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Points for Reflection

Read Eleanor and Alanís Stories. Both have said how they are approached by professionals wanting to learn more about CMT.

Activities

In your clinical practice, what sort of questions might you ask to patients and their families to learn more about a specific condition?

Quotes

"and I was thinking - does it actually matter that itís progressive? Am I going to finish up being a nurse? And I thought well if it does, it does. If it doesnít, it doesnít. Iím quite happy to take that on."

"because itís a progressive condition, you realise something you could do - you can no longer do, but you canít actually say the point in time when you actually discovered or you actually decided you couldnít do it."

"And thatís my quandary as a companion and a carer. Trying to do the best and I never get it right. Thatís the point and there is no way that as a carer you can ever get it 100 per cent right. You are either doing too much or not enough"

Further information

CMT UK

US National Library of Medicine, Genetics Home Reference - Charcot-Marie-Tooth


Location: The Stories
Transcript

You can download the full transcript here

Living with Charcot-Marie Tooth: A husbandís tale
Supporting a wife with an autosomal dominant condition Ė Alanís story

I just want to start off the interview by asking you to tell me a little bit about the condition in your family.

Well Eleanor is my connection with the condition and I knew nothing whatsoever about it before I met her. And when I met her it didnít occur to me initially there was quotes "anything wrong".

She was a bubbly personality but as I got to know her better we talked about things and she said what the problem was and the fact it was genetic. But it didnít really impact in any deep way until we decided that we perhaps ought to get married. And Eleanor said: ĎWell you do appreciate that the condition is a progressive condition.í And I can still recall to this day - we were on holiday in Scotland with a large group of people. We were a mixed group and Eleanor was going off somewhere with somebody and I was going walking with some of the restÖ And I can remember it was pouring with rain, and I was thinking - does it actually matter that itís progressive? Am I going to finish up being a nurse? And I thought well if it does, it does. If it doesnít, it doesnít. Iím quite happy to take that on. So I went back and I told Eleanor and we thought thatís it then and we announced our engagement. Thatís the way it actually worked.

And it was one of those things that I can see the progression over the years. But I think Eleanor didnít actually raise the point, but itís a case of suddenly, because itís a progressive condition, you realise something you could do - you can no longer do, but you canít actually say the point in time when you actually discovered or you actually decided you couldnít do it.

You suddenly realise later that you have stopped doing it and found an alternative way. It does make it very difficult in terms of me not interfering. One shouldnít interfere but itís very difficult not to, but I can see an accident is going to come and so I say ĎWell let me do thatí - and thatís a point of friction at that point! Itís inevitable it will be and we try not to let it grind us down but it does happen. In the sense that I can see that accident about to happen and then you have to turn around and say well should I in fact let it happen and then Eleanor will realise that is something she canít do.

And thatís my quandary as a companion and a carer. Trying to do the best and I never get it right. Thatís the point and there is no way that as a carer you can ever get it 100 per cent right. You are either doing too much or not enough and that doesnít mean to say that the carer and the person being cared for will actually agree on whatís enough you know and where the changeover should come because it doesnít work that way because people are different.

Tell me now a bit about your role or your perceived role as the carer and the companion.

This is the way I perceive it. I am there to support when the support is required and to give the sort of support that is required. Again, there is the question of what that support is and who should determine the support and should it be the carer or the person being cared for. Do I wait until I am called or do I interfere and I say it that way because thatís the way naturally the other person will see it and I try and avoid it but I never get - I get it right sometimes I think. But there is no way I can ever get it right all the time.

Basically because we are different personalities but that care ranges from just assisting with opening things right through to - particularly at the moment - now Eleanor has had a spinal operation, to assisting her with the bath and particularly washing her feet because with a back operation she canít bend at all and that makes it you know fine, thereís no problem. Thatís the range of care at the moment. How far that care will have to go in the future, nobody knows. Because being a progressive condition and Iíve seen progression in terms of a little bit at a time and then almost a jump.

Eleanor was saying earlier that she doesnít walk very far. In my life with Eleanor we have walked 3 miles on ice one New Year in Scotland, but that couldnít happen today. And so it means that the wheelchair is there in the car all the time so that we hopefully are not restricted where we go and what we can do. Clearly we have to be. You canít take a wheelchair up a mountainÖ that sort of thing.

We have been on holiday this year to Australia and Eleanor coped admirably with the wheelchair because everybody was as helpful as they as they possibly could. But there were clearly things that we perhaps might have been able to do in the past but there was no way we could do them now. But thatís the way things happen and thatís what I accepted when I said Ďyes I am prepared to marry you even though you haveÖ and I say, even though not despite.í And I make that very clear it is even though there is a progressive condition that we donít know how rapidly it would have progressed.

What has been your experience of the care you have had from the NHS in supporting Eleanor?

Generally quite good. In the initial stages when she first moved down as she said, getting into the system was appalling, particularly the fact that because itís progressive and in the early days of our marriage it was very slowly progressive and therefore there was no need for any regular attendance. So back to the bottom of the waiting list because it is more than a year since you have seen a neurologist.

Since she had a row with the Health Authority and I think got the impression that the neurologist was quite happy to provide an annual examination, it was just a fact that resources were against him but the authority having stepped in - heís quite happy to do it. But more importantly, itís a case that if you are on an annual appointment, if you need more than an annual or you needed to see somebody fairly urgently, you can ring up and theyíll put you in. Not necessarily the same day, but in terms of Eleanorís back operation and the problems she was having with the pain in her legs they thought it was perhaps CMT orientated so she rang the neurologist and he saw her what, three or four weeks after that point in time with a cancellation of an appointment, and diagnosed the problem which wasnít CMT related. And then of course the problem starts that youíve got to go down the whole system to get that particular condition dealt with by other specialisms within the health service. It can vary. The first thing was that the neurologist said you need an MRI scan and thatís months and months and months waiting list.

But this is where Eleanor has the benefit. She has worked in the health service. She knows, not by-pass the system, how to use the system so she immediately rang up the MRI people and said: ĎI can take a cancellationí. And in fact she got a phone call 4 oíclock in the afternoon Ė ĎCan you be here by 8 oíclock tomorrow morning.í And thatís the sort of thing, fairly soon after that phone call. But itís because Eleanor is in a position to use the system - knowing the system. But apart from that there have been no real problems with the service in terms of the CMT.

What about other professionals like the nursing profession?

In terms of Eleanorís needs, the nursing profession doesnít actually come into the picture very often because itís not a condition that requires regular treatment in hospital or anything like that. But the nursing profession when she has had to go into hospital for perhaps other things not connected with the CMTÖ she says theyíve all been very keen to find out. Thereís no question of that in my mind. You know sheís forgotten to take leaflets and therefore she has really regretted not doing that because there was clearly a demand.

What about genetic nurses? Do you have any contact with them?

I had no connection at all that I can recall with any genetic nurses. The one question where the Health Service did come into its own in terms of the CMT was a registrar in the neurology department at one of the regular annual appointments and Eleanor was saying she gets a thing called restless legs. And a lot of people with CMT suffer from restless legs and this registrar suggested trying a particular drug which was designed for Parkinson disease and so Eleanor tried that and it worked wonders. She canít not take it otherwise she canít sleep at night because her legs are so restless and thatís where the Health Service comes into its own. But then, that was prompted by the individual in the Health Service, not by us.

In terms of as Eleanor said, OT and physiotherapy when sheís needed it - itís been there. Perhaps talking at the meeting a couple of weeks back, the CMT regional meeting, it was quite clear that in this health authority, the service was far better than it was within (another). Because there have been no problems and as Eleanor said wanting some equipment, itís not very expensive equipment that weíve got but its no problem and its done very, very promptly. Handles in the loo, handles on the back door because weíve got a single step and that sort of thing.

If there was anything that you wanted in terms of improving care for yourself and your wife what suggestions would you be able to make? If there was any one thing that you wanted.

At this point in time I donít think there is. We live in a bungalow which we had when we first moved in. We had the loft converted into a room and Eleanor canít get up the stairs. But it is only one room and I use it as an office and a junk room more than anything else and morally thereís no way I would consider saying put a stair lift in so that Eleanor can go up there because there is no need. That seemed to me to be an abuse of the resources. If there were plenty of stair lifts going spare that would be different but as far as I am aware money is very, very tight with all health authorities and therefore there is no need for that sort of thing.

Is there any other point you would like to make that you think could be really important to our project?

I think the one thing I would always say to any health professional in terms of anybody with a neurological condition like Eleanor has got is - donít hide behind that condition. They donít say that everything that youíve got wrong with you must be your CMT. There is an element of that goes on with whatever a condition an individual has. Itís a case of people say Ė ĎWell thatís your CMT. Go away.í And because they have said it effectively, ĎGo away and get on with your CMT.í They say - ĎGo away and get on with whatever else it is.í Well it could well be totally treatable and Eleanor is a good example of that. She has a heart condition which is being treated and doesnít create any problems, but there again we went to see the heart consultant and he said Iíve got a student. ĎIíd like the student to see your CMT not your heart.í

The profession has shown a willingness to learn that from what Eleanor has told me about the earlier experiences in her lifeÖ Wasnít there then, so I think there has been a real change in the attitude of the profession to people with long term genetic conditions that are affecting their life.

Any other points?

Canít think of any but I think that was fairly important one that the professions must recognise that it is not just a one condition that a person can have. You can have several at one time unfortunately.