We've been trying to find the right dosing for Henley and her GCSF shots since we were in Minneapolis a month ago. This has meant lab work three times a week to check counts. It's been exhausting. Sadly, we haven't perfected it yet. Today's latest counts are still low. I give her shots every other day now at home and we are still dipping into severely neutropenic ranges. This makes her extremely vulnerable to infection.
In addition to issues with her white cells (specifically neutrophils), her third line of blood is failing. I think I have explained before, but for those unfamiliar I will give you a little overview. Our bodies have three lines of blood: white cells, red cells and platelets. All are made by our bone marrow. Transplant is typically recommended when two lines of blood are failing. For Henley this has been the case since she was diagnosed with SDS. She has required blood transfusions for her red cells and shots of GCSF for her white cells. Consistently now for over a month her platelets have been below normal range. Normal is 150 to 400 and today Henley's platelets are 96. Platelets can also be transfused, but they will wait until her numbers are lower before doing that. On a bright note her body has been maintaining her red cells for nearly 10 weeks!
These changes in her blood encourage us to do another bone marrow biopsy as soon as possible. That will take place on Wednesday, December 20th at the University of MN Masonic Children's Hospital in Minneapolis.
Schwachman Diamond Syndrome is a bone marrow failure syndrome, meaning her marrow is unable to do the job it was built to do. You can't group all SDS patients into the same exact group, as the syndrome has a large spectrum. We knew Henley's marrow was failing from the beginning, we just didn't really expect her platelets to begin failing as well.
Henley and Jess will head out Sunday. We have appointments in Sioux Falls, SD to see a new GI, then onto Minneapolis for appointments Tuesday and Wednesday.
We thank God that Henley has not shown signs of infection during this last month of trying to perfect medication. We ask that He will continue to protect her and that we will have safe traveling weather for the upcoming week.
