Sunday, November 23, 2014

Trying To Be "Cautiously Optimistic"

The last few weeks have been spent trying to assess the state of Ezra's immune system, but after all the testing, we are still left in a state of uncertainty. We always try to focus on the positive and there is much to be grateful for at this point. Ezra is now on a very low dose of steroids and there has been no sign of the auto-antibodies. We have not yet started to wean the immune suppressant. We are hoping that will happen at some point next month. 

With the decrease in steroids, Ezra's new T cells have started functioning. To assess T cell function, Ezra's doctors run a test to see how the T cells respond to a "general" stimulant (as opposed to a virus or bacteria). Studies have shown that patients can fight off certain infections that are a concern following a T cell depleted transplant with two-thirds of normal function. Ezra's T cells are inching closer to that "two-thirds of normal" milestone. Ezra's T cells performed normally on this test prior to transplant. So not so exciting in terms of a cure, but a very good indicator in terms of T cell recovery post-T cell depleted transplant. 

They also performed another T cell function test relating to the vaccine Ezra received. His T cells were able to respond to one of the strains of bacteria in the vaccine. Ezra's T cells were NOT able to respond to this same bacteria after we attempted to vaccinate him prior to transplant. This is at least some change in his immune function, which we were very happy to see. Based on this result, Ezra's doctor said that she is "cautiously optimistic."

BUT (there's always a "but"), Ezra's IgG levels have dropped. Between his failure to respond to the first round of the vaccine and dropping IgG levels, his immune system is acting just like it did prior to transplant. The doctor decided to test to see if he has made any antibodies in response to the vaccine even though it is likely too soon from the second shot to see a response. If he shows some response at this point, it will be a miracle. Most likely, it is too soon. Or he isn't going to respond at all because of the immune suppressant and his newly rebuilding immune system. We can't help but fear that this part of his system may not come back at all. As much as his doctors believe it will, after all we have been through, we will believe it if and when we see it. 

The tricky issue is whether he needs to get a round of IVIG. We are all concerned about protecting him during the germy season. The problem with giving him IVIG right now is that we lose the opportunity to see if he has responded to the second round of the vaccine because it will be impossible to tell if he responded when he is getting the antibodies from IVIG. The other issue with giving him IVIG is that it will be another 4-6 months before we will know whether he is able to make IgG and antibodies on his own. That means another 6 months at the least of isolation and uncertainty. The older Ezra gets, the more concerned we are about his lack of contact with other children, and another 6 months of isolation is definitely not what we want. We will wait for the vaccine results to come back and go from there. 

As we enter this week of thanks, we are very grateful for how far Ezra has come. Looking back to last Thanksgiving, Ezra had recently developed GVHD, was on high dose steroids, and we were very frightened of the course the GVHD might take. He has overcome many obstacles, and we know more challenges lie ahead. Right now, he is feeling well and is a very happy little guy. And for that, we are more than grateful. 

Sunday, November 2, 2014

Not There Yet

First the good news: Ezra is still 100% donor on all cell lines. This means that those new T cells that appeared a few weeks ago are donor T cells, which is a huge relief. Without donor T cells, we would be nowhere. 

Now, the bad news: Ezra made no antibody response to the vaccine. In addition, he now has lower numbers of the type of B cell responsible for making IgG/antibodies than he did last month. His IgG has also continued to drop, albeit slowly. These results are confusing because he now has a lot more T cells and is on a lower doses of steroids, which should have meant improved immune function. 

There are several possible explanations. When he got the vaccine in the beginning of September, we knew it was a long shot that he would respond because he had very few T cells and was still on steroids and an immune suppressant (as he still is today). Although he now has more T cells, they are new and need time to start functioning properly. He also had plenty of antibodies left in his system from his last round of high dose IVIG in June, which can prevent or mask an immune response. In addition, he only received one round of the vaccine and it can take more than one shot to build immunity (there's a reason kids get so many rounds of the same vaccine). 

The doctor we saw on Friday decided to give Ezra another round of the vaccine. We're not sure it's going to work this time around either. Although he is on a low dose of steroids, we have not yet started to wean his immune suppressant, and that drug can prevent an antibody response. They are also going to run another test on Ezra's T cells later this week that may tell us more about whether the T cells are functioning properly. 

We can't help but feel uneasy about all this. Although Ezra is 100% donor, functionally his immune system right now is looking very similar to how it looked before transplant. We know that some patients, particularly immune deficient patients, do not obtain full immune function after transplant. We are hoping this is not Ezra's fate. Most likely, it is too early to draw conclusions, and we just need to do what we have learned we must always do - have patience with this terribly long journey. 

Ezra had been looking forward to Halloween at the hospital, and he had good reason to look forward to it because the pediatric hospital transforms into a magical place for Halloween. Every area has a different theme; the walls are covered in scenery; and everyone from the doctors to the maintenance staff wear costumes. Sadly, Ezra's day was ruined by the surprise shot. While getting a vaccine is a "normal" kid problem, Ezra has been through too much trauma and pain, and he needs to be prepared in advance for any challenges. That wasn't possible on Friday, and he was very upset. Our poor little Stooge wasn't able to nyuk it up as much as we all expected. 

Thankfully, on Thursday, Ezra's wonderful teachers and therapists came over to give him a Halloween celebration, complete with trick or treating throughout the house. We are very blessed to have such a passionate team working with Ezra who are constantly thinking of ways to help him and bring more life to his days. 

For those of you who don't follow Ezra's Facebook page, last Sunday's Walk for Life was incredible. We raised over $28,000, which will all go to adding more donors to the registry! Ezra was able to attend and cheer on Team Ezra. He was thrilled to have been there. It was an uplifting and inspiring day for us all. These are the moments of good that carry us through the uncertainty that continues to surround us.