Thought we would showcase in one place all the clips of Idan and his match skills we have posted to Facebook:
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!
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!
Wishing everyone a Happy Holiday Season!
There is still time to help us reach our $10,000 fundraising goal. Donate today here: www.hyperigm.org/donate/ or here: https://www.facebook.com/donate/10102802141373022/
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
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.
They say that if you “make it” through the first 100 days (meaning you (1) survive, and (2) have no major complications), your chances of long term survival and overall health are very good. In the past couple years, we have watched too many children succumb to complications from transplant, and have learned of too many more who have lost their lives as a result of Hyper IgM. To say that it was an easy choice to undergo transplant – again – would be wrong on a fundamental level. But we held Idan’s and each other’s hands as we thrust ourselves into the unknown, the vortex, hoping beyond hope that all three of us would come back out the other side.
Today marks the 100th day post-transplant for Idan. We are back in New York, and on the other side of a world of viscous complications. Today, we celebrate that Idan is alive, that he is well, and that he has made it through the darkest days. The next phase of his recovery will still require frequent doctor visits, isolation for at least one year, and many challenges as his immune system slowly recovers, but we can breathe some relief knowing that the life-threatening complications are significantly less likely now than they were up until this point.
In recognizing the meaningful milestone we have reached, we also must pause to recognize the sheer impact of those 100+ days. Going through the vortex was one thing, coming back has been quite another. As you might have noticed from some of our posts, we all grew to love our time in Seattle. For four months, it was the three of us against the world. We struggled together, and we persevered together. We soaked up every single minute we had together, discovering new places, enjoying nature and fresh air, going on adventures, and simply enjoying each other’s company day in and day out. Coming back to New York has been a thrill too – reuniting with our family and friends, sleeping in our own beds, getting back to a normal routine filled with normal things like going to work, scheduling babysitters, walking to the supermarket, as opposed to the hourly medical decisions and actions that commanded the majority of our time in Seattle. But it is also “Zombie central” as Akiva likes to say – one of the densest most crowded place on earth where germs are integrated in the very fabric of this city. And hardest of all is the time we no longer spend together. Idan misses Seattle, as do we. All of a sudden we are back to where we started and it feels incredibly surreal, as though we never left, and as though the whole thing had been a dream.
It turns out that the first 100 days are just the beginning of a very long recovery – both physically and mentally – that necessarily accompanies these sorts of experiences. We may struggle at times on this side of the vortex, but we are uplifted by so many things. For one thing, we are so proud of Idan (and “proud” seems to be such an inadequate word to describe this feeling). He has endured, persevered, and thrived through an experience no 4 year old should ever have to go through. Importantly, we are overjoyed by the fact that, today, of all days, Idan is surrounded by his very large extended family, enjoying pizza and chocolate cake, and laughing like he has not a care in the world.