Saturday, January 16, 2016

Day +102

Day +100 is usually thought of as an exciting day, as that is when people can go home if they have not had too many complications. Unfortunately, it was just another day for us here in the hospital. We have had a stressful week with many changes in plans, dealing with the lack of answers for Raul's issues, and now some not so great news/answers.

Raul's engraftment this time showed his CD3 at 97% (which is slightly lower, but still good), and his CD15 down to 24%. This does not mean his transplant has failed, but it does mean his engraftment has dropped. With the transplants for EB, it is believed that even lower percentages of donor cells can be beneficial. We will check his numbers again in a month. If his CD15 was to drop to 0, with the CD3 still being higher, then a boost of cells may be beneficial to him.

Dr. Tolar stopped by last night to go over all this, and at that time the plan was that we would wean his CSA over 10-14 days instead of 8-10 weeks. However, they got some new test results back later last night, and now we will be keeping the CSA at a therapeutic dose. There is literature about going both ways: stopping the CSA vs. increasing suppression, so this change does not mean we are giving up on the donor cells!

Unfortunately, the new labs that came back last night gave Raul enough met criteria to be diagnosed with HLH (Hemophagocytic lymphohistiocytosis). This is an uncommon, life threatening, condition in which the body's immune system does not work properly. It can become overactive and end up destroying good cells and tissues in the body, which can lead to problems with the bone marrow and organs. Because of this Raul has persistent fevers, low platelets, low hemoglobin/hematocrit, very high ferritin, very high triglycerides, enlarged liver, enlarged spleen, and a high Soluble CD25 level. Due to the risks we will not be checking his bone marrow or spinal fluid, as it would not change the treatment. His kidney failure is a separate issue.
The treatment for this will include continuing the CSA, taking Decadron (a steroid), and taking a chemotherapy called Etoposide. The Decadron starts at a high dose, which is tapered over 8 weeks. After that he will get pulse doses every two weeks for three days. For the Etoposide he will get two doses each of the first two weeks, once weekly for six weeks, and then every two weeks. We do not know the exact length of the continuation therapy, but likely at least several months. There is no guarantee that the treatment will be successful, but the doctors are hopeful that it will be. Sometimes a bone marrow transplant is used to treat HLH, but Raul would not be a candidate for another transplant at this time due to his kidney failure.

Raul is beginning the steroid today, and will begin the chemo on Monday, as that needs to be dosed an hour before dialysis. I am hopeful that this steroid will perk him up like the last one we tried did, as it would be great to have him feeling a bit more like himself! They do not expect him to have too many side effects from the low doses of the chemo, thankfully. And since the chemo is a low dose, it should not kill off the rest of his donor cells, though I would assume that is always a possibility. 


  1. Caroline, can we send cards and/or packages to Raul? I so hope he begins to improve soon. Praying always.

    1. Thank you! Mail can be sent to:
      Raul Stan
      2450 Riverside Ave
      Room C4133 (BMT Floor)
      Minneapolis, MN

  2. Does Raul like interactive books or picture books more? Or both? Anything he would love to receive?