(Yes, I went there.) We are sleepless in Seattle over Idan’s latest chimerism test results. Once again, the numbers are not where we hoped they’d be, and, worse yet, are indicating that the graft is slipping even further. Idan’s T-cell count remained low, nudging up only a few percentage points from 7% to 12% donor from the DLI. This would have been fine if not for the fact that his myeloid cell line, which had been at or near 100% from the beginning, has slipped down to 83% donor.
There are two possibilities for the slip down. The first and more likely is that Idan is rejecting the graft (probably because he does not have a substantial number of donor T-cells). The second possibility is that the myeloid cells are just fluctuating and will stabilize at a high number. We are redoing the chimerism test next week to see if it continues to drop. If so, we know that we are likely facing rejection. Obviously, we are hoping and praying for the graft to hold tight and for the T-cells to continue increasing. We are planning for a second DLI on January 27th, but that plan may change depending on the test results next week.
Without speculating too far into the future, should Idan reject the graft, we will have to gear up for battle again. There is some new research indicating that Idan’s disease makes him susceptible to an even greater array of opportunistic infections than initially thought, and that the CD40 ligand protein is implicated in more areas of the immune system than just the creation of IgGs (hence why IgG replacement therapy is not sufficient to protect Idan).
A second transplant comes with even more risk than the first one and is a very scary thought, so we are praying that we do not have to go down that road. That said, we think back to our initial reasons for choosing Seattle and feel comforted knowing that Idan is still strong and did very well throughout the transplant. There are definitely scenarios far worse than rejection. No matter what the next chimerism results show, we have no regrets.
We are blessed with a child that fills our heart with joy and laughter, and allows us to forget our fears and worries. We hope that we can do the same for him by staying strong, happy and optimistic – but prepared to go back into battle if necessary.
I am praying right along with you all! Love and blessings, Becky
Thinking of you three<3
What can one do but send love and healing thoughts to you all… X
Thinking of Idan and your family. Hoping for the best. As parents, you are so strong and courageous. Idan is brave and joyful. Love to all your family.
Thinking about you and really appreciate your sharing of this journey publicly. My eighth graders ask about Idan every week.
From our family to yours—we are wishing you all well, praying and thinking of you all out there in Seattle! You deserve a break.
Thinking of Idan every day and praying every night for all of you. Sending warm hugs, prayers and love from Ok.
we are all there for you. love, uncle avi
While at first this news seemed so disappointing, on further thought there is much to be positive about. Every case is unique, and Idan may still very well engraft, perhaps it is just taking a slower, more circuitous route. Hopefully things will remain stable enough for the second DLI and that will be the catalyst for engraftment. But even if it turns out that a second transplant is necessary, well, it is a wonderful thing that such an option even exists! While as you indicate, there is more risk the second time, there were also many risks with the first one and Idan did just fine. He exceeded doctors’ expectations, being such an “uninteresting” patient! The best news is that Idan is still a strong and robust little boy. He will find his cure, maybe not as quickly or directly as we all wanted, but he will eventually be rid of Hyper IgM for good. You are all in our prayers. Lots of love and hugs.