Day -19: Finishing Up Campath

Zoe has really impressed everyone around here, including her Mom and Dad, with her strength this week. We’re nearly done at this point, she’s half way through the last dose of Campath, and barring any problems it should be her last.

After yesterday’s unusual reaction, we’ve had a few more folks than usual pop in to take a look at her. It’s not everyday they see Harlequin Phenomenon, among her treatment team only her primary physician had ever seen it before as best we can tell. For the record, it is a harmless phenomenon, and it is not the same thing as Harlequin Syndrome, which is an unfortunate condition indeed.

Today’s counts look good:

[spoiler name=”Click to see counts”]

  • WBC: 4.5
  • Hemo: 10.5
  • Platelets: 22
  • Creatinine: 0.2
  • Bilirubin: 0.3
  • AST: 78
  • ALT: 48
  • Lymphocytes: 0


Because of her platelet count, Zoe will get a transfusion at the end of the day to get her through to Monday. This is not unexpected, the Campath knocks down the platelets along with her Lymphocytes. We’ll be checking in on Sunday to be sure the transfusion does what it needs to.

Next week we’ll be in 3 times for counts, but otherwise it’s a “break” week. Zoe will be on Hydroxyurea to keep her immune system suppressed until the following week when we start Fludarabine and Melphalan, at which point we’ll be admitted in the lead up to the transplant.

It’s been an amazing week. A relief to be starting treatment, sure, but more than that a relief to see Zoe handling everything so well. We still have a lot of anxiety about things, but it’s different now.

Often the anxiety in anticipation of the journey is greater than once the journey has started, and I believe that’s how we’re feeling right now. Glad to have started toward Zoe’s cure, and optimistic that we’ll reach our destination safely.

Afternoon Update:

Without even a whimper, we’re done. Things went so well I’m waiting for something to happen, but at this point there is little left that could. Very proud of Zoe, she’s weathered the week better than we could ever have hoped.

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.


Last Clinic Visit

We had our last clinic visit before the transplant yesterday. It was all together fairly uneventful, which of course these days is a good thing.

Zoe had her last dose of VP-16 or Etoposide, and had already taken her last dose of the steroid, Dexamethasone, on Wednesday. This brings her med count way down, since she has been able to cut one of her 2 blood pressure meds and the Zantac she was taking to prevent the steroid from tearing up her stomach. Morning is 3 meds, evenings 2, down from 9 in the morning and 7 in the evening at her peak.

She will of course have to take other medications post transplant, but it’s nice to see her have a break. She is such a good baby, so calm and rarely fusses much, usually in a great mood and prone to smiles and laughs whenever it’s not clinic day. Letting her just be is really wonderful.


  • WBC: 4.9 (slightly low)
  • Platelet: 474k
  • SEG Neutrophil: 1000
  • Sodium: 130 (up a bit finally)
  • Ferritin: 443 (best count since before the disease became active! Normal in healthy children is 100-200)

Generally looking really good going into the transplant countdown. WBC could be higher and was last week, but she had a bit of a cold or allergies last week, so perhaps the change is due to that.

Next week we start the pre-transplant work up at Duke.

Ups and Downs

One thing we’ve come to understand about this disease from reading others’ stories is, you must be prepared for the ups and downs. It causes a lot of havok, and can generate swings in the body’s natural function.

We have been fairly lucky so far with Zoe, she has weathered it well all things considered. Here’s hoping that will continue.

Last week Zoe’s immune count marker (SEG Neutrophil) was up to 2.0 or 2000, this week it’s back down to .2, or 200. Not a good swing for us.

So this week we’re back in huddle mode, windows closed, no going outside for her to enjoy the beautiful weather until she has rebounded again. Dr. W felt it was likely due to the chemotherapy regimen, but we are wondering if she is not also struggling with her first pollen season.

In retrospect, it was probably a bad week to spend much time out, pollen in our region is still fairly heavy.

Vitals this week:

  • WBC: 3.1 (5.0+ is normal)
  • RBC: 2.83 (3.1+)
  • Hemo: 8.6 (9.5+)
  • SEG Neutrophil: .2 (1.0+)
  • Platelets: 250 (150+)

Rolling with the punches here.

Hopefully next week will be back to where we were last. We’ll have more to report about the outlook for her Stem Cell Transplant soon.