What's in a name?

There's a lot in a name.  Part of the frustration is feeling like a science experiment.  Each kid seems to come up with new problems all the time and their list of diseases is growing.  It's hard for me to wrap my mind around the fact that they have so many problems that seem to get treated individually.  How does one person have 4-5 different diseases?  There has to be one name that sums up everything.  It's not often that people understand this. 
     Yesterday, I expressed some of my anxiety over that and our doctor actually agreed that not having a name is very difficult and not just for us.  Without a confirmed name for this disease, she can only guess at the right treatment.  We have already experienced firsthand what happens when they treat the kids under the assumption that they are 'normal' kids with a few strange problems. 
     In 2007, Shelbie had this same problem with her platelets.  After many failed treatments, they turned to their last resort, VinCristine, a chemotherapy drug. In most ITP patients, Vin Cristine would work wonderfully and with very few side effects.  For Shelbie, she was so sick.  Sick to the extremes and her hair fell out, something they promised her would not happen.  As it turns out, we recently learned, on one of our Seattle trips that SDS kids who get VinCristine will have extreme side effects and it can actually harm their marrow and cause irreversible damage. 
     It's important that those kinds of mistakes don't happen again!  I guess that's why Dr. S had a bit of worry in her own voice and I sense she really is in a hurry to call this disease by it's appropriate name.  There is some discrepancy about how long Shelbie should stay on IVIG.  Our local Oncologist wants to end those treatments in April and begin a new drug.  Our Hematologist in Seattle is very much against the new drug because it is known to cause scar tissue to build up in the marrow.  Scar tissue in the marrow makes a transplant very difficult and risky.  I am confident that the two doctors will come to some common ground but when you start weighing out the risks that come without knowing the exact name of this disease and the process by which it destroys the body it is really scary. 
     Before Dr. S and I finished our conversation yesterday, I asked her what I should call the disease my kids have.  I just need something to hang my hat on .  She said, "They definitely have Bone Marrow Failure Syndrome, and I am comfortable calling Sam 'Clinically SDS' and Shelbie and Spencer are definitely in the arena of SDS.  For now, I am treating them as I do all my SDS patients and refer to them as SDS kids, this is what fits them the best."  I guess for now, that will have to do for me too. 


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