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Motor Neurone Disease (MND)

Tube feeding (PEGs, RIGs, PIGs) and ventilation

When motor neurone disease affects the throat muscles, people may have difficulties with talking, swallowing and breathing. (See also 'Eating, swallowing and breathing' and 'Speech and communication'). Here we look at some of the interventions which can help, such as tube feeding with swallowing and ventilation with breathing. 

Tube feeding - PEGs, RIGs and PIGs.
Many people living with MND find they can continue to eat ordinary foods for a long time by being careful about what they eat and choosing softer foods, including purees and liquids. If eating and swallowing become more difficult, one option is to have a feeding tube inserted directly through the stomach wall - a PEG (Percutaneous Endoscopic Gastrostomy), a RIG (Radiologically Inserted Gastrostomy) or PIG (per-oral image guided gastrostomy), which is less common. The difference between them is the way they are inserted and the stage of the condition where it is appropriate for the operation to be performed, but the end result is the same. A nasogastric feeding tube (passed through the nose and down the throat) may be used as an emergency measure, but is unlikely to be recommended for long term use.

Not everyone wants to have a feeding tube, and this has to be a personal decision. Discussing the options with a speech and language therapist and dietitian will help people make an informed choice. The MND Association website provides - Information sheet 7B - Tube feeding and 7A - Swallowing difficulties.

 
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She was advised to have a PEG because of weight loss. She decided not to have one and feels that...

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Age at interview: 59
Sex: Female
Age at diagnosis: 56
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So I was referred to the local hospital to see the speech therapist and the occupational therapist. The speech therapist and the rehabilitation consultant were talking about me having a PEG because of my weight loss. I felt very strongly that I didn't want a PEG at this time, and fortunately my MND consultant agreed with me. I think I've been proven right, because three years later I've still not lost any more weight. So that was a good thing. And again the speech therapist, I can contact her when and if I need help.

Is there any way the doctors or any of the nurses communicated to you, have there been any good examples or any not so good examples?

I suppose good examples are people saying, you know, 'We're here to help you in the way that you need'. I've appreciated that more than the example of, 'Well, you're a very thin person. Therefore I think you need a PEG'. And that was really quite frightening. Because although yes I am a very thin person and that is one of the troubles with MND, I've only lost about a stone from my original weight, and I was a pretty thin person beforehand. So, yes, 'Let's, let's work together about finding what solutions you need' as opposed to, 'This is what I think you need'. That works better for me. Whether it would work as well for everybody, I don't know. But it certainly works better for me that way, yes.

Several people we talked to had decided to have a PEG or RIG, though not all were using it yet. People were commonly advised to have it done while they were still relatively fit and before they lost a lot of weight, so they would recover well from the procedure. Several said it was reassuring to know the tube was there ready if they did need it. Some people continued to eat some foods by mouth as well as using the tube.

 

She had a PEG fitted while her lung capacity was still good - the procedure was simple. So far...

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Age at interview: 34
Sex: Female
Age at diagnosis: 31
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Yep. So I had lung-capacity testing a few months ago, and that was to check that I could fly. It was also to check my lung capacity because they wanted to, my neurologist had advised me that it might be worth thinking about getting a PEG inserted. And she'd explained why, and that it was really to, so it was there to be used if and when I needed to. And if I didn't want it in the future I could have it taken out. But once my lung capacity got too low they wouldn't be able to do the procedure, and that takes your choice away. So I haven't made a choice as to whether I want to use the PEG or not. What I have made the choice is, I want to have that choice later down, later on and further down the track. The procedure was fine. It was very easy. The hardest thing for them was trying to find my veins, because they're just absolutely thin as anything. But the procedure only took about ten minutes, and that was all fine. It was quite painful for a couple of weeks afterwards, very, very sore, and it was difficult to laugh, to cough and to move. You know, it does affect a lot of muscles. But that was only for a couple of weeks and now it's fine. It doesn't really affect me. And I've been talking to my dietitian about getting a, a flat PEG which doesn't have a tube routinely. So that it's just literally sitting there flush against your skin and doesn't show through your clothes. So I'm going to get that done in a month or so's time.

Have you ever used it at all?

No. We, I have to flush it every day just to keep it clean, but, no, and I'm - no. It's just - I tell people it's just, just an accessory, it's just for show at the moment [laughs]. But I'm looking forward to getting that tube, you know, cut down and just, you know, not having that. It was a bit of a, it was upsetting to first see it and get over that. But that took a couple of days, and it really wasn't a big deal. You, with this illness you, you adapt and you move on, you know, and just as you've mounted the next challenge there's another one that comes along. So, you know, you just, you just do.

This woman found the procedure very straightforward and recovered quickly. Some people took longer to recover and a few developed an infection at the site. Sometimes the 'stoma' or hole (through which the tube passes) tries to heal over. This needs to be treated to stop skin regrowth. One person had to have the tube replaced repeatedly because it kept perishing, which is very unusual. (Different types of tube are used; some last longer than others). 

 

Her PEG was fitted early, to prevent weight loss. She has had two infections, but now hardly...

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Age at interview: 45
Sex: Female
Age at diagnosis: 37
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You've got a PEG, I think you said, but you're still eating and drinking normally as well?

Yeah. I had it put in in March, because I was losing weight slightly. But because I'm not heavy anyway, losing half a stone to me was a lot of weight. And then I was, I take build-up drinks to get, that's kind of kept my weight stable. But there's a lot of evidence that if you have the PEG tube done early, rather than when it's absolutely necessary, the recovery is better, and life expectancy. So I agreed to have it put in just - way before I needed it for feeding - so that it was there if ever there was a problem, rather than having a drastic weight loss.

So do you use it at all at the moment, or?

Not at all, no.

It's just there waiting?

It's just there, yes.

And did, have you had any problems with recovery or infections or anything?

I've had two infections, and it took a long time for the wound to heal. And those are things that I didn't expect. I thought it would be in and over and - but it took more looking after than I originally thought. And luckily I've got an extremely close friend who - and they wouldn't let me out of hospital unless two people could look after it, and I didn't want the children to feel that the main - and she calls every day, and turns and cleans it, washes it out. And now, if she can't come, my daughter does it. But on the whole it's my friend. And I've got another friend, so that if I've got to have a break she's on hand to do it. When I had a, a holiday in the Lakes I took my other friend with me because she can do it. So that gave the one at home a break. And, but I, my theory wasn't just - if I'm not going to need it for feeding get it put in and that's the end of it. I didn't realise that there was an ongoing care.

Do, do you regret having it done?

I did regret having it done in the first six months, because it kept flaring up and I think, well, I've had lots of problems with it. But now I kind of hardly know it's there. But now I have to actually - the PEG itself only lasts eighteen months, so I'll have to have another one put in. Which I had, didn't know at the time. And I think I may have waited a little bit longer, had I realised that that was the shelf-life, really, of a PEG. So I'll have to go and have another one put in.

Footnote' PEG tubes can last much longer than 18 months, and there is no need to replace the tube if it is still working well. Each person must be individually assessed to check whether a replacement is needed and when.

 

His wife was advised to have a PEG. They were very happy with the care. However, the tube had to...

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Age at interview: 73
Sex: Male
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My wife's case has gone very slowly, thank goodness. The throat development, she lost her voice. And then the next thing that happened was she slowly but surely lost the ability to swallow food, solids. And then it became obvious that we had to find an alternative method to feed, and our specialist said that we would have to have a PEG installed with my wife so she could take on liquids. So that was organised. And again a very efficient set-up in the [other city] hospital, and we remained there with all these activities. And they've been wonderful. I can't speak highly enough. Obviously they're set up and capable of assisting people with our problem. 

So my wife was then, had a PEG installed and she lost her ability to take in food via the mouth. And the next problem that developed was saliva, she couldn't swallow saliva. So we've had that problem that we've had to get over or around, or manage. 

One of the things that the specialist informed us with this particular disease is that as long as my wife is kept healthy and strong then she can expect a reasonable length of time that she will manage to deal with this problem. But if she drops her weight or her strength, then this aspect of it will lead to other problems that will obviously cause us very serious problems.

For instance we've had to have the PEG installed, she's just yesterday come from being into the hospital to have it replaced for the fourth time. And this is probably a nearly unheard of amount of times. The first three times it was done in [the city hospital]. And the staff that have been dealing with this problem have said they've never come across this particular failure in the tube. It's a tube that has been giving trouble and has failed, and they've sent it to the laboratories. They can't find out why the tube has failed and why it has perished. So we've had to have it replaced again, for the fourth time, but we don't know how long that... So I mean all these problems are things that we have to deal with and overcome. I mean, they are very worrying because obviously my wife's life depends on the amount of liquids that she's able to take in through this method. Again getting the liquids and the types, they have flavours and things, but again this is another problem which is personal to my wife in that she likes some of the flavours and she doesn't, and all this procedure of going to the doctor's to get syringes, replacement syringes, it's all a considerable problem, but it has to be overcome. 

Footnote' Various types of PEG tube are used; some last longer than others.

A few people had more problems with the procedure itself or with the care afterwards. Sometimes speech difficulties made problems worse because they made it hard to communicate with staff. (See also 'Speech and communication'). One woman said her mother found that lying flat made her want to choke, but staff caring for her seemed not to consider this. Another said staff treating her husband found it hard to insert the endoscope through which the PEG tube was to be passed, because his swallowing reflex was affected. However, other people with swallowing problems had no difficulty with this part of the procedure. 

 

He had problems with the PEG procedure because he couldn't swallow the endoscopic tube and he...

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Age at interview: 54
Sex: Male
Age at diagnosis: 52
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The other experience that we had was when he had his PEG tube fitted last December. The decision was taken that he would, he would have it done. Mike made that decision himself, to have it done at that time, and it was set up pretty quickly really for him to go in to get it done. And the words were -and the speech and language therapist would, would support me on this one - she has changed the way that she speaks to people now as a result of our experience, because she said to us, didn't she, 'It's nothing. It's just a quick process, procedure. You'll be in and out. Don't worry about it. No problem, you'll be fine. They'll take you in the night before' - which they did - 'and then they'll ambulance you across to the main hospital, and everything will be fine.' And that was great. So we were pretty confident, weren't we, that that was okay. So I went with Mike down to the waiting area before they wheeled him in to do the procedure. And of course Mike kept writing on his board, 'Remember to tell them that I've got motor neurone.' And I said, 'I have told them. I have.' And then they came and explained what they were, what was going to happen. They were going to put this tube, you know, down and out through his stomach. Fine. And Mike kept saying, 'They do know I've got motor neurone? And they do know that it's affected all this area?' [indicating face and mouth]. I said, 'I've told them.' Anyway the consultant came up to me and he said, 'We're going to take him in now.' And I said, 'That's fine.' And he was fine about it.

Of course they got him in, and they couldn't get it down, because all the muscles, they don't know what was going on inside. He couldn't take it because the muscles couldn't, he couldn't swallow it, you see, he couldn't react to this tube going down. And it, they just couldn't get it down at all. And of course they have a plastic piece that they put in the mouth to put the tube and the camera, apparently, down the throat. This is what we were told.

And it was on his lip. Well of course Mike can't speak. And what they did when they - they tried about three or four times to get it down. And I could hear what was going on in the room, and I actually thought that they were killing him. He was in such - you couldn't, you couldn't hear him shouting because he can't speak, but you could hear him moaning. And I wondered what the hell was going on. And, and all of a sudden it went really quiet. And the consultant came out, and I remember looking at his face thinking, 'They've killed him' [laughs]. And I said, 'What's gone on?' He said, 'We've got a big, we've got a problem.' I said, 'What's, what's the problem? What's happened?' And he said, 'He just can't take the tube. He won't, I can't get it to go down. And he's fighting it,' because he couldn't get it down, you know, and that. And when he came out of - they brought him out of the ward, out of that room, back onto the, into the waiting area to decide what they were going to do next. And what they'd done is rip the thing off his lip, you see. It was stuck on his lip and Mike couldn't tell them. So they ripped it off. And of course his mouth was out here, and they hadn't got this tube down, you see.

And then the guy that was doing it said, 'The other problem I've got,' he said, 'he needs to have it done.' I said, 'Well, obviously.' He said, 'It's Friday afternoon.' He said, 'And I don't know whether I'm going to be able to get an anaesthetist. He's going to have to go down to theatre to have it put in under anaesthetic, and I don't think I can get an anaesthetist on a Friday afternoon. So you might have to, you'll probably have to come back Monday.' So of course Mike's writing on his board, 'I want it done. I want it done.' Because he'd obviously gone through that process himself of, you know, in confirming with himself that he needed to have it done. And he didn’t want to come away without having it done. And the guy said, “Well, I’ll do my best. I’m going to try and ring round and see if I can call on an anaesthetist.” And we waited and waited, didn’t we? Anyway he came back around about 2 o’clock and he said, “I’ve managed to get somebody to come from [city] who is prepared to, to come across this afternoon.” So they took Mike to theatre that afternoon, and they managed to get it into him.
 

They were surprised he was discharged so soon after the PEG procedure. No-one showed them what to...

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Age at interview: 59
Sex: Male
Age at diagnosis: 58
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Have you been happy with how the, the PEG has turned out?

Oh, it's been very good, hasn't it? We've had - Ken went and had it done in June, didn't you? Sort of backtracking here. It was just, we took him in in the morning. At that stage Ken was still quite, could walk, couldn't you? Took him in in the morning. And they were going to give him a local anaesthetic to do it. And then went back about half past 5, me and my son went to pick him up. And I've got to say I didn't think it was handled very well at all.

Because it was in a ward that closes, you know, a day thing, I felt like - because Ken was about the last person to leave - the nurse just came along, thrust a leaflet in my hand, said, 'Don't use it' - was it till so many hours or something? I said, 'But nobody's shown me what to do yet.' The dietitian wasn't coming till the next morning. And so she said, 'Oh, that's all right. You just read the leaflets. Off you go.' And that was it. Well, Ken could hardly walk. He was like doubled up, weren't you? And there was a wheelchair sitting in the corridor, which I wanted to use, but Ken being strong-willed said, 'No.' So he managed to crawl - luckily we were parked outside [laughs] - crawl to the car. How we got him in and out of it I do not know. Managed to get you upstairs to bed. I think you went, went asleep, didn't you? Our son went out for a few hours in the evening. When Ken woke up, he was in so much pain. He - well, it was awful. It's the worst experience of my life - didn't you? Oh, I just didn't know what to do. I really was, you know. So I phoned the doctor. Of course now it's out of hours, isn't it, which you have to explain to some woman on the end of the phone the problem. She then has to transfer it to a doctor, who then decides whether they're going to come out or not. Well, of course by now, hours - did I give you? Oh, that's it, they said to give Ken painkillers. Well, of course he can't swallow very well at this stage. I don't know if I did manage to get something down you. I don't know, I can't remember now. In the meanwhile our son comes home. I didn't know whether to phone an ambulance - because Ken was, well, it was awful. He was crying, I was crying. Eventually they phoned back and said a doctor would come. I think from phoning to when a doctor came must have been about three hours. And then two of them turned up. They came up, examined Ken. I think at some stage we had got hold of some painkillers from somewhere, hadn't we? Which I don't know if you'd taken and then they'd calmed you down a bit, but then obviously they wore off. And in the end they gave Ken an injection in his leg, which worked pretty quick, didn't it? And then Ken went asleep. But I must admit I feel that Ken should have stayed in hospital overnight. When the dietitian came the next day, which again was a very nice lady, and she said all the hospitals that she'd worked in, they do keep you in overnight. She said, 'I was really surprised that they'd sent Ken home.' And it's normal to get all this pain, because it disturbs all the stomach and they have to push the tube out, not in. And you was really, you didn't have anything... And then she showed me how to do the feed, didn't she? And you was really poorly for a week, wasn't you? It took Ken a good week to get over it.

 

She couldn't tell staff how much pain she was in after the RIG procedure. She could still eat but...

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Age at interview: 63
Sex: Female
Age at diagnosis: 62
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I have only had one hospital stay so far, for gastrostomy, and I suffered a bad reaction - the recovery time was 3 months. The gastrostomy was thought to be necessary because we were planning to have a holiday of a lifetime to New Zealand and the hospital considered it would be sensible to have the stomach tube in place beforehand. The procedure was horrendous. I know everyone is different, and of course had I known too much beforehand I might have refused it.'Months further on, I believe improvements need to be made to prepare patients for the gastrostomy procedure. I am saying this for the benefit of improving care for future patients.

The ward nursing staff seemed to be unpractised with speech impaired patients, possibly due to temporary 'bank' nurses moving from ward to ward. But considering the ward is experienced at RIG and PEG procedures for MND patients, I was surprised they weren't more aware of how to handle a patient with speech loss.

I was in severe pain for 24 hours following the insertion of my stomach tube and, with my inability to talk, I was not given pain management until a young doctor witnessed my distress. Imagine being in severe pain and not being able to tell anyone about it. In fact I was ignored pretty much. On the sixth day the consultant wanted me gone, saying I would recover quicker at home and clearly MRSA was a real worry on the ward, but my husband refused, saying I was too ill, and the nursing staff agreed. So I stayed one more day and I was given an enema before I left. They insisted it was bad luck and probably due to the gas and constipation. If constipation has a major effect on the patient's recovery, could it not be organised through the GP and district nurse that prospective patients have an enema at home a day or so before surgery?

No one asked if I could eat or not, and presumably had no information about my pre-op condition, since I was not offered food, nor was I asked by nurses if I could eat. Early on they twice attempted food by a slow drip into the tube but my painful stomach rebelled. I was not offered food by mouth, but towards the end of my stay I requested some, because I was hungry. It would have been helpful if soup had been available at any time if I felt I could eat it, but it was not an option. I would have liked the staff generally to have been more aware of my condition and considered my difficulties. There were not enough skilled nurses.

The speech therapist and dietitian were caring and helpful and monitored my progress on the ward frequently, but the release letter from the ward did not arrive with the GP until almost a week later. We had to chase up the district nurse. There was no system in place for me.

No one seemed particularly concerned about trying to get me up and moving ahead of my departure from the ward. A physiotherapist should have been involved, because seven days of pain lying in bed made me extremely groggy and my diaphragm was weak. Perhaps I am expecting too much from the NHS.

I was told the stomach tube inserted was a RIG. It is done by a doctor in the Radiology Unit with nurses to assist. Gas is pumped into a tube via the nose to inflate the stomach to make for accurate placing of the tube. All the while I was able to follow the procedure on the monitor. Four injections of local anesthetic are given around the site, and the contraption itself is pushed through the numbed skin straight into the stomach. The connection cap is fitted on the surface of the skin just below the rib cage.

Four months on from the gastrostomy, the fog lifted and I felt more in control of myself again. I got back into dog walking every day, albeit only short distances, and also cooking meals.

We had to cancel the trip of a lifetime because I felt unwell after the gastrostomy and we considered it would have been a huge risk. However we have just had a very successful five-day driving trip to Germany. Because the roads are so smooth, it was not a tiring way to travel.
 

Some people who relied completely on the PEG or RIG for feeding described how they tried to fit it round the family's normal meal-time routine. One man even had supplies of feed sent out in advance to Portugal so he could go on holiday without worrying about taking supplies with him. Another had found it quite easy to obtain a supply of vegetarian feed.

 
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They sit round the table together for meals and Ken does his PEG feed. At Christmas he stuck...

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Age at interview: 59
Sex: Male
Age at diagnosis: 58
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We tend to treat it as a meal, so that Ken sits at the table with me and our son. So we sit and eat our meal and Ken does his feed. We joke about it, don't we? [laughs] Christmas time, Ken made up jokey labels and stuck them on the bottle, and put a label on your Zimmer frame, didn't you? Said people could hire it if they had too much to drink, and things like that. We try to sort of lead a normal life, don't we?

 

They want to be as independent as possible. Mike has a portable pump for his PEG so they can take...

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Age at interview: 54
Sex: Male
Age at diagnosis: 52
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But what I was just going to say is that we took the decision that rather than rely on nurses to come in and do feeds and all of that stuff, we've all along have said we'd prefer to be trained how to do these things. Because we wanted to sustain our own independence, so that we could still carry on doing the things that we want to do. So like now, for example, Mike has a portable pump, because he's fed by a pump once a day. And it's a portable pump [Mike writing on whiteboard] and we just, we link him up to it and he gets fed, and, you know, we still go out and go to a restaurant or, you know, go and see friends and whatever. It means that we've got independence. Sorry? Jug? Your jug for your other feeds, is that what you're talking about? Yeah. His, his feeding regime is that twice a day, morning and lunchtime, he's fed by a syringe, a big syringe and he just, he decants the feed into a jug and feeds it in. So he does that twice a day. And then in the evening he's pump-fed for about two, two and a half hours, in the evening.

People are usually advised to put only the specially prepared feed through the tube, and not other forms of fluid, including liquidised food, in case the tube gets blocked or damaged. 

 
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Sometimes crushed tablets get stuck in the PEG tube. One day when he was trying to put cod liver...

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Age at interview: 59
Sex: Male
Age at diagnosis: 58
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Even the tablets for motor neurone is a tablet, which I think is absolutely ridiculous. So we have to crush everything to put it down the tube, which then sometimes we get a bit, a bit of a blockage, don't we? [Ken laughs] Because no matter how much you crush it, it only needs a little like a bead of sugar size to stop it. We've had a few instances with that, haven't we, over the ceiling. Ken's always taken cod liver oil, always, for years, haven't you? I mean me, I take a capsule, but Ken takes it by the spoonful, which would make me retch. So now he has that down his PEG [Both laugh]. And you was here - was it here on your own, or was our son here? And he couldn't push the plunger. And so he took it out the tube to push it, and it shot across the ceiling, over the lampshade, on the floor, up the door. It went everywhere - I mean it's only a couple of spoonfuls, isn't it? So our son managed to wipe it all off the ceiling, but we now have a nice yellow patch [laughs] on the ceiling. The next day Ken decided to show me what happened, but not meaning to, so he shot it in a different direction this time, didn't you?

Footnote' The MND Association generally advises people to put only the specially prepared feed through the tube, and not other forms of fluid, including liquidised food, in case the tube gets blocked or damaged.

People can sometimes take medication through their tube, usually as a liquid from their pharmacist. Riluzole, for example, is now available in liquid form. Some medication in tablet form can be finely crushed and suspended in liquids for use with tube feeding - this should always be done in consultation with a health care professional in case it risks blocking the tube or disrupts the way the tablet is supposed to work. Some medications may be available as skin patches. (See 'Medication, trials and research').

The tube needs to be flushed daily, even if it is not being used for feeding yet. It also needs to be turned regularly. Some carers said that at first they found it daunting and frightening to have the responsibility of keeping the PEG functioning but they got used to it. One woman said at first it was, “Like an alien that's come in to live with you in a way. But it's so commonplace now.” Some said they came across other staff such as district nurses or hospice staff who needed more training in looking after people with a PEG. 

 

She felt worried and unprepared for dealing with her mother's PEG. To be feeding the person who...

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Age at interview: 40
Sex: Female
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Next day I was invited in for some training on how to operate the PEG. I'd been given no information, actually, about what we were going to have to do to manage it, once it was there. Anyway I went in that morning, and I was given I think ten minutes to be shown how it worked. And a nurse who spoke very little English came to show me, and she put a syringe of, of, of water through the PEG with such speed, and I think for a daughter to suddenly see a mother being fed through a tube in the stomach with such aggressiveness, I mean that bit in itself is difficult. But the kind of just callousness of it all, just shocked me beyond belief. And I remember walking out of that hospital and phoning my sister in floods of tears saying to her, 'We have to manage this every day. Mum's not going to be able to do this for herself, you know, no-one has told me that we're going to have to flush this thing through every single day now.' And I was just, blown away by that, really'The other'

You didn't get any other training after that,

No.

That was it?

No, that was it. Yeah.

You didn't get any written information on it?

No, I had got hold of a booklet which has information, really, that's good for people who know how to use it, but actually not helpful for somebody who's just picking up on it. It doesn't have any pictures, and actually pictures of course are the easiest thing to follow if one's uncertain. I felt that really they needed somebody like me to write it for them, because they're never going to know what it's like to learn to do it, or what you have to overcome psychologically in order to get round to using it. And I think that's quite difficult. And I think also that there are questions that you have like, 'Well, where do you get the equipment?' and you know, 'How often do you throw away a syringe?', and you know, 'Is it safe just to keep it open in the bathroom?' or you know, 'Could I poison my mother?', or - you know, these questions which may seem really silly or elementary are the things that one really worries about. It's like having a new piece of machinery which actually is going to be crucial to your very existence and you don't know how to turn it on [laughs]. It gives you a huge amount of fear. And I worried enormously about that.

You said that psychologically you had to ', 

Mm.

Can you tell me a bit about that?

Yeah, I think the role of your mother in your life is to kind of nurture you and feed you, and that kind of intimacy is one that's mother to daughter, and suddenly there's a switch which is untimely. It's not as if your mother had lost her faculty or anything like that where your mother's life depends upon your care. And that not just that you're not actually putting food in her mouth, it's through a hole in her stomach and for me the kind of connection between the umbilical cord and the PEG tube, you know, it's there, and that seemed like a very difficult line to cross. I think my mother also had an immense amount of dignity and suddenly I was playing a physical role. And I think again that's quite a tricky line to cross, no matter how close your relationship is. You know, I think there are some things that one wishes, one hopes to keep separate, and that wasn't going to be possible. And I think also for her, again there was this business of, 'I'm not going to be able to manage this myself for long.' So it was having to contemplate again the business of failing.

Some people found the tube sticking out under their clothes annoying or uncomfortable. One woman had made a little fabric pouch to cover the end of her husband's PEG tube so it wouldn't dig into his skin when lying down. Another was planning to get a different type of tube which sits flat against the skin.

Ventilation
Experiencing breathing difficulties and choking fits could be both very tiring and frightening. (See also 'Eating, swallowing and breathing'). 

Several people had managed well with non-invasive ventilation (NIV). NIV uses a small machine to support the person's own breathing, by providing extra air through a face mask or nasal tubes. Ventilation can also help support the diaphragm and make it easier for people to speak.

 

Non-invasive ventilation has made a big difference. It helps her sleep properly at night and...

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Age at interview: 66
Sex: Female
Age at diagnosis: 65
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The MND specialist got in touch with a lung specialist at a different hospital. And they got me in there for a week and to learn how to use non-invasive ventilation. You have a mask and it just blows air up your nose and into your mouth. And so I was there for a week learning how to use that.

But it was so wonderful, because I'd not been sleeping. I had to get up in the night and sit in the chair and open the window because I couldn't breathe. As soon as I lay down I couldn't breathe. And it was so wonderful to be able to lie down and sleep. And I've been using it since March and I can be asleep for eight or nine hours. And I have the most wonderful dreams. And it's really made a big difference. And my voice was going very strange before I went on this machine. And then it seemed to cure it quite a bit. And evidently it's the, you're not getting rid of the carbon dioxide, so that's why you feel rotten and it affects your body a lot. And so they test you, they test your blood to make sure you've got the right levels of oxygen and carbon dioxide. And evidently they only used this machine from last January for MND sufferers. And I said, 'Well, what happened before that?' He said, 'Well, you didn't last very long. Two or three weeks.' So I said, 'Well, I was so lucky, wasn't I?' Because if they'd not put me on the machine, I wouldn't be here now.

Gosh. So that, that has the potential to make a real difference to'

It has.

'people's survival.

Yes.

Do you use it during the day?

I do. I sleep, have a couple of hours in the afternoon and that just gets me through the evening, because I wouldn't last all evening. So it's nice, because my husband comes home at lunch and then I go and have a sleep, and then he's home at teatime, and then I can watch all the programmes and have friends round or whatever. And it's made a big difference. And evidently some people don't like it, but I - well, I couldn't manage without it. But it has made a big difference to me, yes.

 

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

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Age at interview: 58
Sex: Male
Age at diagnosis: 56
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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.

For carers, it can take some getting used to when their partner uses a ventilator. One woman said she had moved out of their shared bedroom at that point, “Because it's like someone hoovering all night to my mind - no thank you!” Another said she had now got used to the sound.

 

She felt she had become a carer when her husband needed to use ventilation every night. It was...

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Age at interview: 54
Sex: Female
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I was going to ask you if you knew, if there was any point in time where you thought, 'I'm a carer. I'm actually caring now'?

I think about five years ago, when he started using the Nippy and I would have to put that on him every night. And knowing that he couldn't actually lie down without using it any more. Well, actually, that's not right. It was about three years ago when he couldn't lie down any more without it. But knowing he couldn't sleep without it any more and he needed me to do that. And he couldn't just walk into the kitchen and get the sandwich I'd made for him any more. That had gone. I would say that was about five years ago.

Your experiences of the Nippy. What was that like as not the person who's using it, but as a wife and carer?

Yes. It's strange. We were lucky in our very first one that we had. It had a good rhythm. That sounds crazy. When you listen to somebody breathe [laugh], It, and the old Nippys are quite noisy. I got used to it fairly quickly. But you do sleep at a lighter level because you're listening for this rhythm going on. And the Nippy is breath, what's the word I'm looking for? You can make it breathe for you a little bit more by demanding an extra breath. I don't know how it works, but the person who's using it would understand. I mean you'd sometimes hear that and think, 'Oh, what's going on?' But as I say I got used to that. That was no problem. It went in for a [laugh], for a...

Service?

Service. That's the word. Went in for a service, and it's never come back. And we were given another one, which is very noisy. It sounds like an old traction engine and it winds itself down and then it winds itself back up again. And it's [laugh], we both wake up thinking, 'It's going to stop any minute.' But it doesn't. It keeps on going. And that one's just had a little service. It's a bit quieter. But he's been given a brand new one now, which is very, very quiet. And we both miss the old noisy one [laugh]. But it's strange getting used to the noise. And it's strange getting used to the fact your partner's having to wear this contraption. But a lot of people with sleep apnoea use these things or similar. So it's nothing too weird. But the very fact that it's giving him that breath that he can't get himself, we're very grateful that we've got one. We both are.

Most people we spoke to were comfortable with the idea of non-invasive ventilation, although not everyone will want to consider supported breathing. (See also 'Hospices, respite and thoughts about future care').

 

Non-invasive ventilation hugely improved her husband's quality of life, but now they realise how...

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Age at interview: 62
Sex: Female
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But I think really before he got the ventilator he was in a very shabby state, his breathing and the toxicity, and the headaches, and the falling asleep all day long. The ventilator, a sort of bi-pack machine was absolutely astonishing. It reset his whole system. It gave him energy, he didn't have to rest between every process of getting up, you know, shaving and teeth and, you know, which was really laborious. It absolutely restored his system. Only now I suppose, less than a year later everything else has caught up and it's, he's more and more dependent upon it put it that way, so.

I mean we had problems in the first hospital with a ventilator because it was a machine they'd only just acquired. They weren't familiar with how it worked. That was one of the reasons Bill went back to the hospice to get it set properly, the alarm kept going off and they didn't know why, and in the end they just rang the manufacturers, who said it's simple, you know, why they hadn't done that in months goodness knows. But now we're sorted we go to, a sleep clinic and a different hospital for the respiratory consultant, and there is a, a problem I think with, you know, sort of seamless care. But at the sleep clinic they download from a plastic chip in the card in the machine all the information every three months, how well it's working and if it's leaking and what's going on here. And they actually, for us, because we like information, have given us the greatest information and understanding. When we saw them last they said, 'We can tell you the machine is doing ninety-five percent of the work for you at night'. So we know that without it Bill wouldn't be alive, and they told us, because well then you tend to ask, 'Well so what next?' 'Because you don't know, I mean do they ramp up the pressures? Do they reset the machine? What happens here? And they said, 'Well no basically.' They said the most startling thing actually, when we're asleep at night all our muscles are paralysed, all our motor muscles except our diaphragm that we need to breathe, except in Bill's case of course it's packing up. And that's to protect us from things we may do in an, in, in our dreams or extreme things that we may encounter in our dreams, which is extraordinary'

Yes.

'actually.

It is.

So we had this amazing piece of information. We knew the machine was doing most of the work and the answer to the question about, 'Well what next?' was, 'Well when you are dependent fifteen, sixteen hours a day or using it that much,' because Bill will use in the afternoon for a couple of hours and will use it sort of about twelve hours a day now, 'we say you're ventilator dependent at fifteen, sixteen hours and we'll issue you with a backup machine so that you can have it, you know, in case the other one goes wrong or something like this. And basically from there on you are making choices about, about the quality of your life and, you know, the end of your journey with MND,' which was a bit emotional but was the most helpful thing.

The option of full or invasive ventilation raises further issues. When invasive ventilation is used, the person's breathing is taken over altogether by a tube which is passed through a hole (tracheotomy or tracheostomy) directly into the person's windpipe (trachea). One man explained that he had discussed with his wife beforehand what interventions she would consider and where she would draw her 'line in the sand'. Having a PEG was already close to this line for her, but invasive ventilation would have been against her wishes. 

 

As Teresa became weaker, a PEG was suggested. She developed breathing difficulties and had non...

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Age at interview: 59
Sex: Male
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Unfortunately by this time we're talking approaching Christmas of 2005 and Teresa's condition had deteriorated quite significantly. She was having difficulties, severe difficulties, with swallowing and the hospital were saying to her that she should be having a PEG tube fitted. She wasn't terribly keen on that idea but was allowing the family to persuade her to have it done because we wanted her round for as long as possible. And she said that she would like to get Christmas over and then, OK, she consented that she would have it done. During Christmas she appeared to develop a chest infection which is obviously the thing that we were all most concerned about, the thing which is most dangerous to people with MND particularly ALS or bulbar onset. Shortly after Christmas she was admitted to hospital as a result of the chest infection but with the use of antibiotics and intensive physiotherapy and oxygen she was able to be discharged.

She was discharged less than a week and whether it was another infection or if it was the same infection returning we don't actually know but was then re-admitted again to hospital, not the same hospital as an emergency with breathing difficulties. We did while she was in the hospital this second time in January look at the feasibility of a transfer to the hospital that would do a PEG tube. The reason that we were looking at that was because obviously the primary concern was fighting the chest infection but while Teresa was fighting this chest infection she wasn't taking nutrition. And for somebody who was only seven or eight stone can't afford to lose much and she was clearly losing weight rapidly. With everything going on obviously she probably didn't have much of an appetite.

Eventually the hospital did start to feed her intravenously. Her breathing difficulties got worse and worse. And we went from oxygen to positive pressure ventilation and the use of a, I believe it's called a Nippy Unit, which initially I understood was usually used only at night. But within days she was virtually living in it and still having considerable difficulties in clearing her chest and needed almost constant physiotherapy in order to breathe.

We had at times, at the time when she could communicate effectively I had discussed with her where she herself wanted to draw the line. We did not want to do, embark on any procedure that she, herself, I knew did not wish to consent to. And reinforce that, to, with Teresa, with Teresa had actually given me her power of attorney, an enduring power of attorney so that I could obviously speak for her authoritatively with the healthcare professionals. Her dividing line, the PEG tube was close to it but she had indicated that quite definitely she did not want invasive ventilation. That was, that was her definite line in the sand.

It actually wasn't discussed as an option at the hospital. They were using the positive pressure ventilator and high levels of oxygen. And perhaps they felt that invasive ventilation wouldn't have given any improvement over what they were already doing anyway. I suspect that was the case. And that was the situation when Teresa finally passed away on the 31st of January 2006.

People's views about what care they wanted in future and the use of an Advance Decision to Refuse Treatment (ADRT) form (previously known as a Living Will) are explored further in 'Hospices, respite and thoughts about future care'.

Further information sources:

  • The MND Association produces information sheets to help, such as ’7A: Swallowing difficulties', 7B: Tube feeding' and a guide called Eating and drinking with motor neurone disease, with information and easy-swallow recipes. They have a range of Information sheets (8A-8E) on breathing and ventilation. Their resources can all be downloaded from their website. 
  • The MND Association also hosts an online forum where people affected by MND can share experiences.
  • The National Institute for Health and Clinical Excellence (NICE) has produced Motor neurone disease: assessment and management (NG42). Which includesa clinical guideline on the care and management of MND. The guideline includes recommendations for regular assessment of respiratory function for people with MND (including at or shortly after diagnosis and then every 2-3 months) and urgent referral to respiratory services if problems are identified.
  • The Build-UK discussion forum has a number of articles about ventilation.
  • The MND Association provides the ‘MND Just in Case Kit'. The kit consists of a small box supplied to a named individual through their GP. The GP should prescribe medication to fill the box that can be used to give immediate relief in the case of breathing difficulties, choking or related panic. Leaflets are included that give information and instructions for family carers and health professionals. Most people will never need to use the kit, but just having it readily available at home can help give reassurance and confidence.
     
Last reviewed August 2017.
​Last update August 2017.


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