Sunday, December 22, 2013

Days +111 to +121: How Far We've Come

One year ago, we announced on this blog that Ezra's transplant had officially failed. We had just received the news that he had only 1% donor cells. Faced with dismal options, we wrote "It may be that the right decision is to not do anything. To let him be. To let him live. And to hope that someday soon that long sought after match appears." A few months later our hopes and prayers were answered when Ezra's donor appeared on the registry. After much anguish, we decided that it was not better to let him be and that a chance at a cure was better than life with a sword hanging over his head. And now here we are one year later. The verdict on this transplant is still out, but things are moving in the right direction and so far we are in a much better place than last year at this time. There is much to be grateful for.

The latest round of testing on his T cells is back. There are multiple types of T cells. The type of T cell that he had a small amount of last month is down slightly, but basically the same, which is likely due to the steroids. The good news is that a small amount of another type of T cell has appeared! One step back, one step forward. 

There's been another good sign: Ezra has been able to go longer in between infusions of his immunoglobulin replacement therapy (IVIG). This is the treatment that Ezra has received every four weeks since he was diagnosed. It is what has kept him alive for the past four years. Ezra does not make an immunoglobulin called IgG, or at least he didn't before this second transplant. This was the level that came back drastically low when Ezra was sick with pneumonia at four months old and our first indication that there was something terribly wrong with his immune system. Each week, his levels of IgG are checked to see if he needs the treatment. The level has been dropping, but more slowly than his usual rate. He is now more than 7 weeks without IVIG, the longest he has gone without it. He may need it soon, but the doctors tell us that the fact that he is holding longer means that maybe, just maybe his body is starting to make IgG and his new immune system may be starting to function.

Ezra remains on a low dose of steroids. The wean is going very slowly. Thankfully, there is still no sign of the rash.

Ezra was loving the snowfalls of last week. He had been waiting for the first good snow basically since we got home from the hospital. He ran around yelling "snow angel!" and dropping backward into the snow. Seeing the snowflakes glittering on his newly re-grown eyelashes was a breathtaking sight. A little snow angel indeed.

Happy Holidays!

Wednesday, December 11, 2013

Days +103 to +110: Good News!

Today brought fantastic news: Ezra's T cells are 99% donor!!! And all other cell lines are 100%! We are beyond relieved and so thrilled! The news comes after we had a stressful discussion yesterday with Ezra's doctor where he was preparing us for the possibility that his T cells will be mixed chimerism. With T cell depletion transplants, it is apparently not unusual for the T cells to grow back mixed initially, and then the donor cells take over as time passes. This would have been a difficult scenario for us to handle given what happened with Ezra's first transplant. We knew that mixed chimerism was a possibility from the beginning and that his cruddy T cells are not so easy to knock out, but we were praying that we would never have to see those defective T cells again. Based on these results, it looks like Ezra's CD40 Ligand deficient T cells have hit the road. Adios T cells - you will not be missed!

We are hoping that Ezra's incredible-needle-in-a-haystack-miracle donor feels our joy and gratitude wherever she may be. This is all because of her. We welcome all of her cells and love them and her fiercely.

Ezra, by the way, does not know about any of this. We learned our lesson the hard way with the first failed transplant that we should not tell him much about his new cells until there is more certainty. Until the GVHD stays away and until he has the necessary amount of functioning T cells, we will not give him details. All we tell him is that his counts are good, he is doing great, and that we are so very proud of him. We patiently look forward to the day when we can tell him he has a new, strong immune system and he can go conquer the world. For now, we keep our victory dances for when he is sleeping.

The next steroid wean started last night. He will be on the steroids for about another month at the current rate. The rash has not returned. With these lower doses of steroids, it is more likely that the GVHD may flare up, so we are watching his skin very closely. The amount of T cells will likely be checked again at the end of the week. We are not expecting much T cell growth, if any, due to the steroids.

Thank you to everyone for sharing Ezra's YouTube rocket video! He has over 2,200 views and he is thrilled! He loved hearing all of the awesome comments! Thank you for giving him something to look forward to and to enjoy!

Tuesday, December 3, 2013

Days +91 to +102: Adventures in Steroid Land

Life with Ezra on steroids has been interesting. First, the good news is that the rash has not re-appeared. We have been able to reduce the steroid dose twice, and will be starting a third reduction. The rate of the wean starts to slow down now, so Ezra will be on steroids for at least another month, depending on how things go. It is frustrating because the steroids suppress T cell growth, but we will do whatever gives him the best chance of overcoming the GVHD.

Despite the reductions in steroid dose, Ezra is still dealing with the side effects. The steroids have caused his appetite to increase, which right now is a good thing because he is still regaining the weight he lost during transplant. The problem is that his appetite is mainly for chips. Steroids effect the salt-water balance in the body and can cause bloating and an increase in blood pressure (and blood sugar and many other bad things), so the doctors want us to watch his sodium intake. We have now gone from desperately wanting Ezra to eat and encouraging him to eat whatever he wants as long as he eats, to gently pulling back on the one food he wants more than anything else. Poor little dude must think we are crazy. He also has been experiencing the drastic highs and lows from the steroids. He goes from hyperactive to completely exhausted. Ezra is not a hyper child, so the last two weeks have been interesting. We are hoping that as we go down on the steroid dose, the side effects will subside.

Ezra has reached the Day 100 milestone. Day 100 is often touted as a marker of survival. Studies show that patients reaching day 100 post-transplant are more likely to survive in the long run. In Ezra's case with the type of transplant he had, Day 100 isn't all that significant. The more important milestone will be when he has a strong number of functioning T cells and is able to be free of the steroids without GVHD re-appearing. That will be cause to celebrate.

Ezra has been basking in the light of Hanukkah. Candles, songs, a gigantic menorah, and presents - what's not to love about Hanukkah when you're four years old? This year, the unspeakable thoughts that have tainted every joyous moment since Ezra was diagnosed seem to be a bit more subdued. Ezra's health is still in a very precarious state with so much of the path ahead unknown, but the progress he has made so far is huge. So, maybe day 100 is indeed a moment for feeling a little triumphant in the long fight for a cure. 

Happy Hanukkah! Love, Robin, Evan and Ezra

P. S. Ezra wanted to make a YouTube video of one of his model rocket launches "for other little kids." He likes asking how many views his video is getting, so if you have a few minutes, check out his video at