Month: June 2013

National IDF (Immune Deficiency Foundation) conference

945278_10100734987086782_1867492044_nJune 28: Greetings from Baltimore! We are in the second leg of our journey to find a cure for Idan. We are in Baltimore attending the national IDF (Immune Deficiency Foundation) conference, along with more than 1,000 other patients and caregivers diagnosed with an immune deficiency and over 30 professionals who have experience treating such diseases.

Among the 1,000 patients and caregivers, it seems there are about four families affected by some form of Hyper IgM here (yes, there are several classes of Hyper IgM). We had an opportunity to meet them, and shake the hand of one of the oldest Hyper IgM patients in history – he just turned 40 – which brought tears to our eyes.

We also spoke to several doctors. We had a very long conversation with a doctor in Chicago performing transplants for Hyper IgM patients, as well as a doctor in Seattle who is an immunologist that works closely with the transplant team. Both had yet another different technique for transplant, and we now have even more options to choose from. Seattle, which was not at the top of our list, has moved directly into our radar and we are considering speaking to their transplant team.

We have learned some new facts along the way, including that Idan has the most severe form of Hyper IgM based on the fact that he has no CD40L function. Apparently there are those that have some function to their CD40L (the cell that allows the T-Cells and the B-Cells to communicate with each other), and in those cases the clinical effects of the Hyper IgM can be milder. Idan, however, has zero CD40L, so his Hyper IgM is more severe. That said, there seems to be a concensus that, with the bactrim and immunoglobulin therapy he is taking and the other precautions we are taking to keep him away from germs, he is likely well protected until transplant.

We have one more day of the conference left. We’ll post another update if we learn anything new tomorrow.

Love to all,

Amanda, Akiva & Idan

Update from Duke

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June 22: Thank you for your warm wishes for our trip to Duke, the first of many trips to hospitals around the country. The trip was well worth it as we learned a lot and we now have a good option for transplant (if we can figure out how to relocate to North Carolina for six months of course). Here is a brief summary of our Duke trip and why we have decided, among other things, to change our goal.Our first and perhaps harshest lesson is that, for a baby Idan’s age and with his disorder, a stem cell transplant poses a 10 to 15% chance of death. Why transplant at all then, you ask? Well, after speaking with Dr. Buckley who has been following many Hyper IgM patients from birth, some until their third and fourth decades of life, the risk of death increases exponentially as Idan gets older and is exposed to more potentially fatal opportunistic infections and most certainly malignant cancers. A 10-15% mortality rate for stem cell transplant begins to look pretty good all things considered, and has the potential to be curative. The key now is to determine how to reduce that risk to the greatest extent possible, and how to make sure that we do this once and we do it right.So, we have heard how Duke plans to reduce this risk. The risk comes from three sources: infections, adverse reaction to chemo, and graft versus host disease. Duke recommends using a lower dose of chemo, certain prophylactic medications, and bone marrow as opposed to peripheral blood or cord blood. Each of these steps will help to reduce the risk of mortality, and also increase the chance Idan will accept the transplant.

Importantly, Dr. Buckley, who has seen Hyper IgM patients for the past 40 years and has seen the entire spectrum of how these individuals were affected by the disorder, both for those who opted for transplant and those for whom transplant was not an option, recommends this: Do the transplant as soon as you can with the institution that has the most experience. Idan is the “picture of health” right now, and thus has the best chance of survival and CURE.

So we have only started our journey, but agree that we need to do this once and do it right. This means visiting all the experts and hearing how they plan on reducing the risk. If it were your child, wouldn’t you go to the end of the earth, too?

Now, for our goal. According to materials provided by Duke, the average cost for families across the country to undergo a transplant is approximately $650,000 for the first year. We hope this is a high estimate in our case, where we have insurance that will cover a large portion of the medical expenses of transplant. However, our insurance does not cover IVIG, a very costly treatment Idan will need frequently for the year during and post transplant. Because this is not covered, we will likely need to pay for private insurance, but we understand there is no guarantee we will be able to purchase insurance in such a short time frame that also covers IVIG and also does not charge us a very high premium because of Idan’s condition. Regardless, we have estimated our expenses to range from $250,000 to $350,000, which is why we’ve raised our goal. We have not included in our goal the other non-medical expenses such as the visits to doctors across the country, loss of income for six months or more, and relocating expenses.

We hope you will continue to share Idan’s story, email your contact lists, and post on your facebook pages and twitter so that we may meet our goal, and, more importantly, be connected with any other experts (be they doctors or researchers) that can help us reduce Idan’s risk so that we can do this once and do it right.

Love and best wishes to all,

Akiva, Amanda & Idan

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After a long drive down to Duke, a long day with doctors, and another long drive after, Idan gets his first beach visit for a few minutes before we head back to NYC. I think he liked it.

 

Getting ready for a trip to Duke

photo (3)June 9th Update: dan update: We saw the pulmonologist last week and he believes Idan’s lungs are doing very well and should be strong enough for transplant. This is great news, and should allow us to move forward quickly once we choose a doctor/hospital. We are getting ready for a trip to Duke to visit one of the expert transplant doctors for Hyper IgM and other immune deficiencies. Wish us luck!

Fundraising update: With your help, we’ve raised almost $40,000 – nearly half of our goal – in only a few days! Idan’s story has been read by over 1,000 people, 261 generously donated, and hundreds more have shared Idan’s story through Facebook, email, and Twitter! Through your efforts, we’ve even been put in touch with another doctor who has performed a successful Hyper IgM transplant. Thank you so much!

Idan’s Story goes public on Facebook

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You all know our son Idan, and many of you have followed his adventures on Facebook. Idan now needs your help! Last month, after a 21 day stay in the ICU, Idan was diagnosed with Hyper IgM, a very rare life-threatening genetic disorder that plagues his immune system. We need your help, so he can live a full, long and healthy life: Please see here:https://www.youcaring.com/fundraiseforidan and feel free to Like, Share and pass along. More details and updates to follow. See here for original Facebook post.

How we got here: Idan’s Story

969070_10100769864402362_917188301_n\How we got here: Idan’s Story

Last July, we stood in front of a crowded room and introduced our baby boy to the world.  We spoke of the long journey that we took to get to this moment in our lives.  Akiva’s quest to find a brain surgeon willing to and capable of operating on his brain stem to remove a tumor threatening his life, and unleashing the superhero within.  Amanda’s pursuit of her childhood dreams.  Our love story.  And now, Idan.  A child born to parents who could not be more grateful, more blessed, more thrilled to begin this new journey as a family.  Hence Idan’s name.  Idan is Hebrew for “time,” or “era.”  We knew our little guy would be unusually strong, and we wanted him to have an equally strong name.  What could be stronger and more precious than time? 

Nearly a year later, Idan has proved us right.  He is precious, uniquely strong, extremely brave, and has began his own journey. Idan has been diagnosed with a very rare and life-threatening immune deficiency disorder that affects only 2 in 1,000,000 people.  This is his story, and how you can help. 

In April, Idan started breathing very quickly.  There were no other symptoms to cause alarm.  He played, ate, and seemed perfectly normal, except for his respiratory rate. 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 looking fatigued, we took him back to the pediatrician for a second x-ray.  We were rushed to the hospital, and Idan was taken to the pediatric ICU to be given oxygen.  He was hospitalized for several days before we found out the culprit, a very rare form of pneumonia caused by the most common of fungus that lives in nearly all of us – PCP.  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 he had a severe immune deficiency, which made him uniquely susceptible to this aggressive fungus that threatened to take his life.  We stood by his bedside, for 21 days, wearing gowns, masks and gloves, unable to hold, kiss, or even touch him.  Despite all that, and despite tubes invading every inch of his body, Idan courageously found strength to smile, even to laugh.  We stood by his bed and read to him, sang to him, danced for him, and 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.  

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.  But after Idan was discharged from the hospital, we faced a new challenge – diagnosing the underlying problem with his immune system that made him uniquely susceptible to the fungus that causes PCP.  It was not long before Idan was diagnosed with Hyper IgM, an extremely rare and life-threatening genetic primary immune deficiency disorder where the immune system fails to fight against even the most harmless of germs – hence the PCP.  We have no family history of the disease and never saw it coming.

Today, we are treating Idan’s Hyper IgM with regular weekly infusions of immunoglobulin replacement therapy and antibiotics to prevent reoccurrences of the PCP.  This 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.  We are hopeful, however, that the medical advancements made in the last ten years with stem cell transplantation in children with Hyper IgM will allow Idan to live a normal healthy life.  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.  Thankfully, we have already found a donor who is a perfect match.

How You Can Help

We need your help raising funds to enable us to make sure that, no matter what happens to us, Idan has the best possible medical care, a successful transplant, and enough funds to cover the medical costs he will incur along the way.  This will help to pay for Idan’s hospitalization for the PCP, extensive genetic testing, weekly infusions of immunoglobulin, frequent trips to the pediatrician, immunologist, pulmonologist, and transplant team, as well as the cost of a stem cell transplant and future hospitalization.   We know we cannot do this alone, and, to that end, we have set up this fundraising page for Idan. 

Please post, send to your family and friends, and share liberally.  We appreciate any and all support that you can provide.  Please note that any gifts to Idan are not tax deductible. If you would like to pray for Idan, his full hebrew name is Idan Yarden Ben Esther.

We will literally go to the ends of the earth to find the best medical care for Idan. This disorder is so rare that there are only a few doctors around the world that have even encountered it, let alone know how to treat it effectively.  We are hopeful that, with your help, Idan will have access to these experts and will be cured.

Thank you,

Amanda, Akiva & Idan

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