...update!

As some of you would remember August 2007 was the date my peripheral neuropathy symptoms escalated into the next level - general unwellness, fever, extreme fatigue, muscle fatigue, joint pains, fasciculations etc. Prior to that, starting in June/July 2007, my symptoms had been mostly intermittent burning/electrical currents in the lower legs, sore eyes and some fatigue.

In April this year I developed an extremly bad cough. After four months of coughing and no real answers apart from isolating a Mycoplasma Pn. infection, I finally got so tired of coughing I went to the emergency department of my local hospital about three weeks ago. There I was admitted for six days and had oxygen, frequent nebulisers and antibiotics.

While there I had a HRCT (High Resonance CT scan) and was diagnosed with Bronchiectasis of both lungs. This is irreversible damage to both lungs which will need lifetime treatment vigilance and physiotherapy.

Last Friday I went to a top Respiratory Specialist (head of a Lung Transplant unit) and he said we need to find out what has caused the Bronchiectasis. He has ordered a Bronchoscopy for two weeks time.

Incidentally I also told him of my general ill health and neuropathic symptoms over the past year and although he hopes it is not Amyloidosis , he will be checking for that as a possible cause to both my lung and neuropathic symptoms. He will also be checking for other isolated lung causes.

After he mentioned Amyloidosis, I noticed that Amyloidosis is included in the Algorithm article as a cause for PN. I certainly hope it is not that diagnosis as it is invariably fatal. Interestingly though my most recent SPEP has come back with a feint protein band which wasn't there on the last SPEP seven months before. This is one of the tests for Amyloidosis but can mean other things too.

I hope not.

I had a patient with amyloidosis, who was ultimately diagnosed at Mayo. He had exhausted the local doctors. (there are several good medical teaching facilities here, so I don't know why it was overlooked.)

Kmeb may have some support for you. She has been thru that diagnostic quagmire.

I hope it is not amyloidosis. So do keep us informed.

Sorry to hear you are feeling so poorly....

Yes, I've had a positive amyloid biopsy (fat pad aspiration) - was told "nothing could be done" and sent home with nothing more then "oh well"... I contacted one of the amyloid center of excellences in this country (this is criticial) - I contacted Boston University and spoke to the head of the program - who sent me with her instructions to my local cancer center where they repeated the biopsy, did their own biopsy and also sent tissue to Boston, and got a negative... they still suspect it - but have to find it and it can be very elusive and be there for years before they find it - Ive been told any time any procedure is done to have the docs biopsy for it - and they do.... (this disesase can be anywhere your body) - since you have organs that are directly affected - they should be able to get a very accurate biopsy..... and I hope it is negative.... but in any case - be sure that the tests are confirmed (your tests results can be easily sent to abother institutuion for reading) - just let the docs know ahead of time you want unstained tissue taken also... dont be discouraged - this disease does respond to some treatments - there are groups on the net if you just google that are very very helpful, and a strong support network.....

Again, stay strong - and I hope the biopsy is negative... if it isnt though - rememeber - many many in the medical community, because they see it so infrequenty, dont know much about this disease and you need to step up to the plate at that point and take charge...

Sending good thoughts....

This has nothing to do with neuropathy, however, it has to do with the lung and liver. Check out what your protein electrophoresis is...I just happened to get my notes, and in there it said I was low on Alpha one antirypsin. Looking that up, I found that it is a very common gene in northern europeans ( found in many ethnicities however) and a cause of early emphysema or cirrhosis of the liver. Of course, there are cases where it relates to neuropathy, mostly vasculitic. Just a point of interest. It is amazing what you find in your records if you request and read them....you don't get told everything that is in them.

Now, I can take a simple test to see if I carry the gene....which I likely do, and if my husband carries the gene, our kids may be at higher risk for some diseases.

If you have unexplained lung problems, Alpha one antitrypsin deficiency is often over looked and underdiagnosed. You may want to ask what your status is.

Every one with neuropathy probably goes thru the amyloidosis panic, and I hope for your sake your testing is negative. I have been thru the fat pad aspiration which was painless, and mine was negative. Right now the theory is my neuropathy is autoimmune...(theory changes now and then-I am learning to roll with the punches).

Good luck to you on your search.

Thanks Cyclelops.

I have had two SPEP's (Serum Plasma Electrophoresis) done. The first one done ten months ago, apart from having low serum gamma globulin seemed to be 'normal'. There were no protein bands and the Serum Alpha -GL was 3.2g/L (range 1.7 -3.9) and the Serum Alpha 2 GL was 6.9 g/L (4.3 - 9.4). Are these what you are meaning?

I had another SPEP done in July this year at a different lab and there was a protein band emerging in the Gamma region. Other measurements were done and initially classified as 'normal' however my pulmonologist expressed some concern that the IgG and IgA's had not responded at all well considering the intensity of infections I have recently had in my lungs. He felt they should be significantly higher in the range at least, rather than sitting at the bottom of the ranges.

He wants to do an IgE and other tests when I see him next before my Bronchoscopy.

This area of haematological testing has always eluded me a bit so it's another learning curve as to the significance of each of these things.

When you say: "Every one with neuropathy probably goes thru the amyloidosis panic"- funny thing is that even though it is listed as one of the causes of neuropathy in the Algorithm article I didn't even consider Amyloidosis or even look it up! I'm sure I don't have it even though four days out of every seven I feel pretty bad!

Hi KMEB,

Thanks for your feedback and kind thoughts!

Not a nice place for you to be, in diagnostic no-mans-land regarding Amyloidosis. Wouldn't you be deteriorating fast if you had A? Perhaps that is an assumption on my part that you aren't deteriorating -forgive me, if so!

I suppose my reasoning for myself is that I have been unwell for 14 months now and have not worsened hugely apart from the pulmonary symptoms, and I'm not in the sex demographic for Amyl (66% males - still I suppose that leaves 33% females. Shivers!!)

The Pulmonologist told me that he would be on the lookout for amyloid plaques and doing a biopsy would be one of the things he would perform. I'm sure it will be negative though!

A Serum Protein Electrophoresis (SPEP) is not the current gold standard test of choice for determining if there are rogue monoclonal antibodies (M-proteins) in the blood (which can be associated with amylidosis and other blood disorders.

The more specific Immunofixation electrophoresis of serum and urine should be ordered--this is normally done with an Ig subclass enumeration as well, to see if there are more antibodies than there should be. They wil look for specific bands that may indicate the rogue antibodies and the protein subclasses characteristic of the condition.

An SPEP is not sufficient, as small elevations in the Ig subclasses can be obscured in the overall gamm globulin testing.

See:

http://neuromuscular.wustl.edu/antibody/mprotein.htm

http://neuromuscular.wustl.edu/nother/amyloid.htm

http://www.questdiagnostics.com/hcp/...eralNeurop.htm

Remeber, there could be many many reasons for the PN......

I've been sick for 9 years now - but last year very rapid decline.... my cardiac system is also affected and I suffer daily multiple cardiac events and have to take meds constantly to control... my balance, loss of sensastion etc has gotten much worse, and due to gastroparesis (which is from the nerve damage) has much worsensed much to my chargrin for a girl who loves to eat - ensure and broth does not a big fat cheese burger make! The docs concensous now putting amyloid aside (they want to do another fat pad biopsy but I told them to hang on - because of the severity of the PN at this point I really wouldnt be candidate for any amyloid treatments anyway at this point other then what I'm already getting for guts and heart) is that the PN is genetic - the form they believe it is there is no current genetic test (Boston Mass is working on on) - but a "clinical" diagnosis based on several neuros who have seen me talking together, carfeullly going over nerve biopsies etc., and even though we tried high dose IVIG for over a year it did nothing - they were hoping I would respond even though I had no auto-immune markers and I felt better (I thought) a few days after each infusion) but in retrospect I think it was just relief from getting thru the weekly infusions - which I had many side affects with every infusion and really dreaded them (for those who IVIG works for - its wonderful - dont get me wrong!)...... Now they say there is nothing they can do and we're just working on trying to gain to some weight - and the heart meds so far are keeping symtoms under control.....

Again, the best detector for amy is finding the culprit thru biopsy - also, the light chain test is very helpful - but if the basic markers arent there and there is no severely affected organ or symtoms that really raise some eyebrows - they need to look at the whole PN testing spectrum as others have said...

Hang in there -

Hi Glenn,

Thankyou for your feedback. I will research this area some more and thank you for those links. In this whole research area, I find that information that one reads and discards as irrelevant (for self) at one point in time, can become more meaningful and significant when revisited as one is steered down another alley. I have been down so many alleys these past 14months.

The first SPEP I had done in August 2007 was done in the initial general round of testing for possible causes of PN and the next one was recommended by my endocrinologist in July this year as I had such a bad cough. I don't know that any of them were particularly thinking Amyloidosis at that time - maybe they were!

It was only a week ago that Amyloidosis was mentioned by my Pulmonologist based on the existance of both PN and recent severe lung deterioration. The Pulmonologist said he is going to be doing more blood tests on the 7th October before the Bronchoscopy, so perhaps he will be doing the more specific Amylodosis tests as you suggest. I know he said he would do a biospy if he sees anything resembling Amyl.

Hi KMEB,

I am very sorry to hear of your health decline - I think we all dread this, especially as we have so many plans for the future. A future we have to admit we may not have!

I am very aware that there are many causes of PN and consequently I have already undergone a good number of tests to find a cause. As most of the tests at the time were negative, it was for this very reason that I had all but given up finding a cause as no-one seemed to want to pursue anything further........then the very distressing cough started in April 2008.

Although I have still felt all the PN symptoms over these past months and also become progressively stiff in my muscles, I have found most of my energies have gone into surviving the cough on a daily basis; therefore discovering a cause for my PN had been put on hold.

What I have wondered these past few months; is the lung condition/cough and the PN caused by two different things or are they caused by the same causative agent? This is when I did a lot of research on Mycoplasma Pn. which I have been proved to have and I looked at literature to elicit it's effects on the peripheral nervous system. This I still don't know for sure.

However at least since my hospitalisation some things have been clarified in regard to my lung problems and whether they turn out to be completely separate from PN remains to be seen. Will keep you informed.