Author: Amanda

It is an odd thing to fall in love with a city, but we did.  From the people, who have opened up their hearts and homes without knowing anything more than our name and our story, to constantly finding new places to discover and experience, to the lush green scenery that is now part of our everyday life, we love this city, and Idan does too.  My favorite part has to be the fact that, even though every morning we have woken up to cloudy skies, by the afternoon, the sun is shining brightly in an endless blue sky.  It’s as though the sun is so damn persistent that it will burn its way through all the clouds just to shine down on this remarkable city.

I won’t sugar coat it, though.  It’s not easy to walk the same corridors in Seattle Children’s Hospital as we did once before, to pass by that restroom in the clinic where I had a good long cry after finding out that Idan’s first transplant was failing, or to endure procedures where Idan needs to be anesthetized and goes limp in our arms.  It’s not easy.  But we are comforted by all the familiar faces and the very warm greetings from all the doctors, nurses, physician assistants, pharmacists and all the staff who we got to know so well during Idan’s first transplant.

As expected, Idan has no memory of Seattle as far as we can tell.  In a way, it is a reassuring reminder of how young he is and how much of a distant memory this hopefully will all be some day.  On the other hand, it makes it feel like we’re taking an entirely different child through transplant.  Idan was only 14 months old when he had his first transplant.  Now, he’s almost 4.  He doesn’t recognize the nurse who gave him hugs when he finished a brutal dressing change, or the doctors who we have grown to be friends with and who visited him frequently while he was outpatient.  But he also doesn’t remember the four phlebotomists who took turns soothing him and entertaining him while one of them had to poke multiple times to find a good vein in his chubby arms, or the fact that the blood pressure machine never got a good reading so he had to endure multiple “arm squeezes.”  No, he doesn’t remember anything, but perhaps that is the greatest gift of all.  Idan has heard so much from us about Seattle, the transplant, the hospital and the clinic.  He has watched all the videos from the first transplant, and basically has been preparing for this transplant for the past two years.

Unlike last time, where it seemed that he felt that each probe and poke felt like an assault to his little person, Idan is actually enjoying experiencing each new test or procedure – be it an EKG, echocardiogram, bone marrow aspiration, or even a dental exam (yup).  For Idan, the past two weeks have all been one great adventure.

So instead of screaming during blood draws, he sits patiently, counts the vials, and watches the needle going in without flinching.  Oh, he knows it hurts, and he even announces it will hurt before the needle goes in, but he relaxes his arm and chats happily with the nurse while the vials are being filled.  And he opened his mouth wide for the dentist, taking a bet with her beforehand that she would count 24 teeth.

On Friday, he had his first “surgery” to have a bone marrow aspiration.  He’s heard a lot about surgeries (including his 2013 surgery to place his central line, and the one he will be having on July 11^th to do the same thing), and was so excited to have the surgery that he barely flinched when the IV went in right before the procedure started.  In fact, he was disappointed to wake up and discover that they did the surgery without him watching.  He literally woke up in mid-sentence asking me how they were planning to do the surgery in the back if the IV was in his arm and he couldn’t lie on his side.  When he realized that it was over, he kept repeating sadly, “but I saw when they did my IV surgery, how come I didn’t get to see when they did my back surgery?”

Yes.  This is our incredible, amazingly brave, adorable and joyful little boy.  He wants to experience every ounce of everything, and will muster through the pain and discomfort because he is simply too curious to see how it all plays out.

The past week and a half, we have had a slew of various appointments, and we will have several more tests until July 11^th when Idan’s central line is placed.  We will be admitted into the hospital on July 12^th to begin a 5 day course of chemotherapy, to be followed by a day of rest, and the transplant will occur on July 18^th.  We expect to be in the hospital for at least 35 days, but it all depends on how things go.

In the meantime, we have been having adventures around Seattle.  We went to Bainsbridge Island and Idan took his very first ferry ride.  We visited the Seattle Children’s Research Institute – the very same lab that diagnosed Idan with Hyper IgM before we knew anything about Seattle Children’s Hospital, that processes all his lab results, and that recently published research showing promising results with gene editing as a cure for Hyper IgM.  Idan received a private tour of the science lab and got to see where and how all of his blood gets tested, the different machines they use, and he even peeked into the very cool container storing his blood samples for future research.

So, if you see a smile on our faces in these pictures, it’s real.  We are joyful.  Idan is once again leading the way, and we can’t help but be just a little excited when we’re all together on this great and scary adventure.  We expect some serious storm clouds along the way, but just like the sun in Seattle, together, we are pretty damn persistent, and are hopeful that the clouds will make way for a beautiful blue horizon.

Ever get that feeling, towards the end of an intense dream, that things all of a sudden start malfunctioning or, even weirder, morph into something else?  Where all of a sudden, despite passively accepting all of the nonsensical parts of the dream when it first began, you become aware of the illusion?  Reality seeps in, and you open your eyes.  Maybe sunlight washes over you, maybe the buzz of the alarm shocks you into motion, or maybe it’s the sound of a baby’s cry that gets your feet planted on the ground.  

Sometimes this is comforting, no?  Sometimes you fall into a deep sleep, and the dreams are frightening.  They show you a world where you are alone, fighting, struggling, running.  The moment where the illusion ends is met with a sigh of relief – everything is okay, you’re awake now.  

Sometimes it is the opposite, though.  The dream – the illusion – is a sanctuary against reality.  A peaceful interlude that gives you a break from the chaos.

The other night, I had a dream that I was living in a small claustrophobic room, and directly above my head, there was a hole in the ceiling where I could see trucks and cars passing right over us.  Mindy Kaling from The Mindy Project was asleep on the floor to my right (like I said, nonsensical), and seemed blissfully unaware that we were living right under a highway, and that there was no way out except to climb out of this hole and risk being run over.  As I was plotting our escape, trying to figure out how fast we needed to climb out of the hole and dart across the highway in between the cars zipping by to get to safety on the other side, the walls started morphing, and suddenly our little bunker turned into the back of a truck.  Someone else was driving the truck, and Mindy and I were still in the back, this time being jostled back and forth, not knowing where we were going.  At this point, the illusion started to fade.  I could believe Mindy sleeping on the floor of my underground bunker, but I could not believe that the bunker had turned into a truck.  Reality began to seep in, and I woke up.

I was relieved to be awake.  The sun was shining right through the window, Akiva was sleeping peacefully next to me, and Idan was in the other room, beginning his morning ritual of sweetly talking to himself until either I or Akiva came in to greet him.  

I was relieved to be awake, but had I really woken into reality?  Isn’t our life here, in a way, an illusion?  A peaceful interlude before the chaos of transplant?  The whirlwind hospital days and pre-testing leading up to admission, the unpredictable but inevitable events and incidents post-transplant, the constant trips back and forth to the clinic, the pharmacy, and the hospital, and the gargantuan efforts required to turn the whole trip into an adventure, yet still maintaining some semblance of normalcy, for Idani as every little fiber of his being is tested and challenged… isn’t that reality?  

This week, our home appliances have been taking the biggest hit, each of them taking turns malfunctioning.  The illusion of our peaceful life here is slowly being peeled away, and, come Sunday afternoon, we’ll be jerked into reality.  Maybe it will be the Seattle skyline washed in sunlight, or maybe the wheels touching the ground that shock us into motion, or maybe Idan’s voice sweetly talking to himself as he gazes out the window of the airplane, but we’ll open our eyes, and the illusion will be over.

I don’t know how I’ll feel when I wake up from this particular dream.  I don’t know if I’ll be relieved to be taking our first step towards the end of a horrible nightmare, or if the fear and chaos of transplant will be overwhelming.  But I do know that, as long as Akiva and Idan are by my side, I am ready to wake up.

Today marks the third anniversary since that fateful trip to the ER.  The very first sign we had that our world would come crashing down.  I remember our silent speedy march to the pediatrician’s office that morning.  The devastating news that Idan would need to be transported to the ER, immediately, because he wasn’t getting enough oxygen.  The fights with the doctors to allow us to feed him, with a burning feeling in the pits of both Akiva’s and my stomachs, knowing that his condition was deteriorating right before our eyes.  It’s an indescribable feeling to watch your baby be poked, prodded, and struggling to breathe. To know that the next breath could be his last.

Week 2 in PICU.  April 2013

You all know how the story goes by now… We emerged from the hospital to discover that his pneumonia was simply the introduction to an even scarier world: the world of Hyper IgM.

When we started this journey, we felt so alone.  The disease was so rare, and there was no simple way to reach others who had it.  That all has changed.

After three years, we have built an army of other parents, patients, and physicians who know all about Hyper IgM.  We receive messages and emails on a weekly basis, introducing us to a newly diagnosed family, or patients who have lived with the disease for decades.

Earlier this week, we spoke to a mom of nine children who shared her story with us.  Five boys born with Hyper IgM.  Three of them passed away much too soon.  Two days later, we were contacted by a family who, like us, rushed their son to the ER because of PCP.  And that same week, a family from South Korea, who found the Hyper IgM Foundation’s website and expressed such gratitude that, after so long of fighting this disease on their own, finally they were connected to a community of others facing the same challenges they do on a daily basis.  When these families were introduced to the Hyper IgM community we founded a couple years ago with the few other families we had been fortunate enough to meet, the response was overwhelming – as it always is.  Dozens of comments of welcome, advice, and support.  I read the comments on my way home from work, and burst into tears.

Yes, we were so alone once.  As were all of these families living in different corners of the world.  New York, Louisiana, Ohio, Florida, Texas, Illinois, Canada, Australia, New Zealand, England, the UK, Romania, South Korea – just to name a few.  We have all joined hands, and all of a sudden, one voice was replaced by one hundred voices.  We have supported each other through bone marrow transplants, infections, even cancer and death.  We are not alone anymore.

For the past three years, we have spent every free moment becoming experts on Hyper IgM, consulting with the world’s foremost immunologists and transplant teams, attending conferences and meeting families living with the disease.  Last year, we formed the Hyper IgM Foundation to improve the quality of life and longterm outlook of patients with Hyper IgM, and we have been so grateful for the outpouring of support we have received.  We are already accomplishing our mission, which is in large part due to the tireless efforts of Akiva, who has spent every waking minute working closely with families, physicians, and other immune deficiency networks and foundations.  We have found friends and allies in physicians around the world who volunteer their time to consult with parents about treatment options and frequently lend their wisdom to answer questions raised by members of our community.  Because of the work of the Foundation, there is now heightened attention on critical research that needs to be done to understand more about this rare and tricky disease.  So not only are Akiva, Idan and I not alone anymore, no one with Hyper IgM has to feel alone – ever again.

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.  

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.

Just when we thought everything was going Idan’s way, six days into a happy family trip to Seattle for Idan’s yearly follow up visit post-bone marrow transplant, we received the troubling news that his graft started slipping again.  A chart of all of his chimerisms to date showed us in black and white that, if we follow the same course, we are looking at second transplant or going back to Idan’s horrible disease.

Looking at the chart, Akiva and I were absolutely crestfallen.  The doctor presented us with a few options: either fiddle with the immune suppression and hope it is enough to turn the tide, do a second donor lymphocyte infusion (DLI), or throw our hands up and go into a second transplant.  We chose the second option, a DLI.

So next week, pending insurance clearance etc., we will be admitted into Seattle Children’s Hospital for four days for what can only be described as a mini-transplant.

Before he is admitted, Idan will have a Picc line placed in his arm, similar to the central line, which he will need for the remainder of his stay in Seattle.  On Day -2 of the hospital stay, Idan will receive one dose of a chemotherapy drug called Pentostatin, believed to help aid in engraftment and prevent graft versus host disease (GVHD).  On Day 0, Idan will get his DLI, which is an infusion of the same donor’s t-cells that they’ve been storing for us since his first DLI.  The hope is that the new t-cells act as reinforcements in the battle over Idan’s bone marrow taking place between his own cells and the donor cells he received during his transplant last year.  Idan will hopefully be discharged on Day 1 or Day 2 if all goes well.  There is a 50/50 chance that this will work.  There is a good chance he will get GVHD, which could occur in the liver, the skin, or the gut.  A little bit of GVHD can help bring in the graft, but too much could be deadly.  Idan will need to be in Seattle for approximately two weeks (if all goes well) of monitoring before we’ll be allowed to take him home to New York.

Time can only tell whether we’re making the right choice going forward with the DLI, but in many ways it feels like the only choice.  If we watch this graft slip away, as we have been doing for the last year, and do nothing, we are facing a much harder choice of doing a second transplant or reverting to Hyper IgM.  There is no guarantee this works, and it may do more harm than good, but it’s the best shot we can give Idan’s donor cells to keep fighting for a permanent home in Idan’s marrow.

The weight of these decisions that we’ve been making since Idan’s admission into the hospital over a year and a half ago has taken its toll.  Akiva and I spent several sleepless nights here wondering whether we are risking it all for what has proven to be a poor match, or whether this is our last chance to save the day by grabbing hold of these donor cells, giving them a good shake, and demanding they stay.  We agonize over the effect this will have over Idan.  He’s such a happy good natured little boy, but lurking beneath the surface is very real fear and anxiety that no two-year old should suffer from.

As we were processing the information about Idan’s graft, the doctor went on to answer some of our other lifestyle questions, and surprised us by saying Idan’s immune system, while in a precarious state for the foreseeable future, is actually functioning surprisingly well right now (yet another reason to do everything we can to hold on to this graft).  He is now free to go to the zoo, to a museum, to the aquarium, to have playdates with other healthy children his age, to give his baby cousin hugs and kisses without abandon.  Both Akiva and I released a collective sigh of relief, and I burst into tears for the second time during that meeting.

Although a dark cloud hung over us, the next day, when we took Idan to the zoo for the first time since his diagnosis over a year and a half ago, we felt pure joy.

It’s been an eventful week, to say the least.  As always, we take the good with the bad, and keep moving forward.

Steroid Wean + Cold = Bad Combination

The past few weeks we have been attempting to wean Idan off steroids.  There are several concerns during this process, the most obvious being the return of the GvHD, but a second less obvious but equally dangerous concern relating to the adrenal gland.  When a person is on steroids for a prolonged period of time, their adrenal gland essentially goes to sleep.  The adrenal gland is responsible for many things, from responding to and controlling inflammation, to cuing you to wake up in the morning.  Think of the phrase “adrenalin.”  It kicks in when you need it most, but most of the time you don’t.  As we were weaning off the steroids, it became clear that it would take a bit more time than anticipated for Idan’s adrenal gland to “wake up,” and remember what it is supposed to do.  Not much longer, but at least a few weeks.

Of course in the last few days of the wean, Idan caught a cold.  At the time, his adrenal gland was still a bit groggy, so-to-speak, and didn’t quite know how to “kick in” to fight the cold properly.  So we spent most of Saturday of last week (for those who have been following our Facebook page, that was our 5^th Anniversary) neurotically taking his temperature and blowing his nose every 2 minutes.  Idan’s temperature stayed just below the fever threshold, but just high enough to keep us panicked about a late night trip to the ER.  Idan tossed and turned and whimpered the entire night, a little furnace in between Akiva and I.  But some time between 4:30 and 6, he fell asleep finally.

Thankfully, his fever broke and it was back to playing as usual on Sunday, but this last cold has put us through the ringer.  Every time we fall into a nice routine, we get a kick to the shin, reminding us to be as vigilant as ever.

Chimerisms and Med Changes

Meanwhile, it feels like we’ve been at the doctor’s office every day for the past few weeks.  We are weaning Idan off two other medications as we wean the steroids, which translates to multiple blood draws and vital sign checks each week to make sure his body is handling the changes in dosing and medications well.

But the good – no great – news is that, after all this, Idan will be saying goodbye to three of the thirteen medications he is currently on.  This is a huge step in the right direction.

The latest chimerism results are also in, and – for the first time in 9 ½ months – we can take a short breather.  Idan’s chimerisms are generally stable, with his T-cells hanging on at 32%, and myeloid only dropping slightly to 10%.  For now, the doctors are very cautiously optimistic that the graft has a shot of hanging on for good.  Although we had hoped to start weaning Idan off the other two immune suppressants by now, Seattle would like to keep them on for another 8 weeks at their current dosage and check Idan’s chimerisms again at the end of September.  If the graft stays stable, we should be able to start the long wean off of each of them in October, just in time for our return visit for Idan’s one-year follow-up back west.