Category: Idan’s Blog

Day 227: Finally, some good news!

For the past five years, we have watched our baby boy suffer in so many ways. Today, for the first time in what seems forever, his future seems bright and unlimited.  
The latest chimerism results are in, and, without getting too excited, it looks like Idan’s graft may actually be starting to stabilize! Since we took Idan off immune suppression in December to stop the graft from continuing to fall, Idan’s t-cells have inched their way up, one percentage point at a time, from a low of 79% donor to 85% donor as of last Monday. 85% gives Idan an excellent chance of a cure, so we are hoping that this latest trend continues! What has been worrying us is that, until last week, Idan’s myeloid cells continued to fall, which threatened the long term chances of the graft sticking around. So we were very happy to see that his myeloid cells did not drop this time, and instead went up by a percentage point!  
We are cautiously optimistic that things are finally starting to turn around. Idan is healthy and joyful and has a full head of curly and unruly hair. Transplant and chemo feel like a lifetime ago to us, but every once in a while there are reminders. A couple weeks ago, a song came on the radio that Idan used to love, and he begged us to turn it off. We asked him what happened, and he explained that the song made him feel sick, like he was back in the hospital room getting chemo. Memories of the song playing and me dancing to it to cheer up Idan when he was having a particularly painful day came flooding back to me, and I understand why that same song now has the exact opposite effect. And last week, Idan told us he didn’t want to wear a tank top because sleeveless shirts make his neck hurt. He later explained that he thinks these shirts hurt because the last time he wore them was right after his central line surgery, when his neck was in terrible pain for several days. It turns out that was the last time he wore a tank top. We are 227 days post-transplant, and I guess we were hoping that Idan, who turned 4 the day after his transplant, would forget some of these memories. Then again, these experiences are part of him, and even if we can say goodbye to Hyper IgM, I want him to always remember a few things about transplant:  
1. Idan didn’t just survive, he endured.  
2. He showed the world – and himself – how strong, brave, and fearless he is.  
3. He got to the other side. Sometimes that’s all you can do when things are really bad. 
4. He is loved, cherished, and supported. His parents and family never left his side, and never will. 
5. He witnessed the goodness in people all over the world. From the staff at the hospital, to the strangers in Seattle who embraced us into their community, supported us, and gave us a second home, to the army of supporters from around the globe, he saw and felt the awesomeness of helping others.  
So, even if it can be painful to remember, maybe it’s not such a bad thing that we do.  
While we are waiting to see whether this good trend in Idan’s chimerism continues, we are slowly starting to expose Idan to the happy carefree side of childhood. Last weekend, he rode on a carousel for the first time ever. As we spun around and around, we waved to Idan’s grandparents watching from the side, and I smiled to my mother and shared tears of joy with her in seeing Idan enjoy just being a kid.  
After quietly taking this new experience in, Idan turned to me and said, “Mommy, I will never forget this day.” Neither will I.

Day 176: Cautiously Optimistic:

Over the weekend we heard back from Seattle on the latest chimerism test. For the first time in over a month, his T-Cell line did not fall but rather climbed back up 2%! Idan stopped all immune suppressants (tacro) a few weeks ago and perhaps this result is signaling a stabilization of the donor graft. Some of his other lines still fell a bit, and overall, it is too soon to conclude that we are out of the woods. Still, it was a nice surprise to start the New Year off with as Amanda and I have been expecting more bad news. We will send out more chimerism tests at the end of the month and hope to see continued improvement or stability.

Idan is still quite busy with lots of doctor visits and blood tests and we continue to be in isolation for the time being. We are of course also planning ahead, and, based on these latest test results, we are on track to send Idan to kindergarten in the Fall. This of course has Amanda and I freaking out, as we have become experts in raising a medically challenged child but have neglected to learn anything about normal parenting in New York City. Between the complicated registration process, Gifted & Talented testing and the lack of good zoned school options, we are definitely feeling a bit overwhelmed. Aside from the regular stress of choosing your child’s first school, we’re faced with the daunting task of finding a school that can work with us to make sure Idan stays healthy on the one hand, and that will challenge this ridiculously smart little man. I am sure it will all work out and we will find a good program for Idan to attend next year, as he enters the real world. Regardless of how overwhelming this process is, the fact that Idan will soon be participating in these “normal” things, like story time with classmates, play dates, and riding the school bus, is something of a miracle for this little boy who battled for his life only a few years ago when he had PCP (pneumonia), and has been through two bone marrow transplants since then. Only a few short months ago, Idan told us that he was willing to go through the pain of the bone marrow transplant because he knew that, if he did, he would be able to go to school with other children. Here’s hoping his dreams come true!

Day 154: Waiting and Watching

img_8307Day 154: So far no signs that Idan’s chimerism is improving or even stabilizing; we have been patiently waiting for his donor cells to get back in the game.

As you recall, the chimerism is the percentage of donor cells vs Idan’s old immune cells. Ideally, post stem cell transplant there is no “chimerism,” but rather 100% donor cells on all four cell lines. Sadly, with Hyper IgM Syndrome, full donor engraftment seems to be hard to achieve. For Idan, the most important cell line is the T Cells or CD3, this is where his HIGM defect is. We started in the high 90’s but have watched it fall again in the last couple of months.

We’re testing Idan’s chimerism every 2-3 weeks and, for now, the chimerism still is falling at about the same rate. When Idan’s donor t-cells first dropped, they fell 6% over the course of 4 weeks, and another 6% over the next 4 weeks. Then another 5% over 3 weeks, and now another 2% over 2 weeks. If you plot these numbers on a graph, the trend is about the same with some very marginal (as in, single digit) improvement over the last two weeks, which is within the margin of error so not meaningful.

The plan we put in place last month was to taper off his immune suppressant (Tacro) in order to allow his donor cells to mature and perhaps put up a fair fight against Idan’s old T cells. There is no right or wrong approach, as we discussed in our last post.  After a few weeks, we are almost off Tacro, and expect that things will continue to get worse for now since his donor cells are only starting to wake up after being suppressed since transplant. So far he has not had signs of GVHD (which is the main risk of getting of Tacro and allowing his donors cells to mature and grow), and we hope he stays clear of this complication.

Sometime in January we will have to decide on next steps. If the donor cells continue to drop, we may elect to give Idan a donor cell boost (DLI) to tip the scales in favor of the donor. Of course this DLI comes with an even higher risk of GVHD (he got acute skin GVHD last transplant after the DLI) and there is no guarantee this will work.

For now we are just hoping that we can stabilize his graft with a good high mixed chimerism, which would be enough for Idan’s cure. Hoping that 2017 turns out to be the year we finally have the cure we have been waiting for (sad thinking how many years we have been saying that).

Wishing you all a happy and healthy New Year!

 

Idan’s Story Is Featured In An Article On His Transplant Physician

When our journey first started, we had no idea how our lives would be enriched by the incredible people we met along the way. Dr. Laurie Burroughs is one of the fiercest and most compassionate individuals we have ever met, and she has tirelessly fought side by side with us for a cure for Idan. Her research at Fred Hutch is precisely what drew us to Seattle for Idan’s transplant, but her care, expertise and creativity is what makes us feel confident that we made the right choice. We are so lucky to have her as part of Idan’s team! Article here and below: https://www.fredhutch.org/en/news/center-news/2016/11/stem-cell-transplant-kids-rare-non-malignant-diseases.html

Day 126: Bad November. 

Well I am sure we are not alone in wishing this month could be rewritten. As though to prove nothing in this world is safe, Idan’s graft has been slipping, showing early signs of a possible rejection. We got results back from the chimerism tests last week. This is the test that checks what % donor vs % host (Idan) are in the cell lines emerging from his bone marrow. For those who appreciate the numbers, Idan’s t-cells (the ones we need the donor cells to replace) were 92% donor at Day 27, 96% donor at Day 55, 91% donor at Day 79, and 86% donor at Day 119. For any other transplant, there would be no reason to panic at this initial trend, but this is no ordinary transplant. Because of Idan’s history of rejecting the first graft, and the fact that X-Linked Hyper IgM patients reject at a higher rate than other immune deficiencies, this drop of 10% over the course of two months is reason to panic. 
Unlike the last time, we have a robust amount of donor cells to begin with, so the hope is to stop the graft from falling further, and maybe save the graft. As long as we can keep a good chunk of donor cells (around 20-30%), a cure is still possible.  

The problem is, once it starts falling, there’s no easy way to stop it. It comes down to four options: 1) increase immune suppression, 2) stop immune suppression, 3) give Idan a Donor Lymphocytes Infusion (DLI) if the graft continues to fall, or 4) do nothing and see what happens. Well, we will never be the “do nothing” kind of folks, so we are fighting like hell to keep this graft and save our son’s life. After much discussion with our team in Seattle, who have also consulted with others around the world, we came up with a plan last night. We will be stopping immune suppression with a relatively quick wean of the Tacrolimus he is currently taking, with the hopes that, without this suppression, his donor cells will wake up to the threat around them and begin maturing so they can better defend themselves. In theory, the immune suppression levels the playing field and suppresses both sides until they learn to play nice with each other. With Hyper IgM, however, the t-cells Idan was born with seem to be more aggressive than your average t-cells (perhaps compensation for their other major flaws), and the immune suppression does not seem to be able to quiet them down sufficiently. So hopefully, by easing the restraints on the donor cells, we are allowing them to fight back so that the old cells cannot further erode the new graft. The concern here is that removing the immune suppression might also accelerate rejection since now there is nothing holding Idan’s old cells back. Another big risk with taking off the suppression is graft vs host disease (GVHD), which will complicate things further. The experts around the world are split in half, with one half insisting increasing suppression is the only way, and the other half insisting that stopping immune suppression is the only way.  

If this step does not work, we would then do another DLI (infusion of more mature donor cells). This option likely requires another trip to Seattle (although we are trying to see if this could be done in NY), and requires a surgery to place the line, and also comes with an even higher risk of GVHD than stopping the immune suppression. We will check Idan’s graft again next week after we’ve had a week of less immune suppression. 

Hoping for a better end to this miserable month! Happy Thanksgiving everyone. Hug your kids tightly, and enjoy your time with your families. Love to you all.

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