Day 2

Hi,

Eight weeks ago I sent [many of] you the fantastic news about the birth of my son, Samuel. Today I have less good news. If this is not a good time to read something that is, frankly, pretty damned awful, please feel free to quit now. 

Sam has been diagnosed with a very rare and potentially fatal disease called hemophagocytic lymphohistiocytosis (HLH). I can't say I fully understand it by a long shot, but the gist seems to be that he is essentially being attacked by his own immune system. It will be fatal if left untreated, and it may be fatal anyway.

We took Sam to the pediatrician on Tuesday when he showed unexplained bruising and other discoloration in his skin. The doctor ran some specialized blood tests, recognized a few possible hallmarks of HLH, and made a quick referral to Children's, which is one of a small group of institutions nationwide that have banded together to study HLH and try to establish a standard treatment protocol. It's also where a friend and colleague of his--now our doctor--directs the hospital's HLH program. I think if our pediatrician were not connected to this guy, we might be worse off than we are now.

There are still a number of tests outstanding (some of which will not be returned for a couple of weeks), but at this point I no longer remember what they're for, or if they're now essentially superfluous. Bottom line: today's bone marrow biopsy gave us the definitive marker that the doctors were looking for before making a conclusive diagnosis--evidence that the bone marrow is being attacked from within.

I'm afraid that's just about where the good news, such that it is, starts and ends.

The treatment protocol is very aggressive. Starting tonight, Sam will begin receiving a cocktail of steroids (to get his body to stop attacking itself) and chemotherapy (to break down and eventually disable his immune system). This will take several weeks, during which time he will be very vulnerable to infection and, I gather, myriad other complications. Fortunately for him, chemo is apparently a lot less unpleasant for babies than it is for adults. There are, however, still lots of unknowns, including how his little body will react to chemo and the "massive" amounts of steroids that the protocol calls for.

If that phase is successful, the next step--the only available step if the HLH is to be beaten--is to try to replace his immune system with a bone marrow transplant. I don't know details, but our sense from the doctor is that this, too, is an extremely difficult, drawn out, and risky affair. In any event, it is our only chance to keep Sam from being repeatedly attacked and, eventually, killed by his own blood.

All told, we are looking at a couple of months, minimum, at Children's. (We have been in the pediatric ICU since Tuesday but are supposed to move to hematology tomorrow.) What all of this means for Alyson and me on a day-to-day basis, including work, insurance, etc. is TBD. 

Sam actually looks very good and, if he isn't getting poked or otherwise manhandled, is basically his placid and endearingly squeaky self. At times this is a great--and virtually the only--consolation and source of joy for us. Sam has the luxury of ignorance. He's the only one in the room who isn't emotionally devastated and terrified for his future. But at other times, I collapse in tears when we think that our sweet, big-eyed little boy's life is on a razor's edge, will be for several months, and may, if he lives long enough, become unspeakably difficult before it gets better. It all feels unbearably cruel and unfair to Sam, who has only just begun to smile and lock his eyes onto ours. And who, of course, is so limited in what and how he can communicate with us.

The hospital staff have been great. They're always at the ready, try very hard to explain the whats and the whys, and always--as if they've been drilled on it--ask if we have questions. There are a lot of unknowns at this point, however, so the questions I really have necessarily remain unasked and unanswered. 

Because this disease and the treatment of it are so rare (it's essentially an experimental protocol), there's very little accumulated history, statistics, etc. from which to make predictions. Or, for that matter, to take the time to find a second opinion. The medical community that works on this is apparently small and led by a research group (consortium?) that Children's and our doctor are part of. In short, there's no time for a time-out, and nowhere else to turn.

Anyway, the doctor's tone was guarded, at best. In effect: children have gone through this and made it to the other side; don't think it can't work out. But the details (how many, what percentage, and what kind of life they have afterward) were left ambiguous. Perhaps because the data set is so small, or perhaps because it's pointless to get fixated on anything but the here and now.

I apologize if (!) this is morbid, but I figure that if I care enough to give you the good news I have to trust you with the bad. Besides, Alyson and I will have an especially awful time of this if we remain true to our typically reclusive natures. Which is to say that I'd be very happy to hear from you (even though I can't promise a quick reply.) No worries if you are left speechless or don't know what to say. We don't either. I just need to know that the world--my world, anyway--knows what's going on.

The world is awash in tragedy and hardship, but until now my life has been pretty much free of both; I've been extremely fortunate. I guess my number has finally been called.