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!