Post-transplant Work-up

Today was Zoe’s 100 day post-transplant “work-up”, which means we shuffled through a long day at the Duke Children’s Hospital clinic subjecting Zoe to the same battery of tests that she received before her transplant. She had an echo-cardiogram, the “scream” test for pulmonary function, full blood draws and x-rays. We did not have to have another CT scan, nor did we have to do the nuclear kidney/liver function test. Zoe’s Creatinine and Bilirubin (markers of kidney and liver function) give us no reason to believe she isn’t recovering or fully recovered from her cyclosporine treatments.

The good news is that everything came back just fine so far. Zoe is a fine screamer, her pulmonary test came back better than it did pre-transplant, her xrays looked fine, and her blood counts were almost all in the normal range. The great news is that she did not need “G”, her WBC count is up to 5.6 on it’s own. She’s gone 2 weeks now without a boost, and the counts went up not down. We’re optimistic this means she’ll never need it again.

The visit felt very much like a cap to our experience at Duke. Most of the same tests, only this time with an eye toward making sure everything is fine so we can go home, rather than being fine so that she can be admitted and subjected to chemotherapy. The feeling was entirely different.

Zoe still has a bit of thrush, but given her newfound love of spitting out her medications, we decided to pursue the nuclear option to clearing it up. They gave her a purple dye-like medication that coats the inside of her mouth and throat and suppresses the thrush. The upside to this is it only takes 3 applications over 3 days, the downside is that it is really strong dye.

It’s permanent on pretty much everything except skin I’m told, so Zoe has to wear a little bib for the next few days, and everything she puts near her mouth will end up purple. As we were finishing up the application of it, the nurse lifted Zoe up to a sitting position a little too quickly and she spit up a bit. In a heroic attempt to save her clothes from the purple menace, I caught the spitup in my hand, and was rewarded with a purple hand for my troubles. The dress still didn’t make it. After that, we put on her bib and I gave up any hopes of joining the secret service.

There was a bluegrass band playing in the lobby for the duration of our visit, giving an interesting soundtrack to our Family Circle trek all over the hospital for our tests. There were definitely more smiles than usual everywhere we went, as a result. All in all, a great day.

Day +75: Finding a Natural Weight

As most of you know who have checked in with us at one time or another, Zoe has been on steroids for most of her life at this point. She gained a great deal of weight pre-transplant due to them, and has since retained a decent amount of it. Her percentiles for her age and size put her in the ~90%, meaning she is larger than most babies her age and length.

We’ve been slowly creeping back down from that as she started to grow again and become more mobile, she seemed to stop gaining height for a couple months during the worst of chemo and steroids. Now, she really only retains her steroid cheeks, and even those are in slow decline finally.

During this process we’ve had a heck of a time getting her meds right, as we’ve moved from IV nutrition to milk and solid foods. Zoe’s weight has gone down, her Tacrolimus level (immuno-suppressant chemo) has shot up, and her Magnesium has dropped considerably. Last week and this week have been all about trying to get her eating, trying to figure out if her Tacrolimus readings are “real” or an error of the test, trying to get her Magnesium back up.

Where we are at the moment is, Zoe’s weight is around 8.2 kilos, which is pretty close to where she should be for her size, around the 50th percentile. I’ve come to feel that she’s trying to find her “natural” weight, the weight she would be were it not for all of this mess she’s had to endure. She seems to be much spunkier than she was even a week ago, and feel more comfortable moving around. She sits up easier, rolls on her side easier, and holds herself up on her belly with ease. All part of growing up, of course, but the change seems to be related to the weight. We can feel that she’s lighter. She seems to feel it too.

Zoe’s Tacrolimus level has shot up very high, and they’re inclined to believe it is accurate now — 1800, where normal is 500 for her. This can cause all manner of problems if it continues, so we’re going to suspend it for now, until we figure out the new dosage. Her Creatinine is up as a result, and because the Tacrolimus leeches Magnesium, that is very low. She’s getting supplements of Magnesium now 3 times a day, but she has gotten so proficient at spitting it out that we are struggling to get the levels up.

nom nom

We’re crossing our fingers that all of this settles out once her weight stabilizes. She’s eating pretty well, but the change in diet has definitely wreaked havoc on her med situation. If we can get it figured out without any seizures from too much Tacrolimus, or GVHD from too little, all will be well. We’re a little anxious, however, since those two situations are a possibility at this point.

On the bright side, she is eating solids quite well, her spirits are high, and her personality is really starting to shine through with little happy noises, laughter and cute little eyebrow maneuvers whenever she’s listening to us speak.

Oh, and she’s cutting 3 teeth now.

Day +11: Happy Fourth!

Zoe is doing well today, much improved from yesterday I would say. She is sleeping the day away, and that’s probably for the best. Whenever she is awake she has a hard time getting comfortable, and she has a hard time being held for very long, so it’s not easy to console her.

No uptick in her WBC counts today, sad to say. Everyone here is confident she’s begun engraftment, however. She’s still flushing regularly, skin very warm to the touch at times, and her temperature is staying around 99.5.

We did up her pain medication late last night after having a hard time getting her settled. She is set up on a PCA machine to automatically dose the medication, with “bumps” of it for rough patches if needed via a button. Zoe doesn’t react well to the button, however, she gets very itchy at the sudden higher dosage, then it fades and she is in pain again, so we tend to increase the drip instead. It’s a smaller amount than the button, but it keeps her more at ease when it’s consistent.

Still Cheeky

Zoe’s creatinine came back 0.1, which is suspiciously low given yesterday’s 0.6. Dr. S felt it unlikely that’s accurate, but it does seem likely that it has gone down not up, so we’re ok there. She continues to respond really well to the Lasix, so her weight is stable.

Which is all a long way of saying we’re holding steady. All is well, she’s doing what she’s supposed to — growing a new immune system.

Have a great holiday!

Day +10: The Worst of It (We Hope)

Things are a little tense here at the moment, we’re trying to get through what we hope is the worst of the entire process. We’ve been told for awhile now that around Day +10 we’d see the worst of the mucositis, the worst of the post-chemo struggle, the worst of the fluid retention. That seems to be where we are now.

Zoe's Lounger

Doctors are still feeling very positive about Zoe’s progress. She’s handled everything really well, and her mucositis has not been as bad as it could be by any stretch. She doesn’t have any sores in her mouth right now, just redness and irritation.

She is definitely uncomfortable though. Her pain medication (Fentanyl) has been upped a little more to help her get through this phase, and that has helped with both her blood pressure and her comfort.

The primary concern at this point is the fluid retention. Zoe is noticeably puffier, and we’re trying to let her spend as much time as we can in an upright position to make sure fluid drains well and she can breath without supplemental oxygen. Her oxygen saturation levels are still good, so we are keeping fingers crossed she’s not going to need a tube.

Zoe’s Creatinine, which is a marker for kidney function, has gone up to 0.6, which is significantly up from the 0.2 or 0.3 where it’s been stable these last weeks. We are assuming that that is because of the Lasix she is getting for fluid retention, but we haven’t heard confirmation of that just yet. There are other options for fluid retention that are less hard on the kidney, so we may see a switch to one of those today. We’ll know more later on, we tend to see the docs around 4-5pm most days.

If this is the worst of it, and it is the worst so far at least, then we will be relieved. If on the other hand things continue to get worse as they have these last few days, then we’ll be more nervous. I know from reading the stories of other families that getting through this period is really hard but that it does end, so we’ll just try and keep our eyes on that end of the tunnel.

Almost there.

Update: Feeling a little calmer after a visit from Dr. S. He was excited when he saw her, where I have been very nervous. Turns out he feels that her current condition is the beginning of engraftment — fluid retention, pink-ish flush on the skin. This is a very good thing if he’s correct, and I trust he is. He was not concerned with anything, he felt Zoe doing well since she does not need oxygen at the moment and is flushing out the fluids well so far.

Flushed & Feeling Yucky

Fever rash engraftment, fever rash engraftment — say it as fast as you can, try to keep that in mind”, he said.

We’re likely to see fevers, which can be quite severe, and skin rashes which usually start on the hands if she has them. The rashes are signs of GVH, or graft vs host, and are expected to some extent. There are multiple variations of GVH, most of them mild.

So. We’re upping her Lasix to 3x a day to keep ahead of the fluid build up, we don’t want to “fall behind” on that I’m told. We have canceled her antibiotic, however, since her line cultures are all negative and that medication is contributing to her higher creatinine.

The nurse coordinator who brought us in and took us step by step through the first stages of this stopped by yesterday, it was good to see her. She’s been following Zoe’s numbers each morning, and she was very encouraging.

As she started to leave after we had spoken for awhile, she turned and, smiling, said, “Don’t hold me to it, but I think you might get a little July 4th present. I think we might see a tick up in Zoe’s WBC count.”

Let’s hope she’s right!

Day -1

Well, we made it through Thiotepa. Michelle deserves most of the credit, she had it rough last night with baths and gowns and Zoe refusing to eat out of the bottle. We contemplated having me stay over to help, but technically we’re only supposed to do one person in the room, and with Maya in town and expecting us we thought it better to stick to the plan. Still, kudos to her for getting through what had to be a rough night.

Zoe had her last bath late morning. The baths involve wiping down her skin really thoroughly with damp gauze pads to remove traces of the chemical being exuded through her skin. I didn’t realize this yesterday when I mentioned it, but apparently leaving it on or allowing it to accumulate can cause 3rd degree burns. As such we’re very careful to clean her armpits and little baby wrinkles where moisture accumulates.

Sleeping it off

But, it’s done now. This was anticipated to be the worst day of conditioning, and it was. Zoe is a trooper though. She didn’t like the wipe-downs one bit, but as soon as they were done she would calm down and resume her normal chipper demeanor. We’re very lucky.

Today we simply continue her maintenance meds and get ready for tomorrow, Day 0, transplant day. We’re bringing Maya and my parents in to cheer her on while she gets her transplant since they’re local, so that should be a nice occasion.

A lot of nerves here, but a lot of hope too. We are tense because it’s all come down to these next couple of weeks. We’re ready, but scared for Zoe. It’s a big deal, getting a new immune system. The vast majority of people go their whole lives having no procedures so major as this, and here she is plugging along at 5 months old for it. I find it pretty amazing.

Late update:

Well, as the day wore on things became a little more tense. Zoe’s pain got worse through a combination of the mucositis (from the melphalan), the soreness from blisters on her bum from Thiotepa, and probably some lingering aches from surgery on Friday. In general she had a pretty rough afternoon.

We did get a PCA device set up for her, she’s part of a study that allows parents to administer small amounts of pain medication (dosage regulated and on a timer) when they see their child needs it rather than have to wait for a nurse. It’s basically an IV that can’t be adjusted, but which will dispense a small amount of medicine when a button is pressed to stave off pain. It works wonders.

From here on until she recovers it will be a little bumpier than it has been. We made it through most of her conditioning with little impact to her in terms of discomfort, but now things start in earnest.

[spoiler name=”Day -1 Counts, for the record”]

  • WBC: 0.4 (will likely be “<0.1” by transplant time)
  • Hemoglobin: 10.6
  • RBC: 3.67
  • Platelets: 56 (she’ll get a transfusion tonight more than likely)
  • Creatinine: 0.2 (good)
  • Bilirubin: 0.5 (good)
  • Sodium: 140 (no longer an issue)
  • Lymphocytes: 4% (The way this was explained to me is, she may have a few going into transplant but they are neutralized and cannot multiply. They will slowly be knocked out in the next few days and will not be an issue.)