First up, a wee disclaimer – this is long… very long, but I recall that when I was searching for information about this condition, much of the content I read had been edited for brevity, making much of it pretty useless. You’ll see in no uncertain way, that I’ve avoided the temptation to be brief. Don’t say I didn’t warn you.
Over the course of November 2018, I became aware that some of the exercises that I was doing at the gym were becoming difficult to do. Actually, not so much difficult, but awkward. It seemed to involve any exercises that required a certain degree of grip strength.
This was pretty specific to exercises like the upright row, the lat pulldown and barbell curls. These all required a motion of pulling on a bar. Exercises like bench press or shoulder press didn’t seem to bother me, as these required a push motion with the hands.
I came to the conclusion that for some reason I must have a weak grip and that I needed to work on my grip strength.
Over the next couple of months this become more and more noticeable. I also found that I was a little more clumsy – I fell down steps a couple of times, fell off a ladder and slipped getting into a bath tub.
I didn’t recognise at the time that my legs were any weaker than normal. It wasn’t till later, looking back, that it made sense why I was clumsier than normal. On the occasions when I fell, it was at times when my legs were at a wide extension, with a substantial amount of weight being born on one leg, such as when taking a staircase at two or three steps at a time, or when stretching across trying to get into a bath tub, or whilst navigating around a ladder. There was this particular occasion when I was up a ladder and I decided I could do a mid – air transit from one side of the ladder to the other. I reached around with one leg on the far side, my leg bent – past experience told me I could rely on just one, half bent leg to support my entire body weight – but this wasn’t to be the case this time – so I ended up in a heap on the deck below, with a paint can in my hand. The result wasn’t pretty.
The moment when I realised that my weak grip was something more than just ‘something to be worked on at the gym’ was on a Friday evening when I had a guest at my house for a romantic dinner. I went to open a bottle of wine (a screw cap) and just couldn’t undo the cap. I had to make some excuse and disappeared out to my laundry to find a pair of pliers to open the bottle. Very smooth move – she never suspected a thing.
This was the moment that I realised this had to be some kind of medical issue, so I did what any curious lad does- I consulted google. I searched every possible reason I could find for a weakened grip. The only possible suggestions that I kept coming up with were MS, Carpel Tunnel and Motor Neuron Disease. There were other things that could be immediately ruled out, but these three were the only things that came close to explaining a weakened grip, although all 3 also had other symptoms that I wasn’t exhibiting. I’m fairly resourceful when it comes to data searching, so it really surprises me that I didn’t come across anything about CIDP at that point.
At the time, my GP was on holiday, so I called in to see his Locum. He ruled out carpel tunnel, and pretty much everything else he could think of. He scratched his head and said, “Gee, I just don’t know – let’s just wait and see, and let it ‘declare’ itself”
Now, I’m no Doctor, but that just seemed like the dumbest thing in the world to do, so as soon as my usual doctor arrived back, I saw him and he said we should consult with a Neurologist. We looked at getting a private specialist involved, but the wait times were almost as long as my Doctor anticipated that the public system would take, so he booked me in with Auckland Hospitals Neurology department for the earliest that they could see me. He suggested it could be a few weeks.
Meantime, my feeling was, that whatever this thing was, it wasn’t something normal or ordinary, otherwise the two doctors I had seen would have had some clue as to what it was. So it was my belief that we had to act as quickly as possible as surely the sooner something ‘strange’ is diagnosed, the better the outcome.
So with this in mind, whilst waiting for the Neurologist appointment to be confirmed, I booked myself in to a hand therapist – Hands Out West, in New Lynn.
The Therapist measured my grip strength and it was at 12 kgs. She said that if I was recovering from a hand injury, they would recommend that I not drive until my grip strength had reached at least 12 kg. That was a bit of a shock to think that I might be getting to a point where I wouldn’t, or shouldn’t, be driving. The Therapist couldn’t come up with any explanation either, so she booked me in with an Orthopaedic Surgeon. It was a couple of weeks before I could get an appointment with John Mutu-Grigg. By this time we were into April of 2019.
When I met with John, he did a few strength tests on various limbs and he said we really need to do a nerve conduction test – he said that will determine whether this is a Neurologists thing to deal with or ‘his stuff’ – he said “you really want it to be my stuff”.
The nerve conduction test was booked in with Dr Richard Frith and I rang him to see if there was any way we could get an appointment fast tracked. He had originally said it could be a couple of weeks, but he was really obliging and managed to get me an appointment within a few days’ time, last appointment for the day at 5:30pm.
Meantime, I had a call from Auckland Hospital and they said they had an appointment arranged with me for a couple of days’ time to see a hospital appointed Neurologist. I was really surprised to have heard from them so quickly – my appointment with him was on Wednesday and my nerve conduction test was on the Thursday.
I met with the Neurologist and he did a few tests, tested my reflexes and noted that the weakness in my hands were ‘moderate to severe’ (take note of this term – it’s important later). He also noted that there was ‘mild’ weakness in my legs and in my arms.
This surprised me, because at this stage I really only felt the symptoms were in my hands. I hadn’t twigged that the clumsiness could be a symptom of weakened legs.
The Neurologist also noted an absence of reflexes at the elbow and partial loss of reflexes at the knee and ankle.
He told me that he believed I was suffering from a condition known as CIDP. He explained that it was very rare, but that there were some treatments available that were often reasonably successful, but in order to confirm the diagnosis, he would like to have a nerve conduction test done. He warned me that it could take some time to get an appointment.
I was able to tell him not to worry about the timing, I had a test booked for the very next day. I did wonder however, whether the parameters of testing might differ, depending on whether the testing was looking from an Orthopaedic Surgeons perspective or from a Neurologists perspective. When I presented for the test, I explained to Dr Frith that we had a suspected case of CIDP, just in case that altered the direction in which he might be looking.
The test consisted of sticking needles into various parts of my body and applying electrical impulses into the needles and measuring the reaction time along lengths of the nerves. Dr Frith was collecting and analysing the data as we went, so right there and then he was able to tell me that there was evidence of demyelination of the nerves and he concluded that the Neurologists diagnosis of CIDP was supported by the nerve conduction test.
At this stage we were now near the end of April and I had a follow up appointment with the Neurologist on 1 May to discuss the results of the nerve conduction test. Meantime, however, we now had a confirmed diagnosis so I started googling CIDP frantically.
It was a little daunting, but I clung to the fact that most of the information I read, talked of a 70% success rate, although what constituted ‘success’ seemed to be quite different from case to case. What I did pick up on though, was that it was often stated that success was largely determined by how soon a diagnosis was made and how quickly treatment was started.
It was only a little over a week before I was due to see the Neurologist, but I figured that we may as well get a head start on treatment – the sooner the better right, so I emailed my GP and explained that the diagnosis of CIDP had been confirmed. He emailed the Neurologist who then rang my GP and said let’s start on 60mg prednisone. So this was started in the last week of April.
At the meeting with the Neurologist, he said we’d start off on prednisone 60mg, as well as azathioprine and he’d see me again in 3 months. I asked whether prednisone was the best mode of treatment as I had read about IVIG and Plasma Exchange. He said that most people respond well to prednisone and that it was a lot cheaper. He said it’s very difficult to get approval for IVIG as it’s very expensive and he said plasma exchange is not used very often. He said the azathioprine is designed to work in accord with the prednisone, providing a longer term suppressant effect to the immune system.
After this, I started doing some really intensive research and had read pretty much everything there was to read about the condition. Three pearls of wisdom became apparent, courtesy of Dr Kenneth Gorson. I watched a lecture that he conducted where he outlined the 3 proven methods of treatment (of which azathioprine was not one) and he outlined the mistakes that Neurologists generally make when treating this condition.
He said that they:
- A) undertreat – prednisone, for example, is proven to be most effective at a dose of 1mg per kg of body weight. My bodyweight at the time incidentally, was 84kg.
- B) they don’t monitor results often enough
- C) they persist with a treatment that doesn’t work for far too long.
His advice is that you must treat aggressively, monitor for results and if the patient doesn’t improve, or if they decline, switch to a different form of treatment very quickly.
So it seemed my Neurologist was doing all three – undertreating, not monitoring, and if he wasn’t seeing me for 3 months, how will he know if the treatment was or wasn’t working, so he certainly wouldn’t be able to switch treatment if needed.
I visited my GP, raised my concerns about undertreating and said that if we’re going to do prednisone, let’s do it right and do the dose at 84mg. I mean, if after a while we found that the treatment wasn’t working, we don’t want to then be saying “well maybe the dose wasn’t high enough” – rather, lets hit it hard right up front. After all, Dr Gorson said that the key is to treat early, treat aggressively and monitor often. So my GP emailed the Neurologist and pretty much asked permission to increase to 80mg. Meantime though, I increased the dosage myself anyway. The Neurologist responded shortly after and said he didn’t really feel it was necessary but said it shouldn’t do any harm as long as we monitored for side effects.
As the weeks went on my condition gradually worsened. By mid-May I was having trouble walking, I was struggling to dress myself and stairs were off limits. I could no longer drive. By my 52nd Birthday on the 27th of May I had moved in with my 83 year old mother so she could cook for me, help me dress and cut up my food.
I run my own business so I had to get in to work each day – I was taking an uber to and from the office and the only thing I could actually do when I got there was clear emails, but at least being in the office for a good portion of the day meant that I could keep an eye on things. I feared that if my staff saw me go completely absent that they might think the worst and start looking for jobs elsewhere, so it was important to maintain a presence at the office.
It was pretty clear to me by early June that the treatment was not working and that I needed to shift to IVIG treatment. I got in touch with my GP and got an appointment arranged with the Neurologist.
At the meeting I explained my concerns and said that I felt we needed to move to IVIG. He said “Well it’s very expensive you know, I doubt we’d get approval for it.” He then said let’s examine you and see how you’re progressing. He sat me on the edge of the bench and did the same tests he had done a few weeks prior. He had me hold my hands out straight and he pressed down on my fingers whilst asking me to resist as much as I could, he did similar strength tests with my arms and my legs.
He sat back and said “Well your condition hasn’t worsened at all.”
I was gobsmacked – I said to him “Now hang on, the last time I saw you, I drove here by myself, I dressed myself on that morning and I cut up my own food. This morning I had an uber driver bring me here and the driver had to open the door for me, not out of courtesy, but because I physically couldn’t. I was fed and dressed this morning by my mother and you’re trying to tell me my condition hasn’t worsened – well I can tell you, that your method of measurement is seriously flawed!”
He stammered for a bit and said “Well, I’m looking at my notes here and at the last measure, your hands were “moderate to severe”, and I’d still classify it as “moderate to severe” “. Seriously, as far as a measurement scale goes, that’s a bit like saying “on a scale of 1 to 2…”
I said to him that I think it’s fairly clear from any practical perspective that the prednisone IS NOT WORKING. I said that I want to try IVIG. He said (again) “well, you know it’s very expensive, it’s over $9000 for a treatment.” He really wanted me to know how expensive it was!
Now, I’m a fairly pro-active sort of person (in case you hadn’t noticed), so I’d already done a bit of research. I’d printed out from the Blood Service website the form that a practitioner can submit to the Registrar of the Blood service to get urgent approval for IVIG. I handed the Neurologist the form and said “this is what you’ll need to make the application – they can usually approve, under urgency within 24 hours, please make sure it is for the recommended loading dose of 2grams of IVIG per kg of my bodyweight.”
I had a call a couple of days later from North Shore Hospital and was booked in for IVIG on 17 and 18 June, a total of 168grams.
On the day of the infusion my Mother drove me in and the nursing staff were absolutely wonderful. The process took two days, 8 hours each day and I just sat in a reclining chair the entire time, hooked up to an IV line into which the IVIG solution was pumped.
Let’s divert slightly, and talk about the grip strength meter that I bought a while ago. One of the pearls of wisdom that I had read about early on was that grip strength was a really good barometer of a person’s overall muscular strength and well-being, and in particular, with a condition where the loss of muscle strength is gradual, measuring grip strength gives a really accurate representation of the improvement or decline in strength of the body over-all.
Early on, prior to the diagnosis, the Hand Therapist had measured my grip strength at 12kg. When I had received the diagnosis, I bought myself a grip strength meter and everyday I have charted my strength since.
It showed a gradual decline, with that decline continuing over the period of prednisone treatment. All the way down to 3kg of grip strength.
This data is how I can be so confident that my strength was declining, even though the neurologist was trying to tell me I wasn’t getting any worse. You can’t argue with data, right!
On the day of the IVIG treatment my grip strength was 3kg – less than a 3 year olds. Everyday for months prior my strength had been decreasing steadily. On the 20th of June, just 2 days after the IVIG treatment, my strength increased for the first time since this all started. A modest increase to 5kg. Then a couple days later it was at 6kg, then a couple of days more it was 7kg. This was huge, at 7kg I could actually do up buttons and hold a butter knife. This was a massive relief – at last, treatment was working and I could feel myself getting better. I could start doing normal tasks again.
Within a couple of weeks my life was totally transformed, I could dress myself, feed myself and walk to the train station rather than rely on an Uber.
Within a month I felt ready to return home. My grip strength was 20kg at this time. As much as I so appreciated my mother’s help, it was a huge thing to be able to return to my own home and start getting back to normal.
I was amazed at the improvement in strength. I felt terrible though, I’d gained a lot of weight and the steroids had really taken a toll. Everything I had read was that if steroids don’t work, stop taking them, but the Neurologist had said I should keep taking the steroids in conjunction with the IVIG.
I really felt that this wasn’t a smart move, but just to be sure, I emailed Dr Gareth Parry (through the GBS/CIPD support group website) and asked his opinion. He said that without examining me, he couldn’t be sure, but from what I described it sounded to him as though I should definitely be stopping the prednisone.
That was good enough for me. In conjunction with my GP’s help, I started weaning off the prednisone. I didn’t ‘fess up to the Neurologist about this because I was concerned that if he took umbrage at me going against his advice, he might not be helpful in arranging subsequent IVIG treatments.
After weaning off the prednisone, my strength continued improving, but all the research I’d read had suggested that multiple rounds of IVIG were usually required to treat the condition, typically at 4 week intervals, and the frequency of the interval should be either increased or reduced depending on whether the patient is still improving by the end of the interval, or beginning to decline near the end of the interval. For example, a typical treatment regime might be to start with a loading dose of 2grams IVIG per kg of body weight, and then to monitor for improvement. If effective, the improvement is monitored and a subsequent dose administered at 4 weeks. Near the end of the next 4 week interval, if the patient is still getting stronger, the interval might be extended to 5 or even 6 weeks, but if the strength begins to decline near the end of the interval, the interval may be shortened up to say 3 weeks, or 2 weeks. If improvement is constant, the interval might be extended out and out till eventually it is stopped altogether. The key is constant monitoring.
So I was fully expecting to hear from the Neurologist sometime prior to 4 weeks after the first treatment to arrange round 2 – but nothing – not a word. So I went through my GP to get hold of the Neurologist and arranged a meeting. He seemed surprised that I expected there to be another treatment – what? – did he think this was going to disappear after just one treatment? Possible, but that would have to be the most exceptional case ever heard of.
Of course, I got the lecture again about how expensive the treatment is. I swear, you’d think he was paying for it out of his own pocket. Nevertheless, he conceded, and he applied for a second dose and that was arranged about a week later, around 5 weeks after the first treatment. My concern was that if we didn’t get the second treatment in quickly enough, we might lose the efficacy of the original loading dose – after all, there’s a reason they recommend a loading dose.
Once again, I’ll take a slight diversion here and talk about the azathioprine that the Neurologist had also prescribed. Many studies have been done where azathioprine has been used in conjunction with prednisone and with IVIG as well as studies where prednisone and IVIG have been done alone. Unfortunately the main study in this area had some design flaws so the data isn’t all that reliable, but at this stage there is absolutely no evidence to suggest that azathioprine is effective in treating or aiding the treatment of CIDP.
Nevertheless, the Neurologist had prescribed it- so I took it- but it gave me really severe nausea. I stopped taking it for a while but the Neurologist had said I really needed to keep taking it as it is a second line of defence. So I resumed it again. The early hours of the morning after resuming the axathioprine, I woke up with the most horrendous stomach pains. I had never felt that ill before in my life. I sat on the toilet and seriously thought it would be far more pleasant to just curl up and die. The next day I couldn’t get out of bed, the pain was emanating from the upper part of my stomach, above where I imagined the entrance to my stomach was. I have a small hiatus hernia so I wondered if it was something to do with that. I managed to call my GP and made an appointment and with some help made it to his office. I told him I had just resumed azathioprine the night before, so my GP did a search of the doctor’s version of google and said “No, it can cause nausea but not stomach pain like this”. So he decided it was acid reflux and prescribed a stronger dose of omeprazole and pain killers. The pain killers really helped but I wasn’t convinced of his diagnosis.
I came right after a couple of days but wasn’t keen to try resuming the azathioprine again just in case, but after a week, the memory of the pain had faded so I tried again. Within a couple of hours I was in excruciating pain, but this time I threw up, clearing my stomach and didn’t suffer quite as badly. But it did confirm for me that azathioprine was the culprit. I did a bit more research on it, and it turns out that in some cases it can cause inflammation of the pancreas, a condition which can leave you feeling like death and was consistent with everything I had felt. Acid reflux, my foot!
When I next saw the Neurologist I explained that I just couldn’t tolerate the azathioprine, so he prescribed a cousin to the drug, called methotrexate, which he said will have the same effect, effectively to calm down the immune system.
Back to the story about the second dose of IVIG – after the second dose, near the end of July, my strength continued increasing and life was really starting to get back to normal. But then by the end of October I noticed that my strength gains had started to level off and then to decline slightly. I certainly couldn’t complain about this, because at around 40kg in strength there really wasn’t anything I couldn’t do that I had done before. I was riding my bike again, I was going to the gym again, I began walking on weekends with a hiking group and I was gradually losing the fat that the prednisone had very kindly deposited about my face and midriff.
I was aware, however, that all the research suggests that multiple treatments of IVIG are generally needed in most cases to get the condition under control. I think I’m very lucky to have responded so well to treatment – most people taper off in strength much sooner after a treatment, so to last 3 months is great.
So I contacted the Neurologist again and explained that my strength was starting to decline and that I felt that a further dose of IVIG was in order. It had been 3 months since the last treatment. I feel that we were following the guidelines fairly well here, because we were doing the next dose at the point at where my strength was only just starting to taper off. What really surprised me though, is that without consultation with me, the Neurologist booked in the third treatment at a much lower dose – just 66 grams (should be 88gms).
From what I had read, the accepted protocol is to either reduce the frequency, or to reduce the dosage – not both. So the third treatment was a bit of a token gesture. Nevertheless, it seems my body was responding really well, even to the small amount of IVIG and my strength began improving again within days of the third treatment.
Within a couple of weeks I was back up to around 42kg in grip strength – about where I had got to a few weeks previous before the strength started to taper off (it had fallen away, pre-third treatment, to about 35kg).
According to Google a 50 something year old male has a grip strength anywhere between about 40 and 50 kg, so you could probably reasonably argue that I was back to full strength, but the problem is I never knew what my grip strength was prior to getting sick, given that I only started measuring from well into when I was suffering symptoms. I did feel, however, that I still couldn’t lift the same weights that I used to at the gym. So I had a genius idea on how to figure what my grip strength was likely to have been before I got sick.
I used to arm wrestle occasionally with a very good mate of mine, named Glenn. We’d done this for years ever since we were teenagers and the most recent time was around October 2017. (I remember the date as there was a party, and we had photographic evidence – as drunk as we may have been). Over the years, we were always very evenly matched – sometimes Glenn would win, sometimes I would win. On that last occasion, Glenn had won, but only just.
So I rang Glenn and said, “I have a wee test I’d like you to take”. I had Glenn test his strength on my grip strength meter and it came in at 52kg. From that I can fairly reliably say that my baseline strength should be around 52 kg, so I do believe I still have some way to go yet.
So this is where I get all pseudo clinical – Like I said before, I’m no doctor, but I’ve read an awful lot about this condition, and where as the doctors have to concede, that in certain areas they just don’t have the answers, I have the advantage that I can just make stuff up to fill in the blanks. Having said that, here’s my possible explanation for how the recovery is all working.
The immune system has gone off-road and has started to attack the myelin coating of the nerves. This demyelination process causes damage to the underlying nerve and disrupts the signal along the nerve. It seems that after a dose of IVIG, the immune system is overwhelmed by the influx of normal antibodies, and its behaviour starts to be modified something along the lines of ‘normal’. The nerves get some respite from the damage being inflicted on them, and the myelin and the underlying nerve recovers fairly quickly and the nerve starts sending signals again through to the muscles.
Some of the damage though, will have been more severe and certain nerve fibres have died away completely. Now, it seems that peripheral nerves have the ability to regrow, and they do so from the nerve root. This process of re-growing, however, is a lengthy one and I’ve heard it cited that it can take up to a couple of years for a nerve to regrow all the way from the root, right the way out to the extremities.
So my take on all of this is that in the absence of the immune system attacking the myelin on the nerves, the damaged nerves are repairing fairly quickly and are resulting in the majority of my strength coming back, but the dead nerves are going to take a lot longer to regrow. That final 10kg of strength that I’m missing, I believe is the result of the dead nerves. The 39kg I got back, is the damaged nerves starting to send signals again.
My fear is, that if the immune system goes back to attacking the myelin, that the new growth will probably suffer first, and how long before the nerve root gives up on sending out new growth if it constantly gets destroyed in a yoyo effect of attack and then respite, then attack and then respite.
Hence I feel the need to be extremely vigilant with detecting when there is a decline in strength, and getting back in for a further round of IVIG, so as to sit the immune system back down on its butt and to tell it to back off.
Of course, I may have the science behind this entirely wrong, but then the medical fraternity are quite openly admitting they don’t know exactly why IVIG works, but just that it does, in most cases, when administered in certain ways. It may also sound like I’m a little critical of the Neurologist who has treated me, but in all fairness, he recognised the symptoms very early on, and for that I’m extremely grateful. I guess, in his defence, the only thing I can really fault him with is for being extremely defensive of the public purse, and ensuring that not a dollar more is spent on IVIG than absolutely has to be.
So regardless of the science, there seems to be a process by which this can get better. I honestly believe that the success to date of my treatment is largely due to diagnosing the condition early and to acting really quickly to get into treatment.
I’m very aware that there are people who have not been nearly as lucky with their journey, and my heart goes out to you if you find yourself in this position. If you want to talk, you’ll be able to find me through the support group.
I hope this story will be helpful to anyone else who has been diagnosed or anyone searching for answers to the best approach to treatment – or maybe it will be helpful to someone currently struggling to get the best out of their medical practitioner. I really feel it’s important to be very pro-active and make sure you know more about your condition than your Doctor does – it’s a rare disease and I’m afraid not all Doctors or even Neurologists are as clued up as they could be.
Auckland, January, 2020.