Riding the Roller Coaster -- Four Weeks Post ATG

This past week has been a roller coaster of sorts. Nathan is increasingly bored from having been in isolation for nearly four weeks and we have been effectively just hanging around and waiting. It has been difficult to keep him busy and happy within the confines of these four walls and, if I'm being honest, I'm pretty bored too. Suffice it to say that life here was quite uneventful... until this past Friday when Nathan spiked a fever and his other vital signs showed signs of stress, plus he stayed in bed sleeping the entire day which is very unusual for him. Of course the doctors were concerned because he has no ability to fight infection so he was given a CT scan to check his sinuses and lungs (all clear) and blood cultures were drawn to see if any bacteria was present (also negative). It seems he was simply having a bit of a reaction to weaning off steroids, which I had initially suspected as the cause of his illness given his lack of any outward signs of infection. A stress dose of hydrocortisone seemed to perk him up quickly but he was also placed on a few broad spectrum antibiotics until the blood cultures were confirmed to be clear as well as a systemic anti fungal, thankfully without any major side effects to date. That was much action for one day and I joked that it was a nine bead day; he was quite excited to add all those new ones to his beads of courage. So my point is that I much prefer being bored. And here we are today, bored again, but I'm sure we will have many ups and downs to come.

Clinical Update

From a clinical perspective there is effectively no change in his aplastic anemia. His body doesn't seem to be showing any signs of responding to the ATG treatment and we are now four weeks since he received his last dose. Seems we are indeed headed down the path of a bone marrow transplant and thankfully Camryn and Nathan are perfect sibling matches which is a huge blessing that I talked about at length here and here. Lots of tests and evaluations will occur in the next 1.5 weeks and I am glad that our primary BMT doctor is the attending physician in the hospital HOT (i.e. hematology oncology & transplant) unit this week as I will feel most confident having him here to make all of the day to day decisions leading up the transplant decision and preparation. We continue to wait for some genetic testing but we did find out that he does not have the GATA2 genetic mutation that they suspected some time ago. I am not sure if I'm relieved or sad at this news, clearly something lead to this illness and it sure would be nice to be able to name it, but I can't dwell on that right now. 

Blood Counts
WBC: 0.3  (4.0-12.0)

Neutrophil Count: 141 (1400-1600)

RBC: 3.41 (4.0-5.3)

Hemoglobin: 9.9 (11.5-14.5)
He received a RBC transfusion three days ago.

Platelets: 22 (150-450)

He is getting platelet transfusions every 1-2 days and will get a transfusion again tomorrow as his counts will definitely be in the single digits once again.

Liver Inflammation

AST: 16 (23-58)

ALT: 98 (6-35)

Total Bili: 1.0 (0-1.1)

We likely won't continue to track these numbers as they are effectively normal now.

Medication Notes

While it looks like we are heading closer to a bone marrow transplant, our doctor wanted to try one more medication to see if he could stimulate white cell growth. Nathan is now on both G-CSF and GM-CSF and still his neutrophil and white cell counts are stagnant and showing no real signs of life. His Tacrolimus level has stayed more consistent now at a dose of 10ML. Given his reaction to the steroid weaning, his steroids have been increased again and we are starting the slow weaning process once more, hoping he doesn't bottom out again. He continues on one antibiotic to inhibit bacterial growth and has added Micafungin to inhibit fungal growth as well as medications for his elevated blood pressure (due to tacro) and acid reflux (due to steroids). I don't love the idea of treating an illness and then treating the side effects of the treatment, but it seems that is where we are right now. Bone marrow failure is serious to say the least and the treatment options seem to match the severity of the illness. I am quite certain that our post transplant regimen (assuming we do in fact go in that direction) will be quite intense. But it is all worth it to heal Nathan and get my sweet little guy back home again. There will be plenty of time to work on building his system back up again when this is all behind us. 

Much love to all of our friends and family!