Idan, my hero

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

All fun and games have to end sometime. Yesterday afternoon we found out that Idan tested positive for the Rotavirus. It was just as we came back from a fun lap around the floor with Idan dressed up as a vampire, walking around flirting and blowing kisses to all the nurses. My joke of course was, “I am count Dracula, I am here to suck your bone marrow!” Very funny, Akiva.

We were very surprised about the news, as Idan has been asymptomatic and did not have diarrhea. Nonetheless, it was very scary to hear, as they changed the rules for our room and put us in contact isolation. This means all the nurses and staff have to gown up and put gloves on before they can enter the room. They want to make sure to contain the virus and not pass along to anyone else on the floor. Sadly, having the Rotavirus means also losing all our privileges. Idan cannot leave the room anymore to walk around and see his fans and practice his walking. Amanda and I cannot use any of the common rooms, so no use of the kitchen in the family room, or even go to the complimentary coffee cart that comes by every afternoon.

We have been fortunate so far that Idan’s virus looks like a mild strain, but it is also possible that they caught it at the very beginning and it is going to get worse as days go by. The main symptoms are diarrhea and generally feeling run-down, but he can also come down with a fever and runny nose. There is no way to know if he got the virus now, or if it is a reactivation of the live virus vaccine he got when he was 4 months old, in which case it was just waiting to come back once he had no immune system left. (As a side note, this reaffirms our decision to go into transplant so soon – if he could reactivate a virus he received by way of a vaccination, then years worth of infections and other viruses would pose an even more significant threat during transplant.)

For now, we can control some of the symptoms with more IVIG and fluid replacement if necessary, but we’ll have to wait until his donor cells start working until he can clear the virus himself. So they gave him an early dose of IVIG to help with some of the virus, but they do not know how much that works. One option if he does start having bad diarrhea is to have him drink a bit of the IVIG as well, it tends to help.

The next two weeks will obviously be harder than the first two with this new development, but the very good news is that Idan has been doing so well and is going into Day 9 free of any infections or side effects from the chemo, so he is more than strong enough to fight this virus until the donor cells start coming in, which should be only about a week or so from today.  In the meantime, if you have any ideas for how to occupy an energetic toddler in a room 24/7 for the next couple weeks, send them along!

It’s been a couple days since we last wrote, so we wanted to assure you all that Idan is still doing very well.  We are very blessed to have two weeks worth of “less bad” days, and so incredibly fortunate that Idan has tolerated all the drugs well so far.  Idan has nicely adjusted to his new environment, and we are constantly amazed by his ability to make the best of his situation.

Every day we try to spend at least half an hour with Idan on his play mat.  The physical therapist brought it by when we first arrived, and encouraged us to get down on the mat with him and play so that he can continue meeting all of his milestones.  Sure enough, yesterday, we were playing with Idan on a mat in our room when he pulled himself up to a standing position and pushed against a small chair.  He excitedly gestured toward the door and continued pushing forward.  This little guy wants to walk.  So we put on his sneakers, grabbed him by both hands, and walked him up and down the hallway of our unit.  He was thrilled beyond belief, smiling and flirting with all the nurses, making sure to wave and blow kisses even if it meant letting go of one of my hands.

I can’t help but think back to the very first transplant consult we had at Memorial Sloan Kettering in New York back in May.  We asked if Idan would be chained to a bed again, as he was during his first hospitalization.  The doctor replied with a smile, “No, he should have full freedom of movement.  In fact, we’ve had a few toddlers learn to walk in our very halls.”  First, I breathed a sigh of relief.  Then, I thought, how incredibly sad.  As a mom you always dream of the day you see your baby take his first steps.  In fact, I remember thinking of that very thing when Idan was just a little peanut in my belly.  At the time, I was much more concerned with missing the occasion than I was with the how/when/where of it all.   But when I pictured him walking for the first time in a hospital room that day, it broke my heart.

Having been here two weeks, I see things a bit differently.  First, in the same spirit as Akiva’s last post, I feel lucky and grateful that, when he’s ready to let go of our hands, I might be able to witness Idan’s very first steps.  Second, I am surprisingly overjoyed and bursting with pride that Idan may soon take his first steps in a hospital.  It speaks volumes about this little boy’s personality, resilience, and determination.  He marches forward, no matter the circumstances, no matter the obstacles in his way.  He is my hero, and he can walk wherever he damn well pleases.

At first look, it is a bit unbelievable how unlucky my life has been. I mean, not a lot of folk would think that it is lucky to have a brain tumor at age 25. And while the type of tumor was luckily benign, it was located in the most unlucky of places in my brain – smack in the middle of my brainstem! Every time I went to a new neurosurgeon or neurooncologist there was moment of double-take, where the doctor looked surprised and said, “oh, that’s not a good place!” It meant that my tumor was “inoperable” and I would have been out of luck if my family and I did not find a “cowboy” surgeon with the unique skill-set that was needed to save my life.

My luck turned around afterwards. I recovered from brain surgery, had to stay an extra year at Columbia, but got to meet the love of my life as an added bonus. Fast forward a few years, and Amanda and I were lucky enough to have a beautiful seemingly-healthy baby boy. Months went by with us thinking that we are the luckiest people in the world, happy, in love and enjoying our baby boy’s first milestones together.

But luck turned around again, with a rush to the ER, three weeks in the ICU where our son’s life hung by a thread and ended with a diagnosis of Hyper IGM, a 2-in-a-million genetic mutation that left our son with a faulty immune system unable to protect him. The only cure was a transplant, an expensive and risky procedure that meant leaving our home, our jobs and our family for at least six months.

After 10 days in-patient at Seattle Children’s Hospital, spending 24-7 with Idan, tending to his needs while serving his endless demand for entertainment and company, my father pointed out something very true. He wrote to me that “both of you and idan are soo sooo lucky that you are spending this time together and you are watching him grow and develop every second of his day…which of course means every second of your day… but in today’s world how many parents can say they are able to do this…” My father is right. Despite the circumstances and the hard and scary road ahead, we are so lucky to be able to spend this time with Idan. We are lucky to both be around to see him draw for the first time, and say his first words. We are both lucky to be around to see him so happy and playful and full of joy, 24-7. Sure, we have zero income for the next few months, and both our careers are on hold, but we are saving our son’s life, and getting to spend time with our baby boy while doing so.

When Idan is cured, and we are back in NYC, back at work, trying to balance work and family again, when Idan’s army has moved on to help the next little boy in need, we will know that we were lucky to spend so much time with our first born and watch him become the hero he is destined to be.

Peek-a-boo!

An Update:

It is now the afternoon of Day 4.  Since we last updated, Idan has had two very good days, and today is starting out well, too.  Idan is eating, playing, and after 10 days of refusing the bottle, finally showing signs he is ready to drink again.

Despite having several days of multiple chemotherapy drugs, being tied like a leash to an IV pole nearly 24 hours a day, constantly being prodded by nurses to check his vitals, and being pent up in the same room (albeit with a beautiful view) for the last 10 days, Idan continues to amaze and surprise us.  He is full of energy and light.  When we take a walk around the hall, he still perches forward in his stroller, excited to see the world (even if it is just a row of identical hospital rooms).  When we brush his teeth with a little sponge, he grabs it out of our hands and does it for us.  When he wakes up in the middle of the night, he sits up in his crib, reaches for us, and smiles when we come near.  It is a blessing to be in the hospital for a week and a half and see no signs that his personality has been affected.

Although we still cringe to think what the future might bring, we are thankful that he has gotten to this point without any severe toxicities, side effects, or infections, and we are thankful that we chose Seattle Children’s Hospital.  We hope and pray that we can continue to keep him safe and well, and that the donor’s stem cells are working their way towards Idan’s bone marrow as we speak.

Now, for Some Comic Relief, the Bloopers:

So, as you might imagine, two people sharing a small space can make for a reality TV show as it is, but add in lack of sleep, stress, and a toddler full of energy and you have the recipe for a sitcom.  We thought we’d share with you a few of our slapstick moments:

Making a Big Mess:  Akiva has this very large jug he likes to fill with ice coffee – all the way to the top.  It is one of those 32 oz nalgene bottles, I kid you not.  Anyway, every day when he brings it into the room, refilled, Idan goes crazy for it.  He reaches for it, tries to grab it, and protests when we take it away.  Thankfully, he is less interested in the iPhone now that he has found this jug.  If his Aba treasures it, so does Idan.  So one day, I am feeding Idan, and Idan excitedly reaches for something behind me.  I turn around to look, and before I knew it, Idan’s little fingers wormed their way over to the table that Akiva left his jug open.  As soon as I turned back around, the entire jug was all over the floor, along with about 32 oz of coffee!  The very next morning, this time Akiva is feeding Idan.  Now the jug has found its way into some other corner of the room, and once again, Akiva left it open (says Akiva, “I was drinking out of it!”).  Akiva asks me to pull another pair of pants for Idan from the closet as he had made a bit of a mess.  So I pull open the door, and next thing I know, I am standing once again in a puddle of 32 oz of coffee that was hidden underneath the closet door.  Later that day, Idan got his hands in the Rice Krispies bowl, which subsequently wound up all over the floor.  Needless to say, Akiva has finally learned to cap his jug, I am constantly paranoid I will spill a jug of coffee, and all three of us keep hearing Snap, Crackle, & Pop.

What Day is it Again? We all know how days start to melt together when you have no regular weekend.  So yesterday, on my night off, when I set my alarm for 6 a.m., I did not take into consideration the fact it was set to a “weekday” alarm.  In any event, when Akiva called at 8:30, I jumped out of bed, and rushed out of the house to the shuttle that goes from our apartment to the hospital.  When I arrived to the parking lot, it was practically empty, and the shuttle did not come on time.  Nor did it come ten minutes later.  I called the transportation services number three times, puzzled why no one was answering the phone.  Finally I decided to walk (we are only a mile away), and appreciated the exercise.  When I got to the hospital, Akiva was also puzzled why there was no shuttle.  Akiva also remarked how I missed rounds, but they were much smaller than usual, and everyone seemed casually dressed – how odd?  As if a lightbulb went off in our head, we suddenly put two-and-two together, realizing that the faulty alarm, the absent shuttle, the transportation services voicemail, the small rounds and casual clothes were all because everyone else in the world was enjoying their weekend.  Whoops.