Support the Hyper IgM Foundation!

Donate Now

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.

Day 100: Reaching a meaningful benchmark. 

​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.

Day 92: Leaving Seattle. Again. 

Tonight we set out on our journey home after exactly four months in Seattle. As Idan pointed out to us, we arrived on June 19th and we are leaving on October 19th. We never imagined heading home so soon, and we are fortunate that everything has gone relatively smoothly until this point. 
We still have a long road ahead of us. Idan’s ANC is still causing trouble by dipping low every week or so. This means we need to monitor his neutrophils closely and give him GCSF shots when they drop to the 500 range. After getting a shot last Monday he went only one week before needing another one this past Tuesday. Luckily for us, Idan is a super super champ when it comes to needle pokes and would put to shame most adults to shame with his courage. Going forward, we will be doing these shots at home ourselves, as the set up in NYC is a bit different. 

We will still have to go to Idan’s NYC doctor once or twice a week for check up and blood draws and monitor everything closely. Idan’s immune system is still a long way away from fully reconstituting, so we will continue to be in isolation for the next year. 

Speaking of blood draws, Idan got a special treat this past week. As you all know, Idan is obsessed with playing doctor and performing procedures and blood draws on all his stuffed animals. This week, during his last poke for a blood draw at the clinic, the nurse asked him if he wanted to connect the vial to the vacutanor himself. Idan is an expert. He knows from practice how to insert a vial into a vacutanor and draw blood as he has done it a million times on his “friends.” With a bashful smile, Idan grabbed the vial in his right hand, while the butterfly needle was in his left arm, he stuck the vial into the vacutanor and drew his own blood. He got to do a 2nd vial too. He could not have been happier. 

Tonight we begin the long journey home and hope to avoid sneezing people at the airport. This time tomorrow, we will be tucking Idan into his very own bed. Goodbye Seattle, hello home sweet home.

Day 85: It’s been an eventful week – oh, and we’re going home!!!

Let’s start with the good: On Tuesday, Idan had his central line removed. This is a big deal, a very big deal, and we are very happy to say goodbye to that line. The central line is a long tube that was placed into Idan’s chest on July 11th for the purpose of drawing blood and delivering medications and fluids without having to place IVs – not to mention delivering his new stem cells from his donor on transplant day. The central line is a necessary evil during transplant. Not only has it saved Idan from (taking a guess) hundreds of needle pokes over the past 4 months, it has also delivered some of the more toxic meds like chemotherapy in a safer way. However, the central line poses a significant risk of infection and was a constant source of stress. Did we mention it is a long tube that hangs out of Idan’s chest? Yup. It requires constant vigilance to make sure the line stays clean, and does not get pulled, kinked, or damaged (boys will be boys). Every night before he took a shower (baths were not an option for Idan), Idan’s line had to be meticulously wrapped in a special way so it did not get wet. Once outpatient, Akiva and I needed to flush the line ourselves every single night. Perhaps the most difficult of all, though, was the dressing change. At least once each week, Idan would sit patiently on Akiva’s lap for about an hour while I cautiously replaced the dressing over the line. The last few dressing changes have been especially difficult, as Idan’s skin under the dressing became more and more raw. He would scream in pain when we started the change, but somehow he would muster up enough courage to sit still through the pain. It was emotionally draining for all of us to complete that important task every week. So on Tuesday, the three of us marched gleefully into the hospital at 6:00 a.m. to remove his line. Idan did very well through the procedure and was thrilled to have no more dressing changes. He was especially excited when he discovered a “cool” coincidence on the way to the hospital that morning: “Mommy, do you know what’s so so so so soooooo cool?? My central line surgery to put in the line was on July 11th, and my surgery to remove the line is on October 11th – the same day!!!” Idan’s world lately revolves around numbers, so we were very happy he found some symmetry (we often hear how children get attached to their lines as though they were limbs, and that the removal of the line can be traumatic – thankfully, not for Idan).
Taking a step back – why the line was removed. Idan is doing very well. He does not receive any medications or fluids intravenously, and he tolerates blood draws (and all other pokes) very well. When balanced with the risk of infection that a central line poses, we all decided that the time had come to take it out. Importantly, Idan’s team of doctors thinks he is ready to go home to New York, and we all felt more comfortable making the trip home without the central line in place.

 

In the spirit of saving the best for last, we have had some unsettling blood tests lately. Idan’s ANC (absolute neutrophil count) is still dipping every couple weeks, which is concerning because it should have “recovered” to a more stable and higher level by this point. The cause can be anything from a virus to a drug side effect to graft failure, but none of the tests ran over the past few weeks have been able to pinpoint the cause. In the meantime, Idan is getting an extra shot called G-CSF every time his ANC drops below a certain level, and we just have to hope it eventually recovers. The second unsettling test was his latest chimerism results. The chimerism, which indicates how many of Idan’s cells are comprised of donor cells versus his own cells making a come-back, was tested three times. The first time, Idan’s t-cells were at 92% donor, and by the second time, they went up to 96% donor. When we tested them on Monday, however, they dropped slightly to 91% donor. Normally, this would not raise too many alarm bells, but Idan has Hyper IgM, which we are learning can make it difficult for even a good graft to stick, and he has a history of rejecting his graft. So, in the words of Idan’s transplant doctor, the results are not necessarily worrisome, but they are unsettling. We are going to follow it closely and hope it stabilizes and that Idan’s old immune system does not make an unwelcome come back.

 

And now, for the best news: We are leaving Seattle next Wednesday and will, at long last, return home. It has been quite a journey here in Seattle, and we’ve had our share of ups and downs, but we feel incredibly thankful for the good fortune we have had out here. The Seattle Children’s Hospital and Seattle Cancer Care Alliance are two exceptional centers that have treated Idan with incredible skill, but also compassion. The community here has welcomed us and taken us in for the second time, and once again made us feel like home. And the city, this wonderful beautiful city, has been an oasis in a time of immense pain, stress and uncertainty. But we are overjoyed to be going home – back to our family, our routines, our lives. We still have months of recovery and isolation to go, and just about anything is possible at this point, but we rejoice for being fortunate to have been able to make it to this point with a happy, healthy Idan.

Day 75: Shana Tova!

We wanted to take a break from our regular updates to wish everyone a very happy, healthy and sweet New Year!Tonight marks the beginning of Rosh Hashanah, a Jewish holiday celebrating the new year, 5777. Quite unlike the usual new years’ festivities, which may involve boisterous parties and champagne, the Jewish new year is typically celebrated as a community in the synagogue and then at home with family and friends. It is a time of reflection on the past year and of contemplation of the next. The next ten days are referred to the “Ten Days of Awe,” culminating in Yom Kippur, the Day of Atonement.

In the spirit of this holiday, we reflect back on this past year and think about our hopes for the next.

Last Rosh Hashanah, we were in New York with family, praying for Idan’s first graft to stabilize to avoid a second transplant. We had just launched the Hyper IgM Foundation and began what would become the most important work of our lives.

Over the next year, we watched Idan’s graft and chance of a cure slowly slip away. We made the very difficult choice to undergo a second transplant. Once again, we uprooted ourselves and relocated to Seattle. Idan had his second transplant, and, after an exceptionally difficult summer, we now have hope that our little boy will be celebrating Rosh Hashanah for many years and decades to come.

Through our work with the Hyper IgM Foundation, we have connected with patients and physicians around the world to advance research, improve outcomes and even explore new ways to cure this rare disease beyond the risky bone marrow transplant. We have seen tremendous progress on each of these fronts, which I personally credit Akiva’s gargantuan efforts in this regard. We have also suffered unspeakable loss in our patient community, as three boys with Hyper IgM passed away in the short span of just a few months due to complications following bone marrow transplants that were meant to cure them.

It has been a year of heartache and grief. But it has also been a year of hope, of progress, of joy. We hope this coming year will bring the cure we have been waiting for, and we will keep fighting and raising awareness for Hyper IgM to make sure that every child with this disease has hope for a long and healthy life.

Importantly, we wish all of you, who have held our hand, who have encouraged, supported and listened to us, a very happy and healthy New Year. May you and yours have a meaningful and sweet year, and may you all be written in the book of life.

All our love,

Amanda, Akiva & Idan

Optimization WordPress Plugins & Solutions by W3 EDGE
%d bloggers like this: