Tag: HIGM

It’s Beginning to Look a Bit Like a Cure (!!!)

 We have been pretty delinquent in posting, but wanted to share with you the latest update regarding Idan.  We are very leery of using the “Cure” word, and truth be told, we are many months off from knowing with certainty that Idan is or is not cured, but we have had some very encouraging test results that brought a sense of joy and relief that I cannot even begin to describe.  So we thought we’d share those results with you, and also share the impact this has had on Idan’s life in a very special way.
First, Idan’s chimerisms. We have been monitoring this number like a hawk, and, if you follow our blog, you know that the chimerism results have played with our heart strings in both the first and second stem cell transplant.  In the first, we watched with hope as Idan’s chimerisms in his T-cell line went from only 7% to over 30% with various interventions to save the graft, only to watch it plummet back down in a slow but steady drop over the course of a year.  This time around, we started with 96% in the t-cell line (exactly where you want it to be), and watched in agony as it took a nose dive shortly after transplant. Again, interventions ensued, and we were able to slow its decline, only to see other cell lines slipping.  Although we only need to replace Idan’s T-cells, if his other cell lines are not in harmony (e.g., are comprised of his own cells and not the donor’s), the prognosis for the long term viability of the donor graft is grim, and we would be looking at another rejection at some unpredictable point in the future.
So we have been monitoring this number with a mix of dread and hope, yearning and fear.  A few weeks ago, we learned that Idan’s chimerisms were stable for the first time ever, and the cell lines that had been dropping had even inched up a bit. Over a year out from transplant and chimerisms stable, with t-cells hovering around 87%, we couldn’t be more thrilled.
Second, Idan’s IgGs. When Idan was first diagnosed, one of the most telling indications of his disease was the fact that he produced zero antibodies. None. This has been the constant gaping hole in his immune system.  We have been giving Idan weekly subcutaneous IgG replacement infusions at home every weekend since he can remember.  Well, the first real test of his immune system is whether he now can make his own IgGs.  Over the summer, we stopped giving him his weekly infusions and began testing his IgG levels every few weeks.  As expected, they dropped over the first couple months as the IgGs that were floating around his bloodstream from the infusions slowly dissipated.  But a couple weeks ago, when we tested Idan’s IgG’s, now four months since his last infusion, they held steady at 475. Not zero. 475.  Idan is making his own IgGs!!!!!  An impossibility for a child with Hyper IgM Syndrome.  So, notwithstanding the fact that Idan has had four viruses since starting school (including parainfluenza, which is a pretty nasty one), he has gotten over them with relative ease.  This is the most encouraging sign so far. And it allowed us to give him his first couple vaccines, shots that even Idan welcomed with joy – no tears and no flinching! – understanding the magnitude of achieving this incredible milestone that has always been out of reach.
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Now, Idan.  As you know, Idan started school in September.  What you don’t know is that this is a very special school that caters to children who are exceptionally bright and advanced learners.  This means that Idan is surrounded by children who share his joy and love of learning, and by teachers who are well-trained and sensitized to socialization struggles that often accompany high IQ, which means they have been amazing sources of support for our Idan, who has been thrown into the deep end of socialization after only knowing what it is to be in isolation.
Importantly, Idan walks into school and sees a row of cubbies with his name sandwiched in between all the other kids’ names.  Inside the classroom, his name again appears alongside his classmates’ names – on the cubbies, on the rug, on school supplies, on pictures and projects hanging up on the walls.  When he walks into the classroom, he is greeted by a chorus of sweet voices saying, “Hi, Idan!” (They even all know how to pronounce his name!).  He belongs.  He feels it.  And he’s right where he’s supposed to be.
We were worried that Idan would be overwhelmed or scared by school. Boy, were we wrong.  He walks around with a light inside him so bright, I swear his whole face is glowing. His laughs are heartier, his smiles are wider, his eyes are shining brighter.
For those who have any thought about donating blood or bone marrow, this is what it means to save a child’s life.

Day 6: Another long day, but much improvement.

13681051_1337264952970135_1265469612125622542_nWell, it started out as another rough day, with Idan continuing to suffer with pain, itchiness, and fatigue when all he wanted to do was play. This morning we had a good conversation with the team who noticed right away (without us having to say anything) that, even if the pain was reduced from yesterday, the cocktail definitely needed more adjustment. We changed the dilaudid from one dose every 4 hours to a constant drip, and added a new drug that is supposed to counteract the itchiness that comes with opioids.

By evening, Idan’s mood had lifted substantially and we heard him laugh for the first time in several days. It’s the most beautiful sound. The extreme itchiness seems to have subsided and his skin rash from earlier in the week has also significantly improved.

Idan had his second school session today with another teacher from the community who teaches at the same school as the teacher who came last week. They have coordinated with each other and have already started planning a curriculum for tutoring sessions that are tailored to his interests and skills 🙂. Our heart is full, and we are so grateful to these lovely women who have added some structure, fun and normalcy to his daily routine.

Yes, another long day, but all in all, a good one.

Day 5: Pain (somewhat) under control, but new meds bring new side effects.

13669770_1336467653049865_8170639665652922012_nWe have started Idan on a regular dose of dilaudid to keep him more comfortable. It is always hard to know if a young child is in pain, and this is especially true with Idan, who played and smiled even when he was on a ventilator three years ago. This remains true today. He plays and insists on playing even though his blood pressure is elevated and he is grunting with every breath. He screams when using or about to use the bathroom, and writhes in bed with itchiness from his rash, but if you ask him if it hurts anywhere, he will insist he is fine. He is easily frustrated, very restless, and quite moody. But ten minutes after the dilaudid kicks in, he is breathing easy and having fun. So after a consultation with his team, we’ve decided to give him pain meds regularly instead of as needed. Unfortunately, the increase in pain meds comes with the added side effects of making him more agitated, itchy and tired. Worth the pain relief, but makes for an exhausting day for all of us. In the meantime, his team is working on finding just the right cocktail that keeps him comfortable in all respects.

Speaking of cocktails, during rounds today Idan bravely decided to tell the attending (who happens to also be the head of the bone marrow transplant division) that he does not like the taste of several of his oral medications. He went on to pronounce perfectly the ones he does not like: fluconozole, ursodial, and acyclovir. To his delight (and ours), the attending said we can switch his fluconozole to IV for now, thereby eliminating one of his most dreaded oral meds. Very proud of our 4 yr old for speaking up and for getting his team to change his medical plan.

When all is said and done, we are counting our blessings. The doctors are very happy with his progress and are doing everything they can to help us manage the side effects. Our very good friend Ashley is visiting from New York and has managed to keep him entertained all day. So grateful to all of our family, friends and all of you who have been supporting us along the way.

Day 4: Another rough day brings new challenges, but all is going as expected. 


Day 4: Another rough day brings new challenges, but all is going as expected. 
Idan’s rash continues to drive him crazy (even literally at times), as it would any other person, but thankfully it looks less red and angry. He continues to have some GI issues, but less intense than yesterday. Now that the fever, rash and GI issues are starting to resolve themselves, new challenges are rolling in. Idan is starting to get some mucositis it seems, which is essentially sores and blisters that can develop in the mouth, nose, throat and GI tract, and can be very painful. So pain seems to be the biggest problem of the day. Terrible pain from different places. Managing pain for a 4 year old is very difficult. There are 3 options right now: (1) convince Idan to take another oral med every 4 hours around the clock, which is more benign than the other options, but, let’s face it, unrealistic, (2) give him morphine, which is the most effective at reducing pain but gives Idan paranoia and can make his itchiness worse, or (3) dilaudid, which causes less side effects but is slightly less effective than morphine. We went with (3), and we will see how it goes. 

Idan has tried hard to remain stoic during the pain. It was only because he started breathing funny that we fully understood how badly he feels and how hard he is trying to keep on a brave face. We took him down for an X-ray (which was clear), but as soon as the dilaudid entered his system, he immediately started to perk up and breathe easier. It brings us a great deal of pain just to see our sweet child in so much pain. So, yes, pain is the challenge of the day. But now we know, and we can and will help him through it. 

All that said, we are counting our blessings that Idan is doing well on so many other fronts. His counts have completely dropped to zero, meaning his bone marrow is completely empty and ready for the new donor cells to set up shop. The doctors have no concerns about the side effects – this is all par for course. Importantly, Idan is still eating and drinking very well and taking his oral meds (with M&M bribes – whatever works, right?). We spend most of our time playing and occupying him with activities that keep his little hands busy to reduce the scratching. He said yesterday that he wants to be a doctor when he grows up because “doctors make people better.” His positive outlook and tenacity are helping us all stay strong. 

Thank you for your continued support and strength!

Days 2 & 3: An eventful 24 hours, and another random act of kindness. 

Idan has been starting to feel the side effects of the chemo and other various medications he’s been on and has been in a fair amount of pain. Fevers, itching & burning rash, and (sadly) back to pull ups – all quite a lot to handle. Idan’s aunt Tamara has been visiting and that has been an incredibly welcome and helpful distraction. He also had his first “school” session for the first time back in New York, with a wonderful teacher named Katie who volunteered to tutor Idan once a week (have we mentioned how much we love the people here?). He missed learning so much, so this session brought him a lot of joy. 

Later in the afternoon he started to experience more intense pain and GI issues, and then his rash began to flare up again. We had a good long cry together after an especially intense two hours, and then, once the pain meds started to kick in, he was singing silly songs again and happily playing. 

At the end of the day yesterday, as we were getting Idan ready for bed, he turned to his IV pole (named Ga) and gave it a loving pat and a smile. Then he turned to us and, still smiling, said, “Ga is giving me lots of presents to help the new cell family.” It’s the very last thing we expected him to say about this giant awkward machine that he is constantly tethered to, tripping over, and stubbing his little toes under (not to mention the fact that it’s giving him meds that make him feel sick all the time), but once again he surprises us. Idan’s courage and ability to focus on the goal is just… well, there are no words for that. 

Finally, we wanted to share a sweet story. Akiva popped out to the supermarket yesterday and a woman rushed up to him as he was at the cash register and insisted on paying for his groceries. She was a young mom and she came over when she saw the Seattle Children’s Hospital parent badge around his neck. She told Akiva that she spent a fair amount of time at Seattle Children’s Hospital a few years ago and now every time she sees someone with that badge, she pays for their groceries. A nice reminder that, even when it seems that the world outside is filled with hate and terror, there are truly good people (and you can probably find them in your local supermarket).

Musings

Just barely 4 years old and yet this conversation happened today, just after his fever spiked again:  
Me (Amanda): “Idani, which friends were you talking about yesterday when you said ‘it’s ok to be sick because you get to meet new friends?’ Were these friends the nurses? The CNA?”

Idan: “No. I was talking about the friends I will meet when I go to an outside school that’s a real school.”

In one sentence he manages to break my heart into a million pieces and put it all back together again. He is all too aware of the purpose and goal of the transplant (perhaps more than we know), and he is therefore willing to endure the pain and significant discomfort because he sees a better future for himself. So do we.

Day 1 – Birthday Fevers:


It’s been an eventful 24 hours since Idan started his stem cell infusion yesterday. The cells were infused over the course of a few hours and towards the end Idan started to feel bad and spiked a fever. No way of telling if it was the infusion, the week of chemotherapy and ATG or a new virus/bacteria affecting him now that he has zero immune system. The took blood cultures and gave him Tylenol to reduce the fever. He was pretty tired and not in the best mood the rest of the day yesterday. Around 3:30 AM he woke up upset and spiked another high fever. He took his Tylenol well (getting him to take his oral meds has been a challenge) and went back to bed. At 5 am they gave him a red blood cell infusion as those counts have (predictably) dropped significantly in the last couple of days. 

Today he has been enjoying lots of birthday celebrations. The first thing he wanted to do when he woke up was unwrap some of his presents. He also asked us if, because it’s his birthday, will the nurses give him a break from checkups. Sadly he does not get that break. But they did all come in and sing him happy birthday which was fun. Then another hospital volunteer brought by another big bag full of gifts. A very special nonprofit called The Confetti Foundation organized a whole array of birthday goodies and decorations and a special delivery of cupcakes from Cupcake Royale. Idan insisted that Amanda stay up late the night before decorating the room so it would be ready for him this morning. He then stayed up and watched, announcing he was “too excited to sleep.”
Sadly, the week of Chemo, the lack of immune system, the fever, and a very itchy rash (likely a side effect of chemo) has taken a toll on his mood. He has been very cranky and tired today and, although wanting very much to celebrate, he has just not been feeling up to it. 
Thank you for everyone that organized and sent him gifts for his birthday. In addition to a very fun session with music therapy where insisted that the musician sing 7 Years by Lukas Graham, unwrapping these gifts and seeing the cupcakes and decorations brought him a great deal of joy and are helping him through a rough couple days. 
We will end with a sweet line he send yesterday after all the commotion of transplant and not feeling well. Before he went to bed he said: “Mommy, it’s ok to be sick because you get to meet a lot of friends.”

Glad he knows how to see the positive in everything.

Day 0: Transplant Day!

13697129_1332607503435880_8894533746877192939_n Day 0: Transplant Day! After much delay, Idan’s donor cells arrived and they started the infusion around 1:30PM. As you can see from the photos, there is a little bag of 160ml of stem cells from the amazing human being that agreed to donate his cells to give our son a long and healthy life. Idan was in the middle of lunch, as a true New Yorker he was eating a bagel and cream cheese, and did not let the commotion stop him (or 10 nurses singing to him). Besides bringing the cure Idan’s been waiting for we do hope the donor likes bagels!
Thank you for everyone that has shared Idan’s video. We reached our goal of 180 shares in just a few hours!!! Let’s keep the ball going – keep sharing the clip to raise awareness for Hyper IgM syndrome and the Hyper IgM Foundation.

Day -1: Groundhog Day


Day -1: All over again – Quick update on transplant timing. We heard from the transplant team today that the donor cells will only be arriving at the hospital tonight around 11:30PM. They then need to treat it which takes a few hours. So they have now scheduled the transplant for tomorrow morning (Tuesday). So instead of today being Day Zero it is day -1 all over again. A bit of a Groundhog Day affect but that’s transplant for you!