The Kennedy Krieger Independence Scales-Sickle Cell Disease: Executive Components of Transition Readiness

• Published in: Rehabilitation psychology 2017-08, Vol.62 (3), p.249-257
• Main Authors: Jones, Kelly E., Jacobson, Lisa A., Tarazi, Reem A.
• Format: Article
• Language: English
• Subjects: Activities of Daily Living; Adolescent; Anemia, Sickle Cell - physiopathology; Anemia, Sickle Cell - rehabilitation; Child; Cognitive Ability; Executive Function - physiology; Female; Human; Humans; Life Changes; Male; Self Care - methods; Self-Management; Sickle Cell Disease; Surveys and Questionnaires; Test Validity; Transition to Adult Care
• ISSN: 0090-5550; 1939-1544
• DOI: 10.1037/rep0000158

Purpose/Objective: Youth with sickle cell disease (SCD) are at an increased risk for executive dysfunction and simultaneously have increased self-management needs compared to typical adolescents. This unique combination may contribute, in part, to difficulties during transition to young adulthood. Current measures assessing adaptive skills do not assess the executive components (e.g., initiation, prospective memory) of SCD-related self-care tasks. Modeled on the KKIS-Spina Bifida (Jacobson et al., 2013), the Kennedy Krieger Independence Scales-Sickle Cell Disease (KKIS-SCD) is a new caregiver-report measure that assesses independence with self-management of SCD-specific demands as well as routine daily activities in adolescents with SCD. Research Method/Design: Thirty-three youth with SCD and their caregivers participated in this preliminary validation study examining the construct validity of the KKIS-SCD total and composite scores (Initiation of Routines, Prospective Memory) and exploring relationships of this measure with intellectual functioning, demographic factors, illness severity, and age. Results: The KKIS-SCD exhibited generally good internal consistency (Cronbach's alpha = .733 to .803), and demonstrated evidence for construct and discriminant validity when compared to an existing measure of adaptive function. The KKIS-SCD was significantly associated with caregiver-report of executive behaviors but not with intellectual functioning, demographic factors, illness severity, or age. Conclusions/Implications: Results provide preliminary support for the KKIS-SCD as a reliable and valid tool for the assessment of executive components of self-care management skills for youth with SCD. Identifying specific weaknesses in executive function related to self-care management skills might assist in guiding intervention and individualizing transition planning in these at-risk youth. Impact and Implications As the number of individuals with SCD living into young adulthood and beyond increases with medical advancements, so has the field's interest in these individuals' successful transition to adulthood. However, the impact of the neurocognitive weaknesses commonly observed in individuals with SCD on the acquisition of self-management competencies is not well understood. Individuals with SCD often present with executive dysfunction, which can negatively affect their competence and independence with initiation and completion of medical self-care and other tasks.