Day +112: Living in Limbo.

We got the results of Idan’s latest chimerism today, and they tell an even more puzzling story.  As you may recall, with Idan’s latest rash, we were hoping for an increase in donor T-cells to explain the sudden graft versus host issue.  The good news is that the donor T-cells have gone up – but only slightly.  They are now at 18%, up from 12%.  The bad news is that Idan’s donor myeloid cells have taken another big hit, and those are now at 52%, down from about 80% a few weeks ago.  If the myeloid cells do drop further, we are still faced with possible rejection down the road.  No one can say for sure how Idan’s graft will grow or change over time, but for now we are hoping that the myeloid stabilizes soon, or it may jeopardize the new T-cells that are starting to come in.  We take some comfort in the fact that, even if the graft stabilizes at this chimerism of 18% T-cells, it may go a long way towards patching the gaping hole in Idan’s immune system.

In other news, Idan’s rash is showing signs of improvement, and we will hopefully be able to start to taper the steroids by this time next week.  Steroids, as you might imagine, have sent him bouncing off the walls with a ton of energy and mood swings.  It will take a few months to taper the dose, so we can expect more of the same for some time now.  Because of the rash, we are unable to do another DLI (which is the only way to intervene to increase the T-cells) as it would most certainly cause more severe graft versus host disease – the kind that we would not even trade Hyper IgM for.  Given the fact that the T-cells have inched up over the past couple months, though, we are content to wait and see what happens next.

Next week, we will be doing a test run of life without a central line.  If there’s one piece of transplant that has been the bane of our existence, it has been the central line.  The central line is a thick tube that sticks out of Idan’s chest and is a direct link to a vein above his heart.  It is held in place by stitches and a dressing that we have to change once a week.  For these reasons, the line poses a high risk of infection post-transplant, and with steroids in the mix, it is even more concerning.  From the beginning, the line has caused us grief, causing little Idan to bleed for several days post-surgery and endure multiple dressing changes over the course of 48 hours during his very first few days in the hospital back in October.  In addition, on a daily basis, the line must be protected and flushed every morning.  At night, we cover it completely during bath time and have gone to such extremes to keep the line and dressing dry that we have stopped baths altogether and only do sponge baths.  It also happens to be an attractive nuisance for a curious little toddler excited to grab and yank on anything in arm’s reach.  The time has come to take it out.  So, instead of drawing from the line next week, we’ll test out a needle poke and see if Idan tolerates that well and if enough blood can be drawn.  If so, we will be taking out the line as soon as they can book an OR.

Once the line is out and the rash is under control, we can start thinking about heading home to New York.  Although we still are in a constant state of anxiety over Idan’s graft, we take joy in the fact that in the near future, Idan will be able to splash in a bathtub filled with water, play with toys other than syringes, and – most importantly – be reunited with his many family members back home.