Day +96: Mediport surgery

After a very long day, Ezra is home recovering from the mediport surgery. The surgery was almost called off right before he went into the OR by the surgeon who had concerns over Ezra's platelets. After much delay, the transplant team and the surgeon agreed to proceed with the surgery. Our brave little trooper is in a lot of pain. There was some debate over admitting him, but thankfully he was discharged this evening. We are hoping that the pain lessens by the end of the week and that he heals with no major issues. Another week, another challenge for our rock star.

Day +93: Three Long Months

Today marks three months from Ezra's transplant. He had a clinic visit today and the doctor was talking about how well he is doing. No infections, no graft versus host disease. He's eating and drinking well - even trying new foods (case in point, here's a picture of him trying pizza for the first time-finally!). He has energy and his labs look ok. Basically, we are back to where he was before transplant except that his immune system is in worse shape. His neutrophils still aren't as high has they need to be and he is still not making platelets and remains dependent on transfusions.

We are extremely grateful that Ezra is doing so well at this point. If the transplant was failing and he was sick, this would be an entirely different situation. The plan is still to just taper off the immunosuppressants and see if that makes a difference in the number of donor cells. They are tapering these drugs very, very slowly because, as one doctor explained, you can get graft versus host disease from just 1% donor cells and once you get it, there's no turning back. They won't check for donor chimerism again until after he is off these drugs.

Ezra is scheduled for surgery on Monday to have a mediport implanted. He still has his temporary central line, which can't stay much longer. The mediport goes under the skin and is accessed with a needle from the outside. The area will be numbed beforehand each time it is accessed. It is much less intrusive than the central line, has less infection risk and will allow Ezra to take baths. We know it will take some getting used to for him, but child life has armed us with lots of resources to try and explain this new "button" that will be under his skin. The surgery is more invasive than the procedure for the temporary line and requires more time under anesthesia. We are expecting him to have some pain for a while afterwards, but are hopeful that the mediport will ultimately allow him more freedom. We will let everyone know how he is doing after surgery.

And please keep up the prayers and positive thoughts for those donor cells to grow. We are still hoping for a miracle.

Day +86: Falling

Originally, Ezra's doctor had said that she wanted to wait a while before re-testing his chimerism. She changed her mind and re-ran the test at the end of last week. The results now show that Ezra has only 3% donor cells.

The plan for now is to slowly taper off his immunosuppressive drugs. These drugs are used to prevent graft versus host disease, but they work by suppressing the immune system. The thinking is that the donor cells may be able to grow without these drugs interfering. But the same is true for his old immune system that is right now winning this battle. Ezra's old cells will also enjoy the benefit of no immunospressive drugs.

If there is a battle going on inside him, the underdog is struggling. His doctor says that until the tests say he has no donor cells, she believes the donor cells can still grow. She says she has seen this happen with other patients. But we don't need medical degrees to know this is highly unlikely. If these donor cells are going to win, it will take a miracle. They will re-test the chimerism after the immunosuppressive drugs are tapered off

Words don't do justice to how we feel. Susan, a friend whose little boy also went through transplant for Hyper IgM, gave us this wise advice: Deal with what is happening today as that is the only thing that is real. We are trying to do that, but at some point soon, Plan B, if there is a Plan B, is going to have to be figured out. So many of you constantly ask what you can do. For now, all that can be done is to pray and send positive thoughts for those few donor cells to grow.

One positive note: Ezra was negative for parainfluenza virus today. So some part of that broken immune system is doing something.

Day +78: Sick time, Swing time

Ezra has had a cough for the past week or so. Ends up that he has parainfluenza virus - basically a bad cold virus. There's not much to do about it, except watch him closely to make sure it doesn't get worse. So far, he's holding steady and we hope it stays that way. Mommy also presumably has the same thing, so it's sniffles all around in the Fineman house.

Ezra's donor chimerism will not be tested again for a couple of weeks. The doctors want to give it more time. We will keep everyone posted.

On a fun note, Ezra now has his very own swingset. One of Ezra's favorite activities pre-transplant was going to the park. In the rain, in the snow, and sometimes twice a day Ezra went to the park. For now, public playgrounds are off limits. We were/are afraid that he will be heartbroken over not being able to go to his beloved park. So, in an effort to distract him from what he is missing, we bought him a swingset for our backyard. The swingset came while we were still in the hospital - the issue was finding the time to construct it with our crazy lives. Our wonderful friends Lisa and Brian solved that problem for us. They arranged for Bonim Builders (basically a Jewish Habitat for Humanity) to send an incredible team of volunteers over to build Ezra's swingset. With those volunteers, our super handy friend Joe (Ezra is still saying "Joe is in charge"), and Daddy's persistence through a thunderstorm, Ezra has his dream swingset. He is thrilled. He has also caught on to our intention of providing a substitute park. He promptly declared his swingset "Ezra's Park" and wants a sign for his park. Once again, we couldn't have done it without everyone's support. We continue to be truly touched by all the different ways that people have reached out to help us through this scary roller coaster time of our lives.

Day +72: Headed in a Very Wrong Direction

The results of one of the repeat engraftment studies are in and the donor cells have dropped to only 8%. The doctors are saying that the percentage might still go up and that it is too early to rely on these results. They will repeat the tests in a few weeks. This gives us little comfort. The doctors here are saying that we need to wait and they wouldn't do another transplant until he is 6 months post-transplant because of the toxicity from the chemo conditioning.

The thought of going through another transplant after we just got discharged is unbearable. And Ezra's back-up transplant options are not good. We are going to start talking to the other transplant centers that we had previously considered, gather opinions and go from there. In the meantime, we can only hope that next month's testing will show more donor cells.

For now, this is our burden to bear. Ezra is so happy to be home. He doesn't stay still for a moment and runs from toy to toy all day. He is being really good about getting up early to travel back to the hospital on clinic days. We are struggling to get used to the new "norm" of chasing him around with meds, drinks and food all day long. He also has to take meds during the night, so none of us are getting enough rest. We are still happy to take this crazy schedule over being inpatient, but the latest bad news changes everything.

Some of you have asked if Ezra still needs blood and platelets. Yes, he is still requiring platelets on a regular basis. You can be any blood type to donate platelets. He is starting to hold blood a bit longer, but is still transfusion dependent. He is still O+ blood type. Please contact Mary Thomas at 212-639-3335 to schedule an appointment.

Last, this latest news renews the need for Ezra to find a better source of stem cells if he needs another transplant. If he were to find a perfect bone marrow match, it would be a miracle. We spent two years searching for a match for him to no avail. But there's always the chance for a miracle match to appear. Please register as a donor, encourage your friends to become donors or make a donation to cover the cost of processing a donor test kit so someone else can be a donor at www.giftoflife.org/help4ezra.

Thank you all for the love and support.

Day +66: HOME

Home. There are no words that can adequately describe how sweet and wonderful it is to be home. Ezra is beyond excited. The expression on his face when we pulled up to the house was a mixture of joy, amazement and a little hesitation. He ran around outside for a bit, checking out the backyard, the deck, his basketball hoop. Then he spied his toys through the window and he ran inside. He spent the rest of the day running around re-discovering all his toys. It was amazing and also a bit heartbreaking to watch him reacquaint himself with his home.

We can't get over how quiet it is here - no beeping pumps, doors opening and closing, linen drawer slamming, vital signs checks, screaming children. And there is so much space to walk around. And a table to sit at to eat. The simple things.

It was 78 days ago on an early Monday morning that we packed up and said goodbye to our home. We all had tears in our eyes, including Ezra who knew that this time, the trip to the hospital was different. As we shut the door, we did not know if this family of three would return as a family three. We did not know what challenges were ahead of us and we still do not know what we face. We are grateful for so many things: that Ezra is doing well; that he hasn't had an infection; that he has strength and energy; that he is taking his meds, eating and drinking.

We are thrilled to be home, but it is without a sense of relief or confidence that we've accomplished the cure for Ezra that we were hoping for when we left our home on May 7. Will this all have been for nothing? Will the donor cells grow? Will his old dysfunctional immune system grow back? Will nothing grow back? Will he need another transplant in a few months? These are our questions and we have no answers.

Ezra's health is still in a very precarious state. His neutrophils are not holding and he is dependent on a drug to stimulate growth. He is still at high risk for graft versus host disease. He is also still dependent on blood and platelet transfusions. He still has very little immune function and his immune system is in the early stages of regrowth. He will remain essentially in isolation at home for now.

We will be back at the hospital either Monday or Tuesday and will continue to travel back to Sloan 2 to 3 times a week, and possibly more as needed. He will have labs drawn, be seen by the doctors and receive any needed transfusions or medications. At the sign of the slightest complication, he will be re-adimitted.

Through it all, what has kept us going is the love and support of our family, friends and our new family of strangers. Whoever heard of complete strangers sending gifts, dropping off treats and meals, donating blood, saying prayers all because they heard about a little boy named Ezra who wasn't feeling well? We are touched by everyone's kindness and support and appreciate every note, post and comment sent to us. One day we hope to tell Ezra about how he touched so many people and how those people helped him get better.

We will continue to keep everyone posted as Ezra enters this new phase of his journey toward being cured.

Days + 57 to +61: Getting Closer

Yes, we're still here. The very tentative plan for discharge is Friday. The main issue that is keeping us here is that one of the drugs that Ezra takes for graft versus host disease needs to reach a certain level in the blood for it to be effective. The level has finally started to go up after several dose increases, but it still is not quite where they want it. The doctors have called Ezra a "Super Metabolizer." Not exactly an area where we want him to be an overachiever. Maybe this explains why we've always had trouble getting him to gain weight. In any event, now that the level has gone up a little, his doctor is willing to discuss discharge for later this week.

If all goes well, Ezra will be discharged and will continue his recovery on an outpatient basis. He will need to come to clinic here several times a week for long days of testing, medication and transfusions. This schedule will continue for many months. And at the slightest sign of anything problematic, he will need to be re-admitted. But at least we will be going home in between and that's all that matters. Ezra will also need to remain in isolation at home for a long time, but should be allowed outside to play at our house (swingset has been ordered!). We are waiting to learn the specific instructions and limitations.

Leaving can't come soon enough for Ezra. While he remains an amazing trooper stronger than any of us, he is really getting cabin fever now that he is feeling better. One of his obsessions is Rube Goldberg machines (really complicated machines that accomplish a simple task). He watches videos of Rube Goldberg machines on You Tube and he has now taken to creating his own, which basically consists of setting objects up to knock down. All. Day. Long. Time to go. At least he got a visit from Cookie Monster today (well, through the door) and Ezra slipped him a cookie under the door so that they could munch together.