Management of Sickle Cell Pain

"One of the major developmental tasks for all children is to master functioning in the school environment. This task can be difficult for children with chronic illnesses. Students must be present and engaged to develop positive relationships with same-aged peers and succeed academically. Children with SCD have been shown to have difficulties both academically and socially in school. SCD painful vaso-occlusive crises (VOCs), whether treated in the hospital or at home, are unpredictable, making it challenging for students to be present and fully engaged with their educational experience. Patients with SCD miss an average of 20-40 days of school per year. Approximately 35% of patients miss more than one month of school, a rate significantly higher than peers or sibling controls. Grade retention rates in adolescents have been reported to be as high as 40%, higher than the national average of 5-10% for unaffected students. In addition to pain, other factors related to SCD, such as chronic anemia, neurocognitive deficits, and stroke can impact school absenteeism and performance. Physical changes such as small stature and jaundice, combined with activity limitations that are often placed on students, can impair social interactions with peers. Children with SCD perceive challenges to managing their pain at school and barriers to creating a positive experience in school environment as early as elementary school. Academic challenges extend beyond racial and socioeconomic disparities, as children with SCD have disproportionately higher rates of grade retention and special education services compared with nonaffected children in the same school district. Neurologic complications, ranging from overt stroke to subtle neurocognitive changes, are among the most common complications of SCD. SCD is the most common cause of stroke in children. Patients can also have silent strokes and sickle cell related vasculopathy. Silent infarcts are associated with cognitive morbidity and increase the likelihood of future overt stroke. Patients with normal magnetic resonance imaging results and no history of stroke still may have evidence of neurocognitive decline related to underlying SCD. Full scale IQ typically declines by 1.5 points/year. Markers of disease severity such as low baseline hemoglobin have been shown"--