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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

Day 74: Busy/stressful week, but fevers appear to be gone. 

It has been a while since we posted, and a busy week since we got out of the hospital. As you recall we got out of the hospital the Thursday before last because Idan went 48 hours with no fever. The day we were discharged though, his ANC was very low (in the 400’s, but it should be over 1000) and he needed a GCSF infusion to stimulate the neutrophils growth before they let us go home. The next few days were very stressful as Idan kept running warm at night before he went to bed, and we thought we would have to get admitted again. His temperatures flirted with but never quite reached the level of readmission. With the doctors’ permission, and to reduce our own stress levels, we started to wait until he was asleep to take his temperature, since they always went down once he was settled in bed. His temperature seems to be back to normal now, but we do not know what caused the fevers in the first place.

This past week was also a very busy week as we began our “Day 80” evaluations tests. This work-up is essentially a repeat of all the pre-transplant tests we did, and the purpose is to get a status report of Idan’s overall health at approximately 80 days post-transplant. On Thursday at clinic his ANC had dropped below 1000 again and they had us come in again today to recheck his ANC to make sure he did not need another GCSF shot. Luckily it was back up above 1000 so we will check it again on Monday. Still since something strange might be going on in his marrow, and in order to rule out issues we decided to move up his bone marrow aspiration and biopsy procedure (part of the Day 80 evaluation) that was scheduled for next Friday to this Tuesday. This will give us a better idea of what is going on both with his ANC and his temperatures.
Still, despite the 5 day hospital stay and the many appointments we have had, Idan has been feeling well and enjoyed the long visit he had with his grandparents. He got to enjoy many adventures between and after appointments and a lot of play time with them. He is looking forward to to his aunt (Doda Chana) visiting next weekend, and for another visit of Amanda’s sisters the weekend after.

Day 64: Just when we started to feel homesick, Idan had some surprise visitors :)

Well, it finally happened. After three months, a wave of homesickness came over me like a dark cloud last night. This hospital stay marks Idan’s fifth. It’s the third one where we are searching for a diagnosis. With every hospitalization comes the fear of not knowing what happens next, what side effects will develop, what tolerance Idan will have for the pokes and probes, and of course, the enormous amount of energy it takes to put a smile on our faces and all push forward together through the raging fire of illness. So when I took a break and went back to our Seattle apartment last night, I broke down. It all came flooding back to me the second I opened the door to the apartment. The wave of sadness at the realization that Idan, who has been looking forward to his grandparents visiting from Israel since before we even left for Seattle, is now confined to a hospital room during part or maybe all of their stay. The frustration of coming so close to getting to go home to New York, to not knowing how this set back will affect the length of our stay. The worry and exhaustion of not knowing why his fevers are persistent and sneaky, as though playing with his little heart strings. The hour Akiva and I spent pleading with Idan to take his oral meds without vomiting, trying desperately to undo and reverse the negative association with taking medications in the hospital, after finally getting him past it not so long ago. How cold and empty our apartment seemed last night without the sound of his chatter and giggles filling the rooms. It felt like hitting a wall. 

I broke down so I could put the pieces back together. Remind myself of what we are doing here, remember our strength and all we have been through since the day Idan was intubated in 2013. Piece by piece, reminding myself of all the lows, and how those very same lows led us to all the highs. Idan’s graft is excellent and he is in good health (fevers notwithstanding), and every time he gets knocked down he keeps getting up. It’s no coincidence that Idan keeps singing these lyrics from a Pink song: “You gotta get up and try, try, try, you gotta get up and try.” His resilience astounds me and gives me all the reassurance I need to get up and try. 
Today, Idan’s temperature is *hopefully* showing signs of lowering, and his C Diff appears to be in check. And just in time to really raise our spirits, a couple of surprise visitors showed up at Idan’s door this morning – two of Seattle Sounders’ soccer team players. Idan was jumping up and down with excitement! He asked them a bunch of questions (mostly about numbers – how many players on the team, what player has the highest number, you get the picture) and showed off the soccer field he randomly decided to build with his Plus-Plus yesterday. The soccer players were very sweet and found all his questions pretty amusing. Yes, we’re all starting to feel homesick, but it was a great reminder of how incredibly lucky we are to be here in Seattle and at such a great hospital.

Day 63 – Persistent Fevers:

14329953_1388297231200240_5801254640634437046_nWe have been in the hospital for 48 hours at this point and have not figured out the cause of Idan’s fevers. The first blood cultures taken Sunday night have not grown anything at this point. He spiked another higher fever this afternoon of 101.7 and they took another set of blood cultures to test. This means that we are in the hospital for another 48 hours at the very least, and with every temperature over 100.3 that clock will get reset. Idan continues to get IV antibiotics until the fevers go away, even if the cultures continue to be negative.

Sadly, the side affect of this IV antibiotic has been very loose stools and the return of C. Diff which is not very fun. So we started treating him today with Flagyl again to get rid of the C. Diff and that will continue for another 14 days. Idan is still in a good mood and mostly has had energy but the hospital life (tethered poll, frequent vitals) is starting to take a toll again. In addition, having C.Diff means that we are in contact isolation again so he loses his right to walk the hall or check out the roof garden.

Amanda and I are of course concerned that no cause has been found yet, and are hoping, like our doctor, that this is just a virus that needs to run its course. If fevers continues for two more days or if other symptoms present they will probably order more tests (like a CT and some fungal detection tests) to rule out other types of infections.

We are happy that Idan’s grandparents are here this week so they can help entertain him during the long days in the hospital. We really hope that the fever goes away for good and we can hopefully get out for the weekend, but we are not holding our breath. Hoping for better news soon! Thanks for all the good wishes!

 

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