Month: April 2014

Day 186: A seed of hope.

            IMG_2075As you have probably caught on to by now, the state of Idan’s graft seems to be in flux every time we check in with you all.  Today is no exception.

The last time we checked the chimerism of Idan’s graft, his donor T-cells were stable but low, and his donor Myeloid cells were dropping precipitously.  We started a third immunosuppressant (MMF) in the hopes of stabilizing the graft so that the numbers did not drop any further.

We checked the chimerism again last week, and once again we are scratching our heads.  The Myeloid cells are still dropping, but at a slightly slower rate.  The fact that they are still dropping, though, means that they can still bottom out before the MMF has enough time to save them.  Without any donor Myeloid cells, there is little hope for the long-term stability of the graft.

That said, the donor T-cells, the cells we need replaced to cure Idan’s Hyper IgM, went up from 19% to 30%.  This increase took us completely by surprise as we expected the T-cells to continue hovering around the 19% mark at best, or maybe even start falling this month.  Instead, they jumped up a significant amount.  It may have been from the added MMF or even the DLI back in December, or it may have just been a fluke.

The difference between 19% and 30% may be the difference between whether Idan will suffer from secondary cancers during his adolescence or not.  If we could sustain his T-cells at 30%, we’d be ecstatic.  It obviously isn’t a slam-dunk-cure of the sort we hoped for back when we did the transplant, but it would likely save Idan from a second transplant and worse.  The problem is, without the Myeloid cells, we still don’t know whether the donor T-cells will have enough support to stick around for very long.  Based on the overall trend, it looks like the T-cells are steadily increasing, the tiny number of B-cells Idan has are relatively stable, and the Myeloid and NK cells are still dropping, albeit at a slower rate (see chart with trend lines below).  For that reason, we are cautious about calling the increase in T-cells “good news” until the other lines stabilize.

Chimerism

 

In other news, Idan’s latest recurrence of his GvHD seems to have resolved by slightly increasing the steroids.  We are glad we did not have to increase the dose too much, and hopefully we can start tapering this Friday.  In all likelihood though, we will be doing a dance with Idan’s GvHD and the steroid dose for a couple more months until we can safely stop the steroids altogether.

Also, because his counts have been stable, Idan will not be poked for a routine blood draw after the doctor examines him this week.  It has been seven months since Idan has gone an entire week without having blood drawn, let alone two, so we are very grateful for this news.  Hopefully the trend continues and we can reduce the blood draws to once every two weeks.

Day 179: Murphy’s Law? GVHD is back.

4.19.14             Less than 2 weeks left on Idan’s three-month course of steroids and his skin rash has returned.  Thankfully, we caught it early and it seems that a slightly increased dose is helping to clear it.  A flare-up of GVHD when tapering steroids for GVHD is not entirely uncommon, so it’s nothing to get too nervous about, but it is certainly a frustrating step back.  This flare-up will add at least a couple of weeks, if not months, of the steroids, a necessary evil.  More time on steroids means continued high risk for infection, suppression of Idan’s immune system and graft, and of course increased adverse side effects.  It’s additionally frustrating since we have been slowly tapering the steroids for the last two months now, hoping to come off of them by the third week in April, and now there’s no concrete end in sight.  Sadly, the GVHD does not indicate the strength of the graft, since you can have GVHD from even microscopic amounts of donor cells.

Tomorrow, we will test Idan’s chimerism levels to see if the third immunosuppressant (called MMF) helped stabilize Idan’s graft, which, as of last time, appeared to be in free-fall.  We expect to get the results in a couple of weeks as the blood is sent to Seattle for testing.  If the chimerism levels are stable, we’ll keep the MMF on board for a few more months at the least.  If the chimerism levels are still dropping, that probably means the MMF is not working, but we’ll wait and check again in a month before weaning him off of it.

On a lighter note, Idan participated in his first Passover seder this past week (last year he was fast asleep) and had such a great time.  He sang and asked the four questions — well, hummed it, but that counts! — and ran around room excitedly searching for the afikomen.  It was heartwarming to be surrounded by family this holiday.  For a moment there, life almost felt normal.  To all of our family and friends celebrating holidays this month, we wish you very happy, sweet, holidays.

 

One Year Later.

Puppet Show - Day 1 in the PICU

Puppet Show – Day 1 in the PICU

It’s been a year, believe it or not, since Idan was rushed to the emergency room.  The details of that day have not yet escaped our memory.  Sitting in the doctor’s office and receiving the news that Idan’s X-Ray was very concerning; waiting downstairs with the stroller for the ambulance; the small talk of the paramedics as they hooked Idan up to an oxygen tank; the nurses and doctors whisking in and out of the ER running test after test; being admitted to a room in the ICU upstairs for an indefinite stay; lying in the hospital bed with Idan as we spoke with the doctors; our family members raining down on the hospital like a hurricane, providing much needed support.  Although we hadn’t yet comprehended the extent to which our lives had changed that day, we understood the gravity of the situation.

So today, as we mark the anniversary of that dreadful day, we are flooded with emotions.  It’s hard to pick which is strongest.  On the one hand, we deeply mourn the loss of an entire year of Idan’s precious childhood to hospitals, isolation protocols, and, worst of all, a lot of pain.  On the other hand, we feel enormously grateful that his life was saved that day, that he is still with us and that his PCP was caught before it got the better of him.  We feel grateful that we have identified his immune deficiency so that we can treat it and protect him, and that we can prevent our future children from having the same disease.  But perhaps more than anything, we feel pride.  We are almost bursting with it, in fact.  Our son is strong, resilient, and incredibly brave.  We have asked more of him than any child should ever have to know of, and he has marched forward with such courage and determination – like a brave warrior going into battle.  No matter how frightening, no matter how painful, he has endured and thrived.

Week 2 of ICU In April. Just starting to get over his pneumonia

Week 2 of ICU In April. Just starting to get over his pneumonia

One Year Later. Walking to Riverside Park Today

One Year Later. Walking to Riverside Park Today

A good friend told us recently that, as a doctor, she always looks at the patient for answers when the future is uncertain.  Idan is remarkably strong, and even though he may be losing his graft, he is a survivor.  A year has passed since he was hooked up to a ventilator, and over the course of the last year, he spent a total of 60 days in the hospital, spent six months away from home, and endured weekly infusions and hundreds of tests and clinic visits.  Today, Idan is the size of a child twice his age, can run circles around his parents, is outgoing and social, is a creative artist, and even has his own repertoire of jokes he likes to tell.  If you passed him in the street, you would never know what he has been through, and you would never know he takes an average of 13 medications a day and has a severely compromised immune system.   So even though today is a sad day, filled with longing for what could have been and what should be, it is also a happy day.  Idan has somehow managed to chart his own course, and we could not be more proud of our little boy.

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