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.

Temperature Scare & Other Notes

The day started off very poorly, around 5:25am Zoe’s temp dove to 95º. She began her first chemo medication, the Dexamethasone, which broke her ongoing fever fairly quickly. We had been keeping her only lightly swaddled and unwrapped when her temp was high, and after the med she apparently went from too warm to very cold as a result in part of the exposure. We do wish that it had been discovered more quickly — the nurses had been taking her temp every hour prior to the medication but for whatever reason they didn’t come in for several hours after it’s administration.

After some time of being swaddled with warmed blankets Zoe’s temp creeps back up to 96-97 and hovers there. Fear of infection has set in so they need to draw blood, however her veins are limited at the moment so instead they will put in a catheter to take a urine sample and minimize the blood needed.

5:45, Transport team from Neonatal Intensive Care Unit (NICU) arrives to do a blood draw, nurses on our floor have been having some difficulty and felt the transport team could work better with difficult veins; after a few minutes Transport has gotten the needed draw with minimal pain to Zoe, much quicker than the time spent trying prior to their arrival; relief.

9am, we’re moving to the intermediate pediatric ward, also know as High-Acuity, where they have few nurses per patient and will monitor her constantly until her temp has risen; can’t move to Hemo/Onc ward until a bed opens up, this is an intermediate preventative step after the temp scare due to poor oversight

1pm, visit from Dr. M to check in; Zoe’s Hemoglobin is 8.1, Dr. M says his threshold for a transfusion typically is 8, however because he feels it might help Zoe feel better he orders a RBC transfusion this evening; Zoe is now getting the first two drugs in her chemo regimen the dex and cyclosporine, as well as two of what Dr. M calls prophylactic measure to ensure there is no risk of infection, an antifungal and an antibiotic.

Dr. M introduces us to Ferritin, a protein that stores iron and has become a marker for HLH the more the disease has begun to be understood. Zoe’s is currently 3000, in a normal infant it would be 100; there is no risk from the Ferritin itself however it tends to show evidence of the disease activity. Coming down in Ferritin count is a good sign, the opposite is a worrisome sign.

We also briefly discussed genetic testing. Zoe’s has been sent but can take weeks to return, if she returns with markers for the disease (indicating she likely has FHLH) then we will have Maya tested.

11pm, blood transfusion begins, will last 3 hours; temp down to 96º again, was holding at 98º for awhile

1:15am, heart rate drops below 100 consistently — nurses feel she is probably just a little cold, but wrap her in warmed blankets to ease our concerns

Remainder of the night passes without incident.