Day -9: Admission and Line Placement

Today is considered to be Day -9. The days are counted negative until Day Zero, which is transplant day. Then we start to count up, and hopefully up and up and never again back to a negative number.

Today was difficult, but in some ways easier to manage because we are on familiar territory with familiar faces. After a long morning of bouncing around from exam room to exam room, Ezra had his central line surgery. The line thankfully was placed correctly. This was Ezra's fourth central line surgery, not including his port surgery. Four years old and a chest full of scars - the chest of a fighter. Ezra still has his port, but for the many medications of transplant a central line with three access points is needed.

Ezra had a lot of pain from the surgery, but the pain meds are helping. We are hoping that he will feel better tomorrow after the huge pressure dressing is removed. He also seems to be accepting that the line is bak, better than this morning when it was all tears over the thought of the central line returning.

After surgery, Ezra was admitted to his room. Thankfully, we got a sunny room with a view of Ezra's beloved smoke stacks and the Queensboro bridge. Ezra is excited that he gets to have a sleepover with his pals Bert and Ernie. Dr. Ezra is back and taking new patients. 

Chemo starts tomorrow. The goal of the chemo is to kill off his current faulty immune system and make room in the marrow for the donor's healthy stem cells. It will likely take a few days before the side effects of the chemo start to kick in. 

It is still hard to believe that we are back here starting all over. We are keeping our eyes and hearts on the goal - a healthy Ezra.

This fortune was found in Ezra's cookie the other night. Let's hope this one comes true.

Transplant Take Two

On Wednesday, Ezra will be re-admitted to Memorial Sloan-Kettering for a second attempt at transplant. The good news is that several months ago, a partially matched donor for Ezra showed up in the registry. The bad news is that the donor is only an 8/10 match. When looking for a donor, the doctors look at 10 antigens. A perfect match is a 10/10. Any mismatch away from that perfect 10 brings significant risks for the patient. However, this is the first time that any donor that the doctors would even consider using for a transplant for Ezra has appeared in the registry and that is a big deal.

Due to the degree and type of mismatch, the doctors will use a method called T-cell depletion. The cells will be collected from the donor, and then the lab will process them through a machine that removes the T cells. The T cells are the cells that cause Graft Versus Host Disease ("GVHD"). If Ezra was to receive the cells from the donor without the T cells being removed, he would almost certainly develop GVHD and it would be severe. T cell depletion significantly decreases the risk of GVHD, but it comes with a cost. The T cells that usually come in with a graft provide protection from infection during the period when the patient's new immune system is growing back. Ezra won't have that protection. It will take 4-6 months for new T cells to appear and during that time period Ezra will be at an even greater risk for infection than he would be typically post-transplant. Sloan-Kettering has more experience with T-cell depletion than any other center in the U.S., so Sloan is the best place for this type of transplant.

Whether to proceed with a second, risky transplant has been a devastating decision and one that we have made with heavy hearts. Ezra is automatically at higher risk because this will be his second transplant and his second exposure to high dose chemotherapy. In the middle of our decision making, Ezra's primary doctor at Sloan since he was an infant suddenly passed away, which threw us for a loop. The chair of the department has taken over Ezra's care and we have gained comfort with him. In the end, transplant is the only path that may lead to a long, healthy life without - as one doctor told us - a sword hanging over his head.

If the transplant works, the sword will disappear and Ezra can lead a normal life. He can no longer live this life of seclusion. It is time for Ezra to be able to go to school, play dates, birthday parties - all the normal experiences a child shouldn't think twice about. Enough is enough of this limited life and it is time to make Hyper IgM a thing of the past.

Thanks to the hard work of special friends of ours, Ezra had some very special opportunities over the past months to break out of his limited world. In addition to the NY Hall of Science, Ezra had the chance to visit Liberty Science Center, the Cradle of Aviation and the Museum of Natural History - all after hours (think Night at the Museum). Two weeks ago, Ezra had the incredible opportunity to have a private tour of the Intrepid and the space shuttle Enterprise. To say he was thrilled is an understatement. We are so grateful to all of these institutions for making Ezra's dreams come true and for providing our family with special memories that will carry us through the hard times.

We are so, so grateful that after all these years of searching, a donor has appeared. All we know is that the donor is a female and she is around 20 years old. And that she has been very cooperative with all that has been asked of her so far. We love her already.

We are mustering up all our strength in order to march our son back into the gray prison of the hospital. It was with  indescribable sadness that we broke the news to Ezra. The hardest part, as we suspected, was the news that he will need a central line again. The line caused him a lot of pain last time and dressing changes were traumatic. The surgery for the line will be the first thing to happen on Wednesday. Chemo starts the next day.

Through the months of isolation last year what kept us going were the messages of support, the hundreds of people who donated blood and platelets for Ezra, the meals and treats that were sent with love, the generous friends who paid for our room at the Ronald McDonald house, the gifts sent to occupy Ezra, and the prayers and kind thoughts from people all over the world. They were all reminders that we aren't so alone in this journey. One thing we know this time is that we will not be marching into the hospital alone.

We will update this blog and Ezra's Facebook page (www.facebook.com/help4ezra). Please feel free to share Ezra's story. He needs as many prayers as he can get. His Hebrew name is Ezra Aviel ben Hertzelia Shandel. Please keep him in your prayers and thoughts.

With love,
Robin, Evan and Ezra

Match Number 100!

Today we celebrate our 100th donor match through Ezra's Gift of Life donor circle (www.giftoflife.org/help4ezra)! The 100th donor match is for a 35 year old woman suffering from ALL. This is an incredible accomplishment and one that everyone should be very proud of. Our ability to find matches for so many other patients is due to the support of the thousands of people who have registered, given donations and/or held drives for Ezra and other patients like him. While we continue to hope for Ezra's perfect match, it brings us much comfort to know that we have helped other patients and their families find hope in the possibility of a cure. We pray that one day no patient should ever have to search for a match and that the miracle of a cure will be available to all patients in need. 

It has been a while since we have updated this blog. Ezra has been doing well on his medical treatments. He continues to gain weight and grow, better than ever before. He started in-home pre-school instruction through our local school district and is enjoying his lessons with his teacher very much. Ezra is still largely under isolation restrictions, but with the warmer weather, we have been able to enjoy more outside activities. We will have a more detailed medical update soon, but for now, things are stable, and we are grateful for that.  

Thank you again to everyone who has supported our efforts to find a match for Ezra and others over these past 3 years. It has been a privilege and an honor to help others find their miracle matches and we hope that you all share the joy of this amazing accomplishment with us.

Peter Yarrow Benefit Concert

As some of you may recall, Ezra's all-time favorite song is Puff the Magic Dragon by Peter Yarrow of Peter, Paul and Mary. Since he was a baby, we've rocked him to sleep singing Puff, and if we dared to sing something else, we were promptly reprimanded with a nudge or a cry. Once he could strum a guitar, Puff was his favorite tune to play. And when he was in the hospital during transplant, he would belt out Puff, swinging his IV lines to the beat, while the music therapists played along.

Well, Peter Yarrow learned about Ezra's love of all things Puff (and other PP&M songs) from his grandparents at a recent book signing. When Peter heard about Ezra's situation, he immediately volunteered to hold a benefit concert to raise funds for Gift of Life in hopes of finding Ezra's match.

Thanks to Peter's generosity, An Afternoon with Peter Yarrow Benefit Concert for Gift of Life will be held on Sunday, May 19 at 1:00 PM at the Highline Ballroom in NYC. This will be a child-friendly concert and we hope that you will come out with your children and grandchildren to enjoy a great concert for a critically important cause.

Tickets can be purchased through the Highline website at http://highlineballroom.com/show/2013/05/19/peter-yarrow-of-peter-paul-and-mary/

An anonymous donor has offered to match the first $5,000 in ticket sales, so please buy your tickets soon! We are very grateful to the Highline Ballroom for donating such an incredible space for the concert.

If you belong to a community organization or other group where you can share the information on the concert, please help us spread the word. If you need any additional information, please contact Ruth Miller at rmiller@giftoflife.org.

We hope to see you at this fun event!

Recap of Ezra's 4th Birthday!

We had the pleasure of celebrating Ezra's 4th Birthday this weekend. We are having a hard time believing he is 4 years old (Ezra seems to be having trouble believing it too - before going to bed on the night of his birthday he asked if he would still be 4 the next day). He really seems to have grown and matured this year. After all he has been through and continues to go through, he often seems older than his four years. For his birthday, we'll allow ourselves the indulgence of a little gushing over our little man.

One thing is certain: these have been the best four years of our lives because we have been blessed with a loving, laughing, caring, sweet and creative soul who covers us with kisses everyday. We are so proud of our little fighter. What 3 year old never asks to leave the confines of his small hospital room? Never once asks to go home? Accepts that he has a battle to win, a mission to accomplish - one that he barely understands? We dread the day when we have to explain that the process has failed him; the cure has failed to happen; we've failed him; and that all his brave fighting has been for nothing.

Day after day we continue to be amazed at the way he finds joy in every difficult situation. Whether it be belly laughing at the guy that accidentally crashed his cart into another cart in the hospital hall or concocting a marble run out of oxygen tubing in a tiny clinic room, Ezra always finds something that makes him jump up and down with excitement and passion. Before we started the transplant process we feared that being in pain and in isolation for endless months would break his spirit. Ezra has proved us very wrong. Our hearts burst with pride over how Ezra lives and loves life.

Back to the birthday details. Friday was his actual birthday and we celebrated by playing in the snow from the surprise storm. Then on Sunday we we had a very small adults-only birthday party. We had a presentation from Mad Science, which Ezra loved. The best part was the four model rockets Ezra got to launch in our backyard. He watches model rocket launches on YouTube all the time, so this was a dream come true for him. Apologies to the neighborhood for the wayward rockets...

The other highlight of the party was the unbelievable-out-of-this-world-most-incredible cake we've ever seen! Ezra's space shuttle Endeavour cake was provided by the charity Icing Smiles, which arranges for dream cakes for sick children. The cake was created and baked voluntarily by Krystina of Cake & Co., located in Teaneck, NJ. She is clearly beyond talented. This cake was a true labor of love, and we can't thank Krystina enough. Ezra was so thrilled. If you are ever looking for a special cake or other sweet treat, Cake & Co is your place!

Last year when we watched Ezra blow out the candles on his cake, our eyes were filled with tears because we knew we were headed for transplant and we feared what laid ahead. This year, we felt only happiness as he blew out four candles with all his might. Not because things are better. In fact, the situation is worse. But because we've learned not to look ahead too much and to be present in these moments of joy. And most of all, we've learned that all that truly matters is that our baby boy is happy.

 

 

 

 

 

A Case of the Winter Blues

We are ready for the warmer weather to appear. Ezra had a blast playing in the snow, but the aftermath has not been much fun. The cold, wet, dreary days are getting to all of us, and Ezra has not been interested in leaving the house. He is still largely under isolation precautions, especially during this flu season. It's hard to tell if the reason why he doesn't want to leave the house is simply boredom from his limited activities. We do have one thing to look forward to - his 4th birthday, which is coming up on March 8. He can't have a traditional party with other children, but we have some surprises planned that will hopefully make his birthday a special one.

Healthwise, all is status quo for the most part. He has had some bouncing around with his counts, likely due to a new medication. We also found out that he has dental decay from the chemotherapy. We're making some changes to his dental care in hopes that we can stop the decay from worsening. It's upsetting to be left with only damage from the chemo without any of the benefits from transplant. Although, in the scheme of transplant side effects, dental decay is hardly the worst, so I suppose we'll take it.

On a separate note, for anyone in the NYC area who has not yet registered to be a donor, there are several great upcoming opportunities, starting tomorrow, to get swabbed. These drives are being held for another patient named Wendy Siegel who is in urgent need of a match. You might be Ezra's, Wendy's or another patient's miracle, so please come out and join the registry:

Tuesday, February 26
8:00 AM to 2:00 PM
Grand Hyatt
109 East 42nd St (Park Ave at Grand Central)

and

8:00 AM to 2:00 PM
Brookfield Place
Winter Garden
220 Vesey St (between West and Hudson Ave)

Thursday, February 28
12:00 PM to 8:00 PM
Central Synagogue
652 Lexington Ave (at East 55th St)

A Very Special Day

Ezra had a very special day last week when he had the opportunity to visit the New York Hall of Science for an after-hours private tour. The trip was arranged by wonderful friends of ours (we love you FGP's!) who knew that Ezra would be thrilled to visit the science center. What makes the Hall of Science so exciting for our little space lover is the museum's Rocket Park where they have two former NASA rockets. Ezra has toy models of these rockets, which he plays with all the time, so you can imagine how excited he was to see them in person. When we were driving towards the museum and the rockets suddenly appeared in our sight, he had to turn away because he couldn't handle the excitement! Ezra had so much fun exploring the rockets and playing in the pretend space capsule. Then he had the chance to explore the inside of the museum all by himself. It was like a dream come true for the little scientist. He still needed to wear gloves, but it didn't get in the way of the fun (except when he dipped his hands into a big container of bubble solution and his gloves were soaked, but thankfully that was at the end). The folks at the Hall of Science went out of their way to make sure that Ezra's medical restrictions were accommodated and that all of his many questions were answered. We will always treasure our memories from this special day.

On the medical front, all is status quo. The doctors are doing some extra testing on his immune system to make sure that whatever small smidgen of donor cells is left isn't doing anything to improve his immune system before we make any decisions. His counts are still on the low side and some weeks are better than others, but overall he is stable. We have started the process of consulting with other doctors on our options.

Many of you have kindly asked what you can do to help. It would be a miracle if a matching donor for Ezra showed up in the registry. So, we continue our plea to please register as a bone marrow donor, encourage your family and friends to do the same and consider making a donation to help sponsor the processing of a donor test kit. You can do so at www.giftoflife.org/help4ezra.

As always, thank you for your support and caring.