engraftment

Day +42: Packing Up

/ Evan / 6 Comments

It’s hard to believe, but we expect to be leaving the hospital tomorrow. It’s been a very long and painful trip so far, but we hope that the hard part — survival and cure — is done. Now we have to keep Zoe healthy, stave off GVHD and infections, and see her through to a new beginning, when she’ll be healthy enough to venture out into the world confident that she has a strong immune system.

We will be staying in Durham to be close to Duke for the next few months, give or take. It’s necessary to be within 10 minutes of the treating hospital in case of rashes or other signs of a problem. Once we have reached Day +100, we will be transferred to Wake Forest for ongoing care. Zoe will be seen daily for a couple of months, then weekly, and eventually monthly. After a year or so we’ll be on a yearly schedule of check-ins with her transplant doctors, essentially for life. She should be completely out of danger in a couple of years, but because she is part of a study we will be trying to keep in touch long-term so that the doctors have any data they may need to help other kids.

Zoe is doing well. She is feeling good, very energetic and doesn’t want to sleep very much at all. When she does finally give in, she sleeps soundly through the night. Her WBC count has been good, it dropped a little below 3.0 Monday night so she received a dose of GCSF to bring it back up. No major pain this time, we’ve learned how to navigate that a little more effectively.

The physical therapist came by for a final inpatient visit, and she was really enthusiastic about Zoe’s progress. Granted they are enthusiastic about everything, but she pointed out a few areas where Zoe has continued to improve. Her sitting is much better, she’s learning to catch herself to avoid tipping over, and she’s getting very adept with her fingers, holding and turning toys to examine all sides before giving them a nibble.

It was a relief to hear that Zoe is not “plateauing” in any areas — ie she continues to develop at her own pace, without any stagnation that might point to a long term problem. I have a feeling that once we’re out and she can be around her sister, who she adores already and is extremely active, she will do just fine.

I wrote awhile back about some of the chance encounters we’ve had along the way, in particular with a man on staff at Duke named Mikey. I had an opportunity to speak to him a greater length recently, and he gave me a copy of the poem he recited for Zoe one day. We found it really touching, and Zoe responded so well, calming down during a really rough patch, that I am pleased he was willing to let us have a copy to keep.

He has an interesting story. Raised in West Virginia by two teachers, he spent time in California during the 50’s and 60’s, then Paris, working as a choreographer for most of his life. He ended up in Durham working for Duke as a housekeeper later in life, and when asked why he’s still here he says that he feels God has a plan for him, and that he’s here for a reason.

Mikey volunteered to take on 5200, the transplant ward, after finding that much of the staff didn’t like working here due to the strenuous cleaning requirements. I’ve only seen two people up here doing the cleaning on this wing, both older than the rest of the staff and very dedicated.

The poem was originally written on a whim in response to a child named Daniel on 5200 who rarely spoke, but who Mikey wanted to connect to. He went in one day and recited it, and they were able to make a connection, speaking regularly after that whenever Mikey passed through their room. Since then he has occasionally recited it for kids who seem interested or who he through might appreciate it. We’re glad he’s here. A kind caring voice can mean a lot during hard times.

Under God’s Chestnut Tree

Hello my child, you’re sitting under my chestnut tree,
looking back and up at me.
My child, you’re under my chestnut tree,
the birds and bees fly around thee.
My child, under my chestnut tree, you gaze up
at the stars which look back at thee.
Yes my child, under my chestnut tree,
you look to your friends to play with thee.
Oh my child under my chestnut tree,
your parents began to run and play after thee.
And now my child, under my chestnut tree,
the eagle flies high above thee.
But do you know my child, who sits under my chestnut tree?
God looks down and smiles on thee.
And yes my child, under my chestnut tree,
God has chosen you, for don’t you know, you are he?
— Michael Hairleace-Harris

Day +37: All By Herself!

/ Evan / 6 Comments

Another milestone this morning. Zoe’s counts all went up today for the first time without any cell growth medication or transfusions. Her WBC went from 4.1 to 4.8, RBC was up, and platelets are finally stabilizing and going up on their own.

For quite some time, Zoe has been getting platelet transfusions to keep them up, though she hasn’t gotten one in about a week — they have continued to slowly drop. Her WBC count has also continued to drop on any day she has not gotten GCSF to promote growth, but as mentioned that also promotes bone pain and discomfort. She’s finally growing cells all by herself!

Our departure from the hospital continues to be postponed, mostly because of the continued spit-ups. They had hoped that would be over by now, and while no one is concerned, they say it just takes more time for some kids, they prefer to have her heal a little more before we leave and she’s off monitors completely.

[spoiler name=”Click for counts”]

  • WBC: 4.9 (up from 4.1)
  • Hemoglobin: 9.2 (up from 8.9)
  • Platelets: 144 (up from 112)

[/spoiler]

Day +34

/ Evan / 1 Comment

Still in the hospital, it’s looking more and more like we’ll be here ’til next week.

Zoe continues to have some discomfort, her cell growth has been through the roof on any days that we get growth factor. She went up to 23.8 WBC today, with lots of bands this time. She drifts down about 4 per day in her count it seems typically, 11 to 7 to 3 for example, then she gets growth factor and shoots back up. Those days are difficult, but getting better. 23 is our highest yet though, her ANC yesterday was 21,182.

We’ve been getting day passes, but she came back in with a sniffle yesterday and the doctors recommended we skip it today, so we are playing it safe. Her weight has also been up a little, but she’s also been nursing a little, so we’re not sure if that’s the reason for the gain.

Regardless, we’ll be keeping her in for a few more days until everyone feels like the risk of fluid retention is gone, and she no longer needs any pain medication beyond Tylenol.

Generally things are looking up. We’re roughly on schedule here, she’s engrafted, and with a good chimera result we’re just trying to work through the last remnants of mucositis and the pain of cell growth.

Michelle and I are truly exhausted, I think we’ve been running on adrenaline for quite some time now. With the most recent test results there is a bit of tension release going on, and with it fatigue as things catch up with us. Whenever we do get discharged, here’s hoping we can find a way to just sleep for a few days.

Still, the likelihood that we’ll all be back together is growing each day, so we’ll just keep focused on that sunny tomorrow until we’re out of here.

Day +30: Chimerism

/ Evan / 14 Comments

I had a post written up covering the last couple days while we were waiting for results, but now that I have them, those details seem less important somehow.

First the Chimerism test results, then I’ll try to explain them.

Zoe’s test came back at “Greater than 98%”, which is as high a reading as this particular test offers. That’s good news. The best news, really.

Also important is the line on the report that reads, “no recipient cells detected”. This means that she has none of her old immune system left whatsoever. In short, Zoe’s test results were the best she could get on this test, and the best news we’ve had… well, ever, I suppose.

What does all of this mean?

Zoe “has” (we can’t say she’s cured yet but we’re well on our way) a hereditary condition/disease called HLH which caused her immune system to malfunction and attack her body, as if her body were an infection. The inflammation from this attack caused persistent fevers, enlarged organs, and without treatment and subsequent cure would have resulted in her death in a matter of weeks.

To cure Familial HLH Zoe had to get a new immune system via either a stem cell transplant or bone marrow transplant, otherwise the disease would continue to re-activate. Eventually the drugs used to control it would become ineffective, leaving her defenseless.

In order to get a new immune system, the old one has to be wiped out via a series of drugs (chemotherapy) that destroy the old bone marrow (which produces the white and other blood cells), so that a new one could be transfused. When a transplant takes place, there is a chance that both the old cells and the new cells will co-exist for a time, until either the new cells establish “dominance” as it were, or the old cells reject the new ones.

This rejection is the greatest risk for these kids. If it occurs, they have to go through the process of transplant again until they can establish a new functioning immune system. They can live with a mixed immune system, however, so long as the “dominant” portion are the new cells. Specifically, the old lymphocytes must be destroyed, if they continue to be produced in the marrow they could potentially begin attacking the body again.

This mix of old and new is called Chimerism. The word comes from the Chimera, a creature of Greek myth composed of multiple types of creatures. The test itself tells us what percentage of the old system still exists, and what percentage is new. We want as much as possible to be new, and in Zoe’s case, it appears that it is all new. She doesn’t appear to have any Chimerism, she simply has an entirely new immune system.

I am told that kids can do just fine with as little as 20% new cells, again assuming that the trouble-making lymphocytes are gone and that portion of the immune system is governed by the new cells. We had been optimistic, hoping for results greater than 50%, with room for growth toward that 100%. As time goes on after transplant, it’s expected in kids with a mix of old and new that one of the systems will continue to establish itself, and that they could eventually end up with 100% new cells, despite starting off with 50 or 75% at day +30. This is all part of the process.

Dr. P mentioned that he had been privately expecting and hoping for a 90/10 split for Zoe, due to the reduced intensity regimen and the fact that it can sometimes only partially wipe out the old cells. He would have been happy with that result. He was very pleased indeed with this result. Zoe’s RIC was on the harsher end of RIC, but I now firmly believe we got the best of both worlds.

Zoe was able to receive a lighter dose of the chemotherapy, potentially preserving her fertility and protecting her from the harsher side effects, while still seeing the “right” amount of destruction to her old immune system, leaving her able to accept the new cells successfully.

We will be eternally grateful to Duke University Health for the successful transplant, and to Wake Forest University Medical Center for her early diagnosis and life saving treatment. I now know which basketball team I’ll be rooting for for the rest of my life.

We don’t yet know all the ramifications of this for us or for Zoe. We know we will still need to maintain long term care for her, protect her from infection this next year through isolation, and watch her for GVH. Nothing in medicine is 100%, there is still a chance that there could be GVH or other issues, but we believe the chance much lower than it would be with a more mixed Chimerism.

Our next goal is to reach 60 days, after which the risks to Zoe for infection or GVH should be drastically lower than they will be these next 30 days. The worst is now firmly behind us and I hope that very soon we will be able to say that Zoe is cured. After this year has passed, our family should be able to return to our “new normal”.

Day +27: 11.1

/ Evan / 1 Comment

Zoe’s WBC counts were at 11.1 today, which is significant for two reasons. One, she has white cells! Reason enough to cheer around here. And two, she is off of her cell growth medication, also known as GCF, and her count is holding steady. She was at 11.8 yesterday, her highest so far.

It will likely go down tomorrow now that she is off completely, but we’re optimistic it might not. Without the stimulation of the growth factor (nurses here call it miracle grow for babies), it may drop by as much as half. Still, we went one day without a drop, so that’s good news.

Zoe is still having regular spit-ups as she processes out the mucus, but the pain appears to be mostly in the past for now. We expect to be taken off of the PCA that controls her pain with the button press any time, and they are trying to move us toward oral meds slowly in anticipation of discharge.

The music therapist and physical therapist both stopped by today, and Zoe did great. She is getting much stronger and closer to being able to sit up on her own in the tripod position, though she is still somewhat unstable, having to spend so much time on her back by necessity. That she is trying is reassuring, as are her attempts to roll over, often thwarted by the tether of tubes dangling from her chest.

I suspect the music therapist cheered me up more than Zoe. He did rousing renditions of Happy and You Know It, Itsy Bitsy, You are My Sunshine and one I really loved but had never heard, called Baby Beluga. Zoe was having a round of coughs and spit, so she was less enthusiastic. Still, by the end she was smiling and waving her hands a bit. Certainly a nice diversion, and I’m grateful they have this sort of thing.

Wandering minstrels with lamb chop sideburns who wear Velvet Underground and Bjork buttons while singing Raffi and classic lullabies are more scarce than they should be, I say.

httpv://www.youtube.com/watch?v=2×6-dsqhGJY

On a more somber note, one of the kids had to go to the PICU, or Pediatric Intensive Care Unit, last night. She had her transplant a year ago, but was back here for care due to a brain tumor and surgery. It’s been a harsh reminder that things can take a bad turn with such complicated diseases as the kids here are fighting. Hopefully she will be back up here soon and back on track to recovery.