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.

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.