Crisis averted

OK, maybe not averted. More like endured.

Sam was discharged yesterday evening after a distressing two days back in the Hematology unit. We still don't know definitively what happened or why Sam was off the rails. ER staff seemed pretty confident that Sam was in withdrawal from having been weaned off of Ativan and Methadone a couple of days prior. But Hematology staff were, apparently, never fully on board with that diagnosis because Sam's WAT score ("an assessment instrument for monitoring opioid and benzodiazepine withdrawal symptoms in pediatric patients") wasn't high enough.

Ultimately, Sam was left essentially untreated. Staff "watched" him for 48 hours, gave him prophylactic antibiotics, and made sure he didn't have a fever--all important and appreciated, but a bit unsatisfying given that we knew how distressed he'd been (and, at times, continued to be during our stay).

The doctors were, understandably, very hesitant to put him back on either drug, but they never offered a reasonable alternative diagnosis other than to bandy about theories that he was perhaps colicky or just fussy, because, you know, babies can be fussy. To be fair, Sam was anemic, and they did give him a transfusion. But another of their theories--that the anemia was making him feel bad--felt like a stretch. And I think it was. Two hours after the transfusion was done, Sam was back to behaving like he'd been when we came to the ER in the first place: highly agitated and generally inconsolable.

Take. Me. Home.

Bottom line: we are home again with a smiling little boy.

It's worth saying that on balance, Children's has been terrific. They saved Sam's life last month, and we have every confidence that he is where he should be for the transplant. The weekend was crappy, but I can't complain too loudly.

Back to the ER

We had a great week at home with Sam. He was smiley and comfortable; he was sleeping and nursing well; his ANC (a measure of his risk for infection) was normal rather than depressed, as it was for much of his hospital stay; Tuesday's labs were good, and Thursday's spinal tap drew nothing but crystal-clear spinal fluid; we were told that there are a half-dozen solid marrow donor candidates in the pipeline.

Ambushed by Mr. Duck, the wash mitt.

But a day or two after he was fully weaned from Ativan and Methadone, Sam got fussy. And then really fussy--he could hardly be touched or moved without exploding into shrieks the volume and ear-piercing qualities of which had never before been heard. He refused to eat, and his temperature was a little elevated. (Yesterday morning he was in the low 99s--not quite to the 99.5 threshold they set for us, but well above the mid-97s he'd had all week.)

At Hematology's suggestion, we went to the ER and, with the All-Powerful Red Priority Badge in hand, went right to the front of the triage line (which would have been really satisfying if anybody else had actually been in it). We ultimately spent eight long hours in the ER, getting tests and trying mostly in vain to comfort Sam while ER and hematology staff conferred.

Proper children's hospital ER decor: assorted crayons, random Nat'l Geo map, box of condoms

Early diagnosis: withdrawal. Treatment: a dose of Ativan and admission to the Hematology unit last night*.

Sam's mood (and willingness to feed) had mostly returned to normal by this morning, but he's still not entirely himself, and the diagnostic consensus that we thought we had last night in the ER seems to have dissipated up here in Hematology. As have some of the soothing effects of last night's Ativan squirt. Which means we don't know how long we'll be here or how this is going to play out (e.g., if they'll put him back on Ativan or, if so, what a new wean protocol would look like).

Fortunately, there are no signs of infection or any other potential problems. Still, it's frustrating that poor Sam is stuck in (a rather unpleasant) limbo while the hospital decides what the problem is and what to do about it.

Feeling better this morning

* much to the pleasure--properly qualified of course--of some of the nurses here, many of whom have come to adore Sam

Settling in at home

It's been 48 hrs. since discharge, and the sailing has--so far--been smooth. Little bit of a scare this morning when one of our digital thermometers showed Sam with a temperature of 99.5 (F)--the threshold for a phone call to the hospital, if not also a visit to the ER. Another thermometer, however, read considerably cooler. Sam looked good and didn't seem distressed or uncomfortable, so we rode it out a bit*.

Sam is sleeping (pretty) well, and that's nice for all of us. We had no idea what to expect given that: he's twice as old as he was when he last lived at home (and was just beginning to sleep for up to four or five hours at a stretch); the last five-plus weeks at Children's weren't exactly conducive to reinforcing any kind of normal sleep pattern.

Sam and a suspiciously affable Mr. Shark

Alyson and I administer seven different medications to Sam each day. Most are given once daily, but a few are given several times. Fortunately, Sam is being weaned from a couple of those (methadone, Ativan) and will be off them by early next week. He's also being weaned from dexamethasone, a steroid used to suppress the inflamed white blood cells that were attacking his body. That wean, however, is very slow, and may be halted if future labs show evidence that HLH is mounting a counter-attack.

The home-care nurse made her first weekly visit today to change Sam's PICC line dressing, weigh and measure him, etc. Sam weighed in at 9lb 4oz,--a tiny bit less than when he was first hospitalized. The dressing change went well, and the rest of the visit was, I'm happy to say, entirely uneventful. (I found it reassuring to have a nurse in the house for a bit.)

It's great to have Sam back. Having to administer meds, take his temperature (mantra: Please don't hit 99. Please don't hit 99. Please don't hit 99.), flush his PICC, and otherwise avoid infecting him with, well, anything, is nothing if not anxiety-producing. But Sam smiles a lot, and that goes a very, very long way. (The best, I think, is when he smiles while feeding.)

Apparently unimpressed by mom's new haircut

* The nurse's thermo agreed with our "cool" thermometer, so we've retired the other.

A narrow escape

Sam was brutally attacked by Mr. Shark this evening, but escaped without a scratch. Quite a fighter, this kid.

Tomorrow, barring a last-minute snafu, we'll take him (and Mr. Shark) home with us for some much deserved pre-transplant R&R.

Homeward bound!

The doctor directing Sam's treatment has told us that Sam's white cell count is improving such that we can expect to bring him home "later this week." The details have yet to be revealed, but it sounds like:

• we will need to bring him to the hospital a couple times a week, for what exactly I don't yet know (presumably the weekly spinal tap and chemo treatment, at the very least)
• a nurse will come to the house periodically to do things like change the dressing on his PICC line, which is inserted into (and is about as long as) his upper thigh, and goes directly to his heart
• we will have to flush his PICC line daily and administer the meds he still gets daily, including steroids and antibiotics

He will be immunocompromised, so we are having the house cleaned and tested for mold. We will be able to do some normal things with him (strolls), and will just need to be careful about whom he comes into close contact with. We will not need to wear gloves or masks (or quarantine him à la John Travolta's character in Boy in the Plastic Bubble), but we will need to be vigilant about having clean hands. The primary goal is to prevent infection.

And then we wait. To see if he remains stable, and to hear that the hospital has found a bone marrow donor. The former will be a daily regimen, the latter a weeks-long process. (We've already heard that they've identified potential donors, but those people now need to have their marrow re-profiled, need to be in good health, still need to be willing and able to undergo the donation procedure, etc.)

We're a bit nervous about having Sam home, but we're also thrilled. He's lived at Children's Hospital for nearly half his life. Much too long.

More pix of Sam

Gazing at mom

Smiling at dad

The Thinker

Homeward bound?

Sam and me this morning. Mr. Shark is in the background.

The doctors told us yesterday that the only thing keeping Sam in the hospital right now is his white blood cell count. If/when that count goes up past a certain threshold, they'll send him home with us. Which would be (mostly) great, if only temporary--he will eventually have to return for the long, arduous transplant process.

Mostly? Because the burden of properly taking care of an immune-compromised infant for several weeks would be ours to bear, not the hospital's. They would surely teach us all we need to know about protecting him, taking care of his PICC line and nasogastric tube, administering any meds he's still on, etc.; that part doesn't bother me. What does is knowing that we would surely not be as quick as hospital staff to identify problems (e.g., infection) and, if needed, have them properly addressed. I don't mind taking his temperature several times a day. I'm not thrilled to know that if he's even mildly febrile he'll have to go (back) to the hospital...stat! [Update: also a bit anxious that we will have to try to turn our house--tidy if not genuinely clean--into a more sterile environment, free as much as possible from bacteria, dust, mold, fungus, and whatever else they tell us would pose a threat to Sam. I can easily imagine becoming paranoid in not much time over whether we, Sam's parents, are endangering his health by not doing X, Y, or Z adequately, properly, etc. Anyway, nothing is guaranteed, and we are still probably a couple of weeks away from any of this.]

On the other hand, we would dearly love to not spend all day, every day at the hospital. Not have Sam tethered to a machine. Feed him at home. Take him outside. You know, live something of a normal life.

Sam: The Movie

Here's a quick clip of Sam (w/ hiccups and a raspy throat from the ventilator tube) from the weekend. Special appearances by: Alyson's voice and arm; the ever-present monitor alarm(s), which Sam's wiggling sets off regularly.

Climbing Mt. Everest

Sam continues to improve. He's eating really well again, his labs are in good shape, he seems to be coming off of the methadone fairly smoothly, and when he's awake he's alert, vocal, and active. In addition, this week's spinal tap showed no evidence of HLH in Sam's spinal fluid, so he can forego next week's tap and part of his chemotherapy. Today we played with a rattle, and I saw the first smile I've seen since before we came to the hospital more than three weeks ago. As near as I can tell, Sam is well on his way to being stable and strong enough for a bone marrow transplant (BMT). The hospital has started to look for a donor, and we hope to have an update in the next four to six weeks.

But on Wednesday we met with a doctor from the BMT team, and I couldn't help feel that the progress Sam has made over the last few weeks amounts to little more than getting to Everest base camp during a mandatory attempt on the mountain: necessary but not at all sufficient, and mostly insignificant relative to the long, punishing, and possibly fatal journey that awaits him.

The positive news is that Sam's ancestry puts him in a very good position for finding a bone marrow donor. The doctors don't give us odds or percentages, but they've all spoken confidently (and convincingly) about the likelihood that they'll find a strong match--i.e., a donor whose marrow profile matches up well with Sam's.

There are risks along the way, however.

In the days immediately preceding a transplant, Sam will undergo more (and, I think, more intense) chemotherapy in order to lay waste to his current, faulty immune system. This will put him at significant risk of infection, illness, fever--nothing good for a four-month-old with no natural defenses. The risk of infection will, I gather, remain high until the imported cells set up shop and begin building Sam his new immune system--a process that will take months.

Once he gets the transplant, Sam faces the risk of rejection in the form of Graft-Versus-Host Disease (GVHD), in which some of the transplanted cells attack rather than protect his body. This can be treated, but not always successfully, and not always without incurring damage to Sam's liver and kidneys. (The odds of getting GVHD are reduced by finding a good donor match.)

Sam will be in the hospital post-transplant for 4-6 weeks, after which he will be required to return to the BMT unit several times a week for testing. I'm not sure how long that process takes or how soon the follow-up visits begin to taper off, but it's certainly on the order of months. All told, it sounded like it would be a year before we'd really be able to see this in the rear-view mirror.

The pre-transplant chemo process will cause Sam to lose his hair (which seems relatively minor but which will probably hurt like hell for us), and he may ultimately face a whole host of side effects, some nastier than others.

So that's what's in store for Sam. We, in turn, have to look forward to days and weeks and months of the same stress--fear, anxiety, despair--that's already been the hallmark of this experience. To trying to "take it one day at at time," even if that's excruciatingly difficult to do without obsessing over every lab test, every procedure, every vital sign. To wondering if Sam is suffering, or will be. To not knowing, from one hour to the next, if he'll live through this. Or, for that matter, how we will. 

Your support

I'd planned to describe our meeting with the bone marrow doctor today, but I want more time to get my head around it. In short, it was very difficult. I wouldn't say we're in quite the same state of shock that we were three weeks ago (when we rec'd the initial diagnosis of HLH), but it's not far from that. If the last week's good news has made me lose sight of how difficult the road ahead is, today's meeting brought the brutal reality of the next 3-4 months into sharp focus.

In the meantime, a quick and insufficient note to acknowledge if not fully and appropriately thank everyone for the extraordinary support you've been showing us. Your emails, gifts, gestures, Facebook comments, Tweets, and offers to help really help keep our spirits up. And we need that. Thank you.

The gofundme fundraising site set up* by my friend and co-worker (and former boss) at National Geographic, Barbara Ferry, is extraordinary. To say Alyson and I are grateful--not to mention shocked at what people are doing for us--is understatement. The site has a "send a thank you" function that I have yet to use, but I will. Please just know that your generosity has not not not gone unnoticed (!) and will not go unacknowledged.

* Despite what it site says, I am not the gofundme site's creator. I don't know what happened, but at some point Barbara's name was replaced by mine. I'm not happy about it--I don't like feeling like I'm holding a hat out--but I haven't figured out how to change it back.