Inherited motor neurone disease (MND)

That’s really the way I’m tackling this, is to try and stay one or two steps ahead of the game. If you don’t do that, you inevitably run into crises. I heard about one friend who was stuck upstairs for so many weeks because they hadn’t started the lift early enough. And on the forum, you constantly read about people who run into roadblocks, perhaps through denial, not wanting to face up to the disease. When you’ve had a mother go through it, you can’t really hide. There’s no point in trying to deny it. Quite the opposite.

I would imagine, well I know, there is more available now than there was 50 years ago. We lived in a fairly rural location when I was in my teenage years, and we do now, but I could pull in support from outside, as in from social services, if they’ve got any money left, or from the county council, and I don’t want it to fall all on my family to care for me. I had that and it drained me as a teenager, it really did. Physically and emotionally just drained me for five years.

From your perspective, what was it like, you know, having other people come in to do care instead of your husband?

Fantastic. Because I could ask them to do whatever I liked, and I didn't feel guilty about it. I didn't feel guilty that he's got to do it all day. So I remember the time when we first got the carer in to help me in the mornings with showering and dressing, and it was fantastic. It was such a huge relief. Because we were finding that my husband would be helping me dress and shower and he wouldn't be dressed and showered himself till 11 in the morning. And then that would bother him, and I'd feel guilty about that. And that's, just the start of your day, you know. You've got all sorts of other challenges to come. As soon as we had my lady to come in in the mornings, [husband] was totally freed up. And I would come out in the morning, dressed, ready - ching - and we could have breakfast together and start the day, you know, afresh. And then the challenges that we faced were manageable through the day. You didn't start the day with the biggest task. So that was nothing but positive, having care.

So it hasn't bothered you having somebody else doing the personal stuff?

No. I've, I quickly adapted to it, I think because by the time I took that on, we took that help on, I was really, [husband] and I were really at each other and were really ready to kill each other, you know [laughs]. So I was just glad for someone else to help and take that tension out, so that my husband wasn't my carer. And so it was nothing but a relief. The same, I remember the same feeling when I first used a wheelchair.

We used to live in a house about 800 metres from the beach and it was a nice ten-minute walk. And we used to like to walk down and sit on the beach or go for a coffee at the cafe or whatever. And when my, when my walking became difficult, we couldn't do that anymore. And I used to - you know, we could drive down - but I used to miss being able to walk down. So as soon as we got a wheelchair, “Great, we can walk down again, and we can walk along the promenade.” And it allowed me and freed me up to do things that I hadn't been able to do. So I think because we'd left it relatively late, till things were getting frustrating, then actually it didn't become, it wasn't a big deal, whereas I know other people struggle with going into a wheelchair. But it gave me freedom in some ways. So, yeah, it's interesting.

That's interesting, it is interesting. And I guess, I mean in a way sort of having your husband do all the personal care actually becomes quite unromantic in a way.

Yes, yeah. And it wasn't that he wasn't good at caring for me. He was very good. But I didn't want him doing everything. And I'd feel guilty for that. And, you know, I'd ask him to keep the shower on while he was washing me, because otherwise I'd get cold. No, it was a waste of water. Well, as soon as you get a carer in you can have it how you like, you know. So you don't get any backchat, which when you've got people close to you is harder [laughs]. And I'm finding that now as well where I've got a lot of support from our family here - phenomenal. I don't want to ask them to clean my bathroom or tidy my lounge or, you know, clean the kitchen. I don't feel comfortable asking people who are giving up their time. And I - but yet I'm quite happy to ask things of people that I'm paying, or that that is exactly what they're there for. It makes me, I feel like I'm much more independent, and that they really are an extension of my arms and I can tell them exactly how I want it. Whereas if I ask my, my relatives to clean the bathroom, and they don’t do it how I want it, then I would struggle with that.

Yeah, yeah.

And that would frustrate me as much as it not being done. So, yeah, it’s actually, I’ve actually found it good. What I haven’t found good with care is where the people change all the time. I find that really, really hard. And that one challenge has been probably the biggest challenge for me, moving back to the UK and setting up with new carers is, I’ve set up through an agency to start with and I didn’t have a key worker. And I think I counted that in the first two months of being here I’d met twenty carers. And that is really dull, telling people how you want things all the time. And, “Not like that or like that” and, yeah, really quite tiring. When I have carers in, I just want to babble on. And so that the fact that I’m being washed and dressed becomes irrelevant. You don’t even really think about that, to the point where you think, “Did I, did they put any deodorant on me? I can’t really remember”, because you were gassing on about something. And, but when it’s a changeover of people all the time, I find that hard.

You said earlier about your partner providing a lot of care for you.

Yes, and she was keen to do it. But it’s too much for one person, and I was very mindful of my sister’s experience caring for my mother, so we now have two local people who come and help. One of them comes in every weekday morning and helps dress me and sort things around the house. The other does three days – three evenings a week, doing the bedtime routine. All this takes pressure off my partner. We are now looking at employing a home care company to beef up arrangements, initially at weekends. I expect as I get less, less able to help, we’ll need to do more of that. That’s when I expect my main financial outlay to start. And depending on how long I last, I may eat up my capital, and if I do, I get equity release on this house. So I’m pretty sure I can fund my own care for as long as I’m likely to live. 

I think a point that I would make is that as I mentioned to you before it’s not the doctors that really make a difference to my life with MND. It really is the therapists. So it really is the OTs, primarily for me the OTs, and now the carers, really. And it’s those hands-on people that day to day make my life easier and things more accessible. The doctors aren’t - it’s not that they’re no use to me - but they’re not crucial in this illness at all. But the OTs and having a good OT and having a good relationship with them, really, I’ve been really lucky with the two OTs I’ve had. Having a good relationship with them is really good. And it feels like, they’ve both made me feel like they’re only working for me. And I can ask them for anything and they’ll do their best to get it for me. And that I can try anything as many times or, you know, and that’s really great. Because they are, little things do really make a difference and open doors for you, literally, and make your life easier. And come up with suggestions. It’s very difficult for them because they need to suggest things to me equipment-wise before I might want to face it, but before I’m struggling. So it’s not easy for - I don’t envy their position, it’s not very easy. But having the guts to say, you know, “Have you thought about…?” certainly when I was first struggling with walking, “Have you thought about a frame? You know, that would allow you to get over being able to take a drink through to the balcony.” I would have resisted that at first. But then it’s plant a seed and then, and then, you know, “Oh, okay.” Because otherwise you don’t know what’s out there. And so, and they’re really that link. So you might be struggling on with something, yet they could just say, “Oh, why are you struggling? You could, you know, we’ve got this little gadget or that little gadget.” And things, equipment does make a difference.

But it was only in the August about five months after I’d been diagnosed, that they referred me to an assisted ventilation consultant who put me on to a ventilator, a non-invasive ventilator. That totally transformed things, more or less immediately. Within a couple of days, my appetite came back, I started putting weight back on again, my energy levels increased. Yeah, it was like being born again, it was wonderful.

And… people have often asked me, “Do you feel angry about my condition?” telling me it’s natural to be angry. Like, I almost felt it was abnormal not to feel angry about my condition, but the one thing I guess I have felt angry about is that my MND consultant didn’t pick up on my, you know, on how useful ventilation could be for me. I remember talking to the assisted ventilation consultant about my symptoms and when I mentioned my difficulty in swimming, which at that time had started the year before, he said, “Oh, that’s an absolutely classic sign of it affecting the breathing muscles, the diaphragm.” So it seems that although obviously in the MND centre, they’d been testing my peak flow and other breathing parameters, but they hadn’t been such as to kind of trigger the consultant’s thoughts that I needed to go on to ventilation. Then, yeah… Well, I understand it’s relatively unusual for one of your presenting symptoms to be an effect on the chest muscles, but in my case it clearly was and had been from very, very early on. So I guess I kind of feel disappointed that I kind of lost that summer of 2019.

I mean, I can remember her saying to me as a side issue, I get a lot of pain because the other side of the family, basically we’ve got Marfan Syndrome, the other side of the family. My dad was 6ft7 and I’m very tall, I have the joint hypermobility as my siblings do. So, if and subsequently, both my father and my brother have died of a ruptured aortic aneurism, so it’s definitely Marfan Syndrome. So you add in the joint hypermobility and the muscle weakness that goes with Marfan’s and MND, which means you’re losing muscle so you’re putting more strain on the muscles that you’ve got, and that adds up to pain. So I began to try to talk to her about the pain I was in and she just said, “MND is not painful.” And I’m sorry, that is again, that’s blinkers on. That’s going, “MND affects motor neurones only; it doesn’t affect sensory neurones.” That’s what she was saying.

But subsequently, I know, you know, several people with MND who I’ve met through the association who have a lot of pain. Because, you know, as you’re losing muscle, you’re sitting maybe in a lopsided position or something, you know, your head’s not on straight and it can be painful. And that’s without the joint hypermobility and tall stature that goes with Marfan’s. And she just sat there and said, “No, you don’t get… it’s nothing to do with it.” How can you say that to somebody? You’ve got to look at the whole patient.

Yeah, so basically I filled in an Expression of Wish form which says that, you know, if I do catch Covid, then I don’t want to be admitted to hospital unless there’s a very good chance of my surviving and pulling through. I talked about it with the consultant beforehand, and he – and I guess this was very early on in the pandemic, back in March, before we knew quite so much - but he felt my best chances if I developed Covid were probably, you know, basically to put my ventilator up to the maximum settings and just hope for the best.

What I didn’t want to do was kind of end up in hospital on, you know, for weeks without the ability to kind of say goodbye to my own family, and with no good prospect of coming through. You know, I’d rather if I was going to die, I’d rather do it at home… Luckily, I haven’t needed to act on that so far, but I’m still inclined to respond in the same way. I don’t, you know, the thought of being in hospital with sudden massive progressive organ failure with a cytokine storm, lots of invasive procedures with a relatively low chance of success, it doesn’t appeal to me in the slightest. I’d rather go, if I have to go a few months or a year or two earlier than I’d like to, then I’m willing to kind of take that.

So, you’ve done some kind of thinking around the future and your wishes…

Yeah.

For that kind of thing, yeah.

Yeah. As I say, it’s documented and I sort of keep that in an envelope by my bed, by the ventilator, along with the Do Not Resuscitate forms, so that the medical profession are aware of my wishes. I’ve discussed it also with my brother and my daughter, who are my closest - they’re the kind of nominated family members that the medical profession go to for guidance on this kind of thing, if I was unable to express my own wishes anymore.

We were talking a bit about planning for the future, and it sounds like you’ve done quite a lot of practical stuff already.

Yes, I was talking about a DNR and… really, for the… because of my knowledge through intensive care, so I know at some point I won’t be swallowing very well, I’ll be aspirating secretions, I may be choking on my food, I don’t want to be ventilated because I know that I’ll be even weaker after that, and really, people in intensive care as an adult should be the ones that are going to get out of intensive care. And if you can’t do all the things you want to do, such as walking, making cakes, cleaning, going out, then life is very different.