Objectives 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.
Developmental interconnections in SCD: a bio-psycho-social model of health from childhood to young adulthood
Reggiani, Giulia;Boaro, Maria Paola;Mezzalira, Elisabetta;Colombatti, Raffaella;Arfè, Barbara
2026
Abstract
Objectives 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.
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