14 Apr, 2020

Motor Neurone Disease / ALS, so don’t read if you’re squeamish ….

The title says it all, so if you’re not easy with this topic come back tomorrow.

Anyone reading here might already have expertise in or experience of the MND/ALS world; others might have browsed the internet.  Some could be scientists or medics, some might already know a sufferer, others might have read up on Stephen Hawking, probably the most famous worldwide, or they’ve checked it out because their friend David Peace has it.

What follows you might know already, but these are my own observations as a non-scientist/medic and as someone who had never had any contact with it until nine months ago,  It’s my layman’s take on it, with extra curiosity because I actually have it.  This is the horse’s mouth.  So I’ll say or neigh it my way.

First, how do you spell it?  Some favour ‘neuron’, others ‘neurone’.  As a non-practising Classicist but with a good memory (and an Ancient Greek lexicon) I fancy ‘neuron’ because that’s how the Greeks spelled it.  No doubt you’ll agree, having read your Homer in the original.  The MND Association in UK and its counterpart in America add the ‘e’ however, so for this blog ‘neurone’ it is.

What’s ALS?  Amyotrophic Lateral Sclerosis. No need for the detailed etymology (but thank you, lexicon).  To all intents and purposes it’s the same as MND, and very many countries around the world know it as ALS.

Every movement we make – the raising of a finger, the movement of a leg, breathing, talking – happens because the brain sends instructions to the neurones somewhere in the spinal cord; and the neurones then follow orders, jump into action, and move the relevant muscles.  As I type now, the brain, such as it is, is telling the neurones to make the hand and finger muscles do as they’re told; and they are.

No one seems to know why the system breaks down  For those with MND/ALS  the grey cells continue to churn out their orders but the neurones in one part of the body decide to have a rest and then ‘progressively’ go into permanent retirement. The word gets round; the next set of neurones do the same; and then onwards throughout the body. As the muscles slow down through lack of exercise, they atrophy and then stop completely.   Paralysis sets in and spreads throughout the whole body.

I asked my consultant neurologist whether it’s always contiguous, i.e. if it starts in a foot does it move up through the neighbouring sets of muscle such as the legs, and onwards, or can it jump to muscles further away? She suggested that it’s normally contiguous but not in every case.

The forecast for someone with the bulbar version – starting with the mouth and throat muscles – is as follows: little by little, though no one can predict how fast (a) the voice will weaken and disappear, then (b) swallowing will become more and more difficult and will stop, then (c) the lungs are likely to lose their power, so breathing unaided will be impossible; and so onward throughout the whole body.

There is no prevention, no treatment and no cure. Worldwide only palliative care is available, to ease the effects. Britain has approved just one drug, which seems to extend life expectancy by 3 months. Looking at it from the bulbar perspective the palliative measures are: (a) ‘bank’ the voice by recording it on special software and then, when the sufferer can’t speak, reproduce it by typing; (b) have a peg (percutaneous endoscopic gastrostomy) tube permanently in the stomach to allow feeding, hydration and nutrition when swallowing isn’t possible, and (c) have non-invasive and then invasive ventilation to keep the lungs inflating. Recent news about treatment for the coronavirus has shown how very unpleasant the invasive mechanism is, involving a head harness and a pipe down the throat. And for MND/ALS the measures are permanent. The paralysis then moves though the rest of the body.

Research?  Organisations around the world have been looking into it for years. A major problem is cost, because so few get the illness.  In any 100,000 people there are around 440 new cases of cancer each year. In the same group there are just two cases of MND/ALS.  So in that group, after say 10 years, 4,400 people or their governments will be buying the products of very expensive research.  Is it worth the pharmaceutical companies investing in research for just 20 people?  That isn’t a bitter comment by the way, it’s just an observation.  If I were Chief Exec I’d want to plough money into the most profitable area of research. Someone told me a while ago that the answers to MND/ALS were more likely to come from university research groups round the world, where the profit motive is probably less.

That was a rather longer summary than I intended. Sorry about that but I hope it puts it in context, and at least there’ll be no need to do it again.

How am I doing with the illness?  I’m OK and coping but let’s leave that for a few days. These are weighty matters and there’s no reason for this blog to be unduly weighted down.  Not sure what to move onto next but am hoping the Muse will sit on my shoulder shortly. Or a nice glass of Merlot could do the trick.

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