When our son, Idan, is old enough, he will probably want to know why he has over 1,500 fans on Facebook. He will probably want to know why we have shared dozens of photos and videos of his childhood with strangers. He will probably ask why, when he Googles his name, dozens of news articles come up telling his story. We will tell him that, at first, we shared his story to help him and us deal with the challenges we faced, but later, we shared his story to inspire others who might be facing similar challenges. I hope he understands, and I hope he forgives us for giving him a digital footprint at such a young age.
At eight months old, Idan was rushed to the emergency room by ambulance with a very low oxygen rate and rapid breathing. A healthy and strong baby boy until that point, he spent the next three weeks in the pediatric intensive care unit, two of those on a ventilator, clinging to life. Idan had pneumocystis jiroveci pneumonia, a rare type of pneumonia only a child with a severely compromised immune system could contract. Idan’s pneumonia led doctors to diagnose him with a life-threatening genetic disorder called X-Linked Hyper IgM Syndrome, or Hyper IgM for short. It keeps Idan from being able to produce any antibodies of his own to fight off infection. It meant that without weekly infusions of antibodies and antibiotics prophylaxis, his chances of long-term survival were significantly diminished. A bone marrow transplant was the only known cure.
It had been the hardest three weeks of our lives, and we returned home tired and hopeless. The first thing that greeted us back home was a denial letter from our insurance plan for the antibody infusions, the only thing keeping our son alive. It would be the first of many medical and insurance-related setbacks that we would face over the next couple of years as we fought for our son’s cure. But it set the tone of our struggle and inspired our first battle cry. We decided to share Idan’s story with the world in hopes of not only raising funds to help pay for the medical expenses that were piling up but also to find others like Idan and to learn from them.
In the two and a half years since, Idan has amassed a medical record longer than most nursing home patients. He has endured thousands of needle pokes and dozens of invasive procedures and tests. He has had half a dozen surgeries and one failed bone marrow transplant.
Yet nothing seems to have fazed him or slowed him down. He was known at Seattle Children’s Hospital, where he got his transplant, as the happiest baby they had seen during the procedure. His favorite part was entertaining the team of 14 doctors and nurses who came in during his morning rounds. He learned his ABCs and 123’s during the long hospital stays before most kids say their first word. And he has grown and thrived despite having a severely compromised immune system and many restrictions on human interaction.
But we want more than that for Idan; we want a cure. We recently formed the Hyper IgM Foundation, a patient advocacy organization that will provide support and resources to families living with Hyper IgM, educate the medical community regarding diagnosis and treatment and provide funds to support critical research and advancements in gene editing, bone marrow transplant and other known and unknown therapies that could help Idan and the hundreds of others in the world grappling with Hyper IgM.
Our family has been put through trial after trial, each more trying than the last. But we’ve learned so much, and through the wreckage, we have found something new and beautiful beyond measure: hope. It’s hard not to feel hope in the company of the most charming, loving and bright child a parent can hope for. We are full of pride, and we feel utter joy in his presence. But we know that, without parents like us - empowered, informed and engaged - children with his disease do not stand a chance. Informed parents join their child’s medical team and must constantly make tough decisions. Bone marrow transplants are long and very risky procedures. Even when a match is found, 15-20% might not survive the first year, and many more have long-lasting effects from the chemotherapy and graft v host disease. Parents must be vigilant with the dozens of medications and infusions their children need post transplant as well as with strict isolation procedures.
For all those parents who have the means and the desire to advocate for more research into a cure for a rare disease like Hyper IgM, we encourage you to do so. Patient advocacy groups are vital to medical advancements, are extraordinarily effective tools and support systems and, importantly, can provide a meaningful path to a cure.
