As an update, since my last post (ASXL1 Mutation Detected), I recently came across some very useful articles concerning the CALR mutations & ASXL1 and their ability to be included as Prognostically significant, and naturally wanted to share those findings hopefully to the benefit of others here:
1. "The prognostic advantage of calreticulin mutations in myelofibrosis might be confined to type 1 or type 1-like CALR variants_BLOOD_2014"
This article implies that CALR Types 1 & 2 can render significantly different Overall Survival (OS) outcomes for a patient.
Along the line of the ASXL1 mutation, Vannucchi et al (2013), offers the following:
2. "Mutations and prognosis in primary myelofibrosis"
This is of great interest to me as I am CALR+ Type 2, and in combination w/ ASXL1 means that also according to a new scoring system for MF, (MIPSS70 & MIPSS 70-plus), that my risk stratification has most likely risen too... Some of you might have already seen that article:
"Prognostication in myelofibrosis_ Integrating genetics into routine practice_Hobbs_2018"
Recently, I saw a new specialist, (second opinion), concerning my MPN condition and he knew very little concerning the findings of these articles above, and I found that revelation a tad disturbing. However, in Australia, we do suffer from having a smaller population, and therefore not as much volume takes place in the MPN fields of endeavour... Sad to say of course...
Philosophically speaking of course, one really must learn to be as extremely patient a patient as one can be...
However, yesterday, I underwent my Tissue Type collection at Westmead Hospital, in Sydney.
Unfortunately, for me, I required four (4) different collection persons because of the stubbornness of my circulatory system in providing blood samples. It actually took Seven (7) attempts to acquire x 12 tubes of blood for a variety of tests etc... Hence, my arms are a tad black & bruised today, but they eventually succeeded, and that's really the main game here anyways...
I am getting used to the pain of Blood Tests these days...
Still have not mentioned any of this to my family members as yet because I am still not sure that I want to put my younger siblings through all of this...
There are only two (2) of them; my little sister in the States, & my little brother up in the Blue Mountains. However, there is a great deal to consider really:
* First, there is the obvious interruption to their routines, where the Donor is required to come in to provide their Blood sample to test that they are a 10/10 match, & that is a 1 in 4 chance (25%)
* Secondly, (& if they prove to be an acceptable match), they must first undergo a series of injections over four (4) days, and by the 3rd day Donors will usually experience some unpleasant aches & pains, & nausea... (Those injections are required to help express their Stem Cells (SC) out of their Bone Marrow & into their blood stream for collection)
* Finally, and if donating the SC via the blood plasma method, they will then need to be placed in an Apheresis machine to have the collection of the SC removed and their remaining blood products returned to their system. That process takes circa Four (4) hours usually.
The Donor will possible require some assistance throughout this process, and some reassurance, and a lift home etc...
There is one other option for the Donors also, where they donate the actual Bone Marrow (BM) by a day trip to hospital, and extraction of SC while under a General Anaesthetic.
None of those procedures sounds to me like a pleasant experience...
Then, after all of that:
1. They may prove to have a latent viral infection which could rule their donation redundant
2. They are a match, and the Stem Cell Transplant (SCT) goes ahead but is rejected by the recipient's body, which could leave the donor with pangs of guilt etc...
Of course, on the other hand, it could all go well, and I one might become fully recovered... (?)
However, if I ask my specialist to see if there is a match in the Australian Bone Marrow Registry (ABMR) or an Overseas Registry, I would be forced to accept whatever else is or was available at that time (frozen or not), from a local or overseas donor source... (frozen donations can produce other unwanted side-effects)
So, there is a little to contemplate...
Nevertheless, the process has commenced w/ my own Tissue Tying (TT) completed, and hopefully I won't have any latent viral infections that could complicate the procedure any further than it already is...
Results of the TT generally take up to Four (4) weeks, and then it's all about finding a 10/10 match. So I have some thinking to do...
Happy to hear your thoughts of course...
Best wishes all
Steven
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It’s late in England and I should be asleep, but I wanted to thank you for all the useful links. Very much appreciated.
As for whether to tell yr family and ask yr brother in the Blue Mts to see if he could be a donor: He might feel very sad if you didn't give him the chance to offer to help you. Initially its just a blood test after all.
I take your point Rachel... However, if he is a 10/10 match... There is still no certainty that the SCT will be a success. I am concerned that he might them somehow blame himself. He is my little brother and now my only brother and he is a extremely sensitive kind. Actually, he is an artist and can be very easily wounded... Don't ask me why... He just is... (?)
Whereas, if I just try the ABMR first... I might be successful in that way, or just as successful. However, he would not be able to apportion to himself any part of blame etc...
The odds are not spectacularly in the favour of the recipient, in most cases...
My donor did not experience any problems during or after. My two sisters were devastated that they could not provide stem cells for me. 3/4 injections and sitting around for 3/4 hours to save a siblings life? That is surely a no brainer!
Assuming you have a close family, as not all do, you should share with them and see what they think.
Don’t know about frozen cells but assume Genny, Kathy and Marina who got their cells in USA, Sharon in Oz and Kathy in NZ who all got their cells from Germany are doing fine The databases are worldwide as many of us are mongrels so the cells need to travel
I agree with the others, I think your siblings would be mortified if their stem cells might improve your odds and you didn’t ask them. It is very considerate and unselfish of you to be so concerned about them at such a personally traumatic time.
You say ‘odds are not spectacularly in favour of the recipient’. I assumed odds of success were around 60 - 80% for a median age of 67? Hopefully this figure is continuously improving as new techniques impact results. I suppose a significant factor is whether SCT performed at early or late Stage 2 (Intermediate), especially if higher risk mutations?
Is there an improved GVHD success rate between donating marrow or blood stem cells?
Q. Is there an improved GVHD success rate between donating marrow or blood stem cells?
Personally, I am yet to locate anything definitive. The greatest concern, (other than not surviving the process at all), is developing GVHD in either the acute or chronic forms.
I did find this article below that might interest you, and it does have a few radical treatment options that might hold some future promise. Please do let me know what you think after reading it etc...
Thanks for the interesting link. I posted a video Presentation which implied the risk of GVHD was less than I (and everyone else in the room) were expecting. Will repost link shortly in case you haven’t seen it.
I also posted re a new drug for GVHD - Orencia/Abatacept, which looks encouraging.
The limited initial trial produced (results lat Dec) yielded exciting results for treatment of GVHD. Surprised there has been little follow up in the Press but maybe the scientific community waiting on the second group results, due imminently.
immuno-oncologynews.com/201...
“Results from the second group of 140 patients with matched unrelated donor transplants are expected in the next six months” - this was dated mid Dec 2017 so one to watch out for.
Hello Steven, I am also a CALR type 2 patient, but with a grade fibrosis 1, so still considered as an ET patient. I am using Pegasys and doing very wel for the last year.
Most people have no idea if they are a type 1 ( deletion ) or type 2 ( insertion ) in CALR.
I have no idea if you are already a member of our ET/MF CALR mutation support group on Facebook ? If not, feel free to join us, where you can share also this very informative post.
Lovely to be hearing from you, and thank you for your post.
Tomorrow, May 6th, is my 2nd year anniversary since diagnosis. I pushed to learn of other mutations from the outset as I believed I was still trying to tell myself, (at that time), that I was merely suffering from a case of Reactive Thrombosis (RT).
However, the CALR (Type2) detection, and my BMB put pave to all of those theories...
Wim, I don't do FB, however I am more than happy for you to past any or all of what I have posted for your group... Please feel free my friend.
I see that others have sent good and thoughtful advice regarding this, and I just wanted to add my kind thoughts and best wishes to you. It is a very difficult thing that you are going through and I admire the fact that you care enough for your family to worry about involving them! I hope you do ask them though :-).
Whilst contemplating a transplant I found my siblings more than willing to help. Unfortunately my older brother and sister were considered too old and my other sister was not a match. When you put your thought processes on paper it is clear to see your dilemma. People surprise us all the time though and I believe your siblings should at least be given the option to help. My sister who wasn’t a match was devastated as we are very close. However, in the end I was advised against the procedure anyway. That was a relief in some ways because I could stop my mind playing the same games as yours but now as things stand I do not have a chance for a cure. You do so be a bit more selfish and if you think the procedure is right for YOU then go for it! Your siblings don’t want to lose you and the people who donate marrow are doing it to save lives like yours. Good luck on your future path. Very best wishes, Jan
My brother did actually volunteer, whereas I strongly felt that my younger sister was not so willing. They are also both in the early 50s, hence, time might be of the essence too... I believe the ages are from 18-60 in Oz.
However, knowing that, (even if they did volunteer I mean), does not really allay my reservations.
Philosophically, this is a process, of life, and life... in my view is not an eternal gift. Fascinating all the same, however, I am not in any hurry to leave it...
Nevertheless, I am starting to feel that if I can do this without having to ask my siblings that I will feel better about making my own decision to undergo this process etc.
However, I still have a little more time before I have to make a decision.
Thank you all for your well wishes and generous advice. I am most grateful of course...
Either way Steven you are caught between a rock and a hard place. I understand where you are coming from. As a person you don’t want to put your family out. You are independent. However , if I was your brother and eventually learnt of your predicament I would be horrified you didn’t keep me informed. I do think your family need to be told.
Reading your post has now made me realise that if things deteriorate for me Et Calr, I now know I have to tell my family .
This is not so much about my independence, and more about creating an unpleasant experience, and potentially apportioning any blame to themselves, should things not work out ideally as is most desired...
Naturally, I care about my younger siblings as I am the oldest.
Try for a moment to place yourself firmly in my shoes. If it were possible to achieve this most desirable of outcome w/out having to place them, (my younger siblings), in anyway responsible for a failed attempt. Would you not first want to take that chance...?
Especially in my younger brother's case. He has always tried to prove to me that he is worthy of my venerations... Hates, being referred to as my "Little brother". If it all wen t well, he would be on top of the world, however, the converse would also apply I am afraid...
I have had the occasion to have to rescue him from a precarious situations two in our histories... So I am sure that he would volunteer w/out really giving t much thought at all...
Alternatively, and in the case of my little sister, she would not have to fight her conscious to say that she does not want to do this... She is a beautiful creature and one that chooses to live by her rather unique 'butterfly' standards... and I love them both just the way they are...
Anyways... I am still contemplating for the present.
Hi my friend Steve, you certainly do have a dilemma with regards to your brother and sister and only you can really know and decide to ask them, you know them best and it really comes through that you are the protector, the big brother who is always there for them, and it doesn't matter how old you are, your big brother or sister is someone you feel safe with and protected by, and they always seem invincible. I am coming at this from the angle of being a baby/little sister to 2 brothers and 1 sister, and even though I am now at the grand age of 58, I still look to them for support and advice, I just don't like being told off for using bad language by 1 brother, but having said that, I wouldn't hesitate to give them anything they needed. But, having said all of that, I say again, you know your brother and sister, so you and only you know whether or not they could cope with being a donor. It is a tricky situation for you my friend, I just wish I could help more. Maz x x x
Really lovely to hear from you. I hope all is well in your world. You are an amazing person to everyone here on MPN Voice, and you know how genuine I am when I say that, as I also am aware of what it takes to always be there responding to people out there in our brave new world of IT.
It can be a most thankless task at times I know...
However, Maz, I am sure that everyone here knows just how hard you work to be there to support us all... and I am most grateful, thank you Maz.
You might be the youngest in your clan, however, your wisdom reveals an old soul who gracefully dispenses empathy in ways that are most uncommon...
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Is anyone else bothered with leg and foot cramps? I have PV Jak 2 negative.
