My Mum had Bulbar Onset Motor Neurone Disease (also known as Bulbar Onset ALS or Progressive bulbar palsy) and lived about 20 months with the disease (15 months post diagnosis). We tried various things to work around her various needs and here is an account of what we tried and what worked or didn’t work. Everyone is different though and other sufferers might get on better with some things than she did.
Information and Support
The Motor Neurone Association was our first port of call. They have a good website with lots of information. Soon after diagnosis we had a personal visit from them to Mum at home and this was helpful because it meant that my parents were aware of DLA (Disability Living Allowance) which isn’t means tested. The Motor Neurone Association’s helpline guided Dad through the process and it was very straightforward. The money helped with some of the extra costs they incurred like getting in help around the house or needing a taxi. They also provided some handy little (business card sized) cards which basically said that Mum could understand, but not speak and they were useful in shops and the like.
To be honest that is about as far as the help went. A lot of the assistance seemed to be geared up to those with the Amyotrophic Lateral Sclerosis form of the disease like support groups, but that was difficult for Mum as she already couldn’t speak. We did contact them a couple of times about borrowing equipment or asking advice and they weren’t desperately helpful. We were a bit disappointed to be honest, so much so that at Mum’s funeral we encouraged people to support other charities over the Motor Neurone Association, as we didn’t feel we could wholehearted recommend that people give their money to them.
Speech and Communication
Mum’s speech was the first thing to go and we had to deal with communication issues from the off. To start with she had a notepad and pen and later she moved onto her digital slate. Mum wasn’t very good with technology so an iPad or a tablet wasn’t her thing, but the digital slate was really easy to use. You just write on it and press the button to erase. It’s very lightweight too. Mum used her slate for over a year and I’d recommend trying one as they aren’t expensive either. We also bought a whiteboard and pens because we thought they might be easier (less drag on the pen), but Mum used the slate right up until she got pneumonia.
We also used sign language. Mum seemed to be the world’s worst at sign language. We had no idea what her signs meant. In the end we had to say it was thumbs up and down for yes and no. That was about the extent of it apart from waving and pointing. I did consider whether we should try to teach her more signs – I did study British Sign Language some years ago – but, her left hand started to show signs of deterioration quite quickly, so I’m not sure it would have been worth it.
She did have some cards that were given by the Motor Neurone Association to say she could understand, but not speak. These were useful. I think if you needed to say or do something regularly, it might have been worth creating a few more personalised ones. I also considered making some to indicate things that she needed to say regularly like ‘I need to go to the toilet’ or ‘Turn the heating up’.
As Mum’s mobility became more of a problem, she and Dad found having a call button useful. She’d press the button and he’d come and help. They had a large house with garden and it seemed to have a range that covered all the areas. This was provided for free by the Occupational Therapist through the NHS. The only issue was when one of them failed to wear or carry the button with them.
Swallowing
Mum had to use a thickener for liquids quite soon after her diagnosis. This was provided on the NHS – I think the Speech Therapist asked the GP to prescribe it. Mum hated it to be honest and didn’t drink as much as she should have done because of it. You can try other brands if you don’t like the NHS one. There are loads on Amazon to choose from. I don’t know if they are any better, but it might be worth a go.
Mum found the Puree range from Wiltshire Farm Foods useful. She like the Puree Petite range as she struggled to get through a full meal as she found eating tiring. The fortified yoghurts that came on prescription were one of the last foods she was able to eat as they seemed to sort of slip down. Ordinary custard worked well too.
Using the PEG tube for feeding made it much easier to get both food and water into Mum. She didn’t need the thickener anymore. It was a relief to know she was getting enough fluids and she had a dietitian monitoring her weight and her diet. At first after the PEG all went well and even better after my parents braved the idea of the feeding machine which they used overnight. Sadly though after a while she ceased to gain weight on this and later started to lose weight. This is because the disease means that you expend a lot of energy doing the little you are able to do and so your requirements do increase over time. An overnight feed via the machine and another by hand during the day worked well for my parents, but when they were asked to fit in an extra feed it became difficult. It didn’t fit into their day very well, but more crucially Mum didn’t seem to be able to tolerate the volume and she was sick a few times. This is really dangerous and it was reported. My parents cut back on the feeds again, but that wasn’t a long-term solution either as it would mean Mum losing more weight.
Saliva
Saliva was the bane of Mum’s life. She struggled to swallow her saliva from a fairly early stage of things and got through an enormous quantity of tissues. It had a real effect on her quality of life and it’s one of the things that most annoys me about the health professionals she encountered as I think they let her down on this issue. Her consultant promised her he would sort it out and held her hands in his as he said it, but he didn’t.
What wasn’t made clear to us, until a long way down the line, is that whilst botox is an option to zap the saliva glands and stop them producing saliva, it’s very much a last resort. This partly seemed to be because they didn’t have anyone currently trained up to do it, but I think it is the case that they like to try everything else first. In our experience they want you to have tried all the drugs before the botox and they are reluctant to use botox while you have a swallow capability however small. I would recommend getting the consultant to write a letter to the GP with a list of drugs to try out, how long for, etc. so you can work your way through. Nothing worked for Mum, not even a little bit. The drugs they suggest are ones that have been developed for other things, but have a side effect of a dry mouth. Mum tried taking eye drops orally (tasted hideous apparently), an anti-depressant and an asthma inhaler (which was a dead loss because Mum couldn’t make a proper seal around the inhaler). At the time Mum died we were still waiting for the consultant to tell the GP what the last drug to try was and we had been chasing.
The only thing that really worked for Mum was using a suction machine. This was quite a big thing and not very portable, but works like the machine the dentist uses. It was like carrying around a medium-sized ghettoblaster or something. So it was fine around the house (as long as there was someone around to help), but no good for out and about. We only had the suction machine for the last few weeks of Mum’s life anyway as that referral had seemed to get lost.
Breathing
As Bulbar Onset Motor Neurone Disease progressed, Mum found breathing more difficult. She wasn’t allowed to lie flat or recline to any real extent and the Occupational Therapist provided her with a special chair and bed on the NHS. These were a godsend. Mum also tried Non-invasive ventilation (NIV), where a portable machine supports your own breathing by providing extra air flow through a nose or face mask. She only tried this in the last few weeks of her life and she didn’t like it at all. I don’t feel she gave it a fair go, but it’s difficult to know as she wasn’t writing that much by that time as she found it difficult. One problem that we did encounter was that the mask was too big for her and we had been promised a child’s face mask, but it never arrived. We were also promised a Cough Assist machine to help clear her lungs, but that never materialised despite chasing.
Mobility
Mobility became an issue in the last few months of Mum’s life. She was unsteady on her feet because some muscles were weaker than others and she was having to compensate. This was tiring for her to do. The Occupational Therapist arranged for hand rails to be fitted to the house and these were very helpful on the stairs. A stair lift was not advised as Mum didn’t have the core muscles to hold herself up on it. The OT recommended a move to a downstairs room, but Mum wouldn’t do it. We could have had a lift fitted, but that would have been very disruptive and she would have got very little use out of it. She was able to use the stairs (with assistance) until her last day at home as it turned out.
It became obvious from about July 2018 (Mum died in November 2018) that Mum needed some sort of wheelchair if only to push her round the hospital (which is enormous) for her consultant appointments. We did think about buying a wheelchair, but I’m glad we didn’t as Mum only used the one that we borrowed less than 20 times. The wheelchair we borrowed was very basic and probably not the best for her needs (it didn’t provide enough support and she could have done with one that helped her get up out of it), but it did fold up to fit into a unadapted car and we were actually able to borrow it for free. We paid a £30 deposit that we got back on return of the wheelchair. Getting a wheelchair was one of things we felt as a family we needed some advice for, but it wasn’t easy to get and we could easily have spent a lot of money on something we didn’t need. In fact, we didn’t buy because we didn’t know what to get, but we wasted valuable time trying to find out what we needed. Luckily the Occupational Therapist suggested a local service that would lend us something. We borrowed that with a view to trying it out and seeing how it met Mum’s needs before we bought one. As it happened it was clear soon after doing this that she was never going to use it very much and there was little point buying an expensive wheelchair. It was invaluable to have it for the times we did use it though. We used it at the hospital, when taking Mum to the dentist and I took her for some walks in the park on warmer days. I would recommend borrowing (if you can) or buying a basic wheelchair secondhand, rather than buying something new and expensive for someone suffering from Bulbar Onset Motor Neurone Disease.
We applied for a blue badge for Mum to use when we were transporting her around. To be honest I think the authorities should probably allow you to have one on diagnosis because Mum deteriorated quite rapidly and we were always playing catch up. Getting the blue badge was a faff (despite it being supposedly quicker for terminally ill people). We had to get a passport photo taken of her and that was not so easy to do when photo shops are often on busy high streets with no parking outside – a photo booth wouldn’t have worked as it was quite difficult to get a picture of Mum with her head sufficiently up for a photo. They also wanted a consultant’s letter rather than a GP’s which delayed things too and I don’t really feel it added anything to the information available to the council department dealing with the blue badges. I’d recommend starting the process early by ensuring you have a passport photo in advance and getting a consultant’s letter (if needed) when you have a convenient appointment.
Mum used a zimmer frame for the last few weeks of her life at home. She wasn’t very keen on using it, but it did make her walking better and she seemed steadier on her feet with it. I think it hurt her pride though.
Hand Claw
Mum’s left hand was starting to get a bit clawed in the last month or two of her life. She found it very difficult to move it and wasn’t able to straighten the fingers. She did try (very briefly) a resting splint, but she refused to wear it after the Occupational Therapist left the house.
Neck
Mum was unable to hold her head up in the last few months of her life. She did try this fancy collar, which made her look like Darth Vader and she found uncomfortable. We went back for another fitting and ended up with a much simpler collar. You can get them free on the NHS and should get a fitting to get the best one for your needs. If you are in a hurry to get a temporary solution, you can buy one on Amazon though. Mum couldn’t tolerate wearing the collar for very long at a time. The specialist who fitted the collar said that the jaw bones aren’t really designed to provide that sort of support from below, so whatever you use won’t be comfortable to wear after a while.
Washing
Mum used various things to help her with washing. It proved easiest to wash daily in the sink and a few times a week in the bath. For the sink she used a perching stool so she could stand with some support. This was provided by the Occupational Therapist on the NHS. Mum found the bath lift helpful as a way of keeping her independence. It was tough to find one to fit their cast iron Edwardian bath (original to the house), but the smallest one just about did the job. The problem was that older baths were curved on the bottom, but modern ones are flat, so they provide a better fit for the suction pads on the bottom of the bath lift.
One issue we came across was Mum’s habit of washing her hair in the sink. I’m so used to doing mine either in the shower or the bath and I found this really strange. Mum did start washing her hair when she bathed, as it was easier to get it out of the way when Dad was around to help. I’m mentioning this mostly because the person you are caring for may have different ways of doing things that seem strange and illogical to you. If you find yourself confused about why something is a problem it’s worth asking a few questions to fully understand the issue. Sometimes it will be simpler to do it another way and other times it might be that you want to preserve normality as much as possible for your loved one.
You can read my account of my mother’s illness here.
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