Day +14: 0.2

Great news this morning, Zoe’s count is back up to 0.2 after holding at 0.1 for two days. Everyone is confident that she is growing cells now, so we’re off to the races. Next step will be holding at 0.3 for more than a few days, showing some stability with her counts.

It’s been a fairly busy morning for Michelle at the hospital, she’s begun learning the nursing tasks that we’ll need to handle while we are outpatient locally. After Zoe has engrafted and stabilized, somewhere around the +30 day mark if things are going well, we will be released to our local apartment. We will need to stay there until around day +100 before we can return home, but during that time we will be doing everything that the nurses normally do in terms of dressing changes, line cleaning and blood draws, so we have to learn those things. Michelle got off to a good start today, we’ll both do more over the next two weeks.

Zoe is looking good and seems to have passed the worst of the pain, as hoped. She is sleeping a lot, almost all the time, which is as it should be. She’s growing cells and she needs it.

[spoiler name=”Click for counts”]

  • WBC: 0.2
  • Hemo: 9.9
  • RBC: 3.32
  • Platelets: 26 (she gets a transfusion most every day at the moment)
  • NK: 109 (100-1300 is normal for 2mo-2yrs)
  • Creatinine: 0.3
  • Bilirubin: 0.4


Day +8: Never a Dull Day

Each day seems like a whirlwind here, or at least, a whirlwind as experienced through a dense fog of fatigue. The emotional ups and downs and new medical events prevent it from ever being dull.

Last night was long, Zoe had her first night with the “blow-by” oxygen, which is basically a big tube that blows oxygen past her face to boost her intake. They put it in place because when she was sleeping, her oxygen saturation level would dip down to 80 or 85 out of 100, which is too low for her to continue for any long period of time.

At first I thought it was the machine, it gives very chaotic readings for babies and Zoe is a squirmer, flipping her little legs all over and wiggling, such that the readings are often off. The nurses took a look at the waves this time however, and determined that it was accurate enough that we needed to play it safe. So, we brought out the tube, which lays on the bed near her head and blows a gentle breeze past her face, but makes the room sound like we’re suddenly seated over an airplane engine. It’s about as good for a sound night’s sleep as you’d imagine it is.

Because of the need for oxygen, a whole series of tests were triggered to be sure we have an accurate picture of everything. New blood cultures, preventative antibiotics, a new chest x ray and a new dressing all so far this morning.

Still, Zoe continues to be in great spirits. She still has mucositis, and though the volume of it has gone down there is a little blood spotting it now. She will spit up, get cleaned up, and then go right back to smiling and wiggling or playing with her toys. It’s a really amazing little spirit she has, kids are so resilient. An adult who just spat up what she is each hour would almost certainly be dour and grumpy, I know I would, but here she is ready to play after a few minutes of upset.

We also had a visit from the discharge coordinator, but after a bit of confusion I realized that they check in with everyone well in advance to try and start training and set up class times. We’ll need to know how to do pretty much everything the nurses currently do for us when we leave here. I suggested they check back in with us in a week or so, it’s a little too early for us to manage all of that and not forget something.

Her CBC came back, and we’re back to “<0.1” today, so it could be that the 0.2 was a fluke. Her bum continues to heal, which gives us hope that that is the reason for the WBC count to go down a tick, but it’s really hard to say. It is very early to see any cell growth. The count is especially likely to fluctuate with cord blood transplants, so it could be the start of that we’re seeing.

All in all, we’re right where we should be, perhaps a little better off medically than average. Zoe is not needing much pain medication above what she gets as a drip, is not needing oxygen during the day, and seems to be healing a bit from the worst of the chemo.

Now, about those cells.

Day -22: Starting Campath

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.

Work-up Week is Over


Long week, but very satisfying in some ways because we now know everything about Zoe from head to toe. No more worrying about what we don’t know, now we only worry about what we do.

She’s been poked, prodded, scanned, zapped, scream-extracted, sound-tested, irradiated (twice!) and suffered no end of cheek pinches. The only thing that bothers her is her blood pressure cuff though, she’s a fantastic baby, calm through most everything.

In terms of test results, Zoe is overall in great condition. Sometimes it just seems amazing that she needs a new immune system, the rest of her is in pretty fine shape.

The three main test results we were still waiting for are back, her brain and sinus CT and her kidney function.

The brain scan was completely clear. No swelling, no “gaps”, no shrinkage, and it’s centered (which is something that had never occurred to me to wonder about). This is great, great news. I was deathly afraid that her prolonged exposure to steroids was having an effect, or the intrathecal medication (into her spinal fluid) may have had an effect on the brain, but it has not so far.

The sinus scan came back with a little cloudiness on her right cavity, which to be honest was no surprise. My side of the family has a grand tradition of sinus problems during pollen season, and Zoe has been sneezing and sniffling in recent weeks at the same time the rest of us were. They prescribed a bit of antibiotic which should clear it right up.

The kidney function test is called a GFR, and involved putting a radioactive but non-harmful chemical into her blood stream. Her blood is then drawn at 1 and 3 hour intervals (on the dot! they’re very intense about this). This allows us to know how much of the chemical is being processed through the kidneys and therefore how efficiently they’re functioning.

Zoe’s first GFR came back at 49, which is about half of what they wanted to see. There is a strong feeling from Dr. P that that number may not be quite accurate, but none of us will be surprised if it’s close. She has been on Cyclosporine for a long time now, and a higher dose than is sometimes necessary we’re told. That can reduce kidney function temporarily. She also has had high blood pressure for quite some time we think which is directly related to kidney function. We have fits because the only test she really fights is the blood pressure, she has chubby little arms and doesn’t like the squeezing, and between the struggling and the crying it’s hard to know if it’s ever right. From time to time we get one when she’s asleep, and it’s usually just fine then, if slightly elevated.

Regardless, the test came back low so we’re doing another one on Monday. We’ll know for sure then. If it truly is low, it won’t change our trajectory but it will mean possible adjustments to meds in some situations to ensure we’re not doing permanent kidney damage. From what I understand, what we’re seeing is likely temporary as a result of her meds and her kidneys should should make a nearly full recovery in time.

The rest of her tests for the week showed her to be a healthy little baby, with the exception of her immune system (HLH) and the kidney issue. Again, shew.

Next week we have the GFR again on Monday, a bone marrow aspiration and lumbar puncture on Tuesday. After that we have about a week off, then we begin her conditioning and countdown to the transplant.