Transition planning for youth with sickle cell disease: embedding neuropsychological assessment into comprehensive...

Sickle cell disease (SCD) conveys a high risk of neuropsychological impairment due to chronic anemia, hypoxemia, cerebrovascular ischemia and stroke, in addition to causing pain and other biomedical complications. Few families accessed neuropsychological testing via the traditional referral system. In this paper, we describe a successful alternative strategy for addressing cognitive and behavioral needs of youth with SCD.

The Sickle Cell Disease Program for Learning and Neuropsychological Evaluation (SCD-PLANE) was embedded in 2007 into the SCD Comprehensive Clinic of Children's Hospitals and Clinics of Minnesota. SCD-PLANE includes brief neuropsychological testing, individualized recommendations, patient education and advocacy, interdisciplinary collaboration, and follow-through to assure patients' access to recommended resources. The purpose of SCD-PLANE is to identify and recommend interventions for cognitive, academic, emotional, behavioral, or social problems affecting child development and transition to adulthood.

Access to neuropsychological testing improved dramatically and is well accepted by SCD patients and families. Test results reveal increased risk of problems with executive function, speed of processing, graphomotor function, academic achievement, and parent-reported anxious/depressed symptoms, despite normal intelligence and language abilities. Patients' needs had not been previously identified or served by schools or other community agencies.

The SCD-PLANE, providing brief neuropsychological testing embedded within routine annual visits to SCD Comprehensive Clinic, is an effective approach to detect unmet developmental, educational, and behavioral needs within an underserved high-risk population. As youth move towards adulthood, considering cognitive, academic, and mental health status facilitates successful health care transitions.