Travelling with MG

This time I am going to write about a real trip – not a metaphorical one. I’ve recently been to Berlin, Germany and my MG behaved really well. The fact that I started the increased dose of Mestinon shortly before that helped a lot. I have to say that I was quite anxious before this trip after I had to cancel my holidays abroad over the Christmas break (just before being diagnosed). At that time I didn’t know exactly who is my travel companion but now we got to know each other quite well despite the constant unpredictability of my illness. I am not going to bore you with the details of a trip which I enjoyed to the maximum together with my partner. I would like to take advantage of the hundreds of pictures I took there and share some fragments of my vision affected by MG. The picture which I processed in Photoshop shows actually how I see when I look straight ahead. There is always a degree of ‘doubleness’ and blurriness which can vary from one minute to another (literally in the blink of an eye). It is sometimes so bad (especially when I walk) that I can’t cope without an eye patch when that happens. I've noticed that the gap between images usually increases if I look at my left/right so maybe you imagine how messed up it can get. Luckily I need to close one eye when I look through the viewfinder of my camera so that comes quite handy! 

More Mestinon...

My next stop on the MG route was at a consultant neurologist specialized in muscle. This happened almost two months after I started the treatment with Mestinon. Apparently the guy had a ‘special interest’ in MG which gave me hopes to find out some more information about my illness from a more credible source than the Internet. Once again I haven’t been seen by the neurologist himself but his assistant was really cooperative and answered all the questions I’ve carefully prepared prior to my appointment. However I can’t say that I was overly happy with the answers as many of them were quite vague. To me that reinforced my worries that the doctors I’ve consulted so far might have a ‘special interest’ in this medical condition but none of them seemed to be specialized in MG. Maybe this explains why I’ve been passed from one neurologist to another every time I’ve been booked in (not to mention the ophthalmologists in between). I dared question their expertise in treating people with MG and kindly asked if I could be seen by someone with more experience in dealing with this illness.

As the treatment I’ve been prescribed did not seem to work for me so far, during the same appointment they decided to double the dose of Mestinon. That means that I am now taking 3 x 60mg per day compared to the previous dosage (3 x 30mg). I’ve been recommended to start with a full dose only in the morning (and keep half the dose in the afternoon and evening). I have tried this approach without any significant improvement after one week. As there were no serious side effects involved I decided to go for the full dose straight away. The good news is that there seem to be more frequent spells of ‘normal’ vision (at least when I look perpendicularly). This happens especially one hour after I take the medication and lasts for about 3-4 hours when the beneficial effects of the medication tend to fade away. Despite this improvement, my vision continues to fluctuate immensely which means that it can get suddenly blurry and/or double depending on distances and the angles I am looking at. However, the fact that over the past few days I coped without my eye patch most of the time made me feel happier and much more confident. I am aware that it can change anytime but instead getting worried and stressed about it I just try to enjoy as much as I can the time I am able to do things pretty much normally.

The doctor who has just seen me told me that he didn't think that Mestinon is going to work and asked me to consider going on steroids (immunosupressants) or more specifically Prednisone. I anticipated this route and luckily I’ve done some research around it over the Internet and asked other patients with MG about this line of treatment on various discussion boards. I was personally put off by the long list of side-effects especially psychological ones (behavioural changes, depression, hallucinations, memory problems etc.). I am aware that everyone’s reaction to medication is different and it is difficult to predict the side-effects beforehand. An argument with the doctors on this issue would have been certainly out of question if I didn’t have a choice. The choice apparently is the thymectomy I was writing about in one of my previous entries. I gave it a thought in the meantime and a good reading and concluded that I would rather prefer surgery to steroids. I have the feeling that surgery will happen sooner or later anyway. Sooner would be better as I am still in a fairly good shape both mentally and physically (my condition has not generalized yet as the symptoms are limited to my eyes only). In the first stage I was told that my thymus was too small to be operated and therefore thymectomy was not recommended. I insisted anyway and asked for more explanations. I was not particularly happy with some vague descriptions like ‘small thymus’. Is thymus supposed to show on a CT scan at all?! I’ve read about many cases of people whose scans were normal and after thymectomy their thymus was huge and inflamed (and sometimes cancerous). The doctor confirmed that most likely the illness is related to my thymus but still he was reluctant about going further with surgery (without any solid arguments). 

Anyway it looks like I’ve made my point clear enough as shortly after my appointment the doctor gave me an unexpected call. He said that considered what we had discussed and would like to further refer me to a MG specialist in Oxford which happens to be one of the best MG centre in the UK. Apparently they perform video-assisted thoracoscopic surgery (VATS) - thymectomy. This procedure requires only very small incisions compared to the old-fashioned ‘open’ surgery which can leave a huge scar on the chest. I have the feeling that this is a step further and certainly good news for me.

I haven’t received any confirmation yet of when and where and with whom the next appointment is going to be. The only letter I’ve received so far was from the consultant neurologist I’ve just seen. Apparently he has booked me in in exactly one year’s time! I hope this is going to be only a follow-up appointment and I will hear from them sooner than that... 

The magic gland

Some things seem to be real only when we are children and tend to disappear by the time we become adults. Thymus gland may be one of them. It grows until puberty and then begins to involute in size and activity as we age. At least it is supposed to unless something goes wrong along the process. For instance, if thymus gland gives incorrect ‘instructions’ to immune cells this sometimes results in autoimmunity. For MG which is an autoimmune disease this means the production of the acetylcholine receptor antibodies which attack the neuromuscular transmission and cause fatigue and weakness in voluntary muscles.

I have to admit that I haven’t heard of thymus before being diagnosed with MG. It seems to be strongly related to MG although the connection between the two remains unclear. Most of the individuals with MG have abnormally large thymus glands which need to be removed before they progress into a tumour called thymoma (which sometimes can be cancerogenous). The surgical removal of the thymus gland is called thymectomy. This procedure proved to significantly reduce symptoms in individuals with MG (with or without thymomas). Some research claims that 70% of patients undertaking thymectomy reported reduced symptoms or even complete remission after a number of months/years.

Shortly after I have been diagnosed I was referred for a CT for chest (where this gland is located – just below the neck, behind the breastbone and in front of the heart). I have just received the results and I am not sure whether it is good or bad news... I just take them as they come in a detach way most of the time. The scan has NOT shown any particular abnormalities or at least not to the extent that requires surgery: "CT scan of the chest shows a small degree of tissue in the anterior mediastinum. This may simply represent a small remnant of thymus gland. Whether it is relevant to his symptoms of myasthenia is unclear. Certainly with myasthenia limited to the ocular muscles we wouldn't tend to advocate thymectomy...". However I have read about people with MG who had their thymus gland removed even if it wasn't enlarged (or at least wasn't visible on CT/MRI scans). I don’t even want to think about it right now as I need to work out my beach body for my exotic holidays later this year! Leaving the joke aside, I definitely have to ask more questions about this (and many other things) during my next appointment with my neurologist in a couple of weeks… 

How does it feel?

When my partner has first read my introductory post thought that my feelings in relation to my illness did not transpire very well in my writing. It might be true as I usually tend to detach myself emotionally when an experience becomes too overwhelming. And somehow I managed to reflect this by writing here more about facts than feelings so far. But like any other physical illness, MG certainly goes hand in hand with a variety of feelings most of them negative.

First I was feeling very confused about the unusual sensations in my body especially my sight which bothered and continues to bother me a lot. This was a lot to take for someone who has never had any serious illness and has never been on any kind of treatment. As it took few months to be diagnosed I spent all this time wondering what was wrong with me. I’ve been waiting for doctor’s letters and results for different tests more than I have ever waited for my Christmas presents. And if someone asked me what I wanted for Christmas last year probably it would have been a diagnosis – a name for what I was experiencing, just to know for definite what I am dealing with and eventually get some treatment which would hopefully make me better.

I am a naturally born anxious person so it was not too difficult to build up a great deal of anxiety which culminated with some panic attacks shortly after the onset. I was completely terrified of what was happening to my vision and couldn’t stop thinking about the worst scenario. It could have been a brain tumour, multiple sclerosis or … death. I was feeling that something died in me and I grieved a lot over the person I used to be. I missed my old self and still missing it a lot… There are so many things we all take for granted and one day we don’t have them anymore. That is the moment when we realise how much we missed these things which we are probably not grateful enough for. Grief and sadness shortly developed into depression which hits me every now and then (about once a week at this stage). I am not diagnosed with depression and I don’t have any treatment for it as I haven’t disclosed my feelings to anyone except my partner and some close friends. And believe it or not I am working with mental health problems so I should know better than anyone that I am playing with fire…

The fear is always there. The fear that things could always get worse. That my MG might get generalized one day. That I won’t be able to look after myself. That I would be a burden for others. That I have to leave my workplace which I enjoy so much. That I won’t be able to travel. And the list goes on and on... I hardly can go back to sleep for instance if I woke up in the middle of the night with my arm or leg numb. It could be the next phase of the illness when my limbs will get weak! Or maybe I’ll stop breathing… I sometimes feel short of breath and I can’t tell if that’s because of the tiredness, anxiety, side-effects of the medication or my muscles getting weaker. I’m always over-alert and I am aware that I am probably too conscious about all these new sensations in my body.

Physically I don’t feel any major changes except my vision which went completely berserk over the last four months. Double, blurry, or both, variable with distances… So variable that it may be normal for one minute (most likely if I’m looking straight ahead) and then unfocused again. So variable that I’m sometimes afraid that people would think that I’m making it up or something. The truth is that I can’t cope without an eye-patch at the moment and this took me quite a long time to get adjusted to. Not that it wasn’t helpful but somehow it was like a disclosure of my disability. I was feeling so embarrassed wearing it and people staring at me all the time (or at least that’s what it felt like) but it proved to be helpful in the end and it is one of the few things that help me at this stage. Obviously I struggle with the depth perception and my balance is not so good as it feels like I’m drunk most of the time (some days worse than others)…

But at least I am not feeling guilty. Some people may have unhealthy lifestyles which lead to illness and they bitterly regret after. I tried and still trying my best to keep a healthy lifestyle with a healthy diet and regular exercise, no smoking or drinking (not even coffee), spending my time meaningfully and productively. And I hope that all these things – which I appreciate more than ever – will eventually help me to get through this nightmare…  

Mestinon

The same day I’ve been diagnosed with OMG I’ve been prescribed Mestinon 60mg which appears to be the first line of treatment for MG. It contains an active ingredient called pyridostigmine bromide. This helps prolonging the action of the neurotransmitter called acetylcholine affected in MG, whose role (among others) is to transmit nerve messages to muscles and cause them to contract. In other words, pyridostigmine increases the chances of a nerve signal being successfully transmitted to the muscle. 

The beneficial effects of the medication seem to fade away in few hours. This is the reason I have been recommended taking half a tablet three times a day (30mg x 3). I usually take it 30 min. (up to one hour) before the main meals which means 5-6 hours apart. I have read on different discussion boards that some patients take this medication with food or milk as this may help to decrease the side effects. I personally didn’t find this necessary. The side effects did bother me but only during the first days of the treatment. My stomach was terribly upset and I experienced frequent abdominal cramps and diarrhoea. I’ve noticed that this happened especially after I had rich or spicy foods so I needed to adjust my diet and be more careful with nutrition. As a result, I now manage better the side effects of the medication (except some random muscle twitches which are still there since I’ve started the treatment) as my body successfully accommodated the new substance which was expected to improve my condition.  

I did notice significant improvement shortly after I started the treatment. To be honest, few days before I actually started taking Mestinon my vision was much better when I was looking straight ahead (but not down/on the sides). Following my ‘experimentations’ (patch on/off, looking at different angles/distances etc.) I discovered then that I was coping okay-ish without an eye patch in most of the situations except tasks that required some precision e.g. reading, working on computer etc. This occurred after more than one month of almost constant double/blurry vision which was a great relief. 

Unfortunately my joy was not going to last too long as after only a couple of weeks since I started the treatment my vision got worse and I had to stick my eye patch again most of the time. I was feeling defeated but I had no choice… This was probably going to be just one of the many variations which characterise this disease.  I don’t feel that the medication does the trick at the moment at least not in the dosage I was prescribed so I am going to contact my neurologist soon and seek advice. 

My vision continues to play games and does not seem to stabilize after more than one month of medication. Despite predictions rest does not seem to help either as it feels pretty much the same in the morning even if I had a good night sleep. On the contrary it sometimes seems to be better in the evening when tiredness is expected to actually make it worse. It definitely does not make too much sense to me as there is no visible pattern and all this confusion drives me crazy sometimes…  

Diagnosis: Myasthenia Gravis

In less than three months since the initial symptoms, I’ve been diagnosed with Ocular Myasthenia Gravis (OMG) and have become overnight part of a small 'nation' of approx. 350,000 myasthenics (according to a prevalence of approx. 5 per 100,000). The diagnosis was based on a blood test called acetylcholine receptor antibody titer. Apparently 90% of the people diagnosed with MG have such antibodies detectable in their blood (while for OMG patients the percent is only 70%). This was the first and the only test performed – twice as the first time it was not performed correctly which delayed my being diagnosed with one month. However, I consider myself lucky to be diagnosed so soon as I understand that for some people it might take several years to get diagnosed and prescribed treatment. To note that my neurologist added to the diagnosis the fact that my condition was mimicking the Internuclear Ophthalmoplegia (the initial diagnosis) which might be a sign that they are still considering other causes for my double vision (although the MRI scan I had a couple of months ago eliminated the even scarier scenario of having Multiple Sclerosis).

The pathophysiology of OMG is not known to be different from the generalized MG. Basically anomalous antibodies block acetylcholine receptors with a role in activating muscles and inhibit the effect of the neurotransmitter acetylcholine at the junction between nerves and muscles. This results in muscle fatigability isolated to eyes (OMG) or affecting other groups of muscles (MG) for example muscles which control facial expressions, swallowing, chewing, breathing, limb movements – scary isn’t it?! Eye muscles seem to be susceptible to this disease. If weakness in a limb may pass unnoticed a tiny little error caused by weakness in the eye muscles would lead to the misalignment of the two eyes resulting in blurry or double vision. Different immunologic hypothesis propose that antibodies in OMG may be different than those involved in MG but further research is warranted to confirm this line of thought.

Out of the main symptoms listed for the OMG I experienced only diplopia. This initially occurred when looking down or to the side – significantly worse to the left – and after few weeks it generalized to all directions. Ptosis (drooping of one or both eyelids due to muscle weakness) is another symptom frequently reported by patients diagnosed with OMG which I have personally not experienced yet. I have to admit that at times I was under the impression that my eye lids are not symmetrical and I was terrified of the thought that my eye lid will drop soon. This may well be the result of too much reading about this condition on the Internet and expecting symptoms to exacerbate – which at this stage did not happen (Thank God!).

According to most of the web resources (more or less reliable) I’ve consulted so far 75-85% of all patients already diagnosed with MG initially complained about symptoms relating to their vision (more frequently in females). However, in OMG men seem to be more frequently affected. The average onset for MG is 33 years while for OMG is 38 years. As I was 34 years when I first developed the double vision I am closer to the onset age for generalized MG. However the fact that I am male statistically increase my chances to marry the OMG! It looks now that I am using statistics much like a drunk uses a lamppost – for support rather than illumination (as Vin Scully said) but I am definitely not losing hope that I might be among the 15-20% of the lucky patients whose MG won’t generalize to other groups of muscles. It might be a long waiting but the good news is that if I continue to have only ocular symptoms for three years, there is a good chance my symptoms will not increase!

New beginnings

How would you feel if you woke up one day like a completely different person with an identity you knew nothing about? This has actually happened to me... My story begins one morning in November 2013 when I woke up in a nightmare. The world around appeared completely different simply because my perspective was radically changed – literally and metaphorically as well: I was seeing double! As I haven’t had any problems with my vision in my entire life, I blamed the tiredness and I was sure that it will eventually go away - which did happen but only for a short while...

For the first couple of weeks the double vision episodes lasted for only about 10-15 minutes first thing in the morning. Gradually the images came into one and I was back to normal. I was completely freaked out and went to my doctor urgently. They referred me to an optometrist which performed a series of routine tests just to conclude that my eyes looked healthy and there didn't seem to be any ophthalmological issues. Good news but bad news was just about to come...

More investigations started on the neurological side after repeated blood tests for thyroid and diabetes (potential causes for double vision) showed no abnormalities. There was a long list of other possible causes some of which were life threatening such as brain tumours or possibly a stroke. Doctors had soon eliminated all these with a series of scans (X-rays, computed tomography and angiogram) performed at the A&E department in one day. It was the day I actually realised that something was going really wrong in my body. My mind became very distressed as well. I had a panic attack earlier that day which didn't help clarifying the symptoms as I started to feel pins and needles in my left arm and face (which could have been as well related to high anxiety levels triggered by increasingly upsetting double vision episodes). This was because the double vision was there all the time now especially worse when I was looking down or left. In another couple of weeks it deteriorated to the extent that I couldn't see clearly when I was looking straight ahead.

Doctors recommended me wearing an eye patch as I was able to see perfectly with either my left or right eye (known as binocular diplopia). First I was too stubborn to wear one maybe because I was still in denial and was hoping that things will get better eventually. Or maybe I was bothered by the thought of appearing in public with an eye patch (ah, appearances, and jokes about cyborgs, pirates and so on...). The aesthetic reasons faded away shortly and after another panic attack I realised that I couldn't cope without an eye patch. Honestly, I couldn't wink forever to people just to make sure I see them normally! Only if that helped... First I tried the surgical patches which irritated my skin after long hours of wearing them. Then I tried to attach a fabric patch to my glasses (which I started to wear only for this purpose). I purchased this from the Internet and it seemed to work better and it didn't press my eye too much. I am still wearing it after more than one month now and almost got used to it. To note that I need to cover my right eye otherwise if I cover the left one I can't keep my balance when walking. The supplier provided me with the wrong colour but trust me this was not an issue anymore! It was already the New Year and a new look was on its way...

I'm very passionate about photography so if you are familiar with DSLR lenses then you will understand the comparison between a manual focus and my vision. It felt like someone was changing my ‘lens’ settings all the time. It could have been clear for a minute then it went double again or blurry or a combination of those. It varied for different distances which made things even more confusing and disorientating (especially when I was walking). Most of the time was a ‘bokeh’ effect especially at night. Some people pay good money for Photoshop but I had mine attached to my brain and the ‘special’ effects did not stop to mesmerise (and terrify) me... 

Doctors tried their best and approached my case from both the ophthalmologic and neurological perspectives. The neurologists first suspected Multiple Sclerosis (I even had a provisional diagnosis of internuclear ophtalmoplegia) and only the thought of having it has made me extremely anxious. Long weeks of MRI scans and waiting for the results... Conclusion: my scan was pristine and there were no signs of brain lesions. Next they tested my blood for Myasthenia Gravis (or more exactly for acetylcholine receptor antibodies). First test was not performed correctly and had to wait another month or so to have my blood tested again. This time the result were positive without any doubt. It was the day when I discovered that I might have a life companion...

Paradoxically this happened during one of the best periods of my life so far. I was happy, with a loving and caring partner which continues to support me amazingly. My lifestyle was quite healthy (exemplary diet and exercise). I was confident about my future after just completing a master’s degree and simply loving my job. In other words, no major stress or other signs/symptoms that could have predicted this sudden change in my health and life... That was almost as bad as having my house infested with ladybirds around that time of the year!