Days +111 to +121: How Far We've Come

One year ago, we announced on this blog that Ezra's transplant had officially failed. We had just received the news that he had only 1% donor cells. Faced with dismal options, we wrote "It may be that the right decision is to not do anything. To let him be. To let him live. And to hope that someday soon that long sought after match appears." A few months later our hopes and prayers were answered when Ezra's donor appeared on the registry. After much anguish, we decided that it was not better to let him be and that a chance at a cure was better than life with a sword hanging over his head. And now here we are one year later. The verdict on this transplant is still out, but things are moving in the right direction and so far we are in a much better place than last year at this time. There is much to be grateful for.

The latest round of testing on his T cells is back. There are multiple types of T cells. The type of T cell that he had a small amount of last month is down slightly, but basically the same, which is likely due to the steroids. The good news is that a small amount of another type of T cell has appeared! One step back, one step forward. 

There's been another good sign: Ezra has been able to go longer in between infusions of his immunoglobulin replacement therapy (IVIG). This is the treatment that Ezra has received every four weeks since he was diagnosed. It is what has kept him alive for the past four years. Ezra does not make an immunoglobulin called IgG, or at least he didn't before this second transplant. This was the level that came back drastically low when Ezra was sick with pneumonia at four months old and our first indication that there was something terribly wrong with his immune system. Each week, his levels of IgG are checked to see if he needs the treatment. The level has been dropping, but more slowly than his usual rate. He is now more than 7 weeks without IVIG, the longest he has gone without it. He may need it soon, but the doctors tell us that the fact that he is holding longer means that maybe, just maybe his body is starting to make IgG and his new immune system may be starting to function.

Ezra remains on a low dose of steroids. The wean is going very slowly. Thankfully, there is still no sign of the rash.

Ezra was loving the snowfalls of last week. He had been waiting for the first good snow basically since we got home from the hospital. He ran around yelling "snow angel!" and dropping backward into the snow. Seeing the snowflakes glittering on his newly re-grown eyelashes was a breathtaking sight. A little snow angel indeed.

Happy Holidays!

Days +103 to +110: Good News!

Today brought fantastic news: Ezra's T cells are 99% donor!!! And all other cell lines are 100%! We are beyond relieved and so thrilled! The news comes after we had a stressful discussion yesterday with Ezra's doctor where he was preparing us for the possibility that his T cells will be mixed chimerism. With T cell depletion transplants, it is apparently not unusual for the T cells to grow back mixed initially, and then the donor cells take over as time passes. This would have been a difficult scenario for us to handle given what happened with Ezra's first transplant. We knew that mixed chimerism was a possibility from the beginning and that his cruddy T cells are not so easy to knock out, but we were praying that we would never have to see those defective T cells again. Based on these results, it looks like Ezra's CD40 Ligand deficient T cells have hit the road. Adios T cells - you will not be missed!

We are hoping that Ezra's incredible-needle-in-a-haystack-miracle donor feels our joy and gratitude wherever she may be. This is all because of her. We welcome all of her cells and love them and her fiercely.

Ezra, by the way, does not know about any of this. We learned our lesson the hard way with the first failed transplant that we should not tell him much about his new cells until there is more certainty. Until the GVHD stays away and until he has the necessary amount of functioning T cells, we will not give him details. All we tell him is that his counts are good, he is doing great, and that we are so very proud of him. We patiently look forward to the day when we can tell him he has a new, strong immune system and he can go conquer the world. For now, we keep our victory dances for when he is sleeping.

The next steroid wean started last night. He will be on the steroids for about another month at the current rate. The rash has not returned. With these lower doses of steroids, it is more likely that the GVHD may flare up, so we are watching his skin very closely. The amount of T cells will likely be checked again at the end of the week. We are not expecting much T cell growth, if any, due to the steroids.

Thank you to everyone for sharing Ezra's YouTube rocket video! He has over 2,200 views and he is thrilled! He loved hearing all of the awesome comments! Thank you for giving him something to look forward to and to enjoy!

Days +91 to +102: Adventures in Steroid Land

Life with Ezra on steroids has been interesting. First, the good news is that the rash has not re-appeared. We have been able to reduce the steroid dose twice, and will be starting a third reduction. The rate of the wean starts to slow down now, so Ezra will be on steroids for at least another month, depending on how things go. It is frustrating because the steroids suppress T cell growth, but we will do whatever gives him the best chance of overcoming the GVHD.

Despite the reductions in steroid dose, Ezra is still dealing with the side effects. The steroids have caused his appetite to increase, which right now is a good thing because he is still regaining the weight he lost during transplant. The problem is that his appetite is mainly for chips. Steroids effect the salt-water balance in the body and can cause bloating and an increase in blood pressure (and blood sugar and many other bad things), so the doctors want us to watch his sodium intake. We have now gone from desperately wanting Ezra to eat and encouraging him to eat whatever he wants as long as he eats, to gently pulling back on the one food he wants more than anything else. Poor little dude must think we are crazy. He also has been experiencing the drastic highs and lows from the steroids. He goes from hyperactive to completely exhausted. Ezra is not a hyper child, so the last two weeks have been interesting. We are hoping that as we go down on the steroid dose, the side effects will subside.

Ezra has reached the Day 100 milestone. Day 100 is often touted as a marker of survival. Studies show that patients reaching day 100 post-transplant are more likely to survive in the long run. In Ezra's case with the type of transplant he had, Day 100 isn't all that significant. The more important milestone will be when he has a strong number of functioning T cells and is able to be free of the steroids without GVHD re-appearing. That will be cause to celebrate.

Ezra has been basking in the light of Hanukkah. Candles, songs, a gigantic menorah, and presents - what's not to love about Hanukkah when you're four years old? This year, the unspeakable thoughts that have tainted every joyous moment since Ezra was diagnosed seem to be a bit more subdued. Ezra's health is still in a very precarious state with so much of the path ahead unknown, but the progress he has made so far is huge. So, maybe day 100 is indeed a moment for feeling a little triumphant in the long fight for a cure. 

Happy Hanukkah! Love, Robin, Evan and Ezra

P. S. Ezra wanted to make a YouTube video of one of his model rocket launches "for other little kids." He likes asking how many views his video is getting, so if you have a few minutes, check out his video at 
http://youtu.be/_XFjETWPmus

Thanks!

Days +87 to +90: The Silver Lining

If every cloud has a silver lining, then we found the silver lining of Ezra's GVHD. He now has a very small number of T cells. Although the T cells are causing the GVHD, if we can get it under control, then having T cells is exactly where we want to be. The steroids prevent T cells from growing, so the sooner Ezra is off the steroids, the better. Once he is off the steroids, the expectation is that the T cells will continue to grow and Ezra will be on his way to a functioning immune system. IF he can get off the steroids.

We have thankfully started to wean the steroids. The rash is completely gone. The dose has been cut in half and will remain that way for a week. If there is no sign of GVHD, we will cut the dose again and go from there. If the rash comes back, he will go back on the higher dose. We are anxiously watching and trying not to obsessively check his skin.

The other piece of good news is that the higher level chimerism test showed that all cell lines that Ezra has (not including T cells) are 100% donor. Yes, it is still possible that Ezra's T cells can end up being mixed chimerism, despite the fact that all other cell lines are 100% donor. T cell chimerism will be checked once he has more T cells.

The wean couldn't come soon enough. The steroids are wrecking havoc on Ezra's stomach. We are already seeing improvement in how he feels since cutting the dose. We've been struggling to figure out ways to help Ezra deal with his pain. Fortunately, Ezra has figured out his own way to deal with the pain - with "pain rockets." He pretends that one of his rockets is a pain rocket and he launches it into space where it hits an asteroid and all the pain disappears into space where it can never hurt anyone again. It's working for him, at least for now. 

The super-sized menorah is back on our front lawn ready to light up our Hanukkah! For those of you who have noticed compared to last year's picture, yes, he is most definitely taller! Here's to hoping that the holiday of miracles brings the miracle of no GVHD and a growing immune system.

Days +83 to +86: GVHD

On Thursday, after looking at a picture of Ezra's rash, the doctor decided that it is indeed what we have feared - Graft Versus Host Disease. The rash had not spread, but the rash on his back was worse. She also decided that it was best to be aggressive with treating it and started Ezra on "systemic" (oral) steroids, rather than waiting and trying different topical creams to see if they would lessen the rash. Steroids are a major problem post-transplant because they suppress the number and replication of T cells, delaying immune reconstitution. This is the last thing Ezra needs at this stage when we are anxiously awaiting the appearance of T cells. Steroids are a game changer. They also can cause very serious side effects. But the doctor explained that they have found that if you wait too long to treat GVHD, it becomes much harder to remedy, and it is best to attack it early.

Since starting the transplant process, GVHD has been our greatest fear, short of Ezra dying. This latest set back is very difficult for us to come to terms with. We were told that a T cell depleted transplant (the type of transplant Ezra had) has a 5-10% risk of GVHD. For his first transplant, we were told there was about a 10% chance of engraftment failure. Why our one-in-a-million son ends up on the wrong side of the statistics so often is a question to which we will never know the answer. And yet, we know we are lucky that he has so far landed on the right side of the most important statistic - he is alive and he is fighting.

The good news is that the rash responded immediately to the steroids - it was much improved after just the first dose. The plan is to stay on the higher dose of steroids for a few more days at least and then start a slow wean. The concern is that the GVHD will flare up once the steroids are weaned and that will require an indefinite course of steroids, and result in continuing impact on his immune system and other organs. We will all be watching closely once he is allowed to start the wean.

The confusing part of this GVHD flare up is that as of a few weeks ago, Ezra had no T cells. T cells are what causes GVHD. The new T cells that form from the stem cells from the donor and "grow up" in Ezra are not supposed to cause GVHD. It is typically T cells that come in with the graft at the time of transplant that cause GVHD. With the T cell depleted transplant that Ezra had, only a very small number of T cells come in with the stem cells. Ezra received even less than the level of T cells that Sloan considers "safe" - so small an amount that T cells have been undetectable since the day of transplant. 

So, how does Ezra have GVHD? Well, first of all, labs were drawn on Friday to check again for T cells. If there are T cells, there is no way of knowing if they are the very small number of donor T cells that have multiplied for some reason or new T cells that aren't behaving as expected and are attacking Ezra's skin. If the test comes back showing no T cells, then the assumption is that there are T cells in his skin and organs, but not in his bloodstream, which is why they have been undetectable. His doctor has described GVHD as a "black box" in many respects, and that has been our impression of so much of the transplant process. It is, and always has been, an enormous leap of faith.

Just as we had finally gotten the real Ezra back, we now have yet another version of Ezra - steroid Ezra. Steroids can cause stomach upset and Ezra's weakened stomach is already causing him more pain.  It is so frustrating to go backwards after he had made great strides. 

Please send prayers and positive thoughts that the T cells chill out and leave Ezra's skin alone ASAP (Ezra Aviel ben Hertzelia Shandel). And please wish us all courage as we confront one of our greatest fears.

Days +74 to +82: It's Always Something

Just as Ezra has been starting to do better and his pain lessening, another issue has cropped up. On Sunday morning, Ezra woke up with a rash on his back and stomach. Any type of rash post-transplant is cause for concern. We headed over to Sloan on Monday to get the rash checked. It doesn't look like anything in particular, so the conclusion is that it could be anything. The rash could be a virus, graft versus host disease, a reaction to medication, an irritation, or a food allergy. The tests for common viruses that can cause rashes have come back negative. We are very concerned about the rash being graft versus host disease ("GVHD"). The good news is that the rash is mild and not itchy. However, the doctors think it may be necessary to do a skin biopsy. We are praying that it starts to clear up so that we can avoid the biopsy. The thought of Ezra going through more pain and trauma is devastating. We are applying a steroidal cream and will wait and see what happens. 

On the cell front, we got back one of the chimerism tests and he is 99.8% donor! There is no concern about the .2% - the test has an error rate of 2%. So this is considered to still be 100% donor. This month another higher level chimerism test was sent, which will look at the different cell lines, rather than whole blood. Now that he has B cells and NK cells, they can look at the specific types of cells to see if they are donor or Ezra. Those results are not yet back. 

The other exciting news around here is that Ezra graduated to a "big, big boy bed," as he calls it. He is super proud of his new bed, which is covered in space bedding (of course), although he isn't excited enough to actually make it through the night in his new bed. 

We continue on the roller coaster ride of transplant. A journey of ups and downs with the hope that ultimately in the end Ezra will finish up. The rash is yet another reminder that we never know what tomorrow will bring and to just focus on on the present. We remain thankful for how far Ezra has come, and remind ourselves how quickly everything can change.

Days +67 to +73: He's Back!

After the past week, we can finally say that the real Ezra is back, albeit not 100%, but most definitely back! Since we started the transplant process and in particular the past month at home, Ezra has been totally consumed by the fog of pain and dulled by pain medications. He was shut down and not interested in anything except sitting on the couch and watching movies or reading books. We sat by him with broken hearts feeling helpless and unable to alleviate his pain. But over the past two weeks, we've watched the real Ezra breaking through the fog and returning to us. It is as if he is rediscovering himself - what it feels like to laugh, to be silly, to be mischievous. His inquisitive mind is back to cranking away, mostly about all things space. It is a blessing to see and we are rejoicing with both laughter and tears.

All this being said, Ezra is still struggling with pain. The pain is slowly lessening, and now it is the real Ezra working through it and not the miserable post-transplant Ezra. He is still taking pain medication, but it is basically on an as needed basis and the need is less frequent. We know there may be setbacks, and we also know this is a very long journey. As long as the path is towards improvement, then that is what matters.

Last week, we were sad because Ezra's hair was not yet growing back and his old hair was falling out. This week, we are happy to say that new little hairs have started to pop out. We can't wait for that curly mop head to be back (although we know that hair sometimes grows back differently post-transplant)!

Ezra's counts have been steadily improving. His platelets have actually been in the normal range for the past few weeks. This is incredible because after the first transplant, Ezra's platelets remained low and never returned to normal. His neutrophils have been solid and B and NK cells have been increasing.

So what's missing? Just the most important type of cell - T cells. With the type of transplant that Ezra had (T  Cell Depleted), it takes on average 4 to 6 months for T cells to appear. We may start seeing some T cells around 3 months post-transplant. He's not quite there yet, but those T cells can't show up soon enough for us. Without T cells, Ezra remains highly susceptible to serious 

infection and will not survive in the long run without them. In a non T Cell Depleted transplant, the recipient receives some T cells with the graft at the time of transplant. These T cells give some protection while the new immune system grows and starts to make new T cells. Ezra does not have the benefit of the protection of those donor T cells in this interim period. So grow T cells, grow, and fast.

Another round of chimerism tests will be sent out this week. And another round of extra anxiety for us waiting for word that those donor cells are still hopefully ruling Ezra's immune system.

Days +59 to +66: Rock Star

Ezra made it through yet another challenge like a rock star on Friday. Although we have seen improvements with his stomach and leg pain, the doctors still really wanted to perform the PET scan because he is still having enough pain that there is reason to be concerned. We were so worried that putting him through another round of testing when he is already traumatized by the hospital would make everything worse. But Ezra never ceases to amaze us and he rocked the test like it was no big deal. There was a lot of back and forth about whether he would need anesthesia in order to get through the test, but we knew once we saw his calm reaction when we explained the test to him the night before that he would be fine. Sure enough, he stayed perfectly still and followed all instructions, despite being very tired from an early wake-up call and very hungry from fasting until 2:00 when the test was finally over. He took the instructions not to move so seriously that he held in his laugh before the test even started when Bert photobombed this picture!  It also helped that the machine was called "Discovery" like his second favorite space shuttle and there was a "countdown clock." Somehow Ezra made it more like a ride than a scary test. The most disturbing part for us was being handed a card that said our son would be radioactive for the next 48 hours. Scary stuff.

Thankfully, the results of the scan did not show anything problematic. The assumption is that the leg pain is from engraftment and deconditioning of the muscles and tendons, and the stomach pain is likely left over irritation from the chemo and mucositis. We are working on getting a physical therapy plan in place. He may have a slow recovery ahead of him, but as long as he moves in the right direction, then we are fine with him taking his sweet time.

We have thankfully seen more improvement. He had a good week with less pain, although some days are better than others. More energy, more playing and more giggles. The other night he ran down the hall yelling "I'm really wired tonight!" Um, yes he certainly was!

We have been able to reduce the oral pain meds and he has tolerated the decrease so far. We will hopefully be able to continue to slowly decrease the pain meds as he improves.

Ezra's hair has been falling out again over the past couple of weeks and has gotten thinner. We are hoping it is a sign that new hair will start to grow soon. With the first transplant, his hair started growing back around this time post-transplant, but no signs of new hair yet. It will be nice to finally watch new hair grow in instead of watching old hair falling out. 

It is also incredible to think that we had just been discharged on this exact day (+66) after the first transplant. This time around, we have already been home for over a month. Although it has been an extremely difficult first month home, we are so grateful to not be in the hospital. We are trying our hardest to keep Ezra healthy in hopes that we can keep him home from here on out.

Days +50 to +58: Roller Coaster Ride

We've reached our fourth week of being at home and Ezra continues to struggle with pain. Although we had seen improvement, Ezra regressed at the beginning of last week and was having more pain. Two steps forward, one step back. Then mid-week he started to do better again. This past Friday evening, all of a sudden, Ezra was back. The real Ezra. The pre-transplant, spirited, energetic, space shuttle launching, chatty Ezra. We exchanged looks with each other, asking "where did this kid come from?" We would have cried if we weren't so busy having fun. We were so hopeful that Saturday morning the real Ezra would still be with us, but sadly the post-transplant Ezra was back. 

It has been an exhausting roller coaster ride. While the years of struggling with Hyper IgM and transplant have taught us to never think ahead and take each day as it comes, it has been very difficult to not be anxious over whether each day will be a "good" day or a "bad" day. 

We spent the past week having discussions with multiple doctors, nurses, pain specialists, psychologists and child life in an effort to get a better plan in place for dealing with Ezra's pain. We decided to wean him off his pain patch, which he has had since discharge.  Lately, we haven't been convinced that it is doing much to help his pain and may be doing more harm than good at this point. We removed the patch yesterday and are following a schedule of oral pain medication to make sure he doesn't go through withdrawal. So far he hasn't shown any symptoms of withdrawal. In fact, he had a great day today, but we can't quite tell if he is doped up from the increased oral pain meds or he is truly feeling better. He was feeling so good today that he asked to go to the park. A quiet playground and gloves on made for good, clean fun for everyone! Pure bliss for Ezra. We are hopeful one less pain medication will help get his stomach back in shape and might bring some of his personality back. 

If he is still having significant pain, then he is scheduled for a PET scan at the end of the week to make sure the doctors aren't missing an issue that needs treatment. We are concerned about putting Ezra through more trauma and more radiation with additional testing, but if he doesn't improve, we need to be sure something more sinister isn't lurking inside.

Counts are still steadily increasing, which is great. We are hoping that all this pain is just his marrow chugging away and rebuilding and will soon subside. And that the real Ezra will return to us soon full time.

 

Days +44 to +49: Liking the Cells, But Not the Pain

First the really, really good news: Ezra's chimerism test shows that he is still 100% donor! We are so relieved and thrilled! After hearing the news, we feel the urge to share this incredible news with Ezra's donor, the person whose cells have found a new home in Ezra's body. For now, we'll just keep saving up our excitement and hugs for some day in the future when we will hopefully meet.

The tests show that Ezra does not yet have T cells, but it is too early for him to have T cells at this stage post-transplant and for the type of transplant he had. He does have B cells and NK cells now, which is exactly what is be expected. So all good on that front.

The other small piece of good news is that we are seeing small improvements in Ezra. All infection tests were negative. He is complaining less about his stomach, and his appetite has been a little better. Leg pain is now his primary complaint and he continues to be on a significant amount of pain medication. The past week brought some improvement. He has been able to play a little more and even ran up our stairs, which he hadn't been able to do the weeks before. He still spends chunks of the day in pain, but we are seeing more glimmers of himself breaking through. 

Clinic visits have still been a challenge. It is clear that Ezra is having post-transplant trauma. After all he has been through, it is no surprise that being back at the hospital is very upsetting for him. Someday we hope visits to Sloan will be only a yearly occurrence, but that day is far away right now, so we are doing the best we can to make the visits tolerable. We are also working with the child life staff to figure out how to make clinic visits less traumatic.

Ezra had a special treat this week - his beloved teacher, Mr. Pete, returned. Ezra had started home pre-school instruction this past Spring, which he loved. We saw the biggest smile spread across his face since we started this process when we said Mr. Pete was coming back. His visits are a very welcome distraction.

Now that we know those wonderful donor cells are still growing inside Ezra, we are even more determined to get him back to a place of good health and strength. Day by day.

Days +38 to +43: No Improvement

Unfortunately, Ezra has not improved as we had hoped. In fact, his pain is worse. It has been a very difficult week. Ezra has spent much of the days screaming and crying in pain from his stomach and legs. It is devastating to watch him suffer. His sweet face is twisted and contorted in pain. There are very few glimmers of our fun-loving, spirited, sweet boy. We keep hoping he will wake up one day feeling better. It has yet to happen. His doctors at Sloan have now realized how severe his pain is and have been running more tests and making adjustments with his meds. 

Yesterday was a particularly difficult day. We were at clinic and the doctor saw the intensity of Ezra's pain, and was concerned. We finally got home after a long day when the the phone rang and it was the doctor saying they wanted some more labs drawn ASAP. They had all conferred and were concerned that Ezra may have an infection in his blood, even though he is fever free and otherwise shows no sign of infection. Instead of having us drive back to the city, it was decided that we would go to our local hospital for the labs. Many, many hours later we finally arrived back home at 11:30 at night with an unhappy Ezra. The tests for blood infection take several days to be final. So far, nothing has showed up. A blood infection would be very bad. We are praying that is not the cause of his pain.

It may be that the pain is from his continuing engraftment. And that we are seeing the cumulative effects of two transplants. We will continue to investigate and hope that the changes in his medications will make a difference. 

On a positive note, his counts have remained steady. Chimerism tests were sent yesterday and we should have the results by the end of this week. Prayers that the suffering is not all for nothing and that growing donor cells are the ones causing the pain.

The overwhelming showing of support for Ezra for tomorrow's Gift of Life Walk for Life has been a bright light in these dark days. The two teams walking and running for Ezra are 60 people strong and over $22,000 has been raised for Gift of Life. We feel so honored and privileged that Ezra's story continues to inspire people to support the need to process more donor test kits so that more children and adults can have a second chance at life. 

We are also so grateful for the gifts that have continued to arrive at our doorstep for Ezra. Lately, they are the only things that bring a fleeting smile to Ezra's face. We received some gifts at the Ronald McDonald House that didn't say who sent them. If you haven't heard from us, please send us an email at help4ezra@gmail.com so we can thank you appropriately! 

We are keeping up the fight to get our Ezra back - and hopefully, it will be a new and improved Ezra with a healthy immune system.

Days +30 to +37: A Hard First Week at Home

Our first week home has been a challenging one. We had hoped that Ezra's pain would magically disappear once we were in the safety and comfort of our home. This has not been the case at all. Ezra is still in a tremendous amount of pain from his stomach and his legs. He  spends most of his days on the couch crying and whimpering in pain. He is still on heavy duty pain medication. The plan for now is to wait and see a little while longer in hopes that he starts to improve. Investigating his stomach pain further will mean invasive testing, and no one is in a rush to do that. His leg pain is likely a combination of his bone marrow hard at work rebuilding and muscular deconditioning from all the time spent in the hospital. We are working on getting him evaluated for PT, and trying to get him doing more physically each day. Other than that, we feel very helpless in our ability to alleviate his pain. Watching him suffer is wearing us all down.

Getting Ezra used to going to clinic again has also been a challenge. As one of his doctors put it, Ezra has a bad case of "hospitalitis." Very true. Who can blame him? He also has been having anxiety about his port being accessed again. He forgot what it feels like after having a central line. By our third visit this past week, he was getting better at it, and hopefully the whole process will begin to be more routine as time goes by.

On a positive note, Ezra's counts have been stable, and he has been able to avoid needing the drug to stimulate neutrophils this past week, which is great news. His platelets and hemoglobin have also been good and he has not needed transfusions in a while. We thank each of you who donated blood or platelets from the bottoms our hearts. You have all played a critical role in Ezra's healing, and we believe there is a little part of all of you inside of him. Please consider donating again in the future as there is still a floor full of other children in need of blood and platelets.

Despite the overwhelming pain, we've gotten Ezra to spend short bursts of time outside in this glorious weather. Watching him rediscover his swing set (a/k/a "Ezra's Park") was joyous. A huge smile spread across his face with that first push of the swing. He was so happy he started humming and singing. It felt like each swing was chipping away at the gray days of the hospital. We watched him approach his slide with trepidation and then rejoiced when he reached the bottom and shouted triumphantly "let's do that again!"

Playtime is short as Ezra is weak. He lost a substantial amount of weight during the transplant process. He is eating, but with the pain he is feeling, his appetite is not back to normal. Transplant patients require more calories to support all the re-building of cells and tissue that has to happen. Between that and his pain, eating is a focus and a challenge. The days are spent getting Ezra to eat, take his meds, and attempting to alleviate his pain as much as we can.

Chimerism will be checked again soon - likely this week - so please pray that he remains 100% donor cells. And that he gets some relief soon from the pain.

Days +26 to +29: We Are Home!

To cheers and a standing ovation, Ezra Jordan Fineman, for the second time in his young life, slowly walked himself out of the pediatric bone marrow transplant unit at Sloan-Kettering yesterday. Yes, we are home! We've known for about a week that discharge was on the horizon, but we had no idea of the timeframe the doctors had in mind, and it was all day by day depending on how Ezra was doing. Then suddenly it was you're out of here, pack your bags, adios! In the past few days, Ezra turned a corner and his appetite kicked in and he started eating more, drinking lots and maintaining his electrolytes without IV fluids. That was the sign the doctors were looking for to know he will be ok at home. So here we are.

We still can't believe we're home. Last year we weren't discharged until Day +66. This time it is Day +28. A world of difference.

Discharge day was busy and chaotic with last minute meds and packing. And then came the part we were most nervous about - Ezra needed his central line pulled out. We will now use his mediport for labs and medications. Ezra was traumatized by the last incident when the line broke and has been having nightmares about his central line, so we were very concerned about doing it again. But with the help of his fabulous friend Jessica from child life, he did amazingly well. Please, please let this be the last time we ever have to think about a central line. 

When we first arrived home, Ezra was in a fog of pain, but slowly the fact that he was home settled in and we watched with joy as he ran from toy to toy as if we had let him loose in a toy store. The house feels so bright and clean and the air outside smells beyond fresh. The best part is the quiet - no beeping pumps, vitals all night long, or linens collectors slamming the door open early in the morning. Ah, home.

We still have a not-so-healthy child on our hands. Ezra has only the beginnings of an immune system and remains extremely susceptible to infection. His counts have been low lately and dependent on the drug that stimulates neutrophils. He is also still in pain from his stomach and legs. He remains on pain medication and the hope is once we are settled at home, the pain will begin to subside and we can wean him off the pain meds. 

Ezra will be watched very closely, so closely that we are going back to the hospital tomorrow for a counts check and any necessary meds. Then we will be at clinic at least three times a week. At the first sign of fever or any other issue, he will be re-admitted. Hectic, but we'll take anything as long as we can sleep in our own beds! 

We are grateful to the doctors who have successfully gotten Ezra this far and to the fantastic nurses of M9. Ezra's nurses can best be described as caring, compassionate and creative, with a sprinkle of silliness. They did an amazing job caring for Ezra last year, but this time around, the care was even more seamless because they knew Ezra and his particular ways. They got him (and us) through very tough days in the best possible ways and even made him laugh. The fact that they clearly earned Ezra's trust says it all. We hope to see you again only on the outpatient side!

Last year our homecoming was filled with fear and disappointment as we knew the transplant did not go well. This time, we are allowing ourselves a little bit of hope that things will continue to go well, and in the upcoming months we will see new healthy T cells. As we were sitting in traffic on the FDR on the way home, Ezra was chatting away about all the landmarks he loves to look at during our trips back and forth to the hospital, and he started talking about the bat outside Yankees Stadium. For the first time, we thought maybe, just maybe, he will get to go to a baseball game in the future. Maybe not this year, but it no longer feels like never.

We will continue to keep updating as we enter the next phase of this very long journey. Please continue to pray that Ezra remains infection free, that no GVHD develops, and that his new immune system continues to grow. Thank you to everyone for your support - you have carried us through these dreadful days and we know you are behind us as we continue the fight for Ezra's cure.

Days +22 to +25: Baby Steps

The past few days have been filled with more ups and downs. Last Friday night, the transplant team decided to stop his IV nutrition, which is ultimately a good thing because Ezra has to be eating and drinking on his own in order to go home. The transition to oral intake, however, is difficult and there is a lot of pressure for him to do a lot of drinking (the medical goal is way more than you would expect for a 4 year old) and eating when he still doesn't feel well. The weekend was very rough, and although Ezra was trying, his stomach did not seem to be ready to return to eating and drinking. 

Thankfully, yesterday he seemed to have turned a corner and was able to eat small amounts of food and keep it down. He has also been drinking as much as he can. Hopefully, we are on the way to getting back to a normal diet, as long as that tummy cooperates. Always the trooper, always the fighter.

Ezra's counts are still bouncing around quite a bit. We understand that this is normal at this stage, but it still makes us nervous. Ezra is still very early in this process and we're in it for the long run, but given our prior experience we are grasping for as much reassurance as possible.

We're feeling some sadness that Ezra will be missing sukkot this year. He loves being outside in our sukkah. We are just hoping that this is the last sukkot spent in the hospital.

September is childhood cancer awareness month, and the subject has been on our minds. While Sloan treats diseases of many different types, the majority of patients are cancer patients. Sometimes it is so crowded here that you start to think that every child has cancer. Certainly in the abnormal world we live in, we see more sick children than healthy ones. We have witnessed miracles happening here, and we have seen the devastation caused by pediatric cancer. When a medical code is called for the Peds floor, our hearts sink and we try to pretend that we don't hear the doctors and nurses running down the hall and the machines ringing, and we try to think that everything is ok, when really nothing is.

Some of you may know the shocking statistic that only 4% of federal cancer funding goes to pediatric cancers. Children aren't just mini adults and shouldn't be treated that way. Their bodies react differently and treatments should be specific to pediatric cancer, and not just shrunk down versions of treatments for adults. There are many excellent charities that fund pediatric cancer research. We don't have a favorite one, but certainly Sloan-Kettering is one of the places making great strides in the treatment of pediatric cancer.

One of the many things we have never figured out about this place is why there are only plastic spoons in the pantry. You can certainly get utensils from food service, but many families store food in the pantry refrigerators for their child or themselves. It is obviously very difficult to prepare food with just a spoon. And when your sick child wants something to eat pronto, you don't want to wait on food service. So, we decided to take matters into our own hands and bought a case of 1000 individually wrapped plastic forks from Amazon and have secretly deposited a batch in the pantry and will continue to refill. Our little contribution to making life slightly easier around here. Shhhh...don't tell!

Wishing everyone a chag sameach!

Days +19 to +21: Ups and Downs

As we start week 3 post-transplant and as Yom Kippur is upon us, we are feeling so much gratitude. We are thankful that, so far, transplant number two is going as well as we could ask for at this point. We are also so grateful for all the family, friends and complete strangers who have been supporting us through this journey and keeping us strong so that we can be strong for Ezra. All of the delicious meals and special treats delivered to us, the fun gifts for Ezra, and the messages and prayers for better days and good health are always what puts a smile on our faces and helps us to stay positive. So thank you all so very much.

The focus of this week has been re-introducing food and drink as part of Ezra's life. It seems like it is easy to forget about food when you don't feel well and your body gets nutrition support intravenously. Ezra is very slowly trying more bites of food in small amounts. He is definitely scared that eating will make him throw up, plus he is still having stomach pain, so eating is no easy feat at this point. He can take his time as long as we keep taking baby steps forward.

Ezra's counts have been bouncing around quite a bit. He has some days where his WBC is low, although his neutrophils have remained in the normal range. When his counts are low, he has been receiving a dose of a drug that stimulates neutrophil production because the doctors want to keep his counts on the higher end at this point to keep him protected. The only downside of that drug is that it causes Ezra to have bone pain, which in turn means that he needs more pain meds. We need to start weaning him off the pain medication, but haven't been able to make much progress.

The other issue that has cropped up is that he is having diarrhea again. So far the tests are coming back negative for any bacteria, parasites, etc. For now, the belief is that the diarrhea is likely from a combination of the re-introduction of food and possibly withdrawal symptoms from the small amount of weaning we have done on the pain meds. The plan is to give him more time and see what happens. 

The one area of definite improvement is in Ezra's energy. He has been more active and playful the past few days. He has been walking around more and even had a dance party (check Ezra's Facebook page for a video clip www.facebook.com/help4ezra). He is also working hard at PT and OT to regain some strength. 

G'mar chatima tova!

Days +16 to +18: The Hard Work of Healing

Each day we have been seeing a little more improvement in how Ezra feels. His biggest complaints are still stomach and bone pain. Today he was the most playful he has been since we started this process. It is so wonderful to see his personality coming back (and it's more fun too!). He had not been willing to participate in the services and activities available here for the kids when he was feeling so sick, but today he finally allowed the music therapists to come in and jam. 

Ezra's counts have continued to be strong. The transplant team's focus has definitely shifted toward what has to be accomplished in order for Ezra to be discharged. Several medications have been stopped or are being administered less frequently. The pump is slowly decreasing in size. We also started giving one medication orally. So far, Ezra is taking it like a champ. 

For Ezra, the big challenge will be learning to eat and drink again. He needs  to be eating and drinking fairly normally before discharge can happen. He is still on IV nutrition. Once he is able to eat and drink a little on his own, they will stop the TPN, and then hopefully his appetite will kick in. Chemo affects the taste buds, and we can tell that foods taste and smell differently to him. He also says he is afraid to eat because it will make him throw up. We are starting very slowly with this process. Small bites of chicken soup have gone down ok so far and we will go from there.

We had a short and furry visitor this morning. Believe it or not, the hospital uses beagles to sniff out bed bugs, mites, and other nasty stuff. We were shocked that a dog is allowed in the isolation rooms, but apparently it is worth it to have a beagle's expert sniffing. Ezra slept through the visit and we were glad the cleaning staff came by right after our cute, but not-so-sanitary visitor left. 

We are praying that things continue on the right track. To think that discharge might be in the near future seems too good to be true. We're trying to not think ahead too far because we know things can change in a flash. We are just taking each day as it comes and hoping the next will be better. 

Days +12 to +15: New Year, New Life

First piece of good news: Ezra officially engrafted as of Thursday. The first day of neutrophils over .5 is considered to be the day of engraftment, so Ezra engrafted on Day +11, which happens to be the exact day the doctors predicted based on the size of his graft. Amazing. Ezra's first failed transplant took about 35 days to engraft. A world of difference this time around.

Second glorious piece of news: the new cells are 100% donor! 100, 100, 100! The chimerism test results came back quicker than expected. Tremendous relief and tears of joy. A moment we will always remember. Let it blot out the memory from last year of devastating news. New year, new life. Welcome donor cells. Please don't ever leave!

We wish we could thank Ezra's miracle donor - the reason this is all happening. The rules require that we wait one year before contact and then only if both donor and recipient mutually agree. What a gift she has given us. The gift of hope; the gift of life for Ezra and for us all. For now, we will just have to send our gratitude out into the world and hope she somehow hears us.

We know that there is so much left to this journey. The most important type of cell for Ezra's condition are T cells, and those cells won't begin to make an appearance until 4 to 6 months from now. Those are the cells that need to be donor cells in order for Ezra to be cured. Even though he is all donor now, the T cells could still possibly be mixed between Ezra and donor, so that is something that will continue to worry us. Ultimately, Ezra's immune system has to completely reconstitute and have the ability to make antibodies, which could take a year or more.

This process feels like a marathon with hurdles every few miles that need to be overcome to reach that far off finish line. For now, we rejoice in the hurdles that Ezra has soared over in this race for a cure.

Best of all, we are finally starting to see some small improvements in how Ezra feels. The holiday began with a fever scare. Ezra's fever was not going away and the doctors were getting very concerned. Ezra was sent off for a CT scan to check for pneumonia and other signs of infection. The next step if things didn't improve was to pull out his central line in case that was the cause of infection. Panic time. Thankfully, his fever started to go down and he has been fever free for three days now. 

We have also seen glimpses of his personality coming back. It's like watching your child be reborn. The little chatterbox is slowly appearing again. Yesterday, he wouldn't let the doctor leave the room until he told him about every rocket ever created. And after his CT scan, the doctor came in to ask if Ezra was smiling during the scan because it looked that way in that results. No, he wasn't smiling - he was pretending to snore because the technician had told him to close his eyes. This child never ceases to amaze.

The focus right now is getting Ezra to a place of better health. Intense stomach and bone pain are his biggest issues right now. He needs to do a lot of healing, but we're confident that we will get him there soon enough.

So wherever you may be tonight, raise a glass and toast to Ezra's good news and join us in hoping for better days ahead in the new year and years to come.

Days +10 and +11: Hello Neutrophils

Yesterday, Ezra's white blood cell count went to .2. A little movement. Today, his white blood cell count was 1.5 - a big jump and high enough to run a differential and to see neutrophils! His neutrophil count today is .8 (800). To officially engraft, you need a neutrophil count of .5 or above for 3 consecutive days. Day 1in the bag.

The next step is the one making us very nervous because it is when everything went downhill last time - chimerism. Once Ezra achieves engraftment, a blood test will be done to see whether the cells growing are Ezra's cells or the donor's cells. Last year at this stage, Ezra's chimerism showed only 20% donor cells and they continued to diminish as the months went on. If the neutrophils continue to grow in the right direction, then we are hoping this test will be sent out by the end of the week, and then we anxiously await the results.

This next phase also brings the possibility of Graft Versus Host Disease and continued risk of infection both from outside and from latent viruses that can be passed from the donor through the graft. These viruses can activate once inside of the immune compromised recipient and cause big problems.

Unfortunately, the appearance of the neutrophils has not yet improved how Ezra feels. Yesterday was the worst day he has had so far - intense pain all throughout his body. This afternoon was a little better. He also has been dealing with a fever for the past two days. It may just be part of engraftment, but the doctors are treating it as an infection. We are waiting for cultures to come back and hoping he clears it soon.

With Rosh Hashanah upon us, we are praying that this year will bring Ezra's cure and an end to his life of seclusion and hospital visits. For each of the four past Rosh Hashanahs since Ezra's diagnosis at five months old, we wondered would this be a year of good health? Of illness? Of life or of death? Each year we have prayed that the coming year would bring life and health for our baby boy. But never have the prayers that he be inscribed and sealed in the book of life felt so imminent and critical. Please let this be the year of the cure. Of health and of life.

This will likely be our last post until after Saturday night when the holiday is over. We hope our next post will have good news of engraftment and better days for Ezra. We wish you all a l'shana tova!

Days +6 to +9: No Relief Yet

Ezra continues to be in tremendous pain. We can now add excruciating leg and arm pain to the list of complaints. The doctors suspect that the pain may be bone pain from the drug he started on Friday that stimulates the bone marrow to release neutrophils into the blood, and may be a sign that cells are growing. If that's the case, we'll take it, despite how awful it is to watch him suffer. Gotta get worse before you get better seems to be the theme. We are anxiously awaiting a change in his counts. 

Ezra spent most of the weekend sleeping. It is much better that way. Let him sleep until better days are here. He has had a few lucid moments where the little chatterbox has re-appeared, like in the middle of the night when he decided he had 20 questions about Daddy's bicycles ("Daddy, how often do you ride your bike?" "Ok, that's good. How often do you ride your road bike?" How often do you ride your mountain bike?" Which bike do you buy tires for?" And so on.).

We hope that everyone is enjoying Labor Day weekend. Food services dropped off the Labor Day breakfast specials - what, no BBQ? We will save our celebrating for when we see signs of new life. Grow cells, grow.

Update on Blood and Platelet Donations

We wanted to share with you the words of the Sloan-Kettering blood bank about the donations that have been coming in for Ezra: "The fact is that we have been inundated with calls...You have a wonderfully generous community...This has been an amazing effort with many young new donors." And they don't even know the half of what Ezra's supporters have done for him! We are blown away by this outpouring of love, support and generosity.

While the donor room has been flooded the past few weeks, there aren't as many donations scheduled into September. Platelets have a shelf life of approximately 3 days and blood about one month. Ezra will be needing platelets and blood for the foreseeable future, so please consider donating in the upcoming months. We know the Jewish holidays will make it difficult for some of you to donate in September, so we greatly appreciate all efforts!

Also, as a reminder, you can be any blood type to donate platelets. For whole blood, Ezra is O+ , but if you prefer to donate whole blood, there are many other adorable little patients around here needing blood, so all blood types are accepted!

The information on how to donate is repeated below:

IMPORTANT: Family members related BY BLOOD on either side can NOT donate.

From the Sloan Blood Bank:

EZRA FINEMAN Needs Blood & Platelets

4 year old Ezra is currently a patient at Memorial Sloan-Kettering Cancer Center in New York City. His treatment will require regular blood and platelet transfusions.  Ezra's blood type is currently O+.  For platelets, you can beANY blood type. 

Ezra and his family would deeply appreciate your donation of blood and/or platelets and requests you ask others you know to donate. Donations not used by Ezra will be released for use by other patients many of whom are children.

To benefit Ezra all designated donations must be made in the Blood Donor Room of Memorial Sloan-Kettering Cancer Center

Please visit www.mskcc.org/blooddonations for complete information about FDA donor eligibility and the donation process for blood or platelets.

For answers to questions and to schedule an appointment that is convenient for you please Contact:      

Mary Thomas  -   212-639-3335

Donor Relations Specialist

thomasfm@mskcc.org

The Blood Donor Room –

1250 First Avenue (between 67th/68th Streets) NYC - Schwartz Building lobby

Open Every Day

Fri Sat Sun Mon     8:30am - 3:00pm

Tues Wed Th         8:30am - 7:00pm

212-639-7648

 

The process for donating whole blood takes approximately 1 hour.

The process for donating platelets takes about 2 ½ hours.

 

Appointments are necessary.

 

FREE Donor Parking -

-Somerset Parking Garage, 1365 York Avenue –entrance on 72nd Street, N.W.corner