Day +68: Changes Afoot

Zoe had a great day at clinic — or is having, I should say. We’re still here, getting the last of her Monday infusions.

WBC count was at 2.4, so she did get GCSF again, but as I mentioned previously it seems a matter of routine now, not concern. No one is worried that she will not eventually grow her own WBC more quickly.

The big news of the day is that we’re going to try to get off of TPN. Zoe has been eating more voraciously these past few days, particularly in the evening as the effects of the TPN wane over the course of the day.

We’ll be really pushing to get her eating as much as possible so that our trial run is successful, and she does not need to return to it (ever!).

In addition we’re moving to oral steroid (hydrocortisone), which the doctor indicated may accelerate her loss of cheeks and such a little more. We have the option of trying her off of the Zofran as well, which we’ll be taking. If she has increased spit-ups and nausea, we can always go back.

Next week if all goes well we can move her to oral Tacrolimus, which would have her completely off of IV meds, setting us up to get the lines out and a porta-cath in. That would mean real baths and even some pool action, which would be exciting indeed.

Dr. P stopped by to go over everything, and when I asked confirmed that her chimera results remain at >98%. He was very non-chalant, and indicated that he has no concerns about her graft whatsoever at this point. “It would be rare indeed” were she to have a graft problem with where she is at this point, he said. That’s a big relief, I’ve been anxious, constantly waiting for something to take a turn for the worse with that. I will (try to) stop stressing now.

All of this together means we’re one step closer to that normalcy thing I’ve been talking about. Get her to oral medications, eating on her own, lines out of her chest, and she’ll be much better off. I would say much happier, but honestly I’m not sure there is such a thing as a happier baby.

And that, friends, makes me happy too.

Day +65: Line Breach

We’re still waiting on the chimerism results. It’s a test that they like to discuss, and since results were not expected until this afternoon, we won’t hear about them until Monday. Crossing fingers that we’re at 100%.

Dr. P was very enthusiastic this week about Zoe, he feels that she has been a great case, given how well she’s done with everything, and that by her first birthday, she will be completely normal again. Rockstar is the word he likes to use. Normal is still the word that sticks in my head though, that’s essentially what we’re striving for here — a normal life for our girl.

Thursday’s labs looked good. No chemistry issues other than a drop in glucose related to her reduction in TPN. They’re working on weaning her nutrition so that we can get her off IV meds, the hope is that we’ll be on oral only before long and get her tubes out. A heel stick (blood test) came back normal for the glucose, so no worries there at the moment. WBC was at 3.4, which is where she seems to settle pretty often. She may drop again and need GCSF on Monday, but we appear to be out of the phase of stressing quite as much about that count. No one at the hospital seems worried about it, they’re much more focused on her chemistry’s at this point it seems.

We had our first true snafu this morning. We were closing up her lines (flush with saline, lock with heparin) after her night’s TPN, and when the tube was unscrewed her “cap” came off, leaving her central line open directly into her chest. The “cap” as it’s called is the barrier between her bloodstream and the big bad germy world — having it off for even a few moments is a bit scary. We do cap changes regularly, but we wear masks and Maya has to stay out of the room entirely, the cap is off for a matter of moments only while Michelle and I essentially hold our breath, so anxious are we not to have anything enter that line.

So having the cap come off and go unnoticed as it bumped around in her nightie is, well, not ideal. We noticed it was off when it was time to hook up her Tacrolimus, so it was a matter of a few minutes that it was open. A quick call into the hospital revealed that it was not something we leave unexamined and go on about our day, so Zoe had an unexpected trip to the clinic this morning.

At the clinic they did a line culture, which they’ll test to see if anything is growing in the line that could cause infection, and gave her a dose of Vancomycin, a fairly hefty antibiotic given preventatively in the case of a line breach. Hopefully nothing more comes of it, so far she has no fever or other symptoms of sepsis. That’s what we’ll be watching her for this weekend.

Having something like that happen really brings home how tenuous all of this is at times. Any little infection in her line can really cause huge problems. All the more reasons to get those things out of her.

On a lighter note, being back in Durham has given us a chance to revisit what we liked about the Triangle when we lived here. On Wednesday Maya and I went to (what used to be) Exploris, now known as Marble Kids Museum, and after about 2.5 hours of all-out-play-frenzy, took in an IMAX movie. Great fun. It’s a little strange to be a tourist in a place you used to live on the one hand, but on the other, we know some of the best spots already and can make the best of our free time.

Next week could be a big one. We’re hoping to hear back great news on the chimerism, get her off TPN, and potentially schedule a date to get her lines out. If we can move her Tacrolimus to oral, she will be free finally, having spent now 5+ of her 7+ months with tubes in her chest.

We’ll have to have a party. 🙂

Day +61: Keep on Truckin’

Not much has changed for Zoe, which is exactly what we want. Her counts remain solid, her WBC is holding at 4.2 as of today, though she did have GCSF on Friday. In terms of a trend she seems to be slowly ticking upward, holding on to more and more cells each week.

Her second chimera test blood draw also took place today, so I’ll be on edge until we hear back. They don’t always draw a second chimera test at 60 days, but Michelle and I had already determined to press the doctors to do it anyway — we want to be sure her last test wasn’t a fluke. Which of course it wasn’t, objectively, but at this stage we are still quite prone to worrying that something we can’t see is wrong. Luckily kids who are on the study all get tested again at 60 days anyway for a data point, so it was all part of the plan.

At home we’ve settled in quite nicely. We’re increasingly finding time for work and fun activities during the day, though it tends to be odd hours broken up by Zoe’s med schedule. Zoe has worked her way up to almost 10 ounces per day of breast milk, meaning we may be able to get off the IV nutrition soon. That would take a good hour and a half off of our daily routine, time we’d be grateful to have back.

Maya is doing well, we have a number of activities we’ve worked out to get her out of the apartment and playing with other kids. Keeping up her social life is really important at this age, and we’re lucky to have a number of friends here and back a home a short drive away willing to meet up for play dates.

We did find out that Maya is not going to be able to return to school this year, it’s just too dangerous for Zoe at this point should something hitch a ride home with Maya and get us sick. Her school is a really good one, but it’s just not possible to ensure she won’t bring something home and the doctors said no to her return this year.

Once Zoe is a little further along, likely next spring, Maya can start up again. Thankfully the girls are both very young, she won’t miss as much as she would even a year or two from now.

Day +??

I was walking through the children’s clinic wing of the hospital a couple days ago with Maya on the way to drop off labs, and the thought occurred to me: I don’t know what day + we are anymore. I worked it out, and it appears we are at Day +55 now. 45 more days ’til +100.

What a difference being outside of the hospital has made. We’ve transitioned from micro-management of Zoe’s care, stress and hospital life, to trying to become a whole family again. Fewer worries about which Day+ we are, more worries about making sure Zoe and Maya both have their share of our time. The scares are almost gone, replaced by calmer fretting about Maya having a bad dream, or Zoe not getting enough time on her belly with her lines in the way.

They call it the “new normal”. Finding our routines, our calm, yet still managing the care of a child who needs just a little bit more. Zoe still needs a great deal of care, and will continue to for some time, but we have to work it into our lives in such a way that we can provide normalcy for ourselves and the girls.

Early on in our inpatient stay at Duke, during a visit with Dr. P, Zoe’s primary doctor, the subject of Zoe exiting all of this as a “normal” little girl came up. We were worried, with good reason, that she might not escape this disease without major problems — neurological, developmental, or even death are reasonable possibilities. At the time even the thought of returning to normal seemed too much to ask, even.

Dr. P made a point of saying that, after we made it through all of this, there would come a time where we would have to start treating Zoe as one of our kids, not as a patient. It seems strange to say it, but it’s true — it’s hard not to see everything through the lens of her disease at this point. He explained that it is common for parents to struggle with enforcing rules, establishing fairness among siblings, and encouraging the kids to bump and tumble and, well, grow up.

One day we’ll have to transition out of emergency mode, and into our “new normal”. That’s what this past week has felt like, that we’ve begun that journey. We have worked out the med schedule and we’re maintaining Zoe’s care, but we’re also doing some work here and there, and taking time to do fun things again.

Michelle and Maya made it to the beach for a day trip this past weekend, and Zoe and I stayed here in town and played and watched a series of bad comic book movies on FX. That sort of Sunday would have seemed inconceivable a short time ago. I had been determined to see that Maya got to the beach this summer, but it was looking unlikely there for awhile. I’m thrilled we pulled it off though, Maya had an absolute blast.

On the medical front, we have a couple more weeks of TPN while we transition Zoe back to breast milk. She lost her latch in the transplant, she simply appears to have forgotten how, and so far none of the therapeutic suggestions have made much difference. In the past few days we’ve started giving her a sippy cup, and that has gone really well. She’s a messy eater at the moment, but hey give a baby a break, right?

Her counts have been stable, and her platelets are into the normal range on her own. Dr. P made the joke yesterday that she could start donating platelets now. We’re thinking we’ll hold off on that, but he’ll be here all week folks!

WBC counts are hanging around in the 2’s and 3’s, and Zoe is getting her “G” (GCSF or cell growth medication) about once a week now. The feeling is that she will begin to produce her own WBC any time in sufficient abundance not to need it, but whenever the count goes below 3 she gets a dose. Hemoglobin, minerals, everything else are looking great. The only thing she has needed since discharge continues to be the GCSF.


Day +50: The Med Routine

Things are settling out here as we work out our routines. Now that we’re out of the hospital and in each other’s hair again, everything seems at ocne more hectic and more pleasant — even the unpleasantries.

Zoe has a long routine that we go through each day to maintain the meds she needs. It is very prescribed and intricate for a lay person I’d say, it’s taken us a few days to adjust to everything we need to manage.

Right now, Zoe is still on TPN or IV nutrition, which she’ll stay on until she is eating enough milk or solids to no longer need it. That may be a few weeks, though we are working on it. She is also taking a variety of medications for a variety of purposes, all of which are monitored and adjusted with daily lab (blood) draws that we draw and take in to the hospital.

We were going in every day for the first while, but we’re already on an alternating schedule due to Zoe’s pretty amazing resilience. She has not yet needed any infusions — platelets, blood, GCSF for her WBC — and that’s given the doctors confidence that she is stable enough not to need to come in each day. Her WBC count is holding out around 3.4, and they are resisting giving her any cell growth medication at all in hopes that by stressing her immune system a little, she might begin to produce them herself more quickly.

Here’s what our schedule looks like at the moment on a clinic day:

The Situation

7:30-8am: Zoe’s alarms go off for her TPN and Lipids, which run overnight for 12 hours each night. We flush her lines with Saline and lock them with Heparin, which prevents them from clotting when not in use.

8:15: Blood draws, between 1 and 3 tubes depending on which tests she needs; these have to be dropped off at the hospital within a few hours, but we try to get them in by 9am to ensure the results are back by the time we’re in clinic.

Packing up

8:30: Zoe’s Tacrolimus infusion starts, which runs over 2 hours; this is an immuno-suppressant to keep her immune system from revving up too much, she receives medications to help her fight off infections while it’s kept suppressed. She also receives her morning Hydrocortisone infusion, which is given by hand over 5minutes.

9am: Morning oral meds. Typically 3-5 meds, including Cellcept(to prevent graft rejection), Amlodipine (for blood pressure), Vfend (to prevent infection), and Raniditine (for reflux while her GI tract heals).

Have food will travel

11am: Clinic time. Visits vary widely in length, Mondays being the longest due to our seeing the primary physician that day and getting IVIG(antibodies). Other days we may be out in an hour after they’ve checked her, if everything is looking good with her blood tests and vitals.

2pm: Second Hydrocortisone infusion of the day.

5-6pm: The daily med delivery arrives, bringing everything from syringes to batteries to actual medications in fridge packs.

6pm: The evening prep starts for infusions and TPN. The IV nutrition has to be loaded into the pumps to run overnight, and injected with vitamins. The vitamins lose their potency in the light, so they are injected just prior to administration.

6:30-7pm: Evening infusions. Zoe gets a third Hydrocortisone infusion over 5minutes, Zofran(for her stomach) over 5minutes, and we load her TPN & Lipids for the night. She also starts her second dose of Tacrolimus of the day around this time.

7:30-8pm: Evening oral meds. Usually the same as morning, though a couple are only once a day.

9pm: Tacrolimus is complete, and Zoe is asleep for the night. She typically sleeps through, with perhaps one wake-up at night.

Each of the infusions requires 5ml of saline before and after, then a 3ml Heparin to lock the line. This means Zoe goes through roughly 15 saline syringes per day, not to mention the liquid from the infusions themselves and the heparin. It’s an amazing amount of fluid for her size, good thing those kidneys held up.

Zoe & Maya Playing

Stir in one 3 year old and shake vigorously, and we have a pretty interesting schedule right now. As time goes on, we’ll cut the TPN & Lipids completely, vastly simplifying things.  We’ll hope to cut out other meds, and eventually get down to only the Tacrolimus 2x a day, if everything continues to proceed as planned.