We were saying about my wife and saying how it’s very hard, and I’m finding it very hard to think that she’ll be on her own for years, 20, 30 years. But the other thing is that it’s very hard now. She gets absolutely exhausted. She’s my carer day and night. If I wake in the night, she’s the person who has to get up and get me a bottle so I can go to the lavatory and this sort of thing. If she wants to go to bed early, she feels guilty about sort of staying. Because I’ve possibly slept in bed during the morning. Because one of the things about being in a wheelchair is that you get uncomfortable if you’re in there eighteen hours a day. So if I have to get up fairly early in the morning, I tend to bed slightly earlier. If I know I’m going to be up late, I tend to go to bed slightly later. She’s always up, she’s always running around, she’s got to feed me, she’s got to wash me, dress me. She hardly has any time for herself.

I don’t know why, but the French seem to have got it off to a fine art in terms of wheelchair access. And they’re better, their wheelchair, their disabled parking spaces are really clearly marked as well, blue. Unlike in England, people don’t tend to be outside bank telling machines or fish and chip shops, so they don’t tend to be used by people who aren’t disabled and think, ‘Oh, I’m only going to be there for five minutes or three minutes. It won’t matter. And we drive past and we think, ‘There’s a disabled space’ but it’s full. And we come back, do the circuit, discover it’s still full to somebody else, somebody different.

In [town] there are three beside one of the banks and they’re always full of people, once even with a bank manager, who should have known better, who had gone back to pick up something. The French even have some rather disturbing notices. They say, ‘If you take my space, please take my disability’, which is a kind of crude way of making the point. The reason why we need disabled spaces usually because is that I need a longer space. Because I come out of the back of the car, down the cattle ramp, I need to have about 2′ to 3 metres behind the car for me to unload. Well, the ramp is about 5 feet and then I have to have the length of the wheelchair as well. And some of the disabled spaces are good because they’re longer. Some places are terrible. You find yourselves unloading into the main road into disabled spaces where you back out into the main traffic, which is frightening.

I was upset. I think my wife more so. That’s the difference between us I think. Also I have to say it did flash through my mind that it meant I could retire very soon and I was thinking, ‘Oh, that would be lovely. Perhaps I wasn’t as aware of the consequences, perhaps I wasn’t aware. And I don’t think the doctor then spelt out just how soon, how quick it could be. I don’t think – I’m trying to remember – I don’t think he spelt out, he certainly didn’t say the difference between going central core and legs. I think he said that the diagnosis wasn’t very promising, that it would deteriorate. I think he said something about, ‘People with motor neurone disease don’t live much longer than ten or twelve years. I’m not sure that he said that the average life expectancy was two and a bit. For people from diagnosis with central core probably fourteen months. Of course he wouldn’t have had to say that to my wife, because she probably already knew. She’s been much more aware of medical things than I have.

So we then went along to the motor neurone disease clinic in [city]. And the nurses and the doctor there were great. They answered all the questions we wanted. I think by then – it was a week later – and by then I was in a position to want to know. Perhaps the week before I didn’t necessarily want to know all the details. By then I was getting curious. We didn’t learn everything then, obviously I didn’t ask all the right questions. And perhaps correctly he didn’t tell me all the details of what would go wrong, largely because I suppose nobody knows. I think, as I said, I know probably twenty people with motor neurone disease, and I don’t think I know two people whove started the same way or deteriorated the same way. I know people whove gone legs first. I know people whove gone from the side first. I know people who first discovered they couldn’t swallow, people who first discovered they couldn’t talk, people who fell over for no reason, they just sort of seemed to have had a loss of balance. It’s such a weird disease to predict.

The only slight drawbacks – at home I have something called a Clos-o-Mat, which is like a Swiss lavatory which has a pressure button. It also has arm buttons, but I can’t use my arms. It has a foot pressure button, I can press on that. And it’s a lavatory that enables me to wash myself, so it gives me a certain amount of independence. And they didn’t have one of those at the hospice, which I thought was surprising. But they are a cancer hospice, not a motor neurone disease hospice, though they take people with motor neurone disease. And so perhaps it wasn’t as surprising. But these things are coming in now. The Japanese toilets are quite fashionable these days. They do all sorts of wonderful other things, like play music, and avoid the embarrassment of sound, but they do wash you as well. We first experienced those when my son lived in Japan. And you can actually find Clos-o-Mats now in some disabled toilets. I noticed that the [local music and arts centre] has wonderful disabled toilets. Some of these new buildings really do. Wide doors, which is good, room to turn the wheelchair round, so you don’t have to back out, which is the usual thing. They’re normally too small. But they also have Clos-o-Mats, and they also have hoists in some of them. So people who really needed to get on to a toilet could if they – I think you have to take your own sling. But no, some wonderful ones Anyway, the Clos-o-Mat is useful.

It’s difficult to know exactly what was wrong at first. I think the first symptom that I felt was that I would wake up in the morning and the muscles, mainly of my left arm, which was odd again, because that wasn’t where my first real symptoms occurred. But my muscles of my left arm would feel as though they were of cement, hardened. And it would take me a minute or two to start moving the arm. And it didn’t happen every day, it happened occasionally, and I sort of shrugged it off as old age or something. And then in the summer, looking back on it now, I didn’t know at the time, I was away on holiday, staying in Uppsala with my wife’s family. And I was completely relaxed, absolutely relaxed, and yet I felt as though I had sort of butterflies in my tummy all the time, which I presume was probably the fasciculation that you get, I don’t know what it is, the sort of muscular shivering that you get. And I again didn’t think any more of it. I just thought it was rather odd that I was feeling nervous, because I didn’t feel nervous.

The first time I went to the doctor with any related symptom was in the October of 2001, and I went because I was getting cramps. Again cramps as I knew were something I’d experienced in childhood, so it wasn’t unusual. But I seemed to be getting cramps in unusual muscles. Normally you get it in your toes or in your calf muscles when you’re tired. But I was getting it all over the place. Not regularly, not at any kind of point that I could diagnose, it wasn’t after exercise or anything like that. And of course the doctors laugh this off, because there are about a hundred causes of cramp, and they basically say, ‘Come back if you get anything worse.

Then in the November, a month later, I started finding it difficult to operate the remote control that opens the doors of the car. And that was quite a stiff little button to press. And I obviously had lost some strength between the fingers and the thumb in my right hand, because when I was trying to press this button it didn’t always work. After two or three weeks I sort of got slightly worried that I was losing strength in my hands. So I went to the doctor’s just on the off chance. And the doctor I suspect knew a lot more than he said at the time. And he said, oh, that he would send me to the [city hospital], because it might be a trapped nerve. I think he thought it wasn’t, looking back at his expression, and I know my wife thought it wasn’t either.

I was singing in a church last Friday and it had been designed obviously with a wheelchair ramp for the outside. But the inside had been designed before they thought of wheelchairs. And the ramp was a new concrete ramp. And I actually had to take the footrest off this to get through the door. The corridor was narrow, the door was wide enough, but I couldn’t swing the wheelchair because of the position of the door opening into the corridor. So I had to take the wheelchair footrests off and let my feet hang. That’s a drawback with these wheelchairs. If you make them tall enough for tall people, then the footrests, the wheelchair is so high it’s inconvenient. It can’t go into taxis, it can’t go into disabled vehicles. If you make it lower, and your feet are on the footrests like mine are, then the leg is lifted off the cushion. Even though I have a wedge-shaped cushion it’s still lifted off and you’re sort of perched on the back of your bottom which gets sore after a while. So what I tend to do, which is an anathema to occupational therapists, it sends them into fits and they think this is terrible, is to let my legs hang occasionally. Just lift off the footrests, not take them right off, but just lift them up and let my feet hang, which isn’t very comfortable after a while because you then find your knees hurt. My knees hurt because I haven’t got muscles to hold them. At least my legs are a bit more comfortable.

If you have the footrests lower of course, you wouldn’t be able to go over any kerbs, because the chair is fitted with a kerb-lifter, which I rather upset my occupational therapist by calling a kerb-crawler. Anyway this kerb-lifter thing works, but if the footrests are in the down position on steep curves, the footrests hit the kerb before the kerb-lifter does.

Again the church I was going to had been very carefully – they’d put a ramp in. Unfortunately nobody had thought that perhaps they needed a ramp from the road, where you unload, into the church, onto the pavement, so that you can get into the church. And I had to go about 200 yards down the road to find a place where there was a drive and a dropped kerb, which was a very steep dropped kerb, simply to get in the church.

Dropped kerbs are a problem. Where we live there’s a station, oh, 300 yards away, 200 yards away. And to get to it there are 1, 2, 3, 4, 5, 6, 7 dropped kerbs. One of them wasn’t dropped until I asked. Two of them were newly put in and horrendously steep. With my old wheelchair, when I tried to go up it, the front wheels used to come off the ground. And because the other two were on the hill, they were also very steep. So I rang the local authority, who haven’t – it’s very difficult to work out who do the kerbs, but it turns out to be the county council, which seems to be very illogical. Anyway the county council sent somebody round, who happened to be an ex-pupil of mine, and he and I hatched a plan and so they’ve done these seven kerbs much better now. It’s great. The county have been very good about that.

But there are care homes in [our town], my wife visits a lady who used to be in her choir at a care home in [our town], and as you come out of that there isn’t a dropped kerb. And the residents there used to object. So then they put in one, but there isn’t one on the other side of the road. So you have to cross up to a place where there’s a residence with a garage, and then you go to the next road and there isn’t a dropped kerb, so you go up the road to the first house with a garage and cross. It’s quite a problem. Some places are brilliant.

The only successful medicine that is proven in trials seems to be to take riluzole or Rilutek, one of those is the generic name, I can’t remember which, and vitamin E and vitamin C, which are as antioxidants. I remember being rather disappointed when I discovered that they seemed to extend the average life expectancy six months or so. What I don’t know, and nobody really has done the research to find out, but it seems to be intuitive, is that if you are deteriorating slowly then it extends it longer, and if you are deteriorating very fast, with central core, then you don’t get the full six month benefit, may be three. I don’t think the statistics have been done on that.

Are you taking that?

I take that. It has one slight downside, and that is that one of my liver functions, the function that deals with toxicity, is raised. It should be 30, 50, something like this. In mine it’s been 130, 180. There was a drug trial that we did for a drug from Japan, which proved to be negative, proved to be totally ineffective, which they worried about, they didn’t let me start it the month I was supposed to start it because of this liver function being raised. But I’ve lived with it for five years. It doesn’t seem to have any effect on me. It’s only one of the liver functions. I mean, they test for about five things each time I have blood tests, and it’s only this toxicity test.

I did go on to a minocycline test, well, I need to be tactful here, I did take minocycline, which is the result of one or two tests, which is currently being tested. And I was put on technically a drug trial. And I was taking it originally because I had a bit of a problem with my skin, which I think is probably related to the other drugs I take. I seem to have flaking skin, itchy skin, itchy forehead. Anyway the minocycline would have perhaps cured that. It may have raised that. I’ve certainly been off it now for five or six or eight weeks, because it seemed to raise this particular count in my liver. I’ve recently had another blood test to check if the count has gone down and it was the minocycline. If it hasn’t, it’s just a cumulative effect of the riluzole. I take nothing else.

Footnote’Mincocyline is an antibiotic normally used for severe acne. Previous research suggested it may delay onset and prolong survival in MND, but the most recent clinical trial results reported in May 2007 have been disappointing. Seewww.mndassociation.org/research/research_explained/

And over Christmas, which was just after Christmas, I had to go to see the specialist, over Christmas. I think my wife thought it was MND. How, I don’t know. I thought, well, I might have something, MS, Parkinson’s, some neurological condition. It didn’t seem like a trapped nerve. Anyway I went in the January. They examined me. They said, ‘You’ve got to come back in to hospital for full tests,’ which was in the March. And I went through the full gamut of tests.

Most of the tests are done to eliminate other possibilities. So you get a lumbar puncture, which I suppose eliminates the possibilities of other cerebral or spinal problems. But nobody with MND needs the lumbar puncture. It’s just to eliminate. I was lucky. I was the only person in my ward who didn’t have a stinking headache for 24 hours afterwards. They said, ‘Drink the water. They brought me sort of school jugs of water – I don’t know, a litre and half of water – and they said, ‘You’ve got to drink two of these in the next hour, and you’re not allowed to move,’ which is one of those impossibilities, because if you drink three litres of water you certainly need to go to the lavatory. Anyway I did, and I was the only person who didn’t seem to have a headache.

The tests are the standard tests. They put little electric probes into you. Some of them are passive and they read the electrical connections, I suppose. The others are active and make various bits of your anatomy twitch, mostly in the legs. It’s very disconcerting because you’re sitting there and suddenly your leg jumps. And there’s no logic to which way it goes, either. It’s a bit like starting a steam engine, you don’t know whether it will go backwards or forwards. And you get also an MRI scan, which I suppose is to eliminate other things.

Anyway, so the diagnosis was straightforward. I was one of the ones for whom there was no question. I had ALS and it was standard. My readouts showed all the standard features.

A slight worry is what causes the MND. I mean you know about the two groups of people who have MND in clusters. The first are Italian footballers, who one assumes probably from their diving have eaten too much grass which has been infected, I don’t know, organophosphates. I’ve no idea why Italian footballers – it may be something in their diet. It doesn’t seem to be their lives, so it doesn’t seem to be environmental.

The other are people in Guam, who have a really rather odd diet and they eat fruit bats as part of their diet. This seems to give them toxicity. I think it’s toxicity from the fruit the fruit bats eat, which passes through the fruit bats and gets concentrated. And they have about a 200 per cent chance of getting motor neurone disease greater than average. But apart from that, it doesn’t seem to be related to race, it doesn’t seem to be related to sex, it doesn’t seem to be related to whereabouts in the world you live, to diet. I mean, Japanese people who eat fish and rice are just as likely to get it as people in Europe who eat a meat diet or South Americans or whatever. It just seems to be totally – apart from these two clusters. The only other possible cluster, in [county] we don’t have many people who work in forestry and farming, but maybe people who worked in forestry and farming or gardening have a higher possibility, which gives you the horrible thought that it might be organophosphates. And I happened, I used as a kid to work on a couple of farms and I’ve done sheep dipping and things like that in my past. And you wonder whether some days when I was 20, in my 20s, dipping sheep without gloves and without proper protection, as we did in those days, may just have caused it. I don’t know. I can’t think of anything, but it’s the only possibility that sort of sets me apart from ordinary people. I’ve had a normal diet apart from that – not wonderfully healthy but quite good these days. I don’t seem to have done anything else. So it’s perhaps my days of working with cattle and sheep, on hill sheep farms, hill cattle farms. I can’t think of anything else. Maybe just statistical. I’m the one they choose. Two hundredth thousandth person or something.

Footnote

The reasons why people in Guam have a high rate of MND are still unclear. A recent paper at the International MND Symposium has presented some evidence that the main risk factor may be toxins in flour made from cycad fruit, rather than consumption of fruit bats, but that a genetic factor may also be important in triggering MND. A summary is available at’

www.mndassociation.org/research

Research suggests there is a higher risk of developing MND amongst footballers and farmworkers, but the reason why is still unclear. The MND Association provides a series of questions and answers at’

www.mndassociation.org/research