Idan, my hero

The latest chimerism test results confirmed what we already feared – Idan’s graft is slipping, and it’s slipping fast.  It seems his myeloid cell line is slipping by 20 percentage points every four weeks.  It is now at 31%, and it is likely it will bottom out before May.  Idan’s NK cell line, which had been stable for some time, dropped 12 points to 70%.  Idan’s T-cells remain stable, for now, as do his B-cells, but without myeloid cells, none of the other lines stand much of a chance.

There are a couple options that we have been given.  Wait and see, which means more likely than not that Idan will lose his graft (i.e., rejection).  Or, we stop all immune suppression and see what happens, but risk severe GvHD or wiping out whatever is left of the graft. The third option, which seems the most promising based on our initial conversations with other patients but not by any means a miracle-drug, is adding a third immunosuppressant which may favor the donor cells this time.  Idan’s immune system is already very compromised with the immune suppressant and steroids, and will be at even more risk with a third immunosuppressant on board.  Our doctors are getting back to us next week with their recommendation, but the bottom line is that the picture is looking grim.

It helps that we are knee-deep in boxes and still all-consumed in the day-to-day routine.  It also helps that, for the first time in six months, when we receive bad news, our family is only moments away for moral support.

The last few weeks, we’ve been packing everything up and trying to make sure Idan wouldn’t be traumatized by the move.  He helped us pack up many of his toys to be shipped back to New York, and all of his artwork that he enjoyed pointing at each morning.  We even bought wall decals to match the ones he had in Seattle in his playroom.  But, to our great surprise, Idan felt right at home from the moment we walked in the door.  He had an intuitive sense of where everything was, almost immediately was walking around our home like he owned the place, and — best of all — has been so very happy.  It hadn’t occurred to us that Idan actually missed home because we thought he’d surely forget the place after spending 6 months away at such a young age.  But no, he is literally singing and dancing all day, sleeping peacefully at night, and waking up with a huge smile.  I can’t help but feel that Idan senses that he is “home.”

For the next several weeks, we will continue with the weekly clinical visit, labs, and continue to be on high guard for temperatures above 100.3.  Idan may be many weeks out from transplant, but he still has a long way to go before he is back to his “status quo.”  So we are continuing to follow the isolation protocols that we did in Seattle.  To our many friends in NYC who are hoping to see us now that we are back, we appreciate your patience and understanding.

We’ve been waiting to say this for a long time, so here goes…. “We’re baa—aack!” – in NYC, that is.  After a dreadful and exhausting flight back, we have our feet on solid ground.  As we start to put the pieces of our apartment (and life) back together, we find ourselves still strong and still full of fight.  We can thank the incredible residents of Seattle for that.  Entire communities welcomed us with open arms and made us feel like home.  We owe so much to so many.  Seattle, this post is for you.

When we brought our son out here for medical treatment last September, we did it knowing full well that we were leaving our home and everything behind for at least six months during what would prove to be the most difficult months of our lives.  We knew a handful of people through friends and family, and only a couple of people did we know beyond a name and contact information.  Today, we are proud to say we know so many of you, and are very honored and humbled by the way you embraced our family and made us feel like home.

The members of this community have gone to great lengths to make our stay here not only palatable, but surprisingly enjoyable. Here are just a few of the ways you have brightened our spirits and given us the strength to help our son thrive through his bone marrow transplant:

You picked us up from the airport in the middle of the night when we first arrived.

You opened your home to us — despite never having met us — so that we could have time to find a more permanent place to stay.

You lent your car to us, one of you for two weeks, the other for six months, without any hesitation.

You organized a toy drive for Idan so that he could enjoy every minute of his day that he wasn’t getting probed or poked by someone.

You donated linens, dishes, pots, pans, appliances, and even furniture — so much so that our new apartment was fully stocked in a matter of days.

You donated gift cards to Target, QFC, Safeway, gas stations, and more, so that we would be able to cover our shopping expenses.

You went to Target and other stores to pick up much needed household items for us.

You mobilized so many others in your community to take Idan up as a cause and to make sure that he never wanted for anything.

You prepared and delivered meals three times a week for the past five months, and made sure that every Shabbat we had a challah on the table.

Your children sent Idan cards, handmade gifts, and posters to hang on the walls that he (and we) will forever treasure.

You displayed Idan’s artwork in your school hallway and made him feel so proud of himself, a feeling that not too many toddlers get to experience.

You brought us treats from New York when you were traveling to and from Seattle.

You visited us in the hospital when Idan was in-patient for several weeks and gave us much-needed breaks.

You visited us in our home, took us out for dinner, and have been shoulders to lean on – and sometimes cry on.

You helped pack up and clear out our apartment when it was time to go.

You have prayed for Idan, sent us your well wishes and words of support with every generous gesture.

All in all, you have been the very foundation that we have stood on, and behind the scenes you have been fighting for Idan as part of his army.  For that, we can only say thank you, and hope that some day, some how, we can show the same generosity to you and other families that has been shown to us.

Thank you.

Love,

Amanda, Akiva, and Idan Zablocki

After a very early start (5 a.m. to be exact), skipping breakfast and an anxious couple hours of waiting around, we are very happy to say that Idan’s central line is finally out!  Although he needed to be sedated for the procedure, they removed it in a matter of minutes.  The line has come to represent so many of the challenges Idan has been put through in the past year, so this was an emotional day for all of us.

When his line was place back in mid-October, it was the first real procedure that would mark the beginning of transplant, and we were set to be admitted the next day to start chemo. It was a very long day back then, as the surgery got pushed off from 10 a.m. to 3:30 p.m., and we all had to entertain a fasting and very cranky Idan for hours only to have him taken away to be intubated and have a scary foreign object placed in his chest. Idan woke up then very groggy, and his line did not stop bleeding for three days. The line came to represent all our fears and guilt about what Idan is going through and we could not wait for the day it would come out.

Over the next 4 1/2 months, however, the line would come very much in handy as they could take blood and give meds without poking or hurting Idan. Over 100 blood draws, 4 blood transfusions, 3 platelet transfusions,  several IVIG transfusions, 5 days of chemo, countless doses of antibiotics and other meds as well as immunosuppressants went in and out of that line. And, of course, two large bags of Idan’s donor’s bone marrow went in through that line. Idan got used to and even began enjoying the routine of waking up at 7 a.m. every morning and having us flush his line with saline syringes and heparin, and he quickly learned to sit very still for the 30 minutes during his complicated and tedious dressing change every week. He knew that he was not allowed to pull or touch the lines, and made sure to shake his head and say “No No No” every time he momentarily forgot and decided to yank on them anyway. The line became an extension of himself, and I am sure that in some way he will miss it. But we will not.  The line posed a constant risk of infection, was a nuisance come bath time and play time, and was an incessant reminder of all that Idan has bravely withstood with a smile on his face but that no little boy should ever have to.  Good riddance!!!!

When we left the hospital, the sun was shining bright in Seattle.  Back at home, Idan was able to play without us constantly bothering him to make sure dangling lines were tucked away.  Best of all, now that Idan’s line is out, we can start making plans for our return trip home to New York.  All in all, it was a great day.

As for Idan’s rash, we have been able to slowly start weaning the steroids while keeping the rash at bay.  We hope the trend continues because the steroids have made sleep all but impossible. Regardless, even if we only dream of uninterrupted sleep, we will all be sleeping “easier” knowing that we are rid of Idan’s central line.

It has been a week since they put Idan on the higher dose of steroids for the GVHD and it seems to have done the trick. So tomorrow we will start tapering the dosage and the long road to get off the steroids. With GVHD there is always a chance that it might return when we start reducing the dose, so the doctors will be monitoring his skin closely as we cut down by 10% every 5 days or so. This means that we will have to deal with all the side effects and extra meds that the steroids come with for a couple more months. It has been hard to see Idan have mood swings and laugh or cry for no apparent reason as well as deal with his increased appetite. After a large breakfast at 7:30AM he starts asking for lunch at 10:30AM and so on. On the bright side we are getting a taste of how Idan will be as a teenager so we will be well prepared 🙂

The test poke in Idan’s arm went really well yesterday. As mentioned in our last post we cannot wait to get his central line out and based on this week’s success we hope they schedule this for next week. Amanda and I were so nervous that Idan would have a fit, or that they would need to poke him several times to find a vein that works.  We spent several days preparing him and telling him about it, even acting it out on each other. Alas, we had a great nurse, and with one poke he got the blood (and a lot of it) and our little superhero did not even flinch! So after over 100 blood draws from his central line it looks like we will finally be getting it out!

Still, it is hard to forget that we are still living in limbo and do not know if Idan will reject his graft or reach a point where his donor graft stabilizes. They will test chimerism again on March 10th and like with each of these tests before, it is a toss up and we really do not know what to expect. the good news we are focusing on is our eminent return to NYC. If tapering the steroids in the next couple of weeks goes well we can continue all our care in NYC with our pediatrician and immunologist.  We will be bringing back a  boy with an even worse off immune system, as he is still on two immunosuppressants, but we hope our life can start to return to normal. Keeping Idan safe, healthy, and happy is definitely going to be a big challenge in NYC and we hope that by the time we head back in mid-March the weather in NYC will improve.

For now we have decided that we need to get out and see Seattle a bit more in these last few weeks here as we both came to the realization that there will be no real vacations for us anytime soon. So we are going to squeeze in some more parks and sites of Seattle in between clinic visits and tests.

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.