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 the 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.

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.

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.

Chest supports

This post is about having and using a chest support when sitting in a wheelchair. When I sit in my wheelchair I need to have a support across my chest to stop me falling forward because of limited trunk control. I am not very stable when sitting so the strap holds me in place and gives me extra support. This is totally normal to me but I have not always had to wear a chest support.

The first time I was introduced to one was in secondary school and during that time I was completely against even trying it. The support was more akin to a racing car harness seat-belt that goes over your shoulders and clips on your chest but with a zip at the front. In school, I had much better torso control when sitting down and I would also lean down to the desks to write in my books, but the harness prevented me from moving forward at all.

At the time, it felt too restrictive and as a fiercely independent person it just seemed like it would limit my abilities. I also worried about what it would look like and what other kids might think, and it was enough to completely put me off chest supports for a while at least. I think I tried it for about 5 minutes and said ‘this isn’t going to work’. Whether it could have improved my posture or delayed my scoliosis who is to say, but as a younger person it just did not seem or feel in my interest.

Now I am older, I wear an elastic chest strap all the time in my chair and have been doing so for about 5 years now. Having an extra support is definitely a useful thing when it is needed. I see it as a positive thing for me even rather than a negative, even though it was initially something I was worried about. It holds me up which keeps me comfortable, maintains my sitting position and allows me to better use my arms for driving or using a mouse. Also, it is not completely rigid so I still retain some movement.

Dealing with aches and soreness

Duchenne can cause you all sorts of aches and pains which vary a lot from person to person. I have had various problems with aches and pains myself, the main culprits being my legs, hips, back and shoulders. I have also had other problems with pressure which causes soreness, leading to more obscure ailments such as painful heels. Aside from using painkillers, I have found a number of simple (I prefer ingenious) solutions to deal with some of the pains which I think are worth sharing.

One really useful thing I have found to use is pieces of foam to counteract pressure areas. A good example of this was my ear because I can only sleep on one side. My ear was becoming very sore and the skin was threatening to break down, but we solved this using a thin piece of foam with a cut-out, so my ear does not directly contact the pillow. Similarly, when sleeping I put a lot of pressure onto my knuckles and that has been sorted out in similar fashion.

Foam also has other uses. I use a chest support strap that was causing pressure against my ribs and a bit of foam underneath completely counteracts that soreness. Also, I recently had been having a painful ache in my left thigh but that problem was alleviated by placing another bit of foam under my knee. Another way I have found to combat discomfort is using strategically placed pillows and soft materials to prevent skin from becoming red or helping reduce general aches.

Performing some general exercises regularly yourself can be helpful as well. I try to regularly stretch out my legs when lying down and to exercise my fingers or my neck to help maintain movement. It may not seem like much but I have found it to be beneficial in the long-term.

This all sounds straightforward although it seems useful to tell others what has worked in my experience, because sometimes I have found that nobody tells you information before an issue has developed into something worse.

Services and care for Adults

As you enter adulthood, the amount of services for people with Duchenne appears to decline. From my own experience, the services became more disjointed and some elements of care lessened. I have found that Paediatric services were much more well rounded and encompassing than they are under Adult care. I feel that services for adults should be improved to match those you receive as a child, and this involves care such as physiotherapy and occupational therapy.

Before I turned 18, there was weekly access to physiotherapy, a regular review of either six monthly or yearly with a specific consultant, and easy access to a ward upon being admitted to hospital. This provided excellent coverage of my general health in most cases, even though I would occasionally scoff at the strange nature of review clinics as I saw them at the time. This has changed as I have moved into Adult care, following  a transition period of a few years during which time I saw the same paediatric consultant. Today, I find that the services which provide my care are more disjointed and more separate, and I feel that it could be beneficial to make them more cohesive. This may be a result of problems with funding or planning but I still feel things can be improved. Nonetheless, the level of care I receive is excellent, as are the doctors and others that administer that care.

I recently raised concerns with my health board via a letter in which I asked about creating combined services, providing better care plans and more physiotherapists for Duchenne patients. I have since had a reply which did give me some reassurance, but I am still left thinking that more can be done that would improve services and care for many people. It remains slightly odd to me that someone with a condition that worsens over time would receive less care as they got older.

Medicine

As someone with Duchenne, I have to take various medications to manage particular symptoms. Despite needing more medication as I have got older, I still only have a couple of tablets each day, all of which have been prescribed by doctors and consultants. These deal with my heart for the most part but I also take Vitamin D supplements. The heart medication isn’t entirely out of the ordinary but it helps with the everyday working of the heart and its long term health.

The Vitamin D is good for people who are inactive or sit down most of the time, like me, which is why a consultant prescribed it after an x-ray showed I had mild osteoporosis. It may be useful as a Duchenne patient to ask about Vitamin D earlier to see if it would be beneficial. I wasn’t really aware of it until my late teens so it’s something to find out about.

Antibiotics are also an interesting subject in terms of Duchenne patients due to the greater tendency to develop chest infections and pneumonia. Antibiotics are often mentioned in the news and other media, but usually in a negative context to do with their over-use or resistance. For people with weakened respiratory function, however, antibiotic use is more easily justifiable to counter serious bacterial infections that are potentially dangerous. I was once told off by a GP for taking ‘rescue’ antibiotics when I had a very heavy cold and cough, but a respiratory consultant told me otherwise.

That’s not to go completely against medical advice but some men with Duchenne take antibiotics on a regular basis, so I would say trust you own instincts when it comes to chest infections. You and your family will know more about you and your well-being so you can use that as a gauge for when to intervene or get advice. I’m not a doctor though, so don’t take this as gospel.

Eating

I would not describe eating as a big health problem as such, but it is still important to keep yourself at a healthy and manageable weight. Because of the muscle problems that come with Duchenne, ordinary day-to-day activities, such as breathing, require large amounts of effort and energy so maintaining a good calorie intake is essential. Eating can be a very tiring activity which is therefore a potential issue for your health once you reach about 17 or 18.

In comprehensive school I had very few if any problems with eating and drinking because I still had the ability to feed myself easily, being able to fully use my arms and hands and not tiring. Once I got to college however, I had less chance to eat and it became more energy sapping. I did not consider this to be a problem but slowly my weight was dropping off pretty much unnoticed.

At this point, I was still feeding myself and enjoying my food but in hindsight I was eating much less simply because of tiredness due to the effort of chewing. I would also say that I was quite stubborn so eating myself was a demonstration of my independence. This carried on until I went to one of my usual health check-ups aged about 19, and discovered to my complete shock and dismay that I was just under 6 stone. There was a period of about two years when I had to see dietitians and take high calorie drinks (Fortisip if you’re interested) to get my weight back up, which incidentally is now about 58kg at last count. In that period it was suggested I might even need a PEG feeding tube but this seriously motivated me to eat more. It wasn’t as if I didn’t want to eat as I love food but it was just more challenging.

For the last two or three years I now have most of my food blended in order to maximize the amount I can intake, and I don’t feed myself these days. Despite this, what I eat isn’t like baby food and I am able to have things I enjoy like steak, cauliflower cheese and roast dinner. If you are interested please check out @battle_the_bite on intagram which is run by my sister where she posts some of my meals.

The main things to take from this are to be aware of how much you are eating and don’t be embarrassed or feel down if you need help eating or have pureed food. Also, always eat what you want and as much of it as you like.