What I’m Reading Tonight

Today was a stressful day as the start of our transplant process. Duke’s team couldn’t have been better, but I think there was really no way around our being a little tense at the outset of this.

Among the topics discussed was the possibility of a Reduced Intensity Conditioning regimen. Originally we had thought this was ruled out, I brought it up specifically at our transplant Q&A, but it has come up again. Through discussions with Dr. M and closely following Maya G’s story, as well as some discussions with other parents via email, we continued to stir the pot a bit about the possibility of Zoe being a candidate for RIC.

As it happens, Zoe was a big topic of conversation in the PBMT team meeting, and one of the docs had recently heard Dr. Filipovich speak on the subject. Between our agitating and this coincidence, we’re now back to considering this procedure.

There is a lot to learn, and we have a big decision to make if she is a candidate. We’ll know more later in the week and I will cover the details, but in the meantime I am working through two journal articles on the subject for those hearty travelers who wish to follow along:

The Use of RIC in HLH and LCH Patients (2008 study)

RIC and ASCT in children with malignant and non-malignant diseases (2008)

Invigorating reading, let me tell you.

Those Difficult Thursdays

Thursdays are our clinic days, and in recent weeks Michelle has been going in by herself, feeling that there is no point having two of us out of commission for the day. She’s right of course, but I’ve come to feel that I’m not as in touch with what’s going on, so I’m in with her today. I’d be here alone and give her a break, except that she has to be here to feed Zoe pretty regularly.

Being in the hospital again, even for a day, brings back all the recent memories of Zoe’s worst days. Michelle has remained accustomed to it, but I’m feeling pretty stressed being here, having had a couple weeks away from it. Hopefully, we will be done with this phase soon. Our day is spent waiting and worrying about infection in the lobby, getting labs drawn and vitals, and then sitting in a curtained and claustrophobic observation room for hours while Zoe gets her VP-16.

Compared to her days as an inpatient, it’s not bad, but a sad reminder of what Zoe is going through. At home things seem almost normal many days.

We met with Dr. M first thing this morning to discuss a few things and check in. I’d sent an email to Dr. M and Dr. W on Friday to ask a few questions, foremost among them what contact they’ve had with the team at Cincinnati Children’s Hospital, the U.S. experts on HLH. It’s a touchy issue — I don’t want them to think we are unhappy with our care, we aren’t at all, but I do want to be sure we have the most current information for Zoe before we close the book on her pre-transplant care. It is a rare disease and I feel sure that new information can appear that could help her, given how poorly researched it is relative to other conditions.

We should hear back from Dr. Filipovich soon.

Vitals today:

  • WBC: 12.4
  • RBC: 2.82
  • Hemo: 8.8
  • Platelets: 781k
  • SEG Neutrophil: 8.6
  • Ferritin: 700

Great results overall. Her Ferritin is back down from it’s brief spike, and it is being attributed to the skipped etoposide week (to let her immune levels recover). This means we can continue to taper her steroids, and I have in hand the next few days’ doses, after which she will be done with it, hopefully forever. It will take time to burn off the extra weight she has put on and for her cheeks to go down.

This means we will put a plan in place to move ahead with the transplant fairly soon. If there is a bed at Duke PBMT, we could begin her “work-up” in the next week or two, putting us in the hospital for conditioning somewhere in early June. It’s where we want to be. Doing it during the summer lessens the impact on our work and lessens the impact on Maya’s preschool attendance.

By fall we hope to be having our best year ever as Zoe returns to health. In the meantime, it looks like our mean old Thursday visits will be coming to an end soon.

The Thursday Routine

Each thursday for awhile Zoe will be headed into the outpatient clinic to get her LP (spinal tap), her Etoposide dose, and her blood draws. This complicates her day quite a bit. Here’s a look at her routine for the day:

  • 4am, last feeding for the morning in advance of Zoe’s “8am” surgery appointment
  • 6am, Zoe wakes and starts to fuss for her breakfast, won’t go back to sleep due to hunger. This is very nearly the worst part, listening to a crying baby and not being able to soothe her fully causes me to break out into a sweat almost instantly
  • 7am, we’re up fully now and starting to prepare her meds for the morning
  • 7:20, Zoe takes her meds. We held off on giving cyclosporine because they’re taking her levels in a couple hours — giving a dose on top of a blood draw results in an incorrectly high reading. We also skip her 2nd blood pressure me, the Atenolol, since she’s due to go under anesthesia shortly and we’re scared having two BP meds on top of that might be dangerous. Zoe eats her meds much more easily than usual; there’s something heartbreaking about a baby who takes her meds greedily because she can’t eat.
  • 8am, we’re at the clinic; Zoe gets a blood draw once we’re in the room but her Broviac/central line is so small that it doesn’t give up blood easily — we have to pump her little arms around and roll her back and forth until they get enough blood for the tests. At least she’s getting her exercise.
  • 9am, Dr. M is back, stops by to take a look at Zoe. He’s encouraged by her counts and says that her fussiness, puffy cheeks and hunger should start to drop as we continue to drop the steroid dosage; he advises us we were quite right to skip the 2nd BP med, which is a relief
  • 10:15, they finally come to pick us up for Zoe’s procedure; we were first in line we’re told but I imagine they took care of someone else while we were getting blood draws and the exam
  • 10:45, they start the Lumbar Puncture procedure; the anesthesiologist confirms she hasn’t had anything to drink since 4 and we discuss which meds she took this morning to be sure they know about skipping the 2nd BP dose
  • 11am, it takes 3 sticks in her back to get the LP done; it’s terrifying, no other word comes to mind
  • 11:15, they tip her head down at a shallow angle on the table so that the medication introduced into her spinal fluid can flow down and fully saturate the area around the brain; the goal here is to ensure those few histiocytes they’ve seen in her spinal fluid don’t have a chance to collect in her brain, the medication should suppress them
  • 11:30, we’re moved to observation for Zoe’s VP-16/Etoposide dose for the week
  • 12:30, while on the VP-16 drip Zoe’s face flushes; we’re concerned as to why, she appears to be asleep peacefully on the dregs of the anesthesia; the doctor takes a look, feels it’s her steroids — no other signs anywhere, pulse/o2 is good and bp is 92/44; VP-16 resumes.
  • 1pm, flush is worse, Zoe is an angry red now so we call in the troops; VP-16 is paused and they administer a dose of Benadryl — the determination is to wait 15min and if her color is the same or improved, we resume VP-16 at half speed. This means we’ll be here an additional 2.5 hours to complete the drip.
  • 1:30, Dr. M stops back by to discuss Zoe’s counts. He seems really optimistic and happy about them, more so than ever before. Almost everything has improved but in particular her WBC count has moved out of the infection danger zone, meaning Zoe is a little safer day to day now.
  • 2:15, flush is completely gone. Benadryl is some amazing stuff.
  • 3:30, VP-16 complete, the long day is nearly over; a little stomach upset that seems to pass after a few diaper changes over about 15minutes
  • 4pm, we change Zoe’s Broviak dressing; the process for this is fully sterile, meaning a sterilized dressing pouch and a simple ritualistic routine to ensure no contaminants get near her line opening
  • 4:30, home again; Zoe wants nothing more than to sleep, we settle her into bed for a nice nap
  • 7:30, meds again, and more sleep to round out the long day

It’s strange at the end of the day to realize that we spent a full “work” day in the hospital managing this, Michelle and I. I don’t know how any family could do this without support, it’s really quite daunting. The reward is that Zoe looks beautiful and calm come bedtime. Progress.

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.