Abstract Objectives Sickle Cell Disease (SCD) is a rare, inherited blood disorder causing acute and chronic complications that affect physical, psychological, and social well-being. This study examines SCD patient characteristics using a biopsychosocial model integrating biological, quality-of-life (QoL), and social factors across life stages. Methods This observational study, conducted between November 2022 and July 2024 at a tertiary care hospital in Northern Italy, included patients aged 4–25 years with confirmed SCD. Participants underwent cognitive and QoL assessments; genotype, hospital visits, treatments, and socio-demographic data were collected. Wechsler scales assessed intellectual abilities; PROMIS questionnaires measured QoL. Analyses were performed with SPSS. Results Eighty-nine patients were enrolled. Profiles showed age-related clinical, cognitive, and psychosocial variations. Around 45% reported anxiety and depression, and 50% experienced social isolation and pain interference. Adolescents and young adults (12–25 years) exhibited higher psychological vulnerability—depression, pain interference, and fatigue—than younger groups (p.007). Cognitive performance was below normative means, particularly in executive functions (p=.039). Working memory and processing speed were most affected, declining with age. Significant differences emerged in Verbal Comprehension F(3,82)=6.473, p.001 and Perceptual Reasoning F(3,82)=2.987, p=.036, with poorer outcomes in older participants. Visuospatial skills were relatively preserved but declined in young adults. Conclusions Age-specific risk patterns highlight the need for developmentally tailored, multidisciplinary care. Early detection of cognitive and emotional vulnerabilities, together with preventive strategies, may reduce long-term complications and improve QoL. However, the cross-sectional design of the study limits causal inferences. Overall, findings support holistic care models addressing medical, cognitive, psychological, and social dimensions of SCD.
Fave et al. (Thu,) studied this question.