Month: October 2013

Day -2 & Day -1 – One Bad, One Good

Quick update as we both need to get to sleep tonight so we have energy for tomorrow.

Day -2 was a hard one. Idan seemed to be restless and in a lot of pain. I had spent Saturday night with him, and he was up from 2-7AM and ended up needing morphine to calm down and finally be able to sleep. We are still not sure what the cause of the pain was, but, being on both chemo and ATG, this is expected. I then went home at 8 to take a quick nap while Amanda and her folks stayed with Idan. He did not have a good morning either, still in pain and spitting up his meds. By the afternoon though he seemed to be a bit better and was able to be cheerful again. It was the last day of chemo and ATG and we were looking forward to a day of rest.

Day-1 was a good one and did almost feel like a day of rest. He was mostly happy and playful, but definitely a hand full. His neutrophils dropped significantly and he started antibiotics to give him protection. He also got his first transfusion of blood, and will need those until he starts creating his own blood again once his new bone marrow comes in. We had some good fun on the bed later in the day as you can see here:

Tomorrow, October, 22 is Day Zero, and we have been told that the donor’s bone marrow should arrive in the hospital late at night. The transplant will take place around 11PM and go into the 23rd. So they said we will have two day zeros and day 1 will only be on the 24th. As it is written in the Book of Genesis, “On the third day of creation the Lord said “It is good” twice.” We take away that Tuesday is a good and lucky day, one that is good twice. Personally, I had my brain surgery on a Tuesday so I am happy we transplant tomorrow and have a double “good” day zero. 

Thanks all for your prayers and thought and wishes!

 

Day -3: New Mantra => One more good day = One less bad day.

Day -3Anyone who knows me knows that I am a highly rational person.  Learning how things work, understanding processes, problem solving; these are my coping mechanisms.  But there’s a small part of me (inside all of us perhaps) that still believes – or is afraid not to believe – in jinxes, bad luck, superstition, and all that jazz.  The part of me that waits for the other shoe to drop.  I’ll give you an example.  One day, on my way home from work about seven months ago, I stepped off the subway and saw the sunset straight ahead.  In that moment (and I remember it clearly), I felt like I was the luckiest woman in the world.  I was enjoying my work and planning a huge event for the women’s group about issues immensely important to me and my female colleagues.  Better yet, I got to return home every night to my beautiful baby boy and darling husband who I love with all my heart.  But in the midst of that thought, before I even had time to enjoy it, I gulped and feared when the other shoe would drop.  And in April, only a couple weeks later, my beautiful baby boy was rushed to the hospital, and so began our journey.  I have thought back to that moment, where I thought I had it all, many many times since then.  Did I jinx myself by being happy and believing us to have it all?

Last night, my father said something to me that changed my entire perspective.  Idan has been feeling very well compared to your average chemo patient.  He has been energetic and happy, drawing with crayons for the first time, decorating his crib with stickers (really, I kid you not), and giggling like a maniac when we act silly with him.  And last night, I felt that fear again – when is the other shoe going to drop?  But, as though reading my mind, my father said to me, “I am so glad Idan has been through four days of chemo, and is still feeling great.  Every day he feels good is one day less that he has to suffer from all this.”  And suddenly it all clicked.  Every day he feels well is a blessing, no matter what tomorrow brings.  

And of course, last night Idan did have a terrible night, inconsolably crying, and barely getting any sleep.  He received his first dose of morphine to help with pain.  But I for one am feeling thankful that it took this long, and glad that no matter what tomorrow brings, Idan had at least four less days of feeling bad.

Hyper IGM, How Did We Get Here, and Where Are We Going?

photo (2)Hyper IGM, bone marrow transplant, immunodeficiencies, what does this all mean and why are we doing all this to our son Idan? For those of you still trying to make sense of the whole process, I am going to try and simplify it so that it is easier to follow.

What is X-Linked Hyper IGM?

Our son Idan was born with a genetic mutation passed along on his X Chromosome. The syndrome itself almost always only affects boys as girls have two X’s and are mostly just carriers. Since it never appeared in our family before, we had no way of knowing Idan had this until he got sick in April. Hyper IGM is so rare (2 in 1,000,000) that it is never something that is tested for (and most doctors probably never even heard of it).

So how does it affect Idan? Basically the gene mutation means that he is missing a protein that his T-Cells need to produce (called CD40 Ligand) that is necessary to produce Antibodies, or immunoglobulins. This protein is also used by the T-Cells to communicate (AKA tango with) with the B-Cells and allow them to do their job as needed. This leaves a huge hole in his immune system. Imagine an army with lots of foot soldiers (T-cells) and Special Forces (Immunoglobulins) constantly at battle with the world around us. This army keeps us all safe on a daily basis, and when we encounter viruses or bacteria that the foot soldiers cannot not fight off, the special forces take over. Idan has no  ability to create special forces at all, so he cannot recognize or destroy any viruses (think flu, cold, mono, herpes, etc.) so those can kill him. He also has lots of T-Cells that do not know the basics of fighting because the B-Cells cannot give them proper instructions. Therefore, he is susceptible to many opportunistic infections (like the PCP/PJP that almost killed him in April) that the rest of us just fight off without even feeling sick.

What are the dangers of Hyper IGM?

First, recurrent infections can lead to major organ damage. Idan’s lungs were in very bad shape after beating the pneumonia back in April. As time goes by many Hyper IGM kids have orgran damage to the lungs and liver from these infections. Second, opportunistic infections such as cryptosporidium, a parasite found in water, can kill Hyper IGM patients as there is no cure for cryptosporidium without a functioning immune system. Lastly, cancer, malignancies and liver and central nervous system diseases. As time goes by there are higher risks of all these, leading to a median age of 24 for Hyper IGM patients. Our immune system is constantly fighting off cancers before they even become an issue and our son’s immune system cannot do that.

So what can be done?

In the short term we have been treating Idan with immunoglobulin replacement therapy. These are the weekly infusions of IGG’s which is one of the 4 types of antibodies he is missing, and the only ones medicine knows how to replace.  This, coupled with major lifestyle changes (like isolation, bottled water, etc.) has kept Idan safe since April. Some Hyper IGM patents can live for many years this way, but since Idan’s mutation is the most severe form (i.e., he expresses no CD40L at all), the odds of this being enough are against him. The only known possible cure is a Bone Marrow Transplant which essentially would replace his entire army with a donor’s army that works properly.  But this is a complicated and risky process with high risks and no guarantees.

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 b-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 10/10 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 nursery 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.

 

Day -4 – Last dose of Treoslofen and First Dose of Rabbit ATG – So Far So Good.

photo (1)Day -4 was a hard one on me, but pretty smooth sailing for Idan. After spending most of the night up with him in my arms I was exhausted by the time Amanda came back to the room at 8AM. Idan took a few naps throughout the day and played the rest of it. I was lucky to be able to pop home for a quick nap in the afternoon as well while Idan got his last dose of Treosulfan chemo and the ongoing dose of Fludarabine chemo. He tolerated them well. By the time I came back they had moved us to a room with a nicer view (they said it was a better suited airflow room for Idan, but I think it had to do with Amanda’s jokingly asking the nurse that morning for a room with a view 🙂 ).

In the afternoon, Idan started his rabbit ATG, which is a powerful immunosuppressant. This is the drug the doctors told us would have the worst effect on him, making him sick and giving him flu-like symptoms.  It is a slow infusion over 6 hours, and they gave him Tylenol and Benadryl first. So far he has tolerated it well.  He has a few more hours and he is asleep now. Hoping it continues that way. So far Idan has also maintained his appetite and has been eating his normal food (and lots of Bamba). That is expected to change in the next few days as his counts continue to drop and his immune system completely disappears. Only thing I am really worried about tonight is later on they need to change the dressing on his central line and we hope it does not start to bleed again.  I get to sleep back at the apartment tonight while Amanda stays here, hoping it is a better night than last. I hope to write a good summary of the whole transplant plan right after this post.  Thanks for all your support and best wishes!

Some Morning Fun

Day -5 of Transplant

It’s been almost two days since we drove to the hospital to check in for Idan’s transplant.  As Akiva and I walked side by side down the hallway, wheeling Idan in his stroller down the hallway leading to the bone marrow transplant unit, I had a flashback from my childhood.  My twin Rachael and I were laughing and running up a hill at a national park called Craters of the Moon.  We gleefully climbed the mountain of volcanic soil, holding hands.  We must have been only 7 or 8 at the time.  When we got to the top of the hill, we were in awe of the beautiful view.  But as we turned to go back down the hill, we saw how steep it really was.  We nervously squeezed each other’s hand, and looked into each other’s eyes.  As if on cue, we both said, “I trust you with my life,” and headed slowly down the steep hill, one small foot in front of the other.  Walking with Akiva and Idan yesterday felt eerily similar.  We have gleefully and excitedly arrived at our destination, excited for what’s to come – Idan’s new immune system and all that it entails.  But the path forward is steep and downhill, and we have to hold on to each other tightly, tread slowly, and keep putting one foot in front of the other.

I won’t sugarcoat it; the last couple days have been very difficult.  As we mentioned in an earlier post, the placing of the central line was much later than anticipated.  It also was quite traumatic, as we found out that Idan kept bleeding from his central line for a while after it was placed.  They had to change the dressing multiple times, all before we were allowed to enter the room.  By the time I came to his bedside, the little guy was bent over, still choking back sobs.  He was still drowsy from the anesthesia, but he was scared and angry.  The next morning, we were admitted into the hospital and Idan was finally feeling more like himself.  Unfortunately, in the late afternoon, Idan began bleeding again from the central line site, and, although largely harmless, it was very difficult to control.  Several measures were taken to try to control the bleeding, but nothing was working.  In the meantime, Idan was poked and prodded many more times than planned in this effort to stop the bleeding, and he was extremely upset by it.  There is nothing harder than looking into your little boy’s eyes as he screams in pain and fear, returns a confused and hurt stare, and begs you to save him.  But Idan is strong and, thankfully, very forgiving, and even smiled at the very nurses who moments earlier were forcefully applying pressure to his wound.

After a mostly restless night, Idan returned to his usual self this morning.  Full of life, energy, and jokes, Idan laughed and played his way through the day, and we could not be more proud of him.  He manages to find joy in the smallest activities, from playing peek-a-boo from behind the bars of his crib, to practicing the new words he learned to say, like door, ball, car, and – of course – bamba (his favorite snack).

10.17.13

Tomorrow, we start the ATG, which is another form of immunosuppressant, but likely to cause the most severe side effects.  We have been told to expect that things will get a lot worse for our little one as the days go by.  ATG causes significant flu-like symptoms, among other things, which we will look out for tomorrow and through the next couple weeks.  The effects of the chemo may hit around the same time, so we’re glad Idan is such a strong, resilient little guy!

We are relieved another day of chemo is over, and will send an update again soon.  Thank you for all your support, prayers, and words of encouragement!