Author: Admin

Hi everyone,

 It’s been too long since the last time we sent an email out to Idan’s Army.  Hopefully you’ve been following the blog updates on IdanMyHero.com, latest news, and our Facebook page, Help Fight for Idan.  Regardless, we wanted to check in, give you the latest update and plans for the coming years, and introduce you to Hyper IgM Foundation, Inc., which we recently formed to help others like Idan.

First and foremost, Idan turned 3 on July 20th and is doing very well for a child with Hyper IgM Syndrome. As you may know, Idan’s first bone marrow transplant (BMT) failed, and we have been mourning the loss of his donor’s graft over the last year. The good news, however, is that Idan is a strong and vibrant little man, and his doctors say he is physically ready for another transplant once we give them the go-ahead. Emotionally, it is hard to believe that the three of us will ever be ready to go through that long ordeal again, but the fact that we are familiar with the process will hopefully help us get through the next BMT. In the meantime, we are working with the doctors to coordinate timing – be it this year or next, Idan’s chances of surviving and being cured are significantly higher if the transplant happens before the age of 5.

Idan remains in moderate isolation (which will intensify during flu season, which starts this month, believe it or not).  He has permission to see healthy kids and adults and the ability go on some “adventures” from time to time. He has been tolerating his weekly subcutaneous IgG replacement therapy infusions extraordinarily well, and, true to form, he has thrived, and is a remarkably smart and happy child. Since he cannot attend pre-school he will start receiving home-school services this September. Idan is very excited to start “school” and is eager to learn and advance.

Our lives too have been changed by transplant and we have spent much of our time over the past two years becoming empowered patients and advocates for Hyper IgM and Immune Deficiencies within the medical community. Amanda’s experience has led her to focus her legal career on healthcare and nonprofit law, transitioning out of litigation and into the healthcare practice at her firm Sheppard Mullin Richter & Hamilton LLP.

The knowledge we have gained and the connections we have made in the medical world and among the global Hyper IgM community have inspired us to form a not-for-profit corporation called Hyper IgM Foundation, Inc. Our mission is to improve the treatment, quality of life and the long-term outlook for children and adults living with Hyper IgM through research, support, education, and advocacy.  We are in touch with some of the leading labs across the country that are on the forefront of gene editing for those genetic mutations that cause Hyper IgM and are seeing some early but very promising results. Much of our focus, therefore, will be to support and advocate for research that will further advance these exciting new developments as they hold the promise of a cure for children like Idan without many of the attendant complications of the bone marrow transplant.

In addition, our journey has introduced us to dozens of other families living with Hyper IgM, and parents of children just like Idan, discovering this life-threatening disease much like we did – standing by their child’s ventilator in an ICU – who have found our blog and seek our advice.  All the research, consultations, and connections we’ve made since Idan was diagnosed are now parlaying into a wealth of knowledge and expertise that we can use to help support and educate these families and their doctors as they navigate the quagmire that is Hyper IgM. In that regard, we have established a scientific advisory board that is going to help guide us in developing best practices, treatment plans, and resources for the relevant patient and medical communities.

We invite you to visit the Hyper IgM Foundation’s website, and encourage you to share our story and vision, spread the word and help more children like Idan.

All our best, and, as always, thank you for being part of Idan’s Army.

Akiva & Amanda and Idan

Idan’s New Ride!

 Today Idan turns three, which is a big milestone in every toddler’s life, and Idan is no exception. We are thankful that this past year Idan has kept healthy, and, despite the loss of his graft, we are happy with how well he has been doing and dealing with Hyper IgM. As mentioned many times, our journey to a cure is still at its beginning, but we have learned to enjoy and cherish the small moments of joy along the way, and the small wins that Idan has earned.
This summer we have decided to be more adventurous. With flu season over, and many less cold viruses around we have taken more risks and found ways to allow Idan to get out more and explore. The truth is that most of this is because Idan has turned out to be an extremely conscientious and well behaved kid who has learned to navigate the outside like a germ-busting pro. There are not many kids under three who have learned not to touch their face, not to touch surfaces and to ask for Purel and antibacterial wipes after a fall before asking for the needed healing kiss. You can see the clip below of Idan’s thought out approach to sitting on a stone in Central Park, a method that would put even the best of OCD germaphobes to shame.

This has allowed us to enjoy the summer more and even journey out of state for one of the more memorable experiences Idan has had so far. In late June, we travelled down to New Orleans to attend our second Immune Deficiency Foundation National Conference. We were lucky enough to have Idan’s aunt, uncle and cousin join us to entertain Idan during the long days Amanda and I spent attending sessions and meeting with experts. Idan is such a good traveler he was even excited to receive his weekly infusions of IgG’s in a new setting while playing with the swag he got from the pharmaceutical companies that make immunoglobulin. After three days of swag collecting with his uncle and waking up to his cousin’s knocks on our door, Idan was ready to move into the Hyatt and did not want to return to New York. He has been asking to go back to New Orleans ever since.

For us this conference was a wealth of information and filled with important meetings with some of the biggest names in Immunology. As readers of this blog might recall, it was at this conference two years ago in Baltimore that we met with Dr. Torgerson from Seattle and learned for the first time about their transplant protocols and started entertaining the idea of traveling out there for transplant. This year we returned to the conference and had a chance to sit down with many of the experts we had consulted with two years ago and update them on Idan, and hear about the latest data and studies (soon-to-be-published) on Hyper IgM. No longer are Amanda and I newbies to the immune deficiency world seeking out any information we can find. This time, we returned as patient experts seeking to influence and inspire these experts to bring more focus to research on Hyper IgM and our patient population. 

This weekend we were able to celebrate with family and friends in Central Park and give Idan a relatively normal birthday party for a three year old. Idan is of course not a normal three year old. No, he is the size and weight of a 4 year old with the wit and sense of humor of much older kid. He speaks in full sentences and wants to know why and how everything works. He might still be behind with outdoor playing skills but he can name more shapes than I can, and build a pretty impressive magna tiles castle. He knows that he had a transplant in Seattle, and when watching clips of our time there, he asks to go back, but this time “as a kid.” We tell him we will, but he of course cannot know everything that that really means yet. Until that day comes, we will continues to count our blessings, and plan for the next adventure.

Once again it has been way too long since our last update. With blogs like ours, it turns out that when there is no news, it mostly means that is good news. Since our last post we got results from another chimerism test back in late December which did not show much change. T-cells were still in the 20% range but Myeloid cells dropped even further to 5%. We assume that by the next test nothing will be left of his donor Myeloid cell line (that is the majority of the bone marrow, which includes most of the red and white blood cells). As always we still hope that he might hang on to his 20% donor T-cells, but it is highly unlikely. We have decided to hold off on a 3rd DLI and trip back to Seattle and have been planning our life around the idea that we will head back to Seattle in 2 years for a second transplant.

In the meantime Idan has essentially gone back to his Hyper IGM state. It some ways this is a relief, as most the dangers of the post-BMT year are behind us. We have gotten off the majority of medications and immune suppressants that he was on for almost 15 months.  He only takes some supplements daily and the Bactrim prophylaxis antibiotic to prevent the return of the PCP pneumonia that landed us in the ICU almost two years ago, and his weekly SubQ IgG infusions (antibodies).  We keep him safe with his SubQ and by limiting his exposure to kids and people in general. This has made the winter very hard on us, as flu is everywhere and we sometime go weeks without seeing close family if someone has a cold. And even with all that, he still picks up a cold virus now and then, which goes to show how vulnerable he still is.  The Spring and the end of flu season will be a blessing, and we hope to be able to get Idan out and about a little more.

Doctor visits and blood tests have also been more spread out now and that is a very welcome change. Just three months ago we were going in twice a week for check ups and blood tests and Idan was losing his patience for all the pokes and prodding. If we can manage to keep him healthy we only have one more poke this month to send blood back to Seattle and then we should be able to have an 8 week break from doctors and blood work. We hope 🙂

Idan’s language skills have really advanced in last three months Amanda and I feel like he is more of a 4 year old little boy then a 2.5 year old toddler. His favorite activity these days is putting together a large floor puzzle of the United States, and he excitedly yells “Seattle!!” every-time he sees the state of Washington (at least we know he has warm feelings towards his experience there).  He is very active and happy, and we count our blessings every day that the transplant did not hold his development back significantly. All we can hope for now is to continue to be able to keep him healthy and safe and give him as much of a normal life as we can as we plan for our next big adventure.

Thanks to all of you for checking in and following our story. We hope to keep the updates coming regularly and wish you all a happy Valentine’s Day!

Here is a short clip from his last meetup with his little cousin:

 
Idan putting together his large flood puzzle of U.S.A