Idan, my hero

This week we are celebrating the Jewish holiday of Chanukah, which celebrates (among other things) a miracle:  After destruction of the temple, the Jews used the last remaining oil they had left to light the menorah, with the flame rising from the menorah in the midst of ruins as a symbol of their resilience despite great hardship and against all odds.  They believed the oil would last only one day, but it lasted eight days.  To this day, Jews light candles on menorahs around the world for eight days to commemorate this miracle of survival, resilience, and another seven days of light.

On the 8th and last day of Chanukah, by coincidence, we are headed to Duke to test whether Idan’s remaining donor t-cells, which are so few in number but are nonetheless hanging on, are, like the oil, sufficient. This would be a miracle indeed.

As we mentioned in our last update in September, we are working with our medical team to plan Idan’s second transplant. As time goes by, it looks more and more likely that we will be heading back to Seattle this Spring for transplant. Idan has been doing great, growing and thriving and we have been able to keep him healthy with his weekly infusions of antibodies. He receives home-school services from the New York State Board of Education, and has been learning to read quite impressively for a 3 yr old (we kid you not). So we are once again headed towards transplant with a healthy, active child, and we are once again gearing up for all the challenges that lie ahead.

Before we decide whether to move forward with a second transplant, however, we need to first conclusively determine that it is absolutely necessary.  While he has lost almost all his donor graft, there is around 8-10% of the donor’s t-cells still showing up in his chimerism tests. Although in a perfect world, 10% donor t-cells could in theory be sufficient to provide an individual with a functioning immune system, it appears that the donor t-cells in Idan’s graft are not binding to the B-cells, which is necessary in order to create an antibody response. For this reason, we assume that a 2nd transplant is imminent. However, the tests that have been run so far are not 100% conclusive, and, before going into a second bone marrow transplant, which is without a doubt a life-threatening procedure, we and our team of doctors feel that we need to be absolutely sure that the remaining donor cells lingering in Idan are in fact insufficient to provide Idan with a functioning immune system.  

It is not easy to determine whether Idan can make antibodies with the small amount of donor cells left. He receives weekly infusions of subcutaneous immunoglobulins (SCIG), which provides him with a high level of antibodies. One way to test if he can create antibodies is to give him a vaccine (any of those childhood vaccines we all get). However, because of the constant infusion of antibodies through SCIG, he has a full repertoire of antibodies for almost any vaccine, meaning we would not be able to determine whether he was responding to the vaccine or whether the antibody is from the SCIG.

There is one test that is only available in the United States in Seattle and at Duke called the bacteriophage φX174 or Phage. Essentially we would be giving Idan a *fake* vaccine (through an IV) that contains a small amount of a virus that is used to fight bacterial infections. This is not a virus that people are exposed to, and it is not part of vaccinations anyone gets, so antibodies to this virus would not exist in the SCIG he receives weekly. If Idan can make a response to this vaccine, it will show up as distinct immunoglobulin (antibodies) for this phage and can be measured.

After much research and debate we decided to head down to Duke to do this test. It is a short 24 hour visit, and we will send blood samples back to Duke over the next 6 weeks. Then we will head to Duke again in late January for a 2nd dose and repeat blood tests back in NYC. Sometime in March, Duke will send all the samples to Seattle’s immunology lab for testing and we should get results right before the tentative date of the 2nd planned transplant.

Two and a half years after our first expedition to Duke to find a cure for Idan, we are headed back to Duke this Sunday to test if Idan might perhaps be cured. Based on all lab tests it is very unlikely that Idan will make a response to this vaccine. But, if by some miracle his small percentage of donor T-cells can make a response, this might mean that we can postpone 2nd transplant and see if his donor cells can provide him with enough immune function on its own.

We will begin this test on the 8th day of Chanukah, a holiday commemorating that miracle long ago whereby a very small amount of substance that should have lasted only for 1 day lit the Menorah in the temple for 8 full days. Let’s hope that Idan’s small amount of donor cells can pull off the same kind of miracle.  

“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!