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Research / Re: New video from the US, for sufferers of Wild Type TTR
« Last Post by Miriam Vered on January 11, 2019, 02:04:45 PM »
Professor Hawkins' reply:

It’s under evaluation by NICE, and we remain hopeful that there will be a positive outcome sometime in next few months.
Research / Re: New video from the US, for sufferers of Wild Type TTR
« Last Post by Bryangrist on January 10, 2019, 04:53:20 PM »
Thanks for the video. I am a sufferer from wild type. Any news about availability of patesarin for treatment in gb yet
General Discussions / Re: Close - but no cigar!!
« Last Post by RogerS on January 09, 2019, 02:57:19 PM »
Miriam,  I would like to add my thanks as well.  Your reply helps me to gain a little more understanding of this complex disease.
General Discussions / Re: Close - but no cigar!!
« Last Post by Chirpster on January 08, 2019, 01:17:33 PM »
Hi Miriam, Thank you for taking the trouble to provide such a detailed analysis and my thanks to Sir Mark for his time. This is by far the the most comprehensive explanation I have seen and puts everything into perspective. I very much appreciate it.
General Discussions / Re: Close - but no cigar!!
« Last Post by Miriam Vered on January 08, 2019, 10:40:11 AM »

After reading over the discussion above I discussed it with Sir Mark.  He wanted to emphasise the following important points –

AL amyloidosis is caused by an abnormal protein that is unique in each individual patient and different from the abnormal protein causing AL amyloidosis in all other patients.  No two individual’s AL proteins are ever identical.

That means that each individual patient has a unique disease, even if some, or even many, aspects of the disease and its response to treatment may be similar or overlapping in some patients.  This sets AL amyloidosis apart from many other diseases, even other types of amyloidosis, which are caused by essentially the same underlying disease process in all patients.

AL amyloidosis starts with the abnormal proliferation of one particular unique cell in each patient.  That cell belongs to the class of so-called B lymphocytes that have the unique property of producing antibodies, which are proteins belonging to a class called immunoglobulins.  Each immunoglobulin molecule contains two components, larger ones called heavy chains and smaller ones called light chains.  These two types of chains are normally produced in appropriate proportions to assemble into intact normal antibody molecules.  Only very small amounts of free light chains are released into the body fluids.

The function of B cells is to recognise germs, such as bacteria, viruses and parasites, as well as many other substances that are or could be harmful to the body.  The B cells respond by producing antibodies that specifically recognise and bind to the substance that triggered their production.  The defence of the body requires that there are B cells able to recognise almost any possible substance that could do harm.  There are therefore enormous numbers of different B cells each with its own particular specific antibody product, different from all others.  Under normal conditions, a B cell that meets and recognises its particular target substance responds by dividing into two daughter cells and they divide in turn, and the process continues, to yield a large number of cells that are all identical to the original cell and to each other.  Such cells are called a clone and they all produce the same identical antibody that is needed.

In AL amyloidosis, something goes wrong with the genes in a single B cell, for reasons that we do not understand.  The cell then divides and proliferates to produce an abnormal clone with an abnormal product.  Instead of an intact antibody, the clone produces an excess of abnormal free light chains.  Sometimes the abnormal light chain protein is harmless but sometimes its abnormal structure causes the light chains to stick together in the form of the fibres we call amyloid fibrils.  These accumulate in between the cells of the body’s tissues and they disrupt the structure and then the function of the organs, leading to disease: systemic AL amyloidosis.  We only partly understand why some light chains form amyloid while others do not.

Amyloid fibrils are removed only very slowly from the tissues, once again for reasons that are not understood.  Nevertheless, if we can eliminate, or at least greatly reduce, the abnormal B cell clone that is making the abnormal, amyloid forming light chains, then we arrest progression of the disease.  Over time the existing amyloid deposits are then gradually cleared and organ function can recover.

In AL amyloidosis itself, the clone of cells that causes the disease do no harm themselves.  They are not very abundant and they do not proliferate very actively.  It is just their abnormal light chain product that causes the problem.  Indeed, such clonal proliferations are not rare in the elderly and they usually have no clinical significance provided the light chains do not form amyloid.  In the related condition of myeloma, however, the B cell clone is usually the major cause of illness because the clone proliferates actively and damages the bones.  At the same time, the abnormal immunoglobulin protein, which is produced in large amounts in myeloma, can cause a variety of different problems.  If amyloid forming free light chains are produced then AL amyloidosis can be a complication of myeloma and it occurs in about 15% of all myeloma patients.  The main treatments for AL amyloidosis alone and for AL amyloidosis complicating myeloma are the same and are directed at killing off the abnormal clone of B cells that is producing the damaging proteins.

In each and every AL amyloidosis patient, the abnormally proliferating cells and the abnormal light chains they produce, are different.  This is part of the reason why AL amyloidosis is sometimes challenging to diagnose, to study and to treat.  Care must always be taken in drawing conclusions based on observations and experiences in other patients.  It is also important for patients to appreciate this.  Although you can gain and give invaluable support in sharing experiences between patients, always bear in mind that your own disease, test results and response to treatment are unique to you personally.

Wishing everyone on the forum well in 2019

General Discussions / Re: Happy New Year to alll
« Last Post by patpinchin on January 04, 2019, 07:35:39 PM »
Thank-you everyone for your very warm thoughts & kind words.

I’m pleased to say I’m now slowly recovering at home. I was very fortunate to be cared for & supported throughout by Patrick & our family. I certainly couldn’t have got through the ordeal without them.
Keeping everything crossed,  it doesn’t happen to other members. It turns out my illness was caused by low sodium so my Dr & Nurses have helped me gradually get back to health tho’ I think it might take a while yet.
Will let you know how things go.

I hope 2019 will be a good year for all of us including our NAC team. I for one would be lost without them all. 😊😊

Sent from my iPad
General Discussions / Re: Happy New Year to alll
« Last Post by Richardpreston on January 03, 2019, 03:49:07 PM »
Happy New Year Pat  and everyone.  Keep posting, your resilience is astonishing.
General Discussions / Re: back on chemo
« Last Post by Chirpster on January 02, 2019, 07:48:58 PM »
Hi Anna, so sorry to hear about your symptoms. Some of my own experiences may have some relevance to your situation so I’ll just run through them.
It was over four years ago that I first developed a cough after eating and with it produced a very sticky, clear phlegm. Initially this was only after my main meal in the evening but over time progressed to every meal and now it’s just about every time I eat or drink anything. To be fair the coughing usually stops after the phlegm is ejected but I do have some inexplicable coughing bouts from time to time. Around the same time I developed sinus problems and then a tendency to develop cuts and sores on my upper and lower palate, inner cheeks and tongue. Also I had a tendency to develop large blood blisters in the same areas and bruising on my tongue. These were attributed to allergies and then to chronic fatigue syndrome. Of course I now know that they are the symptoms of systemic amyloidosis in my soft tissue.
As a result, my tongue has enlarged and the the inside of my mouth has become ”softer” and more susceptible to damage. Though it hasn’t deteriorated over time and I have adopted the following to control the problem:-
(1) Avoid sharp food like crisps, toast and especially crackers.
(2) Keep a thorough dental regime.
(3) Inspect your mouth everyday and more often when on treatment.
(4) Cuts and bruises in the mouth start to clear up quickly usually within 36 hours - ensure that they do otherwise seek medical attention.

Very basic sensible advice I’m sure we’d all agree. But I’m not always sensible:- Just before Christmas I fell to the temptation of a blt whole grain sandwich with chips at my local pub. I didn’t feel much discomfort while eating it but afterwards I checked my mouth to discover that my tongue was badly bruised, there were cuts on the roof of my mouth and the inside of my cheeks as if I had been eating razor blades and I had three blood blisters the size of 5 pence pieces scattered around.
I don’t regret it though, my mouth cleared up swiftly and it was great to actually taste something after the chemo and I start the chemo again this week so no such treats for a while!
Best wishes for 2019
General Discussions / Re: Happy New Year to alll
« Last Post by Mark McConway on January 01, 2019, 11:32:06 AM »
Hi Pat,

Thanks for thinking about the rest of us when you're not doing so well, yourself, at the moment.

In addition to wishing you the best for 2019, I just wanted to add something...

I don't think I'll get an argument from anyone here  if I say that your dedication to this Amyloidosis Patient Forum - and the promotion of awareness of Amyloidosis generally -  is second to none.  You have written countless words of encouragement to almost every patient\carer who has left a message on the forum, provided informative links and information for the rest of us to advance our research.  It's appreciated!

On behalf of everyone who has had occasion to benefit from anything you've contributed to this community, I wish you a speedy recovery and a permanent relief from your symptoms sometime soon.

Best regards


General Discussions / Re: Happy New Year to alll
« Last Post by AnnR on December 31, 2018, 05:11:59 PM »
Sorry to hear you are not well and hope you are well enough to go home soon.
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