The tap that keeps on tapping

With apologies to Cyndi Lauper, there's a hole in my back that comes all the way from Rochester. The headache that I've had since last Friday is most likely a result of a CSF leak. This is a side effect of the lumbar puncture I had at the Mayo Clinic, where they stuck a hole in my back to draw out cerebro-spinal fluid, and the hole has not properly closed.

So what's the solution? Poke my back again, and this time inject some of my own blood which will help close the hole. I'm booked into go to shortly to have this procedure done.

This is really getting to me. I agonized as to whether to just keep resting and hope that the hole closed on its own, or to have another uncomfortable procedure to close it, and decided this morning to take this route. It should give me a little more certaintly that the headache will stop once and for all.

Of course I also have to book in for more IVIg juice, and have decided with my neuro to just do one day (instead of the usual two), and do that on Monday after a weekend of rest.

too much juice?

Well, so far, this latest experiment with my medication hasn't been a roaring success. On the first day, the neck soreness was quite severe, accompanied by a sensitivity to light, and that general awfulness feeling (my wife says "you look green"). Fortunately, not much in the way of a headache. On the second day, it wasn't quite as bad. I had the weekend to rest and take it easy, although this morning I woke up with an awful headache. This was probably because of the howling winds that didn't allow me to get the decent night's sleep that I need.

It's quite evident that the juice and I just don't get on well. What I can't stand is that the nasty side effects are very quick to show up, but the positive effects of the treatment usually don't kick in for about a week. That makes me very despondent every time I go in for some juicing. Especially when I wake up in the morning feeling like crap and read puff pieces in the newspaper about little Michael and how IVIg revs him up just like a double espresso. I wish the stuff worked as well for me!

even more juice

Blogger has been playing up lately - very annoying!


I've been tracking my hand strength for the last few months, using a "rig" consisting of a paper cup with some string attached, and a series of scientific weights. A couple of times a week, I measure how much weight each of my fingers can hold up. As you can see from the pretty picture, there are spikes, and these roughly coincide with the IVIg treatments - they seem to have their maximum effect around a week after each treatment.



The legend is a little small - the blue line at the bottom is my right index finger. There are a few interesting things going on here. Firstly, despite the treatment, the best it gets is for my right finger to have not much more than 20% (compared to the left, and the other fingers). I have found this quite disappointing. The spikes are also quite modest. Finally, the pinkie (black) is one of the stronger fingers, and this is uncommon in MMN.


So given that we are reasonably on top of the side effects, my neuro has decided to up the juice. We had been doing 0.78 g/Kg over two days as a maintenance dose, which is already at the high end. The next dose (over the next two days) will be 1.06 g/Kg - an increase of 36% or an extra hour and twenty minutes connected to the juice machine (can you tell I'm a numbers guy?).

I really shouldn't have written about this - all it's done is make me think about how long the treatment is and what I will do. Lately, I've been relaxing more during the infusions - nodding off for a nap here and there because of the phenergan. All of that certainly seems to have helped minimize the side effects. Will have to line up some extra videos to watch or maybe a book (although reading one-handed while lying in a bed is a little difficult).


And in other news, Mayo have responded in the affirmative, and I'm booked in for November. More about that in another post.

Rest is the key

It's been a couple of treatments since my last posting, and things appear to be on the up. I have formed the view that while steroids reduce side effects, they have an adverse effect on the primary condition itself. Having put in a measurement system for the strength in my fingers, I was very disappointed by the improvement in course I had five weeks ago.

So in this last one, we just used anti-histamines (phenergan), anti-inflammatories (nurofen), and regular pain killers (panadol), and these did a reasonable job of reducing the side effects. The most important thing I have learnt from the last two treatment courses is the value of rest! On each occassion I just went to bed and relaxed for 2-3 days, and that was more effective than any drug.

Of course, I would much rather pop a pill (or ten) than have to take 2-3 days off each month. But alas, it is now clear to me that lifestyle changes are required, and one is just to take it easy, and reduce the pace of my life; particular around treatment time.

This last treatment was after a gap of just one month, and the strength in my index finger is the best it has been for around six weeks. That part is very encouraging. Because of some travel plans, my next treatment will be after just a three-week gap, so I'm hoping that will act as an extra booster, and I can revert to a monthly schedule that is more pro-active with respect to the strength in my fingers.

I'm also now exploring some complementary medicine as a way of dealing with some of the limitations of western medicine, particularly relating to the side effects if IVIg. More to follow in future posts ...

rock and hard place

Rock (noun): Deal with the side effects of IVIg using conventional, over-the-counter anti-inflammatories and pain medication, and just wait until they subside.
Hard Place (noun): Deal with the side effects of Prednisone, which rendered me angry, aggressive, hormonal, and bursting out of my skin.

After taking the Prednisone for two weeks, and been off it now for a few days, I've gained a deeper understanding about the nature of different types of headaches. Now that its effects have worn off, I can see Prednisone is reasonably effective in reducing the inflammation reaction to IVIg. Yes, the headaches and neck soreness have returned, so it's back to Nurofen etc to deal with that. Drugs like Excedrin don't work - there is pain, and there is pain. Because mine arises from inflammation, your typical pain killers (even strong ones) don't really do the job.

Being trapped like this between two sets of side effects has me in a bit of a down mood. Maybe I should take something for that?

'roid rage?

I really hate Prednisone. I'm finally tapering for a bit longer after being on it for just over a week at 25mg, and this time, it's really messing with me. Hard to put words on it ... I'm "edgy", not tired at night, sort-of anxious, peckish. Have put on 4-5kg in just a few days, which has made me feel bloated. Is this 'roid rage?

Whatever it is, I can't stand it. It's making me something I don't like, and I can't wait for these side effects to fade away.

It's strange that I don't recall these side effects last time I took Prednisone. This time, we did it as a premedication before the IVIg, so the circumstances and the combination of things going on in my body are a bit different. They have helped to alleviate some of the IVIg side effects, but it has been a very unpleasant journey. In addition, the Prednisone does seem to have a negative effect on my hand strength, so the benefits of the IVIg are held back somewhat. This is something that also happened in my last treatment cycle.

The good news is that my consulting neurologist from Johns Hopkins suggested something different next time. The thought of having to fiddle with doses of Prednisone each time was really depressing me. Instead, he's suggested an IV cocktail of dexamethasone plus a couple of other "mixers" as a one-shot premedication. It's a much stronger form of steroid, and because it's a short, sharp dose, the side effects should be reduced. Anyway, something else to look forward to in a couple of months ...

juicing again

Walked in this morning for the third and final day of this course of IVIg, and Michelle (Jamie has left the position, so Michelle is running the "juice bar" while they find a replacement) greets me with a friendly "How are you?"

"It's fantastic! I woke up this morning with full strength in my hand, and the side effects had all just vanished .... APRIL FOOLS!" Maybe it was funny at the time. Maybe you just had to be there.

I should hardly be surprised. The side effects started showing up an hour or so after the first day of juicing, and have steadily increased. Hard to tell if they are slightly less severe than last time as a result of the premedication with Prednisone - I think it has helped a bit. Will be watching it closely over the next few days as I continue with the Prednisone (plus regular pain medication), and then taper off for a few days. On the bright side, there is a slight improvement in hand strength, and it isn't collapsing under any pressure the way it was a few days ago.

So having established that the juice does help (just that it doesn't quite get on with me), this now turns into a trial-and-error exercise in working out how to manage the side effects: headaches, neck soreness, sensitivity to light and loud noises (like kids crying and shouting). It looks like a mild case of aseptic meningitis (which is what they suspected the first time around), which is usually treated with: steroids! So for each juicing, we will attempt to tune the Prednisone dose to one that works, and hope that other things remain stable for a period of time.

Of course things would be boring if the Prednisone didn't have its own side effects: a touch of nausea, sleeplessness (although I felt quite tired this afternoon), edginess (but who wouldn't be in my condition?) I'm thinking the best thing to do is head off to a spa for a few days of R&R after each juicing. Do you think I can get a prescription for that?

miscalculation

It seems that with all the extra tests I was having, I took my eye off the main game, and overshot the timing for my second treatment. With my 20:20 hindsight glasses, I probably should've done it several weeks ago. In the meantime, my hand strength has deteriorated quite rapidly in the last two weeks, and is now quite severe. Part of the delay was me not wanting to accept the notion of needing regular treatment; I have to deal with that.

The neurologists say that pain is not something that is associated with MMN. Of course, by this they mean that a nerve conduction block will not cause pain. They may be right about this. However, many people with the condition do experience pain, and I think the reason is quite simple. If your hand does not have strength to support movement in certain directions, and you actually do move it or pur pressure in those areas, then the hand collapses. The radial nerve doesn't seem to be that important when it comes to hand movement - I would think gripping (which is the median nerve) was a far more common action that needed support of motor nerves. But surprise, surprise, there are many movements where my hand and wrist collapse in pain - pulling off a t-shirt, turning a steering wheel, shaking hands, ...

Every time I see my neurologist, or discuss it with my pilates instructor (who is also a physiotherapist), I learn so much about the nervous system and biomechanics. While I'm a person who thirsts for knowledge, and people are impressed when I can explain this stuff to them in simple terms, in the back of my mind, I'm thinking "do I really need to know this?"

Anyway, I've started the premedication before the next treatment tomorrow. Hopefully, I will not experience the side effects of last time. This course is just three days, so that might also reduce the chance of side effects. I really need it to work - having my hand like this just isn't doing it for me.

next juice ...

My hand strength has degraded a fair bit in the last week, and with the protracted diagnosis phase all but complete (just a review discussion with my neurologist next week), I decided that it was finally time for my second IVIg treatment. I ought to be pleased because it has been three months since the first one, which indicates a very slow progression, but instead I'm feeling flat and depressed about it.

Approaching a second treatment course is very different from the first. While I am unable to get excited or enthusiastic about any medical procedure involving a close relationship with a needle, at least with the first treatment, I anticipated a successful outcome, and was not particularly concerned about side effects.

This time around, my anticipation is very different. I think the treatment will result in improvement to my hand strength, but the side effects are a concern, mostly because there hasn't been a satisfactory explanation as to why the headaches went on so long. This time, I am going to premedicate with Prednisone, which should help, but as always, the operative word is "should".

But the other thing that weighs down on me is the long-term future. By embarking on this a second time, I'm accepting it as a part of my life - that regular IVIg "juicings" will now be established as a regular part of my diary. While I was already advised that there is no cure, and that this is just about the only treatment, this is the moment when its permanence is truly felt.

next juice?

Well, the most recent set of test results (skeletal x-ray and MRI) are in, and everything does seem to point to MMN. Is this a good thing? Hard to tell. They're ruled out plenty of bad things, and it looks far more like MMN than CIDP. I don't know if one is better or worse than the other, so that doesn't mean a lot.

It's been over a week since I finished the Prednisone, and I have noticed a couple of interesting things. Since finishing, my hands seem to feel a bit better - a bit more freedom of movement. That seems to indicate that the Prednisone had a detrimental effect. However, along with that, the headaches have returned. They are far less intense than a couple of months ago when I had the IVIg treatment, but they are still there. Not severe enough for me for me to be taking something every day, but enough that (a) I notice, and (b) there is no answer as to why they are still there. Perhaps they are less severe because the IVIg is wearing off over time?

So now I play the waiting game. Waiting for my hands to weaken sufficiently that I will embark on the next IVIg juicing. Next time, they will premedicate me with Prednisone, which they think might help with the headaches. No-one really knows; it's a case of trial and error. More and more I feel like I'm the patient on an episode of House. The only differences are that over there in TV-land, he works it out in about 35 minutes, it's all over in 42 minutes, and the total elapsed time is a few days. My episode will being played out over years, and in that context, we've only just begun. In fact, all these medical shows are starting to annoy me. I squirm when they do tests on the patients, especially the ones with needles, and I'm starting to understand far more of the medical terms than the typical viewer.

Perhaps the only good news is that it's been some ten weeks since my first IVIg treatment, and my hands are far better than they were before the treatment. To me, that implies a longer than average cycle between treatments. You might notice that I'm not running back for more in a hurry. It's a fine-tuned balance between a rock and a hard place.

on pain and memory

Chronic pain, and muscle soreness or weakness, is a funny thing. When it's there, you know it. You notice it all the time. Whether it can be medicated or not, it's there in the front of your mind. The fact that it's chronic bears down on the mind as well, and compounds the effect. Unlike a sports injury or the occassional headache, knowing that this is something long term with no end in sight magnifies the feeling with an additional, psychological burden.

Then, if a course of treatment is successful, the symptom may just fade. And when it fades, it's almost as if it was never there. When it's absent, it was really hard to remember what the pain or weakness was like when you had it. You quickly rebound to a state of how things ought to be as if that is how it always was, and there was never anything wrong.

But after a while, the effect of the treatment may start to wear off. The weakness or pain emerges from its dormancy quite quickly, in fact much faster than its original appearance, which was a slow progression over perhaps several years. It announces to the body: "I'm baaa-aack", like a long lost friend who was never lost at all; just travelling briefly. And then you realize that the weakness and pain was never gone at all, and that the time you enjoyed when it was absent was the exception, rather than the norm - just a short dream.

This is where I am at now. After nearly two weeks on the course of Prednisone (which is now finished), and nearly two months since my initial IVIG treatment, the hand weakness reappeared almost overnight and pain a few days later. There is also some feeling of weakness in my left hand, which I've been trying to find words to describe, and of course the cramps in my calves (although I think lots of stretching helps for that).

But now, I am in no-man's land. I'm not going to have another "juicing" with IVIG because we're not sure how to deal with the side effects. But if I do nothing, then the hand weakness will just stay and get worse. Am waiting on some more test results. These might shed some light on the whole MMN vs CIDP thing, which might change the preferred treatment. The only certainty for me is what happens if I do nothing.

'roids forward and backward

Have been on the 'roids (that's steroids, as in Prednisone) for a week now, and they do seem to have had an effect. The headaches have subsided, which is good. The main thing I've been watching for are further possible side effects. Sometimes I wonder what proportion of drugs are taken to avoid side effects of other drugs, and how much goes to treat the primary condition.

Still have some more tests next week that might shed some further light on exactly what thing I have. In the meantime, it's a case of "let's try this and see what happens".

In the meantime, I've noticed in the last day that my right finger has reverted a little, and there is less movement. It seems a bit different to what it was a few months ago before any treatment. My wife thinks I'm really good at describing pain and physical sensations. This feels a little different to before - there is a noticeable heaviness and lack of desire to respond to my brain's instructions to lift. There is also the slightest tingle in the finger.

This could be a side effect of the Prednisone (unlikely, I think), or simply because the IVIG treatment was two months ago and is wearing off. Interesting to note the rate of the decay - it took weeks to start working again, but seems to have stopped working relatively quickly. I am far from ready to try another dose of the juice; still have another two weeks to wind down the Prednisone and assess its effects.

And as usual, a change like this has me feeling depressed. I almost felt like going back to bed and staying there all day. Fortunately, there is enough to do at work so here I am.

and the results are ...

I really need to learn to adjust my expectations. The spinal tap was supposed to explain/reveal so much about specifically what condition I had, and the reason behind the side effects. Instead, the results are inconclusive, and I am left feeling flat, exhausted (is it the hot weather, the return to work, or a side effect?), and as uncertain as ever.

The protein levels were elevated, but not sufficiently to indicate CIDP over MMN. There was slight inflammation, but not sufficiently to confirm aseptic meningitis. Could be that the meningitis was more clearly indicated if we had done the spinal tap sooner after the initial IVIG treatment (it's been over six weeks), and now we are seeing only a residual inflammation. So, to summarize the test results in lay-speak: "it might be this, but on the other hand, it could be that, and the side effects might have been because of the other".

Bottom line: my neuro is putting me on a short (three week) course of Prednisone in the hope that it might reduce the inflammation, and therefore the headaches. They may also have an effect on the primary condition. And I am left still wondering, and have to pick up some more Panadol - the bulk size *sigh*.

that was spinal tap

A spinal tap, or lumbar puncture, is not a pleasant experience, although in hindsight, like so much of this, the anticipation is far worse than the procedure itself. It was done as a day procedure, and not content for just my wife to accompany me, my mother had to come along as well (despite anything either of us could say to dissuade her). In the waiting area, we spotted what looked like a Greek family accompanying someone who was being admitted. They were a party of six plus the patient, so I guess my "entourage" was relatively small.

The whole thing is quite simple, and done under X-Ray. So I'm lying there on my side, and there's a big X-Ray apparatus that slides over the top of me, and the radiologist is able to see live pictures of exactly where to put the needle in so he gets the right spot. Fortunately, he did it quite well, and it worked first time. Indeed, all the of hospital staff were fantastic - very friendly and helpful. Funny how all of the people in the room put on four-part X-Ray "flak jackets" covering every part of their body while I'm lying there in just a gown.

It takes several minutes for the cerebrospinal fluid to leak out of the hole they make, so I'm making small talk with the guy to pass the time. After it's finished, I suddenly get all sweaty and shaky, but this passes after a few minutes. Perhaps the relief of it being over. I had been anticipating this for several days since my appointment, and it has probably contributed to slightly worse headaches than usual.

Afterwards, I have to lie flat for two hours, just in case the hole in spinal sac containing the fluid does not close properly. Fortunately, this appears to be OK, although my headaches have been worse since the procedure. The information sheet advised of possible headaches after having a lumbar punture - is this a side effect resulting from the procedure to help deal with the side effects? The recent heat wave, and my return to full time work after a break may also been contributors to this. Should get some results in a couple of days, which will hopefully give answers on some burning questions, and perhaps an end to these headaches!

more tests

Had a review with the Neurologist today, which confirmed that remains some increased strength in my hands after the initial IVIG treatment. It's not back to 100%, but it's better than it was a couple of months ago. Being just the first treatment, we must now sit back and see how long it lasts.


Some questions remain unanswered, like why I still have headaches, and he suspects that I may be suffering from an uncommon side effect of IVIG - aseptic meningitis (aseptic as in not the nasty form of meningitis that is really dangerous, but rather the benign one that gives headaches instead). There is also still some doubt as to whether what I have is MMN or actually CIDP. He expressed this possibility earlier, because of the way certain nerves were affected, and this doubt was also indicated by comments from another doctor who reviewed my case, and asked for further information.

So the obvious thing to do is to stick a needle in my back and extract some fluid - the magical cerebrospinal fluid which contains (we hope) the answers to the mysteries that lurk within my body. The lumbar puncture, or spinal tap, is something I've seen plenty of times on TV in medical dramas. I guess the only good thing about having one is that I won't see it - I'll be facing the other way trying to think of something distracting. Do you think they'll let me listen to my iPod during the test? My fear of needles keeps being tested by all these damn tests and treatments, and is something I will just have to get used to.

the juice's journey

I feel sometimes that the "juice" is making its way through my body, visiting here and there, and having effects of sorts, some good, some less than good. Every day is something different. The respite of pain in my wrist lasted maybe 48 hours, and seems to have reverted. The strength in the wrist has also diminished. Oh, and the headaches are back, as well as a bit of neck pain. I've felt a bit nauseous over the last few days, although that could just be a result of different eating habits during time on holiday. So much to analyse; so little that can be understood.

Mine does not appear to be a "standard" case of MMN, if there even is such a thing. This appears evident from my doctor's written report, and also from the feedback from another specialist who has reviewed my case. He found the data "fascinating", which may be an appropriate term for an observer who hungers to learn from the many cases he gets to see. For me, sitting inside the observation booth , the term is almost distasteful.

They speak of the "yoyo effect" of treatment, and it seems this exists both at a micro and macro level. On a day to day basis, my mood goes from optimistic to bitterly despondent and depressed. And I've hardly been in this long enough to get a macro perspective!

the analyst

I am an analyst. No, I don't work as an analyst, say for an investment company or an IT firm, Rather, I am an analyst. It's what I am, and it's what I do. All the time. Everything around me is the subject of constant analysis - what is going on and why. Cause and effect. How things work and why.

Had a swimming lesson the other day (it's a long story). Not enough to just take the instructor's word regarding the correct way to do things, but I had to understand why they are correct - the physics or dynamics of the swimming form, and why it works. Once I did, the learning stuck much better, because I knew why I had to keep my head down; why my head movements to come up for breath had to be a certain way.

I enjoyed simultaneous equations in school. N variables and N equations. Didn't matter how many there were; as long as there the number of variables equalled the number of equations, exactly one solution could be found. That's what I like. Determinism. That's why I enjoyed the sciences, and despised the humanities. The latter were so wishy-washy, and there was no way you could know your answers were correct. That's probably why my main business runs computer systems that perform millions of complex billing calculations every month on behalf of our clients.

Now, I am living in a world of non-deterministic MMN. There is no direct or simple cause and effect. There is only fuzzyness.

Yesterday, for the first time in at least a year, the pain in my right wrist subsided, and I was able to put pressure on it (like when doing a push-up). So, a full month after starting treatment, it does seem to have helped, with significant restoration of strength in both the hand and wrist. I look forward to my next appointment where some objective tests will be applied. Also keen to see if my leg spin bowling is any better.

The side effect issue still sits there. Having returned from my holiday, it seems the absence of headaches may be related to being in a calm and relaxed environment (pity I can't stay there all the time). Will pick up another large box of nurofen and panadol before we go away again for another week tomorrow.

good therapy

Important discovery: being on holiday in a fine resort is good for you! Very relaxing over here; side effects have mostly subsided, with the occasional headache. Certainly it seems the quiet over here helps. There is definitely more strength in my hand. Still some pain and weakness, particularly around the wrist. Every day brings different sensations around different parts of my right hand. Cramps in my calves are far less frequent. I'm thinking of developing some of my own objective tests of muscle strength and the like, so I can get a better sense of whether and how the juice works.

Still need to do some further research into the side effects. It seems the extent of mine are not so common. Next visit to the Neurologist is in a couple of weeks, so that should be an interesting discussion. In the meantime, best to make the best of my time away.

happy new year?

Woke up with a splitting headache this morning, and for all the wrong reasons. Usually, it might be due to an overindulgence in celebrating the new year. But a Strongbow, a couple of episodes of The Mentalist, and a 1am bed time hardly add up to a big party. So I was quite surprised that the nasty headaches had returned with a vengeance. How long will they continue? I so hate having to keep taking drugs for them, especially ones that don't reall work that well anyway.

There does seem to be a further increase in strength in my right hand. And yet, I am still left with so many questions about this process. Being on the wrong end of the seeming black art of Neurology is not a fun experience. One thing is clear: nothing happens quickly, and during 2009, there is still so much I will learn about my situation and how to best deal with it.

progress, maybe

Well, it's been over a week since I finished my initial IVIG. There does appear to be a slight increase in strength in my right hand, although in the last day or so, there is pain all through the hand. Previously, there was only pain in the wrist.

Having just watched a few episodes of Heroes (among many other sci fi type shows and movies), it reminds me a bit of the scene where the scientist injects himself with the experimental drug, and goes through some instant (and painful) metamorphosis. You often see the "effect" of the drug working its way through his system as it does its stuff.

I have no idea what is going on inside my hand at the moment. Lots of different opinions about how long it takes the IVIG to work. Have discovered a user community for MMN, so it will be nice to learn about the experiences of others, and possibly find out more about what my expectations should be.

The side effects are still there. Headaches appear from time to time (no more accompanying nausea), but seem to have subsided; neck pain is still very much there. I'm not taking much, if anything, to deal with them.