Thursday, March 27, 2014

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!

Wednesday, March 19, 2014

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. 

Sunday, March 16, 2014

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. 

Wednesday, March 12, 2014

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" ( 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. 

Monday, March 10, 2014

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. 

Friday, March 7, 2014

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. 

Wednesday, March 5, 2014

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. 

Sunday, March 2, 2014

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.