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.

Day +117: Still In The Game

Although Ezra is not yet off his immunosuppressant medications, his doctor decided to re-test his donor chimerism.  The good news is that the percentage of donor cells went from 3% to 5%.  Not enough to feel any real sense of relief, but we are hoping and praying that this small increase is a sign that the donor cells are still capable of growing. At the very least, the donor cells are still present and where there are cells, there is still hope.  And a little hope goes a very long way.  If these donor cells want to take their time growing, we'll have all the patience in the world, as long as they keep growing.  Thank you all for your prayers - here's proof that they are working.  Please keep it up - Ezra needs you!

Ezra also got his game back - his basketball game, that is.  He likes to shoot hoops with a "real" basketball hoop with Daddy's assistance. Our Little Tikes hoop just won't do.  Since the park is off limits, our friends Randi and Arnie have graciously granted Ezra an open invitation to play b-ball with the hoop in their driveway.  Game on!

 

Day +113: Match Number 50

Today we are proud to announce our 50th bone marrow match for another patient in need! A donor sponsored by the Help4Ezra donor circle (www.giftoflife.org/help4ezra), has matched a 29 year old woman suffering from Hodgkins Lymphoma. Through Ezra's Gift of Life donor circle we have found 50 potentially life-saving matches for other patients. That's 50 patients and their families given hope, 50 potential chances for a cure, 50 miracles. When we first decided to start the campaign to find Ezra a bone marrow donor almost exactly 2 years ago (this picture was taken when Ezra was 16 months old and was used on our first drive flyers), we knew that we were and still are looking for a needle in a haystack. But we always said that if we had to go through these challenges with Ezra, then maybe we can make some good out of it and help other patients. We never imagined helping 50 children and adults and still going strong. It is a privilege, an honor and a blessing to be able to help so many people.

Through bone marrow drives throughout the country and the world and an online campaign, thousands of people have registered as bone marrow donors, hoping to be Ezra's match. And equally as important have been the fundraising efforts to move donors off Gift of Life's waitlist and onto the registry. We could not have done it without everyone rallying behind Ezra and understanding how important it is to swab your cheeks and join the registry. Together, we have registered over 4,300 donors! That is an incredible accomplishment and one that brings us much joy.

While we are praying and hoping that a miracle is happening inside Ezra, until we know that he is cured, we will continue to look for his needle in a haystack - his miracle match. So, if you haven't already swabbed your cheek, or told a friend to register, or made a donation to sponsor the processing of a donor test kit, please consider doing so right now at www.giftoflife.org/help4ezra. There are more miracles waiting to happen.    

 

Day +104: Hanging In

Thank you to everyone for your messages to Ezra that he feel better - they are much appreciated! After a rough week, he is doing better with the pain from the mediport surgery. They accessed the port for the first time today. He had some anxiety and tears, but with the help of numbing cream, he did not seem to feel the needle going in. We are hoping that with time he will get used to the process. It has to be accessed each time we come to clinic, which for now is still several times a week, so there are lots of opportunities for him to get used to it (unfortunately). Clinic is exhausting (as you can see), but at least he has his friends with him.

We've been trying to keep him busy at home with different projects and activities. He loves helping Mommy and Bubbe cook and bake. Chocolate chip-sprinkle cookies are his specialty! He has started to ask to go places like the park. It's really heartbreaking to say no, especially when there is no sense of when he will be able to return to his pre-transplant (still limited) activity.

The plan is still to wait until he is off the immunosuppressants before re-testing for donor cells. The doctors are doing a very, very slow taper and it will be a while before he is off them entirely. Usually Day +100 is considered to be a transplant milestone when the immune system starts to kick in more and some of the post-transplant risks become less likely. No celebrations for us on this Day +100.

Day +96: Mediport surgery

After a very long day, Ezra is home recovering from the mediport surgery. The surgery was almost called off right before he went into the OR by the surgeon who had concerns over Ezra's platelets. After much delay, the transplant team and the surgeon agreed to proceed with the surgery. Our brave little trooper is in a lot of pain. There was some debate over admitting him, but thankfully he was discharged this evening. We are hoping that the pain lessens by the end of the week and that he heals with no major issues. Another week, another challenge for our rock star.

Day +93: Three Long Months

Today marks three months from Ezra's transplant. He had a clinic visit today and the doctor was talking about how well he is doing. No infections, no graft versus host disease. He's eating and drinking well - even trying new foods (case in point, here's a picture of him trying pizza for the first time-finally!). He has energy and his labs look ok. Basically, we are back to where he was before transplant except that his immune system is in worse shape. His neutrophils still aren't as high has they need to be and he is still not making platelets and remains dependent on transfusions.

We are extremely grateful that Ezra is doing so well at this point. If the transplant was failing and he was sick, this would be an entirely different situation. The plan is still to just taper off the immunosuppressants and see if that makes a difference in the number of donor cells. They are tapering these drugs very, very slowly because, as one doctor explained, you can get graft versus host disease from just 1% donor cells and once you get it, there's no turning back. They won't check for donor chimerism again until after he is off these drugs.

Ezra is scheduled for surgery on Monday to have a mediport implanted. He still has his temporary central line, which can't stay much longer. The mediport goes under the skin and is accessed with a needle from the outside. The area will be numbed beforehand each time it is accessed. It is much less intrusive than the central line, has less infection risk and will allow Ezra to take baths. We know it will take some getting used to for him, but child life has armed us with lots of resources to try and explain this new "button" that will be under his skin. The surgery is more invasive than the procedure for the temporary line and requires more time under anesthesia. We are expecting him to have some pain for a while afterwards, but are hopeful that the mediport will ultimately allow him more freedom. We will let everyone know how he is doing after surgery.

And please keep up the prayers and positive thoughts for those donor cells to grow. We are still hoping for a miracle.

Day +86: Falling

Originally, Ezra's doctor had said that she wanted to wait a while before re-testing his chimerism. She changed her mind and re-ran the test at the end of last week. The results now show that Ezra has only 3% donor cells.

The plan for now is to slowly taper off his immunosuppressive drugs. These drugs are used to prevent graft versus host disease, but they work by suppressing the immune system. The thinking is that the donor cells may be able to grow without these drugs interfering. But the same is true for his old immune system that is right now winning this battle. Ezra's old cells will also enjoy the benefit of no immunospressive drugs.

If there is a battle going on inside him, the underdog is struggling. His doctor says that until the tests say he has no donor cells, she believes the donor cells can still grow. She says she has seen this happen with other patients. But we don't need medical degrees to know this is highly unlikely. If these donor cells are going to win, it will take a miracle. They will re-test the chimerism after the immunosuppressive drugs are tapered off

Words don't do justice to how we feel. Susan, a friend whose little boy also went through transplant for Hyper IgM, gave us this wise advice: Deal with what is happening today as that is the only thing that is real. We are trying to do that, but at some point soon, Plan B, if there is a Plan B, is going to have to be figured out. So many of you constantly ask what you can do. For now, all that can be done is to pray and send positive thoughts for those few donor cells to grow.

One positive note: Ezra was negative for parainfluenza virus today. So some part of that broken immune system is doing something.

Day +78: Sick time, Swing time

Ezra has had a cough for the past week or so. Ends up that he has parainfluenza virus - basically a bad cold virus. There's not much to do about it, except watch him closely to make sure it doesn't get worse. So far, he's holding steady and we hope it stays that way. Mommy also presumably has the same thing, so it's sniffles all around in the Fineman house.

Ezra's donor chimerism will not be tested again for a couple of weeks. The doctors want to give it more time. We will keep everyone posted.

On a fun note, Ezra now has his very own swingset. One of Ezra's favorite activities pre-transplant was going to the park. In the rain, in the snow, and sometimes twice a day Ezra went to the park. For now, public playgrounds are off limits. We were/are afraid that he will be heartbroken over not being able to go to his beloved park. So, in an effort to distract him from what he is missing, we bought him a swingset for our backyard. The swingset came while we were still in the hospital - the issue was finding the time to construct it with our crazy lives. Our wonderful friends Lisa and Brian solved that problem for us. They arranged for Bonim Builders (basically a Jewish Habitat for Humanity) to send an incredible team of volunteers over to build Ezra's swingset. With those volunteers, our super handy friend Joe (Ezra is still saying "Joe is in charge"), and Daddy's persistence through a thunderstorm, Ezra has his dream swingset. He is thrilled. He has also caught on to our intention of providing a substitute park. He promptly declared his swingset "Ezra's Park" and wants a sign for his park. Once again, we couldn't have done it without everyone's support. We continue to be truly touched by all the different ways that people have reached out to help us through this scary roller coaster time of our lives.

Day +72: Headed in a Very Wrong Direction

The results of one of the repeat engraftment studies are in and the donor cells have dropped to only 8%. The doctors are saying that the percentage might still go up and that it is too early to rely on these results. They will repeat the tests in a few weeks. This gives us little comfort. The doctors here are saying that we need to wait and they wouldn't do another transplant until he is 6 months post-transplant because of the toxicity from the chemo conditioning.

The thought of going through another transplant after we just got discharged is unbearable. And Ezra's back-up transplant options are not good. We are going to start talking to the other transplant centers that we had previously considered, gather opinions and go from there. In the meantime, we can only hope that next month's testing will show more donor cells.

For now, this is our burden to bear. Ezra is so happy to be home. He doesn't stay still for a moment and runs from toy to toy all day. He is being really good about getting up early to travel back to the hospital on clinic days. We are struggling to get used to the new "norm" of chasing him around with meds, drinks and food all day long. He also has to take meds during the night, so none of us are getting enough rest. We are still happy to take this crazy schedule over being inpatient, but the latest bad news changes everything.

Some of you have asked if Ezra still needs blood and platelets. Yes, he is still requiring platelets on a regular basis. You can be any blood type to donate platelets. He is starting to hold blood a bit longer, but is still transfusion dependent. He is still O+ blood type. Please contact Mary Thomas at 212-639-3335 to schedule an appointment.

Last, this latest news renews the need for Ezra to find a better source of stem cells if he needs another transplant. If he were to find a perfect bone marrow match, it would be a miracle. We spent two years searching for a match for him to no avail. But there's always the chance for a miracle match to appear. Please register as a donor, encourage your friends to become donors or make a donation to cover the cost of processing a donor test kit so someone else can be a donor at www.giftoflife.org/help4ezra.

Thank you all for the love and support.

Day +66: HOME

Home. There are no words that can adequately describe how sweet and wonderful it is to be home. Ezra is beyond excited. The expression on his face when we pulled up to the house was a mixture of joy, amazement and a little hesitation. He ran around outside for a bit, checking out the backyard, the deck, his basketball hoop. Then he spied his toys through the window and he ran inside. He spent the rest of the day running around re-discovering all his toys. It was amazing and also a bit heartbreaking to watch him reacquaint himself with his home.

We can't get over how quiet it is here - no beeping pumps, doors opening and closing, linen drawer slamming, vital signs checks, screaming children. And there is so much space to walk around. And a table to sit at to eat. The simple things.

It was 78 days ago on an early Monday morning that we packed up and said goodbye to our home. We all had tears in our eyes, including Ezra who knew that this time, the trip to the hospital was different. As we shut the door, we did not know if this family of three would return as a family three. We did not know what challenges were ahead of us and we still do not know what we face. We are grateful for so many things: that Ezra is doing well; that he hasn't had an infection; that he has strength and energy; that he is taking his meds, eating and drinking.

We are thrilled to be home, but it is without a sense of relief or confidence that we've accomplished the cure for Ezra that we were hoping for when we left our home on May 7. Will this all have been for nothing? Will the donor cells grow? Will his old dysfunctional immune system grow back? Will nothing grow back? Will he need another transplant in a few months? These are our questions and we have no answers.

Ezra's health is still in a very precarious state. His neutrophils are not holding and he is dependent on a drug to stimulate growth. He is still at high risk for graft versus host disease. He is also still dependent on blood and platelet transfusions. He still has very little immune function and his immune system is in the early stages of regrowth. He will remain essentially in isolation at home for now.

We will be back at the hospital either Monday or Tuesday and will continue to travel back to Sloan 2 to 3 times a week, and possibly more as needed. He will have labs drawn, be seen by the doctors and receive any needed transfusions or medications. At the sign of the slightest complication, he will be re-adimitted.

Through it all, what has kept us going is the love and support of our family, friends and our new family of strangers. Whoever heard of complete strangers sending gifts, dropping off treats and meals, donating blood, saying prayers all because they heard about a little boy named Ezra who wasn't feeling well? We are touched by everyone's kindness and support and appreciate every note, post and comment sent to us. One day we hope to tell Ezra about how he touched so many people and how those people helped him get better.

We will continue to keep everyone posted as Ezra enters this new phase of his journey toward being cured.

Days + 57 to +61: Getting Closer

Yes, we're still here. The very tentative plan for discharge is Friday. The main issue that is keeping us here is that one of the drugs that Ezra takes for graft versus host disease needs to reach a certain level in the blood for it to be effective. The level has finally started to go up after several dose increases, but it still is not quite where they want it. The doctors have called Ezra a "Super Metabolizer." Not exactly an area where we want him to be an overachiever. Maybe this explains why we've always had trouble getting him to gain weight. In any event, now that the level has gone up a little, his doctor is willing to discuss discharge for later this week.

If all goes well, Ezra will be discharged and will continue his recovery on an outpatient basis. He will need to come to clinic here several times a week for long days of testing, medication and transfusions. This schedule will continue for many months. And at the slightest sign of anything problematic, he will need to be re-admitted. But at least we will be going home in between and that's all that matters. Ezra will also need to remain in isolation at home for a long time, but should be allowed outside to play at our house (swingset has been ordered!). We are waiting to learn the specific instructions and limitations.

Leaving can't come soon enough for Ezra. While he remains an amazing trooper stronger than any of us, he is really getting cabin fever now that he is feeling better. One of his obsessions is Rube Goldberg machines (really complicated machines that accomplish a simple task). He watches videos of Rube Goldberg machines on You Tube and he has now taken to creating his own, which basically consists of setting objects up to knock down. All. Day. Long. Time to go. At least he got a visit from Cookie Monster today (well, through the door) and Ezra slipped him a cookie under the door so that they could munch together.

Days +53 to +56: Patience

We are all ready to bust out of this place. Ezra is doing great. He is taking his meds like a champ. Eating well too. He is feeling energetic - a bit too energetic for this tiny hospital room. It feels like it is time to go, but the doctors aren't quite ready for us to go. One of his medications that is necessary to prevent graft versus host disease is very level dependent and they haven't been able to get it to a stable point since he switched to taking it orally. This is basically what is holding us up. We are hopeful that we will be out by the end of next week or the following week if all goes well.

On the cell front, the repeat chimerism studies basically show the same 20% donor cells. The message is still that we have to wait and see whether the donor cells grow over time. They will continue to repeat the studies periodically. The other issue is that his neutrophils dropped earlier this week because he was taken off the drug that stimulates neutrophil growth to see whether his body will hold the counts on its own. He was given a boost of this drug to protect him and the counts went back up. Now they are falling again. The doctors say this is common at this stage, but at some point soon we hope to see his body holding the neutrophil count without help.

We have been starting to learn how to care for Ezra's central line, which will include the dreaded dressing changes. Ezra apparently has been paying close attention to how the nurses care for his line because he asked for a cap change kit to play with, said he was setting up a "sterile field," and perfectly changed his therapy bear's caps. Everyone thought this was hilarious, and it was, but we would feel better if our 3 year old son was pretending to do something other than performing medical procedures. Like we said, it's time to go.

We have also been struggling with finding new ways to keep him entertained and challenged. He is becoming bored with his nightly walks around the hall, so the fabulous Jessica from Child Life put together a scavenger hunt for Ezra. This was his first scavenger hunt and he loved it! After he found his prize he literally ran back to his room and wouldn't even slow down for us to take a picture. Mission accomplished.

Last, many of you have kindly asked if Ezra still needs blood and platelets. The answer is a definite yes. He will continue to need transfusions after discharge. He is still O+ blood type. Platelet donations can be any blood type. Please contact Mary Thomas at the Sloan-Kettering blood bank at 212-639-3335 to make an appointment. We are so grateful for the role all of you have played in helping us cure Ezra.

Days +48 to +52: Working Toward the D Word

The past week has been very difficult. Between Ezra being in pain from the central line surgery and the bad news about the donor cells, times have been tough in Room 922. Thankfully, today was a better day, and although Ezra is still on a steady drip of pain killers, he is having less discomfort from his line.

Many of you have asked what the chimerism results mean for Ezra. The fact is that no one knows how this will play out and we just have to wait to see if the donor cells increase. They will repeat the studies periodically and go from there. The important thing is that he has neutrophils - whether old cells or donor cells. These cells are the first line of defense against infection. Without these cells, Ezra would be in a more urgent situation and we would not be waiting to see what happens next. Even if he was at 100% donor, it would still take many, many months for his complicated immune system to reconstitute and it would still be unclear whether he was cured for possibly about a year from transplant. He will be immune compromised for a long time. There are definitely milestones along the way that we will be hoping his immune system meets. But for now, all we can do is pray that the donor cells continue to grow and that his old immune system takes a backseat.

In the meantime, the doctors have been talking about the D word - discharge. We don't yet know when exactly it will be, but sooner rather than later if all remains status quo. In order to be discharged, Ezra needs to be taking all of his many medications by mouth and he needs to be eating and drinking. We have been working on him taking his meds orally for about 2 weeks now, adding medications very slowly. At first, he was very upset and we were worried he wouldn't be able to keep his medicines down because he hadn't eaten or drank in so long and still had so much stomach upset. But then an amazing thing happened: Ezra taught himself to use deep breathing to calm himself down and take his meds. Some of the services here like karate and yoga have talked to him about deep breathing, but we didn't discuss it in the context of taking his meds. Ezra somehow put it together and said in between sobs "I want to stop crying. Take deep breaths." And sure enough, that's what he did, and was able to take his meds. Now he is more used to them and is taking them without tears. We couldn't be prouder of our brave little fighter.

On the eating and drinking front, his appetite has been coming back and they stopped his TPN Friday night. Yesterday, his stomach was not happy with needing to work again and he was sick, but today he is much better and is chowing down. Here he is eating his milk and cereal - the first "real" food he was willing to try.

Day +47: Bad News, Bad Day

The chimerism results are in and they are not good. The neutrophils that have been growing are only 20% donor. The rest is Ezra. The percentages of donor cells for the other type of cells are even lower. The ideal scenario would have been 100% donor cells. Or something closer to 100%. Not 20% - that won't cut it. Despite the doctor's assurances, the chemo failed to kill off Ezra's old immune system. Despite their confidence that the cells growing are all donor cells, that is not the case. 

They are telling us that it is still early with a cord blood transplant and that the percentage of donor cells may increase with time, and that we just have to wait and see. We do know from our own research that with some immune deficiencies, mixed chimerism is fine. All the patient needs are some properly working cells. It may be that if he stays a mix of donor and Ezra it will be just fine. Or maybe he will reject the donor cells entirely in the end and we are left with nothing. The plan for now is to keep repeating the studies periodically to see if things change, but ultimately they wait 6 months before determining whether to do another transplant with one of Ezra's crappy backup options.

That was the news we woke up to. The day then continued on with Ezra needing surgery to replace his central line, which had developed a problem. We decided to allow them to do a bone marrow aspirate during the procedure in case that gives us any additional information. We were back for about an hour and the surgeon came back to tell us that the x-ray showed that the line was not in place and he needed to go back to surgery. This time the procedure was done in the regular OR guided with an X-ray machine so they could ensure proper placement. We have held Ezra in our arms as he was put to sleep more times than we care to remember. It never gets easier.

For now, we are just focused on managing Ezra's pain and hoping he isn't too traumatized.

Days +41 to +44: Sleepless in New York

We never expected to get much sleep in the hospital, but some nights are decent and some nights there is basically no point in sleeping. Lately, the nights have been our enemy and there is no rest for the weary. Ezra has been suffering from a very painful diaper rash and it keeps him awake at night. We keep trying to wean him from his pain meds, but then it flairs up and they put him back on a steady flow of pain meds. They also downsized his IV pump because the number of meds is decreasing, which is a great thing, but the new type of pump beeps constantly during the night. Irritated tush, beeping pumps, regular vitals and frequent transfusions equals long, long nights.

Ezra's counts continue to go up. Some days the numbers are down, but the trend is generally up. And today his neutrophils jumped into the normal range! We know they may come back down, but we'll take it.

The answer to the big question still has not arrived - are these neutrophils donor or Ezra's old cells? The results of the chimerism testing are due in this week. We are very anxiously awaiting the news. Prayers and positive thoughts that these cells are donor please!

In other exciting news, Ezra finally saw smoke coming out of the smoke stacks that he sees from his windows! It only took 8 weeks. Guess the factory workers are back from their vacation in Queens. And Dr. Ezra is back on service. He needed to wear his scrubs for his stroll around the floor.

Days +37 to +40: Breaking Out...

Into the hallway, that is. With his counts on the up, the doctors encouraged Ezra to start walking the halls at night when there are less people around, provided he is covered with isolation gear. It took Ezra's germophobic parents a day or so to get used to the idea and it took Ezra a day to get comfortable with leaving the room that has been his world for the past 7 weeks, and then we were off.  Right away, Ezra was zipping around the halls while we hustled to keep up.  The fabulous Jessica from Child Life made this awesome space shuttle and hung it on the wall in the hall so that Ezra would have a surprise to motivate him to keep walking.  We are now doing nightly outings.

On the cell front, we had a bit of a set back when Ezra's neutrophil counts dropped enough to cause concern, but thankfully, he is now back on track and the numbers are slowly increasing again. The chimerism tests (the tests that look at whether the cells are donor or Ezra) have been sent and results will be available next week. Please keep thinking and praying that these tests show that the new cells are donor cells. We will keep everyone posted on the results.

Days +35 to +36: Ezra's Blast Off

Today Ezra is officially considered to be engrafted! His neutrophil count was .7 today. It has increased by a tenth each of the past few days, so we are headed in the right direction. We say all of this with hesitation because we want to know that these new cells are donor cells and not Ezra's old cells re-growing. We will not know this information until sometime next week. If the cells are Ezra's old cells, then we are back to where we started, but worse off. The doctors are acting confident that these are donor cells. We decided to accept some of their optimism and started filling in this count calendar, which has been hanging in Ezra's room staring us in the face since we got here.  

Assuming that these are donor cells, we now enter the next phase of this roller coaster ride called transplant where we pray that no graft versus host disease (GVHD) develops. GVHD is when the donor cells attack the recipient's cells. It can range from mild to fatal and can effect different organs of the body.  The one advantage of cord blood is that there tends to be less and milder GVHD. We're praying for none.

Ezra's GI system continues to be a mess. The doctors are not yet sure why. The hope is that as his counts come in and they reduce some of his medications, he will feel better.

Today was an incredible day for another reason. Ezra's beloved music teachers Sean and Cindy from Music for Aardvarks came to jam with Ezra. We wish we had a picture of his face when we said they were coming. Pure joy. They are very special people to make the trek to NYC to help bring some happiness to Ezra.  Mission most definitely accomplished. Love you guys!

So here's to hoping Ezra keeps going up, up, up, up toward a new immune system.

Shabbat Shalom!

Days +33 to +34: Slowly I Grow, Tenth by Tenth

Ezra's neutrophils made it to .5 this morning! They went to .4 yesterday and were at .5 today. We are grateful, excited and relieved, but we aren't ready to celebrate quite yet. Two more things need to happen: 1) the neutrophils need to stay at .5 or above for 3 days and 2) these cells need to be confirmed as donor cells and not Ezra's old cells rearing their ugly head. The doctors are acting very confident that these are donor cells, but they will not know for sure until further testing, which is likely to happen next week. No offense old Ezra cells, but we are SO over you.

Ezra is still struggling with GI issues. Now that his counts are going up, they are taking away a couple of the many antibiotics that he is on. We're hoping that this will help with the nausea, vomiting and pain.

His energy is still awesome when he's not snoozing. His nurse let him stay detached from his pumps for about a half hour during a line change today and he had a blast running around the room uninhibited. Daddy raised the bed up high so he could hang from the end of it like monkey bars - take that Physical Therapy!

He is also enjoying being completely spoiled by the nurses here. For the most part, the nurses here have been fantastic - knowledgeable and caring. Ezra's primary nurse is a nice religious guy from Brooklyn who Ezra calls "a silly guy." He has been known to come in on his days off just to check on Ezra - need we say more? Yesterday, one of our other favorite nurses who Ezra has a crush on, gave him a space shuttle t-shirt and a toy Discovery on top of a 747. Ezra is in heaven. Here he is playing space shuttles with Grandpa in his new favorite shirt and jamming as usual.

Days +31 to +32: I Spy Neutrophils

Good news came on Father's Day - Ezra's neutrophils went from .2 to .3. Finally, some movement! Maybe it was everyone's prayers and positive thoughts. We were worried it might go back down, which has happened before, but the level stayed at .3 today. Ezra's neutrophils need to be at .5 or above for 3 days before he is officially deemed engfrafted, so he still has a lot of work to do. This process is going more slowly than expected, but slow and steady wins the race, right?

Speaking of blood, the blood bank has told us that they would like to get more donors on the schedule for the upcoming weeks, especially platelet donors. The longer Ezra's recovery takes, the longer he will need platelets and blood. So please schedule an appointment if you haven't already and continue to tell your friends. We thank you all for truly giving of yourselves and helping to keep Ezra's little body strong.

All else is status quo here. The usual mix of playing, puking, and snoozing. Here's some Father's Day love.

Days +28 to +30: Stalled

There has been no growth in Ezra's neutrophils since the small increase on Tuesday. The level hasn't decreased, but it hasn't gone up either. The doctors are still acting optimistic and saying that sometimes the growth is slow. Despite their assurances, we are nervous. We would certainly feel better about giving it time if we were seeing some growth, but it's hard to be patient when the stakes are so high. We are pushing towards the outer range of days for expected engraftment and it's a place we would prefer not to be.

Ezra continues to have more and more stomach distress and rashes that come and go. They think the symptoms are drug related, but the medications aren't optional, so we just have to deal. The days are really starting to drag for all of us.

Today was Mommy and Daddy's 10th anniversary. We were hoping for a gift of some neutrophils. Maybe tomorrow. So we had some cupcakes from Bubbe and Zadie and decided to celebrate next year.

Days +24 to +27: Waiting

After starting to despair over the lack of neutrophil growth, we finally saw a tiny increase in Ezra's neutrophils on Tuesday. The numbers did not increase today, but stayed the same. The doctor had scheduled a bone marrow aspiration for tomorrow to check the marrow directly, which would tell us whether anything was growing, and, if yes, whether they are donor or Ezra's cells. This afternoon, she canceled the aspiration because the results of the repeat study looking at Ezra's T cells came back showing 17.5% donor cells, up from 10% last week. She was happy enough with that result that she wants to put off the aspiration and wait until next week to see what the neutrophils are doing. We still need those neutrophils to grow - that is what determines engraftment and we need them to be donor neutrophils. We're ok waiting for them as long as progress is being made and Ezra is staying healthy.
On Sunday, Ezra turned to us out of the blue and said, "I feel my new cells growing." It was kind of eerie and we're not sure what he was feeling. We have been saying for a long time that Ezra is always right because he pretty much is always right. We are praying his track record continues with this one.
Ezra is still dealing with some tummy troubles, but otherwise is doing well. His sleeping schedule is totally screwy with him sleeping a lot during the day and then up until 1:00 in the morning. It's hard to break him of that schedule here where night and day aren't so different. Here's Dr. Ezra hard at work and with the space shuttle he crafted specially designed with 5 booster rockets.
Please continue to pray for new neutrophils!

Will the Donor with the Super Platelets Please Stand Up?

First, we want to thank every single one of you who has donated blood or platelets for Ezra. You are all playing an important role in healing Ezra and you have our deepest gratitude. And to everyone who couldn't donate, thank you for spreading the word so much that the donor room has been flooded with donors for Ezra.
Ezra had a platelet transfusion on Saturday that has been holding much longer than his other transfusions. Since he is receiving directed donations, the doctor asked the blood bank to track the transfusion and ask the donor to donate again. We were told that the donor has agreed, but due to HIPAA rules, we aren't allowed to know the identity of the donor, even though that person donated specifically for Ezra (way to go HIPAA). So, donor with the super platelets, if you're reading this, please contact us at help4ezra@gmail.com so we can thank you over and over again!
And we are hoping that other donations will be equally as super! Thanks to all again for hearing the call and answering it.

Days +21 to +23: Good Days and Bad Days

Yesterday was a rough day for Ezra. He woke up vomiting, with a rash and in a lot of pain. No one was sure of the cause - maybe a reaction to medications, blood products or infection. Infection is the biggest worry. The longer Ezra remains without an immune system, the more susceptible he is to serious infection. We ended up having to give him a drug to sedate him because he was in so much pain. It was hard to watch him experience so much pain. This morning he woke up and the rash was gone and he was back to his playful self. So far the tests for infection that have come back are negative and they have added an antihistamine around the clock in case his symptoms were an allergic reaction to a medication or blood product. The doctors are watching him closely.

There is still no progress with his neutrophils. They are nowhere to be seen. The doctors are saying that it is ok because cord blood can take so long to engraft, but that is no comfort on days like yesterday when everyone was fearing infection. The news cells need to grow and the sooner, the better.

Ezra is still doing plenty of playing, but he has been more tired lately. He is sleeping for bigger chunks of the day, leaving Mommy and Daddy to fight over who gets dibs on the iPad. His little body is dealing with a lot right now and we're happy for him to rest, but it's more fun when he wants to play.

Days +18 to +20: A Little More Good News

Word came today that the repeat study looking at the T cells to see if they are Ezra's or the donor's T cells came back showing that the percentage of donor T cells has doubled to 10%. The doctors don't know if it is Ezra's T cells dying off or the donor cells growing. We obviously hope that it is the donor cells increasing, but either way, this is good news. No neutrophils (the cells that typically come back first) have appeared. The neutrophils are what determines engraftment, so we really need them to start growing. Although the average time for engraftment for cord blood is 21 days, his doctor today reminded us that the range is large and it can take much longer to see engraftment. Point being, we need to be patient.

Our job in the meantime is to continue to keep him infection free and happy. His tummy has been bothering him a bit, but that seems to be from the medications and not an infection. Otherwise, his spirits are amazing. He has so much enthusiasm for everything he does here whether space shuttles, volcanoes or arts and crafts. He was even belly laughing in his sleep last night. The downside of him being so active is that we have to constantly keep him entertained - movies don't cut it for this kid. We are making good use of all the wonderful gifts that have arrived so that we have new projects and toys to keep his interest. Here are some pictures of the Superboy hard at play practicing his karate blocks, in downward dog yoga position and playing some hockey-golf.

Grow cells grow!

Days +15 to +17: A Little Bit of Positive News

On Friday, we received a little bit of good news about those lingering T cells. The lab looked again at whether the T cells were Ezra's cells or the donor's cells. This time, there were triple the number of donor T cells from the prior week's test. The doctor says this is good news because it is an indication that the donor cells in the marrow are growing. However, the donor cells are only 5% of the T cells present - the rest are Ezra's cells and could potentially fight off the donor cells. We're definitely not out of the woods yet by any means, but we'll take this little piece of good news.

Day +21 is the average time it takes for the first cells to grow back, neutrophils, to appear for a cord blood transplant. It can sometimes take more than 30-40 days for engraftment with a cord blood transplant. We're obviously praying that the neutrophils appear as soon as possible. Thursday will be day +21, so let's start thinking grow, cells grow for the week ahead.

Ezra continues to amaze us with his resiliency. He is absolutely making the most of being stuck in a tiny room, and enjoyed spending lots of time playing with Grandma who was back in town this week. His latest interest is volcanoes. When Jessica from Child Life heard about his new love, she proposed we make a volcano. And soon enough Ezra was making vinegar and baking soda eruptions over and over again. This is the new daily activity in Room 922. Pretty amazing what can be accomplished in a tiny hospital room. 

Day +15: Prom

Yes, prom. Sloan-Kettering held its annual pediatrics prom today. They bring in dresses and tuxes donated by stores and designers for the patients and hold a dance with food in an auditorium. Patients in isolation, like Ezra, can't attend, so they bring the party to the rooms. Ezra's friend Jessica from the Child Life Department decided that Mommy needed a gown (or two...shhh, don't tell anyone) and brought an arm load of dresses to choose from. Ezra picked out this purple gown (purple is his favorite color, so it was an obvious choice) and Mommy and Daddy put on tiaras and crowns to get festive. There were some wild dance parties and glow sticks and we had a wacky version of prom in Room 922. Only hope that Ezra doesn't remember this prom so that in 15 years he won't show up at the hospital looking for the party.

In medical news, Ezra continues to do well. He did come down with a fever Tuesday night, but it faded quickly and so far all infection tests are negative. The mucositis is doing better. Despite this, he has not been willing to eat the past few days. We suspect that he is filling up from the TPN, although he may also feel funny inside, but not be able to express it.

T cells are still around and remain at the same level. They are repeating the tests from last week to see if they give us any additional information and are waiting for results. We are still hoping they disappear and are getting more concerned as we approach the time period when the new cells are supposed to start growing.

Ezra is still very active and plays most of the day on his mat. He's been on a Puff the Magic Dragon binge (his all time favorite song) after getting a beautiful pop up version of the book from Vicki and Andy. The song stays on repeat for hours. Whatever makes him happy :)

Days +9 to +11: Long Weekend Recap

The weekend started with craziness and ended on a positive note with Ezra doing well. Saturday morning Ezra woke up drenched in blood from his central line. He was bleeding profusely from his line. We had to quickly get the dressing off, which is a nightmare without floods of blood, and it took several attempts to get another dressing stick. Mommy and Daddy then had to sit for 2 hours holding a bag of fluids on the site to put pressure to stop the bleeding while we waited for platelets to come from the bank. Scariest couple of hours ever. The doctors are trying to find the right balance with his platelet transfusions and are checking to make sure he doesn't have any clotting issues. To all who are donating - rest assured, Ezra is using those platelets.

Ezra also has been vomiting blood from the mucositis and having trouble swallowing, but today he did well and was able to to even eat graham crackers and peanut butter. TPN started this weekend so that we don't have to worry when he doesn't want to eat. Ezra is calling it his chicken soup because that's exactly what it looks like in the IV bag. It also resulted in the addition of this monstrosity of an IV pole in our room, which goes off every half hour at night. Awesome.

The main concern remains the presence of T cells. His counts have remained basically the same over the weekend with some fluctuation up and down. There wasn't much information to be had with the primary doctors away for the weekend. They will repeat some tests later this week, but it seems like we won't really know anything until the time when his donor cells are expected to start showing up in a couple of weeks. We guess even miracles take a little time (said the Fairy Godmother to Cinderella).

Despite all the craziness, Ezra spent the last two days playing and even shooting some hoops. He has adopted a rock star's schedule - up until midnight and sleeping past noon. Tonight he's jamming to the new song he made up called "Sack of Potatoes," inspired by Mommy's frustration at him going limp when trying to get him in his pjs.

Ezra's hair has also been slowly falling out over the past week. His head is still covered, but it is thin. Sad to watch that curly mop head go away.

And we remained fueled up by the special people who made the trek to the hospital to bring us delicious food for the holiday and special treats for Ezra. Thank you so much!

Day +8: T cells, T cells Go Away

Ezra's T cells are still hanging around. Despite the fact that they don't look functional, the doctors remain concerned because they shouldn't be there at all after all the conditioning he received before the transplant.  So we'll take all your prayers for the T cells to disappear over this long holiday/yom tov weekend.

Thank you.

Days +6 and +7: T-Cell Drama

The past two days have been anxiety filled. Instead of decreasing to zero, Ezra's lymphocytes, while still low, were increasing. Not what was supposed to be happening. He should have been at zero by Day +2 and he should have stayed that way. When they looked at his lymphocytes more closely, they were T cells, the type of cell that can cause rejection of the donor cells. With the numbers increasing, the doctors started to get concerned and began running more tests. They even started talking about the transplant failing, one of our worst nightmares. Panic time. The first news to come in today was that the majority of the T cells were Ezra's and not donor cells - that was bad news.  Then they ran a test to see if the T cells were "activated."  Thankfully, late this afternoon, the results came in that the cells are not activated and are basically dead cells. Good news and everyone took a sigh of relief.

His lymphocytes went down a bit today, but they still need to continue to stay down until we know it's time for the new donor cells to appear. So we will be waiting and watching.

We also had a scare when Ezra woke up from his nap yesterday with a huge bruise on his hip that came out of nowhere. A quick CBC showed that his platelets had dropped drastically. Within an hour, we had new platelets from one of Ezra's super donors and the bruise is starting to heal. And today brought signs of mucositis, a common side effect of high dose chemotherapy. Ezra has mouth sores and is gagging on the mucus in his throat. He is still being to be a good sport and trying to eat, but it is painful for him. These issues usually don't resolve until the new cells appear, so we may be facing a couple of weeks of mucositis. On a positive note, the doctors had expected these symptoms to appear last week, so we're one week ahead of the game.

The theme of the week has been space shuttles. Ezra LOVES space shuttles and watches You Tube videos of launches endlessly. During our first miserable weeks here, he wanted nothing to do with space shuttles, but his love for them is back in full force (shout-out to Ezra's girlfriend Shayna and her parents for the awesome new space shuttles). Everyone who walks in the room has to watch a launch. The past few days have certainly felt like a space mission - we are definitely exploring uncharted territory for now.

Days +4 and +5: Going Strong, Maybe Too Strong

The last two days have been great. Ezra is doing really well. He is still energetic and playful.  And amazingly still eating well and avoiding TPN.  The only problem is that we found out that he still has too many T cells left. The chemo should have wiped out his T cells, but there are still some floating around. Ezra's T cells need to be gone in order for the new donor cells to grow and take over. The doctors are running more tests to see if the T cells are Ezra's or some residual T cells that came with the cord blood unit. Then we'll wait a couple of days to see if the counts go down. If the T-cells are his and they don't go down, Ezra may need more chemo. Obviously, we really want to avoid putting his little body through more chemo. So please pray that those T cells do what they are supposed to do and go away. We always knew Ezra was strong, but this is an instance where we need his body to give up a little and wave that white (blood cell) flag of defeat.

We've been completely spoiled by all the delicious food coming our way from everyone who signed up to feed us. We can't thank you all enough for your generosity and thoughtfulness.

Special treat for Mama tonight: Daddy went to the RMH this evening and they offered him free Yankees tickets to tonight's game. He correctly declined, telling them he would no longer be a husband if he accepted. Instead of the tickets, they told him that Macys was holding a special event there and he could pick up a new pair of shoes for Mama. Sweet! Now, that's a good husband! Mama is happy with her new dress shoes...now just need a chance to wear them.

Here are some pics of Ezra and his favorite things - playing guitar and space shuttles.