It’s decision time for Zoe.

I’ve talked about this before, about our stress and uncertainty with the decision to transplant Zoe, but until now it has remained a discussion rather than an imminent decision. I’ll try to talk about what’s going through our heads, what this decision entails, and what we’ve decided.

The question is whether or not to have a Stem Cell Transplant, or were she a little older, a Bone Marrow Transplant. They are essentially the same decision. If transplanted, Zoe will have an entirely new bone marrow, or very nearly new with shades of the old one.  Her blood type will become whatever type the donor cell material is, if that helps bring it home the level of change we’re talking about here.

She will undergo a week long conditioning regimen that will destroy her bone marrow and her immune system entirely, taking with it any remnants of HLH. She’ll then receive a transfusion of stem cells over the course of about 2-3 hours into her blood stream, and those cells will “magically” know where to go, into her bone marrow. Within approximately 10 days, assuming a graft takes place, her bone marrow will begin to reconstitute and produce new blood cells — red, white, etc.

I’ve talked about the risks, and there is not a lot more to say about them. They are what they are. There are severe risks, though the percentages are not as high as we once feared.

So how do we decide? Well, there are some factors.

Things that tend to indicate a FHL or Familial HLH case (all FHL cases must have a transplant):

  • Child’s age is under one year
  • Child has no underlying conditions which could “activate” the secondary type of HLH (cancer, epstein-barr or other virus, etc)
  • No measurable Natural Killer (NK) cell function
  • Soluble IL-2 reading greater than 10,000
  • Presence of Histiocytes in the spinal fluid at some point during treatment
  • Presence of one of the genetic markers known to correspond to FHL

Zoe is positive for all of these indicators, with the exception of the one that proves FHL, the genetic marker. We have not yet received those tests back, they take a long time to complete. The other indicators do no by themselves prove anything. Together though, they make it very hard to think she does not have FHL.

In short, we do not know for sure that she has FHL, but we’re nevertheless going to have to make a decision. Given the information in front of us, we have to move ahead with the transplant. We can find no rational reason to hold off, given that she meets nearly every standard currently known.

We have found cases of children under a year who did not have a transplant and who are just fine with only chemotherapy, but we can’t know the details of their cases and have to believe that there is some other explanation. Unfortunately this disease remains mysterious in some ways, and there may be gaps here and there in the knowledge.

What we know for sure though is that we don’t want to take any risks with Zoe’s life. If the disease comes back, it is harder to treat due to drug resistance. Eventually the chemo doesn’t have the strength to fight it off and the child is left without a way to fight at all. Progress with transplant successes has left us feeling like the transplant is the safer option, and it is the only one where we can be sure and not looking over our shoulder. If we can get through it, Zoe will be ok.

We expect to be in Duke’s hands for the pre-transplant work-up within one to two weeks. We’ll have a lot more to share as things pick back up and Round 2 begins. Zoe won Round 1, and we believe she’s got what it takes to go all the way.