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.

Vitals:

  • 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.

2 O’clock and All is (mostly) Well

So far it’s been an uneventful day medically. Zoe has near constant visitors in the form of nurses with medications for her IV, but we have finally reached a point where it is routine and there is no immediate danger.

Zoe’s platelet count is up from 90 to 150 on her own, so that’s a very positive sign. Dr. B came by and we discussed some of our ongoing concerns, one of which is how much should we expect her counts to go back down as a result of the chemo, even as we are watching them rise with enthusiasm. He says that it will take as much as a week to see her counts take a toll from the Etoposide, and in the meantime good news is good news, so we can continue to be pleased by improvements we see. We’ll be watching for news of her inflammation to continue to decrease, watch for ferritin counts to start to drop, and hope that her temperature starts to stay at 98º instead of 97º.

Zoe continues to gain weight from the Dexamethasone, which we’re told will continue for awhile and then level out as her dosage is reduced in coming weeks. I spoke to Dr. B about potential complications from weight, but at this point developmentally she’s really only expected to be working on keeping her head up and perfecting her wiggle. There should be no problems there, and by the time we’re ready to see her trying to roll or lift up, she should be at a more moderate weight. She makes a cute butterball though, I’ll give her that.

Surgery & Etoposide

Zoe starts the day with a NPO or not by mouth order that begins at midnight, so she will spend her night without food to prepare for her surgery in the morning. It’s a rough night, but she’s a real trooper. She fusses and ensures that we all know that she has not had enough to eat for her taste, but around 2:30 or 2:45 she decides to let it go and catch some sleep. She sleeps until 6am when the first of the many nurse and doctor visits of the day begin.

Surgery is scheduled for 8:45am. We get visits from the surgical resident to sign off on paperwork and later from nurses to take her labs and prep her for travel down to the operating room. They also give her 2 more infusions of platelets to ensure she is not at risk of bleed out during surgery, which would have been a serious concern a couple days prior. Around 9am we take the trip down and soon we’re settled into the waiting room, a huge room the largest feature of which is a ~30ft long ship re-creation for kids in the the middle of the room. I almost wish Maya were here, I’m certain she’d have scaled it right up to the false clouds hanging above it by now and be dangling like a little monkey.

After about an hour we speak to the surgeon, who indicates that Zoe has done very well, no problems or complications. The surgery was to install the Central Line or catheter in her chest so that she can begin the 3rd and final of her trio of chemotherapy medications, Etoposide. After another 30 minutes or so to wait for her anesthesia to wear off, we get to go meet up with Zoe for the trip back upstairs.

We have a brief visit from Dr. M mid-morning, he’s about to rotate off for two weeks presumably to do his teaching rotation at WFU. I bring up with him the thoughts listed under Contemplating the Bone Marrow Transplant and he is very receptive to our concerns. He’s able to confirm my fears that there is not a way to 100% rule in the acquired form of HLH, meaning we will have some tense moments ahead if we cannot positively confirm the FHLH. He is, however, open to doing chemo only should there not be any markers for the FHLH variation present in her genetic tests. We won’t be able for some time to know the answer to this.

Later in the day we finally meet Dr. B, the Hemo/Onc doc who is rotating in and will be covering Zoe for her next two weeks. Dr. B is an infectious disease specialist in addition to his Hemo/Onc specialty, so he is able to answer some of our questions in a slightly different way than Dr. M. The whole conversation reminds us of how important it is to speak to more than one doc about such matters, the presentation alone can offer a lot of insights and information that we’d have no way to ask after but glean from the difference in conversation styles.

His infectious disease specialty specifically offers experience with helping children survive the BMT procedure, and he was able to reassure us somewhat that he had seen quite a few children through that process without incident.

We ask after ways to rule in or out the disease variations, and Dr. B mentions the genetic markers which we’ve drawn blood for and which will be sent to Cincinnati. We’ve already discussed these with Dr. M so this is just confirmation, however Dr. B does offer another possibility, NK cell function. I honestly do not yet know what this means, we haven’t yet had time to research it, but it is reassuring to feel that there may be multiple ways to rule in or out the need for the BMT. Additionally, the NK cell function monitoring test can apparently yield results in days rather than weeks.

When asked for ways that Michelle and I might monitor Zoe’s progress toward disease remission, Dr. B offered two indicators of disease activity: Ferritin and IL2/CD25. He will try to keep us up to date on the status of those two indicators so that we know as much as we can week to week where we are.

At 3:45 Zoe began the last of her 3 chemotherapy drugs, this one the Etopocide, the most harmful of the three and the one most likely to cause the types of effects most of us tend to associate with chemo. Zoe was monitored for an allergic reaction during the initial drip, one of the risk factors, but none was present. She was chipper and playing with her hair while the nurses and Michelle and I took turns chatting with her. Zoe will be getting the Etopocide on Mondays and Thursdays each week until she is in remission.

All in all a decent end to a stressful day.