Days +151 to +157: Two Steps Forward, One Step Back

This week we have been able to cross some things off the worry list while adding others. First, the mysterious rash has disappeared. We stopped the Benadryl earlier in the week so that the doctors could see the rash, but it never came back. Phew. They think it was either the parainfluenza virus or an allergic reaction or his new T cells figuring things out. There are certainly a lot of new things going inside his body, which makes it hard to sort this all out. We won't worry about the rash unless it returns, and hopefully we've seen the last of it.

Second, Ezra has cleared the parainfluenza virus! He is still coughing a little, but he is much better. Big sigh of relief on this one. 

Unfortunately, Ezra's liver is back on the worry list. His liver levels jumped back up this week, and another liver function level is now also high. Previously, when this level was normal the doctor said that it was "reassuring" that it was normal. Now that it isn't normal, we're not feeling very reassured. The doctor does not think that it is GVHD of the liver based on how his numbers have been trending, but it may be an infection or other damage to the liver. They are hoping that the high liver levels are still from the parainfluenza and will go down with more time away from the virus. We will see what this week's levels show and maybe we can chip away at that worry list some more. 

Ezra kept busy this week during the snowy, freezing weather by making Minion cupcakes. Of course, Mr. No Sweet Tooth wouldn't eat them, but he had fun making them!

Days +144 to +150: The Good, the Bad, and the Ugly

It has been a heck of a week.  Let's start with the good news. Ezra's liver numbers started to go down this week. They are still way too high, but what matters is that they are decreasing. Second piece of good news, Ezra's whole blood chimerism this month is 99.8% donor. Cell line chimerism will be done this week. Next fabulous news - T cells have increased! One line by just a smidge, but another by a lot. To top it all off, his IgG level increased for the first time ever without treatment and it went up by a lot! We could barely believe this last piece of news. Can this really be happening? No denying it, his immune system, at least in part, is starting to work! 

Our happy dancing came to an abrupt halt when Ezra broke out in a rash that quickly spread all over his body. Total panic time as everyone was thinking his GVHD was back and the doctor was already talking about putting him back on high-dose steroids. By the time we got to clinic the next day, the rash was gone. Ezra is on what should have been his last week of steroids and the dose is very small, so the doctor decided that we would use topical steroids and see what happens. The rash then returned this weekend, but it clearly looked like hives. We gave Benadryl and it quickly went away. Based on pictures of the rash and the fact that it is responding to Benadryl, the doctor thinks that it is either from the parainfluenza virus, another virus or an allergic reaction, and not GVHD. We are giving him Benadryl around the clock for now and so far it is controlling the rash. The question is now what is causing the rash? No doubt this week will be spent figuring this out. We are just praying it does not end up being GVHD because then all this progress will be for nothing and we will be nowhere again. 

Ezra is still coughing, but it is not terrible. The doctor is concerned with the lingering congestion because it can turn into a bacterial infection. He had another chest x-ray and all was clear. Hopefully, his new baby immune system will figure out how to kick the virus soon. 

This coming week will no doubt be crazy as we try to figure out this rash. We are anxious for this latest challenge to resolve (and for some kind of assurance that this is not GVHD) so we can get back to celebrating the good news. 

P.S. If you're wondering what Ezra is doing in this picture, he was filming a "news show" with one of his amazing teachers! The top story was the construction of the new Tappan Zee Bridge :)

Days +139 to +143: Wait and See and Hope

After an insanely long day at clinic, we don't have an answer as to what is going on with Ezra's liver, which is maybe a good thing and maybe a bad thing. The ultrasound was normal, except for a slight increase in the size of Ezra's liver since his last test about nine months ago. The doctors are not too concerned about this increase in size and do not think this is what is causing the increase in his liver enzymes. His liver enzymes increased again on Friday, but not as much as they have been increasing over the past week. The hope is that the increase is from the parainfluenza virus and not something else. If the levels go above a certain number, then he will need a liver biopsy, a surgery we really do not want to put him through. For now, we will just wait and see what happens with the levels over the next week.

Ezra's cough had worsened over the past few days. The doctor detected some congestion in Ezra's lungs on Friday. He is now on an inhaler in hopes that it will relax his airways. He was a little better today. After fighting one battle against pneumonia, the mention of lung congestion is frightening. We are hoping he turns the corner very soon.

We are truly blessed with such a resilient, brave boy. Ezra stayed as still as could be during his ultrasound and asked the technician and radiologist 20 questions, while listening to every word the radiologist said (each time the radiologist said "duct," Ezra said "Did she say duck? Is there a duck in my belly?"). We were concerned about Ezra's reaction to using the mask and tube contraption for the inhaler, but when he saw it, he exclaimed "hey, that looks like my static electricity tube!" The respiratory specialist looked at him and said "how did you know that this is an anti-static tube?" Yup, we're fairly certain that he is channeling Bill Nye. Suffice to say, the inhaler is a piece of cake for him. Ezra behaved better than we did during the way-too-long day, with the help of his amazing friend Jessica from child life and these two characters!

Here's to hoping this week will be a better one.

Days +136 to +138: Update

Thank you to all for your concern about Ezra. We really appreciate all the messages of support - they keep us going. After spending yesterday at the hospital with Ezra getting a full workup, it ends up that he has parainfluenza virus. This is a common virus that causes respiratory illness and cold-like symptoms in people with normal immune systems. In immune deficient patients, it can cause pneumonia, a very bad thing. Ezra's chest x-ray yesterday was thankfully clear and it better stay that way. 

His cough is worse, but not terrible. Ezra actually had parainfluenza virus after his first transplant and managed to recover well from it. Our only choice is to hope and believe that the same will happen this time. There is nothing that can be given to help him fight off the virus. We just have to remain vigilant for any signs of worsening symptoms.

We suspect that he picked up the virus from the hospital because he has not been anywhere else. No matter how many precautions we take, it seems these viruses still find their way to him. The hospital is the worst place for him and yet we have no choice but to bring him there twice a week until his cells are stronger.

Ezra's liver numbers have unfortunately increased. An ultrasound is scheduled for Friday. The doctor thinks that the elevated levels may be from the virus, but we will see what happens on Friday and go from there. We are praying there is nothing more sinister lurking inside.

On a positive note, Ezra never ceases to amaze us. He did so well with all the poking and prodding and manages to find the fun in everything. Whether it's an impromptu puppet show behind the lead vest in the x-ray room or making a game of jumping from square to square in the hallway, Ezra always makes the best out of crappy situations. He also lectured us last night about the Big Bang Theory, atoms and why Uranus rotates differently from other planets. It seems the more isolated he is, the more he wants to know about the universe beyond. 

Thank you for all the prayers and well wishes. Ezra continues to need them and we will continue to keep you updated.

Days +122 to +135: Dealing with the Costs and the Benefits

For the past two weeks, we have been dealing with some of the downsides of Ezra's post-transplant treatment. First, last week his hemoglobin dropped significantly in just three days. It ends up that he now has iron deficiency anemia. The doctors think that the tremendous amount of blood tests that are done twice a week combined with the two months post-transplant when he wasn't eating much have left his iron stores depleted. The plan is to cut back on the bloodwork a bit for now and to try to up his dietary intake of iron. If this plan doesn't work, he will receive iron by IV to boost his stores.

Then on Friday, his liver enzyme levels skyrocketed. They have been elevated since he has been on the steroids, but never this high. If the levels do not come down by Tuesday, then he will have a liver ultrasound at the end of the week. Hyper IgM patients are highly susceptible to liver disease. This combined with two transplants puts Ezra at high risk for liver problems, so this latest issue makes us shudder. It could be any number of things or it could be nothing. We are praying the results are just an anomaly and all will be back to "normal" this week. 

Ezra's white blood cell count and his lymphocyte counts (which include T cells) also dropped this week. They are at the lowest level they have been since he was discharged. The doctors are not too concerned yet. They think that it may, once again, have to do with the steroids, even though he is now at a low steroid dose, and his counts should be increasing instead of decreasing. All we can do for now is wait and hope things turn around soon. It is so hard watching his counts going in the wrong direction. 

On top of all this, Ezra has developed a mild cough. We're hoping it's just the dryness from the heat in this freezing weather, but everyone is watching this cough very closely. Much too much going on right now. Although, if you ask Ezra how he's feeling, he says "never been better." Hoping he knows something we don't.

The one positive that we are holding onto tightly is that he still has not required his IVIG treatment. His IgG level continues to fall, but slowly. He has now passed the 9 week mark since his last IVIG. This is our one sign that something in that little immune system is working. Right now, we will take any sign of a functioning immune system.

We want so badly to feel like Ezra is safe. Just as we thought things were starting to stabilize, these latest issues are a reminder that this journey is very long, and that there are more bumps on the road to good health for Ezra to overcome. 

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!

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!