Sunday, September 28, 2014

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!

Wednesday, September 17, 2014

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 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! 

Sunday, September 14, 2014

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. 

Sunday, September 7, 2014

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:
$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!