Cleared for transplant

Well, almost. The spinal tap came back clean--no rogue white cells found in Sam's spinal fluid--and that's great news. We don't know why his fontanelle was puffy (if only for a day), but if the transplant team is satisfied, we're satisfied. The only things left are a few tests that he's not expected to fail (e.g., hepatitis) but that they have to know he doesn't have. So, one last weekend at home with Sam before we begin the long haul.

Or so we thought.

Yesterday I clipped Sam's left thumb while cutting his nails. Sam didn't flinch, and there was no blood, but I lopped off a piece of skin. I put rubbing alcohol on it (still no flinching) and some antibiotic ointment, then more or less forgot about it.

This morning Alyson noticed that the area around the wound was a little red and inflamed. Infection?!? I called the hospital for guidance--now is not the time to take anything for granted. On their advice, we went to the ER. Blood tests confirmed that his immune system is, for the moment, quite capable of dealing with a minor infection.

But that didn't stop us from being there for five hours while they drew blood, tested it, consulted, ordered antibiotics, infused antibiotics, flushed IV line, etc. We'll return tomorrow for a second dose and will have to give Sam an oral antibiotic for a week. Nothing is quick or easy at the hospital.

We're home now. I just finished doing some stuff in the kitchen that I'd started this morning. Alyson took a nap and is now reading while Sam sleeps on her tummy. Our version of normal.

P.S. Sorry, no pix this time. How about some cat videos?

P.P.S. OK, here's one:

The Great Betrayal: Attacked by his own burp cloth.

Pins and needles

Another busy (and frustrating) week that will end on a note that's either very high or very low.

Background: our original transplant schedule was delayed for a couple of weeks, and we've since been in a holding pattern. The time has gone quickly, and we've continued to love having Sam at home. We started taking him out for walks in a Baby Bjorn-like carrier thing, and it's great...

But on Monday Alyson noticed that Sam's anterior fontanelle was bulging out a bit. This, we'd been told, would be bad. I assumed it meant infection, but since Sam didn't have a fever, attention shifted to the other option: HLH activity in the brain. Although the HLH is very much under control elsewhere in Sam's body, the brain is something of a "sanctuary"--the HLH can hide out there and be difficult to detect and treat.

Sam has been getting periodic (roughly twice-a-month) spinal taps and chemo treatments, and the assumption had been that his central nervous system was in good shape. The fontanelle has since Tuesday looked and felt normal, but the doctors wanted to take no chances. They scheduled another spinal tap and dose of chemo (methotrexate) for today; results are expected tomorrow.

If we get good news tomorrow--no HLH activity detected in the spinal fluid--we will be cleared for transplant and admitted on Sept. 1 to the hospital's Blood and Marrow Transplantation unit for the beginning of the long transplant process (more on that soon). We're both dreading the transplant process, but it's the only way out of this mess--the sooner we get started, the sooner we can be done.

If, on the other hand, they find any evidence of HLH activity, they will delay transplant for several (2-4?) weeks in order to stamp out--or try again to stamp out--the HLH*. And we will be deeply disappointed. And scared that another shoe will drop before we ever have a chance to go to transplant and knock this @#$ thing out.

* Active HLH during transplant is a bad thing. It reduces odds of a successful transplant, and increases the odds that the HLH will come back. And if the HLH comes back, the prognosis for Sam is decidedly less rosy.

Anyhow, here's more recent pix. The last one almost made Alyson pee.

 

Guess which one of us had his nails clipped moments later?

 

Transplant delayed

The donor's not yet available to undergo the procedure, so we're on hold for a couple of weeks. This is disappointing for us--we want to get this damned thing over with--but not necessarily a bad thing. The doctors apparently feel good enough about Sam's current state (and the quality of the donor) that two more weeks at home is worth the wait. So, two more weeks of PICC flushing, squirting meds down Sam's throat, and hoping he doesn't get infected. So far, so good.

Sam is a bit underweight for his age (chemo will do that to a kid, apparently), so the extra time at home gives us more time to fatten him up with the NG tube. At some point we hope to get him to take a bottle again. He took it willingly before and during his first hospital stay, but since we came home last month he's wanted nothing to do with anything but Alyson. He'll need to adapt--once he's back in the hospital and Alyson won't be there to nurse him around the clock, he probably won't be allowed to be fed exclusively through the tube.

Nothing more to report, so here are a few more recent pix.

Sam's busy week

We haven't yet received confirmation that either of the donor candidates has scheduled, let alone undergone, the donation procedure, but the transplant team is proceeding as though that is but mere formality. As such, we have been penciled in to return to the hospital for the final, pre-transplant assault on Sam's immune system on Aug. 17, with actual transplant to occur somewhere near Sept. 1.

In the meantime, the hospital is running all kinds of tests on Sam, ostensibly so they'll have baseline data on all his pieces and parts. Today was a busy day. Blood drawn for labs (exact purpose of this particular draw unknown). Then a GFR. Followed by echocardiogram and EKG. And, finally, a CT scan. This coming week they'll do an MRI, as well as the usual chemo treatment and one final spinal tap to test (and, I think, treat with chemo) his spinal fluid.

Sam was in very fine form today--hardly put up a fuss all day long. I thought he'd hate being immobilized for the 20-minute-long GFR, but he was as placid and cooperative as could be.

Alyson, letting nothing at all hang out the CT Scan unit

Alyson and I, on the other hand, were agitated for most of the day by various hospital staff, protocols, etc. We are, for example, continually dismayed at how frequently we are asked questions of a medical nature that, it seems to us, we have little reason to know the complete answers to and that, frankly, should probably not be posed to us in the first place. To wit:

Anesthesiologist before a spinal tap several weeks ago: What kind of PICC does Sam have--single or double lumen?
My unspoken reply: I have no idea what a 'lumen' is or how many Sam's PICC has. Why don't you look at the PICC [five feet away in Sam's thigh]...or Sam's chart...or at the sign on Sam's bed [also five feet away] that in fact describes Sam's PICC"?

Radiology tech before today's GFR: How much blood is Sam allowed to have drawn each day?
My unspoken reply: How the hell should I know? Why don't you ask his doctors, or check his records, or do something similarly instructive so that you don't draw too much, leaving him anemic and in need of a three-hour-long transfusion?

Yesterday's cake takers came on a Radiology unit registration form that asked us to list Sam's medications, the dosages, and their purpose. Alyson and I were indignant.

Sam has spent one-third of his life under very close medical supervision at this hospital for a complex, unusual, and quickly-fatal-if-left-untreated disease. He has undergone dozens of procedures and tests for this condition--again, at this hospital. And he is now preparing for a major, potentially life-threatening procedure...at this hospital. Surely there exists an authoritative resource--at this hospital--where such information could be obtained without further burdening the patient's frightened, bleary-eyed, baby-juggling, and mostly-medically-illiterate parents?

I'm don't think I'm just being a crank. It's demoralizing and anxiety-producing to be asked questions like this by staff about to perform procedures (some of which are inherently risky enough that we're required to sign consent forms before services will be rendered) on our infant son. It makes us wonder if they know who their patient is, know anything about his condition, and are for some reason unwilling to direct their requests for (ostensibly medically necessary?) information to people who might actually know the correct and/or relevant answers.

OK, maybe I am cranky. It was a long day. But still.

Sam's many moods (the good ones, anyway)

The changing table is not typically Sam's favorite venue, but on this occasion he was undaunted.

Transplant update

We had our second meeting with the transplant team this week. The first time we met, we left feeling pretty queasy about what seemed, at least in the abstract, like a very menacing and risky procedure. This time was different.

For several reasons, the doctor (Dr. Loechelt) is confident that Sam's odds of making it through the transplant relatively unscathed are very good. Top of the list: they have two donor candidates in the pipeline--both young males, which is a big plus--that are as well matched to Sam as they can be without being siblings. A good match means that Sam's chances of having serious problems with Graft-Versus-Host-Disease (GVHD) are lower, perhaps much lower, than they would otherwise be.

It also means that the 14-day pre-transplant drug regimen Sam will soon endure to lay waste to his current immune system can be less intense, and therefore less dangerous to Sam and his internal organs. And that also means a lower risk of GVHD*.

Also very good: Sam's HLH continues to be inactive, and that bodes well for the transplant. It's much better that the disease is basically dormant than still agitating and causing internal mischief.

We talked for awhile about the dozens of possible side-effects associated with the pre-transplant medications and were soothed to learn that a good number of the really nasty side-effects that made us so queasy at the first meeting are, while possible, pretty unlikely. Which means we can spend less emotional energy entertaining fears that coma, major organ damage, and even death are dangers we'll likely face in the coming weeks and months.

It is true that Sam will lose the soft-and-delicate-if-not-yet-thick-and-luxurious hair he's worked so hard to grow; that will be hard for us to see. (As a good friend recalled of his own son's scalp-revealing chemotherapy from a few years ago, seeing the hair fall out means you can no longer even pretend that something isn't seriously wrong.) But we've been through this before, back when Sam was intubated for 10 days and you couldn't look at him without being reminded that he wasn't healthy.

Otherwise, the pre- and post-transplant phases have the potential to be pretty normal. Sam will be hooked up to lots of things again, and he may be somewhat uncomfortable (e.g., nauseated) during chemo. But he won't necessarily have to be sedated or intubated or anything else that would severely affect his general day-to-day lifestyle--except that he'll be in a hospital room being groped and poked and prodded and tested at all hours of the day and night.

If there was anything upsetting about the meeting, it was learning that the months immediately following transplant will likely be, even under the best of circumstances, exhausting and anxiety-filled. Sam will have a brand new but very under-developed immune system, and we will have to watch him for even the slightest signs of rejection and GVHD. If he so much as farts in the wrong direction we will be on the phone and, maybe, headed to the hospital for tests. No fun.

On the other hand, I've recently realized that Alyson and I actually find ourselves talking about life after the transplant. And that's a hell of a lot better than wondering if we'll ever get there in the first place.

* If I have this right, high-intensity chemo is, shall we say, irritating to the body. When the new blood cells arrive, they are more likely to attack the host if they perceive this irritation. So, less chemo means less irritation means less risk of grafted cells attacking Sam.