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!