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Steroid myopathy or Myasthenia or Permanent Damage

Marita 114 posts Jun 23, 2009 7:52 AM	This has been on my mind. I've decided to ask about it and would appreciate help. How does one know if the patient's symptoms are due to the Myasthenia that warrants an increase in medication//or if it is permanent damage from not being treated early enough//or if it is side effects of steroids? How does a physician reach this conclusion? And what if only one aspect, for instance, the eyes are permanent damage or breathing, what measures are normally taken to manage the illness? Marita
alice 379 posts Jun 23, 2009 10:48 AM	Dear Marita, I can only share my own experience with you, with regards to this. like every thing in medicine it is based on logic, and also on what is called-Koch's postulates. which means that if a certain factor/agent leads to a certain symptoms/disease, and if removing that agent leads to improvement and then re-introducing that agent again leads to the same symptoms/disease, then it pretty much proves a causal effect. this is even more if there is a clear dose response, eg-the higher the amount or dose of that agent/factor is the more severe the symptoms/disease is. 4 years ago, after my initial response to plasmapharesis during my crisis, I was given steroids. within a week, I started having a rapid deterioration in my condition to the extent that I almost had to be readmitted to the ICU, but was "rescued" with plasmapharesis. the neurologist that took care of me at that time, thought that although it is known that steroids can cause worsening of MG initially, this was not in the right pattern, and therefore he agreed to my decission to stop the steroids, as he was not sure what was going on. about a year ago, my current neurologist, decided that I had a severe enough illness to warrant agressive treatment and convinced me, that under his care I could safely recieve steroids again as obviously it was not managed properly by my previous neurologist, who did not have his level of expertise and experience. and indeed he gave it to me in a very different way. instead of 60 mg qd, he gradually gave me increasing doses of prednisone, with concomitant plasmapharesis, and indeed although I had some episodic worsening of my symptoms over the first two, I overall did very well, and even had a few days in which I almost regained my normal functional level, after the plasmapharesis. but on the third week, just like had happened 3 years earlier, I started experiencing the exact same symptoms that I had then. and again it was not in the right pattern ( I don't have a clue what the "right pattern" is, but obviously mine wasn't). as this was atributed to my aprehension/fear to go home and other reasons of that sort, I was discharged home in a state that was much worse then that I was admitted with. there was no doubt in my mind that this was due to the steroids, but my neurologist convinced me to preservere with them, as they are sure to be beneficial in the long run. it took me a few months of being almost totally bed-bound and requiring almost constant respiratory support, to realize that this did not make any sense. as according to the Koch postulate that I mentioned before, the worsening in my condition was due to the steroids. to make a long story short, I gradually decreased the dose of the steroids, with some improvement with each dose decresase. (as my illness has extreme fluctations any how, if was hard to be sure, but after a few months the pattern was clear). I was hoping that at some point, decreasing the dose will make my condition worse, because that would suggest that I have a response to the steroids. and indeed when I reached the 40 mg dose (starting from 100 mg initially), I started to feel significant fatigue and weakness, but this was different then my usual fatigue and also my respiratory tests did not deteriorate and even improved to some extent. this was very confusing for me, until I thought that possible I was confussing the withdrawl symptoms of the steroids with the symptoms of my illness. so I consulted an endocrinologist who thought it was quite possible, but was concerned about my myasthenic symptoms, and understandably was not ready to take responsibility for this. so I decided to keep on going down more slowly and see what happens. and indeed with time the withdrawl symptoms were less pronouned and I had further improvement again. I am currently on 14 mg of pred every other day and am pretty much back to what I was before I started this treatment, with regards to most of my symptoms, although the weakness in my legs is more then it was then. it seems in retrospect that I had a very rare type of myopathic response to the steroids, that has mostly been described in ICU patients, but rarely in other patients recieving prednisone. what supports it, is a very clear dose and response curve both on increasing and decreasing the dose of the steroids. and so far it does not seem that there is any beneficial effect of the steroids on my illness. as one would expect that I would be better then what I was before , and not worse if such a response existed. this could be due to one of two reasons-either I have refractory autoimmune MG (which is possible), or some other kind of myasthenic sydrome. I hope this answered your question in some way. alice
Marita 115 posts Jun 24, 2009 10:50 AM	Thank you, Alice. This is very helpful. It is very impressive how you manage to sort out what is happening to you and intervene, when the treating physicians aren't able to, even in emergencies with the IVIG. And with your response to Prednisone as mentioned here. At present, I am feeling lost with what is happening as I remain home bound. I am bedridden now. My only way to communicate with the outside world is by phone ( voice is very weak so usually can't though) letters, or computer. I am now having difficulty holding the phone in my hand even on speakerphone. Increasing weakness arms and legs and wake up at night usually 3 times having breathing difficulties.) I do get relief with mestinon, prednisone, cell cept and don't see how I could lower these because they are keeping my oxygen levels normal. The pattern is to have a positive response with improvement of all syptoms with increase in prednisone but it only lasts a short time (2 days to 2 weeks) then it is increased again ( I told am told the disease is still in "active phase" and that is why). This results in only short-term improvement. This has been the pattern with medications in the past--to stop working after a very short period, usually 2 days. Only to be contending with side effects that are unmanageable. While the current Mg ones are working with the breathing, although could be better still. I also take Tegretol and increased doses keep seizures at bay. I do believe Tegretol weakens my MG symtoms. Thanks, Alice. I do appreciate your opinions on this, as it is confounding. Marita
Marita 116 posts Jun 24, 2009 5:20 PM	One other note: The improvement of all symptoms with prednisone usually takes two weeks to occur. when the dose is raised, initially I become weaker and I've learned the difficult way not to allow my doctor to increase it more than 1- 2.5mg. at a time. I am beginning to think that nothing more can be done for me with this illness. Best, Marita Last Edited on 24-Jun-2009 5:26 PM
alice 380 posts Jun 25, 2009 10:02 AM	Dear Marita, today I saw a neurologsit- a humble caring physician, who told me, after going over my history and reviewing all my files, that he has no doubt that I have myasthenia, although he feels that managing my very unusual illness is like sailing an uncharted sea. he said to me, that obviously I know a lot about this illness, and that no doubt that I had a significant worsening of my symptoms due to the steroids. he said that he has seen one patient (who fortunately was classical seropositive), who had a similar pattern of worsening of her illness with steroids, just like me, and although this is not described in the medical literature, he could not ignore it, as it was very clear. but, he told me that 99.9% of the patients with mysthenia have significant improvement of their symptoms once put on steroids, although some could have initial transient worsening, so it is not surprising that they found it hard to believe. he wanted me to continue going down with the steroids, with the hope that there will be further improvement in my condition, or worsening that will show that they may have some effect after all (although, he thought that at this point it is very unlikely). he also thought that due to the very unusual course of my illness and the severe adverse response I had to almost every treatment I recieved, it may be better to just try and live with mestinon and respiratory support as required, instead of trying other agents. he said that it is very understandable that I wanted to recieve treatment for this illness, and was not ready to accept the fact that unfortuantely in my case there was no magic cure, that would work for more then a very short period with the price of severe side effects. he thought that possibly at least some of my more severe symptoms were due to iatrogenic damage, by multiple treatments that I recieved that probably did more harm then good, and may possibly gradually improve with time. although obviously there was no gurantee for that. he did not think that I had a normal neurological examination, but actually thought that I had significant weakness, that was consistent with the severity of my illness. he also told me honestly that he does not have enough understanding in pulmonology to manage my complex respiratory problems, and that I need to have a pulmonologist as well. he said he was ready to be my partner in finding the way to manage this illness in the best way, so that I could lead the best possible life, as long as I am ready to accept the fact that it is possible that things will not improve signifcantly even if he will do his best. it seems that patients like us, that obviously don't fit the book, need to find a physician that is ready to accept the fact that not all the patients are formed in the same mold. and that some patients require a more personalized approach. every neurologist I encountered until now, has tried to fit me into the procrustrean bed of "myasthenia", even thought they all realized that my illness was very unusual, and all were angry at me for not "fitting". each and every one of them promised me that if I only do what he says, I will be well in no time. and when I "failed" to improve under their treatment (which obviously worked well for the majority if not all of their other patients), I was blamed for it. patients like us, have no choice but to learn and understand their illness, more then others do, but we need to find a partner that is ready to listen to us and accept the fact that after all, the patient is the one that knows best, but still needs guidance and help from the physicians. I hope that I have found that person, as I am very cautious after my previous experieneces, but even if not, I now know what I should be looking for. not someone that gives me false hope, even if it is very tempting, but one that give me realistic hope and is ready to be my partner in achieving those humble goals. not one that has all the answers, but one that is ready to ask the questions and seek the answers with me, possibly never finding all of them, but at least trying. possibly, a few years ago, I would have not been happy with this approach at all, as I was looking for someone to give me a "magic and rapid cure", someone who has all the answers, that I could blindly trust, and the bitter failure was hard to bear both for me and for my physicians. I had to reach the point were I myself was ready to accept the reality and settle for much lesser and more realistic goals. alice.
Hammer 220 posts Jun 26, 2009 6:55 PM	"...but, he told me that 99.9% of the patients with mysthenia have significant improvement of their symptoms once put on steroids, although some could have initial transient worsening, so it is not surprising that they found it hard to believe." But at what cost? Sure, prednisone will ease the MG symptoms, but as it does, it is killing you in other ways. What about the thyroid, liver, heart, lipid values and so on? It's like trying to kill a fly in your house with a shotgun; you will eventually hit your target, but look what it did to your house in the process. Unless prednisone is controlled with other drugs you're just leaving yourself open to other diseases and conditions. But you already know I am not a fan of prednisone, or any catabolic steroid by itself to supress the immune system
alice 384 posts Jun 26, 2009 9:50 PM	Dear Hammer, you say-at what cost? and I perfectly agree with you, as in medicine (like in fact basically in life), one has to take into account the risk/benefit ratio. or in other words what is going to be the cost for what I get, and am I ready to pay this price. I myself have given patients medications that are much more hazardous then prednisone, and carry a much higher short and long term risk, . but, unless you can give me a better way to treat leukemia, I believe that I will continue to give high dose chemotherapy to patients with this condition, as it is definitely worth the price. and I doubt that you would argue with that. from the way you describe your illness, it seems like you have a relatively mild form, and your decission not to take prednisone is reasonable, although some would argue that taking a low dose of prednisone and significantly decrease the risk of progression, is a reasonable price to pay for the potential benefit, but as this is not entirely clear, your approach is reasonable as well. but, there are other patients in which for the same risk, the benefit is much higher. and prednisone may even save their life. it is true though that ideally the risk/benefit ration should constantly be evaluated, and in the ideal world you would have infalable physicians who would do this in a scientific manner on a daily basis, but unfortunately this is not possible, and we all err on both sides. physicians have the advantage of having a broader perspective, and seeing the entire forest and not only the specific trees. whereas patients tend to concentrate more on the momnetary discomfort. how much should those decissions be made by the physicians, and how much should the patients be involved is a very hard question, that I am not sure I have answeres to, and I do not think that any one does. it is not always for the benefit of the patient to know all the details of the risk or the benefit, yet the patient has to be an active partner in this decission process, especially if factors such as quality of life, are invovled. I think that your example of treatment with prednisone of -trying to kill a fly with a shotgun may be true in some patients, but in others it would be more like-tyring to kill an armed burglar who is about to pull a gun and shoot you first. and in a good physician-patient relationship, hopefully, the way to differentiate between the two will be found. and no guns will be used to kill flies, yet those will be used in a timely manner to avoid invasion by armed burglars. and if you have read some of my previous posts you will see that my concern is that in myasthenia this is many times not the case, and flies are seen as burglars and vice versa, and for me specifically it seems that my neruologist kept on shooting in every direction, and destroying my house, not realizing that the armed burglar has found the way to use those bullets against me. very unlikely, I agree, but still that is what happened. alice.
Hammer 221 posts Jun 28, 2009 3:54 PM	Alice I just have a strong prejudice against prednisone, for many reasons, one being that I feel there are alternatives to straight prednisone. I agree, it may help, but it's possible that drinking low levels of mercury might resolve some of the symptoms too, but is it the right solution? (I know mercury won't help, but it also will slowly destroy your body as does prednisone.)
alice 385 posts Jun 28, 2009 9:47 PM	Dear Hammer, possibly you are right, in your thoughts that there could be other alternatives that would be much less harmful. one of the reasons that I have always been invovled in research and not only direct patient care, is exactly this. I am just in the process of conducting a clinical study, based on 7 years of collaborative research in the lab (which I started when I was able to do the work myself and the first paper was based mostly on my own work, whereas in the following ones, I was only able to have intelectual contribution which is much less appreciated, but that's one of my frustrations and is not related to this topic), trying to gain a better understanding of the molecular events involved in the development of a certain type of leukemia. most of the medications that are being used, carry a price, even if it is not as obvious to you as the damage caused by prednisone, and trust me the treatment we currently have for this type of leukemia eventully leads to the failure of the immune system. I fully agree with you, though, that some physicians, are not very concerned about the risks of prednisone or other medications that they use routinely, and use it without putting much thought into the price. one of the reasons I am very dissapointed with my neurologist, is that he kept on giving me this treatment, even though it was clear that it not only did not improve my condition, but actually made it significantly worth. and if it weren't for my own understanding and judgement, I would be still taking 100 mg prednisone, with the unreasonable hope that it will eventually lead to my remission. even though he was told a few times, by other consultants that congenital myasthenia should be considered in my case, he was not ready to even think about it, and got very angry, when me and my husband "dared" to raise this possibility ourselves ( based on what we were told by those consultants). and insisted that I keep on taking high dose prednisone. this is in complete apposition to my dentist ( I have an amazing dentist, who after 3 before him, told me that I can't tolerate any dental procedure and become severely short of breath, because of "anxiety", he was ready to listen to me, and together we found the way to manage it, by more suction, having me upright and him standing etc.), who was contemplating to give me some antibiotics, but when we checked and found out that it could exacerbate MG , although my neurologist had said that the risk for it to happen is low, he decided to try and manage without it, and then he said to me-alice, you know that every medication that we give is poision and we have to have a very good reason to use it. this is my approach as well, and I will not even give tylenol, without good justification. alice.
Marita 118 posts Jul 01, 2009 8:03 PM	Dear Alice, Thank you for your update. I was hoping you were at your appointment when didn't hear from you for several days. I wish you the best with everything. I'm glad you found someone you can trust, who will listen. I hope lowering the Prednisone will help you, and if not, increasing it will make you stronger. I can relate to trying to achieve more humble goals. Not an easy task, though it's exhausting racing all over town for that neurologist who will have all the answers just to make things worse. I do believe the new tick treatment, if a success, could be possibly a good treatment for us, especially since it is more benign and less likely to have the side-effects/risks of other treatments. I'm having a difficult time with the illness but feeling better today. I thank you for the important information you provided on MG Crisis, Sleep Apnea, the dance instruction/exercise (will try when I'm stronger and eyes improved). This is very helpful. Take care and keep in touch. You help keep me strong through it all. Marita Last Edited on 1-Jul-2009 8:40 PM
alice 396 posts Jul 02, 2009 7:12 AM	thank you marita, I just started to understand that I was trying to find the way to cope, with something there was no reason to cope with. my neuro may be one of the best, and obviously has a lot of knowledge and experience and there is no doubt in my mind that he has been able to help many MG patients. but how does that help me? when he is not ready to seriously try and find better management options any longer? and it does not matter if the reason for this is that I annoy him for some reason, or that he is just too busy, or not as open minded as I initially thought, as none of this is something I can change or control. and the same applies to my "local" pulmonologist, a good colleague and friend that I believe really wants my best, but somehow got "stuck" and wasn't able to admit that it is beyond his abilities to take proper care of me. and ended up telling me that I can call him day and night, but practically did nothing other then give me some ill advice. (that fortunately I realized I should not follow). so how does it help me to know that he is a very good, caring physician. it is only very frustrating to know that, because it makes me feel that there is something wrong with me, as why would he treat me in such a way, so different from how I know him? as I think about it, I can't understand why it took me so much time to realise all of this. but, I guess that when we are ill and dependent on others, we don't always think straight. and also, I am a person that always tries to get a long with everyone, ready to make changes and admit my mistakes, so it was very hard for me to decide that this "relationship" that initally was a great source of hope for me, was just not working, and that I have to come to terms with that. and it does not mean that this has to happen again. and hearing about all the people on this forum that had a few neurologists until they found someone that they really felt comfortable with, made me understand that this is not so unusual, and can happen, especially with such a "tricky" illness like this one. and you just have to be ready to search again. and most improtantly, not let anyone make you lose trust in yourself. take care, alice