Idan, my hero

Idan in the SCCA House Play Room

So we got our schedule for next week. The good news is that Idan will not be admitted to the hospital except for the day of his DLI and it’s only a day admission so we can most likely go home that night. The bad news is that next week is going to be a living hell for the three of us.

Here is a summary of our week:

Monday: Kidney Test: We have to be at the hospital at 8:00 a.m. for a kidney test. This requires Idan to fast the night before, and he can’t eat any solid foods until after the test is over —- 8 hours later. So for approximately 16 hours, Idan will not have anything to eat. During this time, they will be placing two IVs, one in each arm, and taking blood from one arm while infusing a solution into the other arm. This test is required to measure the dosing of the pentostatin, the chemo drug he will get on Wednesday.

Tuesday: Picc Line: Another day of fasting all day. The only slot available was for 3:00, and Idan cannot eat anything after 7:00 a.m. We have to be at the hospital by 1:45 p.m., and will have to endure over an hour of waiting before the procedure starts.

Wednesday: Chemo infusion at 10:45 a.m.: 5 hours at the clinic during which Idan will receive a continuous infusion of liquids and the chemo will be infused a couple hours into the visit.

Thursday: Break.  Whew.

Friday: DLI: We’ll be admitted to the hospital first thing in the morning, Idan will have a couple hours of hydration and then the DLI. After he will be monitored until late in the day/evening. We should be discharged by nighttime.

The following week we’ll have a couple clinic appointments to check for GvH, but they don’t expect to see anything for a couple weeks. He’ll have his picc line removed some time that week (or potentially after the DLI friday, but we have to see how many pokes he has lined up for the following week before we decide when to remove it).

If anyone has any ideas of how to keep a starving two year old who is confined in a room and attached to IVs happy and entertained, we’re all ears.

Just when we thought everything was going Idan’s way, six days into a happy family trip to Seattle for Idan’s yearly follow up visit post-bone marrow transplant, we received the troubling news that his graft started slipping again.  A chart of all of his chimerisms to date showed us in black and white that, if we follow the same course, we are looking at second transplant or going back to Idan’s horrible disease.

Looking at the chart, Akiva and I were absolutely crestfallen.  The doctor presented us with a few options: either fiddle with the immune suppression and hope it is enough to turn the tide, do a second donor lymphocyte infusion (DLI), or throw our hands up and go into a second transplant.  We chose the second option, a DLI.

So next week, pending insurance clearance etc., we will be admitted into Seattle Children’s Hospital for four days for what can only be described as a mini-transplant.

Before he is admitted, Idan will have a Picc line placed in his arm, similar to the central line, which he will need for the remainder of his stay in Seattle.  On Day -2 of the hospital stay, Idan will receive one dose of a chemotherapy drug called Pentostatin, believed to help aid in engraftment and prevent graft versus host disease (GVHD).  On Day 0, Idan will get his DLI, which is an infusion of the same donor’s t-cells that they’ve been storing for us since his first DLI.  The hope is that the new t-cells act as reinforcements in the battle over Idan’s bone marrow taking place between his own cells and the donor cells he received during his transplant last year.  Idan will hopefully be discharged on Day 1 or Day 2 if all goes well.  There is a 50/50 chance that this will work.  There is a good chance he will get GVHD, which could occur in the liver, the skin, or the gut.  A little bit of GVHD can help bring in the graft, but too much could be deadly.  Idan will need to be in Seattle for approximately two weeks (if all goes well) of monitoring before we’ll be allowed to take him home to New York.

Time can only tell whether we’re making the right choice going forward with the DLI, but in many ways it feels like the only choice.  If we watch this graft slip away, as we have been doing for the last year, and do nothing, we are facing a much harder choice of doing a second transplant or reverting to Hyper IgM.  There is no guarantee this works, and it may do more harm than good, but it’s the best shot we can give Idan’s donor cells to keep fighting for a permanent home in Idan’s marrow.

The weight of these decisions that we’ve been making since Idan’s admission into the hospital over a year and a half ago has taken its toll.  Akiva and I spent several sleepless nights here wondering whether we are risking it all for what has proven to be a poor match, or whether this is our last chance to save the day by grabbing hold of these donor cells, giving them a good shake, and demanding they stay.  We agonize over the effect this will have over Idan.  He’s such a happy good natured little boy, but lurking beneath the surface is very real fear and anxiety that no two-year old should suffer from.

As we were processing the information about Idan’s graft, the doctor went on to answer some of our other lifestyle questions, and surprised us by saying Idan’s immune system, while in a precarious state for the foreseeable future, is actually functioning surprisingly well right now (yet another reason to do everything we can to hold on to this graft).  He is now free to go to the zoo, to a museum, to the aquarium, to have playdates with other healthy children his age, to give his baby cousin hugs and kisses without abandon.  Both Akiva and I released a collective sigh of relief, and I burst into tears for the second time during that meeting.

Although a dark cloud hung over us, the next day, when we took Idan to the zoo for the first time since his diagnosis over a year and a half ago, we felt pure joy.

It has been a busy few weeks. At this point in transplant things usually calm down, and doctors visits are more spaced out. For us, less tests and doctor visits would be welcome. Little Idan has had to go in at least twice a week for a visit and a blood test wile we are still trying to adjust his immunosuppressant dosing as well as get him off the steroids. He is on a very low dose of steroids, but his adrenal gland is still not functioning on its own. This means he tests very low when we check his cortisol levels and this can be dangerous if he gets sick or is in an accident. The doctors say it will take time for the adrenal gland to wake up and to be patient. Until then Idan will keep braving the needles and being an amazing sport during doctor visits.

One thing that is never really discussed with all the difficulties of transplant is the crazy amount of medications one is on for a long period of time. Besides the weekly infusions of antibodies, most of the months post-transplant Idan was taking between 9-11 different oral meds each day at 6-7 different time points throughout the day and night. Some days I counted 26 different oral syringes. Now Idan has been truly amazing with taking his meds, and we were lucky to not need a feeding tube during transplant, so he always took his meds orally. In the past month, we have been focused on cutting out the ones we can. We were able to cut out one of the two blood pressure meds he was on. Steroids are almost done, so that means we were able to cut out the antifungal he was on as well. Idan’s swallowing has much improved since we took him off the bottle (no pun intended) and he has been drinking exclusively with a straw for the past few weeks. This means we can cut out the liquid thickeners he was on as well as the acid reflex med. It feels really great to wake up on some mornings and only have to prepare 5 meds with his breakfast. Realizing that most parents probably dread giving their kids one medication, I think we are in a good place.

Our next big journey coming up is our return to Seattle for Idan’s one-year follow-up visit. We have started telling Idan that we are taking a trip to Seattle and will be going on a plane. Oddly, he keeps saying “home?” whenever we mention our trip to Seattle, so I guess he still has some memory of his time there. Nothing wrong with being a bi-coastal baby I guess.

It’s been an eventful week, to say the least.  As always, we take the good with the bad, and keep moving forward.

Steroid Wean + Cold = Bad Combination

The past few weeks we have been attempting to wean Idan off steroids.  There are several concerns during this process, the most obvious being the return of the GvHD, but a second less obvious but equally dangerous concern relating to the adrenal gland.  When a person is on steroids for a prolonged period of time, their adrenal gland essentially goes to sleep.  The adrenal gland is responsible for many things, from responding to and controlling inflammation, to cuing you to wake up in the morning.  Think of the phrase “adrenalin.”  It kicks in when you need it most, but most of the time you don’t.  As we were weaning off the steroids, it became clear that it would take a bit more time than anticipated for Idan’s adrenal gland to “wake up,” and remember what it is supposed to do.  Not much longer, but at least a few weeks.

Of course in the last few days of the wean, Idan caught a cold.  At the time, his adrenal gland was still a bit groggy, so-to-speak, and didn’t quite know how to “kick in” to fight the cold properly.  So we spent most of Saturday of last week (for those who have been following our Facebook page, that was our 5^th Anniversary) neurotically taking his temperature and blowing his nose every 2 minutes.  Idan’s temperature stayed just below the fever threshold, but just high enough to keep us panicked about a late night trip to the ER.  Idan tossed and turned and whimpered the entire night, a little furnace in between Akiva and I.  But some time between 4:30 and 6, he fell asleep finally.

Thankfully, his fever broke and it was back to playing as usual on Sunday, but this last cold has put us through the ringer.  Every time we fall into a nice routine, we get a kick to the shin, reminding us to be as vigilant as ever.

Chimerisms and Med Changes

Meanwhile, it feels like we’ve been at the doctor’s office every day for the past few weeks.  We are weaning Idan off two other medications as we wean the steroids, which translates to multiple blood draws and vital sign checks each week to make sure his body is handling the changes in dosing and medications well.

But the good – no great – news is that, after all this, Idan will be saying goodbye to three of the thirteen medications he is currently on.  This is a huge step in the right direction.

The latest chimerism results are also in, and – for the first time in 9 ½ months – we can take a short breather.  Idan’s chimerisms are generally stable, with his T-cells hanging on at 32%, and myeloid only dropping slightly to 10%.  For now, the doctors are very cautiously optimistic that the graft has a shot of hanging on for good.  Although we had hoped to start weaning Idan off the other two immune suppressants by now, Seattle would like to keep them on for another 8 weeks at their current dosage and check Idan’s chimerisms again at the end of September.  If the graft stays stable, we should be able to start the long wean off of each of them in October, just in time for our return visit for Idan’s one-year follow-up back west.