at the screen.

We have spent hours changing the ring tones, getting the buzzer as loud as possible, reminding him to check his phone every hour (and he knows what time it is because of the massive station clock on the wall and the radio programmes he listens to all day) but it’s no use. He has the phone near him as a comforting thing, but not actually a thing with a use.

“Nick, I’ve sent you three texts today. Didn’t you see them?”

“Not yet, no”

“Have you checked your phone at all today?

“I’m sorry. I forgot”

He has a specially adapted landline with a flashing light and an extra loud ring but he either doesn’t hear it or says he can’t get to it in time. If we put it too close by, he just knocks it over, so it has to be put out of immediate reach as he needs it to stay connected to the citywide alarm service.

This time last year he was still picking up the phone to call me, sending me texts or replying to mine, and generally in full communication although he was increasingly finding it hard to press the right buttons on the keypad. Texting must be really hard for him and I keep searching for a solution but the real problem is that he just seems to accept a world where he sits on his own all day and no-one gets in touch.

It is immensely frustrating on a practical level because he is effectively a prisoner. He can’t go out on his own anymore. If the carers don’t turn up for some reason (which they didn’t the other day and thankfully I popped in unexpectedly) then he just accepts it.

On busy days when I might not have time to visit, I just want to check in and say hello and see how he is, but it is one way. He doesn’t reply.

The trouble is, I’m not just fretting for no reason: the danger is real. He has accidents, drops things, smashes things and hurts himself. He’s not really safe to be left alone for long periods. How can I know he’s OK? The only time he gets in touch now is when he thinks he’s running out of wine.

At his request, I stopped hiding the week’s worth of wine and put it all in one place so he knows it is there and does not wake up panicking – but this means he has no reason to keep in contact. 

It’s as if he doesn’t care one way or another and I find it so upsetting. I know he does care and is delighted to have some company but it’s the apathy and closing-in of the illness that is horrible to be around and for all the changes we’ve been through, this is the hardest to bear.

As someone who’s known him all his life, watching him change like this feels painful all the way. I can’t get used to it and I don’t want to. But it’s the way it is and I must.

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!

Second that Emotion

There can be a level of autism in people with Huntington’s as the brain’s more complex functions begin to erode. I see it with Nick in conversation as he vaguely recognizes that some response is required and he tried to offer an appropriate comment. 

For a long time when he was living up north and appeared fairly well in many respects (and to the point that I often forgot about the illness), he would infuriate me on the phone; we’d be talking as normal and I’d tell him something funny or sad but it was as if he couldn’t always tell which was which.

“HOW Funny” he’d reply, or “HOW interesting” – sometimes interchangeably, as if at random. I felt maddened by his pat response. We had been friends and confidantes for so long and this reaction seemed so insensitive, like he wasn’t really paying attention. Now I realize it was the encroaching effects of the disease, slowly stealing his emotional capabilities and empathy.

One time I had to tell him that someone we had both known since primary school had died suddenly, leaving two young children, and I was feeling very shocked and upset. I thought Nick would understand but he listened and then said,

“HOW funny. Guess where I went for lunch today?” 

These days I don’t look to him for an empathetic response – if it suddenly comes along, that’s great, but I don’t expect it. His world is shrinking around him and the thing that seems to dominate emotionally is where and when he is going to get his next drink.  

It’s not that he’s a lost cause; he can still be thoughtful and sensitive and make sweet gestures; he was determined to buy people Christmas presents, for instance (with a bit of help actually getting to the shops and paying for things) and he clearly loves us, his children and his cats.

But it’s hard to tell what he is actually feeling, and perhaps that is a mercy too – that as the illness gets worse and even eating, swallowing, sitting in a chair become Herculean tasks, the brain shuts down its emotional responses to concentrate on the important job of just surviving.