The Enlightenment! (well partial)

CIDP – Drew’s Story (so far)

Introduction

CIDP is Chronic Inflammatory Demyelinating Polyneuropathy, or for the longer version: Chronic Inflammatory Demyelinating Polyradiculoneuropathy (because it involves the nerve roots).  The acute (shorter term) version is GBS - Guillain-Barré syndrome, which apparently is much more common.  CIDP does seem to have other names as well, but I am trying to keep it simple (apart from the spelling)!  CIDP can manifest itself in weeks/months and take months/years to recover from (if ever).  There appears to be numerous effects of CIDP and yet so little appears to be known about how you get it and (from a catchee’s viewpoint) how you recover from it.  Certainly books on CIDP/GBS exist, but they vary enormously in their examples and provided facts.  This is my story, based on my experiences, my interpretation of events, voices my opinion on CIDP and how I am coping/coped with it.

One bone of contention is the medical professions obsession with pigeon-holing cases/patients and attempting to make the symptoms fit what they know!  (e.g. GBS short term max. 4 weeks, therefore try and ensure the symptoms lasted no longer so it fits, when in this case they blatantly did not).  Also as my symptoms were only confined to the legs, this again did not fit their pattern for known cases, so again it could not be CIDP.

Why am I writing this? Because the mystery/uncertainty shrouding this condition makes it difficult/impossible to understand and the medical descriptions scared me, much more than they helped. I read someone else’s account and it contained more useful information (in a readable form) than any other document, so as my experience has been significantly different to theirs; I decided to add to the knowledge, in the hope that someone else reading this in the future would gain a small insight into their condition and thus be better informed (oh and less scared!).

My simple motto: “Hope for the best, plan for the worst”.  So if you know what you have contracted you have a chance for both of these.

Notes on Tests/Procedures Performed

During my entire "ordeal" numerous tests/procedures have been performed on me. I am writing this addendum to assist others understand what these tests are and what they should (or should not!) prove - oh and how it related to me.

• Blood Tests - too numerous to list!
• CT Scan (X-Ray) - detects bones, lungs & chest issues (hard structures) - trying to find cancerous growths
• MRI Scan - detects ligament, tendon, spinal cord (softer tissues) - trying to find tumors
• Pin Test - stick a pin in various locations (with you having your eyes closed) and tell them whether you can feel it or not
• Lumbar Puncture - (Celebrospinal Fluid - CSF), health of brain and nervous system, protein levels in spinal fluid (GBS/CIDP)
• Nerve Conduction Study (NCS/EMG) - for evaluation of paresthesias (numbness, tingling, burning) and/or weakness of the arms and legs.

The two that are supposed to be most relevant to diagnosing GBS or CIDP are the last two, in my case the initial protein levels (June 2011) were 2,000+ mg and later (Feb 2012) were 700 mg, then normalis around 500 mg, so very high first time and high the second but not excessively.

The EMG tests (again carried out in similar timescales) came back as slightly low in the right leg and normal in the left hand from the first test and similar but in the left leg and right hand for the second. Please note that some consultants say it does not matter which arm/leg they use and others say it makes quite a difference!

The actual Neurological Consultant I have been seeing has the most unscientific test ever for detecting improvement or weakness! He asks that you push up/down with a specific part of your bdoy, whilst he applies force in the opposite direction (e.g. elbows up, thumbs out, index finger out, legs up/down...).  The results are then written onto a piece of paper with his score on your strength out of 5.  This is so subjective and prone to errors that I find it ludicrous - yet some of his major conclusions are based on this arbitrary test! (surely there must be a more scientific method?)

His all time favourite is with the feet/ankles whereby he wants you to push up and then down. Due to my RTA! I have severely limited mobility of my feet/ankles (especially upwards) so this test is totally invalid for me, as I will never score highly, yet every time he gets very concerned about my lack of mobility there (highest score 2.5 against 4.5 everywhere else, in Feb 2012) and ignores my comments about it being a bad test - which I find very frustrating! 

The Beginning

Where to start….. (this is far more difficult than it should be).

I will attempt to put things in to a rough chronological order, but due to the time lapse between events and me writing this they may not be 100% accurate. 

I am male, 51 years old, 186cm tall (just over 6’ 1”) and weigh around 69Kg (11st) – at the time of writing this.  I am not un-fit, have never smoked, drink a little and nowadays exercise similarly.  Other historical influences will become clear during this narrative. Before any of this occurred my weight was around 83Kg (just over 13st).

One massive thank you I must say is to my wife, 20 year old daughter, 17 year old son and my mother-in-law, whom without their assistance I would never have got to where I am now (never mind their patience and persistence!).

January 2011

For my 50^th Birthday my wife has arranged a luxury trip down the Nile and in order to go we need some vaccinations.  The trip is scheduled for Mid-February, so we go to our GP for the injections and come out with sore arm(s). Around two weeks later at the end of this month I am suddenly struck down by a flu-like virus that completely knocked me out for 4/5 days.

February 2011

Chaos in Egypt! Holiday cancelled – but that was the least of my worries as it turned out…..

I started to feel very lethargic, couldn’t even mow the grass without feeling knackered.  Had to “just go for a lie down!”.  I also noticed pins and needles in my feet/toes and they were continually cold – which is something I have never suffered from before. Put this down to getting old and tried to get on with life.

We did go on holiday to Malta, but I did feel tired after every excursion and this was not at all normal for me.

March 2011

Lethargy getting worse and feet still cold, starting to affect my work, as really struggling to cope with long days and constant driving around.  Decided to go and see my GP around the end of the month as I had given it long enough to go away on its own.

Initial visit to GP, she listened and took some notes and sent me for blood tests to the nurse.  Had the blood tests and waited for the results.

April 2011

At the beginning of the month was told I had type 2 diabetes (which is the controlled by medicine and diet sort, rather than injections). As both my parents were diabetics and had been for a considerable time, this was not a massive worry to me and I went away with some medicine (Metformin) and the testing kit, plus a diet sheet.  Had fun learning to test myself and my wife worked out what I could and couldn’t eat against a list provided (fun – no fizzy drinks, all diet stuff!).

However this had no effect on my legs/feet/toes, which were starting to feel a bit weird, like they didn’t quite belong to me.  More concerning at the time, was that I now had a band around my stomach that was feeling numb and I had less of an appetite.

May 2011

Went to the GP because of slackness on the right side of my face, it looked like it had dropped!  This was diagnosed as Bell’s Palsy, 10 days later I had it on the left side of my face too – this concerned the GP as in all her years of practice, she had never seen it down both sides before.  Proscribed steroids for 5 days – six pills per day, the symptoms lasted for a few weeks before gradually disappearing.  I did find out much later that this can be related to Sarcoidosis.

 I was in and out of my GP’s surgery, complaining about my stomach area, starting to have problems with bowel movement and eating significantly less.  Additionally I was starting to have walking/balance problems. I was sent to our local hospital (both initially as an out and then as an inpatient), which ran lots more tests, stuck me on a drip and proclaimed it was the Metformin tablets and I should change them. My tablets were changed to Gliclazide and informed that some people just don’t get on with certain types of tablets.  However I was getting very worried that this was not the whole picture and something far more sinister was happening.

Walking became increasingly difficult and I was so lethargic I could hardly do anything, yet sleeping was also difficult.  Towards the end of the month, we went on holiday and I had to visit a local GP because my ability to stand/walk was severely restricted, in fact on one walk I had just fallen over and was unable to move for a considerable time.  Due to an RTA (Road Traffic Accident) when I was 9 years old my right leg has always has been very weak, especially from the knee and below.  My right knee has been prone to give way for no real reason (other than I do not put the weight on it properly) and this was now happening with frightening regularity.

The GP advised we went home and sought urgent medical attention, so the holiday was curtailed.  I was now off work as well.

During our holiday, we visited a church and when I looked up to see the spire I nearly fell over backwards, as I really had more and more problems with balance and feeling in my legs.

June 2011

Further, increasingly worried, trips to the hospital and GP left people unsure what was wrong and I was now unable to walk anywhere at all and had to rely on my arms to propel myself around as from the stomach down I was becoming unable to do anything (yet my arms were fine).  I could no longer manage the stairs in our house so ended up sleeping on an air-bed in the lounge and efforts were made to make the trip from there to the downstairs toilet as easy as possible.

As I had private medical insurance, it was time to invoke it and I went to see a specialist in stomach and bowels.  He recommended a course of scans and tests which took place over the next couple of weeks.  I was gradually getting weaker and weaker in my legs and could no longer even stand un-aided, so it was obviously getting very serious.  I was admitted to private hospital to have a Gastron & colon-oscopy  (stick cameras in top and bottom!), as I could no longer walk I stayed in the private hospital and this specialist recommended I see a neurologist, and had other scans and x-rays of my spine and whole chest area, plus lots of blood tests.

The two camera tests proved there was nothing of concern up or down (good! – I thought).  However the scans/Bell’s Palsy revealed I had a condition called Sarcoidosis, which would require a biopsy to check how bad the condition was (with my lymph glands in my chest).  Also other tests revealed that I had a neurological condition, so I had nerve conduction tests (stick a large amount of current through various parts of your nervous system and see how high/fast you jump!), plus a lumbar puncture (a word of warning about these – they procedure doesn’t hurt too bad at all, but the pain afterwards does and lasts for quite a while, so ask for all the painkillers they will give you shortly afterwards! – I’ve had 2 now and both were very similar experiences).

Both the nerve test and the lumbar puncture came back that I had serious issues with my nerves and signals getting anywhere – in my legs, yet my arms were 100% (in fact I jumped so high and quickly the consultant actually apologised and reduced the current significantly after the first test!).  This confused them massively from two viewpoints:

1.       The words GBS were mentioned, but this had taken too long to occur, so it couldn’t be that

2.       My stomach and legs were affected but not my arms or breathing, so again it didn’t fit

It was then first muted I may have the much rarer CIDP – but things still didn’t totally add up.  So the hospital started to run rigorous tests on my breathing, waiting for this to deteriorate and then wait for results of the biopsy.

I was transferred to another private hospital for the biopsy and this was run and I was then (in theory) transferred back.  This is where it started to go wrong! I was taken by ambulance back to the first private hospital, who refused to re-admit me, due to the fact that I had been initially admitted by the gastroenterologist and it was no longer his case.  In tears I begged them to keep me in, but they refused and I had to get my wife to come and take me home.

This was the worst day/night of my life! As I ended up spending the entire time on/next to the downstairs toilet as I had no idea when I would go and minimal control either.  The next day my wife phoned our GP who referred me to the NHS hospital.  An ambulance was sent to pick me up (lying on the floor, soiled and in considerable pain), they stretchered me to the hospital.  I was admitted to a general ward.

BTW: the results of the biopsy showed minimal issues and though I did have Sarcoidosis it was not particularly malignant and would just need an eye kept on it in the next 6 months or so.

Numerous drips (as I was very de-hydrated and pale according to them) and blood tests from the veins and arm later, the same neuro-consultant who had seen me privately turned up at then NHS hospital and confirmed I had CIDP (at last this problem had a name!).  My weight had dropped from 13st to just above 10st! I could not feel anything from my stomach down and my legs looked like I’d come out of a prison camp! (as virtually all the weight I had lost was muscle)

I remained in this ward for a reasonable time and was given pain-killers and medicine to assist with bowel movement.  I worked out a routine with a chair next to my bed how I could get from the bed to the facilities and back again, under my own steam.

July 2011

About one or two weeks into my stay at the NHS hospital, the neuro-consultant recommended a treatment called IVIG (no I am not even trying to type out this one!).  It was a plasma replacement treatment that was designed to assist patients still “on the way down” to recover quicker and start “on the way up!” (these are the consultants words), it would take 5 days.  The problem was that I knew I was actually getting better at this stage, though the consultant was convinced I was not! The basis for his prognosis was that I could not lift either of my feet off the bed and that I could not push “up” with my feet from the ankle.  I kept pointing out to him that this was the very worst test in the world for me, as due to my RTA I had considerably reduced/limited vertical movement, I never did have and so the test was invalid.

I started to see some neuro-physiotherapists, who started to give me simple exercises to keep me moving and get some strength back into my legs, one interesting point throughout all this was their continued wonderment about how/why my arms were not impacted and that I had full capabilities in this area.

After a few weeks when it was clear I was improving, I was moved to the neuro-recovery unit, along with other people who were recovering from strokes etc. This was good as there were physio-therapists on the ward full time and they devised a stringent routine for me to recover as quickly as possible.  My main problem was that I had gone down so far and my muscles had deteriorated so much that I had to learn to walk again, right from the start. Also I still had considerable lethargy so if I pushed too hard I caused myself more problems. Also as the nerves were recovering at around 1mm per day – it could take up to 3 years for me to recover fully (if I ever did! – that would teach me for being tall). Although I have been told since that the nerves had not been destroyed, just the sheath round them; so it should not take very long to recover at all!

The recovery was slow but I was happy I was on the way up!  Boy was I bored!

August 2011

An occupational therapist came to my house and recommended changes to be made so that I could then go home (Yes!). These included a seat for the downstairs toilet, a bath seat, rails in the bathroom (which had already been fitted by a friend), a higher seat for the lounge (borrowed from my mother-in-law), plus a set of bannisters for the outside of the stairs.  Along with a number of aides: Zimmer frame for upstairs, one for downstairs, two walking sticks, a perching stool so I could make myself a drink…..

Once all these changes had been completed I could go home (YES!).  At this time I had managed to walking using two sticks (or a frame) and despite some falls could actually get upstairs!  It was decided however that I would remain downstairs for a while for my safety and the sanity of my wife (as I was getting up to go to the bathroom at least 3 times a night).

The big day arrived I was discharged and came home (you have no idea how good this felt!) and even though I had to stay downstairs the relief and happiness I felt was extreme.

September 2011 – December 2011

A long series of physiotherapy, hydrotherapy and exercises to do at home, to get some muscles back in my legs and as the feelings slowly returned so did the strength.  Gradually the sensations returned, but large areas remained numb and the most difficult part was sensation of slopes and how to cope with them!  I had to see a slope and then adjust manually, otherwise I fell over (which happened anyway!).  I progressed from frame to two sticks, to one stick (advice get one suitable for you height so you don’t stoop, being tall this was a bit more difficult and one with a proper grip handle helps as well). Home help was from my family which meant I didn’t have to do too much at all apart from get better.

In September I moved back upstairs into my bed (and that was a luxury) as I could get up the stairs on my own without too much drama.

In October I was passed by my GP to drive a car (though I had sent the forms to the DVLA – they still hadn’t finished pontificating, at the time of writing this they still haven’t made a decision!).  This was necessary for me to get back to work.  I have changed my car to an automatic, to make it easier to drive. Over time I have driven further and further without too many issues, just stopping frequently and knowing when I am tired.

In November I started doing some work from home, only small amounts at first, as I would get tired very quickly, but began to build up the periods and effort during the month.

December, I used up all my outstanding leave to use the month as holiday so I could be ready to go in January. Physio and exercises having slow but positive effects, back to around 50-60% feeling in my legs and similar in strength.

January 2012

Went back to work full time, though could work from home for 3 days a week. Such as relief to get into the swing of things, gradually hoped to be able to visit customers and get back to normal!

Had symptoms of numbness around the back of my hands and lower arms, also tingling in my fingers and down my arm, ignored this for 2 -3 weeks and then decided to go to GP and get it checked out.  GP checked for trapped nerves, referred me to specialist so made a private appointment to see the same one I had previously as he knew my history.  He organised a scan of my spine and the results came back negative.

February 2012

In Southampton General after more tests in by private consultant, he reckons there has been severe degradation in reflex response, so need to have tests done and urgently.  Had large amount of blood tests and a lumbar puncture performed, results came back inconclusive to CIDP relapse, so given a choice of two treatments, steroids at home (with risk of high blood sugar levels – whilst taking them) or IVIG at Southampton.  Depending on the results of which I chose they may need to perform the other (if I do the IVIG 2^nd they can perform it at Portsmouth – which is much nearer to home!).  I chose the Steroids first and to go home.

Steroids 60mg every other day, kicking in and causing some discomfort (migraine/headache over left eye for around 4 hours turns to muzzy head, slightly dizzy, not feeling well, plays havoc with my diabetes).

Seen doctor who has said these side effects are nothing to worry about and hopefully they will diminish over time.

Been back to the consultant who has stated the steroids seem to be providing some +ve results and I do seem to be able to "feel" the ground with my feet for the first time in ages. Areas are still definately numb. The consultant stated there are still two possible issues, relapse of CIDP or Sarcoidosis, which they are going to carry out further tests for, plus I need to have an eye appointment to check on my glaucoma.

Long term usage of steroids is not good for you and I now carry a blue card to this effect as I could go "cold turkey" and suffer serious withdrawl symptoms of they are just stopped. Need some calcium like intake, though not directly calcium in case I do have Sarcoidosis and I need the calcium due to the soft bone damage caused by the steroids.

Also starting to reduce the dosage of steroids next week.  Approach appears to be very hit and miss as all they will do is reduce the dosage (50mg next week, 40mg the week after) and see if there is no further improvement or a reversal.  Depending on what happens this is where I will remain (which could mean steroids for the rest of my life)!  No one will comment on this as they all refuse to be drawn.

March 2012
Back to work! Lets see what the future holds.  Mentally seem to be OK, physically still improving and have a fair way to go. I seem to get tired very easily and apprently this is common, like everything else it may never completely clear.

Having to answer questions about what can I do, is it safe for me to...... and I just don't know the answers (no-one does, my doc has no idea and refuses even to answer the question in the vaguest way).  All I can do is take 1 day at a time, try and do something and see if I am OK aftewards.

Seeing the consultant on the 13th to check on the effects of reducing the steroids and my continued improvement (or not). The Calcium, replacement is a hum-dinger, take once a week, sit or stand for an hour afterwards (do not lie down!) and don't eat anything in that period either (apparently it burns!) - getting the stuff tomorrow (Friday 2nd).  As for the Sarcoidosis going for a scan later in the month.