NIH Trip and Information

Whew, Well thank you all for the continuing prayers. It has been a long two days. We have spent most of our time at the NIH clinic. Seeing our own Dr. Uzel and Dr. Jain, the transplant team doctors and spent alot of time getting physicals, talking, seeking answers and trying to learn what we can about NEMO. For me its a complete realization that this is going to be a lifelong battle for Simon with all the things you have to be careful with between the NEMO immune deficiency and the ED, which has alot of affects taht are just not normal. I guess I was hoping to come and hear some miracle had occurred but that has not been the case. Wishful thinking. Since Dr. Jain is the one who discovered the gene mutation he is a good source of information. We saw dermatology as well today who was not very helpful since Simon was not having an outbreak and they basically said we were doing everything right in the skin area. The biggest question was "do we retransplant and if so how aggressive do we need to get". We got a preliminary answer to that question which was they think with the t-cells being at the 30% mark we should try donor lymphocyte infustion first (DLI). This would mean Marriana has to be harvested again. There are two ways to do it and one would entail getting both lymphocytes and stem cells and possibly freezing the stem cells in case this does not work and we have to do a full blown transplant. They think we should go this route as if it works, its always "if" we would avoid more chemo for simon and avoid a full blown transplant, if it does not work, we would have to do the whole transplant over. The problem is that Simons crimerism is somewhere around 15% donor cells. 10% means the whole graft has failed. Since his counts of donor cells has increasingly been dropping the thought is that yes the transplant failed. Is it worth it to give just donor lymphocytes or do we just say we want the whole thing redone and hope that it takes. Yes there is a possiblity that we could totally wipe simons cells out and then the donor cells not take in his body. They told us its almost better to have a mixed crimerism of both cells. We don't know what we are going to do at this point. We have to meet with the drs. in Chicago and discuss all the data that we have learned here. It felt good to come and talk to other doctors and hear them say that things in Chgo. were done completely how they would have done them here. We go back tomorrow for a rap up meeting and protocol information and then we have decided to just get in the car and head home. Its been really hard to be in this dinky hotel room with 3 kids trying to get them to sleep. Simon is the worst as he stands in the pack and play and just laughs and the girls get going and whala craziness occurs. Dave and I settle them down and laugh. We were so out of it from waiting so long and trying to entertain the kids today that we were laughing and couldn't stop and as I walked out of the exam room and the dr was standing there looking at me I said "I'm sorry I have to go to the bathroom" and she smiled and directed me, I looked at her and said "my family is making me laugh", this is after they walked in on all of us as simon was thowing up all over me. Craziness will make you crazy. We did manage to have a nice dinner al fresco last night in downtown Bethesda, which is very nice, expensive but nice and we ate pizza out tonight. We are all anxious to get home. We will have to stop again tomorrow night somewhere and we all miss our home, our beds our routine. Maybe we'll stop off in sandusky and ride a rollercoaster for the day. ha ha. Keep us in your prayers. WE love you all and God Bless. Love Tracy and David