Days +327 to +333: A Good Week

We finally have good news to report this week. First, Ezra's counts were great. His hemoglobin was normal and one of the measures of red blood cell destruction was also completely normal for the first time since he developed the antibodies. Second, the latest test of the strength of the red blood cell antibodies came back negative, showing no antibodies. Huh? Yep. This happened once before when he tested negative for the antibody and then two weeks later tested positive again. The doctor explained that the antibodies are probably there, but not at measurable levels at the moment. The flare-up that Ezra has been experiencing over the past three weeks was likely caused by a combination of the rhinovirus and the decrease in the steroids. Whatever it is, and even if the test comes back positive again two weeks from now, it is certainly good to have a period of calm and healing. 

Even more surprising - Ezra's T cells went up! They are back to where they were in May when they first dropped. The level is still far from where it was before he went on steroids at the end of February and still very far from where he needs to be, but he is headed in the right direction. Ezra's doctor was very surprised at this increase. She did not expect a T cell increase with the current steroid dose. That's Ezra for you - always doing things his own way. 

The question is - can he keep these T cells or is he going to lose them again because of the steroids? The doctor thinks he will keep them provided we are able to continue weaning the steroids. Despite all this good news, the doctor lowered the steroid dose only by the smallest of increments again this week. She is proceeding with caution for now to avoid another flare up. So it remains to be seen if Ezra will be able to maintain this T cell progress. 

By now we know all too well that we can lose this progress as quickly as we gained it. There have been way too many ups and downs on this journey for us to feel like Ezra is at all safe. Despite the number of times we've been knocked down, we are still managing to feel optimistic from these latest numbers. A little hope goes a long way. 

The other exciting news of the week is that Ezra had his first day of school in school! Ezra's teachers came up with the great idea of having Ezra do his home schooling at one of the schools that is closed for the summer so that he can get a sense of what it is like to sit in a classroom and what a school looks like. They had one of the preschool rooms thoroughly cleaned, air conditioners cleared out, and a sign hung on the door welcoming Ezra to school. The look on his face when his teachers told him that he was going to the school was indescribable. He was just so happy. 

Class started with the Pledge of Allegiance. Ezra was very taken by the smart board in the classroom. He was so focused on his school work - it was amazing to see. He will continue to go to the school once a week for the summer. We are very grateful for the creativity and determination of his teachers who arranged for this incredible opportunity. Our hope is that when the day comes for him to attend "real" school with other children that he will be as excited and happy to attend as he is with his class of one. 

Days +320 to +326: A Mixed Bag

It was another week of up and down counts. Ezra's body is still struggling to get back on track. The main issue at the moment seems to be red blood cell destruction. His platelets thankfully remained in the normal range this week. His hemoglobin, on the other hand, has been up and down. At the end of the week, his hemoglobin had improved. However, one of the measures of red blood cell destruction was higher, reflecting that his body is working hard to replace red blood cells that are being destroyed. The doctor was actually happier with this scenario than when his hemoglobin was lower because it means that his body is able to compensate for the destruction. 

The doctor decided to lower his steroid dose by the smallest increment and only on alternate days. We are really hoping that this minor decrease doesn't affect his counts and that we can get back to weaning the steroids. This weekend, we already saw a regression in Ezra's strength and endurance from the higher steroids. Steroids just stink. 

We did receive the results of Ezra's latest chimerism test and thankfully, he is still 100% donor on all cell lines. We take comfort in knowing that when the day comes when he is off the steroids and immune suppressants, Ezra will have donor cells ready to build him a new, strong immune system (we hope). 

Ezra started his summer school session this week. To match his 100% donor cells, he got 100% on his first ever spelling test (we're allowed a little bragging, right?)! Maybe his donor is a good speller. :)

Days +313 to +319: Working On It

Last week started out on an up note and ended on a down one. When we arrived at clinic last Monday, Ezra's counts had improved. Not back to where they had been, but better. He received high dose IVIG, which went fine. We then came back to clinic on Thursday and his numbers were worse. It ends up that due to a miscommunication, the doctor we had seen on Monday had lowered Ezra's steroid dose too much. So we had to go back up again to a higher dose of steroids for another three days. We are returning to Sloan tomorrow to see how his counts responded to the steroids. The goal is to get his counts back up and stable, and to then start weaning the steroids again. 

At this point, the thinking is that the rhinovirus likely triggered the antibodies to reactivate. His counts aren't terrible, but the goal is to prevent any destruction from getting out of control. The strength of the antibodies against red blood cells is still at the weakest level, which is where it has been over the last couple of months. Ezra tested negative for neutrophil antibodies this time around. He has not had an issue with his neutrophils since the day all of his counts tanked, but it is still great that he was able to get rid of those antibodies. We have not re-tested for platelet antibodies recently because the test requires a massive amount of blood, and Ezra doesn't have extra hemoglobin to spare. His platelets have been at a safe number even with the latest set back, and that's what matters most. 

So, as always, we will see what tomorrow brings. Ezra is such an unbelievable trooper about it all - the more frequent (and longer) clinic visits and the increase in the nastiest tasting of all his meds (steroids). He marched into his bed at clinic for IVIG and started exclaiming about everything he could see out the window, even though he has seen that view countless times. He danced around doing his Three Stooges act and had a blast goofing around with the clowns. The rhinovirus is gone and his energy level is great. We're thankful that whatever is going on inside has not affected how he feels. 

Ezra has been waiting to see fireworks again since last July 4th. He got his wish this week. We found a quiet spot to watch our town's fireworks and he of course loved every minute of it. Last July 4th, we had pretty much made the decision to move forward with the second transplant. We remember Ezra's excitement over his first fireworks show and thinking it will be a miracle if he is able to enjoy the fireworks again next year. While too much is still so unknown, we are thankful that today our resilient little man is exploding with so much life. 

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.

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. 

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 (www.giftoflife.org/help4ezra)! 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!

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.