Update 48 - HSCT cure for CIDP - Facts (6)

ISOLATION TESTS

Around 3 time a day, a check of your blood pressure, pulse and temperature.  If your BP goes above 130 (higher) or 90 (lower) then they get concerned and if your temp rises, What happens to these affects the doses of medicines they pump into you.

Your weight is also checked at least once per day (most say pre-breakfast).

IV drugs administered after first set of checks, usually 2 bottles (but size/dose does vary). Plus a pack of pills, which are tailored to you for taking 2/3/4 times a day.

Repeat for quite a while, so relax and enjoy!

As you go through the process initially your results will fall, as the new cells take over they should rise.  Below is an example of the different measures, showing them falling after the first three days - this is normal:

If your platelet count drops to below 10 they will need to infuse you with more to avoid any risk of internal bleeding not clotting.

By around day 7 or so (+ or - a few days as everyone is different), that is when your blood cell count drops to the lowest point.  This is known as Neutropenic State (and your hair comes out!).  This is the day you will know the stem cells are working as the count will now start to rise.

Once you Leukocyte levels reach around 1 million, the strict isolation can (or should be) gradually relaxed - as everything is progressing as it should.  This should be around 2 to 3 weeks after the infusion, meaning that some normality can be resumed and it would be possible to come out of isolation and go home (around 1 week or 2 later).  The timescales are up to the hospital/consultants and your individual capabilities.

There are still 3 to 6 months plus of recovery, with multiple hiccups along the way, but this is a major point of advancement and very good.


Notes:  Whilst in isolation, make sure you have plenty to do, after all you are on your own!
             During all this you will feel, weak, dizzy, tired and in a certain amount of pain
             All the numbers etc. will vary between patients

The best advice I've seen through all this is:

KEEP POSITIVE AND SMILE - YOU ARE BEING HEALED!

Update 47 - HSCT cure for CIDP - Facts (5)

The return of the Stem Cells!

This is simply connecting you to a machine and pumping the stem cells back into your body.  Seems to take a while (half an hour to an hour) and then it's over, however the process has an effect on the body (low blood pressure, dizziness, pressure in chest area...).  So it is not painless, just needs to be gotten through.

As an FYI:  They store your stem cells in cryogenic fluid!

Isolation

As stated previously these are just notes taken from a couple of examples, however each patient may vary.

1. In theory all luggage and clothing goes, so you are supposed to be left in hospital gowns only, but it is possible to keep some clothes?
2. Double exterior windows closed and not to be opened at all.
3. Air Filtration systems turned on and all doors out closed except for bathroom and kitchen visits etc.
4. UV lighting in the bathroom, only off when the patient is in there
5. Staff entering in full covering (gowns, masks etc)
6. Shower, once a day
7. Hygiene washes twice a day, one after the shower
8. No toothbrush, just specific mouthwash -3 times daily
9. Limited food types oatmeal, only baby yogurt, no fresh fruit, nuts, vegetables, lots of other limitations that mean bland food
10. Bedding changed each day and room cleaned thoroughly (1 hour to clean the bed - patient in shower during this time)
11. All dishes washed specially
12. No visitors

Having a Wi-Fi connected computer or device and a pre-paid method for access (hospital permitting) is probably a great life-line to the outside world. Also need to ensure this is sanitised.

Update 46 - HSCT cure for CIDP - Facts (4)

Chemotherapy

During the whole process you need what is called an octopus (or mini octopus) - which is a series of tubes and pipes directly connected to your juggler vein.

This allows the drugs to be administered easily.

The chemo is a series of bottles around 3/4 to 1 pint in size, to be administered  during the day/evening.  It seems there are around 7 to 9 and a couple are for protecting the kidneys, plus a mix of pills for antibiotic, antiviral and gastrointestinal needs.

The chemo is administered over three or four days, but other fluids are administered in similar manner to manage the side effects over the next couple of days.

Other pills include:

Cyclophosphamide 200 mg/kg b.w. (SD) for for 4 days with a single dose of rituximab at the end of treatment (Rituximab 375-500 mg/m*2).

These are provided after the main chemo.  There are lots of potential issues with the process, as it is very invasive to the body.  That is why there are a considerable number of anti-chemo drugs supplied to assist.  The process can rate as medium on a pain threshold scales and the body will "ache" plus commonly difficulty in sleeping.

The side effects of the chemo include:

• Loss of hair - which will eventually grow back, but almost as fluff initially and it takes 3-6 months, or so!
• Chemo brain - this effects a significant number of patients from all chemotherapy, but at quite different levels.  The basic condition is loss of cognitive function (mental abilities) and has been proved to exist.  This is considered temporary. however can last for a considerable time after the treatment.
• Damage to Organs - Heart, Liver & Kidney seem to be the main ones that could have issues, though the drugs and monitoring seem to reduce the issues

On a side issue about how many cells need to be harvested before the process can begin.  The absolute minimum appears to be around 1.5 million, with 2 - 8 million being an accepted amount.  However certain organisations want to have lots more than this, which obviously takes a lot more time and pain, due to the extra stimulation.  Numbers as high as 60 million have been reported, but there seems to be no need for it to be this high and have no benefits to the patient (e.g. faster recovery) that has been proven, up to the time of me writing this,

Update 45 - HSCT cure for CIDP - Facts (3)

Once you are accepted (and have paid!) you will start the treatment. Please be aware setting up payment for this type of treatment is NOT straightforward, mostly due to the country (Russia) and the monetary conditions, but also due to the large amounts required and the security checks put in place by banks (it can be rejected even if they have been informed, so have a plan B).

Notes:

• The process itself is not without risk - apparently there is a 1% change of death, but if you have CIDP and it isn't getting cured, then it will only get worse and it could kill you anyway!  
• Also they put tubes directly into your juguler vein - which looks a lot worse than it is (apparently). These they do insert and remove at various phases

Apheresis

The treatment commences with use of a steroid/anti-inflammatory drug via IV (e.g. Solumedrol) - it helps ease the pain from boosting the bone marrow to produce the millions of brand new cells that are needed for the process. The other medicine injected was to reduce the risk of infection (e.g. Neuprogen).

The drugs can introduce difficulties in sleeping and bone pain (which may not be there initially but can come in over time), plus other side effects like your hand shaking.  If you want the full set of sire-effects please look it up on google. Exactly how much pain is felt, seems to be dependent on the patient and their pain threshold - my view would be this should be acceptable, seeing as compared to CIDP and the hope this brings, it must be worth it?

The next stage is getting those new stem cells out of your body!  This is called apheresis.  It is around 5 hours in a day attached to a machine.  Below is a picture of the type of machine used:

It is basically the same type of machine that is used for kidney dialysis.  It draws all your blood out (twice in 5 hours), filters out the new stem cells and puts the blood back.  Apart from having to lie there for 5 hours solid (and the bodily functions that need to be considered) there seem to be minimal side effects.

This is usually expected to last for 2 days, but it can take longer to collect the cells/  It is expected they need over 2 million of the new cells (this is a rough estimate!). Once the process is complete and they have enough cells the patients are given a day or two of rest.

Apparently in some surgeries (e.g. Dr Burt in Chicago) the chemo can be done before the apheresis, this can assist in the production of new cells, but you can get way more bone pain.

The next stage is the chemo....

Update 44 - HSCT cure for CIDP - Facts (2)

Testing for if you are a suitable candidate to have HSCT

If you go to Pirogov, there appears to be minimal actual testing before you go, apart from general conversations/discussions.

A fee is paid for you to have the initial testing (this seems to be around £3,500), but this doesn't include any flights/transfers, which can easily double the costs, plus if you are actually going with someone else their costs of staying.

The tests themselves seem to last for around four days and the main purposes are to understand what your medical age is, if there are any other issues/illnesses that may prevent you from being treated and likelihood to be able survive the process, this relates to the chemotherapy as you have to be "fit and healthy" in order to cope.

Below is the testing sheet from a specific patient:

The above seems pretty standard for CIDP.

The Dr then goes through the results and assesses whether the treatment is suitable for you and as stated above this depends on your biological age.  The next step if you pass is the treatment itself...

Latest news about Pirogov is there is now an 18-24 month waiting list at this facility :-(

Another centre seems to exist in Beijing China:

China Stem Cells Medical Center

They are advertising on the web: China Stem Cell Center

However I have not found a patient with CIDP that has been treated there and so don't have a lot more information about it.

Update 43 - HSCT IS THE cure for CIDP! - The facts (1)

Continuing on from Update 40 (sorry I got distracted)....

What is HSCT?

In simple terms HSCT is using you own cells (rather a lot of them!) to create new cells once the bad ones have been zapped!  There are three main phases to HSCT as far as the patient is concerned:

1. Preparation and collection of cells
2. Destruction of existing cells - via chemotherapy
3. Replacement and recovery - in isolation

A simple picture outlining the process:

For those who want the medical terms/details:

The actual collection of stem cells is known as Apheresis:

Who Treats CIDP with HSCT?

It seems apart from, four listed below no other clinics are performing any HSCT procedures for curing CIDP:

1. In the US (Northwestern) where Dr Burt is attempting to jump through the FDA hoops (and has been for 11 years!) at stage III.
2. In Israel (Tel-Aviv) CITI  - Prof Shirman Slavin (which seems more focused on cancer but does state auto-immune diseases)
3. In Canada (Ottawa) - where there have been a small number of HSCT treatments (59) for numerous autoimmune diseases (including 2 for CIDP) over the past 20 years, however they do not seem to advertise the fact
4. In Russia (Moscow) the National Pirogov Medical Surgical Center under Dr. Denis Fedorenko, this is where people I know have gone for treatment

(If there are any others, please let me know and I will amend this list - this is as much as I can find after fairly vigorous digging - during which I found an article stating someone had contracted CIDP as a result of having HSCT?)

Clinics do treat other conditions (e.g. MS, Lukemia & Cancer) with HSCT; there are some great stories about the recoveries and don't forget that the actual treatment has been known about for over 30 years.

The rest of the (known) World is holding it's collective breath and waiting for the trials in Northwestern to be approved - this is where the comments about Big Pharma not wanting a cure; cloud the issue and I have to agree that 11 years is a long time when other drugs/treatments are approved in 18 months.

It seems that no insurance company will pay for the HSCT treatment for CIDP (again waiting as above) however they do for other conditions.  What people are doing is raising/getting the money individually to get to Moscow to get treated - this is especially happening from the US.

Currently Pirogov can only handle 87 patients a year and newer/better facilities are being opened there as I write.  I don't know how this will impact the number of patients they can deal with.

The next few posts will contain details from existing patients and the facts from their experience to assist others in making decisions about the good, bad and ugly of HSCT for CIDP......

Update 42 - The Lying Toads!

THIS IS BOTH IMPORTANT AND POTENTIALLY WORRYING

It seems that there is general acknowledgement that vaccinations DO increase the risk of getting GBS!

There have been a number of articles related to this in journals throughout the world. Below are extracts from a couple of web sources confirming this:

November 2013: The Liberty Beacon

A new study published in the Journal of the American Medical Association (JAMA) admits that the vaccine widely administered for H1N1,  also known as “swine flu,” is linked to causing neurological damage in the form  of Guillain-Barre syndrome. The findings confirm several earlier studies in both  the U.S. and U.K. that also linked the H1N1 vaccine to severe neurological  damage.

Reuters Health reports that, for the new study, Philippe  De Wals of Laval University in Quebec City and his colleagues evaluated  rates of Guillain-Barre in Canadians during the six months following the initial  roll out of H1N1 in Quebec. Upon review, the team says it observed a definite  increase in Guillain-Barre cases associated with the vaccine.

Liberty Beacon Link

November 2010: Natural News

Despite extensive denial, The Medicines and Healthcare products Regulatory Agency (MHRA) has recently issued a new statement linking the swine flu vaccine with Guillain-Barre Syndrome. Guillain-Barre Syndrome is a deadly nerve disease that may lead to partial paralysis and death. The news comes after governmental claims were issued that the vaccine had passed all of the required tests and was completely safe for worldwide usage. Those who paid attention to the alternative news media, however, have been fully aware of the dangers that go along with receiving a swine flu vaccination.

Natural News Link

Additional references are contained within the articles. Please read and follow the links as it seeems to expose an underlying acknowledgement of increased risks, but not wanting to tell anyone.

There were a lot of stories spread after the 1976 outbreak and the potential increase in having the vaccination and this appears to be happening again! Also it is other vaccines that can have this effect.

As a person who is 100% certain my CIDP was as a direct result of inoculations, this needs to be bought to people's attentions so they can make an informed choice.

Update 41 - More on Pharmaceutical Companies

The more I read and learn about what the most profitable businesses in the world are and how this impacts ordinary people like me and you, who have a "chronic" illness, the more worried I get about motives!

The following two charts are from a you tube presentation on Medical companies:

This first one shows the sales in billions, from 2006, and the breakdown on how it is used.  With Marketing & Admin being twice the size of Research and Development!  Doesn't that just some it up? These numbers seem t have increased substantially over the past 8 years, but from what I can tell, the proportions have remained the same.

What is also very pertinent is the statement in white at the bottom of the chart that states profits from drug companies run at 20% whereas all companies together is 6.5%?  There are graphs around to confirm this is still the difference today -

So why should the pharmaceutical companies change what they are doing and actually "cure" anyone.  They are businesses and doing very nicely (out of us) thank you!

Here is even more proof that the pharmaceutical companies are more interested in keep things going than actually curing people:

Note:  NME - stands for New Molecular Entities - New approaches?

So out of the 93 new drugs approved 75 were "me too" drugs that are doing the same job as existing medications, that is 80%. 12 were new but had no improvement and only 6 were new/improvements on existing drugs. That is just over 6%!

Thus out of the $37 Billion for R&D only $2.2 Billion is delivering new drugs that may be game changers. If you analyse that against profits that is less than 5% and against marketing it is around 3%!!!!

The link to the you tube clip (and plenty of longer versions) is below:
Pharmaceutical Profits

I will get back to HSCT next time......

Update 40 - HSCT IS the cure for CIDP! - Why it is NOT a good idea?

I have just amended my previous post heading, due to a number of factors.  One more reading of really interesting articles. Two that 2 people I am conversing with are going through HSCT as I write.

The details below (and following blog posts) are a precis of their adventures and the facts (as I see them from the UK) about HSCT.

To lay the ground for my future posts and comments below are some interesting facts/statements that come through from the other posts (to set the scene):

1. More and more people seem to be getting GBS/CIDP and there is still "no cure!"
2. If you take the number of people who contract GBS as 1:100,000 and CIPD as 1:,1,000,000 then with over 7 billion people on the planet this equates to 70,000 cases of GBS and 7,000 cases of CIDP per year
3. The large pharmaceutical companies are making a fortune out of these types of illness, so they are not interested in finding cures as their revenue stream would die out (proof below).
4. In the US it costs about $200,000 per year to "treat" CIDP with IVIG. with an estimated 320 million people in the US if 50% of the people diagnosed with CIDP have IVIG treatment and there are 320 new cases per year the money to be made is growing at $32 million per anum.
5. If you worked this out worldwide (again just for CIDP) this is $7 billion per annum - how about that for a bottom line to a company that has to do no further work/research!
6. Assuming the current number of CIDP patients currently resides at around 400,000 (this is low because I am assuming known about and therefore being treated). Then the existing return is $80 billion per annum
7. If you added in the people with GBS who get "cured" after a treatment of IVIG - assuming an average of 1 months worth of treatment this would add another $6 billion to the total.
8. So pharmaceutical companies are getting $93 billion out of this suite of illnesses in 2014 and it is continually growing at around 9%!

Now you know why the pharmaceutical companies don't want a cure! And are willing to spend a lot of money making sure one is not found! 

What makes the above worse is that I am being conservative in my estimates so the number is far higher.  Yes I do know the there are other treatments (e.g. steriods) - which are cheaper, but have far more serious side effects - this is why I've only used 50%. I am also aware there are costs in creating the IVIG solution.

Even if their costs were half the total above, they still make $46.5 billion in 2014 out of GBS/CIDP.

And this is just from one set of illneses where HSCT can help

HSCT has been known about for over 35 years, yet it is still "in trial!/not proven!" why not.......

Update 39 - Is HSCT the answer for CIDP?

Being well aware of the limited success of recovery from CIDP, I have been trying to find out about the muted alternatives such as HSCT (haematopoietic stem cell transplantation) can be seen as AHSCT (Autologous!).

Now there seems to be a lot written about HSCT being a miracle cure and there appears to be a large amount of smoke and mirrors going on, however these claims seem to be promising at best and dubious at worst.  Yes there is proof that trials have successes against all diseases, but there have also been issues.  In the U.S. limited trials appear to being performed, into MS & CIDP etc.  There also seem to be a significant cost to these treatments and significant risks (that are missed by certain clinics/practices).

I have currently found a number of articles/posts that I believe sum up the current situation:

Report from the US June 2013:

 Abstract

OBJECTIVE:

Only 70-80% of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) respond satisfactorily to the established first-line immunomodulatory treatments. Autologous haematopoietic stem cell transplantation (AHSCT) has been performed as a last treatment resort in a few therapy-refractory cases with CIDP. We describe the results of AHSCT in 11 consecutive Swedish patients with therapy-refractory CIDP with a median follow-up time of 28 months.

METHOD:

Case data were gathered retrospectively for AHSCT treatments in 11 patients with CIDP refractory to the first-line immunomodulatory treatments, intravenous high-dose immunoglobulin, corticosteroids and plasma exchange and to one or more second-line treatments used in 10 of the 11 patients.

RESULTS:

The median Inflammatory Neuropathy Cause and Treatment (INCAT) score within 1 month prior to AHSCT was 6 and the Rankin score 4. Total INCAT and Rankin scores improved significantly within 2-6 months after AHSCT and continued to do so at last follow-up. The motor action potential amplitudes (CMAP) improved already within 4 months (median) after AHSCT. Three of the 11 patients relapsed during the follow-up period, requiring retransplantation with AHSCT in one. Eight of the 11 patients maintained drug-free remission upon last follow-up. AHSCT was safe but on the short term associated with a risk of cytomegalovirus (CMV) and Epstein-Barr virus reactivation, CMV disease, haemorrhagic cystitis and pancreatitis.

CONCLUSIONS:

Our results though hampered by the limited number of patients and the lack of a control group suggest AHSCT to be efficacious in therapy-refractory CIDP, with a manageable complication profile. Confirmation of these results is necessary through randomised controlled trials.

Information from DIADS web site into MS

Hope not hype

Despite the best efforts of researchers, stem cells are often portrayed as a miracle cure, and many MS patients who learn about stem-cell therapy from breathless newspaper articles or television features face let-downs. Even the clinicians had to learn some hard lessons in early studies. "In our HSCT clinical trial, we started with patients who had fairly advanced disease, wheelchair-bound or even bed-ridden," says Richard Nash, a specialist in immunotherapy at the Colorado Blood Cancer Institute in Denver, Colorado "and we found that for many of these patients, even after transplantation they are going to continue to get worse."

The outcomes of subsequent trials have been markedly improved by the recognition that patients with advanced MS might have crossed a threshold of nervous-system degeneration beyond which anything short of neuronal regrowth or replacement is likely to fail. This can be a difficult message for individuals with severe MS to hear. "The only time that patients get mad at me is when we don't offer the transplant," says Burt. "It's hard to get patients to understand that this isn't going to help them."

In some cases, patients have pursued treatment at so-called 'stem-cell clinics' around the world, where they pay tens of thousands of dollars to be injected with cells of dubious provenance in an environment with minimal regulatory oversight. These clinics claim to treat any number of conditions with stem cells but offer little in the way of peer-reviewed efficacy data. At least one published report described a patient who developed tumours after a clinic transplanted fetal stem cells, and in May 2011 Germany shut down the XCell Centre, where a young patient died from complications following autologous MSC transplantation. More recently, a case report from a team of neurologists in Arizona described how a teenage MS patient suffered a severe and debilitating inflammatory response during a course of stem-cell treatments at a clinic in Costa Rica. The physicians were unable to determine the extent to which this strong immune response was attributable to the transplant, but they cite this example as justification for restricting experimental stem-cell treatments to clinical trial settings with proper oversight and safeguards. "We are really fighting those clinics," says Martino, who has collaborated with colleagues in a survey of clinical stem-cell research in MS. "We prepared this leaflet that anybody can easily download from various MS society websites, where we explained exactly what stage we're at with the different types of stem cells."

Most stem-cell researchers see cause for optimism but point out that good science and good medicine require considerable amounts of both time and effort. "The stem cell you use and how you use it will depend on the disease you're treating as well as the stage, and it's just beginning," says Burt. "When I first started I thought I'd have all the answers in five years. But it doesn't work that way — it takes time."

YouTube Web Link to a CIDP story

https://www.youtube.com/watch?v=18-HW1A_18g

To me - all this says progress is being made, but there are still issues and not enough is known/proven.  From an outside perspective it is easy to look at all the good news stories and headlines and go rushing to a clinic and signing up for $40,000??? to go through this, to recover from CIDP.  But the headline from the MS story of Hope not Hype sums it up.

Maybe it is because I have got a reasonably normal life back, so I am not desperate to be cured?  Others will think the risks and the costs are worth it - and good luck to them.

If there is more relevant stuff out there, I will include it and I am still digging!

Update 38 - Latest Consultants Visit

Here is a little anecdote that sums up the NHS in the UK:

I have just been seen by my consultant at QA Hospital Portsmouth.  The appointment was for 12:00 in Wednesday.  So I arrived at 11:45 and asked the receptionist if the surgery was running late as I had to drive to north of Blackburn (around 280 miles) straight afterwards and if it was running late I would get a sandwich before so I could get going promptly.  "No - we are running right on time!" said the receptionist.

At 12:05 the consultant came out and said "Mr Harris?" and another gentlemen got up and went with him to be seen!  I looked across at the receptionist and gave her a questioning stare.  She came over to me and said that was his 11:45 appointment and not to worry I was next.  When I queried the fact that 15 minutes earlier she had told me they were running on time, yet the 11:45 patient was already in the surgery and that I had specifically asked, she replied "Well I didn't know how late he was running until the 11:30 patient came out!".

I gave up at this point because there was nothing to gain by stating that she must have known it was running late because the 11:45 patient hadn't gone in near that time (Sigh!).

Obviously this strategy is used to calculate their waiting times as the NHS always seem to point out how good they are, yet every visit I have ever been to has been around half a hour late of when it was scheduled for.

I eventually went in and was seen.  The conclusions of the visit are encouraging:

1. I have become stronger in my foot flexion and ability to push back with my muscles
2. My reflexes are coming back
3. I have feeling in more areas of my feet and legs

I asked about the constant tiredness, especially after doing anything and he responded that this was fairly normal and likely to continue.  I also asked about the sharp stabbing pains in my feet and lower legs, again he said this would continue and there was little that could be done about it, we discussed pills and I said though sleeping pills did help I was not willing to take them because I felt terrible the next morning and long term usage was not recommended.  So hello to 03:00 for a while longer.  I feel that if I could sleep through the night it may improve my general fatigue issues.

He says as I am slowly improving without pills, so unless I had any sort of relapse I would no longer have to see him again, as I was doing the right things and understood what was required.

I do have to go and see a chest expert for further tests on my breathing and Sarcoidosis, so off to the GP's on Monday to get that arranged.

Update 37 - About CIDP & Diabetes

Having managed to acquire both conditions.  I am seriously considering whether there is a link between the two?  When I did my survey only 10% of people with CIDP/GBS said they were diabetic, but how many don't know they are, as it was only found out for me whilst trying to work out what was going wrong.

Especially as a number of the symptoms are exactly the same (regarding sensory loss in the hands and feet).  From the charts put up last year for the US conference, it seems that people like me need to satisfy all 5 conditions and 3 of them could be diabetes:

One of the doctors attending stated he uses 5 points and if the patient has 3 out of 5 then it is CIDP:

1. Progressive course - it is getting worse slowly
2. Symmetrical proximal weakness
3. Loss of reflex - also EMG's
4. High CSF Proteins
5. Demylinating Features

So actually on their scale I'm not sure I have CIDP???!  But I did and still have, due to the severity and the other issues.  Maybe it is another reason why they took so long to find it.

One great bit of news about changing one of my diabetic pills to Lynaglyptin is that I no longer have to stab myself in the fingers to test my blood sugar levels - as neither of the two I am now on can cause hypo's or low's - which is what they are most worried about.  High's kill you slowly, low's kill you very quickly.  My fingers no longer feel like pin cushions and I am not a significant drain on the NHS for the constant use of the measuring strips.

Update 35 - Pains & Fatigue Explained

This follows on from my previous post and the unsatisfactory answers I received about fatigue from a neuro-consultant. I know a physio who deals with GBS/CIDP on a daily basis, so I asked her the questions I asked the useless consultant. Below are my questions and her answers:

Questions:

I have a number of questions specifically about CIDP:

1.        Why do I still feel fatigued doing normal things and if I do a bit more it seriously takes  it’s toll? (I am like a battery and if I use the charge too quickly the re-charge timeframe is significantly longer than if I keep to minimal running levels)

2.        My toes and feet still get stabbing pains at night which wake me up and so I don’t get too much sleep, is this normal/usual?

3.        My improvements in feeling seem to have either slowed down or stopped, since I came off the steroids – maybe that is a co-incidence – will I ever get close to full feeling again?

For all these questions I wonder whether I have now reached what will be “normal” for me from now on?

I saw a different consultant a short while ago and his answer to the first 2 were “It’s because you’re getting older and to be expected” which I found to be totally unhelpful and whilst relevant for people in general, wasn’t necessarily relevant to people like me who are supposed to be recovering?  I am now waiting to see my usual consultant Dr Gibb as I really felt this new one was a waste of time and frankly condescending towards me.

I am aware that you are not a consultant, but you have real experience about CIDP and see it every day (and I trust your opinion and that you will be honest with me).

Answers:

As you rightly say, I am not a consultant; I shall attempt to answer your queries based on my clinical experience and research knowledge. Please remember these answers are my opinion and far from definitive.

1.      Fatigue: this symptom is a tricky beast. Fatigue in CIDP is likely to be a combination of central neurological fatigue and peripheral neurological fatigue. The mechanisms of these phenomena are not well understood. Current understanding is that peripheral fatigue acts much as you describe: activity beyond a certain threshold leaves you feeling fatigued with an extended recovery period. These effects can be measured in the muscles. Central fatigue is more complicated; it involves changes in the brain in response to signals received from nerves in the body. Central fatigue causes the perception of fatigue without corresponding physiological fatigue exhibited in the muscles. Obviously each individual with CIDP is different and the course of the disease and the symptoms are variable from case to case.

Your CIDP fatigue symptoms are probably a combination of peripheral fatigue (thanks to the interrupted conduction of your damaged peripheral nerves) and central fatigue developed as a knock on effect to "signal failures" in the body. So if normal activities are leaving you exhausted it may be logical to assume that you are suffering mainly from peripheral fatigue:  if you do more activity than a certain threshold you become exhausted. The best way to treat peripheral fatigue is with very gradually progressed exercises during which you work to your threshold (and expect to feel tired afterwards). Gradual progression can slowly lift your threshold. The idea is to maintain exercises (which tire you out) in order to keep your peripheral fatigue threshold above that of normal daily activities. Thus normal daily activities cease to drain your batteries, but exercise still does and should (up to a point of course).

As you have also had CIDP for a while now, so it is very likely that central changes have developed too. This makes your fatigue more complicated, because central fatigue can make you feel exhausted even when your body has not reached its threshold. Central fatigue is like a programming error in a computer, so your brain receives "normal signals" and interprets as them as exhaustion, this makes you feel dreadful and is just as functionally limiting as peripheral fatigue. Central fatigue is more complex to manage. Cognitive Behavioural Therapy has been demonstrated to be effective in some neurological conditions, as has gradual exercise (to a certain extent). However central fatigue takes much longer (years) to reset than peripheral fatigue, and it often seems like it is unconquerable. This can lead to people becoming discouraged, which frustratingly can actually make the central fatigue worse. This is because mental wellbeing has a significant impact on the brain changes involved in central fatigue. Because central fatigue is such a complex process of brain changes and it is influenced by such a wide range of factors, it is difficult to unravel for each individual and results in unpredictable episodes of fatigue even when peripheral fatigue is well managed. The best advice I can give is allow yourself time and keep a positive mental attitiude (much easier said than done of course!)

2.      In my experience night pains are not an unusual symptom to be reported in CIDP. It is important to try to mange these symptoms however because quality of life and central fatigue are both majorly impacted by chronic sleeplessness. There are a number of strategies you can try. The pain is likely to be caused by one of two mechanisms (or a combination of both): either small localised muscle spasms due to abnormal motor nerve conduction or abnormal sensory nerve activity giving rise to parasthetic sensations. The first can often be considerably eased using Quinine tablets (quinine is present in small amounts in tonic water too). The second can often be reduced using neuropathic pain killers (such as Gabapentin or Amiltriptyline). A third option can be sleeping tablets. It would probably be a good idea to discuss some of these options next time you see your consultant or your GP.

3.      Your sensory loss may stay the same, it may worsen or it may improve. Sorry to be so vague on this one. Every person is different and every case of CIDP is different. In general CIDP is a progressive condition, but it can be stable for long periods at a time and it may never change at all for some people. On the other hand some people experience huge improvements in response to therapy or reduced stress. The only way to know how it is with you is to "experiment". You could discuss with Dr Gibb going back on steroids at a low dose and monitor your sensation for a while; but bear in mind the side effects of long term steroid use. Also using steroids may not help the sensation at all anyway. 

        Alternatively you could try sensory therapies, although there is little evidence to support these. Sensory therapies include deep tissue massage, sensory challenges like putting your feet in warm water then swapping to icy water and back again and using different textures to run over your skin alternating between light touch (like feathers, silk and fur) and scratchy "pin-prick touch" (like hessian, sandpaper, Velcro hooks).

I hope this information is helpful to you.


Personally the above makes much more sense to me and I can cope with because I have an understanding of what is/has happened to me.  I have said a great big thank you to the Physio who provided these excellent answers and whilst she is not a consultant she certainly knows more about the real issues and impacts.

Update 31 - Information on CIDP (Part 3)

....continuing from previous blog posts....

In 2009 a supposedly definitive list for qualifying for CIDP was produced (by statisticians and leading Neurologists in the US).  The simplified version is:

Patients with symptoms for over 8 weeks would be classed as CIDP if:

• No genetic abnormality
• No other substances introduced for other issues

AND EITHER

• 75% of nerve tests had a response with over 50% of these showing an issue

OR

• Symmetric weakness in all four limbs and weakness in at least one of the hips and shoulders

However these are not seen as relevant by the majority of NEUROLOGISTS!

Treatments

The only treatments that are proven (clinically in a randomised control trial - RCT) to work on CIDP are:

• Prednisone (Steroids) - 40 to 95% of patients respond positively, after 2 months of usage is the peak benefit.  There is a 30% chance of remission.
• Plasma Exchange - similar responses to Steroids - the main issue is access (thus not the first line therapy)
• IVIG - much later process, though the initial trials were much shorter - 6 weeks. Around 40-50% respond. Later trials showed similar numbers made an improvement (on arms and legs) at 47.5%, though in the same trials 22.5% who had placebo improved which means you get better anyway. But they still don't know how it works!

Dr Barohn states his recommended 1st line treatments are Prednisone or IVIG and if they do not respond then switch to the other (say after a couple of months).  Roughly 50% respond to the first and 50% respond to the second, so 75% overall.  If they are already in the hospital then Plasma is a much easier option as this makes it much easier to administer.

Other potential treatments mentioned (copyright names in brackets):

• Mycophenolate (Cellcept) - Not proven
• Interferon (Avonex) - trials negative
• Etanercept  (Enbrel) - not proven
• Rituximab (Rituxan) - not impressed in neuropahy, not seen much response
• Alemtuzumab (Campath) - too toxic!

SubCutaneous IVIG (SCIG) - new development of IVIG trials being run now.

Improvements are seen first in the muscles nearer to the core (hips, shoulders) before the ones further away (feet, fingers).

CIDP Prognosis

In 1975 (P J Dyck) created a set of stats and this was reviewed in 2007:

                                                   1975               2007
Mortality:                                       11%                3.1%
Working with Disability:                  60%               63%
Not Working:                                  8%              
Wheelchair bound:                         28%                  7%
Recovered:                                       4%                31.1% (however with treatment)
Aids to Walk:                                                       28%

CIDP Caveats

1. Don't undertreat (IVIG especially - min 2 to 3 months) 
2. Don't overtreat (Steroids really nasty side effects)
3. Don't expect too much from the treatments
4. Change treatments if it doesn't work

The likelyhood of patients ever fully recovering is very low, less than 10%. Most people will never recover fully and to tell them so is completely misleading. Strength will be much better - hopefully, but there will be weakness remaining.

Update 30 - Information on CIDP (Part 2)

Further information from the video on CIDP......

Nerve biopsies are not useful for detecting CIDP - a lot of places can't process them properly (in the US? - bar Huston and  a couple of others) and the information can be conflicting as there is no definite outcome. Also the I stands for inflammation and there is not always inflammation shown in the nerve biopsy (maybe the disease needs renaming?)

CSF  examinations (lumbar punctures) show high levels of proteins in 80-90% of cases, however if the other symptoms were there then even with levels at 40 - 50 this would still be CIDP.

Diabetics having CIDP appears more common and the reasons are that Diabetes is a Neuropathy (but without the weakness) and it is possible for the nerve conduction values to creep into the range for CIDP.

The doctor states that: "In his opinion a reasonable number of diabetics get incorrectly diagnosed with CIDP!" 

As the weakness can be there for any other reason and the first 2 or 3 seconds of the tests prove how much weakness they have, so after the first couple of seconds if it becomes easier to push against their movement this doesn't count.

Diabetic patients are the toughest cases to sort out - there must be definite symptoms in all other areas.  As they will usually have numbness in their feet.  The doctor said he would expect significant weakness in the arms as one of the key differentiators.

One of the doctors attending stated he uses 5 points and if the patient has 3 out of 5 then it is CIDP:

1. Progressive course - it is getting worse slowly
2. Symmetrical proximal weakness
3. Loss of reflex - also EMG's
4. High CSF Proteins
5. Demylinating Features

With diabetics this must be much higher! As they can have a number of the above.

Below is the chart shown in diagnosing CIDP:

And this is my version:

The actual terminology used is terrible for the normal person, so I apologise for this.  However the doctor did state that getting hung up specifically on these criteria is not helpful to the potential patient.

I do have diabetes. So by this scale I don't have CIDP? As there was no issues with my arms ever - they were and are 100% OK!  However I did tick all the other boxes.  Unfortunately for me I did and do have CIDP, so the CSF, EMG and complete lack of reflexes in my legs proved this.

More later...

Update 29 - Information on CIDP (Part 1)

I have recently found a video specifically about CIDP, from an expert in this field:

Richard J. Barohn, M.D. - Chair, Dept. of Neurology, University of Kansas Medical Centre

Below is a synopsis of the first part of this hour long video, presented in November 2012, to a audience of medical experts.  It has taken me around 3 hours to de-crypt some of the terms and slides used (so far).

Apologies if I have made this too technical, but I have tried my best to make it understandable.

If you want to watch the full version it is available @ http://www.gbs-cidp.org

What is CIDP

Q: What pattern of Neuropathy specifically defines a sufferer from CIDP?

A: Symmetric proximal and distal weakness with sensory loss

Explanation: Muscle weakness away from the core of the body (i.e. in the arms &/or legs) with loss of feeling within those same limbs. The weakness & loss of feeling being the same down both sides of the body (left and right)

If you just have loss of feeling - this can be caused by other issues (e.g. Diabetes)

If it is not the same on both sides, these are other conditions linked to CIDP/GBS.

How to prove it is CIDP

Q: What is the best test for CIDP?

A: Nerve conduction studies (NCS)

Explanation: There are two types of nerves Motor (control movement) and sensory (control feeling). Both can be tested to see how fast they react to an electrical stimulation. This measures the speed it takes for the signals to get from the one place to another (latency measured in milliseconds) and is performed in a hospital or clinic.

Though a Lumbar Puncture can also be used (CerebroSpinal Fluid - CSF, or Spinal Tap), this measures the levels of proteins found in the spine and is another good indicator of CIDP if they are high,  the presentation states that this backs up the NCS findings

What NCS Values point to CIDP?

 Below is a table taken from one of the slides which just proves how complex and difficult medical people seem to want to make it for the ordinary person in the world to understand what they are saying!

Explanation (I hope!):

NCV - Nerve Conduction Value (metres per second)
DL - Distal Latency (signal travel time between 2 points)
F Waves - time for signal to travel back to central nervous system
LLN - Lower Limit of Normal (you've got to love them!)
ULN - Upper Limit of Normal

Location of Nerves:

So for each of the different nerves in the arm, the NCV needs to be less that 33.6 metres/second (<70%) for them to be fairly certain it is CIDP, with the norm being 48. In the legs this is 29.4 against 42.

For the Median nerve in the arm the time needs to be over 6.7 milliseconds (>150%) against the norm of 4.5 and the Ulnar is 5.4 to 3.6 (why these are different I don't know - I presume they have to be different lengths).  In the legs they are the same at 9.9 against 6.6 milliseconds.

The Time for the signal to travel back to the central nervous system is different for all @ Median: 46.5 to 31, Ulnar: 48 to 32, Peroneal: 84 to 56 and Tibial 87 to 58.

I am now going for a lie down to recover and will put more on this presentation up later!!

Update 26 - Survey Conclusions

My survey into GBS & CIDP will stop running in 1 week (on the 10th of March 2013). Please can anyone with GBS/CIDP who reads this and hasn't filled it in, do so. I have around 500 completed results and would like as many more as possible.  The link is:

http://gbs-cidp.questionpro.com

Conclusions

As I am not a medical person and the sole "experience" of GBS/CIDP is based on what has happened to me. What I conclude below is based purely on how people have responded to my survey and the feedback I have been given to the results:

1. The age range of people contracting GBS/CIDP is around 35 to 64 - which is lower than generally assumed (this could be countered by the fact that younger people use computers therefore are more likely to fill in something on-line)
2. The actual fitness levels of the people who get GBS/CIDP have nearly 90% as active or fairly active. Is this because they have more active immune systems? or because they travel more and expose themselves to more hostile/less sterile environments? or have more injections.....
3. It is definitely the extremities that are mostly affected, with the feet being slightly ahead of the hands.  However CIDP has a higher affected area all over the body than GBS (more areas affected by around 20%)
4. IVIG is the most proscribed treatment and seems to have a reasonable success rate, plasma is an alternative in some countries and steroids are more effective against CIDP than GBS, but all treatments have a fair degree of uncertainty about whether they will work at all and there is no miracle cure!
5. Men seem to complain less than women (that is a generalism about illness and going to doctors anyway).  However women seem to get on with things and cope with pain better!
6. A high number of people with GBS are still stating they have after effects/major issues.  The areas this has shown up in are getting around, nerve pain, how healthy are they now and ability to work.  So if GBS is acute and a one hit illness, people should be able to return to a reasonably normal life afterwards?  It is commonly stated that 80% of people make a full recovery from GBS and 20% from CIDP.  Whilst the results certainly back up the latter, there seems to be a lot less people making anywhere near a full recovery from GBS and as for being acute (it is certainly not "a cute" condition!), a significant number appear to be having long term residual effects. I would suggest that the boundaries and differentials put in place between GBS & CIDP need re-examining and that the leas than 4 weeks - GBS; over 8 weeks - CIDP is not as accurate as the medical people think and there is always the people in between......

The final conclusion is that there needs to be more research and understanding, however, I hope, at least more is known from a patients eye view now than before.

Presentation

The GBS Support Group in the UK have an annual meeting, this time near Liverpool on the 20th of April 2013.  I have been given a 20 minute slot to present some of the results from my survey (as I don't have time to do them all) and then answer questions.  The presentation will be based on the final survey number, around 500.  I will happily send the final version to anyone (after the 20th of April) who may want a copy, so please email or facebook me.

I hope the presentation will be illuminating & interesting and I will represent the findings in a fair and accurate manner.  I will also put in a number of the conclusions from above and try and get the opinions of the medical people at the meeting to my results.

Thank You

I would like to say a great big thank you to all the people who filled in my survey and have made it into the successful venture it has become. When I started out on this I had no idea how it would be received and what would come out. I wish I had phrased some of the questions better and maybe asked others, but I hope that publishing real information based on our experiences, this has assisted people in understanding how others are affected and provided some insights into the nature of GBS/CIDP. 

It was a shame that, apart from minor curiosity from certain medical people and individual support from one or two sections of the GBS/CIDP community, not one of the formal groups actually assisted me in any way or even sanctioned that what I was doing. Yet thanks for your efforts and support.

Now all I have to do is publish the results to the web for the 500!.....

June 9 - Update 11

Further developments on the clinical understanding of GBS & CIDP (and how to stop it occurring).....

Once again I have simplified the information gathered to try and make it easily understood, with apologies to any medical people if I have over simplified parts, but if I put the text in verbatim, then no-one would even read my blog!  I have taken my material from multiple sources (wikipedia, journals,web pages) - I can't reference all sources (as this would also make my blog unfit for purpose of passing the base information on).

I have recently found an article on potential methods of stopping auto-immune diseases and their symptoms.  In order to understand the research further medical terminology needs to be explained (and this is the cut down version):

T-Cells can be sub-divided into different categories, one being Treg - Which are regulatory cells that shut down the immune responses after they have destroyed invading cells (thus causing auto-immune diseases). Normally these cells exist with a ratio of less than 1 to 10 (<1:10) to other T-Cells, the proposed solution increases this ratio to over 1 to 1 (>1:1).  One component in the Treg (IL-5 or IL-5r"alpha") seems to promote activation of relevant antigens (IL-4, CD4, CD25, FOXP3 specific Treg) and these cells would control auto-immune inflammation, by stopping the cells going beyond their initial activities of destroying the invading cells.

Interleukin-5 (IL-5) promotes induction of antigen specific

CD4+CD25+T regulatory cells that suppress autoimmunity

Studies, so far, have been performed in a laboratory in New South Wales - Australia.  The figures below have been copied directly from their research papers (the explanations are in English!):

Note: All animals were given a strain similar to GBS.
        White = no other drug, Grey = Sham/placebo drug, Black = IL-5

Figures A, shows the reduced clinical effect on the rats given IL-5 (first graph) and the reduced effect of weight loss (second graph).  The drugs were administered at 5,000 units/day for 10 days from the onset of GBS (usually day 12) and had an effect within 2 days.

Figure B, shows the difference in the thickness/disruption of the myelin sheaths/demyelination between a control sample and those treated with IL-5 using an electromicrograph.  The differences at day 14 & 21 are significant!

Figure C, shows the reduction in percentage demyelination over 14 & 21 days seen in Figure B.

Figure D, shows the use of an IL-5 blocker (Day 13) on the Grey Triangles sample in the figure, that halted the effects of IL-5 and brought the effects back up to the level of who had not had IL-5 administered.

Further tests were performed to see if the improvements seen in rats can be translated to humans.  The initial trials on our blood samples seem positive.  Clinical trials are hoped to be started in the next couple of years.

This is a completely different way of stopping the disease, by increasing the number of "good cells" that would prohibit the attack rather than directly going against the cells causing the issue.

This treatment could also impact other diseases such as MS. To me the results look very promising.

May 26 - Update 10

STOP PRESS: I now have over 300 completed responses to my survey - Thank You to one and all.  I will be producing more results in July/August, they will be in a separate blog.

.......Following on from the previous

There are different cells in your body for different purposes for the immune system, two main ones seem to be relevant:

• B-Cells - they are the military intelligence, that finds issues/invading antigen
• T-Cells - these are the soldiers that actually destroy the target, also known as killer cells

Vaccinations - Do they have a part to play in this?

The simple answer is Yes and No! (Hang on I'm starting to sound like a doctor?)

OK - There have been a number of studies into viruses, the vaccinations and their affects for increasing the risk of certain diseases (including GBS).  What is a vaccine?

A vaccine is a biological preparation that improves immunity to a particular disease. A vaccine typically contains an agent that resembles a disease-causing microorganism, and is often made from weakened or killed forms of the microbe, its toxins or one of its surface proteins. The agent stimulates the body's immune system to recognize the agent as foreign, destroy it, and "remember" it, so that the immune system can more easily recognize and destroy any of these microorganisms that it later encounters. - Thanks Wikipedia

Thus it is possible to assume that as this is actually a virus/bacteria that is being deliberately introduced into your system and causes the cells above to recognise the danger and so re-act/remember, it could be responsible for causing GBS?

Well Yes and No! (see above)

In 1976 there was a vaccine developed for a swine flu like virus (in the US), that appeared to increase the risk of GBS and this vaccine was terminated.  They did prove that people who were given the vaccine did have an increased risk (roughly two to five times - depending on who's research you read).  Studies from 2000 onwards indicate there is no/minimal links between vaccinations and the rate of infection per se.  In fact the H1N1 incidence in 2009 actually happened part way through a study in Paris into GBS and causes and they found no increased risk due to taking the vaccination.  On top of this, there was a study of 50 million vaccinated people in 5 European countries in 2009 that found 104 cases of GBS, thus the researches could find no link.

So based on these fact the actual normal rate across these 5 European countries is 2.08 per million - significantly different to what was stated in my last post?

As it is the introduction of foreign bodies into your system, then (like in my case - as I had inoculations in January and 2 weeks later had flu like symptoms) there is bound to be some chance, but....... (BTW for my wife's 50th birthday we are going on a cruise to the eastern Med - as long as I am fit enough - one burning question is "Should I have the vaccinations????").  So I have a seriously vested interest in saying "YES!" they do play a part, but I can't find proof that satisfies me!

Anyway.....

Your immune system knows about internal and external cells, but in order to attack your internal ones, it must get very confused? Again there are theories and this does happen in a number of other diseases.

The one thing I do not understand is "Why me?".  What I mean is if there are 1 in 10,000 of me getting this condition from a virus/bacteria/vaccine then why was I the one?  There must be something different in me that triggered this that the other 9,999 don't have.  This is also what the doctors/professionals don't know, unfortunately.  There is research going on and I will try and comprehend where they have got to, but I am still digging.

May 12 - Update 9

GBSSG (UK) Notes - Part 2

This is starting to get complicated (for the ordinary person)!  I have been researching the findings from the meeting and am trying to understand/correlate all the information.  In my travels, on the web, I have come across some wonderful medical terms, one wins (so far):

monosialotetrahexosylganglioside

This is commonly known as GM1 (may as well be called "Bob" for all I cared!).  The relevance is that people with GBS have higher levels of anti-GM1 antibodies and this can interfere with motor neuron function, other antibodies can be relevant depending on the specific strain/type of GBS (e.g. GD3, GD1a, GQ1b - Miller-Fisher).

Please note I am using the term GBS to encompass all the different types (CIDP, AMAN...)

There are a number of interesting "facts" that have come out of my research (not just from the support meeting):

1) Now Polio has largely been eradicated; GBS is the most common neurological disease in the world (Do strokes count?)
2) About 1,500 cases of GBS occur in the UK each year
3) The worldwide rate is approx 1.3 per 100,000 people annually
4) The likelihood of women getting GBS decreases during pregnancy, but increases in the few months afterwards
5) Around 20% of patents have residual neural issues
6) Approximately 5-10% of people die, usually from respiratory complications
7) The amount of research into the "big 5" diseases is astronomical compared with GBS (no surprises there!)

How do you get GBS?

There appear to be a number of different ways of contracting GBS:

A) From bacteria in foods, the most likely source appears to be Campylobacter Jenjuni - which sort of gives you food poisoning.  Then 1 to 3 weeks later a small number of patients get GBS (between 1:1,000 to 1:10,000 roughly)

B) Flu like viruses (e.g. A/H1N1) or general influenza - again you get the virus and a few weeks later you can contract GBS (in similar ratio to above)

C) Epstien-Barr Virus (EBV - the Barr has no relation to Barre in GBS!) a member of the herpesvirus family signs are sore throats and swollen lymph glands (ditto for ratio's)

It is also possible to get GBS from HIV and other infections of this type.

According to the research I have found the numbers stack up roughly 40% from Campylobacter, 20% from Influenza like illnesses, 10% from EBV.  Again I am not a medical "expert" and all these findings are taken from journals and papers/web pages I have visited and taken information from.  You could state they are inacurate, but are my best guess on what I have found.

What seems to be similar is what happens to cause GBS, but first I had to understand the immune system, so here goes:

The body creates antibodies to attack the antigens (antibody generators) in an invading cell, these are like a lock and a key see below:

When an infection is found millions are produced (as opposed to the few "sentries" wondering around in normal times), these then attack the invading cells and neutralise it's effects.  After a time the immune system stops producing these and things return to normal.

So in order to combat the illnesses listed above, the immune system creates antibodies that match the patterns and they then attack the bacteria and wipe it out, now it appears that sometimes these bacteria can be very similar in pattern to antigens of the nervous system, so the immune systems keeps sending out antibodies to attack these as well, so the sheath and axon etc are damaged/destroyed.

Interesting fact: antibodies belong to the family of large molecules called immunoglobulins - hence the IVIG!

I am now going for a long lie down, to allow my brain to recover from this terminology overload, once again I apologise to any medical personnel who think I may have over-simplified things, but I am trying to get this down to a easily understood level and the medical journals etc use far too many long words for "normal" people. 

More later.....