Days +228 to +238: A Better Week

Overall, this past week has been a better one for Ezra. Instead of dropping again at the one week post-IVIG mark, his platelets actually increased. He is now past the two week mark from the last dose of IVIG. Ezra's hemoglobin has also remained in the normal range. Despite these good numbers, several indicators of cell destruction are showing that he is still destroying his own cells. As long as these indicators show that destruction is still occurring, we are not in the clear. There was, however, a small amount of improvement with these numbers - enough that the doctor allowed another small steroid dose decrease last week. 

The most important point is that Ezra's numbers have improved (at least for now) even with decreasing the steroid dose. They have also been tinkering with the dosing of Ezra's immunosuppressant because his blood levels had been showing that it was undetectable. The last test showed that the level of the drug in his blood is at the right point, so we are hopeful that this is also helping his counts to improve. 

The take away from this past week is that the situation is improving, but we know that we can show up any day for clinic and find that the destruction has ramped up. Things remain day to day. 

Ezra has been handling the three times a week hospital visits for meds and bloodwork better than we could ever expect. He regularly asks for confirmation that "we are home more than we are at the hospital," and for now, as long as the answer is yes, he seems satisfied. The days are long and hard, but he remains happy and energetic. We sometimes feel bad for the other patients in neighboring beds because Ezra gets so excited (and loud!) while he's playing. He keeps us all busy with the creative projects that he comes up with - never ceases to amaze with what we can accomplish in a tiny hospital cubicle. Our focus as always remains on making sure he continues to develop and learn despite his restricted life - no small feat, especially with this latest turn of events.

We gladly accept a better week while we remain always on guard for the possibility that tomorrow everything may change.

Days +218 to +227: Birthday Do-Over and The Latest

Last Sunday, we had the joy of holding a "do over" party to celebrate Ezra's 5th birthday. We had a small grown ups only party with a visit from Mad Science, as requested by the birthday boy. Ezra was bursting with excitement waiting for his party to begin, making all of us smile at his happiness. He had a blast with the experiments and projects, and especially the insane model rocket launches from our backyard (still waiting for a visit from the cops!). Ezra was lucky to have an amazing moon cake made with love once again by Cake & Co. in Teaneck, NJ through Icing Smiles. Ezra was surrounded by love as we all sang Happy Birthday, all of us wishing that this will be the year Ezra is finally granted good health (except for the birthday boy who was probably wishing to launch more rockets!). 

For a few precious hours, we got to forget our worries. Everything came crashing back to reality on Monday when Ezra's platelets dropped again. He is responding to the high dose IVIG, but the steroids do not seem to be helping the platelets. This is ok, as long as he continues to respond to the IVIG. The hope is that he will be able to go longer between doses of IVIG. For the past six weeks, he has been able to last only one week between doses. Tomorrow is the one week mark again, so we will see if his platelets hold and he is able to go longer. 

Ezra's hemoglobin has been good, so we have been able to do another small decrease in the steroid dose. The goal is to get him as low as possible on the steroids and let the immunosuppressant take over. This process is going to take months and is only possible if his counts remain stable. Now that we are out of emergency mode, we are focused on the reality of the damage that the high dose steroids are doing to his body. The list of side effects is long and frightening, especially the increased risk of infection and particularly invasive fungal infections. For the side effects that are possible to mitigate - bone deterioration and muscle weakness - we are determined to do what we can to counteract the steroids with increased PT time and making sure Ezra gets plenty of exercise. For the other side effects, all we can do is hope that he can get to a lower dose of steroids as fast as possible. 

On to another week of challenges, unknowns, and rocket launches. 

Days +210 to +217: Improving, But Not Out of the Woods Yet

It has been an exhausting first week home. On the good news front, Ezra's hemoglobin has improved nicely. As of yesterday, it is actually higher than it has been since December when he was first diagnosed with iron deficiency anemia. The level is still below normal, but is much better than it was last week. Ezra's energy has reflected this increase, which is great, especially considering the challenges he continues to endure. 

Ezra's platelets, on the other hand, have been giving us problems this week. On Monday, his platelet count had dropped significantly, so he received another round of high dose IVIG. Thankfully, his counts yesterday showed that he responded well to the treatment. Given the drop in his platelets, it is clear that he is still destroying his own platelets (and likely red blood cells too) and the autoantibodies are still wrecking havoc, albeit with less steam than before. 

Based on the positive counts yesterday, Ezra's doctors decided to attempt a very slight decrease in his steroid dose. We are hoping that he does fine with this decrease and that his counts  do not drop because that will mean an increase in steroids - a place we do not want to go. 

Ezra has been a real trooper so far, dealing with excruciatingly long clinic days that turn into clinic evenings three times a week. He is doing fairly well with the mood swings from the steroids, although he is very bloated and it gets worse every day. 

Despite the difficult clinic schedule, we are very grateful to be home. Ezra was thrilled to be reunited with his beloved teachers this week. We are trying to provide at least some normalcy to his days at home, which of course includes model rocket launches! 

We have big plans for his 5th birthday re-do party this Sunday. We decided not to wait for things to settle down, as they may not settle down for a long time. We've learned time and time again to act in the present and to not count on the future. So no wasting time - party hats are on and we're ready to celebrate!

Days +207 to +209: We're Home!

We're home, thank goodness! The doctors decided that Ezra is stable enough to continue his care outpatient and discharged him today. His hemoglobin is slightly increased and platelets continue to bounce around in response to high dose IVIG. He will need to go to clinic three times a week for labs and IV meds. The plan is to continue with the high dose steroids, the immunosuppressant and high dose IVIG for now. The hope is that we will start to see improving numbers and then we will be able to taper the steroids. We are all very anxious to get him off the steroids before they start causing serious damage. 

We also found out that Ezra has antibodies against his own neutrophils as well. This test has been pending for three weeks, since the day he was admitted. Thankfully, his neutrophil numbers have been fine, so for now at least, it seems like that issue is likely resolved. The doctor said there isn't even a name for someone who has autoantibodies against all three cell lines - red blood cells, platelets and neutrophils - so she's going to call it "Ezra Syndrome." Great...Ezra proves once again that he is a very unique child. 

The disbelief over what has happened over the past three weeks is hitting us hard now that emergency mode is over. We were so close to some sense of safety and normalcy and now the rug has been pulled out from under us. Ezra is now about as immune compromised as he was on Day Zero post-transplant. He is on 5 times more medications than he was 3 weeks ago. While we are grateful that the doctors were able to get him to a safer place quickly while avoiding the drug that destroys B cells, Ezra has a very long road to travel to get his cells behaving again. 

Ezra is his usual amazing self. He is obviously thrilled to be home. The other night, he turned to us and said "I'm going to do my best so I can go home sooner." Our brave little man. We wish this was all in his control. The reality is that none of this is anyone's control. For now, we take comfort in watching him run around the house, laughing and playing, and hope for better days ahead. 

Days +203 to +206: Plan C

Plan C has now begun. After more research and calls, Ezra's doctors have decided to try putting Ezra on an immunosuppressant instead of the drug they were originally going to try. Ezra was on this immunosuppressant after his first transplant and it is considered to be more mild in terms of side effects. The idea is that it will quiet down the faulty antibodies from attacking his blood and platelets. If it works, then the plan is to start tapering the steroids. The downside to the immunosuppressant is that it suppresses T cell function and overall immune function and he will be more immune compromised. The steroids, however, are more toxic than the immunosuppressant, so the goal is to get him off the steroids as soon as possible, if possible. 

While the steroids have stabilized his counts, there still has been no overall improvement. The immunosuppressant may take 2 weeks to have full effect, if it's going to work. So it's more wait and see. If his counts drop again, then it may be onto Plan D. 

Ezra has been doing better now that he is off the mega dose steroids. For the last couple of days, his leg pain has been happening only during the nights, which makes for sleepless nights and tired days, but it's better than pain all day long. We've been trying our best to keep him busy while in isolation with the help of all his hospital friends, and the thoughtful presents that have started arriving from special friends, family and strangers. Today's entertainment was the window washers repelling down the building across the alley. We are so grateful for your support and the well wishes and prayers - we continue to need them. 

Days +201 to +202: Feeling Better, But Counts Not Improving

The past two days have been difficult. The doctors were concerned that Ezra could have sepsis, an infection of the blood that can be very dangerous. High dose steroids can mask a fever, so the doctors are being super cautious. Because Ezra was not acting like himself, they decided to put him on antibiotics in case of infection. Thankfully, he seems to have turned the corner last night when he busted out dancing to "Happy" (http://youtu.be/pdtUraBwDO0). Today was a better day. He will remain on antibiotics and will be watched closely for now, but the fact is that the treatment and drugs he is on for the autoantibodies can cause many issues, so it is not clear what has been causing him to feel crummy.  We are hoping it was the mega dose steroids and that he will continue to feel better and more like himself each day. 

Ezra's hemoglobin and platelets have been stable, but not really improving. His doctors have been researching and continuing to evaluate options that will allow him to quiet this faulty antibody response while avoiding the drug that will kill his B cells and allowing him to get off the steroids. Keeping him on high dose steroids for an extended period of time has very serious consequences, including immune suppression. The longer he stays on them, the more his risk of significant complications increases. A couple of options are in the works. The plan is to see how the next few days go and then move to the next step, whatever we all decide it will be. 

We still can't believe this has happened. We were so close to the cure that we dared to think about the fun things we would start to do with Ezra when that day came. And now it has been all yanked away and he is worse off than he was before transplant. We believe that at some point these issues will resolve, but when and at what cost is unknown. 

Days +198 to +200: Stable, but Suffering

The treatment so far has managed to stabilize Ezra's counts. Hemoglobin and platelets are still low and not improving, but have remained about the same for almost a week now. He received another round of high dose IVIG yesterday and today. However, Ezra is not handling the steroids well.  The steroids have made him crazy and irrational at times. His leg pain has intensified both from his marrow working hard to replace the cells being destroyed and from the steroids. And he is completely exhausted, sleeping for most of the day. The doctors got concerned today over how he's doing, and have decided to drop his steroid dose down to where we started instead of weaning more slowly. They also drew blood cultures to make sure he doesn't have an infection, and started him on antibiotics to be cautious. 

The plan to avoid the drug that kills off his B cells may need to be abandoned. If his counts deteriorate after we drop the steroids then we will have no choice, but to use the drug that will kill off his B cells. And if he has an infection - well, that will be the next nightmare. 

Once again, we have lost our sweet boy to the suffering caused by his treatments. We tried to make his birthday special, but he really could not enjoy it. We feel like we are going through transplant for a third time, watching him suffer and feeling helpless. All we can do is hope that relief will come as we lower the steroids. It can't come soon enough.