Sunday, June 29, 2014

Days +306 to +312: Losing Sight of the Light

What we feared might happen has happened. Ezra's counts dropped this week. In particular, his hemoglobin is lower than it has been since the end of March when we were first discharged and one measure of red blood cell destruction is higher than it's been in almost two months. His platelets also dropped, although they are still at a very good, safe number. Although the timing of this drop in counts coincides with the outbreak of the rhinovirus, it is impossible to know if the drop is being caused solely by the virus, or by the steroid decrease, or both. Whatever the answer is, the drop is significant and the doctors feel something has to be done. 

Here is the plan. We went to a higher steroid dose Friday and are staying at that dose until at least tomorrow. We are going back to Sloan tomorrow for a counts check and, unfortunately, high dose IVIG. The reasons for the high dose IVIG are 1) his IgG dropped to just above the threshold for giving it (which may or may not be a consequence of fighting off the infection); 2) if the platelet decrease is from auto-antibodies, then the high dose IVIG will help stop any further destruction; and 3) it will hopefully completely knock the rhinovirus out of his system and we can take it out of the picture. Thankfully, he has been doing a good job of getting rid of the rhinovirus on his own. He still has a bit of a stuffy nose, but the virus is on its way out. The high dose IVIG should help ensure that it's gone. 

The next step with the steroids will be determined by his counts tomorrow. If they bounce back, then we should be able to drop down to a lower dose, but the doctor is already talking about keeping him at a higher dose than what he was taking for a longer period of time. How long is unclear. Our first question was "then how is he ever going to get T cells?" Well, there was no easy answer to that question. 

We know that it is too soon to feel defeated. If his counts bounce back quickly and stabilize, then the doctors may draw the conclusion that it was the virus causing the counts suppression and he may be able to go back down on steroids quickly. Whatever the outcome, it's hard not to feel heartbroken over another setback. It feels like it never ends - every time we start to feel optimistic, we fall backward. Once again, we thought we were seeing the glimmer of light at the end of the tunnel. And once again, we've lost sight of that light. 

Despite the craziness going on inside his body, Ezra feels well and isn't too bothered by the sniffles. His teachers and therapists are on break for the next week, and then he will continue to receive services during the summer. Ezra has missed so much time that it's great that we don't have to stop again for the summer. His days would be very empty without his teachers to fill them with learning, challenges, and fun. We are trying to keep him busy during break - like spending the afternoon watching PSE&G work on a gas pipe down the road. Thank you to the PSE&G workers for answering Ezra's many questions and for kindly offering for him to climb into the machinery (which he thankfully declined)!

For now, we tuck away our dreams of school and play dates for some other time. We will continue to do what we have learned to do best - find the light in our sanitized world of Purell and Clorox wipes and keep the rest of life at bay for a future day we still hope will come.

Sunday, June 22, 2014

Days +300 to +305: Frustration

As I was posting our update last week, I thought "I hope I'm not 'jinxing' us by writing a positive update." Then I thought, "no, that's silly, we have to rejoice in whatever positive news we can get." Yeah, well, as has been the story of our lives over the past few years, we had another step back this week. 

First, Ezra developed cold symptoms and it ends up that he has rhinovirus - the common cold. Of the viruses that can cause cold symptoms, rhinovirus is the least worrisome, but it can be difficult to clear for immune-compromised patients. Ezra had a really miserable (and nerve racking for us) 24 hours of feeling rotten. He is doing better now, although still plenty congested. 

Complicating everything, Ezra's T cells have dropped by 50%. Ezra has about the same number of T cells that he had in December when they first appeared. We've lost 7 months of progress. Without enough T cells, Ezra remains at high risk for serious infections, and the longer he goes without T cells, the greater the risk. And now he has a cold. If he doesn't fight off the cold quickly, he will get a round of IVIG in hopes that will help him fight off the virus. His IgG has basically remained stable, but without  enough T cells, his doctor is not sure he will be able to clear it quickly, and we don't want it to turn into a sinus infection or anything more problematic. 

Given the T cell situation, his doctor is going faster on the steroid wean this week. Viruses can cause counts to drop, so they are going to keep a close eye on his counts to make sure that between the steroid decrease and the cold, things aren't getting out of control. If he does ok with the steroid wean, then we may move toward weaning the immunosuppressant, while keeping him on a low dose of steroids. All of this is in hopes of getting up his T cells while not letting the autoantibodies make trouble again. A delicate balance. 

We are frustrated, to say the least. It feels like we can never stay in a forward direction for long. As always, please keep our little man in your prayers and thoughts. 

Monday, June 16, 2014

Days +292 to +299: Thriving on the Outside, Working on the Inside

Ezra thankfully had another week of stable counts. His IgG level went up a little, which is great. Too soon to say if his body is getting back into normal IgG production, but it is definitely a good sign that something is cooking. Ezra also got to stop taking two oral medications over the past two weeks (Whoohoo!), and the very slow steroid wean continues. We are waiting for results on the latest check of his T cells. We are praying for some small improvement or at least stability, and no more falling numbers. Ezra really needs more T cells for many reasons. 

In reflecting on the last two months since we were discharged from the last hospitalization, Ezra has been thriving, at least outwardly, for the first time in a very long time. Physically, he seems to get stronger everyday. He has been asking to walk places, when he would only go in the stroller before. This past weekend he rode his bike to the park, which was a huge deal because his leg strength has been poor since transplant and got worse with the steroids. Ezra has also been asserting his independence more and acting more like a typical 5 year old, all of which we are happy to see. His concentration with his teachers has improved now that he feels better. We are very excited that Ezra has been learning how to read! Now we just need the inside to catch up with the outside. 

Today brought a momentous milestone - match number 150 from Ezra's Gift of Life Donor Circle (! We feel very blessed to have been able to help so many others. Thank you and congratulations to all who have helped us with our goal of building the registry!

Monday, June 9, 2014

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 ( 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.

Monday, June 2, 2014

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.