Friday, July 27, 2012

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

Thank you all for the love and support.

Friday, July 20, 2012

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.

Tuesday, July 17, 2012

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.

Thursday, July 12, 2012

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.

Sunday, July 8, 2012

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.

Tuesday, July 3, 2012

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.