Day +101: Life at home

Sam's temperament is pretty cheery most of the time. He has his moments--some of them long--but a lot of the time he is smiley and apparently delighted to be here. In the last week or 10 days he's started to coo and squeal, which never gets old for us. He loves his blankets, can't go for long without a pacifier, usually dislikes bath time, and rarely tolerates being awake and alone in a room.

Sam still has the broviac in his chest, and probably will as long as he gets blood drawn two or three times per week. Transfusions through the broviac (e.g., red blood cells) used to happen weekly, but they are rare now. 

A typical breakfast (and dinner) for Sam. The menu changes slightly as meds and doses are adjusted, but the overall quantity has been pretty constant since discharge. As Sam's immune system develops and he needs fewer prophylactics, we'll be able to discontinue several of these. He was recently weaned off of Lasix, a diuretic that has been depositing too much calcium in his kidneys, and oxycodone (opium-based pain reliever), which he no longer needed but which he'd been unable to kick several times before. We'll be most happy when he's weaned from steroids, which he's on this time suppress graft-versus-host disease. 

Sam's first taste of solid food. (Ratio of sweet potato in stomach to sweet potato on face and bib = approx. 10:1.) He still gets fed through the NG tube* overnight, but that is largely an artifact of the first days after discharge, when he wasn't eating enough (we used the tube to ensure that he got needed calories). He's eating really well now, but the tube is still essential as it's how we medicate him. Although he took meds by mouth over the summer w/o much argument, he's on too many right now for that. He's wised up and would not allow use to stick goo-filled syringes into his mouth 30 times a day.

Sam and me out for a walk. Of the many fabulous things about not being in the hospital, the ability to move around freely and show Sam the outside world ranks pretty high. He likes going for walks, and even a quick trip out into the yard is great way to soothe him. (Sam's hoodie comes courtesy of a good friend's mom.)

Sam can't roll over yet, but he's close. Thanks to spending half his life in a hospital bed (and taking huge quantities of steroids, which suppress bone and muscle growth), Sam is underdeveloped physically--he is literally off the growth charts for a baby his age, closer in size to a kid half his age. But he's getting lots of calories, Vitamin D for bone growth, and physical therapy. And he's still damned cute...

...even if the blissful days of odor-free bowel movements are so over. 

* One big downside to the NG tube: Sam has started to pull it out of his nose, as he did just a moment ago. It's not a huge deal to put it back in, but it's traumatic for him. And a bit disturbing when it comes out his mouth instead of going down his throat and into his stomach.

Day +100: Big transplant news

One thing--one very big thing--that I haven't written about much lately (as if I've written much about anything) is the status of Sam's transplant. To fully appreciate where we are you need to know how we got here.

Back in August we agreed to use a reduced-intensity pre-transplant chemo regimen for Sam because it would be easier on Sam's little body than a high-intensity regimen--less harsh, less poisonous, less likely to lead to long-term side-effects. 

We did not like the idea of using more chemo than necessary, and the doctors felt that a standard regimen would be overkill. Sam didn't need his immune system completely destroyed. He needed only for it to be beaten down and suppressed long enough for the donor's T-cells to establish themselves and learn to keep his T-cells--the ones that get over-excited and start attacking him--in check. 

This equilibrium is known as "mixed chimerism," and every week the hospital tests Sam's blood to see what percentage of the T-cells in his blood are from the donor. 

The first chimerism test after after transplant showed great things: 100% of T-cells were from the donor. But every week that percentage dropped by about 10%; by early November it was below 50% and showing no sign of leveling off. 

I started to sweat bullets. Terrified that the transplant was failing, I ran grim scenarios through my head: that the HLH would flare up if the percentage went too low to keep the disease at bay; that Sam would have to go through the entire transplant process again; that all the extra treatment Sam would require (e.g., chemo, steroids, invasive procedures) would begin to break his body down; that Alyson and I would collapse psychologically and emotionally; that Sam would never again experience life outside a hospital, and that he would eventually suffer a long, slow death not from HLH itself, but from the weight of all the treatments, meds, and side-effects (or an infection somewhere along the line).

A few weeks ago the doctors gave Sam 4ml of donor T-cells in hopes that a "surge" of additional cells would help us get the donor T-cell population back up to desirable level. Then we waited, still sweating, for the effects to show up on the chimerism test results--results that take an agonizingly long 7 to 10 days to come back from the lab.

While we waited the percentage dropped to seventeen.

And then a good thing happened: Sam got graft-versus-host disease in the form of a mild rash. Which suggested that the donor cells were replicating and fighting off Sam's own cells.

And then we got the test results back: 79%. A week later we were at 93%. And last Friday we hit 100%.

So we have stepped away from the cliff's edge. It seems unlikely that we'll stay at 100%, but the docs are very pleased--they say the speed with which donor cell percentage rose bodes well for long-term success. It will still take weeks to see where--and if--we reach the desired equilibrium, but at least we're breathing again.

Today, December 25, 2015, we are celebrating our first Christmas with Sam. Even though Christmas really came a week early.

Merry Christmas!

Day +71: Sam comes home for Thanksgiving

After 82 days inpatient, Sam was finally discharged from prison the hospital last Saturday. Getting him stabilized took almost a month longer than I'd predicted. Four times we were scheduled to discharge, and three times we were forced to abort. Two of those were due to last-minute fevers that, according to BMT protocol, require blood tests, a course of antibiotics, and a mandatory minimum additional 48 hrs. of inpatient observation.

When we'd finally signed the discharge papers, a bunch of Sam's nurses gave him a delightful send-off. They adore Sam, and we will forever be grateful for how well they took care of us--all of us--for the last few months. As thrilled as we are to be gone from the 4th Floor, I will miss them immensely. Their affection for Sam, as well as their good cheer and optimism, kept us afloat emotionally more than anything I can think of.

We are pretty well settled in at home. Sam is a lot of work to take care of in his condition (see below), and he has to go back for outpatient check-ups, blood work, exams, etc. several times a week. But I'm not complaining. We are thrilled to be home with him, and he is visibly happier than he ever was in the hospital. 

In the not too distant future I will elaborate on Sam's condition, the landscape ahead of us, and, maybe, why I've been AWOL for several weeks. 

In the meantime, we have a nice long weekend at home with our little boy. As I type, Sam is asleep on Alyson's chest. And snoring.

Happy Thanksgiving indeed.

Dressed for the trip home. Hat courtesy of Aunt Becca.

The Hematology & Oncology nurses gave Sam a lovely send-off. 

Our first meal together at home. No need to comment on how I look like crap--I've already been told. Repeatedly.

The daily regimen includes administering two dozen doses of meds. 

Bundled up after a bath.

Day +38: Why we're still here

We are halfway through our eighth week at Children's, and more than six weeks post-transplant. In many respects Sam is doing very well. All evidence so far points to a successful transplant (good engraftment), and the HLH bio-markers the docs watch in Sam's blood are in a good place; no worries there. Though it's still relatively early, there are no signs of graft-versus-host disease.

Sam is feeling really good, too. He's alert and happy a lot, he's had only a single (and very brief) low-grade fever in the last 10 days*, and he seems not to have any discomfort. His blood pressures have come down a lot, which means he needs fewer meds (always a good thing), and he's being weaned off of steroids--also good news.

Alyson's first full day with Sam since she started work again this week. Sam was in fine form.

So the big picture looks good.

And yet we are still here, and will be until they can figure out why Sam's breathing rate is sometimes high and a bit labored. He is on oxygen, as he has been for more than a month, and has for 10 days or so been getting treated with nebulized albuterol. The albuterol helps control the symptoms but doesn't address the not-yet-identified source of the problem.

The number in white is Sam's respiration rate, which should be in the 30s or 40s. All the other vitals (green = heart rate; blue = blood oxygenation; pink = blood pressure) are good or very good.

Sam sounds clear in the stethoscope--has for weeks--and none of the testing they've done (multiple x-rays, CT scan, echocardiogram) has provided any clues. But the symptoms persist. The conventional wisdom is that he's still too weak to fight off the rhinovirus (common cold) he tested positive for on Day +1. Another theory: Sam's lungs were damaged by HLH back in June, and he hasn't yet recovered.

Sam gets a breathing treatment (albuterol) 4x/day.

Neither theory is very testable, so until they decide that this is actually a problem (and resort to more extreme diagnostic measures, such as a bronchoscopy), there's not much to do but try to treat the symptoms and wait for Sam to get stronger.

And that means not getting to go home.

* For a while he was crossing the (admittedly low) fever threshold of 99.5 °F / 37.5 °C every 24-48 hrs. That he's so much less prone to having a fever says good things about his recovery and lack of infection. It also bodes well for our discharge, whenever that day comes: the fewer times Sam crosses that fever threshold, the fewer times we'll have to bring Sam to the ER.

Day +29: A corner turned?

Lots of good things happening with Sam these days. First and foremost, they did a second, different test of the spinal fluid the took from Sam 10 days ago and found absolutely no evidence of inflammation. This, in addition to other factors, means that the docs are no longer worried that HLH is on the rebound--a huge relief for us. (One immediate implication: they've started to wean him from the steroids.)

I don't think Sam has had a fever in three or four days--that's the longest he's gone in weeks. His fevers have nearly always been brief and low-grade, but every time he goes above 99.5, they draw blood (to test for infection) and put him on a course of antibiotics. Fewer fevers means fewer meds and fewer reasons for nurses to take Sam's blood (which eventually forces him to get transfused).

Sam's oxygen saturation is better, his respiration isn't as elevated as it had been, and the congestion he's been fighting for weeks seems to have lifted. Maybe he's over the cold? He's also not holding on to fluids so much, perhaps in part because he's not getting so much fluid intravenously anymore (several of his meds are now administered orally). And he's not as hypertensive, which means he needs fewer BP-related meds.

Best of all, Sam is simply feeling better. A week ago, when we were still wondering if the HLH was back, one of our doctors told us that Sam's demeanor would be as important to their diagnosis as any lab result. In that respect, Sam has been killing it lately--he's been alert, social, and happy. The last five days have been his best in at least three weeks.

The docs will likely want another spinal tap in a week or two--unlike the body, whose state can be readily monitored through blood testing and other procedures, there are many fewer techniques for assessing the health of the central nervous system; extracting spinal fluid is one of them--but this was always part of the post-transplant protocol, not evidence of anything amiss with Sam.

More evidence that things are looking good: we are being trained on how to change Sam's dressing so that we can do it at home. Nothing's in ink yet, but if Sam continues on the current trajectory, I'll be surprised if we're not discharged before Halloween.

Day +25: Still reading tea leaves

Last week's tests of Sam's spinal fluid and bone marrow were not positive for HLH. That is, they showed no direct evidence of hemophagocytosis, the process whereby Sam's white blood cells attack his own body. This is good news, of course, but it doesn't seem to have been enough to convince our doctors that HLH isn't still sniffing around.

This view of Sam's bone marrow from June shows hemophagocytosis in action. The large, Pac Man-like white cell is devouring cells that it's supposed to leave alone. This was proof-positive of HLH back in June. Last week's bone marrow biopsy didn't show this, but as Carl Sagan apparently said, "Absence of evidence is not evidence of absence."

Many of the other bio-markers the doctors look at for evidence of inflammation are also inconclusive. They could be evidence of something else--if not something benign, then at least something expected given Sam's post-transplant condition, side-effects of the meds he's on, etc. So, with every symptom, lab, or test result the winds seem to shift a little bit. Right now it seems like the doctors don't feel they have enough evidence to begin an all-out assault on the HLH (that might not really be there), but they have put Sam back on a pretty high dose of steroids (dexamethasone) in order to subdue any inflamed white cells that might be HLH.

We are not happy about this--the steroids weaken Sam's bones, contribute to hypertension, and probably impede Sam's ability to fight his cold--but I suppose it's better than a positive identification of HLH. If we do see definitive evidence of the disease, then we face a Sophie's Choice of sorts. We can treat with steroids and more chemo, which would effectively undo the transplant we just went through (because the brand new immune system we just gave Sam would be damaged, if not completely wiped out, by the chemo), and that would require him to endure some or all of this process all over again. Which, in turn, would subject him to more meds and their side-effects, make him vulnerable to infection, etc.

Or, we can bide our time and hope that Sam's immune system grows quickly enough on its own that it can knock out the disease, whatever there is of it, before it starts trying to eat him alive again. And that is a race that Sam might not win.

Needless to say, we're hoping that the soft evidence is not, in fact, disease-related and will fade as Sam gets stronger.  In the meantime, however, we're watching Sam very closely and crossing our fingers that the reason we don't see stronger evidence of HLH is because it isn't there.

The continued uncertainty is nerve-wracking, but there are some good signs. First and foremost, the transplant itself appears to be very much on track. The donor cells are very well engrafted and Sam's blood counts are coming up faster than expected. His white cell count continues to rise, as does his platelet count. This is exactly what we want and need to happen.

Sam has stopped vomiting, and the quick but sometimes extreme bouts of discomfort he was having last week have disappeared. We're not sure what was going on in either case, but it's nice that he's more comfortable than he'd been, and great to have his awake and alert time filled with curiosity and smiles rather than cries of pain.

Finally, from the "good-news-if-dodging-a-very-big-bullet-is-still-considered-good-news file"...

Last week one of the overnight nurses saw what she thought might be seizure-like activity when she noticed Sam's arm extended and trembling rhythmically. Seizures are a possible HLH symptom, so the docs ordered an EEG with video so they could monitor Sam's brain waves. Sam's head was wired up so that he looked like some sort of electric Einstein (or Medusa?), then watched for 12 hours overnight. The video showed three occasions where Sam's arm did the trembly thing, but there was nothing in his brain activity to suggest that it was abnormal. This was an enormous relief for obvious (and some not-so-obvious) reasons. Suffice it to say that we had a pretty shitty 24 hours waiting for those results to come back.

Now if we can just get Sam to kick his cold, we might actually be able to take him home.

Sam is a boy...

...who adores his mom.

Day +18: An HLH comeback?

Sam's recent labs indicate that there may be some HLH activity in his body. The signals are mixed, however.

On the less promising side, Sam's ferritin and soluble IL-2 levels are elevated--not abnormally high, but higher than expected given his condition. Both are bio-markers for HLH, so both are red flags. In addition, Sam's triglycerides are abnormally high. This, too, is indicative of some kind of inflammation, including--although not necessarily--HLH.

On the other hand, the docs say HLH activity this soon after transplant would be unusual. Moreover, Sam's platelet count is healthy and rising. HLH attacks platelets, so a healthy platelet level would be inconsistent with HLH activity. Sam also recently tested positive for a (readily treatable) bacterial infection, and the associated inflammation could account for at least part of the ferritin and soluble IL-2 values, if not the triglycerides.

In short, we have mixed signals and need more data. Tomorrow Sam is scheduled to get another spinal tap (to see if there's evidence of HLH in his central nervous system) and his second bone marrow biopsy. Results, which we hope to see late Monday or early Tuesday, will tell us a lot more about what's going on.

It's ironic to be rooting for a bacterial infection, but I guess that's where we are.

We still have not yet seen results from the first chimerism (aka engraftment analysis) test, so we don't yet know how much of Sam's blood is his own and how much comes from the donor. Doctors are confident, however, that we will see continued evidence of good engraftment. Sam's donor was perfectly matched, and his blood counts would not be rebounding so quickly if the engraftment wasn't going according to plan.

In the meantime, here's Alyson and Rufus serenading Sam. Background melody provided by the mobile overlooking Sam's bed. Security courtesy of Mr. Shark.

.

Day +15: An unforgettable day

Our room is in the Blood and Marrow Transplant section of a floor dedicated to Hematology & Oncology patients. The BMT unit's 10 or so rooms are behind two sets of magnetized double doors, and visitation is restricted because BMT patients are, by definition, in a fragile state due to their deeply impaired immune systems. Their admissions last weeks or months at a time, and they're here because they have serious, life-threatening conditions that require serious, life-threatening measures. Any way you slice it, it's pretty sobering stuff.

Patients are confined to their rooms except for procedures that have to be done elsewhere (surgery, MRI, CT scan, etc.), so the only people we see around the unit are staff and people that I presume are patients' family. We've never actually met anybody*, however, so we know nothing of the other patients except that they have recently undergone or are about to undergo a transplant procedure intended to cure/relieve/address some kind of blood- or cancer-related condition. We acknowledge one another in the halls, but only briefly and in an obligatory sort of way that suggests courtesy and empathy if not genuine interest. Anything more than that is more than we have the energy to muster. I've got my own horror show to watch. I don't need to know anything about yours. 

It has not been unusual for us to hear crying or screaming through the walls, but since we've never seen any of the other patients, we can't visualize who's making the sounds, or why. (In one case, we learned that the patient had an ostomy that required regular and, apparently, extremely painful attention.)

A couple of days ago we heard noises (cries? shrieks? wails? I haven't found the right word) that were unusually anguished and emotional, and occasionally punctuated by the sounds of impact--a body against a wall? fists on a table? After a few moments I realized that the sounds were coming not from the patient (a little girl, I think, based on the voice we've heard before), but from someone else--someone who soon began to scream "Oh my god. Oh my god." 

Within a few seconds, staff were flying down the halls from every direction into their room. Something was going--or had already gone--horribly, horribly wrong. We--Alyson, me, Alyson's mom, our social worker--traded alarmed glances but didn't speak. The nurse seemed to try to carry on as usual, but it was impossible to ignore the anguish and the commotion 10 feet away even though we could see nothing but flashes of humanity rushing past our door.

When I realized that Alyson and I were both starting to tremble and wither emotionally from the sounds--sounds that I imagined were coming from the mother of a child who was either dead or dying in front of her mother's eyes--I grabbed her and fled down the hall. We eventually made our way to a quiet exam room and closed the door until we received word that it was safe to return. There we sat in a long, quiet embrace, trying very hard to stop hearing that voice, and to stop imagining what might have been happening.

It sounds--and certainly felt--selfish to think about our own well-being when it was abundantly clear that our neighbor was actually experiencing something horrible; we simply wanted not to observe the horror. But under the circumstances, where we spend all day every day trying not to obsess over the possible significance of every vital sign, symptom, and test result, it was hard not to wonder if whatever was happening next door could also happen to us. Could Sam's condition turn south in a single moment like that? And if it did, how we'd react. Is that what I will sound like if Sam dies?**

We have since learned that the patient had a seizure--presumably unexpectedly--but is fine. She has been moved to the ICU, her old room now occupied by another patient. We've recovered from the shock and alarm, though we won't soon forget it. There's no question that the mother won't either.

Sam slept through it.

* You might expect people to want to bond with one another, to commiserate and empathize. After all, we spend hours, days, and weeks here enduring very similar kinds of hell. The hospital does arrange social gatherings for families, presumably for that very purpose (Sunday night pizza in the Ronald McDonald Family Room), but we've never attended. And if our own observations are worth anything, not many parents do.

** I no longer spend much time wondering if Sam will die. I don't know if we will escape from this entirely unscathed, but death now seems pretty unlikely. I used to, however, and I did sometimes fixate on what my precise reaction, both emotional and physical, would look like. Would I cry out? Run? Grab Alyson and collapse into a sobbing heap? I suppose there's no way to know what I will do, but if that little girl dies, I have a pretty good idea what her mother will do.

Day +14: Engraftment!

Sam, sporting camo-colored Sea Bands (which are supposed to reduce nausea*) and an unintentionally matched Where the Wild Things Are shirt.

Two weeks since the transplant and 28 days since the transplant process began, we are seeing good progress:

• The chemo side effects, including the associated discomfort, seem mostly to be gone. We're trying to reduce Sam's morphine dose so that he remains comfortable but not dopey. (Against the odds, he seems to have kept most of his hair.)
• Sam's ANC, a measure of white blood cells that fight infection, is increasing very rapidly. By design, it was slowly reduced by the chemo from about 6000 to nearly zero. At its nadir a week ago it was 40; today it's above 5000. Sam has months before his immune system will be at full strength, but he's off and running.
• Because his ANC has been above 500 for three straight days, he has reached an important milestone: the donor cells have officially "engrafted"--made their way to Sam's bones and started producing new blood cells.
• Although he gets fevers every few days (and is promptly medicated and tested for infection), Sam has yet to test positive for anything except the common cold.
• Near as we can tell, Sam does not feel any discomfort from the fractured vertebra.

Several big questions won't be fully answered for weeks or months:

• Sam's donor was perfectly matched, but how good and permanent is the engraftment? The first of several ongoing "chimerism" tests has been sent to a lab in Minnesota, results to be returned in three or four days. The eventual goal is for the ratio of Sam's blood to donor blood to be heavily weighted toward the donor--evidence that Sam's blood comes from the donor's marrow and not his own (i.e., good engraftment). 
• Will he get graft-versus-host disease, and how much?
• Will we successfully avoid infections while he's still so compromised?

Meanwhile, life goes on...

Mom reading Sandra Boynton's Barnyard Dance! to a possibly alert audience.

* No damned idea how.

Day +10: Back to the minefield

Yesterday reminded me of the earliest days of this little adventure, when I was still reeling and taken aback every time there was yet another complication or side-effect to Sam's condition.

Sam is still on oxygen--has been for about a week. He's on the lowest possible amount, and only needs it sometimes, but he hasn't been able to kick the habit even though the assumed cause of the problem, his head cold, seems to be fading. Docs have not perceived anything with the stethoscope or chest x-rays, so they gave him a CT scan.

They saw nothing of concern in Sam's lungs, but they did see something wholly unexpected: a compression fracture of one vertebra (T-8). Given his size (12 lbs.) and understandably sedentary lifestyle, the only plausible explanation we've been given is that Sam's bones have been weakened from the steroids he's been getting on and off for three-plus months, when they were used in "massive quantities" (our doc's words) to fight off the HLH and keep it suppressed before the transplant.

Sam is not evidently in pain, and there's not much to do about the fracture*--we don't need to put him in a brace, subject him to surgery, restrict his movement, hold him only in certain ways, etc. The docs don't seem alarmed by the fracture, but they are going to start increasing his Vitamin D and calcium intake and try--or at least hope--to minimize Sam's exposure to steroids.

Even so, we still have no idea how it happened or if it's going to happen again. And, if it does, how or if we'll know it. It may be true that weak bones are an expected side effect to prolonged exposure to steroids, but that is only minimally reassuring, if at all. And no matter what we hope to do, it's unlikely that we'll be able to forego steroids, which are a big part of the post-transplant regimen. So Sam will continue to be fragile (but how fragile?) for the foreseeable future.

Fortunately, the news is not all bad. The mucositis does not seem to have gotten worse, and we're not seeing any of the other chemo side-effects. It's obvious that Sam doesn't feel well, but he sleeps a lot and doesn't have to suffer through it much.

Even better: today's lab tests showed early indications that his body is gearing up to produce blood again--i.e., that the transplant is taking hold. This is exciting news, but it also comes with a price: if engraftment occurs too quickly, Sam may experience "engraftment syndrome," whose symptoms (fever, rash, fluid retention) aren't serious, but which would be treated with medications that come with their own risks: diuretics, which are starting to take a toll on Sam's kidneys; and, yes, steroids.

In short, we are now treating the treatment and fighting the side-effects as much as the original diagnosis. Or, as my mom put it, "walking a tight rope."

* I had a compression fracture of two vertebrae when I was 20--skiing accident. I was taken to the hospital, given a shot of Demerol (which I highly recommend), and sent home the same day, presumably with a prescription for something less awesome than Demerol. That was it. There was (very) slight lingering pain and stiffness for a few years, but nothing more.

Day +7: Rock bottom?

No, that's not another reference to Sam's butt. It's a reference to the fact that Sam appears to be getting into the worst of the chemo's side effects. He had a terrible night last night. As Alyson described it, Sam was "either crying or sleeping, and not much sleeping." The discomfort is assumed to be from mucositis--sores in his GI tract that make sucking, eating, and pooping painful.

His morphine dose was increased, and this has made a big difference. He's been sleeping most of the day, and his awake time--what little of it there was--was peaceful if not sunny. We are not thrilled that he's back on opiates given the difficulty we had in late July, but it's worth it if he's comfortable.

Sam had a slight fever again this morning, which prompted another round of blood cultures, tests to see if he's viral (results still pending), and prophylactic antiobiotics. He's also got a half dozen ball bearing-sized bruises around his body which staff are at a loss to explain definitively. The bruises may be related to Sam's depressed platelet levels (or some other problem with his blood's ability to clot), but that's just a theory at this point; we're keeping an eye on 'em.

On the plus side, his oxygen saturation is back to normal (the cannula was turned off and removed from his face), and the cold he's been fighting seems to be improving; he's less congested in the head.

And it's worth noting that if Sam is getting into the worst of it--if he's actually hitting rock bottom--then we're that much closer to getting out of the worst of it.

Tonight is my night to sleep at the hospital. After Alyson and my mom went home, I walked over to Columbia Heights to find dinner and be outside for awhile. On the way I passed a place advertising the following deal:

Somehow I mustered the strength and willpower to keep walking.

Day +4: Sam's butt and other important topics

Sam's diaper rash is now completely under control, thanks to a laborious butt-care regimen started and described 10 days ago. In fact, his little tuches looks so good that he's famous around the floor.

Sam's hind quarters during re-application of skin protectant. (It's not just my eyes that he inherited.)

In other, less salacious news, Sam tested positive for rhinovirus (essentially the common cold) the day after transplant. He's congested and has a cough, but the doctors are unconcerned--pretty ironic given all the concern expressed about preventing infection while Sam has no immune system.

His weight is holding steady at 5.4kg. This is also a good sign--it means he's not retaining fluids as much as he was a few days ago, when his weight had gone up to 5.7kg. His BP vacillates between normal (90/60) and not-so-great (125/70), but hypertension isn't unexpected under the circumstances*. He struggles to keep his oxygen saturation above 95% (the congestion?), so he continues to wear the oxygen cannula.

And, yes, he still has hair. His ANC is continuing to drop, however, which means the chemo he stopped getting a week ago is still working through his system and will eventually reveal itself in the form of unpleasant side effects, like mucositis and, presumably, hair loss.

Sam is comfortable and in good spirits. When he's awake he's nearly always happy and smiling. He loves the mobile above his bed, which he watches very intently.

Down for the night. Or at least until he coughs himself awake.

I suppose all the attention given to vital signs, etc. is tedious to read about, but it's nothing if not illustrative of our day-to-day: lots of navel gazing.

* The downside is that the diuretics he's getting to fight fluid retention and hypertension are leaving calcium deposits in his kidneys that might eventually lead to kidney stones.

Day +2: Pix of Sam

Sam, going nowhere fast. The sock helps keep the oxygen saturation sensor affixed to his foot...but only a little.

Sam and a grandmother.

Sam and another grandmother.

Day +1: Transplant complete

Sam's transplant happened yesterday on schedule, five months after his birth and three after his diagnosis. The donor cells arrived from San Diego early in the morning, and by 11:30 he began receiving the first of two 30ml transfusions. Sam's nearly always being infused with one thing or another (chemo, steroids, anti-fungal meds, anti-bacterial meds, meds to ward off graft-versus-host-disease, diuretics, hypertension meds, blood products of one type or another) so the transplant was, by appearances, sort of a non-event--just more stuff in a syringe being pumped into Sam's little body.

Mom, Dad, Sam, and 30ml of stem cells (in my left hand).

At about 3:30 the second empty syringe was removed, the catheter flushed with saline, and the procedure we'd been looking forward to with both hope and anxiety--a procedure that will either save Sam's life, or won't--was over.

Sam took it well--no fever or other adverse reactions. He then had a bath, his fourth wardrobe change (he may have the makings of a new immune system, but he still doesn't have any bowel control), and a snack. At about 6:30 we went home for the night, leaving Sam in the very capable hands of one of our favorite nurses.

Sam and Mom, transplant in progress.

Alyson and I then went out to dinner to celebrate the milestone and reflect back on how much has happened since June. Things have gone well, relatively speaking, and we're obviously hopeful that our good fortune will continue. There's not a lot to do now for the next few days, weeks, and months but fight off infection; hope the stem cells engraft and start to create a new, properly functioning immune system; hope for minimal graft-versus-host-disease; and, of course, wait to hear that the HLH is indeed gone, gone, gone from Sam's system--in other words, that he is cured.

This morning we received news that Sam had become slightly febrile and that the oxygen saturation in his blood had dropped enough to warrant a return of the dreaded (for Sam as well as anybody who's in the room and has to listen to the beeping monitor) oxygen cannula. Staff are not concerned--reactions like this are common after transplant. The fever's now gone, but Sam is still wearing the cannula. We hope it won't be there for long.

Sam's been a bit more subdued today, but he had some good smiley time and has yet to show any significant chemo-related side effects. The nurses occasionally have a Who has the cutest patient? contest. Sam is a repeat winner.

The hospital: it's easier this time*

So far, this admission seems to be a lot less difficult than the first one. I think there are several reasons.

First, we are no longer in shock, and no longer worried that we might lose our newborn son in a matter of days. Back in June, Sam's fate felt, and I think was, very uncertain. (In the days after his diagnosis, I told my dad and brother, without any melodrama, that they if they wanted to meet Sam--ever--they shouldn't wait to plan a visit.) We still have a long way to go, but I certainly don't feel that Sam's situation is as tenuous as it was then.

It's true that we are months from being out of the woods, and that we still have to make it through a dangerous procedure that not all patients survive. It's also true that if the donor cells don't engraft properly, the HLH could return and cause a lot more damage to Sam than it did the first time.

Sam and Alyson, not long before the former shat on the latter.

But we're no longer reeling. We can see light at the end of the tunnel. (It's a long tunnel, but there's still light down there.) Moreover, Sam is in very good shape going into transplant. The HLH appears to be effectively dormant, and Sam has a perfectly matched donor. Whereas the doctors used to be very cautious about his prognosis (HLH is a humbling disease, they said), they are now upbeat and confident about his recovery. The future simply looks a whole light brighter than it did in early June, when we had no idea how this would really play out, but plenty of dark thoughts about how it might.

We're also more comfortable. The hospital is a dismal place to spend hours upon hours, but we've adapted a bit. We bring more food from home instead of scavenging through the hospital cafeteria's frequently dismal offerings. The big chair--the one we use to hold and feed Sam in--is better, more comfortable, than the last one. It rocks, and when it's in the reclining position it doesn't try to close up like a clam shell. Maybe best of all: since Sam has not needed to have his respiration, heart rate, and oxygen saturation monitored 24/7, we are not tortured by loud and seemingly relentless monitor alarms. None of these things is debilitating on its own, but in concert they were exhausting to cope with precisely when our coping skills were under severe duress.

Finally, Sam. When he was first admitted in June, he was still more or less a blank slate. He had just started to smile and make eye contact, but did neither very reliably. We were still sleep-deprived and felt very much lost at sea vis-a-vis our parenting skills. Sam consumed more of our energy than he gave back. Now, 12 weeks later, he's all smiles, and that makes us smile and laugh--a lot. We now feel a lot of joy being with Sam, and that makes spending hours and hours trapped with him in a hospital room a bit less dismal. (I'd give anything to be able to take him outside for a walk, but that's never going to happen.)

The next two or three weeks are likely to be pretty difficult, but I'm hopeful that we'll be in better shape to handle it all than we were earlier this summer.

P.S. Fun bone marrow transplant fact: Sam's original blood type (O-pos) will change to the donor's type, A-pos.

* But it still sucks.

Day -4: Status quo

I keep waiting for a shoe to drop, for the chemo side-effects to kick in, but nothing's happened so far. Sam's "counts" have not started to drop, so although he's received 10 of 11 doses of immuno- and chemotherapy, we've yet to see the effects--well, except for the two times he's made diarrheal donations to Alyson's lap.

It's not like we haven't been told how this plays out: it won't get ugly for Sam until the chemo's had some time to work. I just keep hoping that Sam is somehow defying medical gravity: He had chemo and never had any side-effects!

All of which is to say that we are doing well, if somewhat in denial about what's still to come.

Alyson and I stayed at home last night. We'd been taking turns sleeping at the hospital, but since Sam's been comfortable and sleeping well lately, the costs to us (a shitty night's sleep) are greater than the benefits to him. I think as long as that's the case, we'll try to sleep at home.

Day -7: Holding steady

Sam's now had two doses of Fludarabine (chemo) and seems no worse for wear--no mouth sores or nausea or diarrhea or other standard chemo-related unpleasantness. The sailing has not been entirely smooth, however.

He's had high blood pressure due to steroids and all the fluids he's been getting, but thanks to a combination of things that seems to be improving*. Sam occasionally gets a fever, but it's nearly always low-grade and short-lived. More important: none of the blood cultures they run when he's febrile has shown any sign of infection.

Sam's put on more than a pound in the last week, due mostly to fluid retention. He's kinda puffy and bloated-looking these days, but the staff don't seem concerned by this except as it relates to the hypertension.

Bath time? Awful. Cuddly after-bath towel time? Not so bad. 

In short, he is holding steady. Whenever the water's been a little rough, the staff have prescribed or tweaked something to get us back to calmer waters. (OK, enough with the damned boating metaphor.)

The only other real struggle Sam's had is with diaper rash, and it's been painful for him and anxiety-producing for us: we don't want him any more uncomfortable in the coming days and weeks than necessary, and we definitely don't want him extra vulnerable to infection.

Fortunately, a change in Sam's butt-care regimen seems to be doing the trick. We used to rub a dab of standard-issue zinc oxide-based butt paste on his rear to prevent diaper rash. Now we slather on specially medicated, house-made paste (his ass looks like a newly frosted cupcake when we're done); place sterile, non-stick Telfa pads on each gooey cheek so it doesn't stick to the diaper and have to be peeled off during the next change; adhere 1/3 of a sanitary napkin to the inside of the diaper to increase absorbency; and, every 2-3 days, apply a liquid skin protectant** after his bath. The extra effort seems to be working, and that's great, even if the diaper change protocol now warrants its own instruction manual.

In other news, we've moved into a new room. We are now completely within the BMT Unit, behind two sets of secured double doors. The old room was squeezed in between the two sets of double doors--it was loud and echo-y. Even better: the room has a fridge and a recliner/rocker that's waaaay more comfortable than the ones we had during Sam's first hospitalization this summer. The "little" things make a big difference around here.

=======

* Average recent BP had been 125/70--not terrible, but neither normal nor desirable. In the last day or so we've seen a few hit the 100/65 range, but there's lots of variation, and it's complicated by the fact that it's evidently damned near impossible to get consistently reliable readings on an infant.

** Funny thing about this stuff: it goes on sticky and stays that way until it air dries. Which, as you might imagine, could lead to some interesting scenarios if you've applied it to an infant's spread-open butt cheeks and fail to let it fully dry...

Day -11: So far, so good

We are four days in. Sam has "spiked" a fever a couple of times, but except for that and diaper rash is in good shape and handling things well. The real nastiness and discomfort are likely to begin next week when he starts getting real chemo. (The Alemtuzumab he's getting now is actually immunotherapy, not chemo per se, and it doesn't wreak as much havoc with the body as chemo does.)

I'd planned to describe our routines in some detail, but I feel like I can barely put two words together today. Suffice it to say that life is pretty monotonous--the hospital room is about as comfortable as a waiting room, and only marginally more stimulating. Sam is not allowed to leave the room--can't risk getting him infected through exposure to the outside world--and he's often hooked up to one or more catheters. If he's connected, he can't travel more than six feet from his bed. If he's disconnected he can move about the room. Today when he was unhooked I took him for a walk around the room...over and over and over. Until we were both bored stiff.

Bath time is not Sam's favorite time.

Physical therapy: too much lying around isn't good for a growing boy.

And away we go...

Sam was readmitted to Children's Hospital's Bone & Marrow Transplant unit yesterday. Not much actually happened, so there was an awful lot of sitting around and wondering why we'd been told to show up at 2pm. (Alyson's answer: "So we could go home at 8pm instead of midnight?" Can't say she didn't have a point.)

Aside from the obvious, Sam is in very good health, and several people have said complimentary things about he looks. We are happy to hear those things. We were also excited to see that the feeding tube, which we'd been using to supplement Sam's breast milk diet, did its job. Sam clocked in at a robust 11 lbs, 3 oz, two pounds more than he was a month ago.

The pre-transplant protocol began today, otherwise known as Day -14. Sam had his PICC removed and had a double-lumen Broviac installed on the right side of his chest. This is how many of his medications will be administered. To our surprise, some of his chemo will be administered "subcu"--that is, as an injection. Talk about adding insult to injury!

Here's how the next two weeks of pre-transplant chemo will play out:

Day -14 thru Day -10: daily doses of Alemtuzumab
Day -9: Rest
Day -8 thru Day -4: daily doses of Fludarabine
Day -3: Melphalan
Day -2 and -1: Rest
Day 0 (Sept. 16): Bone marrow transplant

Sam's hair will probably start falling out around Day -10. The first couple of weeks after transplant (aka Day 1 thru Day 14) are likely to be the most uncomfortable for him, as all of the chemo side effects will be at their height. Those first few weeks will also be the most dangerous and anxiety producing. Sam will have no immune system--no defense against bacterial, fungal, or viral infections--and we won't know yet if engraftment has begun. On top of all that, he'll be uncomfortable and unhappy. This transplant will potentially cure Sam of his HLH, but we won't know for months if that's actually the case.

On the home front, Sam's absence means that we no longer have to spend so much time taking care of him ourselves--administering meds and washing syringes; flushing the PICC; sterilizing water and mixing formula for the supplementary feeds; administering the feeds and washing the hardware. We can sleep and eat without interruption, and our time here at home is otherwise all our own.

But it's quiet here and kind of lonely. We miss him.

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.