Bone Marrow Transplantation, Hemoglobinopathies, SCALLOP

Inclusion Criteria:

1. Patients with a related HLA genotype identical donor and hemoglobin SS, hemoglobin SC, or hemoglobin Sb0/+ and at least one of the following conditions:

1. previous central nervous system vaso-occlusive episode with or without residual neurologic findings, or has an abnormal transcranial doppler exam without neurologic findings, or abnormal MRI/MRA of the brain with or without 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;
5. any of the above symptoms in which the patient is not undergoing chronic transfusion therapy;
6. The patient is undergoing chronic transfusion therapy for symptoms other than those listed and which significantly interferes with normal life activities;
7. Failed hydroxyurea therapy;
8. Indication of pulmonary hypertension on 2 separate echocardiogram examinations;

9. Patients who plan to return to resource poor areas/countries.

   2. Between the ages of birth and 40 years.

   3. Women of childbearing potential must have a negative pregnancy test.

   EXCLUSION CRITERIA:

   1. Patient with biopsy proven chronic active hepatitis or fibrosis with portal bridging.
   2. Patient with SCD chronic lung disease > stage 3 (see Appendix 1). 3. Patient with severe renal dysfunction defined as creatinine clearance < 40 ml/min/1.73M^2.

   4. Patient with severe cardiac dysfunction defined as echocardiogram shortening fraction < 25% or NYHA class III or IV.

   5. Patient with HIV infection.

   6. Patient with unspecified chronic toxicity serious enough to detrimentally affect the patient's capacity to tolerate bone marrow transplantation.

   7. Patient or patient's guardian(s) unable to understand the nature and risks inherent in the BMT process.

   8 Pregnant/lactating women and those unwilling to use acceptable contraception will be excluded.

   9. Patient or patient's guardian who have not signed an informed consent.

   NOTE: Patients who would be excluded from the protocol strictly for laboratory abnormalities can be included at the investigator's discretion after approval by the CAGT Protocol Review Committee and the FDA reviewer.