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Paul - Interview 09

Age at interview: 58
Age at diagnosis: 56
Brief Outline: Paul's diagnosis of progressive muscular atrophy was confirmed 2 years ago (2004) after several years of leg pain and weakness. Now unable to walk and he has limited arm movement. Uses a ventilator, but his speech is not affected. He worked until 6 months ago.
Background: Paul is a retired university IT specialist, married with 2 children, aged 17 and 14. Ethnic background/nationality' White British.

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Paul first noticed symptoms about four and half years ago, especially pain in his legs and difficulty walking. He saw his GP a few months later, who at first thought it might be spinal stenosis (compression of spinal cord and pressure on nerve roots). A year after his first symptoms began to appear, he was referred to a neurologist, and at this point a provisional diagnosis of peripheral neuropathy was suggested (damage to peripheral nervous system).

As Paul's muscle weakness got steadily worse, the neurologist began to think it could be MND and a diagnosis of the progressive muscular atrophy form of the condition was finally confirmed just over two years ago (2004). By this stage Paul was using a wheelchair. He had already done a lot of research on the Internet and thought to himself it might be MND, so he was not that surprised by the diagnosis.

His employers were supportive in helping him stay at work, including providing voice recognition software for his computer, and the Access to Work scheme paid for taxis to work. He was also able to carry on singing with a local choral society until a few months ago, and feels it is important to keep doing normal things as long as you possibly can.

About six months ago Paul decided to stop working and take early retirement, after he caught a virus and had to spend several days in bed. He finds that every time he has to spend time in bed or in hospital, his muscles get significantly weaker. The combination of reduced mobility and increasing breathing difficulties made him feel it was no longer realistic to work. He now uses a ventilator; initially this was just for night time, but he now uses it during the day as well. His speech remains unaffected, and he is able to eat and swallow, although has to be careful about eating because of his breathing difficulties. He takes riluzole, although he is uncertain how far it has benefited him. 

Paul has a live-in carer and a night-sitter. A district nurse from his GP practice visits once a week. He feels it is especially important to have plenty of support in place so that his wife does not have to do everything and they can keep as normal a relationship as possible. He advises people to think ahead about what practical arrangements they may need to get in place to make the most of their life.

 

He lost muscle strength each time he was in hospital and found it hard to get active again....

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I had three one-week spells of treatment with intravenous immunoglobulin, which meant hospitalisation for a week. Now one thing that I hadn't anticipated about this particular condition is that if you don't use your muscles much, perhaps even more for, for most people, then you tend to lose the ability to use them. And on all three occasions what I found was that, after being effectively in bed for the better part of a week, there were things that I could no longer do as well when I came out. In fact after each of those three occasions I very rapidly had an accident immediately afterwards. So for example getting out of the settee, toppling forward and breaking a toe for example. Or spraining an ankle within a few days of leaving hospital. So I think that's something one does have to watch out for. In my particular case it wasn't helped by problems with one of the syringe drivers, which meant that I spent probably more time in bed and less, less time sort of walking around or going to the loo or things of that sort, simply because it had to be reset every time I, I left the bed. So it's, it's worth watching out for that and warning staff, hospital staff that this can be a problem. Because I, I don't think they realise that it could be.

You actually need to keep moving while you're there?

That's right.

And when you got home did you just, how did you build up your strength again?

Just by being active, as active as I could. But the thing about MND is that it's very difficult to recover an ability to do something once you've lost it, simply because the muscles are actually losing their connections.

Have you ever had any access to any physiotherapy?

Yes, yes, the physiotherapy that I, in fact one of the things again perhaps worth commenting on, at an early stage of the diagnosis process, before it was realised what it, what I'd actually got, we were still thinking of peripheral neuropathy. After some initial physiotherapy at the local community hospital I was actually given a pass to the local leisure centre, where they had, you know, the gym, and was encouraged to use that. With the benefit of hindsight that wasn't a terribly successful approach. Of course, because one of the things, the best way of managing MND is to avoid getting overtired. So really encouraging one to pedal furiously on an exercise machine or something of that sort really wasn't doing any good at all, and in fact was simply reducing the amount of energy I had for sort of everyday essentials.

And after you were diagnosed did you keep, did you have any physiotherapy?

The physiotherapy that I've had since diagnosis has been mainly concerned with maintaining the flexibility of joints. You can't really build up muscles as such. The main thing is to try and keep things working for as long as possible. So things, well, very simple exercises, I mean that you can do in a wheelchair if you reach that point for example. But certainly not anything involving very hard work.

 
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He was pretty sure what the diagnosis would be, so it didn't come as a 'nasty surprise' and he...

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Well, I was aware, I mean I did a lot of research on the Internet. Having university affiliation meant that I could dig a little deeper than most people, because I had access to specialist journals and so on. So by the time that, in fact quite some time before we confirmed the diagnosis, I was pretty sure what it was. So it didn't come as any great surprise to me. You know, one just takes these things in one's stride and sees what happens, really.

Is that how you felt when they confirmed that's what it was?

Yes, I wasn't surprised. It didn't come as a nasty surprise, because I guessed there was a very good chance that's what it was.

 

It can take a long time to get equipment or care in place so you have to anticipate future needs....

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Yes, certainly the, one of the biggest problems is that it takes time to set things up. I mean it's the same actually whether you're talking about acquiring aids or about arranging care. You have to anticipate where you're going to be in a, in a few weeks' or months' time, rather than sort of asking for something now that you need now. In the case of setting up the care for example, what we found, and obviously this is just our experience, is it could, it can take six weeks before something arrives. And with a condition like this, you know, things could have changed by then, your needs could have changed by the time you get something.

That's one way you've managed the, the progressive side of it. Is there any other ways you've managed to deal with the progressive side of it, to cope with that?

I suppose some of the time one, one, one, I suppose the hardest part about dealing with a progressive condition is that you know that you're going to deteriorate, you have no idea what the timescale is. And I think, I've certainly found it has been necessary sometimes to think, 'Well, it's probably not worth pursuing that particular avenue'. Because by the time I get something, either my needs will have moved on, or they may, that may no longer be particularly appropriate. Look, something that looks appropriate now, but will probably benefit you for so little time, is not worth pursuing.

 
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Public disabled toilets are often cluttered and badly designed. Using a hoist is uncomfortable if...

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I suppose one of the biggest problems that I encountered, really, in terms of living a sort of normal life was things like when you're travelling, discovering that disabled loo's aren't really disabled loo's, for example. I say travelling, or going to pubs or whatever. But you go to somewhere and you discover that in a pub, for example, the disabled toilet might actually also be used as a storeroom. So it will be cluttered up with ladders and buckets and things of that sort. Or a disabled public loo, for example, might be set up for people just by having perhaps a grab rail or something like that, but nothing more - perhaps no facility for manoeuvring a wheelchair properly. Things of that sort.

On the topic of disabled toileting and showering facilities, it's perhaps worth saying that one of the most useful things that I had in our own installation was a toilet which actually does the sort of washing and drying for you. Because when you have to be hoisted - particularly if you've got breathing problems - when you have to be hoisted on to a toilet, the extra complications of having somebody wiping you and cleaning you up afterwards while you're suspended in a hoist or when you're sort of perched on a commode or whatever, perhaps rather uncomfortably, that's an extra complication you don't really need. The downside is that they are very expensive. So if you can afford them, fine. But if you can't, well, you know, see if you can get someone else to pay for it.

 
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Access to professional journals on the internet helped him know what questions to discuss with...

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I found it very useful with the Internet, both to be able to read about some of the tests that I was undergoing, so that one had a better understanding both of the kind of processes that were being applied, so you can talk more meaningfully to the specialists, and also to get a realistic sort of picture of what the conditions might be that one is being considered for, as it were. I'm talking now not just about what's normally available on the Internet but also specialist journals. Because there's obviously also a lot of rubbish on the Internet. So you're rather picking and choosing. And you don't necessarily use reputable sites. So, you know, again with a university background and with access to online journals and so on through the university affiliation, it meant that I was able to effectively read the same journals as the specialists. So that was quite useful.

 

He uses a ventilator most of the time, which enables him to speak normally. He has adapted well...

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Over the following couple of years my condition has deteriorated to the point where at the moment obviously I can no longer walk, can no longer weight-bear. I have relatively little use of my arms now. But still I'm able to sort of talk and so on quite happily, provided I have a ventilator. Anything more than that and without a ventilator I run out of breath very quickly. Initially I was given the ventilator just for night use. But that was in what? March 2006. But within a couple of months I was using it most of the time because the breathing was being affected, the chest muscles and so on were being affected by the same condition.

So the ventilator is quite a new thing that you've had. How have you got on with it?

Well, I was lucky. I got on with this particular ventilator very well. It's a non-invasive, positive-pressure ventilator. Just using a nasal mask, which among other things does allow me to speak quite normally. And I adapted to it very quickly. I've never had any real problems with it at all. Obviously there are slight concerns now, because using it all the time, one has to take into consideration things like power cuts as a possibility. And one of the things we arranged to get in fact was a battery backup, so that if we do have a power cut, it's quite straightforward to switch over to that.

Well, again with the ventilator you can't leave me for too long. You know, I've really got to be monitored pretty much all the time and have people within earshot. Because with this kind of mask for example, if I have a sneezing fit and my nose gets blocked afterwards, starts running and gets blocked, then with the ventilator blowing away it can be quite difficult to breathe. Or a coughing fit or something like that. Also I cannot reach out and get myself a drink. So you can't just leave me indefinitely. The ventilator dries you out a lot and you get sort of tickly throats and things like that and start coughing and choking. So, you know, you have to have somebody around all the time.

 

Acupuncture relaxed him, but he saw no longer term benefits and it cost too much. It made...

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When I was still at the stage where the diagnosis looked like being peripheral neuropathy, I tried a course of acupuncture. I should say that I'm not a huge believer in alternative therapies, but I'm prepared to give anything a try. And also quite often what you find in these situations is that friends and relatives and so on think you ought to try something. So sometimes I'm quite happy to go along as much as anything else to keep them happy, sort of for their benefit as much as for mine. Anyway I did try a course of acupuncture. Which was all right, in the sense that afterwards for a short time I'd feel quite sort of relaxed. But there's obviously no longer term benefit from it that I was feeling. I continued with it really for quite some time, but obviously after awhile I decided it was too expensive to justify it, just for sort the very minor benefits I was getting. But one unexpected benefit if you like was that when I started receiving, when I was given more hospital tests, checking nerve conductivity, which involves sticking needles into you, I didn't mind that as much as most people. And in fact the specialist concerned was rather surprised by just how well I was tolerating it. Of course the thing there was that after you've had about forty needles stuck into you for acupuncture, then it was really much less of a concern having a few more stuck in just for some electrical tests. But I think in other respects mostly the, I felt it worth going on for some of these alternative therapies, as much as anything else to keep other people happy, rather than having any great belief in it myself.

 

His advice is to keep life as normal as possible and think positive. At the same time you need to...

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I think if you've been recently diagnosed with motor neurone disease probably the most important things are firstly to try and maintain life as normal as possible, simply because that helps to maintain morale. And think positive. There are lots of things you can do. And also, given that it is a progressive condition and nobody knows how long it will take to deteriorate, there are people alive who've been, who've had motor neurone disease for as much as sort of forty years or more after all. You have to be prepared for change. You have to anticipate problems, that problems will arise. And as soon as you become aware that you're losing a particular ability, you need to seize on the opportunity to gain whatever help will enable you to carry on, whether it's getting new equipment or increasing the level of care and other support that you have. You can't really hang around, because if you do then your quality of life will suffer. So I think you've got to keep looking forward all the time and monitoring your condition. And at the same time I think you've got to be completely open about the condition. Because certainly my experience is that friends and colleagues have been extremely supportive.

 

He was glad to have help from Citizens' Advice in filling in the forms. Waiting for claims to be processed can take a long time.

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One of the things that one encounters when one has a condition like this is a huge amount of paperwork. And, and I was interested to see in a recent television programme that the, they, they, they said which of the various government forms were the, considered, generally considered to be the worst ones. And one of them it turns out was the Disabled Living Allowance form, and another one was the Carer's Allowance form. Both of which are quite important when you have a condition like this. We filled in the Disabled Living Allowance form ourselves initially. Which, you know, even with a university degree and so on and so forth was really quite hard work. It was about, I can't remember, it was about forty pages, something like that. When it came to getting the higher level, the highest level of the, oh, dear, what is it called? 'Attendance Allowance, we were offered, it was suggested to us that we should bring the Citizens Advice Bureau into it, because they had people who knew these forms pretty well. So anyway we contacted the CAB and somebody came out and helped us. But they really were very good. That actually speeded things up quite a lot. Certainly saved a lot of sort of scratching of heads and sort of wondering what the best answer was. Now again the Carer's Allowance form is over thirty pages I think. So anybody who doesn't like filling in forms, they would probably be well advised to consider their local CAB, who will usually have somebody who is very familiar with the forms. I think that's probably all one needs to say there.

Has there been any, and actually getting the Disability Living Allowance, did that come through okay finally?

Yes, it took I think about six weeks maybe, something like that, for the Disability Living Allowance, for the, for that to come through. So you do have to be prepared for that. It does take time. And I think the same is true of things like the Carer's Allowance. These things don't happen instantaneously. Although obviously providing you make sure that things are properly time-stamped when they go in, you should get everything backdated.

And have there been any other financial implications from your illness?

I mean I suppose I'm fortunate enough, fortunate enough to have quite a, quite a well-paid job. So on occasion we've bought things ourselves rather than going through the system and sort of waiting a long time to get it. Of course not everybody has that option. But on the other hand, on the other hand things like, we, we converted a utility room to a downstairs shower room with special facilities for me. We, we could have gone through the sort of local authority support with that. But we, we, we worked out that in fact the cost really wouldn't, when you looked at what I was earning and so on, we would probably not be getting much help for it anyway. So there was little point in hanging around waiting for it to come through, since it could have taken six months to get. So that was another of the things that we paid for ourselves.
 
 

The district nurses and MND clinic coordinator help fight his corner. He's also spent a lot of...

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The GP practice that, that, that I use is one which has I think maybe five partners for example and then other doctors as well. So there are quite a lot of people involved. And in general one can never be sure of seeing the same doctor twice. Generally speaking the, the communication between the doctors in this area is quite good. But recently we found it was a good move to try and involve one doctor more perhaps. And so now that I'm pretty much housebound, we try to arrange for the same doctor to come every now and again, just to sort of keep in touch with a home visit. And that system has worked reasonably well so far. We also have a, a district, a district nurse come in once a week from the same, they, they, they work from, out of the same practice. And that's been working very well. In fact it has to be said that the, the district nurses have been a tower of strength when it comes to fighting our corner with problems with the agency for example or with the local Social Services or whatever. They really have been very supportive. And I would encourage anybody to get them, get the district nurses to work for you.

On the whole the services have worked quite well. Where I have some reservations is in the communication between them and in making sure that we actually get what we need when we need it and so on. The district nurse has obviously been very important in bullying various other organisations. Likewise the local MND clinic coordinator at the local hospital has been extremely good at sort of fighting our corner for us and good at making suggestions and so on. That said, a huge amount of mental energy and emotional energy has been consumed at our end I think by making sure that we do get what we want. Things, it has to be said, have not worked very well in some respects, like the length of time it's taken to get particular care arrangements in place. Problems with the agency not always being able to provide staff, so you get a phone call perhaps rather late in the day saying, 'We can't provide anybody tonight' something like that. And making sure you get the right kind of staff. Where we've had some interesting experiences has been, been in, simply in the selection of staff. We've had people suggested to us as night sitters for example who from past experience we know sleep very heavily and have to be more or less kicked awake. So that's obviously not a very satisfactory arrangement for sort of night sitting, where, particularly if you, for any reason if I lose control of the sort of bell push for example, I wouldn't be able to wake them if I had any problems. 

We also had somebody who, coming sort of, coming one night, partly to find out whether she could sleep through the night, so she was coming in effectively for assessment by us when we had another night sitter in, so this would be a, a new potential night sitter, went into complete panic mode and got very confused about where she was and so on. Which again would not have been a very satisfactory arrangement if I have any kind of emergency. So we've had to do quite a lot of rather careful vetting of, of staff and things like that.

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