Aspirin Prophylaxis in Sickle Cell Disease
Condition: Sickle Cell Disease
MeSH terms: Anemia, Sickle Cell
Intervention: aspirin
Intervention description: 81 mg flavored chewable tablets. Subjects between the ages of 2.0 and 4.99 years will receive half of an 81 mg aspirin tablet each day. Those older than 5.0 years will receive a daily 81 mg aspirin tablet. The subject will receive the study drug for a period of 12 months.
Intervention MeSH terms: Aspirin
Phase: Phase 1
Study type: Interventional
Primary purpose: Treatment
Intervention model: Single Group Assignment
Masking: None (Open Label)
Enrollment: 11.0
Sex: All
Minimum age: 2 Years
Maximum age: 7 Years
Healthy volunteers: No
Keywords: sickle cell disease; hemoglobin SS disease; hemoglobin S Beta-0 Thalassemia; silent infarction in sickle cell disease; overt stroke in sickle cell disease; aspirin; transcranial Doppler ultrasound; neurocognitive testing

Summary:

      Neurologic complications secondary to cerebrovascular damage are prevalent in children with
      sickle cell disease. These patients experience both clinically overt cerebrovascular
      accidents and "silent infarctions" demonstrated by magnetic resonance imaging (MRI). They are
      also at risk for neurocognitive abnormalities.We hypothesize that daily, low-dose aspirin
      therapy will safely diminish the incidence and progression of cognitive deficits as well as
      the predisposition to overt and silent stroke in children with homozygous sickle cell disease
      (Hgb SS) or hemoglobin S Beta Zero Thalassemia (Hgb SB-0 Thal). In order to optimize the
      design of a future trial to test this hypothesis, we propose a pilot study to test the safety
      and tolerability of aspirin in young children with sickle cell disease.
    

Eligibility criteria:

        Inclusion Criteria:

          -  1. Children ages 2 - 7.99 years with a diagnosis of Hb SS or Hb Sß0 thalassemia,
             documented by hemoglobin electrophoresis and a complete blood count (CBC). 2.
             Influenza vaccination during the previous year or intended before the upcoming flu
             season. 3. Evidence of past infection with, or immunization against, varicella. 4.
             Negative pregnancy tests in girls of childbearing potential. 5. Informed consent
             signed by the parent or legal guardian.

        Exclusion Criteria:

          -  1. Prior history of overt stroke or cerebral hemorrhage. 2. Known history of allergic
             reaction to aspirin. 3. History of Reye's syndrome 4. Diagnosis of G-6-PD deficiency
             or von Willebrand's disease 5. Prolongation of the bleeding time or abnormal closure
             time, prothrombin time (PT), or partial thromboplastin time (PTT). 6. Active
             gastrointestinal (GI) bleeding or a history of GI bleeding. 7. Hepatic disease (AST or
             ALT >2x upper limit of normal, Direct bilirubin > 1.5 mg/dL) or renal disease
             (creatinine >2x upper limit of normal or 2 mg/dl, whichever is smaller). The exclusion
             criteria laboratory study ranges have been specified as greater than 2 times the upper
             limit of normal. 8. Hypertension (BP >95% for age and height). 9. Current treatment
             with chronic transfusion therapy. 10. Evidence of hemorrhage on MRI. 11. A mean TCD
             velocity > 200 cm/sec. in the middle cerebral artery (MCA) or internal carotid artery
             (ICA). 12. Evidence of Moyamoya syndrome on MRA. 13. Evidence of pregnancy. 14.
             Evidence of an inability to comply with testing procedures. 15. Inability to provide
             informed consent.