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In January I referred to the ALS Functional Rating Scale (Revised) which is useful in tracking the progress of MND/ ALS. In mid 2020, before I had the diagnosis of MND, I reckon my score would have been about 44.  In July 2021 my score was 27.  By January 2022 it was 15.  Now it’s only 6.

That’s a dramatic reduction in my functional abilities; after all, at Christmas time I was able to eat and drink fairly normally.  Yet by March I was effectively ’nil by mouth’.  My left leg is nearly immobile, and my right leg is becoming weak – I can take tiny, slow steps if Sue holds both my hands to provide support.  My arms and fingers are totally immobile – my thumbs are the only digits I can move.  This means that I need help with absolutely everything, from using the toilet to scratching an itch on my nose. 

Sadly, I can no longer have showers because I’m far too unsteady and I get dreadfully breathless, so it’s bed baths only now with hair washing achieved by leaning over the kitchen sink with jugs of warm water.  Just like my mum used to do it for me in my early childhood in the 1950s.

I am now using my ventilator for around 23 hours per day. Without this non-invasive ventilation (NIV) I get very breathless and distressed, particularly when lying down or standing up.  The only time I don’t need NIV is when sitting up in bed immediately after waking up.  Wearing the face mask all the time destroys any last chances of communication. It’s also uncomfortable and can fill with secretions from my mouth.  I do feel a bit self-conscious wearing the face mask when we go out for wheelchair walks.  I do look rather freakish and can frighten young children, and dogs with a nervous temperament.

However, alongside the problems of NIV is the opportunity (at the right time) to withdraw my consent to receive the treatment (with the obvious consequence).  I have always been a highly independent perfectionist with a clear preference to be in control, with everything neat and tidy. I sometimes think this personality type (Myers Briggs INTJ) really is least suited to the chaos that MND brings.  There’s potentially an interesting research opportunity here for a psychologist.

I normally talk about places we have visited in my diary posts.  The fact is we haven’t been anywhere of interest in June.  We enjoyed watching the Queen’s Platinum Jubilee events on television, and we had a lovely time with family at my mother in law’s 90th birthday celebration the following weekend.  We also enjoyed numerous visits from family and friends – opportunities to use my TD Pilot for eye gaze communication.

On Saturday 2 July we have the great pleasure of providing the venue for a family pre-wedding garden party for our son Alex and his fiancée.  A large marquee arrives on Friday which will cover the top part of our lawn.  We are getting very excited! I’ve written a short speech for the event on the TD Pilot, which will be interesting technologically.  The words come out in rather a monotonous way, but at least people will hear my voice as banked nearly two years ago.

This month’s title is a line from the Act Two Finale of Gilbert & Sullivan’s ‘The Mikado’. It accurately describes some of our experiences in May.

The highlight of the month was when friends from Abbots Langley Gilbert and Sullivan Society performed a concert for all our close family at home on a sunny Saturday afternoon. It was a joyous occasion with songs from G&S operas, Carousel, Gypsy and Rent. Afterwards we all enjoyed wine and snacks in the garden. I have been a society member for over thirty years, served on the committee for over twenty, and have the honour of being a Vice President. This was a very special day.

Our first merry dance was one afternoon when I slipped forwards and onto my knees when Sue was trying to lift me off the tall Mowbray seat on the toilet. It’s impossible for me to stand up from a kneeling position – my leg muscles are just too weak. It’s also impossible for Sue to lift me up. Sue somehow managed to pull my pants up and we contemplated phoning for emergency assistance. At that moment the doorbell rang. Two carpenters who were coming to give us a quote for a new long ramp in the garden had arrived early. A funny way for them to meet a new customer but they quickly managed to solve the problem and got me onto my feet!  These guys were fantastic and just six days later we had an amazing new ramp outside, thirty feet long, which descends the equivalent of three steep steps. It means I can now explore the whole garden in my power wheelchair. A truly liberating change for the better.

I mentioned that my legs are weakening. My arms and hands are now practically immobile and I am finding it extremely difficult to hold my phone and write this post  with my right thumb. So posts in future will be a lot shorter as I will have to write by selecting one letter at a time using eye gaze.

Another big recent change is that I now need to use my ventilator nearly all the time. In fact, the only time I don’t need it is immediately after waking up in bed for about an hour.  At all other times I feel very short of breath, particularly when standing.  Accompanying the breathlessness is anxiety and mild distress.  Not nice.  However I now have two ventilators, ostensibly one for use as a backup, although we’re starting to use one upstairs and the other downstairs and outside.  I also was issued with a cough assist machine for use when needed.  It rapidly blasts air in as you inhale.  Two seconds later it rapidly sucks air out.  There are two tricks to learn; inhaling and not swallowing the air coming in, and actually attempting to cough on the rapid exhalation.  I’m practising.

Our second merry dance in May was another very uncomfortable night at the caravan.  At 3am we both concluded that we just can’t continue with the severe difficulties of being away from home any more. We are selling up and I have now left our home from home outside Wareham in Dorset by the River Frome.  We have enjoyed eight wonderful summers there and have many cherished memories, but all good things sadly come to an end.

On 3rd April we travelled to our static caravan in Dorset for an overnight stay with a very full vehicle.  The purpose was to do a quick Spring clean and get everything in place for the new season.  The temperature inside a caravan can often go below freezing point during the winter so all food and bedding is best stored at home, and the boiler, cisterns and toilets have to be drained down.  Obviously, I couldn’t contribute much to the work that had to be done and we were grateful for the help volunteered by Sue’s sister.

Night time brought some challenges.  We installed an electric pillow lifter in the bed, a device that emulates an adjustable bed.  That helped.  But to accommodate my ventilator and to give me sufficient space we had to swap sides in bed – after 45 years on the right it’s extremely disorientating to change!  The fundamental problem in bed though was the low height.  Getting out was very difficult indeed and very hard work for both of us.

During the last couple of weeks, I’ve experienced more symptoms of respiratory failure. I find myself being very short of breath when standing up, and when being showered and dressed.  Accompanying the breathlessness is some chest pain (angina) and also overwhelming exhaustion. My feet and hands often have a purple hue, which is a clear sign of lack of oxygen.

I am now tending to go back to sleep for an hour or two each morning, on my ventilator, and using the ventilator again during the afternoon whilst resting and watching TV. I have been prescribed a low dose of Morphine to help my laboured breathing.

I’ve experienced other changes during April.  Excess saliva remains a serious problem, despite two medications and Botox injections into my salivary glands.  My arms are now immobile, which makes daily living very difficult.  My legs are increasingly wobbly, and I feel very dizzy and unsteady when standing.

In the light of all this our routines have changed, and my world has shrunk. Indeed, I don’t now know if I’ll be able to make it back to Dorset again, which is a very sad thought.

We’ve not yet started the care regime I am entitled to. The reason being that we do have established ways of doing things and, whilst Sue is very busy, we jointly think that ‘two’s company, three’s a crowd’ right now.  In any event, with the rate of physical decline I am experiencing speeding up, I think I’m now on a final slippery slope and my remaining life is measurable in weeks and months and certainly not in years.

I’m not sure if it’s the result of medications or my stage of life, but my mind has been very active.  I’ve found it really easy to think of situations and events from 60 years ago and longer.  Before falling asleep I’ve been on several virtual tours of my grandparents’ homes for instance with every detail in really sharp focus.  It’s quite remarkable!  I’ve also been playing events in the future through my mind; but I’m no longer there.  This sounds very macabre but it really isn’t upsetting, and I fully accept that my days are numbered.

This post is a day or two later than planned.  Yesterday I had a big problem with choking and coughing and I was unable to breathe in without my chest rattling badly.  My cough is practically non-existent now, it’s a tiny splutter and I’m not able to clear my airways at all.  Time to be provided with a ‘cough assist’ machine to increase my comfort levels, I think.  More on this next month, I hope

So, I’m now at the point where my palliative care nurse thinks I qualify for NHS Continuing Healthcare – fully funded care for people who meet certain assessed criteria:

• the help needed
• the complexity of needs
• the intensity of needs
• how unpredictable the needs are, including any risks to health if the right care is not provided at the right time

NHS Continuing Healthcare isn’t provided to many people and, as we know from recent national debate, most people will have to pay for their own personal care up to £86,000 under the incoming social care arrangements.

There are two ways in which a qualified health professional can apply for continuing care.  The first route is based on the completion of a checklist, and the number of points scored is added up.  The result is then assessed by the local clinical commissioning group who make the final decision about eligibility.  The second route is a ‘fast track’ available for people who have a terminal condition which is progressing rapidly.  Sadly, I qualify for the fast track.

I’ll be entitled to up to four carer visits a day.  I can also have overnight care on up to three nights per week provided by the Marie Curie organisation.  Sometimes I do wake up and need Sue’s help four or five times per night (to change position, adjust the ventilator face mask, or deal with my dry mouth etc). Overnight care will allow Sue to have some unbroken nights, however it will require a change in sleeping arrangements.  More than that, it will need us both to accept help from other people – something I think Sue will have more difficulty accepting than me.

On 7th March I was provided (at long last) with a TD Pilot iPad Pro based eye gaze system on a trial basis by the NHS Environmental Control team.  I’ve been providing regular feedback, and on 28th March it was confirmed that I will be issued with a new device on a permanent basis.  For an iPad user like me, with files, photos etc on Apple iCloud, this is a perfect solution and I am one of the first people in the country to receive a TD Pilot via the NHS. A great outcome (that demanded a lot of patience!)

We had a lovely family weekend away in March staying at Hanbury Manor hotel near Ware for two nights. It was sunny and dry; the hotel has a fabulous golf course (that none of us used!). Sue and I had to take a huge collection of MND related equipment with us and it’s likely that this will be last such stay away for me.  Instead, we’ll focus on having time away at our static caravan where we will be able to leave some duplicate pieces of MND kit in place and we won’t have to hurry to get into breakfast. It’s 31st March today and as I write it’s snowing outside.  Last week we enjoyed summer-like temperatures and beautiful blue skies.

Stormy February draws to a close with brilliant sunshine!  

On Friday 18th both our house and static caravan were in the red weather warning area for high winds; thankfully we escaped unscathed.  The day before we’d been back to the National Hospital in Queen Square for a routine respiratory consultation. My ‘peak flow’, cough and sniff functions have all deteriorated, but the specialist nurse was happy that overnight use of a ventilator is keeping my oxygenation up and CO2 level down. So that’s positive news.  It’s amazing how they can download data from the modem installed in my ventilator.  They can check the various settings, see the times of use and even the amount of leakage from around the face mask.

The consultant told me that they’ll happily provide a second ventilator as a spare.  This would also allow me to have one upstairs and one downstairs for the times (probably fast approaching) when I’ll need to use the ventilator during the daytime too.  He also emphasised the fact that use of the ventilator is purely voluntary, and I can withdraw my consent at any time.  When I’m much further down the MND track, giving up the ventilator will lead to death in quite a short period of time.  I’m happy having this knowledge, in fact it’s quite reassuring to know that there is an exit route at the right time.

Before my respiratory consultation, I was lucky enough to be able to have a second round of Botox injections delivered by the MND Consultant Nurse, who had rearranged her diary to accommodate me.  Incredible care in such a busy hospital.  Hopefully the injections into my salivary glands will help this time to reduce the continuous problem of excess secretions.  Daytime floods of saliva and at night-time a drought.

To counter the night-time drought, I have now found Oralieve Gel which works well if smeared all around the inside of my mouth.  Last month I mentioned Xylimelts as a good option, but I found they could detach from my gums during the night presenting a bit of a risk of choking.

Two days later, I drove my power wheelchair out of its overnight parking space in the living room. Unfortunately a hook on the back (for a shopping bag) attached itself to the curtain pull cord. As I sped forwards there was an almighty wrenching sound as the curtain rail, complete with curtains, came crashing down behind me. These electric motors develop an incredible amount of torque, and the incident demonstrated yet again how much extra work MND creates!

The NHS Regional Environmental Control Equipment Service, based at Hillingdon Hospital, arranged for my Housemate device to be reprogrammed to enable me to control my rise and recline chair downstairs and my hospital bed in the study. It’s been increasingly difficult to operate the chair recently.

I’m pleased to say that the RECES team is going to be with me when I’m provided with a £7,000 TD Pilot iPad Pro based eye gaze device on 7th March.  Four months after first seeing and trying the device, the day can’t come soon enough!  Thanks to the NHS CASEE and RECES teams for joining together to support me.

On 25th March coincidentally two ramps were installed for me. One allows me to access the patio from our back door in my power wheelchair. The second walking ramp has been built at our static caravan to enable me to get inside now steps are impossible.  Two liberating (and expensive) jobs done.

The most extraordinary thing happened on Sunday.  I was out in my power wheelchair and a car pulled onto the pavement in front of me to stop outside a house, totally blocking my path.  When she realised that she would have to move, the woman who was driving made a very angry face, slammed the car into first gear, and skidded off the pavement in front of me.  As the car landed back in the road there was a nasty sound as the underside of the car scraped along the edge of the pavement.  Luckily for her I can longer speak or gesticulate!

Finally for February, I’ve written a page detailing the steps I’ve taken to plan ahead. Great to get things organised and done. Positive progress.

Planning ahead

It’s a new year and time for a bit of stocktaking.  Where am I?  From the top:

Speech – almost unintelligible. Learning how to use eye gaze equipment

Swallowing – poor… I’m now having scrambled eggs for breakfast and a daily treat like crème brûlée, otherwise, it’s liquid feeds only via my PEG

Saliva management – a big problem. Taking Glycopyrronium Bromide and Amitriptyline. Recently had Botox injections into salivary glands and waiting for this to kick in

Arms – shoulders and elbows no longer work

Hands – wrists are becoming stiff and fingers are beginning to curl.  Can only type using right thumb on phone.  Can’t hold a pen or use a computer keyboard

Breathing – very shallow and I get quite short of breath when lying flat and when trying to talk. Using BiPap ventilator to good effect all night, every night. CO2 level well managed 

Legs – wobbly and heavy, it’s impossible to negotiate steps unaided.  Using power wheelchair outside the house

Overall – constant feeling of heavy fatigue. I’m happy though that I’m dealing with MND in a pragmatic and positive way. I’m not letting it get me down  

Weight – now 73kg (was 88kg when diagnosed in November 2020, 97kg before pneumonia in March 2020) 

Looking for a more objective way of defining my symptoms, a while ago I found the ALS Functional Rating Scale (Revised) online.  Having had a professional career involving the use of psychometrics, I am interested in trying to measure the unmeasurable.  I don’t know if my neurologists use the ALSFRS routinely (I’ll ask them), but NICE refers to it on their website.

Here’s a link to information about the questionnaire: https://en.wikipedia.org/wiki/ALS_Functional_Rating_Scale_-_Revised

Here’s the questionnaire itself: https://www.outcomes-umassmed.org/als/alsscale.aspx

It rates factors such as Speech, Swallowing, Handwriting, Stairs, and so on.  Had I rated myself when I had initial symptoms, but before I had a diagnosis of MND (February 2020) I reckon my score would have been about 44.  In July 2021 my score was 27.  Now it’s 15.  That’s a dramatic reduction in my functional abilities; significantly, it’s a steeper decline than the typical prognosis line would suggest.  I think I knew this, but it’s good in a way to have it confirmed.

I’ve written before about the problem of over salivation.  I’m now taking Amitriptyline and Glycopyrronium Bromide.  The latter has been very difficult to obtain due to local CCG policy, but my GP has managed to find a way around this just before my prescription supplied by the Peace Hospice ran out.  Glycopyrronium is making a noticeable difference.  In fact, my mouth is now extremely dry at night and I’m being woken up for the opposite reason!  However, for every problem there’s a solution and I’ve found that Xylimelts slow-release Long-Lasting Adhering Discs work really well and keep my mouth tasting fresh and feeling comfortable.

My trial of a Tobii Dynavox Windows tablet based eye gaze system has continued. Technically I’m competent in using it. Just a bit slow. However, I’m at a delicate stage in discussions with CASEE, the NHS communication equipment supplier in Eastern England.  The Windows device doesn’t give me anything other than a means of talking, it stands alone, and I’m not allowed to install any software (such as Word and Excel) to use with eye gaze.  For now, I can still type with one finger on my iPhone, and I’ve learned how to use the Assistive Touch settings to control my iPad with a mouse – allowing me to access and use any number of apps. But I won’t be able to do that forever, and I really need to have an iPad with eye gaze capability. Discussions continue. Maybe we’ll have an answer by the end of February.

Interesting fact for January:

This website was visited by people in 18 countries in the last 28 days. Thanks for dropping in!

December brought three significant deliveries (even before Santa came to call).  The first was 20 packs (240 bottles) of Ensure Compact liquid feed for use via my pump and PEG, or as a bolus feed delivered by syringe into the PEG.  That number of packs takes up quite a lot of space but is 30 days’ supply if you are totally reliant on PEG feeding.  As a start I’m using Ensure Compact as a supplement to try to slow my continuing weight loss.  Since I won’t be drinking from the bottles it doesn’t really matter that it’s all vanilla flavour however, from sampling a little, I know it tastes like melted salted caramel ice cream so I’m happy about that.

The second delivery comprised two new main drive motors for my wheelchair accompanied by the service engineer from Millbrook Healthcare.  Only one motor failed at the start of November, but they wanted to replace both to be on the safe side.  It’s wonderful to have my own wheelchair back in service.  I didn’t realise how comfortable it was until we hired the temporary replacement at vast expense!  I can still ‘walk’ around in the home (although I’m told my left foot is starting to drop).  However, I don’t have confidence to tackle any steps and have a real fear of falling, so no outdoor walking now.

A Tobii Dynavox eye gaze tablet and stand were delivered on 14 December.  I was instructed how to use it during a Zoom call; not an ideal way to get to know a new system and I still have a great deal to learn.  The fact that this tablet uses Windows technology is an added complication as I have been an Apple user for years.  However, the plan is that this device will be replaced with the TD Pilot iPad device in due course and in the meantime, I’m getting useful practice.

It’s frustrating that CASEE, the NHS provider of eye gaze equipment in Eastern England (based in Cambridge) is only concerned about solving spoken communication problems.  If you want to talk about using an eye gaze device for writing emails, or controlling household lights, sound systems etc, you must have a separate conversation with the NHS Environmental Controls team in Hillingdon.  The organisation of the NHS clearly bears no relationship to the capabilities of modern technology and certainly isn’t a ‘one stop shop’.

I forgot to mention in my November post that, while I was in hospital recently, I was prescribed a new medication intended to control my ‘emotional lability’. This fun symptom of MND is particularly prevalent in people with (brain stem) problems such as swallowing and speech.  

Emotional lability can result in either uncontrollable, inappropriate laughter, or increased emotionality causing you to burst into tears at any poignant moment.  Or both.  I’ve not experienced the laughing (fortunately!) but I have been unable to stop myself from crying when thinking sad thoughts or listening to music.  This is quite embarrassing at times.  So, I’m now taking a small daily dose of Citalopram, an SSRI used as an antidepressant.  It works – no more tears.

It’s not usually polite to talk about toilet problems, but as I’m primarily writing for people with MND, I will.  For the last few weeks, I’ve been experiencing bloating and flatulence. It’s been difficult to pinpoint the cause because I’ve started liquid feeds and changed a few medications and there are too many variables.  However, it turns out that the most likely cause is that I’m swallowing air during my overnight use of the BiPap ventilator – a condition called Aerophagia. If you’re fortunate enough to have a PEG or RIG feeding tube, there’s actually a very simple and effective solution.  You’ll need a carer to help though.

The carer screws an empty enteric feeding syringe body onto the feeding tube (with the plunger removed), then releases the clip on the tube.  Now the carer presses on your stomach with firm but gentle pressure.  Immediately air gets expelled from your stomach, up the feeding tube and out through the open syringe with a very satisfying loud gurgling sound!  You’ll find that more air comes out if you’re able to shift your position a bit.  The only tricky bit is to ensure the feeding tube doesn’t contain air when it’s re-clipped and the syringe is removed.  So, before doing that, using siphoning principles, lower the syringe and check that stomach liquid fills the tube. Any small amount of liquid that escapes into the syringe can be flushed away with plenty of water.

On 27 December Alex completed his 1,000 kms running challenge in support of the South Herts branch of the MNDA.  In so doing he has raised over £15,000 in total, 50% above his original target.  For his final run he was joined by two longstanding friends.  It was a very wet day, but we welcomed them home with a Champagne reception!  The way Alex has turned something as negative as MND into something so positive has given us great strength in 2021. 

We enjoyed a lovely family Christmas but look forward to the New Year with a little trepidation, knowing that my time is running out.  However, we remain determined to deal with the increasing problems in a pragmatic and positive way for as long as we can.

Best wishes for 2022

My power wheelchair has broken. Four weeks later the new motors are still awaited, the delay doubtless will be blamed on COVID and Brexit. As it was supplied by the NHS there’s no provision of a replacement; they regard my ‘user operated’ folding wheelchair to be a substitute (except that this user can’t propel a pencil, let alone a wheelchair). We’ve hired a replacement at the cost of £105 per week. Once again, I wonder how those without spare cash could possibly manage with all the unexpected costs that MND brings.

On 10 November, 49 years to the day after passing my driving test, I surrendered my licence to the DVLA.  This was one of the hardest days yet on my MND journey but, as I sat in the driving seat for the last time, it became all too obvious that my arms could no reach the steering wheel.  My much-loved Skoda Kodiaq will be for sale very soon.

Over the course of the next couple of days the paralysis of my shoulders was just about complete, and my elbows are now getting difficult to use.  My wrists get very sore after little use, and there’s a tendency for the fingers on my left hand to curl inwards. These rapid changes are seriously hampering my ability to do hundreds of daily tasks, including typing.  At present I have two ways to type; the first is with one finger on my iPhone, which has the benefit of being relatively light and small, the second to use a mouse to click (one letter at a time) on an onscreen virtual keyboard on my MacBook (found in the Accessibility menu in System Preferences in MacOS 11 onwards). 

I have to say that this monthly update has taken me three days to write so far (on and off) so I do hope someone reads it!  Another big issue is that, because I can’t really type anymore, I can’t use the Predictable app on my iPad to speak for me – at just the time I need it.

Trying to be proactive, we have been looking at pricey eye gaze technology, which has to be the next step. There’s a new device, just the market, called TD Pilot (from Tobii Dynavox), and it was recently demonstrated to us at home.  It’s brilliant and provides speech capability and (because it is hosted on an iPad Pro) you can also use eye movements to operate iPad apps via eye movement.  It costs £7,000. 

I was all set to buy this lifesaver however, working through my ever-supportive SALT (speech and language therapist) and the Communication Aid Service East of England (CASEE) I’ve eventually met the criteria for NHS provision which I’m both grateful for and excited about.  The eye gaze equipment can’t come a day too soon as my speech is now almost unintelligible to everyone including Sue.  We frequently have some funny conversations.

Weight loss is a common symptom of MND. I’m almost ashamed to admit that before pneumonia in Vietnam my weight had crept up to 96 kilos. Two weeks later, arriving home, I was 89 kilos and this was largely the result of not eating. What a great achievement! 

Last week my weight was 76 kilos; my shoulders are incredibly bony, my arms resemble thin sticks and my left leg is following suit. I’ve been told that a three stone weight loss is enough, and a delivery of prescribed food supplement is on its way.

The causes of weight loss in MND are numerous and include muscle atrophy due to lack of stimulation, loss of appetite due to a general feeling of being unwell and taking longer to eat food to avoid choking (often leaving food on the plate).  For me, weight loss without even trying has been a new experience.  It will be interesting to see whether it can now be stemmed.

Towards the end of the month, after a week of self-isolation and PCR COVID tests, Sue and I were both admitted to the NMCCU (Neuro Muscular Complex Care Unit) at Queen Square – me as the patient and Sue as my carer. This arrangement saves the nursing team many hours of care, but it did remind us of being together in the COVID quarantine hospital in Ho Chi Minh City eighteen months ago (but it was considerably cleaner, cooler and more comfortable).  During the two-night stay the pressure and the breathing rate on my BiPap ventilator were adjusted several times and it’s now working well.  In fact, I’d go as far as to say it’s quite a comforting overnight experience, I’m sleeping well and feeling much brighter and refreshed now my blood bicarbonate level (HCO3-) is declining as I’m exhaling carbon dioxide (CO2) more effectively.

All in all, November has been a month of great change.  I rather hope I’m on more of a plateau in December.

Thanks for reading and Merry Christmas!