Days +196 to + 197: Plan B

Ezra's counts have continued to drop, so the team here decided it was time to try another course of action. The first step is to put Ezra on a mega-dose of steroids for three days. He is now on a dose of steroids that is ten times higher than the high dose he was initially put on. This started last night. The hope is that we will see improving counts by Day 3 of the mega high dose of steroids. 

If there is no improvement, then the doctors are proposing the use of a drug that has never been used before for this specific situation. They are trying to "think outside the box" to avoid using the drug that kills off B cells. This drug has been used for similar situations, but not for exactly what Ezra is experiencing. The drug alters the function of the immune system, but the effects do not appear to be permanent. The drug will make Ezra more immune compromised while he is on it and more susceptible to serious infection. We will be in uncharted territory if he needs this drug. We are weighing the option while hoping that we do not get to that point. 

We are obviously not leaving the hospital anytime soon. Ezra's 5th birthday will be celebrated tomorrow in the hospital. We had been planning an adults-only birthday party for him and he had requested a Mad Science show at the party. We plan on re-scheduling for whenever he is home and stable. He doesn't know yet. Hopefully, we can make his birthday special enough that he won't be too disappointed. 

We had been so looking forward to Ezra's 5th birthday as a birthday that would finally mark the beginning of a lifetime of good health. Each of Ezra's birthdays has been clouded by the uncertainty of illness. The past two birthdays we spent knowing transplant was ahead of us. Now, once again, we celebrate his birthday under a cloud of fear and uncertainty. 

Thank you all for the prayers and well wishes. We appreciate them all. 

Days +193 to +195: On the Ledge

After two half-size red blood cell transfusions, Ezra's hemoglobin has increased to the transfuse level, which is an improvement from where he was. He so far has handled the transfusions well in terms of adverse reactions. However, the hemoglobin level has not increased beyond the initial bump from the transfusion, which may indicate that he is still destroying red blood cells. His platelets had increased to a better level, but have been slowly decreasing, which is not a good sign. It is not clear whether the treatment is working. For now, the plan is to sit tight and watch his counts. The doctors are trying to get him out before his birthday on Saturday, but the problem is that we may go home only to be re-admitted on Monday, which will be very hard on all of us. We are not sure how we are going to proceed at this point. And all will depend on his counts in the next two days. 

The biggest concern is if the current treatment does not work, then the alternative is to administer a drug that kills off B cells. The problem is that the B cells often never return. No B cells means no ability to make antibodies. No ability to make antibodies means the transplant will be largely negated. He may still have healthy T cells (assuming we get to that point), but we will be destroying half of his new immune system. This possibility is absolutely devastating. 

One of his doctors told us not to jump off the ledge yet. Well, we feel pretty close to jumping if things don't improve soon. 

Ezra, thankfully has been feeling better. The transfusions greatly helped his energy levels. He is still having leg pain, especially during the night, which makes sleeping difficult. Ezra's child life friend Jessica has been busy coming up with fun ways to keep Ezra occupied, like a homemade piñata. Ezra had a blast bashing at the piñata and got more exercise than he's had in a long time (notice the lefty swing!). 

Please pray that his counts start to improve and that we don't have to turn to the awful alternative. 

Days +191 to +192: Update

Here is the latest on Ezra. His hemoglobin was extremely low early Saturday. They did not want to transfuse because if he is destroying red blood cells, he could have kidney problems if he destroys the cells coming from the transfusion. They held off for a day, but his hemoglobin did not improve, so they were forced to transfuse him today. They gave him half of the normal size unit and ran it very slowly. He is being watched closely for signs of kidney problems. He may need another transfusion tomorrow, depending on his counts. It will be a very scary two days while we wait for signs of kidney problems and to see if his hemoglobin holds. 

Ezra's platelets have started to slowly increase. They are still very low, but better. This is one indication that the treatments are starting to work. His neutrophils and white blood cell count have also improved. 

Ezra feels rotten with all that is going on inside that little body. He is really wiped out. Hopefully, his body ceases fire against the red blood cells and his counts will improve soon. And his leg pain is back with a vengeance as his marrow is chugging away to replenish all that has been lost. Déjà vu. At least his back is feeling better from the bone marrow biopsy. 

The doctors think that these auto-antibodies are the result of his new immune system not yet working properly. He has a lot of B cells and not enough T cells. They believe the T cells and B cells are not yet communicating properly and the B cells are over-producing antibodies. The awful irony of it all. 

For now, it is day by day at Chez Sloan as we watch and wait for his counts to improve and for Ezra to feel better. We will continue to update everyone as we navigate this very scary bump in the road.

Days +186 to +190: Things Fall Apart

Ezra was admitted to the hospital yesterday. His platelets dropped severely. His neutrophils and red blood cells also went down. He has developed antibodies against his own platelets. It is likely that he also has an antibody against red blood cells and possibly against his neutrophils. These tests are pending. This can happen after transplant. Sometimes it is caused by a virus and sometimes it is the new immune system settling in and going haywire. He has been put on high dose steroids and has received two platelets transfusions. He destroyed the platelets that were transfused. They did a bone marrow aspirate and biopsy today. The marrow thankfully looks healthy. The destruction is happening in the blood. There are many tests still pending and not many answers right now. 

In addition to the high dose steroids, Ezra is receiving high dose IVIG. The hope is that the combination of the steroids and high dose IVIG will stop the destruction and "reset" his body. It is unclear how long all this will take and how long Ezra will remain inpatient. 

Ezra was taking this all in stride, but was distraught over the procedure. He is now in pain and is receiving pain medication. He of course wants to go home.  

Everything is very unclear at this time. We will update again when we know more. Please pray for Ezra and for this nightmare to resolve quickly. Ezra Aviel Ben Hertzelia Shandel.

Days +179 to +185: Six Months and the Journey Continues

Today marks six months post Ezra's second transplant. We feel very grateful that Ezra is overall doing well, the donor cells have settled into his body, and they are starting to work. We are hopeful that with time, Ezra will have a new, healthy immune system and we will be able to finally say that he is cured. And yet, we were reminded this week once again that this journey is far from over. 

The issue of the week is that Ezra's platelets count has drastically dropped. The doctors are concerned that he is destroying his own platelets, which sometimes happens post-transplant. They drew a frightening amount of blood (just when his hemoglobin had finally increased a little) to test for antibodies against his platelets. The results can take a week and the test only detects the antibodies 50% of the time. If his platelets drop past a certain point, they will start treatment, regardless of the test results. We will see what his numbers show this week and are once again watching closely for bruising and bleeding - signs his platelets have dropped too low. 

This latest development has left us asking "is nothing safe?" Ezra's platelets have been rock-solid since right after transplant. It was the one thing we weren't worried about. It seems the only thing we can rely on is Ezra's amazing ability to take all the poking and prodding in stride. According to his transplant doctor, he "managed to charm" the serious radiologist and he made an elevator full of cancer patients crack up with his corny jokes. We are so proud of him. We are hoping that his bloodwork this week shows that his platelets are back up and this next crisis can be averted. 

The liver ultrasound showed no change from the last one. Liver levels this week were slightly lower, but basically the same. For now, we will continue to just watch the levels. 

On top of all this, Ezra had a difficult week. His stomach is bothering him and he is exhausted. It seems that the iron deficiency is catching up to him. We have some really fun surprises for him that we're working on with the help of some amazing friends, which we are hoping will cheer him up. And we are all very much looking forward to his upcoming fifth birthday on March 8 (how can he be turning five?). Let the partying begin - we all need it!

Days +166 to +178: Pedaling Along

We have the results back from the latest round of testing on Ezra's T cells. One T cell line went up, although not as much of a jump as last month. The other T cell line did not go up at all. We were a little disappointed in the results, but the doctor says this is absolutely normal, and he is within the expected range of T cells for this point post-transplant for a T cell depleted transplant. The rate of T cell growth is completely unpredictable. He still has a ways to go until he reaches the first T cell milestone. There is no way of knowing if it will take him 6 months or 1 month to reach. As we should have known by now, it's all about expectations - last month, we had zero expectations about his T cells because of the steroids and they jumped up. This month, we expected more of an increase and it didn't happen. So, we will continue to be optimistic, but no more expectations. 

We also received the results of the initial testing of the function of these new T cells in response to a general antigen. He has a good amount of function, but not normal yet. The doctor is happy with the function at this stage. Ezra's defective T cells prior to transplant actually responded normally to this test, so it is not quite an indicator of a cure, but it is a very good sign that his new, healthy T cells are maturing and starting to function. 

We also received the results of the latest round of cell line chimerism tests. His T cells are 99% donor and all other cell lines are 100% donor. The doctor said that she thinks we can feel like Ezra's chimerism is stable because now he has T cells coming in and he is still 99% donor. This was really fantastic to hear, although we're not quite ready to take that sigh of relief. Then again, we may never be able to take that sigh of relief!

The bad news of the week is that Ezra's liver levels have continued to rise. He is going to have another ultrasound on Friday to check for any changes. It is not at all clear what is going on. The doctors are leaning towards it being medication-related, although we also discussed the possibility of a virus. We'll see what the ultrasound and additional tests show this week and go from there. 

This picture was taken a couple of weeks ago before the crazy snowstorms. We were so excited that Ezra got back on his bike because he has not ridden in over a year. He only had enough strength to pedal a few feet, but we are hoping that with a little more time he will be racing along with Daddy!

Days +158 to +165: What Comes Next?

Let's get the not great news out of the way. Two of Ezra's liver levels have basically stayed at the same high level this week, while one level has continued to increase. The only way to know what is going on is to biopsy his liver, but no one is ready to do that yet. So the plan is to wait and watch the levels for now, and hope they decrease. 

The other issue of the week is that Ezra's hemoglobin dropped out. It's not surprising because with all the issues going on lately, they've been taking even more blood than before. We are starting with a different oral supplement. Because Ezra did not tolerate the first oral supplement, we have to add two other meds to help him deal with the iron. It is frustrating to be adding meds when we are at the point where we should be eliminating them. Such is life in post-transplant land - a vicious cycle. And so far, Ezra's stomach is not happy with the iron. 

On to better news. First, Ezra is now completely off steroids. Please, GVHD stay away. The steroids put his adrenal glands "to sleep," which means that he is not making cortisol. It is only a problem if he gets a fever or is seriously injured because his body will not be able to handle the stress. We have an "emergency" drug that we will give him in those instances. Yet another reason to fear infection. The expectation is that he will start making cortisol again within a few months. 

Second, Ezra's IgG level increased again without treatment and is now NORMAL. What can be better than the word normal? We are taking this as good sign that things are cooking in his new immune system. 

So, what has to happen next? The doctors are looking for Ezra's T cells to reach a certain initial level and for them to have basic function. At that milestone, some of the isolation restrictions will be lifted. Then the doctors will look to see if his T cells have CD40 Ligand and if it is functioning. The ultimate test - the one that will tell us if he is cured - is if he is able to make antibodies in response to vaccines. 

We don't know when Ezra's T cells will reach the first milestone, but they are checking numbers and function this week, and we will know more where things stand next week. Please send prayers that his T cells move in the right direction!

This past week, Ezra seems to have decided that he's fed up with this isolated life. For the first time, he had a meltdown over not being able to go somewhere. He has also started asking when he can stop taking his meds. It is heartbreaking, but we're glad that he's talking about it. He's a fighter and he should be angry, even though we have no good answers for him as to when things will change. 

We decided we needed some family fun and bet that between the Super Bowl and the colder weather, the Bronx Zoo would be empty on Sunday afternoon. Ezra had a great time giggling at the snuggling brown bears and riding the bug carousel (twice!). On to the next adventure.