Haplo T-Cell Depleted Transplantation in High-Risk Sickle Cell Disease

Inclusion Criteria: * Homozygous Hemoglobin S Disease, or Hemoglobin S Beta0/+ thalassemia * Patients must demonstrate one or more of the following Sickle Cell Disease Complications 1. Clinically significant neurologic event (stroke) or any neurologic deficit lasting \>24 hours that is accompanied by an infarct on cerebral MRI 2. Minimum of two episodes of acute chest syndrome. 3. Recurrent painful events (at least 3 in the 2 years prior to enrollment). 4. Abnormal TCD study requiring starting on chronic transfusion therapy. 5. At least one silent infarct lesion on a MRI scan of the head. * A familial haploidentical donor without homozygous sickle cell disease * Adequate organ function (renal, liver, cardiac and pulmonary function) * Karnofsky or Lansky (age appropriate) Performance Score ≥50% * Liver biopsy is optional to assess for iron overload in chronically transfused patients. Exclusion Criteria: * Females who are pregnant or breast-feeding * SCD Patients with documented uncontrolled infection * SCD patients who have an unaffected HLA matched family donor willing to proceed to donation * Karnofsky/Lansky (age appropriate) Performance Score \<50% (hemiplegia alone secondary to a previous stroke is not an exclusion) * Demonstrated lack of compliance with medical care. * Clinically significant fibrosis or cirrhosis of the liver * Previously received a HSCT