Last week, Ezra's platelet count had dropped again. Just as the red blood cells had come under control, of course his platelets needed to take their turn again and cause problems. The doctor was planning to see his counts this week and then give another round of high dose IVIG. We were feeling frustrated at how difficult it has been to get these auto-antibodies under control. Then we showed up to clinic on Friday and his platelets had bounced up nicely and are back in the normal range! A pleasant surprise that we will gladly accept. The doctor wanted to do another check of the platelet antibodies, which we haven't done for a while because it requires a tremendous amount of blood. It will be interesting to see what this test shows. Because there always has to be something, over the weekend, one of Ezra's big toes developed redness around the toe nail. It seems to be infected. He started antibiotics and foot soaks. We will be watching it closely. We are hoping the infection clears up quickly and doesn't cause problems with his counts, which it may do. Here we go yet again.The doctor also sent out another T cell count check on Friday (yeah, we're not sure how he has any blood left). Ezra's total lymphocyte count, which includes T cells and other types of cells, has been low, so we have no expectations of what this month's T cell count will show. Please prayers for a T cell increase!As we approach the one year anniversary of Ezra's transplant on August 23, there are a lot of intense memories for us associated with the month of August. This week marks one year from when Ezra was re-admitted to start high dose chemotherapy for the second transplant. Five years ago this past week, a 5 month old Ezra was admitted to our local hospital with labored breathing and would soon be escalated to intensive care. For the first time five years ago, we heard the name "Hyper IgM Syndrome," and life forever changed. August may always be a difficult month for us, but we have plans to change it into a month when we will also always celebrate life and hope.
Just a quick update to let everyone know that Ezra's counts were once again good this week. Ezra's doctor called his counts "beautiful." His hemoglobin was up and all the other numbers were normal. The only issue, aside from the overarching issue of Ezra needing to grow a new immune system, is his liver function. His liver levels have unfortunately increased again. The doctor believes that it is from the increase in steroids. For now, the plan is to wait and see if the levels decrease as we go down on the steroids. There always has to be something of concern on this crazy journey. Ezra also had an eye exam to check his eyes for side effects from his two transplants. They are also going to be watching his eyes closely for cataracts, which can be a side effect of steroids. The doctor said it's too soon to see cataracts from the steroids, but for now, Ezra's eyes got an "A++."We really, really need a period of calm and good counts to get Ezra off the steroids and immunosuppressant. We went down again by a small amount on the steroids, but it's going to take some time before he's even back to where he was on the wean before this latest flare up. Getting off these drugs is the key to allowing Ezra's donor cells to build him the new immune system he so desperately needs. We know those donor cells are there, waiting to be allowed to grow. We can't wait for them to break Ezra free of his life of isolation, and pray that it is only a matter of time.
We finally have good news to report this week. First, Ezra's counts were great. His hemoglobin was normal and one of the measures of red blood cell destruction was also completely normal for the first time since he developed the antibodies. Second, the latest test of the strength of the red blood cell antibodies came back negative, showing no antibodies. Huh? Yep. This happened once before when he tested negative for the antibody and then two weeks later tested positive again. The doctor explained that the antibodies are probably there, but not at measurable levels at the moment. The flare-up that Ezra has been experiencing over the past three weeks was likely caused by a combination of the rhinovirus and the decrease in the steroids. Whatever it is, and even if the test comes back positive again two weeks from now, it is certainly good to have a period of calm and healing. Even more surprising - Ezra's T cells went up! They are back to where they were in May when they first dropped. The level is still far from where it was before he went on steroids at the end of February and still very far from where he needs to be, but he is headed in the right direction. Ezra's doctor was very surprised at this increase. She did not expect a T cell increase with the current steroid dose. That's Ezra for you - always doing things his own way. The question is - can he keep these T cells or is he going to lose them again because of the steroids? The doctor thinks he will keep them provided we are able to continue weaning the steroids. Despite all this good news, the doctor lowered the steroid dose only by the smallest of increments again this week. She is proceeding with caution for now to avoid another flare up. So it remains to be seen if Ezra will be able to maintain this T cell progress. By now we know all too well that we can lose this progress as quickly as we gained it. There have been way too many ups and downs on this journey for us to feel like Ezra is at all safe. Despite the number of times we've been knocked down, we are still managing to feel optimistic from these latest numbers. A little hope goes a long way. The other exciting news of the week is that Ezra had his first day of school in school! Ezra's teachers came up with the great idea of having Ezra do his home schooling at one of the schools that is closed for the summer so that he can get a sense of what it is like to sit in a classroom and what a school looks like. They had one of the preschool rooms thoroughly cleaned, air conditioners cleared out, and a sign hung on the door welcoming Ezra to school. The look on his face when his teachers told him that he was going to the school was indescribable. He was just so happy. Class started with the Pledge of Allegiance. Ezra was very taken by the smart board in the classroom. He was so focused on his school work - it was amazing to see. He will continue to go to the school once a week for the summer. We are very grateful for the creativity and determination of his teachers who arranged for this incredible opportunity. Our hope is that when the day comes for him to attend "real" school with other children that he will be as excited and happy to attend as he is with his class of one.
It was another week of up and down counts. Ezra's body is still struggling to get back on track. The main issue at the moment seems to be red blood cell destruction. His platelets thankfully remained in the normal range this week. His hemoglobin, on the other hand, has been up and down. At the end of the week, his hemoglobin had improved. However, one of the measures of red blood cell destruction was higher, reflecting that his body is working hard to replace red blood cells that are being destroyed. The doctor was actually happier with this scenario than when his hemoglobin was lower because it means that his body is able to compensate for the destruction. The doctor decided to lower his steroid dose by the smallest increment and only on alternate days. We are really hoping that this minor decrease doesn't affect his counts and that we can get back to weaning the steroids. This weekend, we already saw a regression in Ezra's strength and endurance from the higher steroids. Steroids just stink. We did receive the results of Ezra's latest chimerism test and thankfully, he is still 100% donor on all cell lines. We take comfort in knowing that when the day comes when he is off the steroids and immune suppressants, Ezra will have donor cells ready to build him a new, strong immune system (we hope). Ezra started his summer school session this week. To match his 100% donor cells, he got 100% on his first ever spelling test (we're allowed a little bragging, right?)! Maybe his donor is a good speller. :)
Last week started out on an up note and ended on a down one. When we arrived at clinic last Monday, Ezra's counts had improved. Not back to where they had been, but better. He received high dose IVIG, which went fine. We then came back to clinic on Thursday and his numbers were worse. It ends up that due to a miscommunication, the doctor we had seen on Monday had lowered Ezra's steroid dose too much. So we had to go back up again to a higher dose of steroids for another three days. We are returning to Sloan tomorrow to see how his counts responded to the steroids. The goal is to get his counts back up and stable, and to then start weaning the steroids again. At this point, the thinking is that the rhinovirus likely triggered the antibodies to reactivate. His counts aren't terrible, but the goal is to prevent any destruction from getting out of control. The strength of the antibodies against red blood cells is still at the weakest level, which is where it has been over the last couple of months. Ezra tested negative for neutrophil antibodies this time around. He has not had an issue with his neutrophils since the day all of his counts tanked, but it is still great that he was able to get rid of those antibodies. We have not re-tested for platelet antibodies recently because the test requires a massive amount of blood, and Ezra doesn't have extra hemoglobin to spare. His platelets have been at a safe number even with the latest set back, and that's what matters most. So, as always, we will see what tomorrow brings. Ezra is such an unbelievable trooper about it all - the more frequent (and longer) clinic visits and the increase in the nastiest tasting of all his meds (steroids). He marched into his bed at clinic for IVIG and started exclaiming about everything he could see out the window, even though he has seen that view countless times. He danced around doing his Three Stooges act and had a blast goofing around with the clowns. The rhinovirus is gone and his energy level is great. We're thankful that whatever is going on inside has not affected how he feels. Ezra has been waiting to see fireworks again since last July 4th. He got his wish this week. We found a quiet spot to watch our town's fireworks and he of course loved every minute of it. Last July 4th, we had pretty much made the decision to move forward with the second transplant. We remember Ezra's excitement over his first fireworks show and thinking it will be a miracle if he is able to enjoy the fireworks again next year. While too much is still so unknown, we are thankful that today our resilient little man is exploding with so much life.
What we feared might happen has happened. Ezra's counts dropped this week. In particular, his hemoglobin is lower than it has been since the end of March when we were first discharged and one measure of red blood cell destruction is higher than it's been in almost two months. His platelets also dropped, although they are still at a very good, safe number. Although the timing of this drop in counts coincides with the outbreak of the rhinovirus, it is impossible to know if the drop is being caused solely by the virus, or by the steroid decrease, or both. Whatever the answer is, the drop is significant and the doctors feel something has to be done. Here is the plan. We went to a higher steroid dose Friday and are staying at that dose until at least tomorrow. We are going back to Sloan tomorrow for a counts check and, unfortunately, high dose IVIG. The reasons for the high dose IVIG are 1) his IgG dropped to just above the threshold for giving it (which may or may not be a consequence of fighting off the infection); 2) if the platelet decrease is from auto-antibodies, then the high dose IVIG will help stop any further destruction; and 3) it will hopefully completely knock the rhinovirus out of his system and we can take it out of the picture. Thankfully, he has been doing a good job of getting rid of the rhinovirus on his own. He still has a bit of a stuffy nose, but the virus is on its way out. The high dose IVIG should help ensure that it's gone. The next step with the steroids will be determined by his counts tomorrow. If they bounce back, then we should be able to drop down to a lower dose, but the doctor is already talking about keeping him at a higher dose than what he was taking for a longer period of time. How long is unclear. Our first question was "then how is he ever going to get T cells?" Well, there was no easy answer to that question. We know that it is too soon to feel defeated. If his counts bounce back quickly and stabilize, then the doctors may draw the conclusion that it was the virus causing the counts suppression and he may be able to go back down on steroids quickly. Whatever the outcome, it's hard not to feel heartbroken over another setback. It feels like it never ends - every time we start to feel optimistic, we fall backward. Once again, we thought we were seeing the glimmer of light at the end of the tunnel. And once again, we've lost sight of that light. Despite the craziness going on inside his body, Ezra feels well and isn't too bothered by the sniffles. His teachers and therapists are on break for the next week, and then he will continue to receive services during the summer. Ezra has missed so much time that it's great that we don't have to stop again for the summer. His days would be very empty without his teachers to fill them with learning, challenges, and fun. We are trying to keep him busy during break - like spending the afternoon watching PSE&G work on a gas pipe down the road. Thank you to the PSE&G workers for answering Ezra's many questions and for kindly offering for him to climb into the machinery (which he thankfully declined)!For now, we tuck away our dreams of school and play dates for some other time. We will continue to do what we have learned to do best - find the light in our sanitized world of Purell and Clorox wipes and keep the rest of life at bay for a future day we still hope will come.
As I was posting our update last week, I thought "I hope I'm not 'jinxing' us by writing a positive update." Then I thought, "no, that's silly, we have to rejoice in whatever positive news we can get." Yeah, well, as has been the story of our lives over the past few years, we had another step back this week. First, Ezra developed cold symptoms and it ends up that he has rhinovirus - the common cold. Of the viruses that can cause cold symptoms, rhinovirus is the least worrisome, but it can be difficult to clear for immune-compromised patients. Ezra had a really miserable (and nerve racking for us) 24 hours of feeling rotten. He is doing better now, although still plenty congested. Complicating everything, Ezra's T cells have dropped by 50%. Ezra has about the same number of T cells that he had in December when they first appeared. We've lost 7 months of progress. Without enough T cells, Ezra remains at high risk for serious infections, and the longer he goes without T cells, the greater the risk. And now he has a cold. If he doesn't fight off the cold quickly, he will get a round of IVIG in hopes that will help him fight off the virus. His IgG has basically remained stable, but without enough T cells, his doctor is not sure he will be able to clear it quickly, and we don't want it to turn into a sinus infection or anything more problematic. Given the T cell situation, his doctor is going faster on the steroid wean this week. Viruses can cause counts to drop, so they are going to keep a close eye on his counts to make sure that between the steroid decrease and the cold, things aren't getting out of control. If he does ok with the steroid wean, then we may move toward weaning the immunosuppressant, while keeping him on a low dose of steroids. All of this is in hopes of getting up his T cells while not letting the autoantibodies make trouble again. A delicate balance. We are frustrated, to say the least. It feels like we can never stay in a forward direction for long. As always, please keep our little man in your prayers and thoughts.
