Sickle Cell Anemia and Stroke Haematological Disorders Sickle-cell disorders
Conditions
Interventions
Participants will be randomly assigned to one of two groups - the blood transfusion group or the observation group. The blood transfusion group will receive regular blood transfusion therapy. All part
Sponsors
National Institute of Neurological Disorders and Stroke (NINDS) (USA)
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: Inclusion criteria amended as of 12/03/2008: Inclusion Criteria for Screening: 1. Patient must have sickle cell anemia (hemoglobin SS or sickle B) as confirmed by the local institution by hemoglobin analysis after six months of age 2. Patient must be 5 through 14 years of age (i.e., must have attained their 5th, but not their 15th birthday when the screening consent is signed) 3. Informed consent with assent in accordance with the institutional policies Inclusion criteria provided at time of registration: 1. Patients ages 6-12 with sickle cell anemia (Hemoglobin SS or SB) 2. Patient must have a silent cerebral infarct documented by magnetic resonance imaging (MRI) of the brain as read by the neuroradiology panel
Exclusion criteria
Exclusion criteria: Exclusion criteria amended as of 12/03/2008: Exclusion Criteria for Screening at initial screening evaluation and after informed consent has been signed as well as during the interval up to randomization: 1. Patient with a history of a focal neurologic event lasting more than 24 hours with medical documentation or a history of prior overt stroke 2. Patient with other neurological problems, such as neurofibromatosis, lead poisoning, non-febrile seizure disorder, or tuberous sclerosis 3. Patient with HIV infection 4. Pregnancy or lactating females 5. Patient who received treatment with anti-sickling drugs or hydroxyurea within 3 months or anticipate receiving anti-sickling drugs or hydroxyurea during the course of the study 6. Patients on chronic blood transfusion therapy for other reasons 7. Patient judged not likely to be compliant by his/her hematologist and study coordinator based on previous compliance in clinic appointments and following advice. Specifically, families that have missed at least two appointments without notification within 12 months prior to the trial or parents/guardians of potential patients that have been reported for medical or education neglect are not eligible for this trial 8. Patient unable to receive blood transfusion because of alloimmunization 9. Patients who have or anticipate receiving permanent (or semi-permanent) metallic structures attached to their body. (e.g., braces on teeth, body piercings), which their physicians believe will interfere with the MRI of the head to assess the presence of silent cerebral infarct 10. Patients with any person living in the same household who have been randomly assigned in the SIT Trial Exclusion criteria provided at time of registration: 1. Patients with a history of overt stroke or focal neurological event lasting more than 24 hours 2. Patients with other neurological problems, such as neurofibromatosis, lead poisoning, or tuberous sclerosis 3. Patients with a transcranial doppler (TCD) study with a time-averaged mean velocity greater than 200 cm/sec verified by the study radiologist
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Not provided at time of registration | — |
Secondary
| Measure | Time frame |
|---|---|
| Not provided at time of registration | — |
Countries
United States of America
Outcome results
None listed