Day +222: And Then There Were None

The transplant has failed. Ezra has only 1% donor cells left. There is nothing left to do except start the dismal process of talking to the doctors about how just how bad are our bad options. It took us nearly 3 years and countless consultations with doctors across the world to decide to move forward with a less than ideal transplant and now we are back to square one, but with worse options and with the scars of the transplant process fresh on his skin and fresh in our minds and hearts. It may be that the right decision is to not do anything. To let him be. To let him live. And to hope that someday soon that long sought after match appears.

We take comfort in the fact that Ezra is happy and doing well. He has been spending time recently jamming with his beloved music teacher Sean from Music for Aardvarks (a song about pooping on the potty is the new favorite). And we've been taking advantage of his recently granted permission to go to the park when not crowded. We're the crazy ones playing in the freezing cold -- but that also means the park is empty, so perfect playing conditions for us. Ezra has been passing the time at clinic hanging out with his pals Bert and Ernie. Bert "helps" de-access Ezra's port, but Ernie isn't allowed to help because Ezra says he's too much of a troublemaker to be trusted.

We will continue to update this blog with Ezra's progress. Thank you all for your support during these past 7 months, and thank you to those of you who continue to reach out in different ways to bring a smile to Ezra's face. Happy holidays and happy new year.

Day +211: Hoping for a Hanukkah Miracle

This past month has been spent waiting for the immunosuppressants to make their way out of Ezra's system so that we can test the donor chimerism again, which will be done soon.  Now that he has been off the immunosuppressants for about one month, the hope is that the donor cells have been able to grow. If not, then this is probably the end of the road for this transplant, and we will start discussing our options for next steps.

This week we have been joyfully celebrating Hanukkah. Last Hanukkah, we joined up with The Maccabeats, actress Mayim Bialik and Gift of Life to try and find Ezra's miracle bone marrow match. No miracles happened last year for Ezra, although matches for many other patients were found. This year, we are still hoping for a miracle match for Ezra, but first we are hoping that he won't need a match for second transplant and instead his donor cells will grow, grow, grow. That is the Hanukkah miracle we are praying for.

In the meantime, we are spoiling Ezra with Hanukkah fun as much as possible and loving every minute of it. And this year we are celebrating in a big way with a huge menorah on our front lawn. A rabbi from the local Chabad (Jewish outreach organization) randomly stopped by our house and asked if we would be willing to let them place one of their super sized menorahs on our front lawn. We said sure - we could use some extra light in our lives this year. It has been a huge hit with Ezra and the whole neighborhood. We're soaking up the light while we can.

Day +183: Things Fall Apart

The latest chimerism studies show that Ezra's donor cells are back down to 3%.  The doctors had ordered a different type of chimerism test that looks at the specific types of cells to see the percentage of donor cells for each type.  This test came back showing the T cells at only 3% donor and the rest of the cells were 1% or zero.  The only bright spot of these results is that all we care about in Ezra's case are healthy T cells, so of all the types of cells, it's best that the T cells are highest.  But 3% will not do much to fix Ezra's immune system.  After these results came in, the doctors ordered another chimerism test using the same method that we've been using.  Unfortunately, this test also showed a drop to 3% donor cells.

Following these results, he has been taken off of the immunosuppressants.  We were still in the process of the slow taper and had been on hold because Ezra had a rash, and they were concerned it was GVHD.  The rash has thankfully cleared up with a prescription cream and with these bad results, they are no longer concerned about GVHD.  The plan is to wait until the immunosuppressants clear his system, which can take some time, and then re-test the chimerism.  It seems like his doctors do not want to discuss next steps until we reach the point where there are no donor cells.  They seem to want to believe there is still a chance the donor cells will grow.  We know they're right in part because it has happened once, maybe it will happen again.  For now, this all seems ok because we already know the back-up options are bad - very bad - and Ezra is doing well enough that there is no rush. 

Maybe the title of this post isn't completely correct.  Only some things are falling apart.  That which is in Ezra's control is going well.  He is thriving and growing.  For the first time since he was 4 months old when he was first diagnosed, his weight is on the growth chart at the 3rd percentile (Dr. G, hope you're reading this - hooray!).  We are trying hard to keep him occupied at home.  Here is Ezra with the wonderful Sasha from Mini Scribblers Art who has been graciously stopping by to get creative with him.  And he is learning to ride his bike so he can one day keep up with his Daddy.  It is so hard to reconcile the strong boy on the outside with the trouble within.

Safe and Sound

Thank you to everyone for all the messages checking in. We are all fine. We miraculously have power, which is fantastic for many reasons, but most importantly because Ezra takes several medications that require refrigeration. We have some down trees and our street is blocked in both directions (the town told us our street is a "proverbial war zone.") Ezra is on a medical priority list for the town. They checked in on us and said they will work on access to the street for ambulances in case of an emergency.

The biggest issue we've had so far is that our little nature lover started sobbing when he saw the broken trees. Not sure how we're going to get the trees cleared without breaking his heart.

We hope everyone is safe!

Day +151: One Point Higher

The latest chimerism results are in and the percentage of donor cells has increased from 5% to 6%. We are disappointed that the increase was not greater. His doctors are also disappointed and were hoping for a number closer to 10%. We were just hoping for something that would make us feel that Ezra is in a safer place. But we are grateful that there was any increase, no matter how small, because a decrease would put us in a very bad situation. And two months ago, we thought the transplant was definitely failing, so a small increase puts us in a better place than the alternative. So, what do we do now? Wait until the next round of tests and hope that the donor cells continue to grow. We are continuing with the slow immunosuppressant taper. His counts continue to be low, but stable. And his activity is still limited to home, the hospital and the outdoors. There is nothing for us to do except to hope that the graft sticks around and continues to grow, and to keep Ezra infection-free in the meantime.

Ezra continues to make the most out of his limited world and is a constant trooper. When we go to Sloan for his clinic visits, we park in the garage and walk through the tunnels to get to clinic. We pass by a hallway where there is always the smell of woodworking and Ezra asks us each time what they are building. The other day we were with Ezra's pal Jessica from Childlife and we bumped into a Sloan carpenter. She knew about Ezra's woodworking curiosity and we stopped the carpenter to ask about his work. He invited Ezra to come see their woodworking shop. Ezra had a great time checking out the carpentry equipment and talking shop with the carpenters who started calling him their "new employee." And here he is with the plants by the garage that he loves because he thinks they are banana trees. Ezra has a knack for finding fun everywhere - even the bowels of Sloan-Kettering.       

Day +137: Keep on Getting Stronger

Ezra's counts have been starting to stabilize.  His platelets have started to hold without transfusions, and his neutrophils are just above the threshold where medication is required to stimulate growth.  His platelet counts are hovering around the transfuse level, but he has been on the right side of the borderline for the past couple of weeks.  The big question, however, is whose cells are growing: Ezra's old cells, the donor cells, both?  We don't know the answer to this question, but no matter the answer, it is much better and safer for him to be making his own neutrophils and platelets.  We are anxiously awaiting the next donor chimerism test, which will be in the next couple of weeks.  Please, please keep praying for donor cell growth!

Meanwhile, Ezra gets stronger each day.  When we got home from the hospital, he no longer could jump and he struggled with going up and down the stairs.  Now he is like a leapfrog and chuggs his way up and down the stairs.  And he is one happy little dude.  Even on hospital days, he skips around the examination room and sings in the halls, making everyone stop and laugh.  

We've been spending as much time as possible outside, attempting to make up for the summer that never was.  We've been finding quiet outdoor spots that don't involve playgrounds where Ezra can walk around and enjoy the fresh air.  We asked him which season he likes best and he answered "I just like every regular day."  We couldn't agree more.

Day + 123: Happy New Year, Love Ezra

Tonight starts the Jewish New Year and we wanted to take this opportunity to thank our family, friends, and the complete strangers who have entered our lives for the outpourings of support and love for our family this past year.  We have truly learned the meaning of community and are grateful to all of you.

The customary greeting of "Happy and Healthy New Year" seems to have more meaning this year.  We are hoping and praying that this is the year that Ezra is blessed with good health and the chance to live a long life.  Happiness will surely follow.  We are also praying for the health and the end of suffering for all the babies, children and adults who we have met during this journey who are each struggling with medical challenges. 

L'shana tova! Love, Ezra.