Month: July 2016

We wrote this explanation three years ago for a blog post before Idan’s 1st transplant. Thought I would repost this now for those following along. Also adding a link to explanation of Hyper IgM from the Hyper IgM Foundation site:
– So What Is a Bone Marrow Transplant (BMT)?
Currently, there is no way to fix the gene mutation that causes Hyper IGM, but there is a way of replacing the bone marrow and giving Idan a new immune system. One can look at the bone marrow as the army base that the immune system (as well as the blood) is created and trained in. The idea is, if Idan’s T-cells cannot produce the protein he needs, lets give him a new army base, with soldiers that know what they are doing. But it is not that simple:
1) Currently the army base in his body is filled with soldiers (even if they’re not too smart), so there is no vacancy for him to host someone else’s army.
2) Idan’s body (AKA, the equipment, building and land that needs protection) is a perfect match with his current army, and no other army (AKA the donor’s bone marrow) is going to know how to work it in the same way.
3) Any gap in the protection that his army is giving him (albeit a lazy one) will open up his body to infections.
4) In order to create a vacancy in his army base so that a new army can move in, the old one needs to be eliminated entirely. The way they do this is with chemotherapy, which has the nasty side effect of destroying the bone marrow, and in our case we want that. However, that process comes with its own risks of toxicity to the body.
With all the risks involved Amanda and I decided that BMT is the way to go. BMT will give Idan the best chance at a long and healthy life. We did a lot of homework and spoke to dozens of bone marrow teams around the world. We decided on Seattle because of the new type of chemo drug they are using (Treosulfan) that is less toxic than others. If all we need is to clear the army base from soldiers why bomb random cities and cause damage to the whole land?
– How will this cure Idan?
First they will use chemo and ATG to eliminate his bone marrow and suppress his immune system. This is what we are doing from Day -6 to Day -1. We then give Idan a day of rest while the chemo in his system continues to clear out all the soldiers from his army bases (including the civilian side of his bone marrow that produces red blood cells). On Day 0 the transplant date, we will infuse Idan with the donor’s army. His army has been found to be a 12/12 match to Idan’s. While this is great news, it is not the whole picture. This new army is not really going to understand how to relate to all of Idan’s body parts, and will most likely still want to attack some of it (this is called Graft ss Host disease, and it happens with 70% of the transplants from unrelated donors like Idan’s). For this reason they use the ATG, and the other immunosuppressants to allow the new army to come into the empty base, but forces it to unpack, train, and start patrolling very very slowly. Giving the new army more time to get adjusted to Idan’s body is a delicate balancing act that will happen over the next year. Since they are letting the bone marrow come in and start working very slowly there is a longer time where Idan is lacking any working immune system and will be kept in isolation as well as be given drugs to protect him.
– So what is next?
Day -6 to day 0 we are clearing his bone marrow and making room. On Day 0 he gets the transplant, and we start counting days until the bone marrow travels to the base and starts setting up shop. We hope to hear around Day 22 that they have reached the base and that would be engraftment. We will then wait until we see his counts go up. The first type of soldier to come back (or, better put, to “grow”) is his neutrophils (which work fine now, but we have to get rid of them with the rest of the army) and they will wait until that level is good enough to get released to isolation at our apartment. This will happen between day 30 and 50 and then we will count to day 100 before they might let us go back to NYC. From then on and until around 2 years post transplant Idan will still have a weak immune system. Our hope is that around one year post-BMT, his new army will be strong enough and acclimated to his body to start reducing the supplemental protections we are giving him. Along the way there are going to be many scary events but we and the medical team will deal with them as they come up.
In a few years, when Idan can go to school, play with other kids, take swimming lessons, play in the park and ride the swings all with the protection of his donor’s army, we will know we did the right thing to ensure Idan has a normal, healthy and happy life.
For more see here: http://www.hyperigm.org/about-hyper-igm-syndrom/

From Facebook:

Day -6: we checked in today at 8AM and got settled in our room. Sadly, not one with a view ( you would think with all our membership points 🙂 ). He received his first two dosages of chemotherapy (Treosulfan & Fluderabine) and did very well. He played play-doh and ate lunch and then played with his new Magna Tiles. Now he is working on the monthly calendar with Amanda. He still has pain from his central line surgery yesterday that is making hold his head to the side and not wanting to use his right arm too much. Hope that subsides soon. Thank you all for your thoughts and prayers.

It feels like we have been training for this forever. Transplant. And now it’s happening. This Monday, Idan will have his central line placement surgery, and early Tuesday morning we will be admitted to start chemotherapy. Six days later, the donor will donate the “cure” in the form of peripheral blood stem cells (PBSC) and the nurse will infuse them slowly into Idan’s central line. Those cells will find their way to the inside of every bone in Idan’s body. The bone marrow, which will be empty as a result of the chemotherapy drugs and immunosuppressants, will host the new stem cells. Slowly, over many many days, weeks and months, the donor cells will set up shop and start producing Idan’s new blood system, including a working immune system.

They say that transplant is like a marathon. It is long, you have to keep a steady pace and keep going. Forward, onward, keeping your eyes on the goal, the cure. Like a marathon, we have been training for a long time for this. Ever since Idan’s first transplant almost three years ago, we knew that one day we might have to run this marathon again. Idan has grown up knowing that Seattle is where he had a transplant, and where he might once again return for his 2nd transplant. He keeps asking us if he will need another one after this, and we keep saying no, that we only really get two tries.

For the last year, Idan has spent countless hours playing with us and all his babysitters doctor. Like training for a marathon, he has been training by pretending to take blood, to get shots, to send blood back to Seattle, or making believe we are taking a flight to North Carolina or Seattle to bring the blood samples there. He has been preparing for this for so long that anticipation has grown to a point that tonight he told us how excited he was to start transplant this week. He was mainly talking about all the toys that he will be getting once he’s admitted to spend the next several weeks isolated in the hospital (the ones that very kind people of Seattle have given him). Still, the excitement is there, we are all excited (and terrified) to finally start the run, the long walk to a cure.

Everything has been falling into place for now. Idan is as healthy as one can be entering into transplant. Once we start it will be hard, things will get bad and ugly and rough. Amanda and I know this and are dreading the long days, watching the chemo affect Idan, making him sick and tired, all in the name of removing his defective bone marrow and making room for the donor cells to grow. There will be many setbacks and unexpected obstacles, but we have been training for this, and we know, no matter what, to keep our eye on the finish line, the cure.