Idan, my hero

Two days of chemo down, 3 to go. Side effects have been minimal so far, but the hospital setting and painful recovery from his central line surgery has taken a toll on Idan’s spirits, and he has not been his usual happy/energetic self. So you can imagine our delight when his face lit up at the sight of bikes for the kiddos to ride down around the unit. It’s not easy chasing a child with a giant IV pole (which Idan named “Ga”), we have a few bruises on our feet and ankles to show for it, but it’s worth every second to see that sweet smile on his face and to hear that delicious giggle!

From Facebook:

We wrote this explanation three years ago for a blog post before Idan’s 1st transplant. Thought I would repost this now for those following along. Also adding a link to explanation of Hyper IgM from the Hyper IgM Foundation site:
– So What Is a Bone Marrow Transplant (BMT)?
Currently, there is no way to fix the gene mutation that causes Hyper IGM, but there is a way of replacing the bone marrow and giving Idan a new immune system. One can look at the bone marrow as the army base that the immune system (as well as the blood) is created and trained in. The idea is, if Idan’s T-cells cannot produce the protein he needs, lets give him a new army base, with soldiers that know what they are doing. But it is not that simple:
1) Currently the army base in his body is filled with soldiers (even if they’re not too smart), so there is no vacancy for him to host someone else’s army.
2) Idan’s body (AKA, the equipment, building and land that needs protection) is a perfect match with his current army, and no other army (AKA the donor’s bone marrow) is going to know how to work it in the same way.
3) Any gap in the protection that his army is giving him (albeit a lazy one) will open up his body to infections.
4) In order to create a vacancy in his army base so that a new army can move in, the old one needs to be eliminated entirely. The way they do this is with chemotherapy, which has the nasty side effect of destroying the bone marrow, and in our case we want that. However, that process comes with its own risks of toxicity to the body.
With all the risks involved Amanda and I decided that BMT is the way to go. BMT will give Idan the best chance at a long and healthy life. We did a lot of homework and spoke to dozens of bone marrow teams around the world. We decided on Seattle because of the new type of chemo drug they are using (Treosulfan) that is less toxic than others. If all we need is to clear the army base from soldiers why bomb random cities and cause damage to the whole land?
– How will this cure Idan?
First they will use chemo and ATG to eliminate his bone marrow and suppress his immune system. This is what we are doing from Day -6 to Day -1. We then give Idan a day of rest while the chemo in his system continues to clear out all the soldiers from his army bases (including the civilian side of his bone marrow that produces red blood cells). On Day 0 the transplant date, we will infuse Idan with the donor’s army. His army has been found to be a 12/12 match to Idan’s. While this is great news, it is not the whole picture. This new army is not really going to understand how to relate to all of Idan’s body parts, and will most likely still want to attack some of it (this is called Graft ss Host disease, and it happens with 70% of the transplants from unrelated donors like Idan’s). For this reason they use the ATG, and the other immunosuppressants to allow the new army to come into the empty base, but forces it to unpack, train, and start patrolling very very slowly. Giving the new army more time to get adjusted to Idan’s body is a delicate balancing act that will happen over the next year. Since they are letting the bone marrow come in and start working very slowly there is a longer time where Idan is lacking any working immune system and will be kept in isolation as well as be given drugs to protect him.
– So what is next?
Day -6 to day 0 we are clearing his bone marrow and making room. On Day 0 he gets the transplant, and we start counting days until the bone marrow travels to the base and starts setting up shop. We hope to hear around Day 22 that they have reached the base and that would be engraftment. We will then wait until we see his counts go up. The first type of soldier to come back (or, better put, to “grow”) is his neutrophils (which work fine now, but we have to get rid of them with the rest of the army) and they will wait until that level is good enough to get released to isolation at our apartment. This will happen between day 30 and 50 and then we will count to day 100 before they might let us go back to NYC. From then on and until around 2 years post transplant Idan will still have a weak immune system. Our hope is that around one year post-BMT, his new army will be strong enough and acclimated to his body to start reducing the supplemental protections we are giving him. Along the way there are going to be many scary events but we and the medical team will deal with them as they come up.
In a few years, when Idan can go to school, play with other kids, take swimming lessons, play in the park and ride the swings all with the protection of his donor’s army, we will know we did the right thing to ensure Idan has a normal, healthy and happy life.
For more see here: http://www.hyperigm.org/about-hyper-igm-syndrom/

From Facebook:

Day -6: we checked in today at 8AM and got settled in our room. Sadly, not one with a view ( you would think with all our membership points 🙂 ). He received his first two dosages of chemotherapy (Treosulfan & Fluderabine) and did very well. He played play-doh and ate lunch and then played with his new Magna Tiles. Now he is working on the monthly calendar with Amanda. He still has pain from his central line surgery yesterday that is making hold his head to the side and not wanting to use his right arm too much. Hope that subsides soon. Thank you all for your thoughts and prayers.

It feels like we have been training for this forever. Transplant. And now it’s happening. This Monday, Idan will have his central line placement surgery, and early Tuesday morning we will be admitted to start chemotherapy. Six days later, the donor will donate the “cure” in the form of peripheral blood stem cells (PBSC) and the nurse will infuse them slowly into Idan’s central line. Those cells will find their way to the inside of every bone in Idan’s body. The bone marrow, which will be empty as a result of the chemotherapy drugs and immunosuppressants, will host the new stem cells. Slowly, over many many days, weeks and months, the donor cells will set up shop and start producing Idan’s new blood system, including a working immune system.

They say that transplant is like a marathon. It is long, you have to keep a steady pace and keep going. Forward, onward, keeping your eyes on the goal, the cure. Like a marathon, we have been training for a long time for this. Ever since Idan’s first transplant almost three years ago, we knew that one day we might have to run this marathon again. Idan has grown up knowing that Seattle is where he had a transplant, and where he might once again return for his 2nd transplant. He keeps asking us if he will need another one after this, and we keep saying no, that we only really get two tries.

For the last year, Idan has spent countless hours playing with us and all his babysitters doctor. Like training for a marathon, he has been training by pretending to take blood, to get shots, to send blood back to Seattle, or making believe we are taking a flight to North Carolina or Seattle to bring the blood samples there. He has been preparing for this for so long that anticipation has grown to a point that tonight he told us how excited he was to start transplant this week. He was mainly talking about all the toys that he will be getting once he’s admitted to spend the next several weeks isolated in the hospital (the ones that very kind people of Seattle have given him). Still, the excitement is there, we are all excited (and terrified) to finally start the run, the long walk to a cure.

Everything has been falling into place for now. Idan is as healthy as one can be entering into transplant. Once we start it will be hard, things will get bad and ugly and rough. Amanda and I know this and are dreading the long days, watching the chemo affect Idan, making him sick and tired, all in the name of removing his defective bone marrow and making room for the donor cells to grow. There will be many setbacks and unexpected obstacles, but we have been training for this, and we know, no matter what, to keep our eye on the finish line, the cure.

It is an odd thing to fall in love with a city, but we did.  From the people, who have opened up their hearts and homes without knowing anything more than our name and our story, to constantly finding new places to discover and experience, to the lush green scenery that is now part of our everyday life, we love this city, and Idan does too.  My favorite part has to be the fact that, even though every morning we have woken up to cloudy skies, by the afternoon, the sun is shining brightly in an endless blue sky.  It’s as though the sun is so damn persistent that it will burn its way through all the clouds just to shine down on this remarkable city.

I won’t sugar coat it, though.  It’s not easy to walk the same corridors in Seattle Children’s Hospital as we did once before, to pass by that restroom in the clinic where I had a good long cry after finding out that Idan’s first transplant was failing, or to endure procedures where Idan needs to be anesthetized and goes limp in our arms.  It’s not easy.  But we are comforted by all the familiar faces and the very warm greetings from all the doctors, nurses, physician assistants, pharmacists and all the staff who we got to know so well during Idan’s first transplant.

As expected, Idan has no memory of Seattle as far as we can tell.  In a way, it is a reassuring reminder of how young he is and how much of a distant memory this hopefully will all be some day.  On the other hand, it makes it feel like we’re taking an entirely different child through transplant.  Idan was only 14 months old when he had his first transplant.  Now, he’s almost 4.  He doesn’t recognize the nurse who gave him hugs when he finished a brutal dressing change, or the doctors who we have grown to be friends with and who visited him frequently while he was outpatient.  But he also doesn’t remember the four phlebotomists who took turns soothing him and entertaining him while one of them had to poke multiple times to find a good vein in his chubby arms, or the fact that the blood pressure machine never got a good reading so he had to endure multiple “arm squeezes.”  No, he doesn’t remember anything, but perhaps that is the greatest gift of all.  Idan has heard so much from us about Seattle, the transplant, the hospital and the clinic.  He has watched all the videos from the first transplant, and basically has been preparing for this transplant for the past two years.

Unlike last time, where it seemed that he felt that each probe and poke felt like an assault to his little person, Idan is actually enjoying experiencing each new test or procedure – be it an EKG, echocardiogram, bone marrow aspiration, or even a dental exam (yup).  For Idan, the past two weeks have all been one great adventure.

So instead of screaming during blood draws, he sits patiently, counts the vials, and watches the needle going in without flinching.  Oh, he knows it hurts, and he even announces it will hurt before the needle goes in, but he relaxes his arm and chats happily with the nurse while the vials are being filled.  And he opened his mouth wide for the dentist, taking a bet with her beforehand that she would count 24 teeth.

On Friday, he had his first “surgery” to have a bone marrow aspiration.  He’s heard a lot about surgeries (including his 2013 surgery to place his central line, and the one he will be having on July 11^th to do the same thing), and was so excited to have the surgery that he barely flinched when the IV went in right before the procedure started.  In fact, he was disappointed to wake up and discover that they did the surgery without him watching.  He literally woke up in mid-sentence asking me how they were planning to do the surgery in the back if the IV was in his arm and he couldn’t lie on his side.  When he realized that it was over, he kept repeating sadly, “but I saw when they did my IV surgery, how come I didn’t get to see when they did my back surgery?”

Yes.  This is our incredible, amazingly brave, adorable and joyful little boy.  He wants to experience every ounce of everything, and will muster through the pain and discomfort because he is simply too curious to see how it all plays out.

The past week and a half, we have had a slew of various appointments, and we will have several more tests until July 11^th when Idan’s central line is placed.  We will be admitted into the hospital on July 12^th to begin a 5 day course of chemotherapy, to be followed by a day of rest, and the transplant will occur on July 18^th.  We expect to be in the hospital for at least 35 days, but it all depends on how things go.

In the meantime, we have been having adventures around Seattle.  We went to Bainsbridge Island and Idan took his very first ferry ride.  We visited the Seattle Children’s Research Institute – the very same lab that diagnosed Idan with Hyper IgM before we knew anything about Seattle Children’s Hospital, that processes all his lab results, and that recently published research showing promising results with gene editing as a cure for Hyper IgM.  Idan received a private tour of the science lab and got to see where and how all of his blood gets tested, the different machines they use, and he even peeked into the very cool container storing his blood samples for future research.

So, if you see a smile on our faces in these pictures, it’s real.  We are joyful.  Idan is once again leading the way, and we can’t help but be just a little excited when we’re all together on this great and scary adventure.  We expect some serious storm clouds along the way, but just like the sun in Seattle, together, we are pretty damn persistent, and are hopeful that the clouds will make way for a beautiful blue horizon.

Today was our first day back at the SCCA clinic to start the process of the various pre-transplant testing . We started with a nurse visit to go over a lot of basics and get our schedule for the next few days.  It is very strange to be back here. Everything is the same, including most of the staff that we meet. It is a bit of an awkward balance between the “how nice to see you again” and the “I’m so sorry you had to come back to do this all again!” Overall, it is nice to have familiar faces and people we feel like we know. For Idan, everyone is new. He has no memory of any of these people and cannot understand why they all seem to know him so well.

After the nurse visit, we had Idan’s first round of blood tests of many many to come. He did very well and sat still while they poked him and took 17 vials of blood. Yes, 17 vials of blood! Not bad for his first draw here. They will take a few more before his procedure scheduled for Friday as he was at his max today for how much they are allowed to take.

We have a nice long break for lunch and went to eat outside. We went down to the marina to see the old boats. Idan could care less about the boats and manly enjoyed dragging Amanda around up and down the trails and just jumping around.

SCCA House Playroom

Later on, we had our first visit with Idan’s primary care physician who went over all Idan’s history and did a full physical. All went well and Idan was very excited to come back to the SCCA House, the clinic-related hotel we are staying at, so he could go to the play room.

Our schedule for the next couple of days is busy. Tomorrow we have another full day at the clinic, mostly with meetings (social worker, nutritionist and a 90 min consult with the attending to go over the transplant protocol). He also has an EKG in the morning.  Friday will be a tough day, as Idan will have a bone marrow aspiration and biopsy at 12 at Seattle Children’s Hospital and will have to fast all morning since he gets anesthesia. So we expect a hungry and cranky child.

At the moment, unless something changes, we have a couple more weeks of testing, and will be admitted to the hospital on July 12th to start chemotherapy conditioning.  The transplant itself will occur around July 18th or so. Good news is that we have found an apartment in the same complex we lived last time. We should be moving in there on July 1st, and it will be nice to have a temporary home as opposed to a hotel room. Idan is not as happy as he says “there will not be a play room” in our apartment. Not easy to please! 

We will keep you all updated.

Our first full day in Seattle and we are getting used to our temporary living situation (SCCA House) and getting reacquainted with Seattle. Our appointments start on Wednesday. As before, the Seattle community has been so amazingly welcoming and have gone out of their way to help us get acclimated (including lending us a car to get around while we’re here). One very special 12 year old has organized for all her friends, as part of her Bat Mitzvah cause, to get Idan new toys and activities to keep him busy during the long days ahead. Below is just the first batch of many amazing toys she has organized for Idan. We are so thankful too her and the many that have donated these items to bringing a smile to Idan’s face and keep him busy.

Ever get that feeling, towards the end of an intense dream, that things all of a sudden start malfunctioning or, even weirder, morph into something else?  Where all of a sudden, despite passively accepting all of the nonsensical parts of the dream when it first began, you become aware of the illusion?  Reality seeps in, and you open your eyes.  Maybe sunlight washes over you, maybe the buzz of the alarm shocks you into motion, or maybe it’s the sound of a baby’s cry that gets your feet planted on the ground.  

Sometimes this is comforting, no?  Sometimes you fall into a deep sleep, and the dreams are frightening.  They show you a world where you are alone, fighting, struggling, running.  The moment where the illusion ends is met with a sigh of relief – everything is okay, you’re awake now.  

Sometimes it is the opposite, though.  The dream – the illusion – is a sanctuary against reality.  A peaceful interlude that gives you a break from the chaos.

The other night, I had a dream that I was living in a small claustrophobic room, and directly above my head, there was a hole in the ceiling where I could see trucks and cars passing right over us.  Mindy Kaling from The Mindy Project was asleep on the floor to my right (like I said, nonsensical), and seemed blissfully unaware that we were living right under a highway, and that there was no way out except to climb out of this hole and risk being run over.  As I was plotting our escape, trying to figure out how fast we needed to climb out of the hole and dart across the highway in between the cars zipping by to get to safety on the other side, the walls started morphing, and suddenly our little bunker turned into the back of a truck.  Someone else was driving the truck, and Mindy and I were still in the back, this time being jostled back and forth, not knowing where we were going.  At this point, the illusion started to fade.  I could believe Mindy sleeping on the floor of my underground bunker, but I could not believe that the bunker had turned into a truck.  Reality began to seep in, and I woke up.

I was relieved to be awake.  The sun was shining right through the window, Akiva was sleeping peacefully next to me, and Idan was in the other room, beginning his morning ritual of sweetly talking to himself until either I or Akiva came in to greet him.  

I was relieved to be awake, but had I really woken into reality?  Isn’t our life here, in a way, an illusion?  A peaceful interlude before the chaos of transplant?  The whirlwind hospital days and pre-testing leading up to admission, the unpredictable but inevitable events and incidents post-transplant, the constant trips back and forth to the clinic, the pharmacy, and the hospital, and the gargantuan efforts required to turn the whole trip into an adventure, yet still maintaining some semblance of normalcy, for Idani as every little fiber of his being is tested and challenged… isn’t that reality?  

This week, our home appliances have been taking the biggest hit, each of them taking turns malfunctioning.  The illusion of our peaceful life here is slowly being peeled away, and, come Sunday afternoon, we’ll be jerked into reality.  Maybe it will be the Seattle skyline washed in sunlight, or maybe the wheels touching the ground that shock us into motion, or maybe Idan’s voice sweetly talking to himself as he gazes out the window of the airplane, but we’ll open our eyes, and the illusion will be over.

I don’t know how I’ll feel when I wake up from this particular dream.  I don’t know if I’ll be relieved to be taking our first step towards the end of a horrible nightmare, or if the fear and chaos of transplant will be overwhelming.  But I do know that, as long as Akiva and Idan are by my side, I am ready to wake up.

Three years ago when our baby boy Idan was diagnosed with Hyper IgM Syndrome (HIGM), a rare and life-threatening immune deficiency, an army of supporters came together to support Idan and our family as we headed into a bone marrow transplant to cure our little boy. Although the transplant was unsuccessful, something unique and special came out of that first battle:  the Hyper IgM Foundation. As Amanda and I immersed ourselves in everything Hyper IgM in order to find a cure for Idan, we learned of the dire need for a community and voice for the Hyper IgM patients around the globe. HIGM is rare among an already rare family of genetic diseases categorized as primary immune deficiencies. The little information we could find tended to be outdated or inaccurate. We set out to not only fix this but to gather patients and families from around the world to create a stronger voice for Hyper IgM Syndrome patients in hopes that a combination of advocacy, education, and focused research will accelerate a cure and better outcomes for all families with Hyper IgM.

Three years later, we are going into battle again for our little boy, and behind us, we hope, stands the same army that helped us in our original fight. As we head back to Seattle for Idan’s 2nd transplant, we are calling on you to help not only us, but to help all children with Hyper IgM Syndrome. Idan is one of the lucky ones, he has several matched donor options for a transplant, but for many others transplant is not possible.  And the cure itself comes with significant risks, with transplant complications taking the lives of two other little boys with Hyper IgM in the month of May alone.  Our goal is to raise funds for research into gene therapy and other techniques that can provide curative options for all HIGM patients.

The Hyper IgM Foundation aims to improve outcomes and quality of life for Hyper IgM patients and their families through promoting and supporting research, creating and providing educational tools for the patient and physician communities, and creating a community to support Hyper IgM patients and their families as they undergo treatment and cope with this disease.  Between Akiva’s extensive experience in public health, Amanda’s legal background in healthcare and nonprofit law, our fellow board member Dr. David Hagin’s cutting-edge research into gene editing for Hyper IgM, and our scientific advisory committee that consists of the foremost experts on Hyper IgM in the world, we are confident that we can make a tremendous impact.

We hope you can join us again in support for a cause that will impact the lives of many children and adults living with HIGM.

Consider a donation today!

For those of you interested in following Idan’s journey, we will be updating Idan’s blog and Facebook page on a regular basis. We are heading back to Seattle on June 19, and expect to be admitted to Seattle Children’s Hospital in July for 4-6 weeks. For the second time, we’re packing our bags and uprooting our lives. The decision to move forward with a second transplant was even more difficult than the first time, knowing what we do now about the risks of transplant, but we have to believe that Idan will be cured, and that he will have a brighter future.

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The Hyper IgM Foundation is a recognized 501(c)(3) charity and all donations are tax deductible. We thank you for your support and invite you to stay up-to-date on the Foundation’s efforts by connecting with us on Facebook by liking the Hyper IgM Foundation Facebook page, www.FB.com/HyperIgMFoundation, and by visiting our website, www.hyperigm.org.

As a reminder, the Hyper IgM Foundation is entirely a volunteer run organization. We have no salaries, no rent and limited overhead. This means that 100% of your donation goes to fulfill our mission to improve the treatment, quality of life and the long term outlook for children and adults living with Hyper IGM Syndrome through research, support, education, and advocacy.