Welsh cross-party meeting on Muscular Dystrophy

I recently attended a Senedd meeting on Zoom about Muscular Dystrophy, which focused on how Covid had affected people with neuromuscular and muscle wasting conditions in Wales with regard to their medical care. It was attended by politicians, doctors, patients and other clinicians who discussed various issues that have affected the levels of treatment afforded to those with muscle wasting conditions both throughout the pandemic and prior to it. A prominent politician present was Member of the Senedd Rhun ap Iorwerth, who intended to help lobby Welsh government for more action.

Those present spoke of the negative impact of Covid shielding on the care of neuromuscular patients, which caused lack of access to care, disruption to services and delays in receiving treatment. Over the pandemic, Muscular Dystrophy UK carried out a survey of people with muscle wasting conditions across Britain which showed the problems caused by the pandemic. However, some of the problems highlighted were present even before 2020. These included lack of funding, not enough specialist staff and a reliance on the goodwill and efforts of individual health staff who end up burnt out and leave their roles. The pandemic meant that people with muscle wasting conditions missed out on appointments and treatment, and were left isolated from society whilst shielding. I was interested to hear the thoughts and feelings of other people with forms of Muscular Dystrophy, and those in charge of their care as many of the things spoken about are issues that I have dealt with personally and have seen for myself. As such, it was good to have confirmation that it is a common experience and that things do need to be done.

In the meeting, Muscular Dystrophy UK went through the findings of this survey and discussed five priority areas that were needed to ensure care for those with neuromuscular conditions continues and improves going forward.

1. more funding – the allocation of more money and more consistent funding directly for the care of neuromuscular patients

2. creation of a network manager role – this individual would help to push through directives and implement change with regard to patient care and the Wales Neuromuscular Network

3. more specialist staff – particularly psychology and mental health services, but also neuromuscular physiotherapists, nurses and neurologists etc

4. easy access to new market drugs – providing better and timelier introduction of specialist drugs to those with neuromuscular conditions

5. more staff in the community – introduce more trained neuromuscular staff who can work in the community to bridge gaps within the system.

The importance of improving the services for people with neuromuscular conditions cannot be overstated. People with muscle wasting conditions have complex needs which require complex long-term care from multi-disciplinary care teams. Not having adequate care can reduce the quality of life of those with neuromuscular conditions and potentially reduce life-expectancy, so making sure improvements are made to care services is vital. Increasing funding and awareness are therefore paramount to making meaningful change in this area.

Attending hospital appointments in Covid times

I went for an in-person hospital appointment a couple of weeks back for the first time in about 18 months. It was a slightly strange experience after spending such a long time shielding and staying at home because of Covid, and it went without a hitch. It was a cardiology appointment which I was glad to have because I had been feeling some anxiety about my heart rate and having not been seen face to face for an extended period. I was a bit apprehensive going into a hospital but it was no problem at all. I wore a mask, followed the one-way system and the place was very quiet so I felt at ease. I felt only one moment of slight unease when five people entered the tiny room to look at my echo scan but that was still okay. All of the tests were fine and I was reassured by chatting with the consultant and the nurses.

Despite not going to a hospital, I have still been attending virtual appointments over the last two years and seeing respiratory nurses and others at home occasionally. Having telephone consultations and video calls have been very useful during the pandemic for people with neuromuscular and muscle wasting conditions to access some services but face to face meetings remain vitally important when it comes to medical care. A recent report undertaken by Muscular Dystrophy UK (linked below) has highlighted this point, as difficulty in getting appointments has been shown to be detrimental to the physical and mental health of people with neuromuscular disabilities.

https://www.musculardystrophyuk.org/static/s3fs-public/2021-11/POL14%20-%20WalesVersion-Impact%20of%20COVID%20report.v4.pdf?VersionId=5OjOr0ezmTJQ3AEL8C7xVdn725MAojfG

Importance of comfortable seating

Making sure that your wheelchair cushion is comfortable and supportive is very important, especially when you sit for most hours of the day. Having the correct seating is good for my posture and balance, but I can be thrown out of position by very small alterations to my seat. Often when I have had discomfort it has been related back to the seat and how supported I am feeling. When the gel-pack my cushion leaked recently, I had to have a new one and it changed my height in the chair. This had knock-on effects onto my legs and head position. A change of just 1 or 2 inches meant that I had to put things under my feet to lift them up to stop my legs feeling painful.

The cushion is not the only thing that keeps me comfortable. In conjunction with my lateral support and chest strap, I am held in a relatively stable position that has kept me seated well for a number of years. Sitting all day and wanting to be in a good position has implications for normal activities, and being comfortable is especially necessary. Fortunately, over the years I have worked well with some great seating and wheelchair healthcare professionals to keep me in a good position and they have provided lots of input. This service is definitely an important one for people with wheelchairs, so it really should be seen as an essential part of adult healthcare.

One good thing we have found to prevent discomfort is occasionally giving the gel pack a bit of a beating to push the gel around and spread it back out properly. If I am uncomfortable it does seem to help as the gel tends to settle in one spot after a while. I currently have a JAY J2 cushion on my chair.

Anxiety about future medical issues

A couple of weeks ago, I had an annual check-up with some of the medical team I am under the care of. During the meeting, the subject of the future and advanced care planning was brought up. I was given some information about medical procedures that I may want to consider undergoing in the future, and it caused me to feel anxiety and stress. They started to give me details of advanced care planning, which entailed a discussion of topics such as feeding tubes, tracheostomy and resuscitation. All of these subjects were scary to broach and as I have not often been someone who looks forward too far, being faced with potential future problems was slightly upsetting, more so than I thought it would be.

As Duchenne muscular dystrophy is a progressive disease, issues that come with it are inevitable so the described ideas and procedures were not much of a surprise to me, but they were still very worrying nonetheless. Because I had gone into the meeting not expecting conversation, it probably hit harder considering just how significant having these medical procedures can be to your life. Perhaps the biggest source of anxiety is thinking about the choices. Making decisions about what to put your body through is a really big thing, especially when you are healthy currently. The ball is put firmly in your court and you must make the ultimate decision. Also, there is no definite timeframe for problems to arise, so ‘when’ becomes another factor as there is no way to predict the future. Thinking about “what ifs” and “when shoulds” can be a lot more difficult than one would think. I would not usually consider myself as an anxious person, and even though there was no pressure on me to make any decisions there and then, it certainly gave me a sinking feeling and it was still slightly hard to hear having not thought too far ahead as a usual matter of course.

The discussion was to give me more information about my future. Having that information is useful in order to make informed decisions and I definitely do see the benefit of talking about future issues in good time, before they become a problem. Also, making sure your wishes are set out properly in case they are needed is important too, for myself and others. Having said this, I think it is very important to approach certain subjects with a large amount of sensitivity and it is also important not to make people feel like they have been ambushed in anyway.

Food I miss

Food has become more difficult for me to eat and the vast majority of my meals are now blended and put through a sieve to make it easier. Losing weight is a big concern for someone with DMD and has to be maintained. Not being able to eat whatever I want is a challenge, it’s not just the concerns about weight or choking but also that it can get me down mentally. However, we always try different dishes to see if they can be liquidised or made in a way that I can manage. It isn’t quite the same as eating something whole but it can still work.  I generally have a positive attitude towards this part of my routine but there are certainly foods that I miss eating, and I want to share some of them with you.

Rice – This may seem like quite a boring one but I did used to enjoy eating rice-based dishes like fried rice with vegetables or chicken and rice. It does not go through a blender very well and likely would not make something with the right consistency for me. Carbs like rice and pasta cause this problem so I steer clear from them.

Sausages – They were one of my favourite things to eat. It could be on the BBQ, a fry up, in the oven, sausage and mash, toad in the hole, these are all great things that I really enjoyed. I definitely crave a sausage sandwich with ketchup on occasion. However, I do still eat sausages as part of my diet albeit in blended form as it works well in soups or casserole.

Fish – This is a more difficult thing blend and is much less enjoyable after so we tend to eat it less. We used to eat fish a lot and I would normally pick it when we went to restaurants. I really miss fish and chips as well. But again, this doesn’t mean that I can’t eat it at all in different ways. I have had fish in different sauces and also as a mousse so it can work even though it isn’t quite the same.

Withings ECG watch – my review

I was recommended by my doctor to buy a watch that could record and take an ECG if I needed. I got the Withings ECG and here is my short review of the product.

It cost a little over £100 which feels maybe a bit expensive but it can be used to record a number of different measurements. These include steps, tracking workouts, heart rate, and an echocardiogram. The watch is used alongside an app on your phone and that was fairly easy to set up and is very intuitive. The battery was dead when I tried to use it for the first time despite a supposed 12-month life, but that may have been caused by not using it for a number of months. The battery was easy to change after finding a YouTube tutorial.

The watch itself is very simple with a basic clock face and another dial which illustrates when the ECG is in progress. It is lightweight and very comfortable to wear all day. There is only 1 button to press to start an ECG and you can take readings on the go with or without your phone nearby. It uses Bluetooth and syncs to the app each time you open it. A big issue for me is that to take an ECG requires placing your other hand over it and touching the edge of the watch for 30 seconds. This means that I need help to use it but that would normally be the case for me regardless.

You able to watch the ECG in progress, view the results as a pdf and send them to your doctor from the app once you set up an email, although this part could have been made easier. It tells you whether your sinus rythym is normal and gives your heart rate. It would be useful if you were able to download each ECG when I log in on my computer to make the process easier unless I have missed an obvious option.  The watch and app can store a lot of information which is another plus point. Overall, it is a solid product and does the job as a smart ECG watch.

Universal Credit – My experience

Recently, I started claiming for the Universal Credit benefits. Applying for Universal Credit was quite a saga for me and my family, and I want to share my thoughts and go through some of the parts of the process. The application can be difficult to navigate in places and there are several obstacles to overcome as someone with Duchenne before you can receive UC benefits. It took nine weeks from the time I applied to when I received my first payment. Fortunately, I was not in a situation where I desperately needed the money but this could certainly be a problem for other people if they had to pay for carers or other necessities.

In order to begin claiming you need to create an account. This seems straight forward, but I encountered an issue at the very beginning with proving my identity. I needed three items and there were limited choices of documents to use as proof on the UC website. I do not have a driver’s license or my own mobile phone contract for example, so someone had to come to my house in order to prove who I was. To further complicate matters, my father is authorised to act as my appointee (to help me with things like signing documents) so we also needed to prove this too.

After setting up an account, there are several forms to fill in and documents to send in order to get the ball rolling. One problem with the website is there is nowhere to select what specific benefits you are applying for which seems to unnecessarily complicate the claims process. Once I began the claim, I needed proof of my disability. It was necessary to provide a note from a doctor to prove what medical condition is preventing you from working. I then had to fill in the ‘work capability assessment’ form, a very long form detailing your everyday activities and what you can and cannot do, within a month.

Despite already going through the process for Personal Independence Payments, which entailed filling in a very similar form about day to day living, and having qualified for the higher rate, there is no way to show that my disability has already been verified by the Department of Work and Pensions. As Duchenne does not improve and has no ‘cure’ currently, it should not need to be assessed twice or more. Therefore, I think it would be good if the DWP could check your history and check medical conditions before asking for the same documentation multiple times.

I had to complete an interview over the phone as well to go through a further assessment. Completing the forms and sending the doctor’s note before this interview turned out to be very useful as I avoided having to repeat certain answers about how you are disabled and what problems you have. At the end you agree on certain commitments such as attending medical appointments, and then I began receiving payments.

Sometimes there is a need for a face to face interview, but my answers on the assessment form were sufficient to show that my condition qualifies for the benefit, meaning I was placed into the ‘limited capability to work’ group without having to be seen.

Hopefully this post gives people a better idea of the process. Please leave a comment if you liked it.

Using Mouth Piece Ventilation (MPV) – Non-Invasive Ventilation

I have been trying out MPV over the last week and I want to share my initial impressions.

Firstly, I should explain my usual use of ventilation during the day. Normally, I use a face mask connected to my NIV machine during the day when I need a bit of help with breathing. This is around 2 to 3 hours per day, mostly after meals when I feel a bit full and need a boost.

With MPV, I’m using a small straw to take in air instead. When I suck the straw, it activates the machine which pushes in more air and gives me a bigger breath. The small size of the straw helps to increase the pressure of the air going in, increasing the volume to give me a bigger breath. The NHS has provided me a Philips Respironics Trilogy, which is quite a new product.

So far, I have found MPV to be very good at giving me deeper breaths. It works better than the face mask even though it takes a little more effort to activate the NIV. It feels that I get just as much relief and comfort from the straw, and I have been able to adapt to using a mouth-piece very easily. Not having to wear a face mask is definitely a plus because I can keep talking without sounding muffled or quiet. Furthermore, it also made a positive impact in terms of my breathing. I had a blood gases test after four days using the MPV which showed a decrease in CO2 retention and very high oxygen saturation.

Additionally, there is an option to attach the machine to my wheelchair so it would be portable and I could get the benefits of the NIV when going out and about. Hopefully, there will be a practical solution because I think it would be great to have.

Regular ‘Maintenance’ physiotherapy for Duchenne

I am of the opinion that having regular physiotherapy is an important part of managing muscle-wasting conditions like Duchenne. I have had physiotherapy for a number of years and I think it has done me a lot of good. I am a firm believer in its usefulness for Duchenne patients to maintain the condition of limbs, joints, and muscles. I have had physio regularly between the ages of 11 and 24.

It was provided especially when I was under paediatric care throughout primary and secondary school, and took place every week or so. When I was younger, physiotherapy wasn’t necessarily my main concern and it did seem like a drag. In primary school for example, I remember occasionally trying to avoid having physio by hiding with my friends or pretending I had forgotten what day it was.

But, I came to realise how important it was to keep me feeling comfortable in my wheelchair and keeping me flexible enough to be able continue to do everyday activities such as putting on clothes easily. However, the amount of physio I have had has decreased over time, and now I moved completely into adult care services I no longer have any at all. It was explained to me that the support for regular maintenance physio no longer exists. You are still able to see a physiotherapist if you have specific problems, but for the most part you have to do it yourself. Although this is manageable, it would be great to see more professional support for adults with Duchenne.

This again seems to come down to funding and availability. In my experience, child services are well funded and prioritised (and are excellent) whereas adult services drop off almost completely. It is baffling that services fall so dramatically for adults considering that conditions like Duchenne progressively worsen and need more help as you get older.

I have read articles on a number of websites that discuss the importance and benefits of physiotherapy for muscular dystrophy patients to prevent contractures or reduce pain. A quick search also reveals a lack of physio services for neuromuscular conditions across Britain. Having more services for adults with muscle-wasting conditions makes a lot of sense to me. Physiotherapists are very important in this regard, and I think that greater support for and also access to these services is necessary.