Two Steps Forward, One Step Back

It seems like the nature of treatment that we are never able to quite get clear of things before we have a setback. We progress, things improve, and then something happens and we are back where we were a few months ago in terms of medications and precautions. It IS improvement in a big picture sense, but it’s bittersweet and stressful day to day.

Zoe is back on track now, and our worst fears have been put to rest. She does not have a recurrence of HLH, her graft came back at >98% again, the same as before and a clear sign that her transplant has taken and HLH is gone (forever we hope). The fear of a return of HLH was due to a rash that appeared very similar to an HLH rash, but the chimerism result reassures us that is not the explanation.

GvHD Rash before treatment

She does however have GvHD, which has required her to restart Tacrolimus and go on a short term run of Prednisolone, the old nasty steroid, again. Why she has GvHD was a big point of confusion to us given her chimerism results, so I will do my best to explain briefly.

Zoe has a new immune system, and that immune system is completely donor material. This is the result we want, since her old immune system was dysfunctional and susceptible to HLH. Her new immune system however has not yet come to terms with it’s new home, meaning her old body is still considered a threat to it to some degree. When left unsupressed, her new immune system attempts to fight her old body, causing the rashes and other GvHD symptoms.

We had gotten her off of almost all of her meds slowly over months of weaning, when the rashes started to appear. It seems that she is not quite ready to be off of them, so we have restarted the routine and we’ll see where she is in another 3 months or so.

There is a question of whether or not she will be ready to enter pre-school this fall, but I’ve been told by reliable sources — Zoe’s two teacher-grandparents — that she is too young to need to be in school anyway. Yes it was what we had planned and yes it would be helpful, particularly socially, but it’s not going to hold her back to wait another 6 months or year to be safe.

In the meantime, she is back to looking good and feeling good most days, which is a relief. We are going to finally be able to remove her port despite the complications of these past weeks, so that too is progress. At this point I’m wishing that we had started putting little patches on Zoe’s well-worn diaper bag for each medical scare, not unlike notches in a gunslinger’s holster or emblems on a jet fighter. She’d have them racked up by now.

 

 

Reality Check

These past few months we’ve found that it’s really too easy to lose sight of the reality of this disease when things are going well. I suspect it’s due in part to how hard things have been when Zoe had an active disease and in the 2-3 months post-transplant when she was in pain or discomfort often. Once those times pass, all we want to do is forget the bad.

We had a bit of a wake up call these past two weeks. Zoe developed a bit of a rash around her mouth and nose, and at first we were convinced it was a slight food allergy. Even the doctors felt it was nothing to be concerned with (yet). Another week went by, and instead of disappearing, the rash spread slightly and began to make us very nervous. We took Zoe in to her home hospital, Wake Forest, and after some consultation decided to take her straight back to Duke to decide what to do.

At Duke we determined that Zoe does have some slight GVHD, which is very distressing since she hasn’t had any worth noting this entire time. We’ve begun treating it with Prednisone, which is one of the steroids we had hoped never to see again. While on the one hand the steroids saved her life, on the other, all of the worst side-effects Zoe has had to endure are the result of the steroids. Our goal is to nip this and get her back off of it as soon as possible. Zoe is days from crawling we believe, she is crawling a couple steps here and there already, a little more each day, but a return to steroids could change things.

Turn your head for a moment and anything can happen with these two

Zoe is also getting a little Nystantin cream for a couple of spots on her skin, and some Nystantin liquid for her thrush, which is still hanging on and never seems to go completely away.

Zoe’s spirits are very high despite these issues, and she is growing well — she’s up to 67th percentile for height now — so it’s possible the GVHD will only be a minor speed bump in her progress. Having her do so well for so long then have the rashes appear is certainly disconcerting though. Hopefully it’s not a sign of anything major.

We have another milestone visit to Duke coming up, we’ll see how things look then.

Update:

After a few days on the steroids, Zoe’s rash has cleared up nicely. She’s in good spirits and we’re back on track we hope, we’ll know more at our next milestone clinic visit.

Day +75: Finding a Natural Weight

As most of you know who have checked in with us at one time or another, Zoe has been on steroids for most of her life at this point. She gained a great deal of weight pre-transplant due to them, and has since retained a decent amount of it. Her percentiles for her age and size put her in the ~90%, meaning she is larger than most babies her age and length.

We’ve been slowly creeping back down from that as she started to grow again and become more mobile, she seemed to stop gaining height for a couple months during the worst of chemo and steroids. Now, she really only retains her steroid cheeks, and even those are in slow decline finally.

During this process we’ve had a heck of a time getting her meds right, as we’ve moved from IV nutrition to milk and solid foods. Zoe’s weight has gone down, her Tacrolimus level (immuno-suppressant chemo) has shot up, and her Magnesium has dropped considerably. Last week and this week have been all about trying to get her eating, trying to figure out if her Tacrolimus readings are “real” or an error of the test, trying to get her Magnesium back up.

Where we are at the moment is, Zoe’s weight is around 8.2 kilos, which is pretty close to where she should be for her size, around the 50th percentile. I’ve come to feel that she’s trying to find her “natural” weight, the weight she would be were it not for all of this mess she’s had to endure. She seems to be much spunkier than she was even a week ago, and feel more comfortable moving around. She sits up easier, rolls on her side easier, and holds herself up on her belly with ease. All part of growing up, of course, but the change seems to be related to the weight. We can feel that she’s lighter. She seems to feel it too.

Zoe’s Tacrolimus level has shot up very high, and they’re inclined to believe it is accurate now — 1800, where normal is 500 for her. This can cause all manner of problems if it continues, so we’re going to suspend it for now, until we figure out the new dosage. Her Creatinine is up as a result, and because the Tacrolimus leeches Magnesium, that is very low. She’s getting supplements of Magnesium now 3 times a day, but she has gotten so proficient at spitting it out that we are struggling to get the levels up.

nom nom

We’re crossing our fingers that all of this settles out once her weight stabilizes. She’s eating pretty well, but the change in diet has definitely wreaked havoc on her med situation. If we can get it figured out without any seizures from too much Tacrolimus, or GVHD from too little, all will be well. We’re a little anxious, however, since those two situations are a possibility at this point.

On the bright side, she is eating solids quite well, her spirits are high, and her personality is really starting to shine through with little happy noises, laughter and cute little eyebrow maneuvers whenever she’s listening to us speak.

Oh, and she’s cutting 3 teeth now.

Day +25

Zoe’s WBC count hit 6.8 this morning, up 1.3 from yesterday. Just amazing. No ANC yet, we aren’t getting daily differentials right now that she has entered normal cell range. I have been waiting for it to go down some, we’ve been warned repeatedly that it tends to go up and down, but so far she’s just building.

At the moment Zoe’s only real symptoms/side effects are the continuing mucus discharge (spitting up as she heals), pain twice a day when receiving the cell growth factor medication, and some pain around her bum breakdowns. All in all, we’re making progress in that she’s not having as much daily bone pain, nor does she have any more sores in her mouth.

As mentioned yesterday, there is a possibility we’ll be discharged in the near future. I’ve been asking around a bit about that, and it seems they look for good progress on counts — engraftment, no infections, and for the parents to be “ready” to handle the care.

When we first were in discussions to get Zoe into Duke and planning her transplant, one of the things that came up was that the fastest anyone had ever been discharged from the Pediatric Blood and Marrow Transplant wing is +23 days. At that time, being the guy I am, I thought, “Zoe can beat that!”.

Now, I have no interest in that. All I care about is making sure she is at optimum health before we’re released, and that we all know exactly how to handle things. The next few months will be stressful while we wait to make sure her graft holds and she is clear of GVHD.

On Monday they will do her Chimera test, which is a method of determining what percentage of cells are her old immune system, and what percentage are the new. We want to see mostly new cells. I have been cautioned that patients can get by with far fewer than 100%, as little as 20% donor cells, and live normally, but again we are hopeful for something north of 50% for this first test, the closer to 100% the better. We’ll get results back on that later in the week, it will be a big news day when we know.

httpv://www.youtube.com/watch?v=pJmVgRAGQ_Q

Day +13

We had a visit from Dr. S yesterday afternoon, and it was very positive overall. Zoe was feeling good and smiling, and we were able to discuss her status.

Her WBC was 0.1 yesterday, but on Mondays they do a manual differential, which means that they actually look under a microscope and count the cells. The daily cbc is done by machine. So they actually saw white cells under a microscope, no possibility of machine error there. Dr. S felt that we are exactly on track for the start of cell growth, maybe a few days early — day 14 he said was typical, we were day 12.

We talked about the Reduced Intensity Conditioning study that Zoe is on, which Dr. S heads, and so far they have used this protocol with 7 other patients. Only one of them had any notable GVH, most of them had very very mild if any at all. Zoe has had a very mild bit of GVH on her cheek (the redness seen in recent photos), but he felt it was at best a 1 on the scale, so not a concern.

All 7 of the kids they have used her protocol on have had successful grafts. Very very good news. They feel that Zoe is shaping up exactly as expected, which in medicine I gather is a great thing. Wonderful news.

Zoe’s mild tissue breakdown in her mouth from mucositis is healing, as is her bum. We haven’t needed the “open air” strategy or any special medicines for it for a couple days now, just her cream.

The WBC count today was again 0.1, which means we seem to be holding steady at the moment with some cells. It will likely go up and down, what we’re hoping for initially is 0.3 or so for a few days, that will indicate some stable progress with cell growth.

Assuming things continue as expected from here, we’re looking at a nice long stretch of waiting while Zoe’s cells grow. We may see some increased redness from GVH if the cells rush in too quickly, but it’s not anticipated. Zoe may have a higher chance of that because she is the youngest on the study, and she got the most concentrated cell dose, which is a good thing in most respects. It does mean her cells may grows fast enough to generate a reaction, however. We shall see.

Zoe has been feeling well enough to start playing with the bottle again. She’s been unwilling to take it for more than a moment, we assume due mostly to mouth pain, but it’s a good sign that she’s willing to take a little now as she’s healing.