Health Literacy - Neurocognitive Screening in Pediatric SCD

Pediatric sickle cell disease (SCD) is a blood disorder affecting approximately 70,000 to 100,000 individuals in the United States. Approximately 80% of individuals affected by SCD are African American, with approximately one out of every 346 individuals in this racial group diagnosed. Sickle cell disease is associated with broad neurocognitive impairment. Compared to their healthy peers, children with SCD demonstrate deficits in intellectual functioning, verbal abilities, visual-motor, and visual-spatial skills, short-term memory, executive functioning, attention and focus, and processing speed.

Neurocognitive deficits can result from primary disease-related factors including chronic anemia, hypoxemia, or cerebrovascular ischemia; as well as secondary factors such as missed school and a higher prevalence of socio-economic disadvantages in African American children. While direct neurological impact appears to be associated with neurocognitive performance, it was found that even children with no MRI abnormality evidence lower intellectual functioning skills than healthy controls, suggesting that "biological, socioeconomic, and environmental" factors are all implicated in neurocognitive impairment.

Because of these deficits, children with SCD are more likely to be retained in school relative to other African American students at the local and national levels. In addition, poorer cognitive and academic performance is associated with a decrease in patient-reported quality of life and general psychosocial functioning. In turn, greater levels of stress and negative mood are correlated with higher levels of reported pain, greater health care utilization, and reduced physical activity. It appears that sickle cell disease not only contributes to poorer neurocognitive and social-emotional well-being, but that declines in these domains can further exacerbate sickle cell disease symptoms.

Because of the deficits in neurocognitive functioning of children with SCD, routine screening is recommended to assess for cognitive deficits and provide appropriate recommendations. Unfortunately, prior research in SCD suggests limited follow-up on referrals and recommendations. For example, it was found that 25% of children with SCD evaluated by a neuropsychologist met the criteria for ADHD, but only 21% of the children subsequently diagnosed with ADHD were prescribed medication. In addition, it was found that children with SCD were not universally receiving appropriate school accommodations and interventions based on their cognitive deficits, despite the recommendation that school-based services be implemented immediately for children with SCD.

The disconnect between the cognitive, emotional/behavioral, and academic deficits are typically seen in children with SCD and the actual support received may be due in part to low health literacy and the provision of complex reports following testing. Health literacy has been found to be low in adolescents and adults with sickle cell disease. Another study found that caregivers of children with SCD had a low level of baseline disease-related knowledge but that this knowledge did improve with in-person education; however, this knowledge appeared to decline over time. Unfortunately, materials developed to improve knowledge often do not fit criteria for broad population health literacy. One study found that patient education materials developed for patients with sickle cell disease and their families ranged from an 8th to 12th grade reading level, with limited potential to translate written recommendations into concrete actions.

Broadly, research suggests the need for a change within the historical format of neurocognitive evaluation reports. It is noted as a need to make written reports provided to families more efficient, readable, and effective. Providing appropriate feedback to families appears essential, as it can improve health-related quality of life, coping, and understanding. An informal calculation of the current reports provided to families in our clinic's neurocognitive screening program suggested that reports averaged a reading level grade of 12.3 and approximately 17,500 words, standing in stark contrast to health literacy recommendations. Addressing the poor health literacy of neurocognitive feedback evaluations has two primary problems. First, these reports often have multiple intended audiences beyond caregivers, including teachers, school psychologists, physicians, and other professionals working with the child. These individuals may benefit from more detailed and technical information. In addition, some of the wording used in neurocognitive reports is standardized and can lose meaning or be technically incorrect if simplified. Thus, it appears essential to evaluate whether the addition of a health-literate passport card will help address disparities in understanding of results and patient/family follow-through after receiving a neurocognitive evaluation.

This study is innovative in being the first to our knowledge to investigate the use of a tangible tool, namely a "passport" style printed card, to address health literacy concerns by conveying the most important findings and recommended follow-up after completion of a neurocognitive evaluation. In other contexts, healthcare passport cards have demonstrated the ability to improve communication among families and other providers, make information and recommendations portable, and enhance continuity of care. However, the use of passport cards has not been evaluated as a potential solution to the problem plaguing neuropsychological evaluations, namely the provision of overly lengthy and complex reports following testing.