Day +30: Chimerism

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”.

14 thoughts on “Day +30: Chimerism

  1. GO DUKE, especially 5200,

    GO Zoe, I can’t wait to meet you 🙂

    GIve your Grandma and Larry a big smile for me. They are the best neighbors and friends.
    Love, Barbara

  2. Evan, You’re gonna have so much fun when Duke plays Wake.

    But I’m thinking baseball at the moment, as that child has hit a grand slam!!!!!

    Love, Mara

  3. To Evan and Michelle – congratulations on the great news from the Chimera test!!! I hope your level of stress has been lightened and replaced with a huge level of relief. To Zoe – you’re such a fighter, a strong little woman – if this fight you’ve had for this short of life means anything, you are going to be a force to be reckoned with throughout your entire long life. Good luck to your whole family that things continue to improve as they have!!

  4. What wonderful news! Gene and I are so happy for all of you. What a great birthday present for Maya.
    Margaret

  5. To everyone who reads this blog,

    I have written a letter to my contacts about Zoe’s illness and Evan’s wonderful blog. I know he and Michelle want to get their blog out to as many people as possible, so that it helps as many people as possible.

    If anyone who reads this comment wants to do something similar and would like to see what I wrote, please email me at evansmara@hotmail.com. I’ll send the letter on to you. You are welcome to use any or all parts of my letter. Or none of it.

    I thought it might save you time or encourage you to send something to your contacts, so that this terrific blog starts circulating, as is their wish.
    Best, Mara

  6. Evan and Michelle,

    What wonderful news!!! I know you have been waiting for these results from the beginning and praise God for the great news!!

    I hope Maya had a wonderful birthday and I am so sorry we were unable to be there!!

    Love to you all,

    Gail

  7. What a GREAT day!!!!
    Yea Zoe!!!!
    Happy birthday Maya!!!
    You are all an inspiration to me!!!
    Hugs all around!!!

  8. This is amazing news! Once again showing what an exceptional young lady she is!

    Congratulations to your whole family and wishes that you can return to normalcy sooner than later!

  9. Evan and Michelle:

    What wonderful news! We are so happy to hear that little Zoe is making such great progress. Your blog is so inspirational.

    Happy birthday to Maya!

    Catherine

  10. HI MY NAME IS VANESSA AND MY 8 MONTH OLD HAS HLH IS THERE ANY INFO THAT YOU CAN GIVE ME TO COPE AND ARE THERE ANY QUESTIONS I CAN ASK THAT CAN HELP ME THROUGH THIS?? SHE’S WAS DIAGNOSED JULY 18TH HER SECOND MONTH MARK. THESE SYMPTOMS OF HLH HAPPENED RIGHT AFTER SHE GOT HER 2ND ONTH IMMUNIZATION SHOTS I WONDER COULD THAT HAD BEEN THE REASON PLEASE RESPOND BACK I WOULD GREATLY APPRECIATE IT

  11. Hi Vanessa,

    Immunization is a vitally important part of protecting children, and I feel confident saying it has nothing to do with having HLH.

    By my understanding all HLH under the age of about 2 years old is the result of a genetic quirk, it has no external “cause”. It can be triggered by an illness, but children who have the genetic form will get sick and need treatment no matter what.

    I am very sorry to hear about your child, I know how hard it is and coping is very difficult. What I can say is that if she has been diagnosed then she has a very good chance of making it through with prompt, proper treatment and a transplant.

    If you have specific questions please feel free to email me at hlhjournal@gmail.com and I’ll do my best to answer.

    Best of luck to your family,

    Evan

  12. I an student and was searching all over the net to understand what this Chimerism testing is for and what the results mean.
    Thank you Evan for 1- Giving the good news and 2- Explain the test so well. You would have made a great teacher!

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