Ken - Interview 15
More about me...
Ken first noticed symptoms about 6 years ago in 2001. The first thing he noticed was a heavy, stiff feeling in his left arm when he woke up in the morning. Then he started to feel butterflies in his tummy for no obvious reason. He went to see his GP when he started getting cramps, but the GP did not think it was anything unusual. Not long afterwards he went back to the GP, because he was losing strength in the muscles of his right hand and could not operate the remote control for his car. This time the GP was immediately concerned and referred him to a neurologist. At the time, the GP mentioned it might be a trapped nerve, although looking back Ken and his wife wondered if he knew more than he was saying.
Ken was admitted for tests in the spring 5 years ago, and was told it was a clear diagnosis of MND. His wife was more upset at the time than he was. At first he did not want to know the detail of what to expect, but by the time they had a further consultation a week later at a specialist clinic he felt ready to ask more questions. He went back to work as a teacher, but by this time he was having trouble writing on the board in class, and very quickly decided to retire. His wife also took retirement. They decided to enjoy several holidays together while they still could.
Gradually his left arm also became weaker, and his occupational therapist provided arm supports to help him eat and use the computer. He found these really useful in maintaining his independence for as long as possible. His legs were also becoming weaker, and he started to need help getting up, but he could still walk independently until two years ago. Now at home they have a hoist, a lift and a wet room, and a special toilet which enables him to wash himself. They have had a car (a people carrier) specially adapted. He has a wheelchair which he can operate with his chin, and an infrared remote control he can use to operate electrical equipment at home, such as the TV and lights. He has found some useful voice recognition software which converts his speech into text.
At present his wife provides all his care at home, but she does get very tired. He has had one respite stay in a hospice, and they are considering getting more help in at home. Both he and his wife still sing in choirs, and he would like her to be able to continue to go out and sing even when he becomes more dependent. He has been generally very happy with the care and services provided, and has always found staff willing to answer any questions they can, but of course no-one can answer the question of what causes MND or why people are affected in such different ways. He himself wonders whether exposure to organophospates from farm work in the past had affected him, or possibly a period of stress and depression in his life some time ago. Ken is glad that in his case the condition has progressed more slowly than he originally expected.
He had various tests including a lumbar puncture. He was the only person in the ward not to...
He had various tests including a lumbar puncture. He was the only person in the ward not to...
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.
He has read up on suggested causes. He worked in farming and wonders whether exposure to sheep dip affected him, or whether it's just chance.
He has read up on suggested causes. He worked in farming and wonders whether exposure to sheep dip affected him, or whether it's just chance.
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
He had some liver problems taking riluzole, especially when he was also taking minocycline to...
He had some liver problems taking riluzole, especially when he was also taking minocycline to...
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. See www.mndassociation.org/research/research_explained/
His arm felt stiff when he woke every morning, and he noticed fasciculations and cramps. His hand...
His arm felt stiff when he woke every morning, and he noticed fasciculations and cramps. His hand...
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.
The location and design of ramps and dropped kerbs is often a problem but his local council has...
The location and design of ramps and dropped kerbs is often a problem but his local council has...
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.
He has a Clos-o-Mat toilet at home which gives him independence. They are now available in some...
He has a Clos-o-Mat toilet at home which gives him independence. They are now available in some...
He is glad the doctor did not tell him straight away the worst that could happen. No-one can...
He is glad the doctor did not tell him straight away the worst that could happen. No-one can...
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 who've started the same way or deteriorated the same way. I know people who've gone legs first. I know people who've 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.
Wheelchair access and disabled parking seem easier in France. People are more firmly discouraged...
Wheelchair access and disabled parking seem easier in France. People are more firmly discouraged...
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.