Idan, my hero

“Based on the rate of growth and the location of your tumor you have around two years to live.” That was the sad prediction I heard from dozens of physicians in the summer of 2005. From New York to Boston to Los Angeles and Jerusalem, everyone around the world we consulted had a different approach, but they all agreed: The brain tumor was “inoperable.” I was twenty five years old and one year away from graduating Columbia University. I was way too young to die.

I am still here. Ten years later, I am alive and well and tumor free and the only prediction that came true was the one given by Dr. Robert Spetzler in Phoenix, Arizona. With a big smile on his face he said: “I am 95% confident I can get your whole tumor out, and leave you in not much worse condition than you are now.” At first, it seemed his proclamation might have been an overstatement. On November 8, 2005, Dr. Spetzler successfully remove my whole tumor. While my family was assured the next day that the tumor was all out, and I would be fine, at the time it did not look like it. I was in an induced coma, intubated and had tubes sticking in and out all over my body. I only woke up four days later, when they were finally able to extubate me. I could not feel or move the left side of my body. I could not talk, walk, swallow or do much of anything. The right side of my face was paralyzed and numb, and I could not smile or even hold water in my mouth without it dripping out. Like a newborn baby, I was cradled and tended to by my family and an amazing team of nurses at St. Joseph’s Hospital in Phoenix. Every day Dr. Spetzler came by during rounds, looked at my broken and weak body, and proclaimed, “he is doing great!” Everything will be okay. Eleven days later, I was transferred by plane and wheelchair to NYC to Rusk Rehabilitation Center for what we were told would be a very slow and long process of rehab. There, I learned to walk, talk, swallow and was able to be released to outpatient in three weeks. I continued my rehabilitation out-patient while I resumed classes at Columbia. The right side of my face was paralyzed, and numb, and I had set a personal goal to smile once again.

Two years later, in November 2007, I had my diploma from Columbia and went on to obtain an MPH at the Mailman School of Public Health. I was dating the love of my life and was doing great, physically and professionally. But I still could not smile. Sure, I could mostly drink without spilling water out of the side of my mouth, and I had even perfected a method of blowing up balloons. But I had not reached my goal. The cranial nerve that controlled my face was cut or damaged and was not coming back. The doctors had told me over and over again: most deficit improvement is seen in the first 3-6 months. After that, some might still get better in the first 2 years post surgery. But after those two years, things will not improve or come back. My fate was sealed. I may have been reborn as the superhero, iPatchman, but I was destined to always be defeated by DC Comics’ Two-Face.

Two years later, November 2009, marked some of the happiest days of my life. I just married my true love. I had a dream job in healthcare consulting and life was good. I had outlived all predictions and was tumor free for four years. And despite the utter joy, I could not fully smile. I had perfected the Zoolander Blue Steel look for photos. My wife, Amanda had fallen in love with my half grin and I could not complain. Most people in my situation did not live to tell the tale, and I was complaining about a smile? I continued to refuse offers to surgically stitch my face in a permanent Joker grin and continued my own physical therapy and exercise to keep the little muscle tone I had on my right side of my face.

Two years later, in November 2011, now six years post-brain surgery, I sat at Nonna’s Italian Restaurant on the Upper West Side for my birthday dinner with Amanda. She handed me my birthday present. A little red box with yellow teddy bears. Inside, little baby booties and a handwritten note on tissue paper, with the message “To the greatest love we will ever know.” She was informing me that she was expecting. We were going to have a baby in eight months. Inside, my joy was endless, and yet I still could not smile. Just my regular half grin in response to the best news anyone can get.

Two years later, by November 2013, we had experienced both extremes of any parents’ emotional spectrum. From the new happiest day of my life, the day my son Idan was born, to the scariest and saddest day of our life, the day he was intubated for PCP pneumonia. Even if I wanted to smile, I could not. We were filled of hope now, and longing for a normal life again. Just a few weeks after Idan’s bone marrow transplant, I celebrated my 8th year tumor free. I was full of hope that by my ten-year anniversary Idan would be cured and everything will be back to normal. I stopped believing that I would ever smile fully again, even if I wanted to.

Today, two years later, Idan is not cured. We are heading towards a second bone marrow transplant in the spring, and life might never be normal. But hope is still part of every drop of my being. The same unrelenting perseverance that allowed me to live through an inoperable brain surgery has kept my son alive and well and found him the best care he could receive. My education, life experience and optimism serve me now as the president and co-founder of the Hyper IgM Foundation. Our mission is not only to cure Idan, but to work for a cure for all families and children living with Hyper IgM. I know this can be achieved. I know happiness is possible. I know normality is a state of mind. I know all this because I spend the day smiling. A full smile. I spend the day smiling when Idan wakes up in the morning with a million questions as if the night was just a fleeting moment. I spend the day smiling when I see Idan play with his little cousin and they both laugh uncontrollably. And I spend the day smiling when Idan is solving puzzles way too advanced for his age or building lego sets made for six-year olds.

Sometime, over the last two years of trying to achieve normality in a post transplant world, my smile just appeared. Despite all medical predictions of how or when deficits can recover, I am able to smile with both sides of my face now. Ten years, brain tumor free, and I am smiling every morning when I wake up next to Amanda and when Idan calls us into his room. If my smile can appear again ten years after it was removed along with a tumor that had my name on it, I know that anything is possible. I invite you all to help me make that which is impossible a reality. Help me bring a smile to the families across the world dealing with Hyper IgM Syndrome.

Donate today to my ten year brain-tumor-free fundraising page!

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.