Idan, my hero

Day 1 in the PICU, April 10, 2013

“It’s going to be a rough two years, but after that, he’ll be cured, and everything will be fine.”  It’s a statement I heard multiple times from well-meaning family members in the corridors of the pediatric ICU at Cornell Hospital.   I held onto those words tighter when I looked over at my darling baby boy attached to the vent.  Even tighter when I would wake up in the middle of the night to find a team of nurses surrounding him, one nurse straddling him on the bed furiously pumping air into his little lungs when the ventilator failed or when a tube got yanked out of his nose.  I held onto those words for the next two years, and here we are.  It’s been a rough two years, and he’s not cured.  But everything is starting to feel more fine.

Week 2 in the PICU, April 2013

When I think back about our time in the PICU, there are a few more potent memories.  There’s the memory of the masks, gowns, gloves – constant fixtures in our days in the PICU.  Yelling at the doctors when they refused to let him eat or drink in the first two days, then sudden comprehension that they believed he would be intubated, and could not risk aspiration.  The conferences with the doctors where they explained the possible causes for his lung failure, and the trial and error diagnoses that began to mimic an episode of House.  The impossible torture of not being able to give my sweet baby boy a gentle kiss for fear of sharing germs or tripping over life-saving tubes.   Leaving the hospital for the first time in several days, collapsing on the floor of my childhood home choking back the sobs that I denied for so long, seeking comfort and strength from my family so that I could return to Idan’s bedside, renewed. 

Most potent for me, however, is the memory of us leaving the hospital with Idan.  After three weeks spent in a tiny box filled with the constant hum of machines, we were allowed to take Idan home.  After his diagnosis with PCP, we were hit again with a second diagnosis of a severe immune deficiency, and informed that he would spend his life in constant fear of being re-hospitalized for new and (if you could believe it) scarier illnesses as his immune system would simply fail to adapt to the germs around him.  Yes, I remember leaving the hospital vividly.  Holding Idan tightly in my arms as we walked out of isolation into the busy corridor of the PICU.  The sounds of coughing, sniffling patients, children playing in the waiting area, five people waiting for the elevator.  The same hospital that cured him was now a minefield.  I tightened my hold on Idan as the elevator doors opened, trying to nestle his face into my shoulder to protect him from any exposure once crowded inside.  When the elevator doors opened, Akiva and I made eye contact, and as if on queue, sharing the same instinct, we started running.  We ran through the main floor, dodging patients and hospital staff.  We ran all the way to the car, out of breath by the time we sat down.  Idan was safely in his car seat, and suddenly “safe” took on an entirely new meaning.    

And we all know how the rest of the story goes.  Two years searching for a cure, building an army, learning the hard way how strong we all could be in the face of adversity. 

I’ve always believed that time is not quite linear.  Yes, it marches on ever steady, but there are moments and events that are forever linked to you.  Those three weeks in the PICU are long gone, time having granted us two years of wonderful memories and joyous occasions since then.  But we keep circling back, and carry those memories with us every day.  Because when we were rushed into the hospital two years ago today, we were a normal happy family, and the moment we walked through those doors, we became something new.  We found our strength, our courage, our fight.  We became a force to be reckoned with.  We’ve persevered despite all odds, and Idan is a blissfully happy and brilliant child, who is surprisingly aware of his condition and limitations but never lets that stop him from feeling joy.  And the next two years will be another rough two years, and we hope and pray that we’ll have a cure.  Today, we’re stronger than ever, and everything is going to be fine.

Week 1 n the PICU, April 10, 2013

April 2015

It has been two years since that awful day we rushed our 8 month old son to the ER with an oxygen mask. That April was much warmer than this one, and we had spent much of the time in the two weeks leading up to that day outside in the park and playground. He had just started enjoying the swings and there was always a playground stop after the visits to the pediatrician leading up to that fateful day. I am still amazed by the little boy’s resilience and happy mood. Idan was breathing 80 breaths a minute while the Pneumocystis pneumonia (PCP) was quietly destroying his lungs, but he was as happy as could be and could not wait to play.

It has been two years since we watched our son intubated in the ICU  while his lungs gave in to the PCP, not knowing if he would ever recover. We spent three weeks isolated in that room hoping to wake up from a nightmare that was just beginning. We spent three weeks researching PCP survival rates, and immune deficiencies, trying to piece together the mystery of how a healthy happy boy could suddenly fall so ill.

The Idan Do Campaign September 2013

It has been two years since we first heard that words Hyper IGM and CD40 Ligand Deficiency, since our son came back to us and we settled into our isolated life in the center of the busiest island in the world. Our life was changed forever, and our dreams crushed as we faced ever growing uncertainty as to our son’s survival. It has been two years since our baby inspired us to become better people, and gave us the strength to make the hard choices we faced.

It has been two years since our son galvanized thousands of people to open their hearts to our family and form an army of supporters to fight with us. It has been two years since our son empowered us to become experts in his medical condition and gave us the resilience to carry on with our lives. For it is Idan’s resilience, in the face of all that he has endured, that has kept us going all this time.

Your average toddler during this period would learn to walk, talk, play and jump. They would start asking questions and start learning some letters and numbers. They would learn to kick a ball, do puzzles and stack blocks. And they would grow taller and bigger and brighter. It is all a parent can hope and wish for their child during this age.

October 2013

Idan reached and surpassed all these milestones despite Hyper IGM. He laughs, entertains and sings despite having spent over 65 days in hospital rooms. He learned the ABC’s and his numbers while getting prodded and poked thousands of times. He can put together a 54 piece puzzle of the USA all on his own despite receiving three types of chemotherapy. Nothing has slowed him down or taken away his constant excitement and curiosity, not even the social isolation he has had to endure.

The road ahead is long and rocky. Idan will have to face another transplant, more isolation and many more pokes, meds, and surgeries. It will be more than two years until the cure we hope for him will be in our reach. But despite this, we know that Idan will continue to thrive and develop and inspire us to carry on. He is truly our light and our guide throughout this journey and we will not let him down. Idan is My Hero!

April 2015

Idan building his truck

Today marks a year since our return home to New York after Idan’s bone marrow transplant (BMT) in Seattle. It truly is amazing how time flies, and so much can change in our life, yet at the same time still be the same. We left to seattle in September of 2013 full of hope that we would bring home a cured baby and be able to start our normal life again. When we returned to New York last March on day 140 post transplant, we already knew that the cure was slipping away from us.

In those months out in Seattle, the transplant days went by with no major glitches and Idan did well despite the chemo and immunosuppressive treatments. Besides some minor setbacks, it seemed at first that he engrafted and we were sent to outpatient sooner than expected. But as time went by, we learned that the most important part of the donor graft, the T-cell line, never really took hold. We did our best to save it, going back to our generous donor and asking for more cells. In December 2013, we give Idan his first donor lymphocyte infusion (DLI) and waited. By January, we knew that it only gave him a small bump, and unfortunately that small bump came with graft vs host disease (GVHD) of the skin, which required increased immune suppressants and steroids. Throughout this whole time, Idan never really complained and was a happy and inquisitive baby who learned to walk in the halls of Seattle Children’s Hospital and the wonderful outdoors of the Seattle neighborhood we lived in.

When we were allowed to return home last March, we were still full of hope that his graft would take hold and that a cure was still on its way. When we brought Idan back, he was a little toddler full of joy to be reunited with all his family in NYC.

When we returned to New York, our life was so different from the March before. The previous March, before he was diagnosed,  we spent our time going to the park, meeting up with our friends with young children, and taking swimming lessons at the JCC. That March, however, passed in isolation, venturing out only for twice-a-week doctor appointments and blood draws. Compared to Seattle, NYC felt like a zombie-filled nightmare with virus spreading strangers at every corner. It was good to be back with family but it was hard to entertain an almost 2 year old boy with this kind of isolation. Idan was on 13 daily medications with 26 different dosages at  every time of day and night. Going to his grandparents for the day meant packing ziploc bags with two dozen syringes and planning his nap and meal schedule around the medication times. Idan grew tired of the weekly blood draws and started fighting off the needles. And still, his graft continued to slip away.

Idan in March 2014 packing our shipping boxes

In the fall, we returned to Seattle and made one last effort to save the graft with a second DLI.  When the graft continued to slip in the weeks and months that followed, it became clear to us that the graft was no longer a graft, and now just a memory – an unfulfilled promise of what could have been.

This past year flew by and we find ourselves a year later very much in the same place we were before leaving to Seattle the first time. Idan has grown up, and turned into a smart, inquisitive and very curious little boy. In July, he will turn 3, but he feels older and wiser for his age (he is also very tall and sturdy, something that is unusual for transplant kids). We are comforted by the fact that he is no longer in post-transplant mode, but he has regressed back to Hyper IGM life. Thankfully, his daily meds consists only of 3 vitamins and supplements and twice a week prophylactic antibiotics to prevent the return of the PCP that landed him in the ICU back in April 2013. Blood draws are infrequent now, and if all goes well, his next draw will only be in May. We still give him weekly infusions on Saturday mornings with antibodies that keep him safe, and he takes those three needle pokes like a real superhero.

Amanda and I have spent the last two years learning everything and anything about Idan’s rare condition. I doubt any doctor or patient out there has read more medical journals on the subject then we have and we continue to try to understand the disease while we asses the risks involved. The facts remain the same though: the median life expectancy is 24 and over 80% of patents don’t make it past their third decade of life. We knew this before transplant and took the high risks then in order for our son to have a long and happy life, and we will do it again in a heart beat.

What this experience has taught us more then anything is that we are goal oriented people, ever focused on our vision of tomorrow, ready to face the challenges along the way.

Baking cookies with his parents

Ten years ago, when I (Akiva) was diagnosed with a so called “inoperable” brain tumor in my brain stem, I set a goal to be completely cured, regardless of what 30 of the top doctors were saying my chances were. And I met that goal by risking everything and having complicated brain surgery in Arizona with the one doctor in the world that was confident enough to take me on as a patient. Two years ago when Idan was diagnosed, and after the initial shock of the ICU, we set a goal to learn about Hyper IGM and talk to as many experts so we can make the best and most empowered decision on how to cure our son. And we still stand by our decision to uproot our life, leave our jobs, and move to Seattle for six months to cure Idan. Despite the failure of the 1st transplant, we have never given up hope.

We have once again set a goal, to transplant again within the next two years, again in Seattle and with the hope of a better matched donor. Idan will still be under five when we try again, which is the age that the risks of transplant are better controlled.

In the meantime, we continue to live our lives and help Idan thrive and grow. You may have seen the posts on Facebook, as the kid is happy and an extremely energetic two year old. He talks non-stop, expresses his feelings, and loves to learn and build and just have fun. He has always been a happy child, weather in the ICU, getting chemo, tethered to an IV pole or being cooped up in a NY apartment for most of the winter. No matter what, he always wakes up with a smile and declares “Wake up!!  Its time to play!”

Creating a large Magna Tile triangle on his own

We would like to thank all of your for your continued support. Idan’s army always has a special place in our heart and it makes us stronger and gives us hope that we can enter again into this battle for a cure and hopefully this time come out triumphant. Someday, in the future, when germs are not the big boogieman anymore, we hope to thank more of you in person for your support.

It has been two months since our last real blog update. We’ve been updating Idan’s Facebook page more regularly, but it has been a few weeks since we shared any real news. Sadly, it has been a rough few weeks and neither Amanda or I have been in the right place to put it all down in words. I will jump right in then:

We came back from Seattle at the beginning of November (after spending most of October there) with the hope of saving Idan’s failing graft.  After putting Idan through more chemo, several long fasting days, dozens of pokes, a PICC line surgery, and a second DLI this October, we checked his chimerism again right before Thanksgiving and did not get good news. The DLI did not work, and his graft is still failing. We were heart broken. We were faced again with the options as before: risk a third DLI or do nothing and start preparing for a second transplant. After many conversations with our Seattle doctors, family and NY doctors we are no closer to making a decision but we have decided to wait for another data point next week to see how fast the donor graft is falling. A third DLI would be at a higher dose, comes with high risk of GVHD, and, given our track record, has a minimal chance of working.  A second transplant also comes with more risks than the first one, another long stay in Seattle away from home and our family, and the possibility of rejection.

What has made things harder this month has been the fact that Idan has spent most of the past 4 weeks sick with a cold. Every parent of a toddler dreads when their child is sick, but for us there is always the added stress of being one step away from the ER. Like with a newborn, any fever above 101 or requires doctor visits, blood cultures and a good chance of hospitalization. While Idan can fight a cold virus with the help of the weekly antibodies infusions we give him, any cold can all too easily turn into a pneumonia and we always need to rule out an infection.

Idan has been getting stronger and better at fighting us off when we try to take his blood and there have been way too many blood tests this month. We cannot wait for a time where we can space out these tests and hope and try to keep him safe so that we can avoid unnecessary colds and infections.

The good news is that Idan is still growing, developing and thriving and has an endless amount of energy. He has been talking more every day, and we potty trained him over the Thanksgiving weekend and he has been golden ever since (no pun intended). He has grown an inch and a half since November 1st and he has been developing his art skills along with his imagination.

All we can wish for is a better and healthier 2015 for Idan and the rest of his little Hyper IGM counterparts around the world who inspire us with their bravery and perseverance.

Wishing you all a happy holiday season and a happy and healthy new year!