Days +285 to +291: We'll Take It

Thankfully, Ezra's counts were good this week. His platelets actually made it to the normal range for the first time since February when the auto-antibodies appeared. His hemoglobin and other indicators of red blood cell destruction were good. His IgG level dropped again, but it was not as much of a drastic drop as the prior weeks. The tests looking more closely at Ezra's immune system showed that he has the type of B cells capable of making IgG. Based on these results, his doctor decided to wait on giving him IVIG. She set a threshold number for his IgG level and if he drops below that number, then he will get IVIG. These test results were reassuring because they show that Ezra's immune function has not been completely suppressed by the steroids and immunosuppressant. We were also happy to see that these B cells were still there because Ezra did not have this type of cell prior to transplant, which means that the donor cells are still working on building a new, functioning immune system.

The steroid wean continues, but the rate of decrease has slowed. The wean has to proceed more slowly now because it is more likely to see flare-ups at lower steroid doses. We of course want him off the steroids as fast as possible, but clearly this is going to be a long process. Good thing we have plenty of practice being patient. 

Even Ezra's liver levels, which had jumped up again in the past month or so, improved this week. Ezra's doctor is very pleased with his progress. We don't know if two weeks of good results make a trend. Maybe it does and maybe it doesn't, but either way, we'll take it.

No exciting visits or adventures to report this week, but the memories of Ezra's visit with Big Igi and his day at the ballpark continue to bring smiles to all our faces. We are also so grateful to those of you who continue to make donations to Ezra's Gift of Life donor circle (www.giftoflife.org/help4ezra) and to other organizations in merit of Ezra's complete recovery. We are touched by the continuing support for our little man. We never imagined that so many people would care so much about a little boy's medical challenges. Thank you for sharing Ezra's quest for a cure with us and for helping others along the way.

Days +278 to +284: Take Me Out to the Ballgame

First, before we get to the exciting news of the week, here is the medical update. As has been the trend lately, there is good news and bad news (better than all bad news!). The good news is that Ezra's counts were great this week. Platelets went up and were higher than they have been since this latest crisis began. Hemoglobin was also up and one of the indicators of red blood cell destruction was down. And this was all with a steroid wean, so good stuff indeed.

The bad news is that Ezra's budding new immune system has been beaten up pretty badly by the steroids and immune suppressant. Either he is no longer making IgG or he is making very little of it. His IgG levels are dropping and he will likely need IVIG again - not because of his platelets, but because he is no longer making enough IgG to protect him from infection. So, we are back to the state of his immune system prior to transplant. When Ezra's body started making IgG post-transplant, it was a huge deal for us because Hyper IgM prevented him from making IgG. It was the first sign of a cure that he was making IgG. And now we lost it, and it feels like a punch in the stomach.

The doctor, however, is confident that this is a temporary issue, and that once Ezra is off the steroids, his immune system will come back. We trust this, but also feel like we have to be guarded - we will believe it when we see it. We are waiting for some additional test results looking more closely at the different cells of his immune system, and then she will make a decision as to whether he needs IVIG now or if we wait a bit longer and continue to check levels. His long-awaited T cells have also taken a deep nose dive. Despite this negative state of immune system affairs, we know that Ezra has the strength to rise again from the ashes. It is just a matter of time and keeping him healthy in the interim. The question is how much time, and that no one knows.

The day we were discharged from the hospital following Ezra's second transplant, we wrote in this blog about how we looked at Yankees Stadium as we sat in traffic on the FDR, and thought that maybe, just maybe, Ezra would get to go to a Yankees game this year. Well, it ends up that his immune system is not strong enough to handle being in the crowds at Yankees Stadium. But that did not stop some very special friends who have made it their business to make Ezra smile. Thanks to these special miracle workers and the generosity of Yankees General Manager Brian Cashman, Ezra got his first day out at the ball game!

Yesterday, Ezra had the opportunity to watch a Yankees game from the safety of a private suite at the stadium. We didn't tell him until yesterday morning, for fear that something would happen and we wouldn't be able to go. We handed him an envelope with the tickets inside. When we explained what the tickets were for, he ran into our arms crying from overwhelming happiness. It was an emotional, beautiful moment.

The weather was spectacular, the view was spectacular - the entire experience was spectacular (except for the Yanks who lost, but hey, there's just so much we can control). We didn't think Ezra would make it through the entire game, but his eyes were glued to the field and he wasn't going to miss a minute of the game.  Thanks to all those involved from the Yankees organization, as well as the devoted people at Project Sunshine who helped with the magic making, no detail was overlooked. The suite was stocked with hot pretzels (Ezra's favorite snack), his name was on the jumbotron, he took home a bag of souvenirs, and they provided purell and sanitizing wipes! Ezra happily fell asleep last night singing "Take Me Out to the Ball Game."

One of the most difficult aspects of Ezra's medical journey is the lack of control we have over his health and really the course of his, and in turn, our lives. What we do have control over is making sure Ezra is as happy as he can be given the circumstances. We are lucky to have people in our lives who feel the same way and have helped us to override the horrible days with incredible days.

Days +270 to +277: In Love With a Big Blue Bear

It was another frustrating medical week. On the plus side, Ezra avoided another round of high dose IVIG. At clinic on Tuesday, his platelets had gone up a little. They are not back to where they were, but because the platelets went up, the doctors did not give IVIG. Platelets remained the same on Friday. One of the indicators of red blood cell destruction was a little elevated this week, but his hemoglobin remained solid. He is clearly still destroying cells, but not at a rate where it is affecting his counts. More importantly, his doctor allowed another steroid wean this week. We will see how his counts look next week after the wean. It all remains day to day, but then again, it always has been day to day.

 

On the downside, we got back some results looking at the state of Ezra's immune system, and it is definitely suppressed. While we need it suppressed so that he can deal with and get rid of these auto-antibodies, it is so, so hard to basically lose the progress Ezra has made over the past six months. Ezra's primary doctor was out of the country this week, and we will have a better understanding of where things stand once she is back. It may be that he bounces back quickly once he is off the immunosuppressants, but there is no time frame for that and once again, we are faced with the reality of an endless journey.

 

The bright spot of our week came in the form of a giant blue bear. Some of you may have seen the video we posted on Ezra's Facebook page of Igi sending love to Ezra (http://youtu.be/OT1KxWoGyXA). Well, Igi is a therapy bear made by Baxter to help children with primary immune deficiency deal with and understand their treatments. Ezra's Igi is called "Ivig" (sounded out), and Ivig has become an integral part of Ezra's life over the past few years. We first introduced Ivig and the medical supplies that he comes with to Ezra when he was two years old. He took to him right away, but it wasn't until Ezra's first transplant that the relationship became critical to Ezra's medical and emotional care. According to Ezra, "Ivig used to live in a bag, but then I took him out and now he's always with me." Ivig became our way of explaining all the terribly difficult challenges that Ezra had to endure. His child life specialist made books starring Ivig to explain every difficult milestone of transplant and beyond. Whatever happened to Ezra, happened to Ivig.

During his second transplant, Ezra saw a picture of the life-size Igi at a conference. Ezra went crazy - laughing and begging us to get giant Igi for him. To have anything bring a smile to our son's face while he was suffering so much meant everything. We know one of the patient advocates with the program and shared with her Ezra's excitement over seeing big Igi and his love for Ivig. We laughed about it and that was that. Then this past February, we heard from her, and it ends up that she had been working for months to arrange a visit for Ezra from big Igi! We couldn't believe it. We set up a date for the visit and eagerly waited for the day to arrive. Unfortunately, it turned out that Ezra was in the hospital for when the visit was originally planned. Everyone was heartbroken (Ezra thankfully did not yet know about the visit).

Touched by Ezra's story, the amazing folks at Baxter arranged for the video of big Igi to cheer him on. Once Ezra was stable, we were able to plan a makeup visit, which happened this past week. There are no words to describe the joy on Ezra's face when he saw giant Igi strolling down the street waving and then turning into our driveway. He then spent an afternoon racing cars with Igi, playing tag and goofing around. For us, the best part was that Ezra was free to hug and hold hands with Igi. Ezra has learned to be so cautious about touching anyone or anything that he isn't certain is safe. We knew that Igi was newly redone for his visit with Ezra and he could be uninhibited with loving that blue bear. We got to watch our son have the time of his life and forget all the pain and suffering for an incredible afternoon.

We are tremendously grateful to everyone who made this visit happen. As you can see from the pictures, it was a dream come true for our little man.

 

 

 

Days + 263 to +269: Never a Straight Path

After our positive update last week, we had another harsh reminder this week that we are not in the clear and this latest set back is not yet behind us. There were two issues this week: 1) the re-testing of the antibodies against red blood cells showed that Ezra is now positive again; and 2) Ezra's platelets decreased on both Tuesday and Friday.  With respect to the red blood cell antibodies, it is a "weak" antibody. Hid doctor said that she has no way of knowing if he is "on the edge of detectability;" in other words, he could just have easily tested negative as positive, or if there has truly been a change. However, the doctor said that we will go by "the whole picture," and his hemoglobin and another indicator of red blood cell destruction has been stable. 

Ezra's platelets, on the other hand, dropped this week. His platelets were getting closer to normal and the drop wasn't as drastic as it has been in the past, but it was enough that his doctor is concerned. His primary doctor was not supposed to be in clinic on Friday, so when she showed up, we knew it was not good news. The plan is to see what his numbers are on Tuesday and then unless the platelets come back up, he is going to get another round of high dose IVIG. It has been 7 weeks since the last dose of IVIG, and we all thought he was done with needing it. The doctor also halted the steroid wean until we know more. We are praying that we show up on Tuesday and his platelets are back up, and this week was all a blip. 

Ezra overall is feeling good and had a great week with lots of outside time. He even walked outside this weekend more than he's ever walked, an impressive feat while on steroids. Best of all, his curls are back! They have not yet appeared on the top of his head, but the back is covered in curls. We can't stop touching them and Ezra thinks we're crazy. May we never again have to wipe clumps of those curls off his pillow. 

We are frustrated with this latest step backwards. Once again, we thought things were improving and now we fear that it is all getting yanked away. We always remain grateful for what we have - and we have a lot - but we also want what every parent wants. We want to feel like our child is safe. Is that too much to ask for?

Days +249 to +262: Laughing in the Rain

Thankfully, things continue to head in positive direction for Ezra. His hemoglobin has been stable, and his platelets are slowly increasing. We found out that he is still positive for neutrophil antibodies, although the antibodies are now considered to be "weak." We are anxiously awaiting results of a re-check of the red blood cell antibodies. He was negative last time, but the doctors are keeping a close eye to make sure that the red blood cell antibodies do not re-surface as we wean the steroids. 

We also re-tested his chimerism. The whole blood results show that he is still 100% donor. We are waiting for the cell line chimerism results. Assuming all is ok on that front, it is comforting to know that when the day comes that he is off steroids and the immunosuppressant, he will hopefully be back on track with building a healthy, new immune system. We have also thankfully been able to stay at twice a week clinic visits for the past two weeks, which makes a huge difference. 

Ezra has been feeling good and loving being able to spend more time outside. We were out for a walk yesterday, and all of sudden the skies opened and we were caught in a downpour. Ezra thought this was hysterical, as we got soaking wet running back home. He could hardly breathe he was laughing so hard! It felt so good to feel so alive, to be away from the stale gray of the hospital, and to be able to laugh in the rain. 

Days +239 to +248: Headed in the Right Direction

We are happy to report that this past week was a good one! The doctors repeated the antibody tests last week. The good news is that Ezra is no longer testing positive for red blood cell antibodies. The bad news is that he still has antibodies against his own platelets. BUT, even though he still has platelet antibodies, his platelets increased this week and he has not needed IVIG. Tomorrow will be four weeks from his last high dose IVIG treatment. This is a huge improvement considering that last month he couldn't make it a few days without needing treatment. 

When we asked the doctor if the red blood cell antibodies can return, she said "not usually," but we could sense the hesitation in her voice, as things with Ezra often don't follow the "usual" path. She is certainly proceeding with caution on the steroid wean and going very slowly. Ezra remains on a significant steroid dose and we remain very concerned about the side effects. 

The other good news is that we are doing a trial run of an oral medication that can replace the medication that Ezra was receiving by IV three times a week at the hospital. This means that if all goes well we get to move down to clinic visits twice a week. Ezra is really excited about this change - we just hope the oral med works out. When we were explaining that for this week, Ezra would not need to go to the bed area at the hospital unless his bloodwork shows he needs medicine, Mr. Comedian responded with "ah, back to the good 'ole days of waiting in the waiting room." This kid needs to stop spending so much time with grown-ups in so many ways! 

It was exactly two months ago that this crisis began. At the time, we felt like we were on the ledge, with a child in serious danger and at risk of losing his transplant again. We are grateful for his doctors who thought outside the box to get Ezra to a better place in a way that was best for him. While we know he is by no means in the clear yet, we are grateful for where he is at this moment and that we still have our happy, energetic boy keeping us on our toes. 

Days +228 to +238: A Better Week

Overall, this past week has been a better one for Ezra. Instead of dropping again at the one week post-IVIG mark, his platelets actually increased. He is now past the two week mark from the last dose of IVIG. Ezra's hemoglobin has also remained in the normal range. Despite these good numbers, several indicators of cell destruction are showing that he is still destroying his own cells. As long as these indicators show that destruction is still occurring, we are not in the clear. There was, however, a small amount of improvement with these numbers - enough that the doctor allowed another small steroid dose decrease last week. 

The most important point is that Ezra's numbers have improved (at least for now) even with decreasing the steroid dose. They have also been tinkering with the dosing of Ezra's immunosuppressant because his blood levels had been showing that it was undetectable. The last test showed that the level of the drug in his blood is at the right point, so we are hopeful that this is also helping his counts to improve. 

The take away from this past week is that the situation is improving, but we know that we can show up any day for clinic and find that the destruction has ramped up. Things remain day to day. 

Ezra has been handling the three times a week hospital visits for meds and bloodwork better than we could ever expect. He regularly asks for confirmation that "we are home more than we are at the hospital," and for now, as long as the answer is yes, he seems satisfied. The days are long and hard, but he remains happy and energetic. We sometimes feel bad for the other patients in neighboring beds because Ezra gets so excited (and loud!) while he's playing. He keeps us all busy with the creative projects that he comes up with - never ceases to amaze with what we can accomplish in a tiny hospital cubicle. Our focus as always remains on making sure he continues to develop and learn despite his restricted life - no small feat, especially with this latest turn of events.

We gladly accept a better week while we remain always on guard for the possibility that tomorrow everything may change.