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.