2nd Matched Sibling BMT for SAA

[Lisa S.]

When my son was 9 years old-bloody noses, petechia, weakness and then June 2013 admission for red blood cells, platelets, followed by eventual diagnosis a month later of SAA. Sept 2013 his 6 year old brother donated Bone Marrow to him, and it worked well, great response, but 3 months after the cyclosporine stopped it came back -18 months after BMT #1. So we tried again, BMT #2 in March 2015. And it was rough, 59 days in Boston Tufts Hospital, and to shorten this post, he is home on cyclosporine-he is now 10 months from BMT #2, and the cyclo is rough on his kidneys, so he takes 500mls of Normal Saline through his broviac at night. He used to take 1000mls of NS, but the doc decided to try Sirolimus and taper the Cyclosporine. But now after 4 weeks of slowly going down counts, I saw the Lymphocytes rising and his ANC went under 1000. This game changer has him back on a higher dose of Cyclosporine and tomorrow we are headed to clinic to get him a GCSF shot to stimulate his Marrow to release some neutrophils. Doctor wants him to stay on Cyclo and the Sirolimus dose for 3 months and then taper one of them off. I don't know about this sirolimus, it was great for his kidneys, but what good is that if it can't keep his lymphocyctes from attacking?? Feeling helpless....Any Advice!!

[Marlene]

What a difficult situation Lisa. It's so rare to hear about relapses after BMTs. The only thing I can think of is to get a fresh opinion from a different BMT center. I know many really like Dr. Margolis. He has lot of pediatric experience with SAA and BMTs.

[Lisa S.]

I did talk to David Margolis via Phone and email prior to my sons's second transplant. My doctor called him and they consulted and my son's pre-transplant conditioning changed the second time-to Campath, Cytoxan, fludarabine, and Methotrexate. He did rATG, Cytoxan, and Methotrexate the 1st BMT. I am tempted to contact him again in regards to the sirolimus as I can't find a lot, of information on it via the internet.

[Marlene]

Was the Cytoxan post BMT? I know they've been doing high dose cytoxan post BMT to combat GVHD. Is it possible he's dealing with GVHD or virus causing the spike in lymphs?

[Lisa S.]

My son was given Cytoxan with both of his BMT's for pre-transplant conditioning. I do not know what is causing his lymphs to go up. I do know that I just got off the phone with the Doc and he is sending me Neupogen/GCSF shot for once a day for three days. I am an RN so I am happy I can give him the shots from home instead of traveling to the clinic that is 2 and 1/2 hours away. We travel to Boston on Sunday for a visit on Monday, that hopefully will show a jump in his ANC.

[Marlene]

Hope the GCSF helps and you get some answers next week.

Wishing you the best....Marlene

[Lisa S.]

Back from Boston. My sons WBC and ANC count went up as hoped for with Neupogen. Yes, it is temporary as WBC last only 2-3 days but I think he will regulate back to his low normal. His platelets fell to 77, but his Hemoglobin stayed the same as last weeks at 9.5. Doctor seemed to think that my son was not on a therapeutic level of sirolimus long enough to give it a chance. The plan is to stay on cyclosporine and sirolimus for the next three months, then he wants to taper one of them off. He has not said which one. I spoke up and said I want a slower taper, not so fast, and he said he is doing what all the other hospitals do, the protocol. I mentioned that each patient is different and with my sons history of loosing the first transplant I feel it warrants a slower taper. For now I am glad the counts are holding, except the platelets fell, but we will see what next weeks labs will bring. Give me a stable Chimerism and a stable count, then I will give the Doctor the go ahead to taper his way, but for now, I am a worried, and very tired Mom....who will advocate for her son's best interest.

[Marlene]

Hopefully he will consider modifying the "protocol" in your son's case. You've have a compelling case and I'm sure he'll give it some consideration.

I'm glad things are stable and hope they improve over the next three months.

Stay strong.....

[Lisa S.]

Labs today WBC 3.3, hemoglobin 9.0, Hematocrit 26.4, ANC 1.5, Platelets 155, the up in cyclosporine has helped a lot. He is still on 1 liter of Normal Saline at night and that is keeping his BUN, Creat, and Potassium almost normal. It has been 10 months since transplant #2. No results on the most recent Chimerism's yet. But I am happy with his labs for now. He did cough up a couple blood clots last night, but nothing since.

[Lisa S.]

Happy to report the Chimerism's showed an increase from 60% in the CD3 line to 77%! That is a jump of 17% in just a months time. The Doc had mentioned that the Chimerism probably would only show a small change, well that is a Big change-and for the better! The other 3 lines, are 97% or higher. Whole blood is 86% donor over all. Now to keep the change either stable, or maybe even go up some to more donor.

[Marlene]

Great to hear. I'm sure it will continue in the right direction.