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.

Checking In

After last week’s lower immune counts we had skipped the VP-16 dose for the week, so today Michelle is in the clinic “catching up” a bit.

Zoe’s vitals are back up nicely for the most part, with the exception of her sodium and ferritin. Her sodium has been hovering at a lower than expected count, 127 (where 133-142 is normal), and the doctors are not sure why. Michelle’s sodium intake is quite low, so we’re going to take their advice and up her Gatorade (and junk food?) intakes. More bouillon cubes please!

The ferritin count is trending back upward, a worrisome sign. Ferritin if you recall is one of the markers for disease activity, so we want to see that trending down or remaining static, particularly as we look to move ahead on the SCT soon. We do not want to see disease activity right on the verge of starting her transplant process. Hopefully we’ll know more next week, they’re doing another count today and she’s getting the VP-16, which should have an impact.

There is some concern that we might be seeing the beginning of drug resistance in that ferritin reading, and the feeling is that we will need to move forward soon on the SCT if that is the case.

Feeling a bit tenuous here. Low sodium can mean hospitalization, high ferritin could be a warning sign of a return of the active disease. Better immune counts are our silver lining at the moment. I really want to see her go into her transplant with all systems in top shape.

Vitals for the week:

  • WBC: 9.1 (5-19.5 normal, this is way up thankfully)
  • SEG Neutraphil: 1.456 (also way up, a positive sign)
  • Sodium 127 (133-142 normal)


A New Hope

After a short break for Michelle and I over the weekend that included a movie for myself (Shutter Island) and a birthday party and play date for Michelle and Maya, we’re back on schedule at the hospital. It was a really needed break and I’m grateful my mother is here and willing to sit in with Zoe while we try to see what normal life is like for a few hours.

There was a new doctor on over the weekend, somewhat younger and who mentioned she had only ever seen two cases of FHLH in her career. In both cases, they found it much much harder to treat the child than they have in Zoe’s case, lending us new hope that this may not be the Familial form of the disease. The reason they are making that judgement is her age, very young to present the acquired version, but as previously mentioned she was very very ill prior to presenting, which is one way that the acquired form manifests itself. We may have our hopes dashed soon enough when the genetic tests start to come in from Cincinnati Children’s Hospital, but in the mean time it’s what we are holding on to as a way to escape this disease without further harm to Zoe.

An update on Zoe’s numbers:

  • Her weight has again dropped a bit (not a bad thing) to 13.5lb
  • WBC: 3.2 Sat, 4.3 Mon
  • Hemoglobin: 8.8 Sat, 8.9 Mon
  • Platelets: 191 over the weekend and then 217 today
  • Ferritin: relatively unchanged at 880

In general these are positive or neutral signs, though the WBC count is just under normal. Ferritin is a slow moving indicator, which is why they don’t check more often for it’s level. Platelets are steadily rising, a very good sign, and WBC and other counts are holding around normal range.

Right now we are on track to leave the hospital as of Friday assuming no symptoms reappear. We’ll be in regularly for medications and Zoe is set to begin getting a spinal tap every week for the next four weeks starting this thursday. She has to have general anesthesia each time along with the other inherent risks of surgery, so that’s a little unsettling. Every taste of good news seems to come with a bitter side dish around here.

One Tube Down & Other Notes

Zoe is down to her central line only now, down from a peak of 4 tubes to manage attached to her at all times. Her oxygen and heart rate monitor came off today, she’s been off oxygen for a couple days and is finally holding steady right where she should be. I’m sure she must feel better and we’re certainly glad she’s getting that much closer to normal.

Dr. B’s visit brought news that her IL-2 level is at 11,000, where normal is 3,000. This is not necessarily bad news, just the first level we’ve received news of so we have something to base progress off of. This along with Ferritin will offer us a way to track disease activity without a bone marrow biopsy.

We discussed our next steps, and Dr. B feels that it is in the realm of possibility that Zoe will be able to go home the end of next week or the following week. We have to reduce her steroid dosage gradually and make sure she holds up without it, and obviously ensure other symptoms do not reemerge in the meantime. If we are able to take her home, she’ll still be coming in regularly for her Etoposide regimen and we’ll be administering her other 2 chemotherapy medications at home, but at least she’ll be out of the hospital. Progress is progress.

Zoe has also gone down slightly in weight, 13.8lbs down from 14.11 the day before, which I take as a positive sign. We attribute it to coming off the iv fluids, which she had been on at the same time she was breastfeeding. For a while there I thought we were going to need the Willy Wonka juicing machine.

Ferritin Levels & Blood Draws

First the great news: Zoe’s ferritin level has decrease from 5000 to 880, a huge and positive response to her medications. Normal ferritin level is ~100 for most people, and high levels are not toxic in and of themselves, but ferritin is our primary indicator of disease activity — a decrease in that is a decrease in disease activity. We’re thrilled and Zoe is beside herself with wiggles at the news.

We talked to Dr. B about temperature reading a bit, given that Zoe is staying around 97º consistently. He indicated that the 98.5ºish temperature that we all know and love is really an average, not an absolute. Many people naturally range higher or lower than that, though not significantly, and as such a slightly lower consistent temp isn’t cause for panic. Zoe’s 97º might be closer to her norm than we had first thought. My first thought at this news was that it explains so much about why I always want the A/C high and Michelle is always cold when I’m comfortable, but that’s a story for another day.

We spent our morning getting blood drawn — myself, Michelle, and Maya. Mom and Dad went first so that we could show off our boo-boos to Maya, who took the news that she was going to be poked much easier as a result. There were tears, but no struggle, and now we are much closer to knowing if she can be a donor for her little sister. The tests will be sent off to Duke Pediatric Bone Marrow Transplant unit for testing along with Zoe’s.