Give us another update, someone asked. So here it is.
Mouth and throat muscles almost completely paralysed. Thin liquids and solid foods are impossible to consume: liquids flow anywhere without control, and solid food, also uncontrolled, is rejected by the throat and can slide into the trachea and choke me. The local supermarket is rather missing my old shopping list!
Main problem is the tongue, which has very limited movement. It therefore (a) stops me making many of the sounds needed in order to speak (hence very depleted speech production), and (b) doesn’t direct food to where I can chew and then swallow (hence a serious risk of choking), and (c) can’t move thin liquids to the right areas (so they can also choke me), and (d) doesn’t automatically direct saliva to where it can be swallowed (hence continual build-up of saliva in the mouth, and loss of control). Worse, regrettably, is that even if I wanted to I couldn’t stick my tongue out at idiots ….
Lips are still sensitive to touch but muscle strength has completely disappeared, so they cannot hold anything in the mouth. Result: anything that goes in comes out, and saliva comes out. For the saliva there’s a cloth always to hand, and for the rest see the two paras below.
How to deal with these? All food is puréed so that there are no solids that could choke me. Even puréed food can slide down the trachea and cause choking (and frankly there have been a few times when that’s been serious) but so far the limited tongue movement has generally been able to direct it to the right area. All liquids are mixed with a special thickening powder so that the tongue, again with its limited movement, can get it to where it can be swallowed safely. But again there have been some instances of choking, fortunately cleared in time. So, a thinning of food and a thickening of liquid, and that works so far.
To keep the puréed food in I eat with a teaspoon, head right back, gazing at the ceiling so that gravity keeps it in the mouth, otherwise the lips would let it out. Then I manoeuvre it for a bit of chewing and then little by little swallow it. I’m now an expert in the details of my ceiling, and one day I’m going to zap that damned cobweb!
Food: a selection of supermarket items, combined and then mashed or power-mixed to a puréed consistency: and frozen puréed dinners are delivered by a special contractor.
Liquid: the thickening powders do work well. To my surprise they also work with lager – a lot of foam that then settles, but the taste is the same and also the enjoyment. Haven’t tried it with wine yet. Perish the thought.
Saliva: to try to dry the mouth, every day I take a prescribed liquid and every three days I change a special plaster behind an ear. They’re both supposed to help, and they do, but it’s still very troublesome at times. The specialist told me it’s the Pavlov thing: when we think of food or drink that’s the trigger for saliva to increase; and the mouth automatically deals with it. You don’t notice but I do because my mouth doesn’t deal with it. By exception, Pavlov proved!
Lungs mostly OK. Still this strange phenomenon: I can walk for a few miles in silence but if I talk I’m immediately out of breath. It’s a great way to shut people up – the sympathy card.
Weight: in the last 18 months down from 90 kilos to 78. That’s still more than 12 stone, perfectly acceptable for someone of my height and age, and it seems to have stabilised so I’m not worried.
Feet and ankles: anyone reading the 7 August blog entry will know that the frozen toes and ankles aren’t linked to MND/ALS. It’s something similar to Housemaid’s Knee. Last week a physio visited and recommended a programme of exercises, so hopefully that problem should disappear in time. Meanwhile, a gait not dissimilar to Charlie Chaplin’s.
Voice: a growl that goes lower and lower throughout the day. An hour’s conversation can reduce it to almost zero. Another great way of bringing talking to an end, with me announcing we have to stop and then watching carefully for expressions of pleasure and relief.
That’s about it, as a summary.
The PEG. Again, the 7 August entry said I’d agreed to have the PEG operation – the pipe in the stomach. The National Hospital for Neurology and Neurosurgery which is looking after me has given a provisional date of 7 September for the operation. Apparently I’ll be admitted and prepared for battle on the 6th, then on the 7th I’ll be taken to UCLH (University College London Hospital) for the operation itself, and then taken back to the National for 2 or 3 days recuperation. The dates are not confirmed yet. They could easily be delayed. All being well though, after the operation all food and liquid will go in via the tube, and the effort and risk of eating and drinking by mouth will have disappeared, together with the pleasure of taste. ‘Nil by mouth’ will be the norm, but I’ll definitely be adding ‘except thickened lager’.
At the same time in hospital the plan is to have botox injections into the salivary glands. Apparently that could reduce the saliva flow for quite a long time. I’ll be boasting botox thereafter.
Am I looking forward to these? No. But if the result is that the current effort and risk of eating and drinking are at an end, then perhaps life will be a little easier.
Covid 19. There’s been a lot of news recently about the effect this pandemic has had on cancer treatment and other illnesses. Specialists are reporting that large numbers of sufferers have had treatment delayed for long periods, and that research has been disrupted in order to focus on the virus. Regrettably the same is true for MND/ALS sufferers, mainly in terms of research. From a national newspaper:
“Research on motor neurone disease has also been seriously disrupted. A Europe-wide survey to determine if the drug Tudca could halt the progression of the disease – which affects about 5,000 people in the UK – had to be suspended in March and efforts are still being made to restart it at the five British centres involved in the study.
“Around 50% of people with motor neurone disease will die within 18 months of their first diagnosis,” said Brian Dickie, the Motor Neurone Disease Association’s director of research development. “These individuals are rapidly running out of time, so suspending projects like these is utterly heart-breaking.”
This is a terrible pity. Months ago I offered to be used in research. The particular project was to look at the 10% of sufferers who might have a genetic link, but they then found that I didn’t have such a link. I’ve registered for other projects but haven’t been called up yet. Covid probably intervened.
Frankly I’ve never held up great hopes of a cure or a medicine to halt the illness during my lifetime. Even in normal times the figures are bleak. In every 100,000 people around 440 will be diagnosed with cancer each year. In the same 100,000 just two people will be diagnosed with MND/ALS. Why would a pharmaceutical company invest large sums in MND research for so few sufferers, when the profits from a cancer breakthrough will be so huge? A university research establishment might be more interested in MND/ALS, but their resources could be far smaller.
We are where we are, though. And we make the best of it. If my operation dates change I’ll let everyone know. So from a housemaid proving Pavlov and anticipating botox, over and out for now.