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.
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.
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.
Due to weakening of muscles over time, breathing and coughing can become a little more problematic as you get older. Doctors begin taking a greater interest in your respiratory system and you have more check-ups. Respiratory clinics may seem worrying at first but they do not need to be. Having consultants and nurses describing your breathing and respiratory function can feel nervy at times but after a while it becomes just a normal part of life.
I use two main pieces of equipment to help with my coughing and breathing but they are not at all scary so don’t be put off or disheartened when you first come into contact with them. I have a ‘cough-assist’ machine, which increases the strength of the cough reflex, and an NIV (Non Invasive Ventilation) machine that helps with breathing overnight and occasionally in the day. Both probably sound like serious and complex medical treatments, but the reality is they are simply useful pieces of kit that give your lungs a boost.
I now know that these machines are a common treatment for Duchenne patients but initially I was completely nonplussed, so don’t feel unnerved by them. For example, the first time I was presented with a ‘cough-assist’ machine it felt like a massive deal and I was very hesitant to use it as it presented a negative change. However, surprisingly I became accustomed to it very quickly and it has actually been incredibly useful whenever I have had a chest problem.
One of the major issues related to Duchenne that I want to discuss is scoliosis. This is when the spine develops a curvature to the side which normally affects people aged 11 to 15. Scoliosis can happen for many different reasons, but young boys with Duchenne can be particularly susceptible to it as I discovered. It was one of the most significant health problems for me that I have experienced in my life so far.
For me, the curve developed when I was 14, gradually worsening until I was eventually sent for an x-ray. The curve was about 67 degrees at its worst point and required surgery. Before I found out about the extent of the damage, some of the physiotherapists I was seen by suggested solutions to try and correct my posture, such as a chest harness for example, but at the time I did not want to be restricted physically as it would have prevented me from doing things I wanted to do. Despite this, I feel like it should have been impressed upon me and explained more clearly as nobody had really highlighted scoliosis before I was told just how far it had gone.
I had surgery just after my fifteenth birthday which was successful despite me ‘mis-behaving’ on the operating table according to the anaesthetist, whatever that meant. However, I think that much more attention needs to be drawn to the potential of scoliosis as soon as possible because it can have a huge impact on your life. Mine was lucky to be caught and treated in time but it should never have reached such a dangerous stage. It probably could not have been entirely prevented but doctors should definitely give their patients some more information when it comes to this.
I also think that spinal surgery should be considered earlier than it currently is, to make sure the opportunity is not missed. Not treating a curve could stop people using their wheelchairs which would be devastating for someone with Duchenne. If I was given surgery sooner my body position would be much better than at present. Surgery certainly should not be delayed for any reason, and especially not because of petty disputes between consultants and surgeons, for example.
Hopefully this information will be useful and give some food for thought. I was taken by surprise when I found out about the scoliosis but I don’t think others should be, so don’t be afraid to ask about it.
There are a number of health issues associated with Duchenne that I feel could have been highlighted to me earlier or that would have been useful knowing about sooner. Finding out about the condition can be a scary prospect so is something I have never been particularly interested in. I haven’t often researched the potential long-term implications of Duchenne in detail, but it would have been good if some things were explained to me earlier by the health professionals I came into contact with. Some knowledge can go a long way in reducing some of the stress and confusion that comes with a new challenge.
Because of this, I want to give details about some of the issues I have come into contact with and offer my own advice. Hopefully it will give some welcome reassurance and a bit of information about Duchenne. Remember, each problem you face can be overcome and sorted out, so don’t ever feel too dejected! As my Mum often says, ‘one day at a time.’