New to site

[white.karen5]

Great to find some information on AA,i was beginning to think 1 was on my own as have not met anyone else with same diagnosis.Here is my short story so far.
Diagnosed with AA in June with BMB,AGT rabbit treatment in July,had line infection and serum sickness in Freemans Hospital Newcastle 2 weeks.Was on cyclosporin for 2 weeks but got body tremors for 5 days in Whitehaven Hospital,have now stopped cyclosporin all together.Tapering off Prenisolone. Consultant says i should wait a couple of months to see if AGT works.My CBC for this week was PLT 13 HB 9.2 WHITE 3.5 NEUT 1.2.Have had about 13 transfusions so far,mostly platelets every week, back to hospital Monday and will probably get more.

BMT is an option for me and my brother has sent off his bloods for a match also the BMT team are looking for unrelated match.I guess this is in case the AGT hasn't worked.

My consultant stated i may have Hypoplastic Myelodisplasia.My bonemarrow is 10% cellurality and without blasts.
Any feedback would be great,Are no blasts normal?

Cheers Paul

[Neil Cuadra]

Paul,

This must all seem to be happening very fast since you were only diagnosed a couple of months ago but it sounds like your case has been handled by the book so far. Even serum sickness and tremors are, unfortunately, common side effects of the drugs you've been given. Giving the ATG time to show its effects makes sense. Note that the initials are A T G, which stands for anti-thymocyte globulin.

It shouldn't take long to check if your brother is a match or search the registries for an unrelated match, so you'll know if you have that option.

Bone marrow in aplastic anemia patients is hypoplastic, meaning there's a lack of cells. As a result, that introduces the possibility that it's really a form of MDS that is also hypoplastic. In MDS the cells are damaged, not just missing, but when you have very few cells the doctors can't get easily get a sample, so they don't know if your cells are damaged or not. It doesn't sound like there's evidence that you have MDS, just no way to rule it out for now. Doctors will probably want to do further bone marrow biopsies to keep an eye on your marrow.

Normal bone marrow has a small percentage of blasts (immature white blood cells), usually about 5%. Like other bone marrow cells, yours are apparently scarce.

[evansmom]

Hi Paul,

Find out from your doctors what protocol they are following as first line treatment for your aplastic anemia. Usually doctors will choose horse ATG over rabbit ATG for the first go but perhaps your doctor is following another protocol. Also, the use of cyclosporin or another immunosuppressing medication in it's place should definitely be part of the treatment. ATG is a strong immunosuppressant that takes your immune system right down in an effort to stop the immune system's attack on the stem cells (assuming the AA is an immune-driven disease) and that effect is then maintained by cyclosporin.

If the ATG's work is not maintained by the cyclosporin, then I'm not sure how your doctors are going to guage your response to therapy. If cyclosprin gave you tremors, perhaps the doctors should have lowered the dose as perhaps your blood level of this medication was too high.

Furthermore, there are many who are on cyclosporin who have had to learn to live for a while with tremors as the benefit of the cyclosporin FAR outweighs the inconvenience of the tremors.

Aplastic anemia is a very serious disease that requires a clear, concise and definitive plan of care to get you well again. There are protocols for treating doctors (ideally hematologists/oncologists) to follow and experts for them to refer to if they are unsure about something.

I fear your treatment plan may have some holes in it and it will have to be up to you to advocate for the best care for your disease.

Inform yourself and read, read, read. Using this forum the way you are is also very wise.

Best wishes,

[white.karen5]

Hi Neil

Thanks for that.Looks like i've got a bit of a journey and plenty to learn! Getting a lot of info from this forum.

Do you know what deleted chromosome 13q means?

Thanks Paul

Hello Evansmom

Hope you and your family are well.Thanks for the feedback,its just what i am looking for.

I believe horse ATG has been stopped in England at present.I to thought it strange to stop Cyclosporin altogether(0n 50mg twice a day.don't now if this is high dosage)
consultant said there were no alternatives.I wondered if it could be serum sickness from ATG?Which i finished 5 weeks ago.I was getting used to the tremors,it seems to be the only exercise i am getting!!!
Back to hospital Monday so i will be asking my Consultant a few more questions

Thanks for the tips.Paul

[Neil Cuadra]

Originally Posted by white.karen5

Do you know what deleted chromosome 13q means?

From what I've read it's an abnormality that occurs in about 2% of MDS patients. Was it reported in your bone marrow biopsy results?

I believe horse ATG has been stopped in England at present.

For years horse ATG has been the standard in the U.S. while rabbit ATG was the standard in Europe. Even though a major study has now found that horse ATG should be preferred for initial treatment of severe AA, acceptance in Europe has been a slow process.

[BerryP]

Hi i'm in the UK and had pretty much the same treatment except that when I got the shakes they said tough. Deleted 13q as I understand it means that part or all of your chromosome 13 has bits missing from it, hence the deleted. I have something wrong with my chromosome 15. From what the docs told me this is not something you were born with but something the disease has caused in your body. Rabbit ATG is the only thing approved for use in Europe at present and in my case it didn't work. Hope things run smoothly for you and nice to have the option of BMT. I am 58 and the risks are too high so managing on tx but with me platelets sit around 40, whites 0.5 and hb 8 so they give me packed red cells and shortly an iron pump. Its a hard disease to explain to anybody who hasn't suffered it cos the effects seem so random, changing from day to day. I wish you luck with the treatments and stay in touch, this website is very good, its helped me a huge amount.

[white.karen5]

Originally Posted by Neil Cuadra

From what I've read it's an abnormality that occurs in about 2% of MDS patients. Was it reported in your bone marrow biopsy results?


For years horse ATG has been the standard in the U.S. while rabbit ATG was the standard in Europe. Even though a major study has now found that horse ATG should be preferred for initial treatment of severe AA, acceptance in Europe has been a slow process.

Hi Neil

Yes,The abnormality was found in my bone marrow biopsy taken 2 months ago

[white.karen5]

Originally Posted by BerryP

Hi i'm in the UK and had pretty much the same treatment except that when I got the shakes they said tough. Deleted 13q as I understand it means that part or all of your chromosome 13 has bits missing from it, hence the deleted. I have something wrong with my chromosome 15. From what the docs told me this is not something you were born with but something the disease has caused in your body. Rabbit ATG is the only thing approved for use in Europe at present and in my case it didn't work. Hope things run smoothly for you and nice to have the option of BMT. I am 58 and the risks are too high so managing on tx but with me platelets sit around 40, whites 0.5 and hb 8 so they give me packed red cells and shortly an iron pump. Its a hard disease to explain to anybody who hasn't suffered it cos the effects seem so random, changing from day to day. I wish you luck with the treatments and stay in touch, this website is very good, its helped me a huge amount.

Thanks for that and hope you are well.I have just been for blood count,(neut 1.5 hb 9.1 whites 3.1 and platelets 8) consultant is happy that they seemed to have levelled out,but to go back Thursday as he is not wanting to give me platelets as often if i have no bleeding and is not keen to put me back on cyclosporin.

Good hear from somebody close to home the kids were at Chester zoo only last week.

Cheers Paul

[Neil Cuadra]

Paul,

There's some discussion of 13q deletion in the Chromosome 13 abnormality thread. Statistics show that some particular abnormalities are relatively better or worse than others for prognosis or can influence treatment choices, but I don't think 13q deletion is one that's significant in either of those ways. However, it's significant that you have any chromosome damage. I assume that's why you were told it could be a myelodyspastic syndrome (MDS).