bilirubin

Post-transplant Work-up

/ Evan / 4 Comments

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 -1

/ Evan / 5 Comments

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.)

[/spoiler]

Day -20: Campath Poster-Baby

/ Evan / 4 Comments

The evening went well, Zoe slept very fitfully through the night. We saw a little bit of rash again this morning just as she was getting her Hydrocortisone pre-med, which may have been coincidence since Hydrocortisone usually treats rashes, not causes them.

We’re told Zoe is looking more and more like a “Poster-baby” for

Lucky the fish

Campath, her reactions so far are the mildest most of the staff have seen. I’ll take it.

Today is expected to go just the same as yesterday, no changes to treatment. If we finish out the week tomorrow without anything else eventful, we can breath a big sigh of relief for our first successful week.

Morning Counts:

  • Creatinine: .2 (down from yesterday, which is good)
  • Bilirubin: .3 (unchanged)
  • AST: 78
  • ALT: 36
  • Sodium: 141
  • WBC: 11.5
  • RBC: 3.38
  • Hemo: 10.2
  • Platelets: 111 (down from the campath, we may hold her hydroyurea if they get too low)
  • Lymphocytes: 0

Very quiet today so far. Our appointed mascot, Lucky the fish, highly approves.

Afternoon Update:

Once again, we made it all the way to the end of the day before seeing any reactions. Zoe is having a bit of a flush this time, in what you might call an unusual pattern. Her entire left side of her face is red, with a line right down the middle where it ends.

A couple of NP’s came in to take a look, and they hadn’t seen a rash quite like this before. But, it doesn’t appear to be bothering Zoe much, she’s a little squirmy but vitals are fine.

This reaction is definitely curious. I promise we didn’t leave her in a tanning bed on one side too long, but that sure is what it looks like.

Still, one of the NP’s said he’d never seen such a happy Campath baby, so I guess all things considered we’re doing ok.

Late update: It’s called Harlequin Phenomenon, and we’re told it’s harmless.

Day -21: Campath Full Dose

/ Evan / 3 Comments

Our second day of conditioning brings the first full dose of Campath, and a much higher chance of a reaction. So far this morning we’ve done her pre-meds with the addition of Dexamethasone as the steroid, and begun her Campath. The chemo is still administered on a stepped schedule, a small dose, then a slightly higher dose, then the full amount for the remainder of the day.

Zoe’s counts look good:

  • WBC: 18.2 (higher)
  • Hemo: 9.9
  • Lymphocytes: 1 (her partial dose almost completely wiped them out — this is intended)
  • Sodium: 138
  • Creatinine: .3 (up from yesterday, we’re told it will go up and down with the conditioning)
  • Platelet: 331
  • Bilirubin: .4 (still fine)
  • AST: 44
  • ALT: 21

So, no major changes other than a drop in platelets and lymphocytes, both expected. I’ll update later with any reactions and changes, she has only just begun the Campath so it’s calm at the moment.

Afternoon Update:

Everything has gone really smoothly again today.

Minor Campath Rash

Around 3pm, Zoe started some minor rashes on her face and head, no where else. She’s been very sleepy and calm all day and we were beginning to think she would plow on through day 2 without any side effects. So far, things have gone much better than we expected.

We finished the Campath and her rashes showed up just as we began the saline flush, exactly the same as yesterday’s slight fever. Nothing to be concerned about, Zoe is doing great with her treatment at this point.

Blood pressure has held steady around 100/65 most of the day, temperature averaging about 98 degrees.

So far, so good.

Day -22: Starting Campath

/ Evan / 2 Comments

We’re off and running now. Campath started today at 11:30am on a stepped dosing process, which means she gets an increasing dose every 15 minutes under observation until we reach the full test dose. The test dose will run for several hours, and is 20% of what she will receive the rest of the week.

Zoe was given pre-meds to help her manage the chemo: hydrocortisone (steroid), benadryl (antihistimine), tylenol (fever), and demerol (pain).

She is asleep, no surprise. We’re told to expect hives, rashes and fevers, so we will see how things go.

Zoe’s blood counts brought good news today, something we are grateful for on this day in particular. Her Creatinine, a marker of kidney function, is now down below .1, which is as low as can be measured. Her most recent test on Friday was .2, which means that her kidney function has improved measurably. Go Zoe!

The numbers:

  • WBC: 5.0
  • Hemo: 10.4
  • Sodium: 137
  • Platelets: 667
  • Seg Neutrophil: 38
  • Lymphocyte: 55
  • Creatinine: <0.1
  • Bilirubin: 0.5
  • AST: 37
  • ALT: 20
  • ANC: 1900

These are the numbers we’ll be following most closely from now on. The creatinine as mentioned is a marker of kidney function. Bilirubin is a marker for liver function, and it’s currently perfectly fine.

AST & ALT will be monitored to determine if we need to hold the Hydroxyurea. If they rise to 3x “normal” or mid-range, then we hold it. 3x “normal” is ~180 for AST, ~66 for ALT.

ANC means Absolute Neutrophil Count, which is a somewhat complicated calculation that measures immune system function. We can expect that to go up and down, but mostly down, during conditioning. After Zoe’s transplant this number will become our holy grail. We’ll be looking for her cell counts to go up and her ANC to rise and stay above 500, as I understand it. We’ll learn more about this closer to the transplant.

The campath will quickly reduce the Lymphocytes, which is what it is there to do. The “segs” will remain elevated and the ANC, which counts both, may therefore stay high initially. Eventually we expect it to go way down and not recover until after transplant.

Afternoon Update:

Zoe’s first day test dose went very well. No major reactions today. She had an elevated heart rate and very mild fever for about an hour, but was otherwise fine.

Because Zoe did so well, we will be giving her Dexamethasone for the next 3 days during Campath instead of the normal steroid given. This is because Dexamethasone penetrates the blood/brain barrier more effectively, and with HLH it can do more to suppress any abnormal cell activity than the traditional steroid given with RIC.

Tomorrow we continue the chemo and step up from the today’s test dose (which was 20% of normal strength) to the full dose. We expect if we’re going to see a reaction we should start seeing it tomorrow.