Goodbye ICU, Hello 4th Floor

After more than two weeks in PICU, Sam is stable enough that he no longer needs a dedicated nurse (whose desk is two feet from his room) and round-the-clock observation. So we have moved to "the floor," hospital-speak for anything that's not an intensive care unit. In our case, this means the Hematology and Oncology unit (4th floor).

Leaving PICU is genuinely a good thing, but we are careful not to get over-confident. We were moved to the 4th floor once before, about three days after we were first admitted. We made it all of one night before the doctors saw something on Sam's chest x-ray that they didn't like. Back to PICU we went, followed by more than a week with Sam on a vent (among other things).

We are very happy to have Sam in such a good place again. He looks and sounds better than he has since this all started on June 9. But as we have been reminded, HLH makes for a long journey. There is still lots of time for things to go awry, and it's possible, if not likely, that we'll have to go back to PICU for one reason or another.

In the meantime, we are expecting to talk to the bone marrow transplant team sometime this week. It'll be good to learn more about how that process is supposed to play out. And it feels good to be able to think--even in the abstract--about what a light at the end of our tunnel might look like.

MRI results

Another really good day. The MRI results finally came back, and they showed no impact of HLH on Sam's brain. Which, obviously, is an enormous relief.

The MRI--and the prospect that Sam had suffered some kind of brain damage--has been lurking out there almost since the initial diagnosis of HLH, when Dr. Greenberg (the team leader) uttered the vaguely euphemistic phrase "brain involvement" in reference to the HLH. The hypothetical became a bit more real when the first spinal tap found evidence of HLH activity in Sam's spinal fluid.

Sam first went for an MRI 10+ days ago, but he wouldn't sit still for the exam. Then he went on the ventilator, and for whatever reason the PICU staff waited a full week to take the sedated (and conveniently motionless) Sam back to MRI for another attempt.

I have tried not to think too much about brain damage. It's very upsetting, and though potentially serious, we haven't had much emotional energy to spare. It's also somewhat abstract--it could conceivably take years to reveal itself. So, first things first.

Plus, I was optimistic. Since Sam came off the vent yesterday, he's seemed very much like himself. He's vocal, he wiggles around, he makes solid eye contact, he chomps away at his pacifier. His body has nearly always seemed to behave as expected. His major organs, although occasionally taxed from the treatments he's receiving, have always operated properly. Sam has not shown evidence that any of his most important pieces and parts--except for the broken immune system that brought us here in the first place--are defective or otherwise malfunctioning. These are all good signs.

As such, I'd quietly and hopefully been telling myself that maybe he hadn't suffered any neurological damage. Or, if he had, that it was really minor.

Even so, when the doctors told us this morning that the MRI showed nothing abnormal, I made them repeat it several times. "So you're saying it's totally clean? No damage? Normal normal?"

Yes, they said. Each time.

And that ultrasound? Not sure what that's about, they said, but not to worry. Ultrasound is not nearly as powerful or precise as MRI, so if nothing shows up in the MRI, there's nothing there.

Good Great answer.

Extubated

That's a fancy way of saying that Sam is off the ventilator! The PICU staff had been talking about it for several days, always saying that "later this week" (i.e., Th or Fri) would be a best-case scenario. At rounds this morning they announced immediate plans to directly test Sam's ability to breathe on his own. I assumed those tests would take most of the day--these things are usually managed in 12 or 24-hour increments, and almost nothing at the hospital happens when you think it's supposed to--but I was wrong. And more to the point: Sam aced the tests and was unhooked by early afternoon.

He still has an oxygen cannula in his nose, but we can now hold him again. (He's connected to a half-dozen wires and catheters, but there's enough slack to take him to a bedside chair.) This afternoon he was alert, making great eye contact, and slowly finding his voice again. He's a bit hoarse and congested, but we're told that'll clear up shortly. He didn't seem too interested in a pacifier, but after eight or nine days with a tube down his throat, who can blame him?

We're still waiting to hear the results of yesterday's MRI of Sam's brain. We're nervous about it, but for the moment we are just enjoying our little boy's return from his long, morphine-fueled slumber.

One big way you can help

Several of you have asked if you can help defray the likely-to-be-exhorbitant financial costs of this little exercise. We do have insurance, but it's hard to see how the fees associated with 6-8 weeks in the hospital (let alone the add'l costs of a subsequent bone marrow transplant) aren't going to eventually spill over the dike that is our policy.

Courtesy of some friends and family, there's now a fundraising site at http://www.gofundme.com/samfry. We're very grateful for anything and everything. Thank you. (By the way, that $5 anonymous donation listed on the site? A test.)

P.S. Either way, please continue to send us notes at michaelwfry[at]gmail.com and/or alyson.foster[at]gmail.com. They're really nice to get.

Day 14

A good day for Sam. They've been lowering settings on his ventilator (to reduce the support it's providing him), and he's been handling it well. If he holds steady over the next day or so, they'll start adjusting the meds so that he can come off the vent and return to consciousness.

Tomorrow or the next day he'll get another dose of chemo (etoposide). They're also talking about trying to do the long-discussed MRI while he's still sedated.

Two weeks down. Six to go.

Day 13

Not much to report today. Sam is "stable," whatever the hell that means. Nearly all of the seemingly infinite number of vital signs, numbers, and levels that the hospital staff are tracking are in a good place or moving in a good direction. They've taken him off the blood pressure meds, they are starting to wean him from the ventilator, he's being given more breast milk (and he's crapping it out like a pro), and he gets pissed when they change his diaper. (Well, as pissed as he can be given that he's mostly sedated and woozy.)

So these are all good signs. And yet I can hardly bring myself to tell people that he's doing well. Because he's not. He still has HLH, and that will kill him if these treatments don't work, or if his body decides that the 6-8 weeks of pounding he's going to endure (plus the bone marrow transplant, should we get that far) is just too much to handle. It's been less two weeks since we admitted him, and already most of his major internal organs and bodily functions have come into play. Brain and nervous system. Heart and blood. Lungs. Liver. Spleen. Kidneys. Pancreas.

A week or so ago, they came to check his heart. My first thought--which I later recounted to friends--was: "Wait, that's at risk, too? How many mines are in this @#& field?"

One of said friends replied, "It doesn't matter how many mines are in the field. The only ones that count are the ones you step on."

He's right. But it's still a minefield.

Father's Day

I'm not big on Hallmark holidays, but I can't say this is how I pictured my first. I'm hardly alone, though, in a hospital filled with patients and families who would rather be elsewhere.

I look around the floor and wonder why all these other (mostly) kids are here. Children's hospitals specialize in conditions of a certain type, not people of a certain age, so there are patients here--patients with "kids' diseases"--who are in their late 20s. Most are younger, much younger than that. Sam is very young, but he is not the only infant I've seen in PICU.

One 11-year-old whose father my dad befriended a few days ago was in a horrible head-on ATV accident. He came here via Medevac* from a rural area near Camp David, MD (up near the PA border) and, at some point, had a stroke. Beyond that I know no details. When I see the dad we exchange vague pleasantries ("we're doing well today") but avoid more than the superficial. I don't have the energy to retell Sam's whole story, and it doesn't feel fair to ask him for anything more than I can give myself.

After 10+ days here, other faces, both parents and kids, are starting to look familiar, but we don't know one another's stories and we do little more than greet one another cordially, as if to acknowledge that, yes, we are both still here.

Occasionally I see a pre-school-age boy being pulled down the hall (by dad?) in a little red Radio Flyer wagon, his medical apparatus (monitor and such) in tow. It's briefly cute but mostly sad; he seems to know that the exercise is kind of forced and hollow, a pale imitation of life on the outside.

* Which his dad said cost just short of $50000.

Pix of Sam

Alert in the bassinet

Apparently dissatisfied

Bath time

Nap on the couch

One of his first smiles

Day 10

Several pieces of good news this morning.

Sam's chest x-ray showed improvement, which suggests that the treatment is having an affect on the HLH (and fluid?) in his lungs. That's good for a few reasons.

With less crap in his lungs, the team has decided that it's now safe to start feeding him breast milk again (via a tube in his nose). The milk replaces a nutritional supplement (whose name I forget) that was going in via IV. I'm not sure how it all pieces together, but the fact that Sam is on a ventilator meant that they were reluctant to put fluid into his tummy that he might aspirate. He's still on the ventilator, but somehow the risk of aspiration is reduced.

Anyway, it feels good to know that Sam is again getting the best food out there, one that includes important antibodies from Alyson. It's also nice to know that he won't feel hungry anymore.

The x-ray result means Sam is also on a path to be removed from the ventilator--maybe in the next couple of days? And that means that he would not need to be sedated anymore, could again be held and breastfed. We are dying to hold him, look into his eyes, and know that he's looking back.

Finally, with less fluid in his lungs, they have started (or will soon start) to wean Sam from the diuretic they've been giving him to help him excrete all the fluids he's been getting. It's nice to have him getting one less med, and nice also to think that his kidneys will get a break. The more we can spend the next couple of months just dealing with the HLH, the better.

Day 9

We've made it through Week 1 of a long (8 weeks?) treatment protocol for the HLH. Sam has undergone more medical procedures in the last week (including lumbar punctures [aka spinal taps], x-rays, CT scans, echocardiograms, ultrasounds, and more) than Alyson and I have had in our entire lives. He receives steroids and chemo to stop the HLH and break down his immune system, as well as a slew of other medications intended to stave off infection, fever, and/or high blood pressure; help drain fluids from his lungs; help him pee; keep him sedated and comfortable; etc.

The HLH appears to be in Sam's lungs, which has made it difficult for him to breathe easily on his own. He now breathes through a small tube in his mouth. He is sedated, and that, in turn, means that we can no longer make meaningful eye contact with him, feed him ourselves, or hear any of his many vocalizations. For the most part, then, we can now only watch and hope that Sam continues to be stable, responds to the treatments, and continues to tread lightly through what feels like a minefield of possible side effects.

The HLH is also in his spinal fluid. The implications of that are unclear to me, but at least it's being treated.

There are some other good signs. His blood levels (e.g., platelets) and some of the other markers of HLH have improved since the treatments began. His heart and kidneys are in good shape, his liver and spleen aren't so enlarged anymore (a by-product of the HLH), and most of his other vital signs are normal for someone of his age and size. We are hopeful that his lungs will start to clear up.

Every day seems to be its own roller coaster--a bit up, a bit down--but we have no real sense of what the end will ultimately look like or if we're making any meaningful progress toward a good outcome. So we ride. What choice do we have? The best we can hope for, I guess, is that we get to ride again tomorrow. And that one day in the not-so-distant future, we'll get to take our little boy home, dress him in pajamas with little bunnies on the feet, and Alyson can sing to him to sleep.

Alyson has begun reading to him here in the ICU. I love that she does it. I like to think that he finds the sound of her voice soothing, and maybe even healing. Either way, it's probably good for her to spend time here doing something meaningful (and distracting).

We have stopped spending the night at the hospital. Although it's hard to leave him, the ICU staff takes good care of him. And since we can't feed or hold or him anymore, there are no longer any compelling reasons for us to stay. At least now we can be assured of getting a decent night's sleep--impossible here at the hospital.

We are enormously grateful for your notes, gifts, prayers, visits, offers to help, and efforts on our behalf. They are a huge boost to our spirits, and we thank you. I'm sorry that most don't receive a response (I'm overwhelmed), but please know that we read everything, sometimes repeatedly.

We are happy to receive visits here in Pediatric ICU, but can't guarantee much of anything: who will be here, what kind of hosts we'll be, whether you'll be allowed in the room, etc. (Official PICU policy is two people in the room. We've almost never been held to that, but one never knows if/when the hammer will fall.)

If you want to visit, please err on the side of caution if you've recently been or think you might be sick. (Sam is in reverse isolation, so you'll have to put on a robe and mask.)

As far as gifts go, we're happy to receive them as long as they don't include flowers or fresh fruit, both of which are apparently potentially dangerous to Sam.

I have no idea what all this means for us financially--I haven't yet had the energy to pay attention to anything but making it to tomorrow. Nobody ever indicates which of the myriad procedures, meds, facilities, tests, staff, and services are covered by our insurance, or to what extent they're covered. I'm not sure it would matter--Sam's fragile little life is on the line, and nothing they've done has seemed optional.

There are rumors that somebody at work is doing something (or wants to be) about a fundraiser event/site, etc., but I don't know any details. If that comes to pass, I will keep you posted. (I cannot imagine either of us asking for help, but we know that we cannot turn it away if it's offered.)

Several of you have mentioned donating bone marrow. Thank you--to say we're touched is an understatement. I know almost nothing about bone marrow transplants, but two things have been made clear to me. First, one's blood type has nothing to do with whether one's bone marrow would match Samuel's. Second, the hospital will be responsible for finding a donor, not us.  That doesn't mean you shouldn't donate. The hospital would search for a match, so it only helps to have a bigger pool from which to draw even if your marrow goes to somebody else.

Day 6

It's still too early to say anything about where this is all headed, but so far Sam is both handling the treatments well and, given how some of the numbers and such look, seems to be responding. (The lead doctor is very cautious but he, too, shows signs of being encouraged.) Fortunately, Sam also continues to be more or less himself. His intake and output are very good, he's as vocal and ever (which they say is always good), and he looks like himself, if a touch puffy from the steroids. 

Next hurdles: the MRI and spinal tap (aka lumbar puncture), both designed to see if there's been any of what the staff euphemistically call "brain involvement". They tried the MRI yesterday, but we couldn't get Sam to doze off. I think they'll have to knock him out to get him to stay still. The LP requires his platelets to be higher than they've been (they need to be sure that his blood will clot if they're gonna poke him internally), so we'll see if/when that happens. 

Alyson's mom stays the night with him sometimes, which is enormously helpful--it gives us a chance to get a full night's rest in our own bed, distance from the hospital, and the very soothing knowledge that Sam will be watched and held by somebody who loves him. ICU staff are great, but family is family. 

It's looking like Alyson's mom is going to stay here on a long-term basis, which would, among other things, give us a second car, extra hands around the house, a nanny of sorts, a medically literate advocate, and Alyson's best friend all wrapped in one. It would also make it easier for me to go back to work (where we get our insurance).

We are enormously touched and gratified to have received visits, gifts, notes, and to hear of concern and efforts on our behalf. It's humbling and flattering and, admittedly, a bit uncomfortable for people who aren't very good about accepting help and are generally wary of feeling that we'll be imposing. (I realize that's absurd under the circumstances, but it's still true.) 

Everyone asks if there's something they can do, but the truth is that right now we don't know; we're sort of taking things hour by hour. We need to eat (both there and at home), but don't know who's going to be where to receive food or groceries or do anything useful with it before it goes bad, etc. (Just yesterday I learned that there's a fridge at the hospital for family use, so that's good to know.) We benefit from time away from the hospital, but again, don't know yet how to see into the future to plan such things. Visits are also nice, but traffic in the ICU is supposed to be limited, and with Alyson's parents and my dad currently here (if in and out a lot), it's hard to say that X or Y is a good time. Or maybe it's not at all hard, and we're just too overwhelmed to see how easy it actually is?

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.