Idan, my hero

Today, we have much to celebrate.  Idan has officially engrafted.  His ANC has been above 500 for four days now, and it has jumped to 1,030 this morning!  Since Idan is eating well and is on no pain meds, after over a month in the hospital, we can be discharged — today!

The last few days have been incredible.  The first step towards discharge was the ANC staying stable over 500.  Once that happened, we were able to switch Idan’s last remaining continuous IV med to oral, so he only has to be hooked up at night.  Finally, Idan is no longer tethered to Johnny Five!  He has much more freedom of movement (limited as it is in the hospital).  In addition, since his meds are all in the morning, late afternoon, and evening, we have several hours that Idan isn’t being bothered for anything.  We have our first taste of freedom.  They even gave us a “therapeutic pass” to leave the hospital for a few hours on

We still have a long road ahead of us.  In order for the transplant to be a cure, Idan needs to accept the donor cells.  We tested the percentage of his cells that are the donor’s this morning.  Those results should be back next week.  Even if it is 100% donor, there is a chance that Idan’s own immune system can come back.  So we wait and see.  Until then, we have another 75 days or so when Idan is at the highest risk for infections, viruses, etc., and when GvHD usually presents itself.  So even though we can leave the hospital, we must keep him very isolated for now.  His immune system will start to kick in around Day 180, and it will be an entire year before it is fully functioning.Sunday and Monday.  We visited IdaniWonderland (a.k.a. Idan’s playroom in our apartment a mile from the hospital), and Idan took a nap in his own bed.  These short excursions back to the house have been a much needed mental break for Akiva and I, and have given Idan a chance to slowly adjust to our new apartment here in Seattle.  Despite being in a hospital, this room has been home for him for over a month.

Nonetheless, we have a lot to be thankful for today.  Above all, Idan is leaving the hospital in much the same spirits he entered.  He still wakes up with a smile and shows us all of his funny faces.  He still manages to throw a ball across the room with the force of a much older child.  He still dances and still sings.  We owe a lot of that to all of you.  Idan went into this battle with an army behind him, and it has made all the difference.

Playing with stickers he received from a fan.

It’s been several days since our last update, and we wanted to let you know all is going very well!  Since we last wrote, Idan’s counts have went from 50 to 262!  We are more than halfway to 500, the magic number we need to exceed for two days before Idan can be discharged.  His rash is significantly better, and we hope it will disappear altogether.

The last few days have been a bit of a blur – in a good way.  Idan’s aunts Sabrina and Elisabeth came to visit, and we all had such a fun time.  They gave us much needed breaks throughout the weekend, including to run back home to get the apartment ready for Idan.  The IdaniWonderland is now filled with fun toys and the walls are all decorated.  We can’t wait to see his face when he gets to play untethered to Johnny Five (his IV stand) for the first time in over a month!  This room is a product of Team Idan.  So many of you have sent toys and wall decals to help make his stay in Seattle fun, and we’ve brought many of them to the hospital to entertain him while he’s hospitalized.  Thank you for bringing so many smiles to his beautiful face.

As we start to approach discharge, we’ve had some time to reflect on our stay here at Seattle Children’s Hospital.  It’s truly a place of healing.  The staff here are cheerful and extremely accommodating to crazy NYC parents, and the rooms are spacious, allowing for all sorts of play and activities.  Despite Seattle’s reputation for being a cloudy/rainy city, we’ve been fortunate to see many sunny days (and a few sun showers), and on those days we can see Lake Washington from our window.  Perhaps one of the most unexpected sources of healing has been the widescreen TV that is hooked up to the internet.  A “toy” that we initially thought would be more fun for us has actually become a wonderful way for Idan to keep in touch with his extended family.  Aunts, uncles, cousins and grandparents alike have sent Idan videos which we play off a private Youtube playlist on the big screen.  He excitedly dances along to grandparent’s nursery rhymes and his uncles’ lullaby’s, and sweetly listens to his aunts read his favorite books to him.  He especially squeals when he sees his cousins on the big screen.  When we chose Seattle Children’s Hospital, our main reason was the chemotherapy protocol, but looking back on this overwhelmingly positive experience, we’d choose Seattle again even if they were using the same chemo as every other hospital.

It’s been eight years since Akiva’s “Day 1.”  For those of you who are unfamiliar with my husband’s own story, he underwent very risky brain surgery to remove a tumor from his brainstem on November 8, 2005.  Akiva, this post is for you.

When we first locked eyes across that small table in Lerner (Columbia’s student center), my heart skipped a beat and I had to catch my breath.  Never in my life had I felt that feeling before.  I felt as though my life had just changed course.  Yet, despite intuitively knowing that in that moment I became the luckiest woman on earth, I had no idea just how lucky.

Idan’s disease is inherited from the mother.  It is what they call “X-linked.”  This means that – no matter who the father – there is a 50% chance that my children would be affected by the disease.  Every boy I have has a 50% chance of having the disease as Idan does, and every girl I have has a 50% chance of being a carrier, and thus would carry the burden of knowing that she could pass this along to her children as well.  So when Idan was born with Hyper IgM, it was nothing short of a miracle that he is also Akiva’s son.  Who better to fight Hyper IgM than the son of a superhero?

It’s not just that Akiva learned how to navigate the healthcare system because of his own brush with death eight years ago – which, don’t get me wrong, was instrumental in helping us find Seattle and getting Idani cured – it is his personality.  Akiva is optimistic, certain, determined, and resilient.  He is incredibly funny, silly, and loves to make others laugh.  He will drop everything to lend someone a helping hand.  So how does this play out?  Well, he got me and Idan through the worst days of our lives.  He can entertain a toddler for all his waking hours without breaking a sweat.  In the middle of the night, he wakes up if he so much as hears Idan turn over, and will rush to Idan’s bedside at the slightest hint of discomfort.  Akiva dropped everything to take care of Idan full time when we first received the diagnosis so he could ensure Idan stayed healthy and happily unaware of all the things we had to do (or not do) to keep him safe. Akiva called doctors, fought with insurance, and gave me the strength to continue forging forward despite the many obstacles in our path.  When we thought we’d have to come up with a million dollars to get Idan to Seattle, Akiva smiled and said, “no worries.”  When we needed a jet to get to Seattle, Akiva shamelessly posted the question on Facebook to make sure Idan did not have to fly commercially to his bone marrow transplant.  And in the middle of the night, when Akiva is sleeping at the apartment and I am at the hospital, I get an email asking how things are going.

Akiva, thank you for giving Idan his smile, his resilience, his determination, and most of all, his silly sense of humor.  I could not have asked for a better father for my son.  Happy re-birthday.

We are at the end of Day 15 and Idan got some good news today. He tested negative for the Rotavirus!!! So they lifted his isolation restrictions and he is once again allowed to walk the halls of the transplant unit and blow kisses to all the nurses. This made us all very happy. Not clear how he got rid of the virus – either it was a mistake to begin with (false positive) or his donor’s immune system cleared it (although it would be a bit early for that to happen, we know for sure that Idan’s old faulty immune system could not have cleared it this fast). Either way we are happy it’s gone.

Unfortunately, the skin rash/GvHD is still bothering him. It was very itchy all last night and neither him or Amanda got much sleep. The rash on his back comes and goes so that might be a good sign that it is a mild form of GvHD and it might clear up as his ANC comes in. We started today to treat it with a topical steroid ointment and hope that clears it up.

We are excited for the weekend as Amanda’s sisters are coming out again from NY and Idan will be very happy to see them. It will also give Amanda and I a much needed break and allow us to prepare and clean the apartment for an eventual discharge, we hope soon 🙂

Idan during his Music Therapy

In our never-ending saga of ups and downs, Idan has GvHD.  Thankfully, it is mild for now, but we’re keeping a close eye on it.  Last night we noticed a rash on Idan’s back and side which looks like the start of some skin GVHD. It was better this morning, but now it is red and blotchy again. The doctors say that this is common with the beginning of his counts coming back and the new donor system coming on board to see a hyper-acute form of skin GVHD. They are not concerned yet, and will follow it and if it does get back they will treat it with steroids. As the donor’s bone marrow is taking up shop in Idan’s body GVHD is to be expected and we are just hoping for a mild run with it. The good news is that this likely confirms that the counts we are seeing are in fact the donor cells and not Idan’s old immune system returning.   Overall, the transplant team is still very happy with how Idan is doing. The attending we have had for last three weeks was on his last day of rounds today, and he joked that Idan is the least interesting patient medically, but that they all keep coming around just to play with him 🙂 He said that they are going to start training us now on taking care of his central line, and giving him fluids through it and learning how to flush it with the syringes. They want to get us as ready as they can to go home, as they expect his counts to keep rising and once he is at ANC 500 for a couple of days they will transfer us to the out-patient clinic. Idan is happily taking his oral meds (usually a big hurdle for patients who have undergone chemo) and they hope to transfer most of the IV meds to oral over the next few days. What to expect: If all keeps going well they might discharge us next week. If his GVHD gets worse then it would probably mean two weeks as they would want to treat and monitor. Once we are discharged we move back to the apartment we rented and are still in mostly isolation there. We would have 2-3 days a week which we need to go to the out-patient clinic or the hospital for checkups, tests, meds, or infusions of blood, IVIG or Platelets. The point is to follow him and keep him safe and infection free as his immune system comes back slowly. Once we reach the 100 day post transplant mark they will start talking to us about going back to NYC. Still a long way away. But we are happy that he is doing well and we are excited for returning home, taking Idan to the park, and letting him crawl around without Johnny Five (his IV stand) shadowing him wherever he goes!

This morning, we saw Idan shed his first hairs, a painful reminder of what he has been through in the past few weeks.  But as we say goodbye to Idan’s beautiful hair, we say hello to his new immune system.  Today, we dare to hope.

It’s been almost two weeks since the donor’s cells were infused into Idan’s bloodstream.  Now, we are seeing small signs that they may have made a home in his bone marrow.

There are a few levels that are being monitored on a daily basis to determine the success of the transplant.  The first and only one we need concern ourselves with for the next few months is his neutrophil count.  This is the first type of cell to grow from the donor’s stem cells. Yesterday, after being at 0 for the last two weeks, Idan had 50 neutrophils.  The doctors believe this is an early sign of engraftment – i.e., success – because it is around the time when we’d expect the donor’s cells (now Idan’s cells) to create neutrophils.  We won’t know for sure until Day 28 whether these cells are the donor’s cells growing, or Idan’s own immune system coming back, but given the time frame, the doctors are optimistic – and so are we!  The counts will bounce around a bit over the next few days (and may even go down as neutrophils commonly do), but once they exceed 500 and stay above 500 for at least two days, we will know Idan has engrafted and we will be discharged from the hospital.

Assuming this is indeed a new immune system, the next hurdle we have to be prepared for is graft versus host disease.  It can be acute or it can be chronic, and the levels of severity differ greatly from patient to patient.  We hope that in choosing Seattle, in choosing the treosulfan chemotherapy and an institution with the highest survival rate and excellent treatment of GvH, this will be a minor obstacle at worst.

As for the rotavirus, Idan remains asymptomatic, which means it has not affected him in the slightest – Idan is his usual cheerful and energetic self.  We are still in isolation because it can be contagious and everyone on this floor has a compromised immune system.  The rotavirus will still show up in his labs until he has a fully functioning immune system, so we may be discharged long before we’d be allowed out of isolation.

Thank you all for your continued prayers and support!  Many of you have sent gifts and toys to entertain Idan and they are tremendously appreciated.  We feel blessed to have gone into this transplant healthy and strong, and we owe Idan’s good health all to you.

All our love,

Amanda, Akiva & Idani