And More Waiting...

Unfortunately the initial review of the bone marrow asperate does not give us a definitive diagnosis. They can see that the bone marrow is making fewer cells than in his last biopsy, meaning his bone marrow continues to fail, but our daily labs already indicated that. I am hoping that the actual marrow will provide more insight but that may very well not be the case. It seems he is stuck between a diagnosis of autoimmune (ie non-viral) hepitits associated aplastic anemia (HAAA) and hemophagocytic lymphohistiocytosis (HLH). And those two diagnoses have somewhat different treatment paths. Meeting with the liver specialist again tomorrow then devising a plan for the coming week or two. More talk of a possible liver biopsy later this week but I want to have a clear idea of the benefit before I give them the go ahead because a liver biopsy, even one done through his jugular vein due to his low platelet counts, carries risks. We are continuing to wait for the HLH genetic testing which has been expedited but it is still a long process. Since he continues to appear quite well and managing his internal distress, our doctors do not seem likely to move forward with a bone marrow transplant as would normally be the case with a child who has aplastic anemia and has an HLA matched sibling. They are considering other possible treatments, weighing the side effects, and we are trying to decide together how confident we need to be with any given diagnosis to start its accompanying treatment. So, we are waiting some more. Patience is the word yet again

In the meantime, Nathan is getting platelet transfusions every other day at this point with specially matched platelets (which don't deem to make a huge difference at this point over non matched platelets) and red blood cell transfusions every two weeks give or take a couple days. His body is eating up platelets at a rapid pace and that is specifically what is keeping us so close to the hospital. They are letting his platelet level get down to 10 or lower before transfusing him. If he only needed transfusions a couple times a week, we could do all of this from home.

His current medication regimen is meant to hold his over active immune system at bay and minimize the damage while they make a complete diagnosis. His meds include:

• Methylprednosolone - immunosuppressive steroid
• Tacrolimus - another immunosuppressive targeting T cells
• Ursodiol - a bile acid supplement to aid the gall bladder
• Lansoprozole - acid reflux medication due to the steroids
• Magnesium oxide supplementation
• Mouth care rinses as needed to control any oral bacterial growth. 

The above will be changed based on the official diagnosis, but the basic idea is to calm down his immune system. If he has HAAA, they will add a medication called ATG, or go straight to a bone marrow transplant. If the diagnosis is HLH, they will change him to a different steroid and add a chemotherapy drug called Etoposide. Or it could be something else entirely, they are not taking any possibility off the table at this point. 

On another note, Camryn woke this morning with a cold so they left for home a day earlier than expected. It is tremendously difficult for all of us to be apart yet it's also the best thing for us all... Jay gets more work done at home or can go into the office, Camryn gets a slice of normalcy and space to process everything at home, Nathan gets some much needed down time and I get to continue to hyperfocus on his needs for at least a few days each week.