Friday, 12 September 2014

Final decision: thymectomy

Thankfully it’s been a relaxing summer despite the tension in the background while waiting for the results for the tests with the Myasthenia Clinic in Oxford earlier in July. I think there was a spark of denial in me as I was hoping that the blood test would eventually reveal a misdiagnosis (I sometimes still can't believe and accept what I am dealing with...). The blood test measuring the antibodies to the acetylcholine receptor (anti-AChR) which are present in a high proportion of patients with myasthenia gravis has been repeated. Not only that the test hasn't invalidated the diagnosis, but the levels of anti-bodies apparently rocketed to 28 compared to just above 5 in January shortly after the onset (to note that normal levels are considered below 5). I don’t have an explanation yet about what this increment means. All that I can think about is that my thymus is getting enlarged and is secreting more anti-bodies which risk to affect other muscles in high concentration. As well this may be just an obsession I have that something wrong is going on inside me and is out of my control. Unfortunately this kind of tests are not administered very often and I don’t have any scans scheduled in the near future to confirm or infirm my hypothesis.

Paradoxically I don’t feel that my condition has worsen so much over the summer leaving aside the thoughts about denial. On the contrary, I had some really good days when I managed to cope without prisms glasses until late hours of the night. Warm weather didn't seem to affect me despite the predictions from the doctors and other sufferers I've been in touch with on discussion boards and on-line support groups. There seems to be a pattern of good vision in the morning which gradually deteriorates in the afternoon (when muscles and especially ocular ones tend to weaken). The deterioration continues further in the evening to the point that the double vision sometimes can’t be controlled without prisms glasses. I continue to have doubts about the effect of the medication (Mestinon) which does not seem to help too much in the evening despite going back to 3 x 60mg daily.

The bad news is that the results for the other test (Electromyography - EMG) indicated that my muscles did not react normally to the electric impulses which suggests that my condition is in an early stage of generalization. I was afraid that this is going to happen sooner or later and now there is clear evidence supporting the worst scenario (not sure about 'the worst' but definitely is not a happy one). My current physical shape does not seem to match the findings though. I've been feeling well and religiously committed to a healthy life-style with a very balanced diet and regular exercise (five-six times a week). I haven’t noticed any weakness except the overall feeling of fatigue more pronounced in the afternoon (which is a drop in energy levels experienced by most of the people as far as I know). Now if this is related to a generalization of MG is difficult to say as it does not affect a particular group of muscles e.g. limbs. Random twitches of various intensities continue to be very annoying and for some reason usually gets worse after I take the medication (or shortly after the meal following the medication). It is a common side-effect of Mestinon and I wonder whether all the twitching biased the results of the EMG test in any way. I haven’t had the chance to speak to my neurologist about this since I received the results but I’ll definitely ask this question during my next appointment.

Following this change in my circumstances I am feeling more pressured than ever to take some action and make a final decision with regards to the next treatment avenue. As I am not a big fan of medication of any sorts it didn't take me too long to push my decision towards the surgery. I have recently spent a lot of time reading about different treatment options I have at the moment, mainly thymectomy and steroids. I've come across some interesting pieces of research that explore the role of thymectomy in people with ocular or generalized myasthenia. The results sound quite promising although there might be a risk that my reading was 'selective' in order to justify a decision I've almost made before actually the results for my tests came through. Anyway I’ll list below some of the more or less recent articles I found more relevant with a brief description of findings and a link to the source:


Schumm, F., Wiethölter, H., Fateh-Moghadam, A., Dichgans, J.

  • The severity of ocular symptoms was rated using a score developed for this purpose. The score progressively declined after surgery to an average of 70% of its initial amount in 80% of patients. Full remission occurred in three cases. No patient developed generalized myasthenia. Antibody titres against acetylcholine receptors if elevated preoperatively also dropped following surgery, with one exception. Clear criteria for the expected therapeutic success of thymectomy could not be identified. Based on our results, and on the assumed significance of the thymus gland for pathogenesis, thymectomy should be considered in patients with pure ocular symptoms.

Roberts, P.F., Venuta, F., Rendina, E., De Giacomo, T., Coloni, G.F., Follette, D.M., Richman, D.P., Benfield, J.R.

  • Transsternal thymectomy (n = 55) and transcervical thymectomy (n = 6) resulted in cure in 31 (51%) patients, improvement in 12 (20%) patients, no change in 16 (26%) patients, and worsening of symptoms (including 1 postoperative death) in 2 patients. Patient outcomes were statistically independent of the duration of preoperative symptoms (mean 9.5 months), patient age, or the presence or absence of thymoma. In patients with ocular myasthenia, 70% were cured or improved after thymectomy; in the subgroup of patients with ocular myasthenia and thymoma, 67% were cured or improved. Thymectomy is an effective and safe treatment for patients with ocular myasthenia gravis.

Liu Z, Feng H, Yeung SC, Zheng Z, Liu W, Ma J, Zhong FT, Luo H, Cheng C.

  • None of the patients experienced a myasthenic crisis, progression to generalized myasthenia gravis, or mortality. Hyperplasia of the thymus was present in 106 of the 115 patients (92.2%). Among 110 patients on whom follow-up was done postoperatively, 29 (26.4%) were in SCR, 64 (58.2%) showed improvement, 7 (6.4%) remained unchanged, and 10 (9.1%) had a worsening of their conditions. The results of the review indicate that ETT is a safe and effective treatment for OMG, especially in patients with illness of shorter duration.

Geoffrey B. Blossom, MD; Raina M. Ernstoff, MD; Gregory A. Howells, MD; Phillip J. Bendick, PhD; John L. Glover 

  • Improvement after thymectomy was noted in all 37 patients. Complete remission was achieved in three patients (8%) and pharmacologic remission in 23 (62%). The remainder improved in stage, medication requirement, or both.

Maggi G, Casadio C, Cavallo A, Cianci R, Molinatti M, Ruffini E.

  • The results of thymectomy in the treatment of myasthenia gravis (MG) arereviewed in the light of a personal series of 662 MG patients, operatedupon during the last 15 years. In 500 MG patients without thymoma, thefollowing results have been achieved: remission 37.9%, improvement 49.4%,unchanged or worse 7.4%, dead 5.2%. There is no sex prevalence and theremission rate is higher in patients under 40 years of age (P less than0.01), with mild disease (P less than 0.05), with a MG duration of lessthan 1 year (P less than 0.05) and with a follow-up length of between 5 and10 years (P less than 0.01). 
And the list goes on and on and on... 

To conclude with, statistically there is a high chance to make some improvement or even go into complete remission following thymectomy. I’ve been offered the video-assisted surgery option which takes longer to perform compared to the trans-sternal one but the recovery is a lot shorter (only a couple of weeks).

I have read about steroids as well and I admit that this would be a quicker and more efficient solution at least on a short term (there is research that claims that the treatment with steroids prevents the generalization of MG). Many participants in research studies or people I have met online have reported a significant improvement following steroids treatment but at the same time they complained about the numerous side-effects which are more noticeable after months of treatment. This is actually the main reason I declined my doctor’s offer of starting a course of steroids. The most common side-effects mentioned by MG sufferers already on steroids are:
  • Osteoporosis (thinning of the bones)
  • Increased chance of infections (as steroids may suppress the immune system). 
  • Increase in blood pressure
  • High blood sugar
  • Skin problems
  • Weight gain
  • Muscle weakness (?!)
  • Increased risk of developing cataracts
  • Increased risk of duodenal and stomach ulcers.
I am perfectly aware that people react differently to steroids and they do not necessarily experience all the above mentioned adverse effects (which may vary with different types and dosages of steroids). However I don’t feel like taking any risks and ending by being further medicated for health problems caused by the steroids.

So here I am, ready to go under the knife! I’ve just been officially referred to a surgeon and now I’m waiting for an appointment to discuss the surgery in detail and arrange a ‘date’ with my thymus... I’m feeling incredibly relaxed overall although I have to admit that all this MG affair hits me really hard when I’m feeling depressed. So far I’ve managed to get up and fight without complaining too much about all these strange sensations in my body and accompanying worries and negative feelings.