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.

.