Prioritizing Mental Health in Sickle Cell Disease: Implementing Routine Psychological and Neurocognitive Assessments in a Comprehensive Sickle Cell Clinic
Authors: Franco Emily , Gonzalez Lisbel , McGirr-Crowley Allison , D'Angelo Christina , Jennings Ernestine , Gardner David , Kochhar Manpreet , Musula Lydia , Karkoska Kristine , McGann Patrick T. 
Published: December 2024

Introduction: Sickle cell disease (SCD) is an inherited hemoglobinopathy that can lead to significant physical and psychosocial challenges. Mental health concerns and neurocognitive deficits are highly prevalent but underrecognized in individuals with SCD. Cognitive deficits begin early and affect academic achievement, school retention, and job attainment. Unfortunately, formal neuropsychological testing is often a barrier for patients, as it requires several visits and includes up to 8 hours of testing. Thus, a clinic-administered assessment of executive function, in addition to well-validated measures of depression and anxiety, are appealing screening options. In this study, our objective is to describe the implementation of routine assessments of depression, anxiety, and neurocognitive status within routine outpatient sickle cell care.
Methods: We implemented in-clinic screening for depression and anxiety through use of the Patient Health Questionnaire-9 (PHQ-9) and the Generalized Anxiety Disorder-7 (GAD-7) scales. The scales were self-completed by patients ages 12 and older during routine comprehensive SCD clinic visits. Importantly, these assessments were administered without any pre-screening or referral to psychology or social work. Additionally, through a linked study requiring informed consent due to the extra time commitment, we performed a brief, neuropsychological assessment using the NIH Toolbox: Cognition Battery to assess cognitive functioning. This test is administered via tablet application, has been validated in individuals ages 3-85 years, and tests components of executive functioning and attention. The Toolbox results are standardized to that of a full-scale intelligence quotient with a mean 100 and standard deviation 15.
Results: A total of 74 patients &gt; 12 years of age were screened with the PHQ-9 and GAD-7. The mean age of screened patients was 22.9 ± 9.8 years. The sickle genotype distribution was reflective of our SCD population (54 HbSS, 13 HbSC, 7 other). Overall, of the 74 screened patients, 31 patients (42%) screened positive for at least mild depression (score &gt; 5), and 27 patients (36%) screened positive for at least mild anxiety (score &gt; 5). 21/74 patients (28%) screened positive for both anxiety and depression.
A total of 21 patients completed the NIH Toolbox with a median age of 10 years. 19/21 patients were pediatric patients under age 21. The assessment was demonstrated to be a feasible, in-clinic assessment of neurocognitive functioning. The mean composite cognition score was 90.4 ± 15.6. Abnormal results of the NIH Toolbox composite cognition scores were common but not universal, with 8/21 patients (38%) scoring lower than 1 standard deviation below the mean, but no patients scored lower than 2 standard deviations below the mean (considered intellectual disability).
Discussion/Conclusions: There is no question that mental health and neurocognitive concerns are highly prevalent among individuals with SCD. Thus, it is crucial that we identify practical and effective ways to screen for these concerns in all patients. In our pediatric and adult Lifespan Comprehensive Sickle Cell Clinic, each patient is seen by a multidisciplinary team, including hematology, psychology, social work, nursing, nutrition, research, and community health work. When patients screen positive for depression or anxiety or they demonstrate low composite scores on the NIH Toolbox Cognition Battery, our psychosocial team can connect them with resources or prioritize them for more formal assessment or management in real-time. If we depended upon referral by the provider due to mental health concerns or formal neurocognitive testing, many patients with real concerns would be missed. The PHQ-9 and GAD-7 are easy and quick to complete. The NIH toolbox cognition battery is more efficient than formal neurocognitive testing, but requires approximately 30-40 minutes to complete, which was the main limiting factor in having more patients complete the testing during a clinic appointment. Overall, in our comprehensive SCD clinic, patients expect and welcome seeing providers from multiple disciplines, and using our comprehensive care model to implement neuropsychological screening has proved to be an effective strategy to provide high-quality, patient-centered care with the ultimate goal of promoting overall mental and physical health.