Monday, July 28, 2014

Days +334 to +341: Got To Keep It Up

Just a quick update to let everyone know that Ezra's counts were once again good this week. Ezra's doctor called his counts "beautiful." His hemoglobin was up and all the other numbers were normal. The only issue, aside from the overarching issue of Ezra needing to grow a new immune system, is his liver function. His liver levels have unfortunately increased again. The doctor believes that it is from the increase in steroids. For now, the plan is to wait and see if the levels decrease as we go down on the steroids. There always has to be something of concern on this crazy journey. 

Ezra also had an eye exam to check his eyes for side effects from his two transplants. They are also going to be watching his eyes closely for cataracts, which can be a side effect of steroids. The doctor said it's too soon to see cataracts from the steroids, but for now, Ezra's eyes got an "A++."

We really, really need a period of calm and good counts to get Ezra off the steroids and immunosuppressant. We went down again by a small amount on the steroids, but it's going to take some time before he's even back to where he was on the wean before this latest flare up. Getting off these drugs is the key to allowing Ezra's donor cells to build him the new immune system he so desperately needs. We know those donor cells are there, waiting to be allowed to grow. We can't wait for them to break Ezra free of his life of isolation, and pray that it is only a matter of time. 

Sunday, July 20, 2014

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. 

Sunday, July 13, 2014

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. :)

Sunday, July 6, 2014

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.