Inclusion:

• Patients with a haploidentical related HLA donor and hemoglobin SS, hemoglobin SC, or hemoglobin Sb0/+ thalassemia and at least one of the following conditions:

  1. previous central nervous system vaso-occlusive episode with or without residual neurologic findings;
  2. frequent painful vaso-occlusive episodes which significantly interfere with normal life activities and which necessitate chronic transfusion therapy;
  3. recurrent SCD chest syndrome events, which necessitate chronic transfusion therapy;
  4. severe anemia, which prevents acceptable quality of life and necessitates chronic transfusion therapy.
• Patients with a haploidentical related HLA donor and homozygous b0/+ thalassemia or severe variants of b0/+ thalassemia and require chronic transfusion therapy.
• Women of childbearing potential must have a negative pregnancy test.
• Between the ages of birth and 65 years.

Exclusion:

• HLA identical or 5/6 HLA matched sibling donor
• Biopsy proven chronic active hepatitis or portal fibrosis.
• SCD chronic lung disease > stage 3 Severe renal dysfunction defined as creatinine clearance <40 ml/min/1.73 M2.
• Severe cardiac dysfunction defined as shortening fraction <25%.
• HIV infection.
• Unspecified chronic toxicity serious enough to detrimentally affect the patient's capacity to tolerate Stem Cell Transplant.
• Patient or guardian(s) unable to understand the nature and risks inherent in the stem cell transplant process.
• Pregnant or lactating females and those unwilling to use acceptable contraception.