Idan, my hero

By Anne Cohen:

Read more: https://forward.com/news/181861/jewish-babys-rare-immune-disorder-attracts-global/

Any Jewish mother thinks her baby boy is special. But Amanda Zablocki’s son is one in a million — and in one of the most heart-wrenching ways imaginable.

Idan Zablocki is 1 year old. Three months ago he was diagnosed with a genetic disorder called Hyper IgM syndrome, a rare immune deficiency that actually affects two out of every 1 million people.

“It’s similar to what you know as the ‘Bubble Boy syndrome,’” explained the boy’s father, 33-year-old Akiva Zablocki. “Hyper IgM is like one level down from that. “ Idan cannot drink tap water. Bath time means pouring out bottled water into the tub. Guests must take off their shoes, wash their hands, and sometimes even wear masks and a clean change of clothes.

Though his parents can still touch him and hug him, they also must take precautions. His father, who has the sniffles, has been wearing a mask for the past week in order “not to risk anything.”

“We’re kind of living in our own bubble to make sure we’re not taking anything from the outside and bringing it to him,” said Amanda Zablocki, 28. “Sometimes I hear someone sneeze on the train and I actually change train cars.”

Idan was born healthy, but roughly eight and a half months later he started breathing abnormally quickly as he was recovering from a cold. A couple of weeks later it happened again. In the end his respiration rate got so high that his parents rushed him to the hospital.

“He was diagnosed with a very rare form of pneumonia, caused by a fungus that’s extremely common,” his mother explained. ”It’s almost like getting sick from dust. People who get sick from that are people who have very compromised immune systems.”

Because he has almost no ability to produce antibodies, Idan cannot come into contact with germs. He has an extended family and cousins his own age in Israel and the United States, but he has never met them, as an ordinary cold could be a death sentence.

The Zablockis are no strangers to medical hardship. Eight years ago, Akiva Zablocki was diagnosed with a brain stem tumor that doctors deemed inoperable. Refusing to give up hope, he looked for a doctor who would operate.

A brain surgeon in Arizona proved to be the answer and saved his life, removing the tumor entirely. Though he sometimes has to wear an eye patch, he has regained his quality of life.

His son’s condition “makes my brain surgery look like a paper cut.,” Akiva Zablocki said .”But [he] is strong, and I think he’ll overcome it.”

People with Hyper IgM have three options: They can do nothing, which condemns them to death in their mid-20s from cancers and opportunistic infections; they can use intravenous immunoglobulin, the blood plasma replacement therapy that Idan currently undergoes once a week, or they can have a bone marrow transplant.

After consulting with doctors and medical professionals in England, Canada, Israel and all over the United States, the Zablockis have finally made their decision: They are making Seattle their temporary home as Idan undergoes treatment at Seattle Children’s Hospital, where both bone marrow transplants and Hyper IgM were discovered. He is lucky: Four potential donors have already been identified.

The Zablockis chose Seattle because doctors there use a drug called Treosulfan as part of the chemotherapy that will wipe out their son’s original immune system. Though still not FDA approved for general use, it is being used in clinical trials. The drug is believed to reduce the three fatal complications of bone marrow transplants: complications from chemotherapy, infections and graft-versus-host disease.

“We want to choose the [option] that gives him the best chance of long-term survival with the least complications,” his mother explained. “With a bone marrow transplant, there’s a chance for a complete cure. It takes one to two years to fully recover, but if it’s successful, then he’s a perfectly normal child and he grows just as any other person does.”

But all this comes at a cost. The hospital estimates the medical bill will be between $600,000 and $1,000,000. Akiva Zablocki, who takes care of Idan full time, doesn’t work. His wife is a lawyer, but she will have to take six months of unpaid leave in order to be with her son.

To alleviate some of the costs not covered by their insurance, the Zablockis have launched an online fundraising campaign, and what started as a couple of Facebook posts and emails to friends has turned into a strong network of support: They have raised $124,507 toward their $250,000 goal, with 117 days left to go.

“The response has been amazing,” Akiva Zablocki, said. “Overall, we don’t know the majority of the people donating to the campaign. We sent it to a few friends on Facebook and email, and a lot of people have been sharing it, sending out emails to their congregations. It was very scary for us; we didn’t know how we were going to deal with this.”

“People are praying for Idan,” his wife added. “People we’ve never met. It’s been a very powerful experience.” The Zablockis acknowledged the efforts by Jewish communities in New York and abroad, saying they have been an incredible asset.

“One of the things that has been extremely touching for us has been the large volume of emails from the Jewish community offering to bring us meals, to help, to baby-sit,” Amanda Zablocki said.

“Just the overwhelming response of ‘We’re with you,’ ‘We’re there,’ ‘What do you need?’ We’re trying to draw strength from where we can, and we’re definitely drawing strength from the Jewish community, from New York and all over the world.”

As for little Idan? Well, his name says it all. “Idan means ‘era’ [in Hebrew], like ‘time,’” his mother said. “It’s from the Bible. We were opening up a new era with Idan. For me, the association between era and time, time being so precious and yet so powerful, there’s something very meaningful in that. Idan is very strong, but he’s very precious.”

Contact Anne Cohen at cohen@forward.com

Read more: https://forward.com/news/181861/jewish-babys-rare-immune-disorder-attracts-global/

After 3 months of exhaustive research, the choice is clear.  Seattle, here we come!

Dear friends, family, and supporters of Idan,

Three months ago, when Idan was diagnosed with an extremely rare immune deficiency, we decided that nothing would stop us in our search for a cure. We sought out and consulted every Hyper IGM expert and transplant center that might have experience treating this terrible genetic disorder, and today we can happily announce we have found that place, and hope to be moving forward with transplant in September.

Like his father had done before him, we created a Team Idan, and read and gathered any piece of published data on Hyper IGM. We consulted and/or visited dozens of experts in the US, Israel, Canada, and Europe, including doctors in New York, Boston, Durham NC, Cincinnati, Philadelphia, San Francisco, Los Angeles, Texas, Chicago, and Seattle, as well as the National Institute of Health, Toronto, the U.K., and Israel.   We spoke to patients from Florida, Utah, Illinois, New Jersey, and England.  We dove deep into the science behind the immune system, and learned the names of a dozen chemotherapy drugs, immunosuppressants and steroids as well as their side affects and toxicity levels.  We have become what they call “e-patients,” determined to be fully engaged in Idan’s treatment and cure. But in the end, we heard over and over again, both from doctors and from other patients, we should go with our gut. After all the research was done, after every rock was unturned, we should go with the centers and doctors we trusted the most. That center is Seattle Children’s Hospital.

Of course, the first and main reason we chose Seattle is that they have the expertise to transplant and cure Idan, boasting lower risks of death, long-term complications, and toxicity from the chemo. As we have mentioned before, there are three categories of “fatal” complications in a bone marrow transplant:  (1) complications from the chemo; (2) infections; and (3) graft versus host disease.  Each hospital has a different approach to managing it, but we’ve found that there is one drug that is being used only in Seattle that can substantially reduce all three of these risks here.  This drug is called Treosulfan.  This drug is widely used in Europe and Asia, but has yet to get FDA approval in the United States.  Seattle was using the drug as part of a clinical trial several years ago, and has been granted an IND to continue using the drug.  The rest of the hospitals use a drug called Busulfan that carries significantly more risks of short term and long-term side complications (including sterility), and yet Treosulfan is equally capable of wiping out the immune system to allow for full engraftment.

To add to this, the bone marrow transplant was actually invented in Seattle and the lab that our immunologist runs there was the first to discover Hyper IGM; they are without a doubt both pioneers and leaders in this field. The transplant floor is brand new, built last year by the Gates family, and is clean and high-tech. If we have to live in a hospital 24/7 for 4-6 months, this new facility is a huge plus. Lastly, we truly loved the doctors and nursing staff and the rest of the friendly people we met at the center. We have been getting good vibes from Seattle for a couple of months now, and turns out that was the gut feeling we were waiting for.

It is still going to be an uphill battle. Even before Idan starts the chemo to make room in his bone marrow for the donor transplant, we are dealing with dire financial issues that no one in our situation should ever have to deal with. The estimate we have received from the hospital for the transplant is between $600,000 and $1,000,000. They, like other centers, gave us information about fundraising, like contacting local communities and having a bake sale. Our insurance plan will likely only pay about half of this, and rest will be on us. And to add to this, we both have to move away for 6 months and take an unpaid leave, while paying out of pocket for the health plan through COBRA and probably purchasing a second plan.

This is where you come in. We cannot thank you enough for your help and support in the past few months. We never would have had the strength necessary to find the right doctor and hospital for Idan if not for the constant support and encouragement we have received from our family, friends, and strangers worldwide.  We have raised more funds then we ever thought possible, but we are still less then half way to our goal. We hope we can once again call on you to help spread the word about Idan. We have seen amazing responses every time Idan’s story goes out to a community, synagogue or church listserve. We have seen great responses every time someone writes an article in a local paper. And we have seen amazing responses when you shared our story to your email contacts, or on social media. Please continue to share our story and help us cure our son.

Last night, there was a moment when we felt it was all for nothing – that all of our consultations, trips, and hours spent into the night researching conditioning protocols, complications, and long term risks, would be totally derailed by an inability to afford the care our son so desperately needs.  And in that moment, we felt utterly defeated.  This morning, after scrolling down and seeing the thousands of wishes and prayers and words of encouragement on Idan’s fundraising andFacebook page, we know we can face this challenge too.

With much gratitude,

Akiva, Amanda and Idan

P.S. Don’t forget to follow us on Facebook for more updates and cute pictures and videos of Idan.

We were so touched to discover that Idan’s story was featured in Columbia University School of General Studies’ News Letter. As proud and active CU alums, it was humbling for us to know that we had the support of the community.

Running. That’s what I remember most.  Running down the corridors of the hospital with my son Idan in my arms, running all the way until I reached the car.  I ran believing it to be the only way to protect Idan from the germs that lingered in every corner.  I ran to escape from the experience I wished so desperately to leave behind.  We spent three weeks in that hospital, watching our sweet baby boy battle a life-threatening illness, requiring a ventilator to breathe, and even fully recover, only to find out the battle had just begun.

Idan was born a healthy beautiful boy, and has lit up our lives ever since. His smiles are contagious and his laughter can fill a room.  In many ways he is like his father, Akiva, who I met when we were students at Columbia University.  Akiva wore an eyepatch over his right eye, and I soon learned that he cheated death.  Diagnosed at age 25 with an “inoperable” tumor in his brain stem, he was told he had only a few years to live.  He could not accept that as his fate, and sought out every neurosurgeon in the country until he found one willing to operate.  He is now a brain tumor survivor, and a real life superhero who has saved many lives by sharing his experience.  When we chose Idan’s name, we knew we wanted it to convey the miracle that brought him into our lives, and symbolize how he was always interwoven with our past and inextricably linked to our future.  We chose the name Idan, Hebrew for “era,” because what it really means is the passage of time.  Time is ubiquitous but elusive, pure, precious, stubborn and unwavering.  There could not be a more perfect name for our son.

When Idan was 8 1/2 months old, after recovering from a mild cold, he began breathing very quickly. It was every parent’s worst nightmare.  The cold that never quite went away.  No fever, no chills, no problems eating, drinking or sleeping – just a quickened breath – and a hidden danger lurking beneath the surface.  We went back and forth to the doctor, everyone believing it was a sneaky virus that would take its course and go away.  When two weeks passed and Idan began to look fatigued, we took him back to the pediatrician for a second x-ray.  This time, we were rushed to the ER, and Idan was taken to the pediatric ICU to be given oxygen.  He was hospitalized for several days before we found out the culprit, pneumocystis jiroveci pneumonia (“PCP”), a very rare form of pneumonia – caused by the most common of fungus that lives in nearly all of us.  It wreaked havoc on his baby lungs, and he had to be placed on a ventilator for 13 days to help him breathe.  The doctors busily ran tests to determine why he developed PCP, a condition that usually affects those without an immune system.  But he was our healthy 8 month old boy, right?

We soon discovered his immune system was severely compromised, which we would later find out was due to an extremely rare and life-threatening genetic primary immune deficiency disorder called Hyper IgM, where the immune system is unable to fight against even the most harmless of germs such as the fungus that causes PCP.  For 21 days, we stood by his bedside wearing gowns, masks and gloves, unable to hold, kiss, or even touch Idan.  Despite all that, and despite tubes invading every inch of his body, Idan courageously found strength to smile, even to laugh.  We read to him, sang to him, danced for him, and yet, deep down, we knew that he was the one comforting us the entire time.  His smiles and intuitive stares, ones which belonged to a much older adult, not a child, told us he was strong and going to survive the PCP and conquer his immune deficiency.

After three excruciatingly long weeks, Idan’s lungs finally recovered.  He regained his strength and was discharged from the hospital.  The day he was discharged, we ran through the hospital, Idan in my arms, trying to avoid all germs possible so he wouldn’t get sick, but mostly in disbelief that we’d ever make it out of there.

Idan came home and resumed many of his normal routines, and you would never know he spent weeks in the hospital fighting for his life.  Nonetheless, we live in constant fear of another infection.  We are treating Idan’s Hyper IgM with weekly infusions of immunoglobulin replacement therapy to fill some of the holes in his immune system, and antibiotics to prevent recurrences of the PCP.  The immunoglobulin replacement therapy will protect him from the majority of infections, bacteria and viruses, but cannot protect him against everything.  To date, most individuals with Hyper IgM have not survived past their third decade of life, and are often plagued with recurrent infections and high incidence of cancer.  When his lungs have fully healed, Idan will need a stem cell transplant, which, though it comes with its own set of risks and challenges, is the only known cure.  We are incredibly thankful to have already found a match, but now we need to find a hospital capable of transplanting him.  Because his condition only affects 2 in a million people, the doctors who have treated this disease are few and far between.  Just like Akiva did eight years ago, our family is again on the hunt for the doctor who is doing it best, and doing it right.

I have since learned that I am a carrier of Hyper IgM.  Ironically, my husband and I underwent extensive genetic testing prior to having children, but Hyper IgM is so rare it is never tested for, so we never knew I was a carrier.  As a carrier, I do not have any symptoms, but it turns out that there is a 50% chance that my sons will be born with the disease, and a 50% chance that my daughters will be carriers and pass the disease to their sons.  In many ways, we feel incredibly fortunate that this is the first instance of Hyper IgM in my family, considering that the many men in our family who apparently had a 50% chance of being born with the disease were born healthy instead.

As a mother, it tears me apart that I unwittingly passing along this horrible disease to my beautiful baby boy.  All I can do now is protect and fight for my son to make sure he gets the very best treatment, has a successful transplant so that he can live a long, healthy, and normal life.

My husband, who wears his eyepatch with pride, gives me hope that Idan, too, can be cured.  In June, we set up a fundraising page for Idan to assist with medical expenses, and, since then, hundreds of people have lined up to show their support for Idan and to help us through this next battle.  We now have an army behind us, and we’re ready to face whatever comes next.

Our story is a story of hope, resilience, and perseverance against all odds.  Idan, just like his father, will overcome.  After all, he is the son of a superhero.