Abatacept for GVHD Prophylaxis After Hematopoietic Stem Cell Transplantation for Pediatric Sickle Cell Disease

"(Standard of Care) Premedication~Diphenhydramine: 1 mg/kg IV or PO q 8 hours (maximum=50 mg)"

"(Standard of Care) Premedication~Acetaminophen: 10-15 mg/kg PO q 6 hours (maximum=1000 mg)"

"(Standard of Care) Premedication~Methylprednisolone: 0.25-0.5 mg/kg IV q 6 hours"

"(Standard of Care) Premedication~Use as needed (PRN) Meperidine: 0.5 mg/kg IV q 4-6 hours (for rigors)"

"(Standard of Care) Immunosuppression~Alemtuzumab:~A test dose of alemtuzumab, 3 mg, should be administered IV over 2 hours the first day. If the test dose is tolerated, administration of three treatment doses should begin within 24 hours. The three treatment doses should be administered on consecutive days. 10 mg/m2 should be given the first day, 15 mg/m2 the second and 20 mg/m2 the third."

"(Standard of Care) Immunosuppression~Thymoglobulin: A 4 mg/kg dose of anti-thymocyte globulin should be administered in place of each alemtuzumab dose not completed."

"(Standard Conditioning Regimen)~Fludarabine should be administered 30 mg/m2 IV daily for five days. It should be infused over 30 to 60 minutes."

"(Standard Conditioning Regimen)~Melphalan should be administered 140 mg/m2 IV 3 days before marrow infusion. It should be infused within 60 minutes of preparation and over a maximum of 30 minutes. It should be infused immediately after the fludarabine infusion is complete."

"(Standard Conditioning Regimen)~Thiotepa should be administered 8 mg/kg IV 3 days before marrow infusion. It should be infused immediately after the fludarabine infusion is complete. The thiotepa should be infused over one hour."

"(Standard GVHD Prophylaxis) Calcineurin inhibitor~Cyclosporine: Administration will commence no later than at least 36 hours before marrow infusion; Cyclosporine doses will be adjusted to maintain a level of 150-300 ng/ml."

"(Standard GVHD Prophylaxis) Calcineurin inhibitor~Tacrolimus: Administration will commence no later than at least 36 hours before marrow infusion; Tacrolimus doses will be adjusted to maintain a level of 8-15 ng/ml."

"(Standard GVHD Prophylaxis)~Methotrexate will be given at a dose of 15 mg/m2 IV on day 1 and a dose of 10 mg/m2 IV on days 3, 6 and 11. Dosing shall be based on actual weight."

"(Investigational)~Abatacept will be administered intravenously at a dose of 10 mg/kg based on actual weight with a maximum of 750 mg. In cases where the calculated dose is less than or equal to 110% of a simple multiple of a 250 mg vial: 250 mg (275mg), 500 mg (550 mg) or 750 mg (825 mg), the dose may be rounded down to the nearest multiple. No rounding up of the abatacept dose is permitted."

"(Standard)~A procedure that infuses healthy cells, called stem cells, into your body to replace damaged or diseased bone marrow. A bone marrow transplant may also be used to treat certain types of cancer"

"(Standard GVHD Prophylaxis)~Calcineurin inhibitor (cyclosporine or tacrolimus or with permission of the sponsor: sirolimus or mycophenolate mofetil (MMF)) administration will commence no later than day -2 (at least 36 hours before the stem cell infusion); cyclosporine doses will be adjusted to maintain a level of 150-300 ng/ml. This range assumes monitoring by mass spectrometry. If an immunoassay is used instead, the equivalent range for that immunoassay should be used."

"(Standard GVHD Prophylaxis)~Calcineurin inhibitor (cyclosporine or tacrolimus or with permission of the sponsor: sirolimus or mycophenolate mofetil (MMF)) administration will commence no later than day -2 (at least 36 hours before the stem cell infusion); cyclosporine doses will be adjusted to maintain a level of 150-300 ng/ml. This range assumes monitoring by mass spectrometry. If an immunoassay is used instead, the equivalent range for that immunoassay should be used."