Author: Admin

Somehow, despite all odds, here we are with a healthy happy child on his way to kindergarten. We are still waiting to see whether Idan is cured, but his immune system is showing us very positive signs that it is ready to be put to the test. This summer, we begin a slew of procedures and tests as part of Idan’s one-year follow up. We started this process on Friday – right where it all began.  
It felt a bit unreal to be speaking to the very same physician who first spoke to us in the ER, when I was cradling my weak and breathless baby, feeling what I can only describe as rage. That feeling you get when the world buzzes around you like a tornado, threatening to take everything – everyone – you hold dear in an instant. Dr. Worgall came down to see us after reviewing Idan’s chart. He pulled a chair and sat directly in front of us, and began speaking slowly and carefully, reading the rage (fear) on our faces. He told us that Idan was breathing far more rapidly than his little body could handle, and if we didn’t get to the bottom of this, his lungs would eventually tire out and simply give up. He admitted he did not know why Idan was so sick, but told us that, “common things being common,” he would look for the things that are common, and although there was a long list of potential other causes, he did not want to speculate that the cause was something more rare unless he had a reason to. Shortly after that conversation, because of the severity of Idan’s clinical symptoms, we were taken to the pediatric ICU.  
As Idan’s breathing deteriorated over the next 48 hours, the intervention necessary to support his lungs intensified. Dr. Worgall was worried there might be some bleeding in the lungs, and recommended we do a bronchial lavage, a procedure requiring intubation. When the procedure was over, the ventilator became a permanent fixture in Idan’s airway until his underlying condition could be sufficiently treated and his lungs regain their strength. When the results from the lavage came back, Dr. Worgall and the PICU attending told us that Idan had PCP, a rare pneumonia with a 50% survival rate. Now that we had a diagnosis, we could treat it, and increase Idan’s chance of survival. To this day, we believe Dr. Worgall was instrumental in keeping our son alive.  
It had been over 4 years since we had seen Dr. Worgall, but when we saw him yesterday, we greeted him as old friends. It’s amazing the bonds you make along a journey such as ours. Dr. Worgall’s face lit up with joy at seeing a grown Idan, healthy and strong. He told us that he often speaks to his students about Idan and our family – from the difficult diagnosis, to the arguments with the residents and PICU team when things seemed to be spiraling for our baby boy in the first couple days after arriving in the ER. The experience was almost as memorable for him as it was for us. 
While a reminder of a painful time, the visit reinforced how far we have all come from that day in the ER. Idan is a healthy boy, despite having had two stem cell transplants and nearly dying from pneumonia. And we formed the Hyper IgM Foundation, which has already had a positive effect. The Foundation recently gave a grant to a promising study on gene editing for Hyper IgM, and has reached 150 families, often in remote locations, giving them comfort and a vibrant and close community. Importantly, the Foundation is now a powerful and recognizable voice in the medical community – an active participant in the dialogue about how to better diagnose, treat and cure Hyper IgM. 
A couple weeks ago, we found ourselves at the Immune Deficiency Foundation’s conference in Disneyland. Idan met other kids and families with Hyper IgM, including a little boy his age. Having just received clearance from the doctors to go swimming and be in crowds (for the first time since his diagnosis after that hospital stay four years ago!), we made sure to make the most of it. Idan splashed in the pool, rode the rides at Disneyland, and played with other kids without fear. And, for another first, Idan skipped his weekly dose of subcutaneous IgG. It was the first entire week in our collective memories that Idan was entirely free of needle pokes. 
And Akiva spent his first Father’s Day ever without worry over Idan’s future, without fear, without restraint. 
In the next few weeks, we will be raising funds to support the Hyper IgM Foundation in honor of Idan’s transplantversary, and hope you will join us in ensuring that others living with Hyper IgM have as bright of a future as our little Idan. 
To donate today please visit: www.crowdrise.com/idans-journey-to-a-cure/fundraiser/hyperigmfoundation

Apparently, new-found freedom and normal kid activities come with normal kid injuries!

Idan fell off the playground today and broke his wrist. We took him to the ER at Cornell (a few too many flashbacks as we walked right past the same room he was first taken to 4 years ago when he had PCP). As usual, they took very good care of him there. We had our pediatrician call ahead to let them know he was post-transplant and they cleaned a room for him and had it ready within minutes after we arrived. Idan spent the first few hours in a lot of pain until they could do the procedure to move the bone back into place and set a cast. Idan was stoic as usual, and put on his brave face as he got poked and prodded right where it hurt. The procedure to set the bone was successful, and the doctors expect the bone to heal nicely.

After a long and exhausting day in the ER without being able to eat until 6 (at which time he gobbled down a PB&J sandwich), Idan was able to come home just in time for a nice chocolate treat for dessert.
Hopefully, we are looking at only a few weeks of this cast. Once his bone heals nicely and fully, he can finally get back on track with kids activities.

It’s been a while since we last wrote, and as in the past, no news is good news. At the end of April, we tested Idan donor chimerism again. In a reversal from the March results, which showed a dip in chimerism, the donor t-cells went back up to 89%. We were very pleased with his results. His other cells have been dancing around, but overall, if this stays the case, Seattle believes this may indicate a stabilization of his graft. Of course, as always, it is too early to know for sure, but as we approach the year mark for Idan’s transplant this July, we will feel good if his grafts stays at this level. 

The next step we are starting to explore is getting Idan off the last few medications he is on and maybe entertaining a break in his immunoglobulin infusions. We are waiting for word from Seattle if they feel it is a good time to do this. As you may recall, Idan had been getting weekly infusions of antibodies since his diagnosis at eight months old. These antibodies have been the main thing keeping him safe all these years, but with his new donor immune system, it may be time to see if he can produce his own antibodies and make a response to vaccines. This would be the main test for a functioning immune system. 
In the meantime, our NY transplant doctor has run many functional tests and feels like it is safe for Idan to start partaking in normal childhood activities. Last month we signed him up for soccer classes and he got to be with other kids his age for the first time. We started to get reacquainted with play grounds, which Idan has missed out on for a long time, and he is loving exploring all the different ones in our neighborhood. We got the green light for him to play in the grass, pick up twigs and sticks, acorns or rocks, things that may seem mundane but Idan has felt excluded for far too long from these childhood activities. Think about that for a second. Idan is almost five but, fearing unknown bacteria, fungus or parasites that lurk in fresh soil, Idan has never picked up a stick or rock in the park in his life! He gave a big bashful smile when we told him he can do this now. 
Up next we have a lot planned. First, he and we are open for play dates with kids both inside and outside, so let us know if you are Upper West Side folk 🙂 Next month we will head to to Anaheim, California for the National immunodeficiency Foundation Conference and enjoy a couple of days in Disneyland! Yes, you heard us, Idan is going to Disneyland!!!! None of us know what to expect, but we are all excited for the adventure! 
As always, we are thankful to all of you for following our journey and supporting us and our cause. Look out for Idan’s One Year transplant anniversary in July along with a fundraiser for the Hyper IgM Foundation (or as Idan cutely calls it, the Hyper GGM Foundation) in honor of Idan’s excellent health. Our hope is to break last year’s record and help accelerate a cure for all children with Hyper IgM Syndrome.

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.