It seems like the nature of treatment that we are never able to quite get clear of things before we have a setback. We progress, things improve, and then something happens and we are back where we were a few months ago in terms of medications and precautions. It IS improvement in a big picture sense, but it’s bittersweet and stressful day to day.
Zoe is back on track now, and our worst fears have been put to rest. She does not have a recurrence of HLH, her graft came back at >98% again, the same as before and a clear sign that her transplant has taken and HLH is gone (forever we hope). The fear of a return of HLH was due to a rash that appeared very similar to an HLH rash, but the chimerism result reassures us that is not the explanation.
She does however have GvHD, which has required her to restart Tacrolimus and go on a short term run of Prednisolone, the old nasty steroid, again. Why she has GvHD was a big point of confusion to us given her chimerism results, so I will do my best to explain briefly.
Zoe has a new immune system, and that immune system is completely donor material. This is the result we want, since her old immune system was dysfunctional and susceptible to HLH. Her new immune system however has not yet come to terms with it’s new home, meaning her old body is still considered a threat to it to some degree. When left unsupressed, her new immune system attempts to fight her old body, causing the rashes and other GvHD symptoms.
We had gotten her off of almost all of her meds slowly over months of weaning, when the rashes started to appear. It seems that she is not quite ready to be off of them, so we have restarted the routine and we’ll see where she is in another 3 months or so.
There is a question of whether or not she will be ready to enter pre-school this fall, but I’ve been told by reliable sources — Zoe’s two teacher-grandparents — that she is too young to need to be in school anyway. Yes it was what we had planned and yes it would be helpful, particularly socially, but it’s not going to hold her back to wait another 6 months or year to be safe.
In the meantime, she is back to looking good and feeling good most days, which is a relief. We are going to finally be able to remove her port despite the complications of these past weeks, so that too is progress. At this point I’m wishing that we had started putting little patches on Zoe’s well-worn diaper bag for each medical scare, not unlike notches in a gunslinger’s holster or emblems on a jet fighter. She’d have them racked up by now.
3 thoughts on “Two Steps Forward, One Step Back”
Want to send our condolences for the loss of your father. We send continued prayers for Zoe!! and your family. Thanks for keeping us udated and pray for continued healing forward with no more steps back!!
As one of the reliable sources I can say that Zoe is really very, very bright. She may pass everyone regardless of when she starts school! And goodness are we all blessed. Things could have been much worse.
We JUST embarking on this exact same journey for our two year old daughter. It is surreal to read about it here. Thank you so much for sharing this. It does worlds of good for terrified parents out here that are facing the same fight.
We are 4 weeks into the HLH protocol, awaiting donor. Planning to go straight to transplant following protocol in 4 weeks.
God Bless you and keep you all in his tender loving care.