Idan, my hero

It has been a busy few weeks. At this point in transplant things usually calm down, and doctors visits are more spaced out. For us, less tests and doctor visits would be welcome. Little Idan has had to go in at least twice a week for a visit and a blood test wile we are still trying to adjust his immunosuppressant dosing as well as get him off the steroids. He is on a very low dose of steroids, but his adrenal gland is still not functioning on its own. This means he tests very low when we check his cortisol levels and this can be dangerous if he gets sick or is in an accident. The doctors say it will take time for the adrenal gland to wake up and to be patient. Until then Idan will keep braving the needles and being an amazing sport during doctor visits.

One thing that is never really discussed with all the difficulties of transplant is the crazy amount of medications one is on for a long period of time. Besides the weekly infusions of antibodies, most of the months post-transplant Idan was taking between 9-11 different oral meds each day at 6-7 different time points throughout the day and night. Some days I counted 26 different oral syringes. Now Idan has been truly amazing with taking his meds, and we were lucky to not need a feeding tube during transplant, so he always took his meds orally. In the past month, we have been focused on cutting out the ones we can. We were able to cut out one of the two blood pressure meds he was on. Steroids are almost done, so that means we were able to cut out the antifungal he was on as well. Idan’s swallowing has much improved since we took him off the bottle (no pun intended) and he has been drinking exclusively with a straw for the past few weeks. This means we can cut out the liquid thickeners he was on as well as the acid reflex med. It feels really great to wake up on some mornings and only have to prepare 5 meds with his breakfast. Realizing that most parents probably dread giving their kids one medication, I think we are in a good place.

Our next big journey coming up is our return to Seattle for Idan’s one-year follow-up visit. We have started telling Idan that we are taking a trip to Seattle and will be going on a plane. Oddly, he keeps saying “home?” whenever we mention our trip to Seattle, so I guess he still has some memory of his time there. Nothing wrong with being a bi-coastal baby I guess.

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.

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.

Wow, I cannot believe it has been a whole month since our last blog post. Sorry to have been away so long. I know we post small updates on Facebook, but that is never enough to keep our friends and family really updated. It has been a very busy month and I am going to try to make sure I cover the important parts:

Idan had his first cold in over a year:

This was the scariest part of the month and had us very close to being admitted to the ER. Two weeks ago, on a Monday morning, Idan woke up with what appeared to be a cold. Now, post transplant patients like Idan receive regular infusions of IgG’s (antibodies), which can help Idan clear a common cold and certain viruses, but it can nonetheless quickly turn into a nightmare. Seattle was most concerned about the risk of this turning into a chest cold or pneumonia which Idan would have a much harder time clearing. Idan was running warm and the rule from our immunologist in NYC was that if he hits 100.4 we need to go straight to the ER. We had Idan’s immunologist confer with and prep the bone marrow team at Columbia to see what protocol they had and if we could bypass the ER (which is a germ infested place).  Amanda stayed home from work as Idan’s temperature rose to 100 and it looked like we would be going in.  As the day went on and Idan’s temperature hovered around the 100 mark, Seattle said they wanted us to go to the doctor to have a virus test done to know what we were dealing with.  Thankfully, the fever went down the next morning, and the test results came back shortly after confirming he had the Rhinovirus, a.k.a. the “common cold,” and upped the temperature threshold for going to the hospital to 101F (which he never reached).

All in all it was a scary few days of feeling that we might be admitted any day (last time we were admitted in Seattle for a fever of 100.4 we had to stay for 8 days), and poor little Idan had been suffering with sneezes and a very runny nose. The strangest part about it all is that, because Idan has a Primary Immune Deficiency and has recently underwent transplant, we have been keeping him so safe and healthy that this was his first cold since the PCP last April! Luckily, by the end of the week, Idan’s cold symptoms went away and we were all able to relax a bit.

New Chimerism Results:

Last Monday we sent Idan’s blood to Seattle again and got the result on Friday. Once again very confusing results. As you may recall, last month we were upset to see his donor T-Cells drop from 30% to 15%, and this month they shot back up to 32%. This is very strange behavior and no one really knows what to make of it. Sadly, while last time his Myeliod cells held tight at 20% they have not dropped down to 13%. What this all means is still not known, except we do know that Idan’s donor graft is struggling, and may ultimately fail. But we are holding on tight to our sliver of hope that, if he can hold on to that 32% of donor t-cells, he has a chance at a cure and we would not need to transplant again. So we are still praying and hoping it sticks. Nothing changing with any of his treatment course, and we still hope we can get him off the steroids for the GVHD in the next month or so.

Moving, growing, thriving:

The other big news of the month, and what has kept us very busy is that we moved apartments last week. Since Idan got sick we knew we would need a larger place for him to grow and run around in. A one bedroom is okay for a baby, but not for an active toddler that can’t go to the playground and spends most of the day at home. Luckily, a larger 2 bedroom apartment opened up in our building and we did not have to move very far. Idan is loving the new place. We painted his room colors he chose and he runs around playing hide and go seek and enjoys exploring all the nooks and crannies (still unpacking so there are a lot of them). Idan has also been talking more, putting together sentences and thriving like most boys his age. We are extremely grateful that he has not missed a beat even with all the medical care and constant medical treatments he has to endure.

Next month Idan is turning two and we are very excited (and unbelievably fortunate and blessed) to be able to throw this incredible little boy an intimate birthday celebration for him with close family in our new home.

Hope we can update again soon. Make sure to follow Idan on Facebook.

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.