That is how the attending physician of the Pediatric ICU described Logan a week ago. It has to be true.
I’ve started writing this update several times. But its been continually interrupted by the sheer volume of developments and new information coming at us, and the need to take a pause from this madness to properly celebrate Logan and Riley’s 9th birthday on May 13.
I will preface this post by saying that the recent developments have entered the realm of super complicated to follow, even for those with an advanced medical degree. I will try my best to lay this all out sequentially and coherently.
From the last post, you know Logan was in a tough spot. He had the Adeno virus brewing, a bacterial infection, was febrile, and had no immunity after a round of post-transplant chemotherapy. Then following a GI bleed, he was sent to the PICU. At that time, we prayed for Logan’s immune system to kick back in and for his white blood cells to recover and start fighting off the infection and virus. Now the situation seems profoundly more difficult, at least on the current assumptions everyone is operating under.
We mentioned that last week, in addition to these other symptoms, Logan also started to develop what turned into a very dramatic skin rash. At first we thought it was a heat rash from the fevers. Then the theory was that it was potentially a viral rash or a medication-induced rash from the antibiotics he is on. By Thursday of last week, the rash seemed hyper-inflamed and spread from his back to his abdomen and arms. He also had very high fevers. That was the day that Dr. Martinez—the director of the transplant program—returned to service and saw Logan for the first time in a while. Immediately upon seeing the rash, she posited whether the rash was potentially being caused by something far more serious . . . GVHD, or “graft vs. host disease.”
GVHD is a serious and rare complication that can occur when the immune cells within the transplanted organs (the donor cells) actually start to attack the cells of the host (Logan’s cells). This is essentially the reverse process of typical organ rejection, in which the primary concern is the host immune system recognizing as foreign the transplanted organ and trying to attack it. The affected cells with a GVHD reaction are typically those within the skin, liver and intestines. But in Logan’s case, given that the liver and small intestine were actually transplant from the donor, those organs would not be attacked by the donor’s own immune cells. GVHD most commonly occurs in patients that have received bone marrow transplants. But rarely, it has also been noted to occur in multivisceral transplant patients.
On Friday, the doctors took a biopsy of Logan’s skin, and that came back showing the presence of female chromosomes in the cells. Logan’s donor was a female. This is pretty definitive diagnostic evidence that the skin rash is in fact being caused by a GVHD response from the donor’s lymphocytes.
In addition to the rash flaring profoundly, Logan had really high fevers last Thursday. On Friday, the doctors said they had some “tough choices” to make, and decided to start Logan on a three-day course of high-dose steroids to tamp down the inflammation. This is essentially the course of treatment that would be done if he in fact had GVHD, which they weren’t certain of at the time. The steroids proved very effective at calming the fevers and rash and returning Logan to being comfortable. He has actually been in very good spirits the last few days and is out of the PICU and back on the Oncology floor with all of the nurses that he loves dearly.
Most alarming and pressing about this whole situation is that Logan’s immune system has still not come back on line, and we are now a month out from when he received chemotherapy. The strong suspicion is that the GVHD is contributing to also attacking Logan’s Bone Marrow and preventing his white blood cells from recovering. That is so scary. Needless to say, without an immune system Logan is at severe risk of infections and serious complications.
In the backdrop of all of this, the main Bone Marrow transplant doctor here at Columbia, Dr. Prakash Satwani, got involved and has been more or less directing Logan’s care for the past several days. He is starting a new medication aimed at stimulating bone marrow production in tandem with continuing immunosuppressive medications to try to inhibit the donor T-cells from continuing to attack Logan’s cells. This is all very rare and complicated, and not deeply studied or “evidence based,” as Dr. Satwani explained. They are acting on principles and anecdotal treatments that have worked in these unique and difficult situations in the past.
In the midst of the changing landscape, it has been hard for Becca and I to get a feel for expectations on when they might hope to see a positive response or when they would consider other potential treatments to stimulate bone marrow production. The ultimate lever to pull if Logan’s bone marrow is irrevocably suppressed would be to undergo a bone marrow transplant, but we’re still not sure if he is even a candidate for that extreme procedure in his current state. They will extract a bone marrow sample tomorrow, conduct testing, and that will likely give valuable information as to the extent of damage to Logan’s bone marrow production and potential actions to consider.
For those ardent followers of the blog that have been following along with Logan’s story since the early days of receiving chemotherapy in the fall, you probably remember that Logan’s platelet count was always an issue. Everyone thought this would be fixed by removing his enlarged spleen (which “sequesters” platelets) during the transplant surgery, but his platelets never rose to the level they would’ve expected even after doing that. This probably indicates that there was always an underlying issue with Logan’s bone marrow production not being robust, it just had not manifested in the dramatic ways we are seeing now following his counts being completely knocked out by chemotherapy and the onset of other GVHD symptoms.
We are doing our best to stay up to speed on all of this and make the best decisions. A shout out to the SDNY family for putting us in touch with a GVHD specialist at Sloan Kettering who offered to “put his head together” with Dr. Satwani to theorize potential treatments and when to sequence them. And an enormous debt of gratitude is owed to our nurses and other hospital staff who came out in force to celebrate the twins’ birthday on Tuesday. Despite all these challenges, Logan felt incredibly loved and appreciated. He was literally showered with gifts (Adriana), tended to like a prince (Michala), and even had a very special nurse come in on her day off to celebrate with Logan (Morgan), to name just a few of the innumerable acts of kindness and support from so many.
We are scared and mystified at the complexity of this situation, to name some of the many emotions we’re experiencing right now.