This can be a problem for me and I have had some experiences of really painful cramping and trapped wind, potentially from sitting down for most of the day but we have never found a definitive reason as to the cause. It does not happen often, but when it does it has knocked me out of kilter for a couple of days.
The first time I had severe bloating it caused lots of pain, my stomach was distended and hard, I couldn’t eat and the gp even suggested hospitalisation because of it. Fortunately, the problem went away on its own but it is important to manage as the problem can be very acute. We have since found out and come up with strategies to reduce the problem.
Lying down and using a hot water bottle were two things that have provided some relief when an episode occurs. Taking a medicine called Mebeverine has proved to be very effective for me on the last two episodes of bloating. It was recommended and prescribed by my gp so it may be worth asking about it if you have problems. Also, I did alter my diet to have more beans and legumes but it is difficult to know how effective it is.
I have been feeling increased levels of anxiousness recently, probably increased over the last two year by problems like the pandemic and my own health. Being concerned about my health and possible difficulties in the future has brought with it some worry and fear about the future. To counteract these negative feelings, I have been using mindfulness for the first time. I have been trying meditation to help turn negative thoughts and feelings around. It has only been three weeks so far, but I do think it can definitely help me going forward. Accepting that negative thoughts are just thoughts and nothing more, and having a sense of calm and letting go has been good for me. Short periods of meditation also don’t take too long and still provide the benefits which is another good thing.
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.
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.
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.
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.
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.
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.
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.
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.