Bulbar Onset Motor Neurone Disease or Progressive Bulbar Palsy: An Account of my Family’s Experience

My mother died of Bulbar Onset Motor Neurone Disease (also known as Bulbar Onset ALS or Progressive Bulbar Palsy)  in November 2018.  I say she died of it, but it went on the death certificate alongside Aspiration Pneumonia, which is what caused the crisis that led to her death.  She wouldn’t have got Aspiration Pneumonia without the Motor Neurone Disease though.  My mother was born in 1947 and until she was diagnosed with MND she had been very healthy.  She was a slim, active woman and she was forever running around the local area.  She always seemed to be a few minutes late and would run to make up for that.  She’d run in a number of races over the years and had fairly recently been placed in the Great South Run for her age group. We thought she had another 20 years plus ahead of her, not least because her mother was still alive and only died a year before Mum.  The only health concern was diabetes which seemed to be a family trait as she wasn’t a typical physical type for it, but she was able to manage this through diet.

Mum first showed signs of symptoms in the late winter, early spring of 2017.  We went on a trip to Wales (me, my brother, Mum and Dad) at Easter that year and she was slurring her words.  She’d been doing it for a few weeks by then.  She’d been to the doctors and seemed to have been fobbed off a bit (a virus).  It was unlike Mum to allow herself to be fobbed off by a doctor as she’d been a nurse for many years.  I suspect that she already had an inkling what was wrong, but perhaps wasn’t ready to face it.

Over time the slurring got worse.  The GP referred her to an ENT specialist.  She went along and came back reporting that the ENT consultant had said he could do nothing for her and had referred her to a neurologist.  Mum said ‘ that’s never good news’ – I think she already knew by this point. In the summer of 2017 the speech continued to get worse and we pushed Mum to chase up her appointment.  Her neurologist referral seemed to have got lost in the system and the GP chased that up.  We arranged for Mum to see a private neurologist and then have a private MRI scan to speed things up a bit.

By August all the bits were together and Mum had an NHS neurology consultation to go to.  Alongside the MRI scan she had a letter from the private consultant which clearly said that Mum had spoken to him about her concerns about her problems possibly being caused by Motor Neurone Disease.  Coming back from this appointment my parents reported back that it was probably Bulbar Onset Motor Neurone Disease.  I’m not sure if the hesitation was the consultant or them.  After that I went to the vast majority of the appointments, so I had a clearer idea of what was going on.  At the next appointment in November 2018 I went with her and the diagnosis of Bulbar Onset Motor Neurone Disease was confirmed.

Mum was referred to a speech therapist following the first NHS consultant appointment (they started treating it as Bulbar Onset Motor Neurone Disease immediately), who visited her at home.  She was the person Mum saw most regularly over the course of her disease and she came to the house throughout. I thought she might help Mum improve her speech for a little while at least, but this didn’t happen.  The focus in later visits certainly (I started going to these when I could in the autumn) was around swallowing and breathing.  Mum started drinking with a thickener.  She didn’t really like the taste and she barely drank anything really for a few months.  She went from drinking multiple cups of tea a day to drinking one, then none and it was much the same with the water.  I think it was just too troublesome.

By October 2017 Mum could barely speak – it had taken just over 6 months for her to lose her speech.  Even with an ear that had got accustomed to her slurring, it was getting impossible.  Mum was the type of person who enjoyed speaking to people that she met in shops, or at the bus stop or at one of her many classes.  This was now not feasible for her.  Eating got ever more difficult too.  She couldn’t eat a lot of foods, basically anything that required a lot of chewing.  It had to be soft, but not too liquidy.  Something like a thick stew or a yoghurt was fine. I remember she managed a bit of Christmas dinner, but it was the portion of a small child.  She had stopped doing her exercise classes and Tai Chi by Christmas 2017 – I don’t think she had the energy.

Mum was losing weight.  She didn’t really have any spare flesh to lose so this was very worrying.  Medical staff advised a PEG (Percutaneous Endoscopic Gastrostomy), which is basically a feeding tube in your tummy that goes directly into your stomach.  This apparently had to be done at a specialist centre in Stoke and we live in Birmingham.  This seemed daft as we have loads of big hospitals in Birmingham and there are other places with hospitals that are closer and easier to get to than Stoke, but apparently they are specialists in respiration.  This seemed a bit silly to us as non-medical professionals as Mum didn’t seem to be struggling with her breathing, but I don’t think she always told us all about her symptoms and it later came out that she was having to prop herself up in bed at night.

One of the main issues with going to Stoke was the fact that they never gave my Mum a date for the op, but wanted to ring with cancellations.  I think this was because they wanted to fit her in quickly, but it did add to the stress for the whole family.  We eventually persuaded her to get it done in February when they rang up the second time with a cancellation.  Mum really needed it by this time. We only had a day or two’s notice and had to rush around buying her new pyjamas, etc. She found that she felt frozen cold, even in that hot hospital, because of how little flesh she had on her.  It was a relief to get her home again and to settle into the feeding regime. Dad really stepped up to the mark with this and I think it suited him as he’d been a lab technician.  It was onerous though, as she was being fed 5 times a day and she wasn’t gaining weight. Mum was still able to manage a little bit of food though at this point.  We bought specially prepared foods from Wiltshire Farm Foods and also used the fortified yoghurts provided by the doctor.  Mum said she dreamed about food.

From February we did insist that they needed more help around the house (Dad was more than happy for this, it was Mum that needed persuading).  We arranged for a cleaner, but Mum was still insisting on doing the ironing for a few months longer.  From February to July we had a bit of a pause in her disease.  She did still decline overall but it was less noticeable, and there was even some improvement short-term after the PEG was fitted, because of better nutrition I think.

Mum and Dad found a way of working things between them.  They would go to the shops together on the bus.  My brother would get any heavy stuff for them, so they just needed to buy the fresh stuff. They tried a machine for feeding overnight and that helped as it freed them both up.  It did cause a bit of stress while they got used to it, but overall it worked well for them.  Mum was still able to walk down to church on a Sunday and she came to my son’s last school sports day at primary school.  Her left arm was quite weak by this stage, but her right arm was better so she could still write (in fact the last time I saw her write was on the Friday before she died – it was clearly harder than it had been and she wrote single words or short phases and it was sometimes difficult to read, but she could still communicate that way).  Her legs weren’t too bad either although not as strong as they once were.

In July 2018 though she went downhill very badly within about a week.  One week she walked to church, the next week she got a lift, and the third week she didn’t go at all.  From July onwards you could probably count up the number of times she went out of the house.  She went to appointments, a few family meals at the local pub and I pushed her round the local park in a wheelchair on sunny days.  She could no longer manage the stairs really and we wanted her to move to the ground floor, but settled on the middle floor (she lived in a three storey house) in a room with easy bathroom access.  Stair lifts aren’t recommended for people with Motor Neurone Disease because of their weak core muscles. Around this time she got a hospital bed, a special chair, a bath lift and various small adaptations were made to the house. She couldn’t hold her head up, so we tried out collars.  They did help a bit, but got uncomfortable to wear after while.

August and September saw her go down gradually, but fairly quickly.  Walking around the house was problematic.  Then she got a cold in early November.  Dad had had it for a week, so it wasn’t a surprise.  Mum found it hard to breathe.  It was very scary.  She had to stand up to try to breathe sometimes, but needed help to do it.  We had the GP to the house and the priest.  She did recover though.  I think it did weaken her, but it’s difficult to be sure as she was getting weaker every day anyway.  I knew pneumonia was the danger and thought it might even be a kindness, but I don’t think any of us were ready then (if you ever are).

In November she carried on getting worse.  She couldn’t keep awake for more than a few minutes at a time, but she couldn’t sleep at night either because of her breathing problems.  We tried various machines, but the only one she really used was a suction machine to take away the saliva (when you can’t swallow it builds up and this caused my mother much distress as she kept dribbling).  There was no quality of life left really.

One evening in mid/late November 2018 she struggled to get up the stairs to bed.  This came a bit out of the blue, although it had been getting progressively more difficult to get her up the stairs at night.  My brother was called and he carried her up.  All seemed normal.  Then about 10pm she needed the toilet and Dad went to help her.  She fell in the bathroom and Dad couldn’t get her up.  My brother came round again and helped get her back to bed.  The next time she needed the loo, she fell to her knees on the way back to bed and Dad decided it was time to call the ambulance.  They came and took her into hospital.  This was on the Sunday morning (early hours).  She was distressed and not really with it when we went to see her later that day.  The next day she was much quieter and more peaceful, but also clearly lower in herself.  On Tuesday I went to see her in the hospital and she seemed the same, but lower still.  She died whilst I was there that day.  There was no drama: she wasn’t hooked up to monitors by that stage.  I noticed that her colour was greyer and her chest had stopped moving.  I already knew what had happened, but asked a nurse to check.

As I said earlier Mum died of Aspiration Pneumonia, which is caused by something going into the lungs that shouldn’t and causing an infection.  In her case this was either food from the PEG (she had been sick a couple of times, although not that close in time to her last illness) or more likely from her own saliva.  You or I can clear things that go down the wrong way by coughing, but people with Bulbar Onset Motor Neurone Disease can’t do that. Or at least they can’t in the later stages of the disease and even before that it, it becomes difficult (there were a number of meal times where my Mum ended up retching on her dinner before she gave up eating).

I have written this for other people to read that are either suffering from Bulbar Onset Motor Neurone Disease or are close to someone who is.  I found it helpful to read other people’s accounts of the disease as a way of preparing myself for what lay ahead.  Having read other people’s stories and spoken to the relatives of other sufferers, it’s clear that each path through Bulbar Onset Motor Neurone Disease is different.  That said, there’s a lot that is similar too.  One way of describing this form of the disease, as compared to the better known Amyotrophic lateral sclerosis (ALS) form of Motor Neurone Disease, is that it happens from the inside out, rather than the outside in.  It’s a bit simplistic I know, but this helped me tell other people about what was happening to Mum, so I’m mentioning it here in case it helps you too.

When Mum was first diagnosed I sought out Progressive Bulbar Palsy stories to understand what would happen to her. I’ve shared her story here in the hope of helping others faced with a diagnosis or with someone close to them with a diagnosis of Bulbar Onset Motor Neurone Disease or Progressive Bulbar Palsy. I’ve written another post about what things worked and didn’t work for my mother during her illness.  You can read about what seemed helpful for her as a sufferer of Bulbar Onset Motor Neurone Disease here.