Month: January 2014

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.

(Yes, I went there.)  We are sleepless in Seattle over Idan’s latest chimerism test results.  Once again, the numbers are not where we hoped they’d be, and, worse yet, are indicating that the graft is slipping even further.  Idan’s T-cell count remained low, nudging up only a few percentage points from 7% to 12% donor from the DLI.  This would have been fine if not for the fact that his myeloid cell line, which had been at or near 100% from the beginning, has slipped down to 83% donor.

There are two possibilities for the slip down.  The first and more likely is that Idan is rejecting the graft (probably because he does not have a substantial number of donor T-cells).  The second possibility is that the myeloid cells are just fluctuating and will stabilize at a high number.  We are redoing the chimerism test next week to see if it continues to drop.  If so, we know that we are likely facing rejection.  Obviously, we are hoping and praying for the graft to hold tight and for the T-cells to continue increasing.  We are planning for a second DLI on January 27th, but that plan may change depending on the test results next week.

Without speculating too far into the future, should Idan reject the graft, we will have to gear up for battle again.  There is some new research indicating that Idan’s disease makes him susceptible to an even greater array of opportunistic infections than initially thought, and that the CD40 ligand protein is implicated in more areas of the immune system than just the creation of IgGs (hence why IgG replacement therapy is not sufficient to protect Idan).

A second transplant comes with even more risk than the first one and is a very scary thought, so we are praying that we do not have to go down that road.  That said, we think back to our initial reasons for choosing Seattle and feel comforted knowing that Idan is still strong and did very well throughout the transplant.  There are definitely scenarios far worse than rejection.  No matter what the next chimerism results show, we have no regrets.

We are blessed with a child that fills our heart with joy and laughter, and allows us to  forget our fears and worries.  We hope that we can do the same for him by staying strong, happy and optimistic – but prepared to go back into battle if necessary.

Idan being playfull while trying to avoid bath time

It’s been a long week.  It started with yet another medical mystery.  On Tuesday, we took Idan to the hospital to test his swallowing ability.  Since his first hospitalization in April, he’s had random choking fits while eating or playing that have gotten worse since transplant.  We initially thought it was acid reflux, but decided to take a closer look to make sure bacteria wasn’t quietly entering his lungs with these fits.  It turns out, Idan does have a problem swallowing liquids.  He is having some mild aspiration, which is concerning enough that we are now thickening all of Idan’s drinks to make sure he doesn’t choke.  It doesn’t seem to be connected to the transplant since it started after his intubation last April, but it’s not clear what is causing this problem.  In any event, we are hoping Idan grows out of it.

Getting his X-rays

Shortly after the swallowing test, Akiva felt a scratch in his throat, and we had to separate him from Idan until we knew for sure he did not have a cold or, if he did, he was free of symptoms for at least 48 hours.  Thankfully, the scratch in his throat went away, but the scare meant Idan and I were on our own for two days.  Ever since we came to Seattle, Akiva and I have worked in unison to care for Idan as the three of us hunkered down for his bone marrow transplant. It hadn’t quite dawned on us just how much care and energy our situation necessitates until I was without my partner.

Our routine starts at 7:00 a.m., with Idan’s first medication of the day – tacrolimus.  This must be given every 8 hours.  Idan is usually up by then, so we give it to him right on time.  Then, his central line must be flushed.  His central line splits into two lines.  Both lines must be flushed once a day.  This requires first cleaning the two lines with an alcohol swab, then pushing a saline and then heprin syringe into each line.  After breakfast, we take Idan’s temperature, and then measure out and prepare his 6 or 7 oral medications (depending on the day of the week), and give them to him a ½ ML at a time (more syringes).  After oral meds, we can play, go outside, and do “normal” activities until it’s time to leave for the clinic.  We go to the clinic two or three afternoons a week for Idan’s regular exams, blood draws, infusions, to pick up new meds, and to talk to the pharmacist about any changes to the meds.  These visits can last anywhere from 1 ½ to 3 hours, depending on what’s lined up for the day.  We usually have to pack his 3:00 dose of tacrolimus to make sure he gets it on time.  The clinic visit often ends with a cranky child who, although loves visiting all his friends, gets pretty frustrated that he can’t crawl or walk around.

After clinic, we give Idan more medicine for his acid reflux, and then we have Facetime/Skype chats with family on the East Coast over dinner.  After dinner, Idan gets 5-6 more oral medications.  Then we can get Idan ready for his bath.  Before the bath begins, the entire tub must be thoroughly cleaned, a few fresh towels and washcloths are set aside in a clean area, and Idan’s dressing must be covered.  Small pieces of film are wrapped around the ends of his line to prevent moisture from coming in, and a piece of gauze is placed on some Press N’Seal wrap to cover his entire dressing.  Idan’s entire chest and stomach are covered, and he gets a bath standing up – no splashing in water for him.   Before he goes to bed, he needs another bottle to meet his fluid goals for the day, so we put on some Curious George while he finishes up.  We do one last temperature check before he is carried into the bedroom.  One of us will put Idan to sleep, and the other works on cleaning the rest of the house so it is ready for Idan in the morning.  Our night ends at 11, when we give Idan his last dose of tacrolimus for the day, trying not to wake him.

Imagine doing all of the above with an active toddler who weighs over 30 lbs, and on only a few hours of uninterrupted sleep!  It was a happy reunion when Akiva was able to join us again.  During his period of isolation, Akiva finally got a chance to deal with our insurance and bills that are starting to come in – and finally got a haircut!  I learned how to do all of the above with no help (although Idan does enjoy helping me wrap his central line before bath), and in spite of it all, had a ton of fun with the cutie.  So, not all bad…

Next week is a very busy week.  We have the next chimerism test on Monday morning, bright and early.  The rest of the day/week will be filled with a repeat of all the tests that were done pre-transplant for the post-transplant evaluation typically done between Day 85-100.  These days don’t matter nearly as much for us given Idan’s difficulty engrafting on his T-Cell line, but it is important to know how well his organs have withstood the chemo, etc.

We will have lots to report in a week, so stay tuned…