Month: August 2016

Day 40: Moving along

IMG_4908It has been 10 days since our last update and we apologize for not updating sooner. Following other people’s medical blogs I have learned that no updates is usually a good sign 🙂.

Before diving into the latest about Idan (which is all good!), we would like to give a big Mazal Tov and thank you to that very special 12 year old that organized a large toy drive for Idan for her Bat Mitzvah. We heard that she included him in her speech this past weekend and has had her whole community praying for our little boy. She is a wonderful person and we are so very honored and incredibly touched to have been included in her big day!

Things have been going well in the past week and a half. We have been keeping busy with clinic visits and building a routine for ourselves here in our temporary home in Seattle. Idan’s blood work has been doing well. He has consistently surpassed his 50oz of oral fluid intake a day and only needs to be hooked up to his IV fluids at night for 2 hours to administer his magnesium. We had hoped to switch the magnesium to an oral medication by now, but it tends to be hard on the tummy. We tried and failed last week, and we will try a different version of the oral magnesium this week with the hope that he tolerates it well and we can get off the IV. Besides that he has been doing very well with the rest of his 10 or so oral meds twice a day and has been eating well.

The trick now is to keep him healthy and safe which is not always so easy. The team feels that he is at lower risk for reactivation of some of the bad viruses as it has been 40 days already with almost no immune system and it has not happened yet. So, while still possible, the risk for CMV, EBV and Adenovirus is lower now. That said, new viruses are always scary and despite all the medications he is on we need to be vigilant in keeping his safe. Today he woke up with a slight cough and a few sneezes, so we are of course worried he is coming down with a cold, which could land us back in the hospital. So far no fever and he seems to be in good mood and high energy. Tomorrow morning we have clinic and blood work so we will talk to them then and hope for the best.

Amanda went back to work this past week from the home office we have set up for her, and I have been trying to take care of more of my work for the Hyper IgM Foundation as well. This has left Idan to enjoy a few hours a day with some new faces. He had a teacher come in once or twice a week for some school, which he absolutely loves. We have also been assigned two wonderful volunteers from Side-by-Side, a program affiliated with Children’s. Idan gets to play with them one afternoon a week and has had a lot of fun showing him all the gifts and toys he has received from all of you! We have also been very lucky to find a few babysitters in our apartment complex who can help entertain him when Amanda and I both need to get things done. One is a BMT nurse that literally lives right above us, and another is a hematology nurse that lives near by. So we feel pretty good about Idan spending time with them, as he has been teaching them the proper way to do a central line dressing change on his Piggy and how to do transplant on his puppy, Blue 🙂

Day 30: A very good sign.

We tested Idan’s chimerism on Monday and received very good news. As a reminder, chimerism is the percentage of Idan’s new cells that belong to his donor versus his own defective immune system making a return. The results came back showing that a whopping 92% of his new T-Cells are donor t-cells, all of his myeloid cells are donor myeloid cells and 89% of his NK cells are donor NK cells. Translation: according to these initial results (which may change over the next few months), the transplant appears to have worked! A cure is in sight!!!
We have spent the last 24 hours in a bit of a haze. It is difficult to rejoice knowing what challenges lie ahead (viruses, infections, GVHD), each of which can turn back the clock, or worse. But for the first time in a very long time, we are enjoying the luxury of hope. For the past three years, “hope” had become a four-letter word. Each time it reared its head, our hearts would be crushed soon thereafter. But unlike Idan’s last transplant, with a starting point of only a tiny T-Cell graft (7% at first) that we tried everything we could to salvage, we are coming out of this transplant with a very strong graft, and a very healthy child. We have real cause to hope that a cure is indeed in sight.
We also received some very sad news yesterday. We learned of the passing of a 4 yr old boy with Hyper IgM who had his transplant in Argentina only a few weeks before Idan. We had been following his story and talking to his mother about the complications that came up post transplant. He died from a vicious virus that snuck up on him after he was released from the hospital. Our thoughts and prayers are with this family as they grieve this tremendous and unspeakable loss. Sadly, this little boy is the third Hyper IgM child in just a few months to die from complications after a bone marrow transplant, which is the only known cure for this disease. For a disease that affects only 1 in 1,000,000 boys, 3 is an alarming number of deaths. It is also a very painful and unwelcome reminder that the first year after transplant is a treacherous road, with very real dangers that can happen at anytime. We will continue to raise awareness for Hyper IgM with the hope to accelerate a safer and better cure for Hyper IgM than the risky stem cell transplants that has taken so much from these families.
As always we thank you for being part of Idan’s army and part of a movement to continue to fight for all of these precious boys so that transplant is not the only option for a cure.


Day 24 – Building a Routine:

It is definitely good to be out of the hospital and back at our temporary home here in Seattle. Idan has spent the last few days enjoying his freedom from the tethered IV pole and the frequent vital checks he had in the hospital. He has been getting a lot better at taking his oral meds and is very close to meeting his daily oral liquid intake goal of 50 oz. Amanda and I still have to hook him up at night to an IV pump that gives him extra fluids and magnesium and then unhook him and flush his central lines in the morning. We are all looking forward to the day we can switch over to oral intake of magnesium and not have to do any nighttime IV fluids. Idan has told us that he plan for clinic Monday is to ask the doctors about this 🙂

We are on a twice a week blood test and clinic schedule, which ends up always leading to more visits as things come up from these tests. Today, for example, we were supposed to only have a blood draw at 7:30 a.m., and then Idan enjoyed a well-deserved freshly baked Top Pot doughnut in the Marina/Lake Union area. Just as we were heading home we got a call from the clinic that the results from his morning blood draw showed that his ANC dropped down to 1000 (it has been trending down for a few days). So we made a U-turn and headed back to the clinic for Idan to get a G-CSF shot. This shot will help stimulate growth in his bone marrow and hopefully get it to trend back up again. This is quite normal at this stage and not anything they are worried about. Idan, being the trooper he is, did not even flinch during the shot in his arm, and we were back out again in no time heading back to play more doctor at home.

The last few days have been very fun for Idan, with his Uncle Yitzi visiting for a couple of day and joining us on all our adventures and today with the arrival of two of his aunts for the weekend. On Thursday we took advantage of the good weather and went out to Discovery Park for Idan to see the beach and light house. We try to find places that are not crowded and don’t present other risks for his very week, almost non existent immune system.

As always we are very thankful to all the support Idan’s army has shown us in the past few weeks. Today, Idan came back from his unexpected shot at the clinic to a big surprise in the form of two large boxes from Amanda’s co-workers at Sheppard Mullin back in NYC. These thoughtful toys and activities (and some pampering items for us) will keep him busy and us sane during the long days ahead.

We are checking Idan’s chimerism next week, which will tell us whether he has engrafted with donor cells or not (the best measure of the transplant’s success). We expect results by Thursday or so. We will keep you all posted!

Day 21: And just like that, we are on the other side. 

It’s been quite an exhilarating couple days preparing for discharge. Today, at 1:30 pm, Idan marched proudly out the doors of the BMT Cancer Care Unit at Seattle Children’s Hospital for (what we hope is) the last time. After three days of cleaning, packing, and home care lessons, we were given the green light to go back to our temporary home in Seattle, and it feels so good. 

As one of our doctors said to us the other day, we have jumped through so many hoops to get to this point and for Idan to be doing so well. That is a huge blessing and quite uncommon. There are still quite a few scary hurdles to overcome over the next few weeks and year (GVHD, viruses, infections, just to name a few), but at least the initial long hospital stay is in our rear view mirror. 
When we think of all that Idan has been through and recovered from over the last few weeks, we are simply in awe of this remarkable resilient child. In no particular order: traveling 3,000 miles from home, saying goodbye to family, friends and his teacher, multiple moves from a hotel to an apartment to a hospital, 3 weeks of poking and tests before transplant, painful bruising after the central line surgery, an entire week of chemotherapy, fevers, a terrible itchy rash all over his torso, intense pain in his GI tract, reversion to pull-ups, getting tethered to an IV pole and losing all freedom, independence and privacy, midnight and 4am vitals interrupting his sleep, losing most of his hair, being forced to take a dozen new medications by mouth, C. Diff stomach bug causing more pain and more discomfort, and his first night terrors, just to name a few. Through all of this, he endured. But we were not without help. Visits from family and friends, constant support from the Seattle community, meals and household goods and appliances from Margot and her army of incredibly giving people, toys galore from Amy, her daughter Eliza and their family and friends, a car for our entire stay from Robert and Polly, the school teachers, Nomi and Katie, who volunteer their time to teach Idan each week, the family, friends and even a couple strangers who have sent toys, cards and notes of support, the stream of comments and outpouring of support from people following this page, it all adds up to be a formidable force – an army. Today, as Idan marched ahead, he walked quickly and effortlessly. He laughed and danced with our nurse. He is eating and drinking better than he did before transplant, takes all meds, and loves playing doctor more than ever. We couldn’t possibly be more proud of him.  
We will keep you all updated as we check his chimerism in a couple weeks, which will be the first indication of whether this transplant has resulted in a cure, and Idan will continue to be monitored closely for signs of GVHD. In the meantime, we are excited to share this incredibly important and meaningful milestone with all of you. Thank you for being part of Idan’s army.

Day 18: A really ridiculously good day. 

After an uneventful day yesterday and a very restless night last night, Idan’s ANC shot up from 1546 to 2316 this morning. So far the medication he is taking for his C. Diff seems to be working. He has officially transitioned to oral meds for all his medications (except for nighttime fluids which he will continue to take for several weeks), and we are getting trained for home care. If everything continues to go smoothly over the next few days, we are looking at getting discharged on Tuesday!!  

In the meantime, with Idan’s ANC safely above 500 and his C. Diff at bay, we were allowed a four hour break from the hospital today!!! Idan now can be unhooked from his IV for the entire day and he had total freedom today for the first time in such a long time. We went to Magnuson Park and then back to our apartment for playtime. For the past four weeks, we have had a constant buzz around us – people floating in and out of our living space at all hours, machines beeping, footsteps and call lights ringing in the hallway, the hum of the air vent, the frequent opening and closing of cabinet doors when supplies are delivered to the room – so you can imagine the peaceful stillness of entering our quiet apartment with Idan. He played completely uninterrupted by vitals and probing by strangers and did not have to drag a monstrous pole with him every time he wanted to move an inch. 

We are back in the hospital now, but hopefully not for too much longer!

Day 16: C. Diff. Two Steps Forward One Step Back: 

We had this mantra during last transplant and we have shared this with other families going through transplant. The feeling of two steps forward one step back always holds true. After very positive news yesterday, Idan has not been feeling so good today. 
His ANC has held above 500 and they now consider him to be engrafted. We were able to stop his antibiotics IV and another IV med today and are making the right steps towards a possible discharge next week. But his gut had been acting up yesterday and today and that concerned us because it could mean any number of things. He woke up at 5am feeling sick and has had a rough day.

Later on today, after many potty breaks, the results of the stool test came back positive for C. Diff, which is always a risk when you combine a compromised immune system with a long hospital stay and broad spectrum antibiotics. This was of course not what we wanted to hear, but at least we caught it early and know what it is. They started him on antibiotic treatment (Flagyl) tonight and he will take it by mouth 4 times a day for ten days with the hope that it clears his C. Diff. 

Our hope is that it clears quickly and does not cause Idan too much discomfort and suffering.

Day 15 – ANC 576! 

Today we woke up to some good news that Idan’s Absolute Neutrophils Count (ANC) is up to 576. As you may recall from our previous posts, the neutrophils are part of the white blood cells. They are therefore part of the body’s first defense against infection and form an essential part of the innate immune system. The ANC coming in is the first sign of engraftment, and, once Idan has over 500 for three days, they will consider him “engrafted.” Of course we will not know if these neutrophils are the donors or Idan’s for a few more weeks, but we are working under the optimistic assumption that they donor cells. 
If all continues to go well, we may be able to be discharged earlier then the 2-3 weeks we thought we had left inpatient. Possibly even at the end of next week. Our goal today and for the next few days is to wean Idan off any meds he does not need and switch the rest of his IV meds to oral. Since he has not been in pain in a few days, we have been weaning the Dilaudid and today we stopped it all together (along with the 2nd continuous narcotic drip he had to counter act the itchy affect of the Dilaudid). We also discontinued the oral Zofran we started a couple days ago, and so far Idan has not been nauseous. Tonight we will switch his continuous IV drip of tacrolimus to oral at 9PM as well. Tacrolimus is a chemo drug that Idan will be taking for the next 6-12 months to suppress his immune system and allow the donor cells to come in very slowly to reduce the risk of developing severe graft vs host disease (GVHD).  

Idan was thrilled this morning when we woke him and told him the good news about his ANC. He was in a good mood all morning, enjoying another drum session with the music therapist who already knew all his favorite songs by Twenty One Pilots. He then had a great session with one of the great teachers that have been volunteering their time and had a good lunch. He then jumped back into bed and took a 4 hour nap 🙂 Got to have energy to make those cells keep growing. 

It is a long long long road ahead with more dangers then one would like to outline here. Once we transition to outpatient, the journey will still only be in its infancy. We will have to wait two more weeks to test his chimerisom for the first time to see what percentage donor his cells are. We have to hope that Idan does not get GVHD, or more realistically to hope that the GVHD is acute and mild. We hope that he stays virus and infection free and has no reactivations of any viruses hiding in his DNA. And we hope that his new immune system continues to grow and thrive as we wait for it to become fully functional.

Day 14: Another day in the right direction, and another happy occasion celebrated in the hospital.

13912898_1343132032383427_4962615802868756776_nToday marks Akiva’s and my 7th anniversary. It’s not the typical way we celebrate our anniversary (dinner, wine, a sentimental gift). Instead, we are sleeping in different places out of necessity. I woke up at 4:30 a.m. to a very alert Idan who could not fall back asleep (yesterday’s very long nap seems to have affected his sleep cycle), and Akiva rushed over at 7 a.m. with much needed coffee and breakfast. The excitement of our day was not flowers or romance, but unhooking Idan from his IV pole for exactly enough time for him to race around the halls of the unit untethered in his favorite John Deer bike. Instead of going out to a restaurant to eat tonight, we will be taking turns negotiating with Idan to take all his oral meds. No, it’s not a traditional way to celebrate, but we are celebrating nonetheless. We are together on this incredible journey, and there’s as much love and romance in that brief moment catching each other’s eye as Idan’s giggle bounces off the halls of the BMT unit down as there could ever be sipping a glass of wine.

Today, Idan cheerfully listed all the occupations he will be when he grows up: an astronaut, doctor and lawyer, in that order. Boys with Hyper IgM have a median life expectancy of 24 years. Not nearly enough time on this earth, let alone enough time to enjoy the luxury of building a career (or three, as the case may be…). But this transplant has given Idan a chance to live a long and healthy life, and he has all the reason in the world to dream big. His doctors are very happy with his progress and we are starting the slow process of transitioning his IV meds to oral so we can be discharged after his ANC count reaches 500 and stays there for three days. We are expecting at least 2-3 more weeks inpatient, but we could be discharged sooner if his counts climb more quickly. No way to tell if these new cells are Idan’s old immune system making a return or the donor’s cells starting to grow until we test chimerism in a couple weeks, but it will be an important milestone nonetheless.

Day 13 – Teenager:

13882107_1342571062439524_7192353666645223481_nToday Idan woke up late and said he was very hungry. He had a big breakfast and took his morning oral meds. Then climbed back into bed and did not want to do anything but watch his YouTube clips (Once Upon a Time… Life– an educational cartoon about the body). He fell asleep in bed a half hour later and then continued to sleep for 5.5 hours. We had to wake him after 5pm so he did not sleep the whole day. I guess in true teenager form his body is doing a lot of growing (mostly his bone marrow), and he needs his rest.

During dinner (awake and starving, just like a teenage boy…), Idan insisted on playing his new favorite song over and over again. We all had to listen to Stressed Out by twenty one pilots. Every time the nurse walked in she would say “this song again? I love this song!” Teenagers!

His counts have continued to slowly come in over the past three days, and his ANC has climbed to 138. We closely watch this number as we hope to see a slow increase over the next week or two with the goal of getting to above 500 for three days in row, which will tell us that he has engrafted. We also started to wean him slowly on his pain meds and we will see how he handles the lower dose.

Tuesday will mark two weeks since transplant day, and three weeks since we checked into the hospital. We are all feeling the stress of isolation and long repeated days. It’s only fitting that we keep listening to Stressed Out! But, all things considered, everything is going well so far.

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