Methylphenidate to Address Attention and Executive Deficits Among Children With Sickle Cell Disease

Prior treatment

• Two or more prior episodes of priapism
• Stimulant medication within the past two weeks
• Previous adverse reaction to methylphenidate
• Currently prescribed another investigational medication.
• Currently prescribed any of the following:
  • Phenobarbital (anticonvulsant)
  • Phenytoin (anticonvulsant)
  • Primidone (anticonvulsant)
  • Warfarin (anticoagulant)
  • Antipsychotic medications
  • Selective Serotonin Reuptake Inhibitor (SSRI) medications
  • Tricyclic antidepressant (TCA) medications
  • Vasopressor medications

Other illness

• Uncontrolled seizures (seizure within the past 6 months)
• Cardiomyopathy or known congenital structural cardiac defects
• Stenotic valvular disease, left coronary artery stenosis, or history of myocarditis or pericarditis

Lab values

• History of heart arrhythmia including ventricular tachycardia, ventricular fibrillation, supraventricular tachycardia, QT prolongation or concomitant use of medications associated with QT prolongation
• Diagnosed with sickle cell-hereditary persistence of fetal hemoglobin (HbSHPFH), sickle cell homozygous C (CC), Hb S/N-Baltimore, or other rare genotypes that would preclude the participant from having the classic symptoms of sickle cell disease

Age

• Blood pressure \>95th percentile at the three most recent visits consecutively (i.e., \>95th percentile reading at all three of the most recent hospital visits to St. Jude).
  • If blood pressure is \> 95th %ile compared to age-norms on the day of the baseline visit, a repeat blood-pressure reading will be performed both electronically and manually to confirm findings.

Other

• Primary language other than English
• Score below the 2nd percentile on the Wechsler Abbreviated Scale of Intelligence (WASI)-2 intelligence quotient (IQ) test (ever)
• Severe sensory loss
• Inability or unwillingness of research participant or legal guardian/representative to give written informed consent.

• Diagnosed with SCD
• Enrolled on the institutional protocol: Sickle Cell Clinical Research Intervention Program (SCCRIP)
• Between the ages of 8.0 and 17.9 years
  • \*Included if performance measure, rating scale or diagnostic criteria met (within the past 2 years):
• \*Score at or below a Standard Score of 85 (16th percentile) on any 2 out of 4 performance measures:
  • NIH Toolbox Flanker
  • NIH Toolbox List Sorting
  • NIH Toolbox Dimensional Change Card Sort Test (DCST)
  • Wechsler Intelligence Scale for Children (WISC) -5/ Wechsler Adult Intelligence Scale (WAIS)-4 Digit Span Forward (DSF)
• \*Score at or above a T Score of 60 (84th percentile) on any 1 out of 2 parent rating scales:
  • BRIEF-2 Global Executive
  • BASC-3 Attention
• \*Have a documented diagnosis of attention deficit / hyperactivity disorder (any subtype)
• English as the primary language
• Research participant and one caregiver willing to participate and provide consent/assent according to institutional guidelines
• Negative pregnancy test in female participant of childbearing age