Idan, my hero

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.

Today we officially mark the 100th day post-transplant milestone. They say that the first 100 days of transplant are the hardest, and carry the most risk of infection and GvHD. Idan has made it to this point in great shape and has continued to grow and thrive despite everything he has been through. This is of course a reason to be very happy that we reached this point. Then again, Amanda and I always thought that by day 100, we would be well on our way towards a cure for Idan and preparing to return to our home and family in NYC. Instead, we are still in limbo and nothing much is changing at this point. We are staying in Seattle, for now, and Idan’s central line will stay in as well. We stay on most of the oral meds he has been on for the last 3 months including the immunosuppression that both keeps his immune system low but also carries other side effects. And we are also no closer to knowing if Idan will be cured, with only 12% donor T-cells and a graft that has not shown itself to be stable yet.

And yet, we stay positive and hopeful that things will get better and Idan will be cured. Having an optimistic approach to Idan’s challenges is the only way I know how to deal with adversary. Any other approach, in my mind is futile and lacks utility. I don’t spend my time thinking of the “what if’s” or second guessing decisions I have made. My positive attitude is directly correlated to the fact that Amanda and I have made our own informed decisions regarding Idan’s health. It is for this reason that I am positive that we both made the right decisions and that things will be okay. After all, this is the belief that allowed me to make it to this point in in the first place.

I was not supposed to be here. At least, if you go according to a couple dozen top neurosurgeons and neuro-oncologists. Eight and a half years ago I was diagnosed with an “inoperable” brain tumor in my brain stem. I was told I could not have it removed, and I was told if I tried, I would most likely die, or become so disabled that quality of life would not be worth the risk. Then, like now, I was not content to give up, and instead spent months, with my family, researching and finding the best option, one that can give me a chance. As with Idan’s Seattle Children’s Hospital, my saviour would be a so-called “cowboy” surgeon in Arizona. I was told by almost every other doctor not to to go down there and was “fired” as a patient from some for doing so. But it was my decision, and my true conviction that things will be okay, and that I can beat this, that allowed me to leap off the cliff and face Fear himself and survive.

It was that decision, to live, that led me to this point. It was the fact that I needed months of rehab to recover that led me to staying an extra year at Columbia. It was that extra year at Columbia that allowed me to meet the girl I had crushed on for 4 years, and it was a cumulation of all my decisions until that point that made me the man Amanda fell in love with. And out of that love, came a new one, a new era, our little Idan.  This is why I know that things will be okay, that Idan will be cured and will survive and do great things in his life. It is because he was not just born to strong willed parents that are survivors, it is that he has an inherent positive outlook as well. He is the one sitting through all the poking, prodding, and procedures. He is the one that received chemo, infusions and surgery, and he is the one that has kept smiling and thriving despite it all.

The 100 day mark is another badge of survivorship that Idan and our family wear with pride. It is not the end of our journey, it is still just the beginning. Today we are thankful that we made it to this point and Idan has stayed healthy and we mark another badge of honor on the wall of our life’s journey.  We have many more voyages ahead, and probably some stormy weather, but we know that we can face these challenges and that we will come out stronger. Idan has been our hero since this all begun and he continues to be the brave little boy leading our way.

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.