Andrew Heitzer, PhD

In medicine, it is easy to focus on physical symptoms, especially with a condition such as sickle cell disease, which is known for characteristic pain crises that occur due to sickle-shaped red blood cells sticking in the body’s vascular pathways. But the impacts of sickle cell disease extend far beyond the physical. My clinical research focuses on addressing the significant need to develop and implement interventions that address the consequences of neurocognitive difficulties in patients with sickle cell disease. 

Before we can start developing supports for patients, we must first identify any difficulties they may have with neurocognitive function. For those with sickle cell disease, this can be difficult due to the great variability in how these difficulties present in individuals. Previous research efforts identified hematologic, neurologic, demographic and genetic risk factors that contribute to neurocognitive difficulties, with many beginning early in life. There is a continued need to assess and identify risk factors for neurocognitive shifts in children with sickle cell disease so we can begin  interventions early that preserve neurocognitive function and quality of life.

Beyond the challenges of identifying neurocognitive risk factors in individuals with sickle cell disease, there are challenges in implementing the services that would best support these patients. While the American Society of Hematology 2020 Cerebrovascular guidelines proposed standards to address the significant variability in how neurocognitive difficulties are assessed and monitored across care centers, discrepancies and gaps in care still exist. My collaborative work assessed barriers to the implementation of neurocognitive assessment services for people with sickle cell disease and proposed methods to implement service across care centers. As a leader of the Sickle Cell Assessment of Neurocognitive Skills (SCANS) program at St. Jude,  I work to ensure the SCANS program continues to be an example of how neurocognitive monitoring can be implemented as an aspect of care for patients with sickle cell disease, with the aim of sharing outcomes and implementation strategies with other care centers.

There are no behavioral or pharmacological interventions that have established efficacy to improve neurocognitive performance among individuals with sickle cell disease. Prior attempts to address neurocognitive difficulties in patients with sickle cell disease have historically displayed poor feasibility and acceptability, limiting potential effectiveness. Addressing barriers to intervention adherence and increasing accessibility is critical to success. To address these limitations, our group has collaborated closely with patients, families and providers to understand the needs and preferences of patients with sickle cell disease. This information is used to adapt evidence-based interventions to increase access and improve engagement. Ongoing work is examining whether these adapted interventions are feasible and acceptable prior to testing their effectiveness. 

My clinical research efforts focus on the identification of barriers to interventional, proactive neurocognitive care for patients with sickle cell disease. By prioritizing this often-overlooked area of care for this inherited hematological condition, my work hopes to improve the care patients with sickle cell disease receive to prioritize the preservation of mental function and improve quality of life.