Eyes on the wrong ball

What a year 2020 had been.  Reflecting on the build-up to my diagnosis through the year – the aspiration pneumonia, the onset of weakness in my arms, difficulties with speech and swallowing… I came to realise that I’d actually been focusing on the wrong things for many years.

In 2003 I’d had the first of two heart attacks at the age of only 48.  Just six years later I was diagnosed with prostate cancer.  In the first ten or so years of the new century I’d had several angiograms, two sets of stents in coronary arteries, and a robotic radical prostatectomy to sort out the cancer. 

So I was ultra-cautious.  I was careful not to over exert myself or to get too stressed. Either could have had consequences for my chronic coronary artery disease.  I was also watchful for other symptoms that may have been associated with the cancer. I was really fortunate enough to be able to retire early, to change my lifestyle, to travel, to relax, and to enjoy time together with Sue.

Little did I know that I’d hit the jackpot and would go on to develop a third fatal disease! My eyes had been on the wrong ball and I guess the earliest symptoms of MND had passed me by as minor inconveniences.

More than this, after our traumatic experience in Vietnam – the awful COVID quarantine hospital, the pneumonia and the dash to get home on the last flight before lockdown, that illness really was high in my consciousness and it got the blame for a number of the subsequent MND symptoms. My voice sounding hoarse, increasing tiredness, another chest infection etc.

But I also reflected on how lucky I had been. Some people sadly are passed from pillar to post, from consultant to consultant, trying to diagnose their MND over many stressful and frustrating months. Thinking about it, the first real indications that I had something seriously wrong with me were at the beginning of August 2020 and just days later my GP had referred me to a neurologist. In early September I was told that all the symptoms sadly pointed to MND. Four weeks later that was fully confirmed. The fact that this speedy process of diagnosis happened during the COVID pandemic makes it all the more remarkable.

I’ve also been very lucky to have been referred on to ‘The National’ (Hospital for Neurology and Neurosurgery) at Queen Square in London, just 35 minutes from home by train and a world class centre of excellence with the most supportive and co-ordinated clinical team one could ever hope to come across. And it’s all free of any costs under the NHS. Amazing.

First visit to the National Hospital for Neurology & Neurosurgery

Thursday 12 November was a memorable day – we took the train to London for my appointment at Queen Square. Kings Langley station was eerily quiet as we waited for the ‘rush hour’ train because most people still worked at home. 

My consultation was really helpful.  So kind and supportive.  Firstly, I had a full examination and then a frank discussion about the condition and its likely progression.  I was prescribed two medications – Teglutik (Riluzole in suspension) and Lorazepam to calm the anxiety caused by choking on food.  

Riluzole is the only primary medication for MND at present – it’s thought to work by protecting the nerves in the brain and spinal cord from too much of a natural substance called glutamate that may be part of the cause of nerve damage.  Studies have shown it can delay the need for ventilation and increase life expectancy by two to three months.  Not a great impact really, but probably worth taking if you don’t experience side effects.  I was prescribed Tiglutik, which is Riluzole in suspension and therefore easier to take.  However, it tastes artificially sweet and has the really odd effect of numbing the mouth and throat immediately after you’ve taken it.  

We talked about benefits to which I was now entitled, and the consultant said that the hospital would provide me with a DS1500 form to enable me to make a fast-track application.  The DS1500 states that death within six months could be ‘reasonably expected’.

We also discussed the right time for the provision of a feeding tube.  It was pretty clear that, even though the consultant was reluctant to give my MND any particular label, its first devastating impacts were on my abilities to speak and to swallow.  We talked about the options of my having a PEG or a RIG.

The neurologist arranged for me to have blood tests, and for me to be seen by a respiratory consultant later in the day.  There was some concern about my lung capacity and lower than normal blood oxygen saturation, which I’d been monitoring at home.  An overnight home sleep study was arranged for a few days later to monitor the quality of my breathing while asleep.  It is bit difficult to sleep with a sensor under your nose, a finger sensor and a device strapped around your chest but a useful experience as it showed that I wasn’t in immediate need of non-invasive ventilation.

Hospice visit

On 19 November we went to the Peace Hospice in Watford for a first meeting with the community nurse. We covered a lot of ground and, having thought about the matter a lot, I was able to talk about my future wishes for the time when MND gets the better of me.

I’m not very brave and I hate pain and suffering. I would also hate to inflict stress, sadness and incessant high demands my loved ones. So I said that, once I reach the end of the road, the hospice will be a more appropriate place for me to be than home. That wouldn’t be everyone’s decision, but it’s my choice for now.

I authorised a ‘do not attempt resuscitation’ form in the event of cardiopulmonary failure and this has been countersigned by a doctor. I now have it in a folder with my Lasting Powers of Attorney, and my Advance Decision to Receive Treatment (from Compassion in Dying). These will accompany me should I ever get admitted to hospital.

I’m really pleased to have these issues resolved. It seems to me that once you have the terms of your death sorted out, you can get on with living your life.

Next steps


After a negative COVID test, I had a videofluoroscopy examination on 19 October arranged by the Speech & Language Therapist. We shared a concern about my swallowing, and the test would show the extent of the problem. It was a very easy test – you sit in a chair with an X-Ray camera to the side and a large TV screen in front. You then are given a series of different things to eat and drink either coated with, or mixed with, a small amount of barium. On the TV screen I could see my head, mouth and swallowing action with the various samples. I’ve always hated the taste of banana and a coating of barium didn’t enhance the experience!

The result of the test was clear evidence that there was ‘airway penetration’ when I swallowed ‘thin’ liquids like water and tea. In other words, it might be easy for me to get a chest infection from some liquids entering my lungs. There wasn’t a problem with thicker drinks. Crunchy biscuit also caused problems of a tickle in the throat, a cough and the risk of choking.

I was prescribed Resource Thicken Up Clear to add to all drinks in future – for example two scoops in a mug of tea or a glass of water or wine. Thickened wine may sound like a travesty, but the slightly jelly-like consistency is actually very nice. Subsequently I have found that naturally ‘thicker’ drinks (like a cappuccino or flat white), or fizzy drinks (like lager) are not so much of a problem.

MND Association

We made contact with the MNDA in a number of ways. I’d already been looking at their online forum on the website, and I’d looked through many of the really valuable pages of online information. The resources are really good.

We ‘met’ our MNDA Area Support Co-ordinator on a Zoom call, and the local South Herts branch of the association made contact with us. We were in abnormal times during the COVID pandemic, but the local branch organises monthly Zoom calls for carers (which Sue always attends) and also monthly Zoom calls for people with MND. I attend this one less regularly as my speech isn’t great and I find it difficult to participate.

We also were introduced to our really supportive MNDA ‘visitor’ who is in regular contact. She wasn’t able to visit us due to COVID restrictions but monthly phone conversations have been much appreciated, and she is always available to take a call if we have questions or concerns.

Quotes for ‘through the floor’ lifts

There’ll be a specific page in this blog about wheelchairs and lifts, but during October we had various sales people around to provide quotes for through a through the floor lift to would take me in a power wheelchair from our dining room to my newly designated study bedroom above. These lifts are expensive, but much cheaper (in our case) than an alternative option of converting our well used garage into a bedroom and new bathroom. We did have a quote for one very nice looking lift that, on close inspection, was not actually big enough to accommodate a power wheelchair. We could have made a very expensive mistake, as others have done.

Network of care providers

Towards the end of October we were getting quite confused by all the names, so we started drawing a map of all the people we were having regular contact with, providing us with care and support. You can see how complex the network has become!

Diagnosis Day

It was a cold and wet morning on 2 October when we went to St Albans City Hospital for a follow up consultation with the neurologist.  After the two sets of tests my NHS consultant’s amazing PA arranged a special ‘overbooked’ appointment for me to be seen at the end of his Friday morning NHS list.

It was very quiet in the outpatient’s department because of the coronavirus protocols, and I didn’t have to wait for more than a couple of minutes before being asked in to see the consultant.  Meanwhile Sue sat in the car, in the rain.  Only patients were allowed to enter the building. 

The consultant was very candid once again.  As we’d previously discussed, everything pointed towards motor neurone disease and he now could definitely confirm the diagnosis.  The nerve conduction tests had revealed quite extensive denervation; the MRI scan only showed ‘normal wear and tear’ and certainly no indication of a brain tumour that might have been responsible for MND-like symptoms.  

I count myself as being very lucky because there isn’t one definitive test for MND and an awful lot of people seem to be passed from pillar to post, from one specialist to another, in a well-meaning attempt to rule everything out before finally landing on the devastating diagnosis of MND.  I’m really grateful to my consultant for going with the balance of probabilities, for his pragmatism, and for not tempting me by offering false hopes.  He asked me how I felt about having a feeding tube and non-invasive ventilation when needed.  I said I would be quite happy to have both.  The prospect of choking to death on food or drink (although rare) is truly horrible, so a feeding tube or ‘gastronomy’ would be great and would alleviate that problem.  I said ‘yes’ to non-invasive ventilation but said I would never want a permanent tracheostomy.  He told me that, in practice, this doesn’t routinely happen in the UK, but he added my request to my notes.  I told him that when I can no longer breathe, that will be the time for my life to come to an end.

A number of things would be arranged.  Firstly, he would refer me to colleagues at the National Hospital for Neurology and Neurosurgery at Queen Square in London for follow up and care.  This sounded like great news to me as I knew the hospital has a world class reputation.  Secondly, I should re-start Atorvastatin as it was clear that I didn’t have statin induced muscle weakness.  Thirdly, having been diagnosed with a notifiable condition, I would have to contact the DVLA so they could determine my ongoing fitness to drive. Having passed my driving test in 1972 this was one of the hardest parts of the whole day.

I went back to the car where Sue had been waiting.  There wasn’t much to say as we had already known full well that I had motor neurone disease.  We went home for lunch to start making plans for a very different future.

First neurology consultation

On Wednesday 8 September I met a really knowledgeable and empathetic consultant neurologist at Watford General Hospital.  After a discussion about my symptoms, he carried out an examination and checked my reflexes with his little hammer.  One of the most startling and indicative tests (as I read later) was a test of the reflexes in my feet.  I displayed the ‘Babinski Sign’.  When the soles of my feet were tickled, my toes instantly and dramatically curled up instead of down.  Apparently, this reflex is normal in infancy but when it occurs later in life, it’s a strong indication of damage to the central nervous system.

Before the appointment I’d asked the consultant if he would level with me about the possible diagnosis.  Without prevarication he told me that all the signs pointed towards MND.  The first step was for me to stop taking statins for a while (there’s some indication that they can cause muscle weakness, and this had to be ruled out).  Secondly, I should have a series of tests – nerve conduction and electromyography studies, and an MRI scan of my brain.  Thirdly, I’d be referred to a specialist Speech & Language Therapist for help with speech and swallowing.

Within ten days I had my first consultation with my Speech & Language Therapist.  This was long before any formal diagnosis of MND.  We talked about my experiences of coughing or choking when eating and the types of food and drink that I should avoid as they could cause problems.  Foods that are too dry, flaky, sticky, crisp or hard should be avoided.  Thin liquids like water and tea would also cause problems and great care should be taken.  I’d sort of worked this out for myself already; my own experience was that I could enjoy a creamy cappuccino very easily, and fizzy drinks (including beer!) were OK because the bubbles seemed to help the liquid to be transported when swallowed.

The other subject for early discussion was voice banking.  While I could still speak fairly coherently, without too much of a slur, I should record my speech so it could be synthesised for use when I can no longer speak.  What a scary prospect that was, and this spurred me into action.  I’ll write a separate section on the subject of voice banking because it gets quite involved (but is very satisfying).

On 24 September I had nerve conduction tests and electromyography studies at Hemel Hempstead Hospital.  A great doctor and nurse team, and it wasn’t as bad as it sounds.  Yes, it does involve fine needles being placed in your arms, legs, chin, and yes some mild electric shocks too… but it’s quite bearable and not really painful.

On 29 September an MRI scan, back at Hemel Hempstead Hospital.  This procedure was quite difficult because you have to lie flat and very still on a hard plastic surface for about 45 minutes while the scanner does its extremely noisy work.  Lying flat on my back wasn’t ideal because I couldn’t clear my throat properly so it was quite uncomfortable.

This is getting serious now

August came and suddenly the muscles in my left arm were quivering… all of the time.  I opened my mouth to look at my tongue and was shocked to see that it too was quivering uncontrollably.  I was pretty sure that my tongue somehow looked thinner and ‘wasted’ too.  We looked up ‘muscle tremors’ (fasciculations), ‘slurred speech’ and ‘cramps’ on Google.  The answer was motor neurone disease.

I booked an appointment to speak to a doctor about my symptoms on 6 August.  The only one available was with a doctor at the Watford Extended Access Hub.  It was a telephone consultation and we talked through my numerous symptoms.  I said, ‘I think I might have motor neurone disease’.  The doctor didn’t disagree, and he said he’d refer me back to my GP practice for a further consultation.

The next day I had a video consultation with a GP from my surgery.  After talking through the problems again the doctor asked me to lift some weights on camera and she looked at the fasciculations in both my arms and in my tongue.  She immediately initiated an urgent appointment for me to be seen by a neurologist.  The possibilities to be explored were either a brain tumour, or MND.

I could now feel fasciculations all the time in both my arms and in my back around my waist.  It was a funny tickly feeling that has never gone away.

I can’t fully raise my arm. Should I be worried?

I could no longer raise my left arm comfortably to open the garage door, and actually both arms seemed to have insufficient strength to lift things above my head.  It became very difficult to continue to do my online keep fit classes because it was just too exhausting.  My speech was definitely getting much more slurred to the point where I began to avoid answering the phone.

More alarming changes

By June some new problems emerged.  My voice was really lacking volume and I began to notice that I was slurring words – it was as if I’d had too much to drink all the time.  

How odd.  Now there was something wrong with my left hand.  It was really difficult to squeeze shampoo out of the bottle, and almost impossible to use nail clippers to cut the fingernails on my right hand.  Around the same time, it also became impossible for me to cut my toenails – I just couldn’t bend down to reach without instant, painful cramp in my legs.

I still had a slight cough, and I had a real tickle in my throat when drinking water and cups of tea.  Very uncomfortable.  On 19 June I remember choking when eating some crisps.  Three days later I developed a chest infection that required antibiotic treatment.  It was hard to get help for a chest infection in the days of COVID-19 and the first hurdle had to be a negative test result.  Naturally, we associated this chest infection with the pneumonia in Vietnam.  Later it became obvious that both events were probably actually cases of aspiration pneumonia caused by choking and inhaling particles of food into the lungs.