Sunday, December 21, 2014

T It Up!

Good news on the T cell front - Ezra's T cells have reached the normal range! We also received the results of the general function T cell test and his T cells have surpassed the "two-thirds of normal" immunological marker. They aren't functioning normally yet, but they are making slow and steady progress towards a safer place. Looking back to September, Ezra still had very few T cells, and it felt like we would never see the day when he would have normal T cell numbers. Now, three months later, he made it!

Although we have reached this milestone, nothing changes as far as his treatment and isolation. In fact, Ezra had normal levels for these tests prior to transplant, but this is an important milestone for a post-T cell depleted transplant patient. We still need to see if he can make antibodies in response to the vaccine. This will be the true test of whether he is cured. Another round of tests to see if he responded to the vaccine have been sent to the lab, and we should have the results in the next couple of weeks. Please send some positive antibody thoughts Ezra's way!

We have also continued the slow wean of the immune suppressant. Getting off the immune suppressant and steroids is another challenge that Ezra needs to surmount in order to have a properly functioning immune system. His doctors are keeping a close eye on his counts to make sure there is no autoantibody flare up while we wean. So far, so good. 

We have been fully enjoying the light of Hanukkah this year. It isn't just the giant menorah that is once again on our front lawn. It is the love that has been bestowed upon Ezra. He has received gifts from many people who we know wish him good health most of all, but want him to also enjoy himself in the meantime. He is having a blast! 

We also had the opportunity to celebrate the holidays at Menorial Sloan-Kettering's annual pediatric holiday party. We spent time with Ezra's wonderful primary nurses from his time in-patient and celebrated with the doctors, nurses and staff who have gotten us through the most difficult times of our lives. It is also Ezra's favorite time of the year at the hospital because hidden away in the basement level is a magical hallway transformed into a winter wonderland by the operations staff, including a model train display that always captivates Ezra. We've become experts at finding light in even the darkest of places. Happy Holidays to all!


Sunday, December 7, 2014

Living In Limbo, But It's Ok

We had a chance to speak with Ezra's primary transplant doctor this week at clinic about the state of Ezra's immune system. As we wrote in the last blog post, we have been feeling very unsettled by the fact that the part of Ezra's immune system that isn't working right now (the ability to make antibodies) is exactly the main part that wasn't working prior to transplant. We got back the results on the vaccine antibodies test from a couple of weeks ago. He may have responded to the polio part of the vaccine, but for complicated reasons, the results are not clear. He definitely did not yet respond to the other viruses/bacteria in the vaccine. Ezra's doctor believes it is too soon. They will check again in two weeks. 

If he still has not responded, Ezra's doctor assured us that she does not think that it is because of Hyper IgM Syndrome. She said that the communication between the T cells and B cells that is needed for B cells to make antibodies (and what Ezra's immune system was not able to do prior to transplant) is the last function to come back post-transplant, especially in T cell depleted transplants. She is optimistic that if we can get him off the immune suppressant, then with time the ability to make antibodies will come. His IgG also stayed the same for the past two weeks. It would be reassuring to all if we can see an increase in IgG, but we will certainly take stability - and it buys us another two weeks without IVIG. 

Ezra's doctor also explained that Ezra is in a safer place in terms of handling an infection now that he has more T cells and their function is improving. They are most concerned when there are low numbers of T cells and they aren't functioning. We obviously don't want to test this out, but it was very good to hear that he is making progress. Another T cell check is cooking in the lab and we should have the results in a couple of weeks. 

Thankfully, we have started weaning the immune suppressant. The wean will be slow over several months. He is still on a very low dose of steroids and we will hold him on this dose for now while we wean the immune suppressant. We are praying that he doesn't have an auto antibody flare up while we are weaning. 

Despite all the uncertainty, we are starting to feel like there are slivers of hope for the future. For the first time, I bought a shirt for Ezra during the holiday sales to put away for next year. I had never before allowed myself to buy something in advance for him. It always seemed presumptuous to think there would be a next year. Throughout this journey we've learned the hard way to never think ahead and to take each day at a time. We are starting to allow ourselves little glimmers of a healthy future, and we so hope we aren't proved wrong. 



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. 


Sunday, October 19, 2014

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. 


Sunday, October 12, 2014

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. 




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! http://www.goodthreads.com/SharedDesign/TeamEzra3.design


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 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! 


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: 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!




Saturday, August 23, 2014

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 

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. 


Sunday, August 17, 2014

Days +354 to +360: We Will Get There

We got back the results of Ezra's monthly T cell check and unfortunately Ezra's T cells decreased this month. The T cells are not as far down as they were in June, but it's enough movement in the wrong direction. The decrease in T cells is likely from the increase in steroids following Ezra's auto-antibody flare-up in June. After giving us the results, Ezra's doctor reassured us that "we will get there." We hold on tight to the belief that we will indeed get there. When and at what cost are the unknowns. We always say we're ok with this process taking a long time - after all, a life saving treatment shouldn't be easy - as long as we are moving forward. It is falling backwards, especially with losing precious T cells, that hurts. 

Ezra's toe is still a little red, but improved. We are continuing with the antibiotics for a bit longer and will go from there. His hemoglobin was down this week, but one of the indicators of red blood cell destruction was normal, so the doctors weren't too concerned. We are sitting tight on the steroids for this week and then hopefully be able to wean again next week. 

One piece of positive news: the platelet antibody screen came back negative. As of now, Ezra is testing negative for antibodies for all three cell lines! A negative screen doesn't mean that the antibodies are totally gone, but it is a sign that things are quieting down. They sent out another red blood cell antibody screen test on Friday because as we go down on steroids, the antibodies can flare-up again. We are so hoping these tests stay negative. 

While the summer is great for us because we can do so much outside, it is also challenging to go to any outdoor public places because so many more people are around in the summer. We did make a great discovery to keep Ezra busy and challenged - mini golf. We discovered a great mini golf place close-by. We are able to go at quiet times and keep distance from the other players. Ezra loves it and even hit a crazy hole-in-one! Last year on this day, Ezra was on Day -5 of chemo in preparation for transplant number two and was already very ill. So grateful to be breathing fresh air far away from the gray of the hospital. 



Sunday, August 10, 2014

Days + 342 to +353: The Revolving Door of Issues

Last week, Ezra's platelet count had dropped again. Just as the red blood cells had come under control, of course his platelets needed to take their turn again and cause problems. The doctor was planning to see his counts this week and then give another round of high dose IVIG. We were feeling frustrated at how difficult it has been to get these auto-antibodies under control. 

Then we showed up to clinic on Friday and his platelets had bounced up nicely and are back in the normal range! A pleasant surprise that we will gladly accept. The doctor wanted to do another check of the platelet antibodies, which we haven't done for a while because it requires a tremendous amount of blood. It will be interesting to see what this test shows. 

Because there always has to be something, over the weekend, one of Ezra's big toes developed redness around the toe nail. It seems to be infected. He started antibiotics and foot soaks. We will be watching it closely. We are hoping the infection clears up quickly and doesn't cause problems with his counts, which it may do. Here we go yet again.

The doctor also sent out another T cell count check on Friday (yeah, we're not sure how he has any blood left). Ezra's total lymphocyte count, which includes T cells and other types of cells, has been low, so we have no expectations of what this month's T cell count will show. Please prayers for a T cell increase!

As we approach the one year anniversary of Ezra's transplant on August 23, there are a lot of intense memories for us associated with the month of August.  This week marks one year from when Ezra was re-admitted to start high dose chemotherapy for the second transplant. Five years ago this past week, a 5 month old Ezra was admitted to our local hospital with labored breathing and would soon be escalated to intensive care. For the first time five years ago, we heard the name "Hyper IgM Syndrome," and life forever changed. August may always be a difficult month for us, but we have plans to change it into a month when we will also always celebrate life and hope. 


Monday, July 28, 2014

Days +334 to +341: Got To Keep It Up

Just a quick update to let everyone know that Ezra's counts were once again good this week. Ezra's doctor called his counts "beautiful." His hemoglobin was up and all the other numbers were normal. The only issue, aside from the overarching issue of Ezra needing to grow a new immune system, is his liver function. His liver levels have unfortunately increased again. The doctor believes that it is from the increase in steroids. For now, the plan is to wait and see if the levels decrease as we go down on the steroids. There always has to be something of concern on this crazy journey. 

Ezra also had an eye exam to check his eyes for side effects from his two transplants. They are also going to be watching his eyes closely for cataracts, which can be a side effect of steroids. The doctor said it's too soon to see cataracts from the steroids, but for now, Ezra's eyes got an "A++."

We really, really need a period of calm and good counts to get Ezra off the steroids and immunosuppressant. We went down again by a small amount on the steroids, but it's going to take some time before he's even back to where he was on the wean before this latest flare up. Getting off these drugs is the key to allowing Ezra's donor cells to build him the new immune system he so desperately needs. We know those donor cells are there, waiting to be allowed to grow. We can't wait for them to break Ezra free of his life of isolation, and pray that it is only a matter of time. 


Sunday, July 20, 2014

Days +327 to +333: A Good Week

We finally have good news to report this week. First, Ezra's counts were great. His hemoglobin was normal and one of the measures of red blood cell destruction was also completely normal for the first time since he developed the antibodies. Second, the latest test of the strength of the red blood cell antibodies came back negative, showing no antibodies. Huh? Yep. This happened once before when he tested negative for the antibody and then two weeks later tested positive again. The doctor explained that the antibodies are probably there, but not at measurable levels at the moment. The flare-up that Ezra has been experiencing over the past three weeks was likely caused by a combination of the rhinovirus and the decrease in the steroids. Whatever it is, and even if the test comes back positive again two weeks from now, it is certainly good to have a period of calm and healing. 

Even more surprising - Ezra's T cells went up! They are back to where they were in May when they first dropped. The level is still far from where it was before he went on steroids at the end of February and still very far from where he needs to be, but he is headed in the right direction. Ezra's doctor was very surprised at this increase. She did not expect a T cell increase with the current steroid dose. That's Ezra for you - always doing things his own way. 

The question is - can he keep these T cells or is he going to lose them again because of the steroids? The doctor thinks he will keep them provided we are able to continue weaning the steroids. Despite all this good news, the doctor lowered the steroid dose only by the smallest of increments again this week. She is proceeding with caution for now to avoid another flare up. So it remains to be seen if Ezra will be able to maintain this T cell progress. 

By now we know all too well that we can lose this progress as quickly as we gained it. There have been way too many ups and downs on this journey for us to feel like Ezra is at all safe. Despite the number of times we've been knocked down, we are still managing to feel optimistic from these latest numbers. A little hope goes a long way. 

The other exciting news of the week is that Ezra had his first day of school in school! Ezra's teachers came up with the great idea of having Ezra do his home schooling at one of the schools that is closed for the summer so that he can get a sense of what it is like to sit in a classroom and what a school looks like. They had one of the preschool rooms thoroughly cleaned, air conditioners cleared out, and a sign hung on the door welcoming Ezra to school. The look on his face when his teachers told him that he was going to the school was indescribable. He was just so happy. 

Class started with the Pledge of Allegiance. Ezra was very taken by the smart board in the classroom. He was so focused on his school work - it was amazing to see. He will continue to go to the school once a week for the summer. We are very grateful for the creativity and determination of his teachers who arranged for this incredible opportunity. Our hope is that when the day comes for him to attend "real" school with other children that he will be as excited and happy to attend as he is with his class of one. 






Sunday, July 13, 2014

Days +320 to +326: A Mixed Bag

It was another week of up and down counts. Ezra's body is still struggling to get back on track. The main issue at the moment seems to be red blood cell destruction. His platelets thankfully remained in the normal range this week. His hemoglobin, on the other hand, has been up and down. At the end of the week, his hemoglobin had improved. However, one of the measures of red blood cell destruction was higher, reflecting that his body is working hard to replace red blood cells that are being destroyed. The doctor was actually happier with this scenario than when his hemoglobin was lower because it means that his body is able to compensate for the destruction. 

The doctor decided to lower his steroid dose by the smallest increment and only on alternate days. We are really hoping that this minor decrease doesn't affect his counts and that we can get back to weaning the steroids. This weekend, we already saw a regression in Ezra's strength and endurance from the higher steroids. Steroids just stink. 

We did receive the results of Ezra's latest chimerism test and thankfully, he is still 100% donor on all cell lines. We take comfort in knowing that when the day comes when he is off the steroids and immune suppressants, Ezra will have donor cells ready to build him a new, strong immune system (we hope). 

Ezra started his summer school session this week. To match his 100% donor cells, he got 100% on his first ever spelling test (we're allowed a little bragging, right?)! Maybe his donor is a good speller. :)


Sunday, July 6, 2014

Days +313 to +319: Working On It

Last week started out on an up note and ended on a down one. When we arrived at clinic last Monday, Ezra's counts had improved. Not back to where they had been, but better. He received high dose IVIG, which went fine. We then came back to clinic on Thursday and his numbers were worse. It ends up that due to a miscommunication, the doctor we had seen on Monday had lowered Ezra's steroid dose too much. So we had to go back up again to a higher dose of steroids for another three days. We are returning to Sloan tomorrow to see how his counts responded to the steroids. The goal is to get his counts back up and stable, and to then start weaning the steroids again. 

At this point, the thinking is that the rhinovirus likely triggered the antibodies to reactivate. His counts aren't terrible, but the goal is to prevent any destruction from getting out of control. The strength of the antibodies against red blood cells is still at the weakest level, which is where it has been over the last couple of months. Ezra tested negative for neutrophil antibodies this time around. He has not had an issue with his neutrophils since the day all of his counts tanked, but it is still great that he was able to get rid of those antibodies. We have not re-tested for platelet antibodies recently because the test requires a massive amount of blood, and Ezra doesn't have extra hemoglobin to spare. His platelets have been at a safe number even with the latest set back, and that's what matters most. 

So, as always, we will see what tomorrow brings. Ezra is such an unbelievable trooper about it all - the more frequent (and longer) clinic visits and the increase in the nastiest tasting of all his meds (steroids). He marched into his bed at clinic for IVIG and started exclaiming about everything he could see out the window, even though he has seen that view countless times. He danced around doing his Three Stooges act and had a blast goofing around with the clowns. The rhinovirus is gone and his energy level is great. We're thankful that whatever is going on inside has not affected how he feels. 

Ezra has been waiting to see fireworks again since last July 4th. He got his wish this week. We found a quiet spot to watch our town's fireworks and he of course loved every minute of it. Last July 4th, we had pretty much made the decision to move forward with the second transplant. We remember Ezra's excitement over his first fireworks show and thinking it will be a miracle if he is able to enjoy the fireworks again next year. While too much is still so unknown, we are thankful that today our resilient little man is exploding with so much life. 


Sunday, June 29, 2014

Days +306 to +312: Losing Sight of the Light

What we feared might happen has happened. Ezra's counts dropped this week. In particular, his hemoglobin is lower than it has been since the end of March when we were first discharged and one measure of red blood cell destruction is higher than it's been in almost two months. His platelets also dropped, although they are still at a very good, safe number. Although the timing of this drop in counts coincides with the outbreak of the rhinovirus, it is impossible to know if the drop is being caused solely by the virus, or by the steroid decrease, or both. Whatever the answer is, the drop is significant and the doctors feel something has to be done. 

Here is the plan. We went to a higher steroid dose Friday and are staying at that dose until at least tomorrow. We are going back to Sloan tomorrow for a counts check and, unfortunately, high dose IVIG. The reasons for the high dose IVIG are 1) his IgG dropped to just above the threshold for giving it (which may or may not be a consequence of fighting off the infection); 2) if the platelet decrease is from auto-antibodies, then the high dose IVIG will help stop any further destruction; and 3) it will hopefully completely knock the rhinovirus out of his system and we can take it out of the picture. Thankfully, he has been doing a good job of getting rid of the rhinovirus on his own. He still has a bit of a stuffy nose, but the virus is on its way out. The high dose IVIG should help ensure that it's gone. 

The next step with the steroids will be determined by his counts tomorrow. If they bounce back, then we should be able to drop down to a lower dose, but the doctor is already talking about keeping him at a higher dose than what he was taking for a longer period of time. How long is unclear. Our first question was "then how is he ever going to get T cells?" Well, there was no easy answer to that question. 

We know that it is too soon to feel defeated. If his counts bounce back quickly and stabilize, then the doctors may draw the conclusion that it was the virus causing the counts suppression and he may be able to go back down on steroids quickly. Whatever the outcome, it's hard not to feel heartbroken over another setback. It feels like it never ends - every time we start to feel optimistic, we fall backward. Once again, we thought we were seeing the glimmer of light at the end of the tunnel. And once again, we've lost sight of that light. 

Despite the craziness going on inside his body, Ezra feels well and isn't too bothered by the sniffles. His teachers and therapists are on break for the next week, and then he will continue to receive services during the summer. Ezra has missed so much time that it's great that we don't have to stop again for the summer. His days would be very empty without his teachers to fill them with learning, challenges, and fun. We are trying to keep him busy during break - like spending the afternoon watching PSE&G work on a gas pipe down the road. Thank you to the PSE&G workers for answering Ezra's many questions and for kindly offering for him to climb into the machinery (which he thankfully declined)!

For now, we tuck away our dreams of school and play dates for some other time. We will continue to do what we have learned to do best - find the light in our sanitized world of Purell and Clorox wipes and keep the rest of life at bay for a future day we still hope will come.


Sunday, June 22, 2014

Days +300 to +305: Frustration

As I was posting our update last week, I thought "I hope I'm not 'jinxing' us by writing a positive update." Then I thought, "no, that's silly, we have to rejoice in whatever positive news we can get." Yeah, well, as has been the story of our lives over the past few years, we had another step back this week. 

First, Ezra developed cold symptoms and it ends up that he has rhinovirus - the common cold. Of the viruses that can cause cold symptoms, rhinovirus is the least worrisome, but it can be difficult to clear for immune-compromised patients. Ezra had a really miserable (and nerve racking for us) 24 hours of feeling rotten. He is doing better now, although still plenty congested. 

Complicating everything, Ezra's T cells have dropped by 50%. Ezra has about the same number of T cells that he had in December when they first appeared. We've lost 7 months of progress. Without enough T cells, Ezra remains at high risk for serious infections, and the longer he goes without T cells, the greater the risk. And now he has a cold. If he doesn't fight off the cold quickly, he will get a round of IVIG in hopes that will help him fight off the virus. His IgG has basically remained stable, but without  enough T cells, his doctor is not sure he will be able to clear it quickly, and we don't want it to turn into a sinus infection or anything more problematic. 

Given the T cell situation, his doctor is going faster on the steroid wean this week. Viruses can cause counts to drop, so they are going to keep a close eye on his counts to make sure that between the steroid decrease and the cold, things aren't getting out of control. If he does ok with the steroid wean, then we may move toward weaning the immunosuppressant, while keeping him on a low dose of steroids. All of this is in hopes of getting up his T cells while not letting the autoantibodies make trouble again. A delicate balance. 

We are frustrated, to say the least. It feels like we can never stay in a forward direction for long. As always, please keep our little man in your prayers and thoughts. 


Monday, June 16, 2014

Days +292 to +299: Thriving on the Outside, Working on the Inside

Ezra thankfully had another week of stable counts. His IgG level went up a little, which is great. Too soon to say if his body is getting back into normal IgG production, but it is definitely a good sign that something is cooking. Ezra also got to stop taking two oral medications over the past two weeks (Whoohoo!), and the very slow steroid wean continues. We are waiting for results on the latest check of his T cells. We are praying for some small improvement or at least stability, and no more falling numbers. Ezra really needs more T cells for many reasons. 

In reflecting on the last two months since we were discharged from the last hospitalization, Ezra has been thriving, at least outwardly, for the first time in a very long time. Physically, he seems to get stronger everyday. He has been asking to walk places, when he would only go in the stroller before. This past weekend he rode his bike to the park, which was a huge deal because his leg strength has been poor since transplant and got worse with the steroids. Ezra has also been asserting his independence more and acting more like a typical 5 year old, all of which we are happy to see. His concentration with his teachers has improved now that he feels better. We are very excited that Ezra has been learning how to read! Now we just need the inside to catch up with the outside. 

Today brought a momentous milestone - match number 150 from Ezra's Gift of Life Donor Circle (www.giftoflife.org/help4ezra)! We feel very blessed to have been able to help so many others. Thank you and congratulations to all who have helped us with our goal of building the registry!


Monday, June 9, 2014

Days +285 to +291: We'll Take It

Thankfully, Ezra's counts were good this week. His platelets actually made it to the normal range for the first time since February when the auto-antibodies appeared. His hemoglobin and other indicators of red blood cell destruction were good. His IgG level dropped again, but it was not as much of a drastic drop as the prior weeks. The tests looking more closely at Ezra's immune system showed that he has the type of B cells capable of making IgG. Based on these results, his doctor decided to wait on giving him IVIG. She set a threshold number for his IgG level and if he drops below that number, then he will get IVIG. These test results were reassuring because they show that Ezra's immune function has not been completely suppressed by the steroids and immunosuppressant. We were also happy to see that these B cells were still there because Ezra did not have this type of cell prior to transplant, which means that the donor cells are still working on building a new, functioning immune system.

The steroid wean continues, but the rate of decrease has slowed. The wean has to proceed more slowly now because it is more likely to see flare-ups at lower steroid doses. We of course want him off the steroids as fast as possible, but clearly this is going to be a long process. Good thing we have plenty of practice being patient. 

Even Ezra's liver levels, which had jumped up again in the past month or so, improved this week. Ezra's doctor is very pleased with his progress. We don't know if two weeks of good results make a trend. Maybe it does and maybe it doesn't, but either way, we'll take it.

No exciting visits or adventures to report this week, but the memories of Ezra's visit with Big Igi and his day at the ballpark continue to bring smiles to all our faces. We are also so grateful to those of you who continue to make donations to Ezra's Gift of Life donor circle (www.giftoflife.org/help4ezra) and to other organizations in merit of Ezra's complete recovery. We are touched by the continuing support for our little man. We never imagined that so many people would care so much about a little boy's medical challenges. Thank you for sharing Ezra's quest for a cure with us and for helping others along the way.


Monday, June 2, 2014

Days +278 to +284: Take Me Out to the Ballgame

First, before we get to the exciting news of the week, here is the medical update. As has been the trend lately, there is good news and bad news (better than all bad news!). The good news is that Ezra's counts were great this week. Platelets went up and were higher than they have been since this latest crisis began. Hemoglobin was also up and one of the indicators of red blood cell destruction was down. And this was all with a steroid wean, so good stuff indeed.


The bad news is that Ezra's budding new immune system has been beaten up pretty badly by the steroids and immune suppressant. Either he is no longer making IgG or he is making very little of it. His IgG levels are dropping and he will likely need IVIG again - not because of his platelets, but because he is no longer making enough IgG to protect him from infection. So, we are back to the state of his immune system prior to transplant. When Ezra's body started making IgG post-transplant, it was a huge deal for us because Hyper IgM prevented him from making IgG. It was the first sign of a cure that he was making IgG. And now we lost it, and it feels like a punch in the stomach.

The doctor, however, is confident that this is a temporary issue, and that once Ezra is off the steroids, his immune system will come back. We trust this, but also feel like we have to be guarded - we will believe it when we see it. We are waiting for some additional test results looking more closely at the different cells of his immune system, and then she will make a decision as to whether he needs IVIG now or if we wait a bit longer and continue to check levels. His long-awaited T cells have also taken a deep nose dive. Despite this negative state of immune system affairs, we know that Ezra has the strength to rise again from the ashes. It is just a matter of time and keeping him healthy in the interim. The question is how much time, and that no one knows.

The day we were discharged from the hospital following Ezra's second transplant, we wrote in this blog about how we looked at Yankees Stadium as we sat in traffic on the FDR, and thought that maybe, just maybe, Ezra would get to go to a Yankees game this year. Well, it ends up that his immune system is not strong enough to handle being in the crowds at Yankees Stadium. But that did not stop some very special friends who have made it their business to make Ezra smile. Thanks to these special miracle workers and the generosity of Yankees General Manager Brian Cashman, Ezra got his first day out at the ball game!

Yesterday, Ezra had the opportunity to watch a Yankees game from the safety of a private suite at the stadium. We didn't tell him until yesterday morning, for fear that something would happen and we wouldn't be able to go. We handed him an envelope with the tickets inside. When we explained what the tickets were for, he ran into our arms crying from overwhelming happiness. It was an emotional, beautiful moment.

The weather was spectacular, the view was spectacular - the entire experience was spectacular (except for the Yanks who lost, but hey, there's just so much we can control). We didn't think Ezra would make it through the entire game, but his eyes were glued to the field and he wasn't going to miss a minute of the game.  Thanks to all those involved from the Yankees organization, as well as the devoted people at Project Sunshine who helped with the magic making, no detail was overlooked. The suite was stocked with hot pretzels (Ezra's favorite snack), his name was on the jumbotron, he took home a bag of souvenirs, and they provided purell and sanitizing wipes! Ezra happily fell asleep last night singing "Take Me Out to the Ball Game."

One of the most difficult aspects of Ezra's medical journey is the lack of control we have over his health and really the course of his, and in turn, our lives. What we do have control over is making sure Ezra is as happy as he can be given the circumstances. We are lucky to have people in our lives who feel the same way and have helped us to override the horrible days with incredible days.





Sunday, May 25, 2014

Days +270 to +277: In Love With a Big Blue Bear

It was another frustrating medical week. On the plus side, Ezra avoided another round of high dose IVIG. At clinic on Tuesday, his platelets had gone up a little. They are not back to where they were, but because the platelets went up, the doctors did not give IVIG. Platelets remained the same on Friday. One of the indicators of red blood cell destruction was a little elevated this week, but his hemoglobin remained solid. He is clearly still destroying cells, but not at a rate where it is affecting his counts. More importantly, his doctor allowed another steroid wean this week. We will see how his counts look next week after the wean. It all remains day to day, but then again, it always has been day to day.
 
On the downside, we got back some results looking at the state of Ezra's immune system, and it is definitely suppressed. While we need it suppressed so that he can deal with and get rid of these auto-antibodies, it is so, so hard to basically lose the progress Ezra has made over the past six months. Ezra's primary doctor was out of the country this week, and we will have a better understanding of where things stand once she is back. It may be that he bounces back quickly once he is off the immunosuppressants, but there is no time frame for that and once again, we are faced with the reality of an endless journey.
 
The bright spot of our week came in the form of a giant blue bear. Some of you may have seen the video we posted on Ezra's Facebook page of Igi sending love to Ezra (http://youtu.be/OT1KxWoGyXA). Well, Igi is a therapy bear made by Baxter to help children with primary immune deficiency deal with and understand their treatments. Ezra's Igi is called "Ivig" (sounded out), and Ivig has become an integral part of Ezra's life over the past few years. We first introduced Ivig and the medical supplies that he comes with to Ezra when he was two years old. He took to him right away, but it wasn't until Ezra's first transplant that the relationship became critical to Ezra's medical and emotional care. According to Ezra, "Ivig used to live in a bag, but then I took him out and now he's always with me." Ivig became our way of explaining all the terribly difficult challenges that Ezra had to endure. His child life specialist made books starring Ivig to explain every difficult milestone of transplant and beyond. Whatever happened to Ezra, happened to Ivig.

During his second transplant, Ezra saw a picture of the life-size Igi at a conference. Ezra went crazy - laughing and begging us to get giant Igi for him. To have anything bring a smile to our son's face while he was suffering so much meant everything. We know one of the patient advocates with the program and shared with her Ezra's excitement over seeing big Igi and his love for Ivig. We laughed about it and that was that. Then this past February, we heard from her, and it ends up that she had been working for months to arrange a visit for Ezra from big Igi! We couldn't believe it. We set up a date for the visit and eagerly waited for the day to arrive. Unfortunately, it turned out that Ezra was in the hospital for when the visit was originally planned. Everyone was heartbroken (Ezra thankfully did not yet know about the visit).

Touched by Ezra's story, the amazing folks at Baxter arranged for the video of big Igi to cheer him on. Once Ezra was stable, we were able to plan a makeup visit, which happened this past week. There are no words to describe the joy on Ezra's face when he saw giant Igi strolling down the street waving and then turning into our driveway. He then spent an afternoon racing cars with Igi, playing tag and goofing around. For us, the best part was that Ezra was free to hug and hold hands with Igi. Ezra has learned to be so cautious about touching anyone or anything that he isn't certain is safe. We knew that Igi was newly redone for his visit with Ezra and he could be uninhibited with loving that blue bear. We got to watch our son have the time of his life and forget all the pain and suffering for an incredible afternoon.

We are tremendously grateful to everyone who made this visit happen. As you can see from the pictures, it was a dream come true for our little man.