6 months post atg

[N dean]

Hi all
My son was diagnosed with vsaa in dec 2020, he then went on to have H-ATG, currently we have seen an increase in neutrophils (0.02 before atg) and currently sitting between 0.6 and 0.9 but we have seen no reduction of time between blood and platelet transfusion which still sot at platelets every week and blood every fortnight. I was wondering if anyone has had this kind of response and if it was likely that he would eventually get a full response or if we should be heading to transplant which is currently in discussion with the hospital
Thanks for any replys in advance

[Matthew42]

Dear N Dean,

My mother has SAA and it's been 5.5 months since her horse-ATG. She recently had a big rise in neutrophils for about two weeks (averaging about 1200, as opposed to about 500 for the past 5 months. A big rise in neutrophils does mean something. What else would be kicking them up besides the ATG? Only prednisone or some steroid can cause a false rise in neutrophils.

My mother has also had a rise in RBC and hemoglobin, but then will drop sharply. She's never had rises in hemoglobin after a transfusion - only a steady decline. She normally gets a blood transfusion every 9-11 days, but recently it's been 2 times a week. You say that she's getting transfused more often...but she's getting these "spikes" in hemoglobin between transfusions. That has never happened before. We're still not sure, but something seems to be changing in her marrow. Platelets, however, have not improved in the least (she still gets 2 platelet transfusions a week).

At any rate, horse-ATG can take 9 months or more to really kick in (several people on here where it took up to a year or more!). If there had been no change at all in your son's blood counts at 6 months, then I might say that horse-ATG seems to be not working, but that is not the case just yet, as he has had a rise in neutrophils over time since ATG.

I am not sure if vsaa vs saa makes a real difference in response time. It's just that vsaa means your average neutrophils are under 200, whereas in saa, the neutrophils are higher.

I wish your son the best of luck and health. May he heal from this disease. My mother has been suffering greatly. She's in the same boat as your son. It's been 5.5 months out from horse-ATG. She thinks that she will never get better and cries every day. I think she's given up, but I tell her that the horse-ATG can still work. Her neutrophils are now averaging over 1000 and she has sharp rises in her RBC count and hemoglobin (but they still fall after a few days).

Keep believing, keep strong. I give your son much love. This is hard. He can do it. Just believe....

[N dean]

Thank you matthew for your response, our consultants said that they wanted to see a significant increase in neutrophils and that they wanted him transfuse free by 4 months due to his age and impact on hos life as he is unable to go to school ect, we have had a bone marrow biospy today and speaking with consultant again tomorrow, depending on the results of this they will determine if ATG has failed or not as they seem to think that we are not going to improve much more so our next step is transplant which we have a 9/10 donor so we will just have to see tomorrow. I can understand your mums despair its awful this isnt an easy thing to live with and watching in hopes that u see an increase in everything and not getting the results you want is devasting but as you say there is still time. Im on the understanding that with vsaa they want to see a more defined reaponse in counts and transfusions sooner due the the damage that it may have already caused to the marrow. I also think that with children they do not tend to like to leave the time as long as 9 months. Our consultant has daid that here its 4 months for children before they look at other options and then obviously you still have a chance of a response whilst everything is being put i to place. I hope your mum shows sign of improvement soon

[Matthew42]

Originally Posted by N dean

Thank you matthew for your response, our consultants said that they wanted to see a significant increase in neutrophils and that they wanted him transfuse free by 4 months due to his age and impact on hos life as he is unable to go to school ect, we have had a bone marrow biospy today and speaking with consultant again tomorrow, depending on the results of this they will determine if ATG has failed or not as they seem to think that we are not going to improve much more so our next step is transplant which we have a 9/10 donor so we will just have to see tomorrow. I can understand your mums despair its awful this isnt an easy thing to live with and watching in hopes that u see an increase in everything and not getting the results you want is devasting but as you say there is still time. Im on the understanding that with vsaa they want to see a more defined reaponse in counts and transfusions sooner due the the damage that it may have already caused to the marrow. I also think that with children they do not tend to like to leave the time as long as 9 months. Our consultant has daid that here its 4 months for children before they look at other options and then obviously you still have a chance of a response whilst everything is being put i to place. I hope your mum shows sign of improvement soon

Dear Neal,

I appreciate your explaining your son's situation better. You're correct: with children, they don't like to wait it out. The next option is a bone marrow transplant. I've heard of young adults waiting it out, but usually children are encouraged to get a BMT if horse-ATG does not work at 6 months.

Also, vsaa might be looked at a little bit differently than saa in terms of waiting-and-watching timeframe because excessively low neutrophils can lead to infections.

I surely hope your son fully recovers and can go back to school so he can live a normal life that he deserves.

Even though I am a stranger, I give my love and support to your son and to your family. You will get through this somehow. There is light at the end of the tunnel.

Keep us posted, please!

[N dean]

Hi so we have been to see our consultant today, with the results of his bone marrow biospy (his trephine results wnt be back till next week) but the marrow shows that atg has failed and is still empty, we are going to transplant hopefully on the 5th of oct, our donor has confirmed and had tests done (consultant had put all this in place jusy incase) only negative is donor is currently testing positive for covid so nothing more can be done until he is testing negative on a pcr test. Hopefully he will be testing negative by the 5th and we can proceed. In the mean time we are looking at chemo options, and the possibility of fertility treatment and radiation (these are dependent on the trephine results). Thanks for all you support it helps when people can relate to the current situation or have been through tor have been the same long road either themselves or a close family memeber

[Matthew42]

Originally Posted by N dean

Hi so we have been to see our consultant today, with the results of his bone marrow biospy (his trephine results wnt be back till next week) but the marrow shows that atg has failed and is still empty, we are going to transplant hopefully on the 5th of oct, our donor has confirmed and had tests done (consultant had put all this in place jusy incase) only negative is donor is currently testing positive for covid so nothing more can be done until he is testing negative on a pcr test. Hopefully he will be testing negative by the 5th and we can proceed. In the mean time we are looking at chemo options, and the possibility of fertility treatment and radiation (these are dependent on the trephine results). Thanks for all you support it helps when people can relate to the current situation or have been through tor have been the same long road either themselves or a close family memeber

Thank you for letting me know. Please keep us updated.

I believe your son will do beautifully with a BMT. He's young, which is in his favor. Very young people are encouraged to get BMT's for aplastic anemia. He had no improvement in cellularity, either, which does indicate that the horse-ATG isn't probably working. But you will never know for 100%.

My mother actually gained 4-5% in bone marrow cellularity after 4 months post-ATG, but blood/platelet levels had still not been up (just neutrophils were a little better). But her hematologist said that that was enough improvement to believe the horse-ATG could end up working down the road. She also has a small PNH clone in her marrow, which is supposed to indicate a favorable response to ATG. But nothing is ever a guarantee, is it? My mother can't get a break for nothing, it seems.

We are still not out of the woods, despite a few spikes in her hemoglobin lately. We are keeping our fingers crossed. You just never know with this disease.

If my mother's horse-ATG doesn't kick in by mid-November, she will have to do the rabbit-ATG (stronger). My mother can still get a BMT if all fails if she stays healthy enough. But we just don't know what will happen.


Keep in touch.

Wishing you all the best!

[Hopeful]

Hi N Dean,
I am sorry to read of your son's struggles with this disease. Be sure that they have tested your son for all of the genetic variations (Fanconi, SDS, etc), as the conditioning regimen for transplant can be different.

The transplant success rate is high for children, and it is considered a cure.
I hope the road ahead is smoother for him!

[N dean]

Hi hopeful

My son had all other possibilities and genetic tests done and ruled out before any treatment or donor search was originally done in dec 2020.

We are currently in hospital 5 days post transplant with a 9/10 donor as there was no change with atg from what i previously wrote.

So far things are running relatively smoothly and we are back to the waiting game to see if the transplant is working and when we can get discharged altho this is looking to be at the minimum 20 after transplant providing that he has neuts of 0.5 and no pther issues.

Thanks
Natalie

[Matthew42]

Originally Posted by N dean

Hi hopeful

My son had all other possibilities and genetic tests done and ruled out before any treatment or donor search was originally done in dec 2020.

We are currently in hospital 5 days post transplant with a 9/10 donor as there was no change with atg from what i previously wrote.

So far things are running relatively smoothly and we are back to the waiting game to see if the transplant is working and when we can get discharged altho this is looking to be at the minimum 20 after transplant providing that he has neuts of 0.5 and no pther issues.

Thanks
Natalie

I am hoping that your dear son will be forever cured.

Neutrophils of +500 are is not bad; only when the get under 500 (more so, 200 or less), do doctors get very concerned. My mother's hematologist says that neutrophils of +1000 are all that is needed really for decent protection. She says that the range 500-1000 is okay, though, particularly if the person takes some precautions (masks, washing hands, staying away from crowds, etc.)


I am wishing your family love, health and happiness.

[Hopeful]

Hi N dean,

I will keep your little guy in my thoughts and prayers. Please keep us informed of his progress. We are all rooting for him!

[N dean]

Thank you all.. so far we are day 13 post transplant amd no neutrophils as yet (full white cell count is below 0.1 so we are in full isolation in an air filtered room) engraphtment of cells is at the earliest day 10 but can be some time after. Again still a waiting game he is doing well considering happy in himself and moving on to all oral meds again which is great. Just need those cells theough now.

[Matthew42]

Originally Posted by N dean

Thank you all.. so far we are day 13 post transplant amd no neutrophils as yet (full white cell count is below 0.1 so we are in full isolation in an air filtered room) engraphtment of cells is at the earliest day 10 but can be some time after. Again still a waiting game he is doing well considering happy in himself and moving on to all oral meds again which is great. Just need those cells theough now.

Thank you so much for the update.

When should he start to see neutrophils?

I am wishing your son all the best. He can do it! I know it in my heart.

Much love to your family!