Author: Amanda

This morning, as I was pulling up Idan’s shorts, he noticed the scar on his leg.  He touched it, furrowed his brows, and — struggling to find words — asked me, “what’s that?”  For the past several months, he has been finding his voice.  Putting sounds together to make words, stringing words together to make sentences, but I was in no way prepared for this question.  Not yet, at least.

I looked in his eyes and my heart broke, knowing full well what that scar symbolizes and how it got there, but not knowing if or how to explain it to him.  Maintaining my composure, I responded, “When you were little,” —when you were little?! —“you spent some time in a hospital.  That scar is from an IV tube, and that IV tube helped save your life. There was a pesky teeny tiny fungus that got caught in your lungs, and medicine was delivered to you through this IV, and it helped the fungus go away.  This was how we learned you had Hyper IgM.”

I know this will only be the first of many questions and conversations about his time in the ICU, the bone marrow transplant, and everything since, and undoubtedly the answers and explanations will be more complicated.  How does a little boy process this information, and for that matter, how does anyone process the information that their very life once hung in the balance?  But I hope, just as Idan found the right words to ask me this morning, we will find the right words to tell him.

Well, it’s official.  Idan will wake up on Sunday morning a 2 year-old.

With everything we’ve been through lately, anniversaries and milestones are reminders to stop for a moment, take a deep breath, and reflect.  Mostly, we feel extraordinarily thankful.  Regardless of the time we’ve “lost,” we’ve gained so much along the way.

Idan is truly a spectacular person who is at once bashful and yet can entertain a room of people.  He is strong-willed, and focuses intently even while playing.  And yet, on a dime, his concentration can be broken into a bout of silliness, songs and dance.

It is therefore somewhat surprising that he is only now turning two.  And it is even more surprising how someone who has been in your life for such a short time can completely take it over, give it new meaning, shape and redefine you so that you are a better person for it.   Not even two years old, and he has already changed the course of our lives.

 Idan, my hero, here is my birthday wish for you:  May you continue to always bring sunshine into a room.  May the sun always shine on you, and may you live another 120 (or more!) years with the best of health, happiness, and goodness.

Well, it seems like we never really get to have an easy chimerism test.  For every glimmer of good news, there’s an alarming bell sounding in the background.  This month, it is no different.

As you may recall, last chimerisms showed a nice uptick in the T-cell line but still a substantial drop in the myeloid cell line.  The T-cells are what we need to replace to cure Idan, but the myeloid cells are also important and necessary to sustain the new T-cells over time.  Idan needs these new T-cells to stick around for the rest of his life, so we’re fighting this battle on two fronts to make sure he not only has a cure, but that he has a cure forever.

With the addition of the new immunosuppressant, MMF, it seems (without jinxing anything) that we may have stopped the myeloid cells from dropping any further.  But, and here’s the real kicker, the T-cells dropped by half this month – from 30% to 15%.  This could be because the T-cells are really dropping, or it could be a random fluctuation.  Once again, we have no certainty and no clarity.

Our hearts sank when we saw the T-cells dropped, and we were only moderately comforted by the fact that the myeloid cells seems to have stopped dropping for now (for the first time in a few months, by the way).

We’ll be repeating the chimerisms next month, and hopefully the news is better and clearer.

Once again, we are reminded of how much support we have, from our family to co-workers and friends.  Akiva’s parents are visiting from Israel and Idan cannot be more thrilled.  The last time he saw them in-person was in Seattle, and things were so different then.  He has Skyped with them nearly every day since and was overjoyed to see them arrive this week.

And this week I received a sweet surprise from several of my co-workers who raised funds for Idan and ran in the NYC Brooklyn Half Marathon in honor and support of his courage and strength.  Since returning to work only a few weeks ago, I’ve learned of the great lengths the staff and co-workers have gone to make my transition smooth and ensure I don’t pick up new germs from the office.  They scrubbed every inch before I returned and even placed Purell in the common areas.  It is truly humbling and encouraging to work with such wonderful people, and Akiva, Idan and I could not be more grateful.

So, as sad as we are to receive yet another set of disappointing results, we’re feeling as strong as ever.  In fact, since returning to New York, not a day has gone by that we’re not happily run ragged by our very energetic little boy who is constantly on the move, constantly surprising us.  He counted to ten for the first time last week, and it was ridiculously adorable.  His energy, sense of humor, and incredible capacity for love isuplifting.

We wanted to share with you a quick update, and a little story that goes back to last summer that has touched our hearts.

First and foremost, Idan is doing well, still looking for ways to get into trouble while keeping us all laughing. We started a third immunosuppressant, which we hope will save the graft from slipping any further. His immune system is now extremely weak, and this means more isolation and more precautions necessary to keep him safe. Thankfully, the drug does not have many side effects and Idan seems to be tolerating it well so far. If it does not work (i.e., the chimerism keeps going down at the same rate), then we will come off the drug after 2 months. If the chimerism holds, then we’ll stay on the drug until it seems his graft has stabilized, which could be several months to a couple years. As they say, there are no guarantees in life, and there are no guarantees this drug will save Idan’s graft, but we’re not ready to give up just yet. In the meantime, we’ve been keeping busy with weekly doctor visits and blood draws and making sure Idan gets to make up for lost time with his grandparents, aunts and uncles.

And now, for our story. After Idan was hospitalized in April, you may recall that we did everything we could do to find out everything we could about his disease and raise money for the astronomical medical expenses down the road. This included a newspaper article in the New York Daily News, among other things. Unbeknownst to us, when our doorman Gus saw the article in the paper, he clipped it and made a bunch of photocopies and distributed them to all of our neighbors. He wanted everyone to help keep Idan safe and stay off the elevator if they were sick and they saw us inside, but mostly, Gus knew we’d need friends in the building looking out for us. We only found out when we saw cards slipped under our door, donations made on our Youcaring site, neighbors frequently asked us how we were doing, and offered to check the mail and bring in packages for us while we were away. When we did find out, we were so touched and caught off guard by the random act of kindness, and all the other random acts of kindness that followed.

Well, this single gesture carried further than we thought. Last week, we were greeted in front of the elevator by our neighbor Gloria. She was deep in a conversation with the doorman when we first walked in, but she glanced in our direction and immediately her eyes welled with tears. We hadn’t seen her since last summer, but, then, we hadn’t exchanged more than a couple pleasantries in the past, so it was a bit surprising at first that she was so emotional. She approached us and joyously exclaimed, “You’re home! You’re home! I’m so glad you are home and he is here, sitting in his stroller, looking so healthy!” It turns out, ever since the day Gus left the copy of the Daily News article in her mailbox months ago, Gloria has been praying daily for Idan. She tells us her family members and friends in Puerto Rico are also praying for him and constantly ask about him. His picture is hanging in her apartment and she thinks of him often.

Later that week, we bumped into our mailman, who also was very excited to see us home. He said there were others in the building asking about us, and to know that we had a lot of people looking out for our little boy.

Many of you know that I grew up in Manhattan and have spent my whole life living in apartment buildings. Despite having dozens of “neighbors” in Manhattan, it is almost unheard of to act neighborly. So I must believe it is something about Idan that brings out the very best in people, and that his cry has reverberated around our building and around the world for a reason. We feel very blessed to know these wonderful people, to live just a few doors down, and know that Idan is better and stronger for it.

We got the results of Idan’s latest chimerism today, and they tell an even more puzzling story.  As you may recall, with Idan’s latest rash, we were hoping for an increase in donor T-cells to explain the sudden graft versus host issue.  The good news is that the donor T-cells have gone up – but only slightly.  They are now at 18%, up from 12%.  The bad news is that Idan’s donor myeloid cells have taken another big hit, and those are now at 52%, down from about 80% a few weeks ago.  If the myeloid cells do drop further, we are still faced with possible rejection down the road.  No one can say for sure how Idan’s graft will grow or change over time, but for now we are hoping that the myeloid stabilizes soon, or it may jeopardize the new T-cells that are starting to come in.  We take some comfort in the fact that, even if the graft stabilizes at this chimerism of 18% T-cells, it may go a long way towards patching the gaping hole in Idan’s immune system.

In other news, Idan’s rash is showing signs of improvement, and we will hopefully be able to start to taper the steroids by this time next week.  Steroids, as you might imagine, have sent him bouncing off the walls with a ton of energy and mood swings.  It will take a few months to taper the dose, so we can expect more of the same for some time now.  Because of the rash, we are unable to do another DLI (which is the only way to intervene to increase the T-cells) as it would most certainly cause more severe graft versus host disease – the kind that we would not even trade Hyper IgM for.  Given the fact that the T-cells have inched up over the past couple months, though, we are content to wait and see what happens next.

Next week, we will be doing a test run of life without a central line.  If there’s one piece of transplant that has been the bane of our existence, it has been the central line.  The central line is a thick tube that sticks out of Idan’s chest and is a direct link to a vein above his heart.  It is held in place by stitches and a dressing that we have to change once a week.  For these reasons, the line poses a high risk of infection post-transplant, and with steroids in the mix, it is even more concerning.  From the beginning, the line has caused us grief, causing little Idan to bleed for several days post-surgery and endure multiple dressing changes over the course of 48 hours during his very first few days in the hospital back in October.  In addition, on a daily basis, the line must be protected and flushed every morning.  At night, we cover it completely during bath time and have gone to such extremes to keep the line and dressing dry that we have stopped baths altogether and only do sponge baths.  It also happens to be an attractive nuisance for a curious little toddler excited to grab and yank on anything in arm’s reach.  The time has come to take it out.  So, instead of drawing from the line next week, we’ll test out a needle poke and see if Idan tolerates that well and if enough blood can be drawn.  If so, we will be taking out the line as soon as they can book an OR.

Once the line is out and the rash is under control, we can start thinking about heading home to New York.  Although we still are in a constant state of anxiety over Idan’s graft, we take joy in the fact that in the near future, Idan will be able to splash in a bathtub filled with water, play with toys other than syringes, and – most importantly – be reunited with his many family members back home.

Idan has a pretty serious skin rash.  We took him to the doctor and she confirmed that he has acute graft versus host disease (GvHD).  We started him on a course of steroids today, which should help calm the rash down.  The steroids will be given at full dose for about 7-10 days and then tapered over the course of the next two to three months.

The bad news is that GvHD is dangerous, can be a bit unpredictable, and throws steroids into the mix.  Steroids cause many bad side effects, including eratic behavior and mood swings, insatiable hunger, weight gain, and facial swelling.  In addition, steroids will suppress his already-weak immune system even further, causing us to retreat just a little bit more into our bubble.

The —-potentially—– good news is that this kind of GvHD is usually associated with (take deep breath and knock on wood) an increase in donor t-cells.  If so, we will take the GvHD and the steroids, and let out a huge sigh of relief.  If not, we will be at a worse position than we were last week, because that would mean Idan is not only failing to engraft but also having to suffer needlessly with GvHD.

We will be testing Idan’s chimerism on Monday, so look out for an update from us by the middle to end of next week.  Until then, we’re happily going to keep ourselves very distracted and busy entertaining a little boy on steroids.

After the dropping chimerism from last week’s test results, we were dreading the news of this week’s chimerism test.  All lines seem to be stable, thank God.  While we are far from where we had hoped to be by Day 100, we are nonetheless grateful that the Myeloid graft appears to be holding on – at least for now.

Looking back at the overall trend, our doctors in Seattle consulted with every known expert on earth about what to do next.  Multiple doctors have voiced the opinion that it is best to sit tight and wait, hold off on the next DLI, for now, and see if the numbers start to go up.  They are all reassured by the fact that his counts have actually remained relatively stable, all things considered.  For this reason, they believe that another DLI at this juncture could do more harm than good as it carries the risk of both GvHD and annihilating Idan’s bone marrow.  The plan for now is to re-check the chimerism on February 10th, and re-consider the DLI if his counts drop.  Until then, a 12% T-cell graft may not be enough to cure Idan’s Hyper IgM, but, if it remains above 10%, it may just be enough to ward off the cancers that often plague children with the disease. 

Amazing how a little morsel of good news (the graft is not in free-fall as we feared) can go a very long way.  The sun was out the past couple days here in Seattle, and if you look really closely, you can see tiny buds beginning to grow on the trees in our neighborhood.  There’s nothing quite like hint of Spring to fill you with hope and the promise of life, no matter how bleak things may look at the time.

(Yes, I went there.)  We are sleepless in Seattle over Idan’s latest chimerism test results.  Once again, the numbers are not where we hoped they’d be, and, worse yet, are indicating that the graft is slipping even further.  Idan’s T-cell count remained low, nudging up only a few percentage points from 7% to 12% donor from the DLI.  This would have been fine if not for the fact that his myeloid cell line, which had been at or near 100% from the beginning, has slipped down to 83% donor.

There are two possibilities for the slip down.  The first and more likely is that Idan is rejecting the graft (probably because he does not have a substantial number of donor T-cells).  The second possibility is that the myeloid cells are just fluctuating and will stabilize at a high number.  We are redoing the chimerism test next week to see if it continues to drop.  If so, we know that we are likely facing rejection.  Obviously, we are hoping and praying for the graft to hold tight and for the T-cells to continue increasing.  We are planning for a second DLI on January 27th, but that plan may change depending on the test results next week.

Without speculating too far into the future, should Idan reject the graft, we will have to gear up for battle again.  There is some new research indicating that Idan’s disease makes him susceptible to an even greater array of opportunistic infections than initially thought, and that the CD40 ligand protein is implicated in more areas of the immune system than just the creation of IgGs (hence why IgG replacement therapy is not sufficient to protect Idan).

A second transplant comes with even more risk than the first one and is a very scary thought, so we are praying that we do not have to go down that road.  That said, we think back to our initial reasons for choosing Seattle and feel comforted knowing that Idan is still strong and did very well throughout the transplant.  There are definitely scenarios far worse than rejection.  No matter what the next chimerism results show, we have no regrets.

We are blessed with a child that fills our heart with joy and laughter, and allows us to  forget our fears and worries.  We hope that we can do the same for him by staying strong, happy and optimistic – but prepared to go back into battle if necessary.

Idan being playfull while trying to avoid bath time

It’s been a long week.  It started with yet another medical mystery.  On Tuesday, we took Idan to the hospital to test his swallowing ability.  Since his first hospitalization in April, he’s had random choking fits while eating or playing that have gotten worse since transplant.  We initially thought it was acid reflux, but decided to take a closer look to make sure bacteria wasn’t quietly entering his lungs with these fits.  It turns out, Idan does have a problem swallowing liquids.  He is having some mild aspiration, which is concerning enough that we are now thickening all of Idan’s drinks to make sure he doesn’t choke.  It doesn’t seem to be connected to the transplant since it started after his intubation last April, but it’s not clear what is causing this problem.  In any event, we are hoping Idan grows out of it.

Getting his X-rays

Shortly after the swallowing test, Akiva felt a scratch in his throat, and we had to separate him from Idan until we knew for sure he did not have a cold or, if he did, he was free of symptoms for at least 48 hours.  Thankfully, the scratch in his throat went away, but the scare meant Idan and I were on our own for two days.  Ever since we came to Seattle, Akiva and I have worked in unison to care for Idan as the three of us hunkered down for his bone marrow transplant. It hadn’t quite dawned on us just how much care and energy our situation necessitates until I was without my partner.

Our routine starts at 7:00 a.m., with Idan’s first medication of the day – tacrolimus.  This must be given every 8 hours.  Idan is usually up by then, so we give it to him right on time.  Then, his central line must be flushed.  His central line splits into two lines.  Both lines must be flushed once a day.  This requires first cleaning the two lines with an alcohol swab, then pushing a saline and then heprin syringe into each line.  After breakfast, we take Idan’s temperature, and then measure out and prepare his 6 or 7 oral medications (depending on the day of the week), and give them to him a ½ ML at a time (more syringes).  After oral meds, we can play, go outside, and do “normal” activities until it’s time to leave for the clinic.  We go to the clinic two or three afternoons a week for Idan’s regular exams, blood draws, infusions, to pick up new meds, and to talk to the pharmacist about any changes to the meds.  These visits can last anywhere from 1 ½ to 3 hours, depending on what’s lined up for the day.  We usually have to pack his 3:00 dose of tacrolimus to make sure he gets it on time.  The clinic visit often ends with a cranky child who, although loves visiting all his friends, gets pretty frustrated that he can’t crawl or walk around.

After clinic, we give Idan more medicine for his acid reflux, and then we have Facetime/Skype chats with family on the East Coast over dinner.  After dinner, Idan gets 5-6 more oral medications.  Then we can get Idan ready for his bath.  Before the bath begins, the entire tub must be thoroughly cleaned, a few fresh towels and washcloths are set aside in a clean area, and Idan’s dressing must be covered.  Small pieces of film are wrapped around the ends of his line to prevent moisture from coming in, and a piece of gauze is placed on some Press N’Seal wrap to cover his entire dressing.  Idan’s entire chest and stomach are covered, and he gets a bath standing up – no splashing in water for him.   Before he goes to bed, he needs another bottle to meet his fluid goals for the day, so we put on some Curious George while he finishes up.  We do one last temperature check before he is carried into the bedroom.  One of us will put Idan to sleep, and the other works on cleaning the rest of the house so it is ready for Idan in the morning.  Our night ends at 11, when we give Idan his last dose of tacrolimus for the day, trying not to wake him.

Imagine doing all of the above with an active toddler who weighs over 30 lbs, and on only a few hours of uninterrupted sleep!  It was a happy reunion when Akiva was able to join us again.  During his period of isolation, Akiva finally got a chance to deal with our insurance and bills that are starting to come in – and finally got a haircut!  I learned how to do all of the above with no help (although Idan does enjoy helping me wrap his central line before bath), and in spite of it all, had a ton of fun with the cutie.  So, not all bad…

Next week is a very busy week.  We have the next chimerism test on Monday morning, bright and early.  The rest of the day/week will be filled with a repeat of all the tests that were done pre-transplant for the post-transplant evaluation typically done between Day 85-100.  These days don’t matter nearly as much for us given Idan’s difficulty engrafting on his T-Cell line, but it is important to know how well his organs have withstood the chemo, etc.

We will have lots to report in a week, so stay tuned…