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. 

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. 

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.