and now?

[rui]

Hello!

My name is Rui and I’m from Portugal.
My father was diagnosed with AA Mar 2010. He is 70 years old.
Before that doctors thought it was a MDS and only stated that “there was nothing to do”. One doctor decided to go further and with myelogram and bone biopsy it came SAA diagnosis.
He was hospitalized on 13 May 2010 and underwent treatment with ATG (rabbit) + cyclosporine according to the protocol of the EBMT.
Was hospitalized for 59 days due to complications from lung infection.
Was discharged on July 15.
Since then still treated with cyclosporine 210 mg x2 day and made leukocytes inducers and erythropoietin (Filgrastim 480 u / day and Darboetina 150/semana).

Since then he has made weekly blood transfusions and one platelets transfusion as well.
Then put some values of hemogram before and after ATG treatment:

Data Hemoglobin Leukocytes Platelets
28-01-2010 5.6 4.6 19
30-01-2010 8.2 3.56 9
31-01-2010 8.7 3.26 10
03-02-2010 9.4 3.96 15
10-02-2010 8.7 4.3 12
24-02-2010 7.8 3.4 14
04-03-2010 8.2 3.17 6
12-03-2010 8.9 3.42 11
Hospital ATG treatment
03-08-2010 7.5 8.52 6
08-09-2010 6.7 11.68 4
13-09-2010 7.5 15.12 5
21-09-2010 7.7 13.23 3
29-09-2010 8.4 23.4 4
04-10-2010 6.7 3.09 3
08-10-2010 7.8 2.62 4
11-10-2010 8.8 30.66 4
13-10-2010 9.6 14.26 2
27-10-2010 7.5 8.27 4
There have been one episode of gingival bleeding quicly controled with a platelet transfusions.
In last consultation doctor told my dad that the treatment had failed and it was time for a 2nf ATG course.
I honestly do not know what to do and start thinking about having another medical opinion.

I appreciate all the support and information that I can be assured.

Sincerely

[Neil Cuadra]

Hi rui.

A second round of ATG is often successful, but when you're not sure of the most appropriate treatment, a second opinion is a very good idea, if your father agrees. If another doctor makes the same recommendation that will give you increased confidence to proceed.

Forum member paulaespada lives in Portugal and maintains a blog.

[Hopeful]

Hi Rui,

I would also recommend getting another opinion on the case. It is very hard to tell the difference between MDS and AA in the absence of chromosomal abnormalities in a hypocellular marrow. It would be good to have another pair of eyes reviewing the BMB slides or even possibly redoing the biopsy. AA is rarely seen in the elderly, and it would be prudent to confirm the diagnosis as well as to get another opinion on the next course of treatment.

[rui]

Thank all..
Yes I know, but my father is in defensive, is afraid of the future, the first ATG last 59 days and was very hard psicologial and physical. Analyzind the values what do you think? Did it work?, is too soon for a decision?.
I’m desperate.

[Hopeful]

Hi Rui,

Did your father see any bump in his numbers just after the ATG? Am I interpreting correctly that his platelets have been below 10k since the ATG but that he has had only one platelet transfusion? What has his ANC been?

I would get a second opinion before redoing ATG.

[Lisa V]

Hi Rui,

I too am curious about your father's platelets. How is it they haven't transfused him more than once if they remain so low? Our doctor would always order a transfusion when Ken's platelets fell below 10k, although I've heard that other doctors and patients use different target numbers.

Generally speaking, it is advised to wait 3 to 6 months after ATG before you decide that it's not working. Occasionally a patient will start to see a response after the six month mark, but if you haven't seen any signs of improvement up to that point, it seems less likely that you are going to see it after that without repeating the ATG. It is not absolute, but the majority of responders will respond by then. If he had his first treatment on May 13, then he is getting very close to the six month mark. Looking at the counts you have posted, I'm afraid I am not seeing any clear signs of improvement in that time. The clinical definition of response is to no longer need transfusions, but have you seen any increase in time between transfusions? If so, that could be a good sign.

I'm certainly not going to argue against getting a second opinion. It never hurts to get some input from another qualified specialist. If they agree with his current doctor, then that will help you and your father accept the decision. If they disagree, then hopefully they will give you some valid reasons why he should either wait or pursue a different course of treatment. Frankly, however, I'm not sure what that other course would be. At his age, I doubt they would consider a transplant, so (assuming his diagnosis is correct) it seems like the main options are to do another ATG or to do nothing.

A second round of ATG can be more successful than the first. Although my husband responded both times, his second round produced a stronger response than the first. He was lucky to get in and out of the hospital fairly quickly, however. I can understand with all that your father has been through that he would not want to repeat that experience, but unless his counts start to improve soon, another round may be his best hope.

[wapost3]

Hi Rui and everybody else,

I certailny don't know everything about this disease since I'm just out of the hospital a couple months after being diagnosed, so take my response accordingly. I have not seen in these forums that I have read much if any mention about plasmapheresis (http://www.mdausa.org/publications/fa-plasmaph.html). I lost count of the number of these treatments that I had received to bump my platelets up. Yes I had ATG Rabbit, prednisone and cyclosporine, but it was the plasmapheresis that got my platelets count up in the end. Has any of you also received these treatments?

[Lisa V]

That's a very interesting link, Wilbur. I've heard of plasmapheresis, but never heard of it used as a primary treatment for AA. Maybe you can start a new thread in the "alternative treatments" section where more people can find it.

[wapost3]

Lisa,
Keep in mind that when I went to the hospital with platelets below the 10K mark closer to 5K that I was first diagnosed with TTP(Thrombotic Thrombocytopenic Purpura http://emedicine.medscape.com/article/206598-overview)
. Thats when they started the Plasmapheresis treatments, I continued on these treatments for approximately two months, sometimes twice a day. I go to the Dr tomorrow to see where the blood counts are at. My platelets got up as high as 337K but more recently were around 247K. I can only hope that tomorrow's results will be similiar. RBC's have been slowly going down so that is what I am currently most concerned about.

[Lisa V]

Wilbur, I hate to hijack Rui's thread, but I have several questions I'd like to ask about your treatment. Perhaps if you start another thread, you can address them there.

I'm curious about the timeline of the plasmapheresis and the ATG/cyclo, i.e. which came first and why, and what changed your diagnosis. If the PP came first, and your Plts got that high, I'm wondering what made them decide to do ATG, unless your red and white counts were dangerously low. If that is the case, and if removing the antibodies from your blood was that effective in calming the autoimmune attack that was causing your Plts to be low, why would it not also be just as effective on the other two lines? On the other hand, if the ATG came first, then what made them decide to do PP, and how do you know it is the PP and not the ATG that has caused the rise?