This Is Either Good News or Not So Good News

We received some confusing medical results this week. Ezra's T cells shot up - not to normal, but well past the initial level that the doctors were waiting for him to reach where certain changes are made on his medical care. This is a surprising result given that he is still on steroids, a full dose of immune suppressant, and he has barely had any T cells for a long time. We would have been jumping for joy over this news, except it came with a confusing result - his IgG is down. With more T cells, his IgG should have increased. So, does this mean the new T cells aren't functioning properly? Is it because of the steroids and immune suppressant? Or are the T cells too new and not yet fully communicating with his B cells in order to make IgG?

Or, scariest of all, could the T cells possibly be his old, defective T cells and not healthy donor T cells? Our minds immediately went to that thought. We have not checked his chimerism for three months because of all the other issues going on and the effort to conserve blood. This is the longest we have gone without checking chimerism. We can't help but worry that this burst of T cells is made up of old, defective Ezra T cells. Ezra's doctor does not think that this is what is going on, but anytime we have been told that something about Ezra is medically "surprising," it has always been a bad thing. We are trying to be positive and focus on the most likely explanation is that the lack of IgG is from the immune suppressing drugs and that these are very new T cells that need more time to properly communicate with his B cells.

The plan is to send out a chimerism test and a slew of other tests later this week to try and get a better sense of the state of his immune system, assuming his hemoglobin can handle the blood work. We know that most likely, there will be no black and white answer - the situation will be filled with gray. The most likely outcome will be that we need more time for everything to sort out. That's just how this journey seems to go. It will be an anxiety-filled couple of weeks until we have his test results back - especially the chimerism test. 

Thankfully, we have next Sunday's Walk for Life to distract us from our worries! We are so grateful to everyone who has generously donated and to all who are joining us on Sunday. You can still help us celebrate life and help add donors to the registry by donating at http://support.giftoflife.org/site/TR/WalkForLife/General?px=1293644&pg=personal&fr_id=1150

If you plan on attending the event, please register in advance - you will save on registration fees! It will be a fun and inspiring day!

We hope that by our next update we have at least some clarity as to the state of Ezra's immune system. It will be a miracle if the appearance of these T cells ends up being good news. Even if it ends up that things are still mostly covered in gray, at this point any step toward the light will feel like a leap to us. 

Iron Boy!

After two rounds of IV iron, Ezra's hemoglobin is back in the normal range. His iron stores were severely depleted - almost gone. We will continue to monitor his iron storage levels to determine how often he needs the IV iron. Unfortunately, regularly taking significant amounts of blood from Ezra to monitor him for infection and all the other issues we have been dealing with is a necessary evil. Going forward, it will be a balancing act between getting these tests done and making sure he doesn't become anemic.

After seeing his counts this week, Ezra's doctor feels confident that the drop in hemoglobin was not from a flare-up of the auto-antibodies, and it was the lack of iron. This is a relief. She allowed us to get back to the slow wean of the steroids, which is great because our number one goal right now is to get Ezra off the steroids. 

We are waiting another two weeks before checking to see if Ezra has been able to make antibodies to the vaccine, assuming his hemoglobin is high enough to handle the blood work. Another T cell check is currently at the lab. It would be a huge relief to finally see some T cell growth this month. Until he gets an adequate amount of T cells, Ezra remains at risk of life-threatening infections, and the longer it takes, the scarier it is. A critical amount of functioning T cells is his ticket out of isolation and into the world. Please T cells, grow, grow, grow!

The IV iron has helped improve Ezra's energy, although he continues to struggle with strength and endurance issues following these 2 and a half years of brutal transplant-related treatments. Last night at bedtime, Ezra proclaimed "I am VERY healthy!" We're hoping that he has the inside scoop and maybe he knows that his T cells are growing. At the least, we are happy that he feels "very healthy." Our hope is that he should only know what it feels like to be healthy from this point forward. That would truly be a dream come true. 

Have the Happy, Need the Healthy

It seems that always when we reach a period of calm and we start to see a glimpse of light, it disappears before we can even breathe a sigh of relief. Over the past two weeks, Ezra's hemoglobin has been dropping. We spent the last several days in a panic because the cause of the decrease was not clear. We were very fearful that it was the auto-antibodies ramping up again. Ezra's doctor was already talking about increasing the steroids again, and we were in a state of despair. We just received word tonight from his doctor that it appears that Ezra is once again iron deficient. He has had a lot of blood drawn over the past month for various reasons, and it may be the case that the increase in blood draws put him over the edge. 

We never thought we would be relieved that Ezra is iron deficient, but compared to the other options, we are relieved. We don't have a complete picture of what is going on because Ezra's doctor wanted to try reducing the amount of blood taken this past week and did not want to add any additional tests. We are praying the culprit is "just" the iron deficiency and not the auto-antibodies. He will start IV iron again this week. It will likely take some time to rebuild the stores as it did last time, but hopefully his counts will improve quickly. 

Ezra's doctor wants to wait a month after the vaccine to test for antibodies to give him more time to respond, although now with this latest issue, we don't know when we will test. We also got back results on testing performed on a certain aspect of T cell function. The results show that the small number of T cells that Ezra has are functioning fairly well. Not normal function, but improving. This is great, assuming he can avoid any increase in steroids from here on out. 

Once again, we head into a new year hoping that it will finally be the year of the cure. Last year on Rosh Hashanah, we found out that Ezra engrafted and was 100% donor. We had many high hopes for this past year. We remain grateful for what we have and hopeful that this coming year will bring health for our sweet boy. Wishing all a happy and healthy new year. 

P.S. If you are ordering a Team Ezra shirt and want it before Oct. 26, please order this week! http://www.goodthreads.com/SharedDesign/TeamEzra3.design

Ordering Team Ezra Shirts

A couple of questions have come up about ordering Team Ezra shirts. You will receive a standard Gift of Life shirt with your registration, but if you want to show your Team Ezra pride, you can order at http://www.goodthreads.com/SharedDesign/TeamEzra3.design. The store can only be viewed on a computer. The Team Ezra design is only available in Men's, Women's, Youth and Toddler short sleeves styles. 

If you plan on wearing the shirt to the Walk for Life, please order in PURPLE. If you are not attending the event, feel free to order in any color. Purple is Ezra's favorite color and we know that he will be thrilled to see everyone in purple!

Please do not make any changes to the file on Step Two of ordering. 

When you add the shirt to the cart, it will ask you to input the sizes you are ordering. 

Please order at least 3 weeks prior to Oct. 26 to ensure that you receive your shirt in time, so order soon!

$5 of every shirt goes to Team Ezra's fundraising for Gift of Life!

Thank you all for your support and enthusiasm for Team Ezra! 

Come On Antibodies!

Ezra received the vaccine at clinic on Friday. They took baseline levels beforehand so that the doctors will be able to tell if he responds to the vaccine. We are waiting either two or three weeks before checking for antibodies. The doctors will also be running another test to check if his T cells respond to the vaccine, which will give us more information. They have told us that if he does not respond, it means that his immune system is just not ready. And if he does respond, then celebrations all around. So now we wait and think positive antibody thoughts. 

We received the results of the latest T cell check. The amount of T cells was almost exactly the same as last month. An indicator of new T cell growth that has been zero for many months actually registered on the test this month at a very small amount, which gives us a sliver of hope that maybe next month will result in some T cell growth. Maybe. 

Ezra's IgG level went up a little this week. It's too soon to know if he will be able to maintain his IgG levels without treatment, but he certainly is not tanking, which is great. 

And because we can never go long without an issue cropping up (life would be so dull), Ezra's toe infection decided to return this weekend. We are trying topical antibiotics and soaks for a day or so to see if we can avoid another round of oral antibiotics. Toes crossed that the least invasive route works!

Ezra was upset over the shot, but he really used all the coping skills he has learned over the years to deal with his fears. It was amazing to see. Ezra's special child life friend Jessica brought in her two-headed monster to help Ezra get through the day. It was Ezra's idea to practice giving the two-headed monster a shot. Thankfully, Ezra behaved better than his patient. 

We have heavy hearts this weekend after learning that another Hyper IgM family lost their brave teenage son who was about six months post-transplant. This family already suffered the loss of another son to Hyper IgM. Our prayers are with them. He will be remembered during the Miss America pagent tonight, so tune in and send his family love. As long and as difficult this journey with Ezra has been, we feel very blessed that he has come so far and that he is still fighting hard. 

An Interesting Plan

Ezra's counts have been stable for the past two weeks and there thankfully has been no sign of the auto-antibodies. The doctors have slowed down the steroid wean now that Ezra is on a lower dose and the chance of a flare-up is higher. Frustrating that we are going so slowly, but it's better than a flare-up. His liver levels have been close to normal over the past couple of weeks for the first time in longer than we can remember. Another T cell check is cooking in the lab. We have zero expectations for his T cell numbers, but are hopeful as always for an increase. 

Ezra's doctors have come up with an interesting plan. They want to try and vaccinate him. Typically, they would not try to vaccinate until he reaches a certain number of T cells. The reasons are complicated to explain, but basically, he has now reached a dosing on the steroids where they think he may be capable of having an immune response. The reason for trying now is that it has been almost two and a half months since his last dose of IVIG. They can only check for an antibody response to vaccination when time has passed from the last IVIG as IVIG provides the antibodies that Ezra hasn't been able to make on his own. Ezra's IgG levels have unfortunately been dropping due to the immune suppression and he is at the point when we would normally give him IVIG. Now is an ideal time to hold off on the IVIG to do this vaccine challenge because the germy season has not yet hit. The hope is also that his body will kick in on the IgG production in the meantime now that the levels are low. 

Ezra will be given the vaccine at the end of the week, they will get a baseline, and then test for antibodies two weeks later. If he makes an antibody response, it won't necessarily change anything about his current situation because he remains at risk for life-threatening infection until he has more T cells. But it would be HUGE if he is able to make antibodies in response to the vaccine. There are a lot of issues with Hyper IgM, but the primary issue is the inability to make antibodies. If he responds to the vaccine, then we know the few T cells he does have are working and we are on our way to a cure. If he doesn't respond, then his immune system just isn't ready yet. It's worth a shot ;)

As everyone is returning to school, Ezra had the last of his weekly visits to "school" this week. As you can see, he loved every minute of it. We had decided to do the second transplant when we did with the goal of getting Ezra healthy in time to start kindergarten. We've had to come to grips with the reality that kindergarten is not happening - at least, not yet. It's heartbreaking, but at this point, all we want is a healthy Ezra. We are bringing kindergarten to Ezra - tomorrow he starts at home with a wonderful kindergarten teacher, in addition to his beloved teachers and therapists. 

Team Ezra is off to a great start! Team Ezra T-shirts are now available! Check out these awesome, Rube Goldberg theme purple (Ezra's favorite color) shirts: http://www.goodthreads.com/SharedDesign/TeamEzra2.design
$5 of every shirt goes to Gift of Life/Team Ezra fundraising for the Walk for Life. Special thanks to Jodie Hein and Ken Bowser for designing these fantastic shirts! Show your Team Ezra pride!

Day +365: One Year - A Celebration of Life

One year. A year like no other. Looking back on the past year, it feels like it can be divided by all the challenges Ezra has faced. First, round two of high dose chemo, transplant, engraftment and the unspeakable suffering of that time. Next, home and the horrific pain that Ezra suffered, which made him cry all day, as he sat covered in hot packs in a fog from pain medication. Then, just as the pain resolved, came skin GVHD and the round of immune-suppressing steroids used to stop it. Next up, parainfluenza virus and liver issues. And just when he had gotten over the virus and stopped the steroids, the auto-antibodies against his red blood cells, platelets and neutrophils started their vicious attack. Back in the hospital for the month of March with this frightening turn of events, a problem that continues to plague him and drugs that continue to prevent his new immune system from growing.

We still do not know how we ever mustered the strength to walk our baby back into the hospital and subject him once again to the nightmare of transplant. It was solely our belief that transplant was his best chance at a long, healthy life. And that we owed it to him to try and give him that life.

While there is much of transplant that we try not to remember, there is too much that we will never forget. The moment of transplant. Eyeing that impossibly small syringe and not believing that small amount of cells could save our son's life and grow into a new immune system. Everything - all of our lives - hanging on a minuscule amount of cells. At that moment, remembering how hard it was to breathe behind those masks - they make you feel like you're hyperventilating. And maybe we were. But the mask did not stop us from covering Ezra's chemo-soured head with kisses and wondering when he would once again smell like baby shampoo.

We have never gone into great detail as to the horrors chemo and the transplant process inflicted on Ezra's little body, especially the second time around. Suffice to say that it was horrible. But what is amazing, is the way that his body has healed and dealt with all the changes in the past two and a half years of two transplant journeys. From pre-transplant to the horrors of transplant to post-transplant weight loss and hair loss to the bloating of steroids to the still puffy, but not as puffy of today - it is breathtaking to see how his body has changed. We have been trying to teach Ezra to appreciate the way his body can heal and to take comfort in his body's incredible ability to heal.

Our hope that Ezra will someday be cured would be impossible without Ezra's miracle needle-in-a-haystack donor. Our hope when we had none. We owe everything to her and we hope she knows how much she means to us. We feel so much gratitude that a cure even exists, as difficult as it may be, and that Ezra's doctors have gotten him this far.

In celebration of Ezra's life, we are forming Team Ezra for the Gift of Life Bone Marrow Foundation Walk for Life taking place on Sunday, October 26 in Paramus, New Jersey. The event is a 5K walk or run and includes a donor-recipient meeting, kids area and entertainment. All net proceeds from the walk go to adding more donors to the registry. Please join us in honoring Ezra and helping others have a second chance at life. Register for Team Ezra at http://www.giftoflife.org/njwalk/teamezra. If you can't make the walk please consider donating to Team Ezra or to our family fundraising page 

http://gol.convio.net/site/TR/WalkForLife/General?px=1293644&pg=personal&fr_id=1150

We are also in the process of designing an awesome Team Ezra shirt - stay tuned!

We celebrated the one year transplant anniversary over the past few days with cake and balloons and a special shirt for Ezra that says "I survived TWO stem cell transplants" (who knew they made such shirts? And in kid sizes?). Our journey for a cure is sadly nowhere near over. We enter year two post-transplant with a certain amount of fear of what may come, but mainly we are hopeful. Hopeful that this year may be the year that Ezra is cured. 

Ezra has a daily calendar that he fills out each day with his teacher. It has a spot for feelings. Everyday Ezra chooses "Happy" because, as he says, "I am always happy." This is what we hang onto more than anything - our happy little boy who leads us through the darkest of days so that all we see is light.