On Thursday, after looking at a picture of Ezra's rash, the doctor decided that it is indeed what we have feared - Graft Versus Host Disease. The rash had not spread, but the rash on his back was worse. She also decided that it was best to be aggressive with treating it and started Ezra on "systemic" (oral) steroids, rather than waiting and trying different topical creams to see if they would lessen the rash. Steroids are a major problem post-transplant because they suppress the number and replication of T cells, delaying immune reconstitution. This is the last thing Ezra needs at this stage when we are anxiously awaiting the appearance of T cells. Steroids are a game changer. They also can cause very serious side effects. But the doctor explained that they have found that if you wait too long to treat GVHD, it becomes much harder to remedy, and it is best to attack it early.
Since starting the transplant process, GVHD has been our greatest fear, short of Ezra dying. This latest set back is very difficult for us to come to terms with. We were told that a T cell depleted transplant (the type of transplant Ezra had) has a 5-10% risk of GVHD. For his first transplant, we were told there was about a 10% chance of engraftment failure. Why our one-in-a-million son ends up on the wrong side of the statistics so often is a question to which we will never know the answer. And yet, we know we are lucky that he has so far landed on the right side of the most important statistic - he is alive and he is fighting.
The good news is that the rash responded immediately to the steroids - it was much improved after just the first dose. The plan is to stay on the higher dose of steroids for a few more days at least and then start a slow wean. The concern is that the GVHD will flare up once the steroids are weaned and that will require an indefinite course of steroids, and result in continuing impact on his immune system and other organs. We will all be watching closely once he is allowed to start the wean.
The confusing part of this GVHD flare up is that as of a few weeks ago, Ezra had no T cells. T cells are what causes GVHD. The new T cells that form from the stem cells from the donor and "grow up" in Ezra are not supposed to cause GVHD. It is typically T cells that come in with the graft at the time of transplant that cause GVHD. With the T cell depleted transplant that Ezra had, only a very small number of T cells come in with the stem cells. Ezra received even less than the level of T cells that Sloan considers "safe" - so small an amount that T cells have been undetectable since the day of transplant.
Since starting the transplant process, GVHD has been our greatest fear, short of Ezra dying. This latest set back is very difficult for us to come to terms with. We were told that a T cell depleted transplant (the type of transplant Ezra had) has a 5-10% risk of GVHD. For his first transplant, we were told there was about a 10% chance of engraftment failure. Why our one-in-a-million son ends up on the wrong side of the statistics so often is a question to which we will never know the answer. And yet, we know we are lucky that he has so far landed on the right side of the most important statistic - he is alive and he is fighting.
The good news is that the rash responded immediately to the steroids - it was much improved after just the first dose. The plan is to stay on the higher dose of steroids for a few more days at least and then start a slow wean. The concern is that the GVHD will flare up once the steroids are weaned and that will require an indefinite course of steroids, and result in continuing impact on his immune system and other organs. We will all be watching closely once he is allowed to start the wean.
The confusing part of this GVHD flare up is that as of a few weeks ago, Ezra had no T cells. T cells are what causes GVHD. The new T cells that form from the stem cells from the donor and "grow up" in Ezra are not supposed to cause GVHD. It is typically T cells that come in with the graft at the time of transplant that cause GVHD. With the T cell depleted transplant that Ezra had, only a very small number of T cells come in with the stem cells. Ezra received even less than the level of T cells that Sloan considers "safe" - so small an amount that T cells have been undetectable since the day of transplant.
So, how does Ezra have GVHD? Well, first of all, labs were drawn on Friday to check again for T cells. If there are T cells, there is no way of knowing if they are the very small number of donor T cells that have multiplied for some reason or new T cells that aren't behaving as expected and are attacking Ezra's skin. If the test comes back showing no T cells, then the assumption is that there are T cells in his skin and organs, but not in his bloodstream, which is why they have been undetectable. His doctor has described GVHD as a "black box" in many respects, and that has been our impression of so much of the transplant process. It is, and always has been, an enormous leap of faith.
Just as we had finally gotten the real Ezra back, we now have yet another version of Ezra - steroid Ezra. Steroids can cause stomach upset and Ezra's weakened stomach is already causing him more pain. It is so frustrating to go backwards after he had made great strides.
Please send prayers and positive thoughts that the T cells chill out and leave Ezra's skin alone ASAP (Ezra Aviel ben Hertzelia Shandel). And please wish us all courage as we confront one of our greatest fears.
